REVISTA BRASILEIRA DE NEUROLOGIA

Órgão Oficial do Instituto de Neurologia Deolindo Couto Vol. 56 • Nº 3 • JUL / AGO / SET - 2020

Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 1 ISSN 0101-8469 CODEN RBNEES ISSN eletrônico: 2447-2573 Acesso pelo LILACS-Express REVISTA BRASILEIRA DE NEUROLOGIA Órgão Oficial do Instituto de Neurologia Deolindo Couto

Editorial...... 4

Pulmonary capacity of patients with total traumatic injury of the brachial plexus submitted to neurotization with the phrenic nerve: a case series...... 5 Camilla Fernandes de Melo, Armèle Dornelas de Andrade, Fernando Henrique Souza, Helen Kerlen Bastos Fuzari, Juliana Fernandes, Silvya Nery Bernardino, Daniella Araújo de Oliveira

Conhecimento da população sobre Acidente Vascular Cerebral em Torres RS...... 11 Valmir Soares Machado, Lidiane de Medeiros Hahn, Maria Isabel Morgan Martins, Luiz Carlos Porcello Marrone

Guillain-Barré syndrome: advances and future perspectives...... 15 Rabello, Francisco de AP Cabral Júnior; Pupe, Camila Castelo Branco; Nascimento, Osvaldo JM.

Infarto da artéria de Percheron: relato de caso...... 21 João Carlos Cervelin, Matheus Muller da Rosa, Rafael Frizon, Denise Kriege

Creutzfeldt-Jakob disease: one hundred years of participation in the design of the transmissible spongiform 25 encephalopathies...... M. da Mota Gomes

Zeus’ pain: did he experience a thunderclap headache?...... 29 Giuliano da Paz Oliveira, Thiago Mendes Barbosa, Giordanno Santana Mazza, Raimundo Pereira da Silva-Neto

Guido da Vigevano and the first human neuroanatomical figures...... 31 Eliasz Engelhardt REVISTA BRASILEIRA DE NEUROLOGIA Órgão Oficial do Instituto de Neurologia Deolindo Couto

Fundador: CIP-Brasil - Catalogação na Fonte Deolindo Augusto de Nunes Couto Sindicato Nacional dos Editores de Livro - RJ Ex-Editor-chefe: Clóvis Oliveira R349 Revista Brasileira de Neurologia/Instituto de Neurologia Editores-chefes: Deolindo Couto, Universidade Federal do Rio de Janeiro Eliasz Engelhardt [vol 1 - nº 1 (1949)] Marleide da Mota Gomes Continuação do: Editores associados: Jornal Brasileiro de Neurologia [vol 19 - nº 6 (1983)] Abelardo Queiroz Campo Araújo Trimestral a partir do vol 37 - nº 1 (2001) [descrição Marcos Raimundo Gomes de Freitas baseada no vol 34 - nº 6 (1998)] Osvaldo José Moreira do Nascimento 1. Neurologia - Periódicos brasileiros. Comissão científica: I. Universidade Federal do Rio de Janeiro. Instituto Alexandra Prufer Araújo de Neurologia Deolindo Ana Cláudia Celestino B. Leite Couto. II. Título: Jornal Brasileiro de Neurologia. Andrea Camaz Deslandes Claudia Marcia Nacif Drummond 98-1980 CDD 616.8 CDU 616.8 Claudia Domingues Vargas Clynton Lourenço Corrêa Denise Madeira Moreira Jerson Laks ISSN 0101-8469 José Mauro Braz de Lima CODEN RBNEE José Vicente Pereira Martins Leila Chimelli Luíz Antônio Alves Duro Marco Oliveira Py Marzia Puccioni-Sohler Envie seu artigo científico para publicação na Péricles Maranhão Filho Revista Brasileira de Neurologia somente ON LINE: Regina Maria Papais Alvarenga Sergio Augusto Pereira Novis RBN: http://revistas.ufrj.br/index.php/rbn Bibliotecárias: Luzinete Alvarenga Núbia Tavarez Gomes

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Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 3 EDITORIAL

This issue of Revista Brasileira de Neurologia is dedicated to Prof. Raimundo Edson de AraúJo Leitão in his birth centenary. On December 14, 1950, he graduated from the National Faculty of Medicine of the University of Brazil having started his career at the Rheumatology and Physiotherapy Service of Santa Casa de Misericórdia do RJ, directed by Prof. Waldemar Bianchi. In August 1954, Prof. Araújo Leitão was part of the group led by Waldemar Bianchi for the foundation of the Sociedade Brasileira de Fisioterapia, name changed in 1959 to Associação Brasileira de Medicina Física e Reabilitação Prof. Araújo Leitão participated in the development of professional rehabilitation at Instituto Nacional de Previdência Social - National Institute of Society Security ( INPS), in the 1960s. The golden period of professional rehabilitation by physicians in Brazil occurred between 1970 and 1982. At that time, professionals in rehabilitation centers lived in a dynamic work environment for the professional rehabilitation of the patient. However, at that time, there was already a shortage of physiatrists in Brazil. From 1959 to 1990, Prof. Araújo Leitão worked at the Neurology Institute, and, from 1970, he became head of the Rehabilitation Service of the Institution created by Prof. Deolindo Couto. This Service had an area of more than 300 m2 with the inclusion of a doctor's office, therapeutic academy, sectors of electrotherapy and thermotherapy, hydrotherapy (Hubbard tank, and tourbillon devices), and occupational therapy. Another important achievement of Prof. Araújo Leitão was the creation of the Academia Brasileira de Medicina de Reabilitação - Brazilian Academy of Medicine and Rehabilitation (ABMR), founded on January 26, 1972, at the headquarters of the Sociedade de Medicina do RJ. It was born under the influence of academic physiatrists Odir Mendes Pereira, Hilton Baptista and Araújo Leitão, its first president. The academic Araújo Leitão took over the coordination of the physiotherapy discipline at FM-UFRJ, after the retirement of Prof. Camillo Abud, and he also became the postgraduate coordinator for the Sector. On the occasion of his birthday celebrations, on July 28, 2020, Prof. Araújo Leitão was greatly honored by ABMR, where he is his honor president, and the National Academy of Medicine, where he is an honorary member. This is an expression of the importance of their commitment to strengthening the rehabilitation of patients with disabilities and, also, of the affection and respect of his disciples and confreres.

Editors Rev Bras Neurol 56(3):5-10, 2020. Pulmonary capacity of patients with total traumatic injury of the brachial plexus submitted to neurotization with the phrenic nerve: a case series.

Capacidade pulmonar de pacientes com lesão traumática total do plexo braquial submetidos à neurotização com o nervo frênico: uma série de casos.

Camilla Fernandes de Melo1, Armèle Dornelas de Andrade1, Fernando Henrique Souza2, Helen Kerlen Bastos Fuzari1,3, Juliana Fernandes1, Silvya Nery Bernardino4, Daniella Araújo de Oliveira1,3

ABSTRACT RESUMO

Total traumatic injury often requires surgical intervention such as A lesão traumática total freqüentemente requer intervenção neurotization using the phrenic nerve with the aim to recover the cirúrgica, como neurotização usando o nervo frênico, com o elbow function. However, its repercussions on the respiratory objetivo de recuperar a função do cotovelo. No entanto, suas kinematics are unknown. Objective: To evaluate the ribcage repercussões na cinemática respiratória são desconhecidas. volume in tricompartments division, kinematics of Duty Cycle, Objetivo: Avaliar o volume da caixa torácica na divisão dos and shortening velocity of the respiratory muscles after nerve tricompartimentos, a cinemática do Duty Cycle e a velocidade de phrenic transfer. Methods: Five participants (4 male), aged 18 to encurtamento dos músculos respiratórios após a transferência 40 years old (32±2), diagnosed with total brachial plexus injury do nervo frênico. Métodos: Cinco participantes (4 do sexo and with nerve phrenic transfer. The optoelectronic masculino), com idade entre 18 e 40 anos (32 ± 2), com plethysmography (OEP) was the instrument to evaluate volume diagnóstico de lesão total do plexo braquial e transferência do in quiet breathing (QB), inspiratory capacity (IC) and vital nervo frênico. A pletismografia optoeletrônica (OEP) foi o capacity (VC) of the rib cage in its tricompartments division instrumento para avaliar o volume na respiração silenciosa (QB), (pulmonary rib cage, abdominal rib cage and abdomen rib cage) a capacidade inspiratória (IC) e a capacidade vital (VC) da caixa and in each hemithorax, as well as the shortening velocity of the torácica em sua divisão tricompartimental (caixa torácica respiratory muscles, and respiratory rate. Assessments occurred pulmonar, caixa torácica abdominal e caixa torácica do 30 days prior and 30 days after surgery. Results: There was a abdômen ) e em cada hemitórax, bem como a velocidade de decrease in the total compartmental distribution in QB with encurtamento dos músculos respiratórios e a frequência statistical difference only in the abdominal compartment (p <0.05). respiratória. As avaliações ocorreram 30 dias antes e 30 dias Four patients showed a reduction in the shortening speed of the após a cirurgia. Resultados: Houve diminuição da distribuição left diaphragm muscle. It was not possible to perform a group compartimental total no QB com diferença estatística apenas no analysis of respiratory kinematics and volumes in CV, IC due to compartimento abdominal (p <0,05). Quatro pacientes the variation found in each patient analyzed. Conclusion: There apresentaram redução da velocidade de encurtamento do was a reduction in volume in the rib cage as well as a change in músculo diafragma esquerdo. Não foi possível realizar uma the speed of shortening of the respiratory muscles after the análise de grupo da cinemática respiratória e dos volumes em transfer of the phrenic nerve one month after surgery. CV, IC devido à variação encontrada em cada paciente analisado. Conclusão: Houve redução do volume da caixa Key words: Plethysmography, Brachial plexus, Phrenic nerve, torácica e também alteração da velocidade de encurtamento dos Nerve phrenic transfer músculos respiratórios após a transferência do nervo frênico um mês após a cirurgia.

Palavras-chave: Pletismografia, Plexo braquial, Nervo frênico, Transferência de nervo frênico

______1-Department of Physical Therapy, Universidade Federal de Pernambuco (UFPE), Recife/PE, Brazil, 2-Department of Peripheral Nerves, Hospital da Restauração, Recife/PE, Brazil, 3-Postgraduate Program in Neuropsychiatry and Behavioral Sciences (POSNEURO), Universidade Federal de Pernambuco (UFPE), Recife/PE, Brazil, 4-Department of Electroneuromyography, Hospital Getúlio Vargas, Recife/PE, Brazil

Corresponding author: Daniella Araújo de Oliveira, Av. Jornalista Aníbal Fernandes, 173 - Cidade Universitária, Recife - PE, 50740-560. E-mail: [email protected] .

Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 5 de Melo et al. Neurotization with phrenic nerve. Research Committee with Human Beings at UFPE under the CAEE number: 59986616.9.0000.5208. INTRODUCTION

Among the traumatic plexopathies, total brachial plexus Plestismography procedure injury presents the worst prognostic due to total denervation of the muscles of the ipsilateral upper arm leading to loss of Optoelectronic plethysmography (OEP) (ETS) (BTS capacity and performance in activities of daily living.1 Bioengenireeirng, Italy) was used to evaluate the total chest Two types of donors exist, the extra-plexal nerves such as cavity volumes, thoracic cavity volume per compartment phrenic nerve, intercostal nerve and accessory, and the intra- (pulmonary, thoracic and abdominal) and volumes in both plexal nerves including proximal stump of the roots or collateral hemithorax and the kinematic variables in the inspiratory motor branches of preserved brachial plexus nerves. In TBPI, an capacity, vital capacity and breathing at rest. Eighty-nine option of treatment is the transfer of nerve phrenic (C3-C5) to reflective, hemispheric markers, 6 mm in diameter, with musculocutaneous nerve (C5-C7)2,3, this technique has been hypoallergenic tape were fixed on the thorax of the volunteers, used since 1970 to restore the function of the biceps brachii 43 on the anterior part, 37 on the posterior part and 10 on the muscle in order to improve elbow flexion1,4. sides of their thorax, from the clavicles to the anterior superior The main reasons to choose the phrenic nerve as a donor iliac spines, along pre-defined horizontal and vertical lines11 include easy anatomic localization, large number of motor For the data acquisition, subjects were in a seated neurons (average 800), and high frequency and amplitude position, with their feet flat on the floor, knees and hips 90º conducting nerve impulses.8,9 However, the time frame between flexion, erect spine, arms supported, elbows flexed at 90º and the injury date and the surgical intervention (should not exceed a forearms in neutral position. From this arrangement, subjects year) as well as other cardiorespiratory issues, once this nerve is were asked to breathe calmly for three minutes, while avoiding the main responsible for innervate diaphragm muscle, should be speaking or changing posture in order to record the resting considered.5,7 volumes fromwhich only the most stable minute was used for Understanding the importance of the phrenic nerve in evaluation. A further three minutes of vital capacity (VC) respiratory function, we believe that the evaluation of respiratory maneuver were also recorded and the highest value out of the function before and after nerve transfer surgery with this nerve is three was used for analysis. relevant2,3,7. Although promising, knowledge regarding the Images captured by the eight cameras at a frequency of repercussions of this surgical technique on the patient’s 60 Hz and generated by the infrared light reflections of the 89 pulmonary volumes and capacity are limited,6,7 markers were then triangulated through the Gaussian theorem, In this scenario, the use of optoelectronic allowing the visualization of the rib cage in its three plethysmography (OEP) as a non-invasive tool capable of compartments: pulmonary rib cage (Vrcp) , abdominal thoracic accessing each phase of breathing in a three-dimensional way cavity (Vrcab) and abdomen (Vab), as well as in its entirety (i.e. and thereby detecting changes in the volumes of the total rib total chest wall, Vtcw). Each compartment was evaluated in cage or separated by compartments, as well as in each terms of volume variation throughout the recorded respiratory hemithorax, offers an additional advantage to study the impact cycle, in an absolute way, and divided between the right and left of neurotization on the respiratory system. OEP has been used hemithorax. Inspiratory capacity (IC) and vital capacity (VC) for analysis in several populations, such as post-stroke patients were obtained following the same methodology. Variables such and in different clinical conditions10, however there is a lack of as inspiratory time (Tinsp) and total cycle time (Ttot) were studies targeting patients who have suffered from TBPI. calculated to obtain Duty Cycle (Tinsp / Ttot). Therefore, this study aims to evaluate the distribution of From the ratio of Vrca and Vab by inspiratory time, the pulmonary volumes and capacities in patients who had total shortening velocities of the diaphragm and the abdominal brachial plexus injury and underwent phrenic nerve muscles were obtained, respectively. To obtain the variable neurotization. shortening speed the inspiratory muscles (external intercostal muscles, ribcage muscles and diaphragm), the calculation was made based on the ratio between Vrcp variable and inspiratory METHODS time12. The OEP was performed in the preoperative phase (within the last preoperative month) and in the postoperative A series of cases were carried out from August 2016 to phase, respecting the period between 15 and 30 days after the August 2017 including five patients (4 men), aged between 18 procedure, which was established by the coincidence according and 40 years (32.6 ± 2.48). Patients were referred from the to the postoperative reassessment by the surgical team. “Peripheral Nerves Ambulatory of Hospital da Restauração, Recife, PE” with a clinical diagnosis of TBPI confirmed by Statistical Analysis complementary exams of electroneuromyography (EMG) and Magnetic resonance Imaging (MRI). The data were tabulated using Microsoft Excel 2016 Volunteers with TBPI who underwent neurotization with software and then analyzed using SPSS Statistics® software the phrenic nerve were included in this study. Patients had all version 22.0. For the descriptive analysis for the five study roots avulsed (four on the left side), using the graft for participants, the absolute values of each dependent variable were transferring the phrenic nerve in their surgical procedure. The used, as well as their percentage variation calculated using the mean time between trauma and surgery ranged from 5 to 8 formula (Post-surgical volume - Pre-surgical volume / Pre- months and the phrenic nerve was used completely in the surgery volume x 100). The data are expressed in absolute cervical region (Table 1). Exclusion criteria included any chest values and the volumes of the rib cage both in quiet breathing trauma (rib fracture, pneumothorax, etc.) and/or diagnosis of and IC and VC are also expressed as a percentage of pre-existing lung disease(s). All assessments were performed at contribution to the Vtcw. Wilcoxon's non-parametric test was the “Laboratory of Cardiopulmonary Physiotherapy (LACAP)” used to analyze the comparison between the pre- and post- and at the “Learning and Motor Control Laboratory (LACOM)”, operative periods of lung volumes in total lung box, as well as in the Department of Physiotherapy at the Federal University of separately between the right and left hemibodies. A 95% Pernambuco (UFPE). The study was approved by the Ethics and confidence level and a p-value <0.05 were used for all tests. RESULTS muscle decreased its shortening speed in the postoperative period. Table 1 shows the characterization of the studied sample. Therefore, we may suggest that after neurotization, patients No patient had postoperative complications during the decrease the speed of contraction of the diaphragm causing a evaluation period. Regarding the compartmental distribution in compensatory shortening of the abdominal muscles and, the pulmonary rib cage, although there was a reduction in lung consequently, decreasing the volume distribution in this volumes after surgery, only the decrease in Vab was statistically compartment. This fact can be explained by the necessary significant (Before: 423.00 ± 314.73 ml; After: 187.00 ± 148.68 antagonist nature between the diaphragm and the abdominal ml, p-value <0, 05) (Figure 1). Additionally, it was observed that muscles, which allows the chest wall to expand while keeping this decrease in volume of the abdominal compartment occurred the larger volume in the abdominal rib cage13. both on the right side (Before: 114.89 ± 95.33 ml; After 76.01 ± Patient 1 had thoracoabdominal asynchrony, that is, during 84.43 ml, p-value <0.05) and on the left side (Before: 124.70 ± inspiration there was a gain in volume in the right pulmonary rib 103.91 ml; After : 82.62 ± 88.80 ml, p-value <0.05) (Figure 2). cage and a reduction in right abdominal volume. On exhalation, the volume of the right pulmonary rib cage decreases and the right abdominal volume increases. Such asynchrony may be related to a significant decrease in the speed of shortening of the abdominal muscles, which leads to an increase in the speed of shortening of the inspiratory muscles, as seen in Figures 3 and 4.

Figure 1. Quiet Breathing (QB) compartmental distribution in the three pulmonary compartments before and after the surgical procedure. * p-value <0.05

Figure 3. Thoracoabdominal asynchrony in patient 1 during quiet breathing. It is noted that during an inspiration, represented by green arrows, there is a volume gain of the right lung chest and a reduction of right abdominal volume. This same asynchrony is seen during expiration (red arrows).

Figure 2. Compartmental distribution of volumes in Quiet Breathing (QB) on the right and left sides before and after the surgical procedure

Checking the behavior of the respiratory musculature in relation to its shortening speed, it was observed that the left diaphragm muscle decreased its shortening speed in three patients with left plexus injury (patients 3, 4 and 5) and one with Figure 4. Shortening rate of respiratory muscles (seconds) Pre op.: preoperative; right plexus injury (patient 2). There was a reduction in the Post op: post-operative; Inspiratory M R: Right Inspiratory Muscles; Inspiratory M L: Left Inspiratory Muscles; Diaphragm M R: Right Diaphragm Muscle; speed of shortening of the abdominal muscles in patients 1, 3, 4 Diaphragm M L: Left Diaphragm Muscle; Abdominal M R: Right Abdominal and 5, all with lesions of the left plexus (Figure 4). Muscles; Abdominal M L: Left Abdominal Muscles Analysis of the respiratory kinematics and volumes in vital capacity (VC) and inspiratory capacity (IC) was not When the side of the lesion is analyzed, the patient with a possible due to individual variability within the sample (Table 3). total brachial plexus lesion on the right and neurotization of the phrenic nerve on the right may demonstrate greater respiratory repercussions. These repercussions were demonstrated by the DISCUSSION patient in the respiratory rate (RR), which was approximately 12 bpm before and 17 bpm after the procedure. This can be The aim of the present study was to evaluate the distribution interpreted as a compensation to maintain ventilation of lung volumes and capacity in patients with total brachial considering the RR is increased to guarantee greater uptake of plexus injury submitted to neurotization with the phrenic nerve. O2 (Table 2)14. In addition to this change, the patient presented a In view of the results presented, it was observed that the reduction in volume in all compartments and obtained an abdominal compartment decreased in volume and the diaphragm increase in the shortening speed of the inspiratory muscles; in other words, with the reduction of the total lung volume, an Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 7 increase the RR is observed consequently using more inspiratory can be performed early and preventively in this population so muscles to guarantee ventilation. Thus, it is likely that the fact that there are no possible long-term clinical repercussions. that the right hemicupula of the diaphragm rests on the upper hepatic surface forming a physiological mechanical barrier, makes this hemithorax the most impaired in phrenic nerve CONCLUSION 15 transfers . In the assessments of respiratory capacity after about A study with 165 patients with brachial plexus injury who 30 days after the surgical procedure, it was possible to observe underwent nerve transfer surgery used radiographs to determine possible changes in the volumes of the rib cage in individuals a diaphragmatic height index. In this study it was found that the with total brachial plexus injury submitted to neurotization with right side of the hemicupula is higher than the left side. the phrenic nerve. A decrease in the total abdominal volume and Additionally, a non-significant difference in the height of the on the left side, in addition to changes in the diaphragmatic diaphragmatic hemicupula in patients who underwent phrenic shortening speed on the left side were observed. In some patients, nerve surgery was found when compared to the control group14. it was possible to observe greater repercussions in terms of When we analyze the results presented by volume and changes in respiratory kinematics. Pornrattanamaneewong and colleagues regarding the difference This study proved to be a pioneer by using OEP which is considered a more reliable instrument to evaluate the variation of in diaphragmatic height with the results presented by our study the three-compartment volume of the rib cage when compared to with OEP, we can infer that there is a visible anatomical other methods. difference by radiography in patients after phrenic nerve surgery, as well as a change in the distribution of volumes by CONFLICT OF INTEREST compartment as detected by OEP. Radiography is a simple, low- The authors declare that there is no conflict of interest. cost method that uses ionizing radiation to capture images, to obtain anatomical differences in these patients; however, it is FUNDING STATEMENT subjective to the evaluator's interpretation and the patient's The Pro-Rectory for Research and Graduate Studies at the position at the time of capture. On the other hand, despite OEP’s Federal University of Pernambuco (PROPESQ-UFPE), high cost, this method is a more sensitive tool to detect minimal Institutional Program for Scientific Initiation Scholarships changes in volume within the three-compartments in the lung, (PIBIC) and the coordination for the improvement of higher education (CAPES). which allows us to infer possible repercussions and compensations acquired by patients after injury. There was a loss in volume values in total vital capacity in patients 2 (13%), 3 (5%) and 5 (21%), while patients 1 and 4 REFERENCES were able to compensate with other compartments and even 1. Siqueira MG, Martins RS. Surgical treatment of adult traumatic brachial plexus increased their total vital capacity (32 % of patient increase 1 injuries: An overview. Arq Neuropsiquiatr 2011;69(3):528-535. and 9% patient 4).This was probably due to the fact that even 2. Rezende M, Silva G, Paula E, Mattar Junior R, Camargo O. What has changed though all patients present a loss in abdominal volume varying in brachial plexus surgery? Clinics2013;68(3):411–8. between 5% and 202%, patients 1 and 4 were able to 3. Ferrante MA. Brachial plexopathies: Classification, causes, and consequences. compensate more effectively from other compartments thus, Vol. 30, Muscle and Nerve. 2004. p. 547–68. 4. Gu YD,; Meng-kun Ma. Use of the Phrenic Nerve for Brachial Plexus adding volume to the total vital capacity. Reconstruction. Clinical Orthopaedics and Related Research 1996;323:119- The inspiratory capacity analysis showed that patients 121. 1,2,3 and 5 had a loss of volume in the inspiratory capacity in all 5. Socolovsky M, di Masi G, Bonilla G, Paez MD, Robla J, Cabrera CC. The compartments with patient 2 obtaining a 2% increase in the phrenic nerve as a donor for brachial plexus injuries: is it safe and effective? postoperative period. Inspiration requires the diaphragm to be Case series and literature analysis. Acta Neurochir (Wien). 2015;157(6):1077– lowered and the accessory muscles of inspiration to increase the 86. 6. Liu Y, Xu X-C, Zou Y, Li S-R, Zhang B, Wang Y. Phrenic nerve transfer to the diameter of the rib cage thus, obtaining the inspiratory capacity. musculocutaneous nerve for the repair of brachial plexus injury: If any of these muscles are altered, muscular compensation Electrophysiological characteristics. Neural Regen Res 2015;10(2):328–33. occurs and a consequent change in the volume in the inspiratory 7. Cardoso MM, Gepp R, Correa JFG. Outcome following phrenic nerve transfer capacity is observed. Thus, patients who had a loss of volume in to musculocutaneous nerve in patients with traumatic brachial palsy: a total inspiratory capacity, obtained a gain in inspiratory capacity qualitative systematic review. Acta Neurochir (2016) 158:1793–1800 DOI in the thoracic compartment where the accessory muscles of 10.1007/s00701-016-2855-8 8. Chuang ML, Chuang DCC, Lin IF, ;Vintch JRE,; Ker JJW., Tsao TCY. inspiration were activated. Ventilation and exercise performance after phrenic nerve and multiple Although the phrenic nerve has long been used as a donor intercostal nerve transfers for avulsed brachial plexus injury. Chest. nerve to restore muscle function after brachial plexus injury, 2005;128(5):3434–9. little is known about the possible change in lung function in the 9. Monreal, R. Restoration of elbow flexion by transfer of the phrenic nerve to long term. Most studies suggest that phrenic nerve surgery does musculocutaneous nerve after brachial plexus injuries. Hand.2007;206-21. not result in long-term consequences for lung function4,5,10,14; 10. Luedemann W, Hamm M, Blömer U, Samii M, Tatagiba M. Brachial plexus neurotization with donor phrenic nerves and its effect on pulmonary function. J however, another study shows that regardless of the surgical Neurosurg . 2002;96(3):523–6. technique used for phrenic nerve neurotization, patients will 11. Parreira VF, Vieira DS, Myrrha MA, Pessoa IM, Lage SM, Britto show a decrease in the pulmonary function test from the first RR.Optoeletronic plethysmography: a review of the literature. Rev Bras Fisiot post-surgical week which will be gradually recovered starting at 2012;439-53. the third post-surgical month16. 12. 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We suggest that future studies test for phrenic nerve dysfunction. J Neurosurg [Internet]. 2012;117(5):890–6. 15. Elshafie G, Acosta J, Aliverti A, Bradley A, Kumar P, Rajesh P, et al. Chest wall be carried out including a larger number of patients and long- mechanics before and after diaphragm plication. J Cardiothorac Surg term evaluation while also incorporating pulmonary function 2016;11(1):25. tests to verify possible clinical repercussions. 16. Xu, W.-D., Gu, Y.-D., Xu, J.-G., & Tan, L.-J. (2002). Full-Length Phrenic Nerve Limitations associated to this study include the small and Transfer by Means of Video-Assisted Thoracic Surgery in Treating Brachial heterogeneous sample number and the short time for follow-up. Plexus Avulsion Injury. Plastic and Reconstructive Surgery, 110(1), 104–109. Therefore future studies should include a longer doi:10.1097/00006534-200207000-00018 follow-up and a larger sample, so that respiratory physiotherapy Table 1. Sample characterization of five patients submitted to phrenic nerve neurotization after traumatic total brachial plexus injury. Patients Age (Years) Injury side Type of accident Date of injury Date of surgery

1 31 Left Motorcycle May/2016 October/2016

2 33 Right Motorcycle June/2016 November/2016

3 36 Left Motorcycle Augst/2016 January/2017

4 28 Left Car September/2015 May/2016

5 35 Left Motorcycle June/2016 February/2017

Table 2 - Data on ventilatory kinematics, tricompartmental distribution of thoracic cavity volumes and rates of contraction of respiratory muscles in each hemithorax on the pre- and post-operative period. Voluntary RR DUTY Vtcw VRCP VRCA VAB Speed of Speed of Speed of Speed of Abdominal Abdominal

(min) CYCLE (mL) (mL) (mL) (mL) inspiratory inspiratory the the speed R (s) speed L (s)

Muscles Muscles L(s) diaphragm diaphragm

R(s) R(s) L(s)

Voluntary 1

Pre 17.23 0.45 423 259 110 54 63.92 90.80 34.05 31.66 16.13 16.13

Post 10.284 0.44 784 502 333 -52 94.52 99.15 61.73 66.74 10.42 9.65

Voluntary 2

Pre 12.71 0.44 755 235 202 317 55.582 40.04 50.87 43.33 70.66 79.60

Post 17.476 0.46 528 173 129 226 62.38 51.04 49.78 36.55 66.16 83.18

Voluntary 3

Pre 11.25 0.37 929 256 233 440 61.94 65.44 60.44 55.94 105.40 114.39

Post 16.26 0.36 415 58 78 279 17.58 24.91 27.11 30.04 106.96 97.44

Voluntary 4

Pre 17.65 0.35 609 158 66 384 59.24 63.91 26.50 24.94 144.97 154.33

Post 16.40 0.33 504 125 51 329 48.07 50.43 20.49 18.91 122.93 136.33

Voluntary 5

Pre 7.917 0.54 2278 1036 322 920 102.63 121.22 25.28 44.30 95.07 103.72

Post 14.62 0.47 472 194 125 153 42.45 55.58 36.38 26.28 36.89 40.43

Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 9 Table 3 - Three-part distribution of thoracic cage volumes (ml) of each hemithorax on the pre- and post-operative period

VRCP VRCP VRCA VRCA VAB VAB Vtcw Voluntary Right Left Right Left Right Left Voluntary 1 VC (Pre) 613 (33%) 694 (38%) 195 (10%) 229 (12%) 40 (2%) 65 (3%) 1837 VC (Post) 530 (21%) 551 (22%) 725 (29%) 740 (30%) -41 (-1.8%) -66 (-2.7%) 2439 Gain and/or Loss - VC (%) -14 -21 272 223 -202 -202 32 IC (Pre) 450 (29%) 714 (47%) 126 (8.3%) 186 (12.6%) 21 (1.4%) 20 (1.3%) 1517 IC (Post) 413 (27%) 510 (34%) 509 (34%) 585 (39%) -291 (-19.5%) -240 (-16%) 1486 Gain and/or Loss - IC (%) -8 -29 204 115 -1382 -1300 -2 QB (Pre) 107 (25%) 152 (36%) 57 (13%) 53 (12%) 27 (6%) 27 (6%) 423 QB (Post) 245 (31%) 257 (33%) 160 (20%) 173 (22%) -27 (-3.4%) -25 (-3.1%) 783 Gain and/or Loss - QB (%) 129 69 181 226 -300 -296 85 Voluntary 2 VC (Pre) 499 (15%) 397 (12%) 536 (16%) 371 (11%) 755 (22%) 772 (23%) 3330 VC (Post) 416(14%) 296 (10%) 506 (17.5%) 356 (12%) 672 (23%) 638 (22%) 2884 Gain and/or Loss - VC (%) -17 -25 -6 -4 -11 -17 -13 IC (Pre) 358 (15.4%) 381 (16.4%) 315 (13.6%) 312 (13.5%) 430 (18.6%) 515 (22%) 2311 IC (Post) 345 (17.5%) 297 (15%) 385 (19.5%) 310 (16%) 323 (16.4%) 309 (15.6%) 1969 Gain and/or Loss - IC (%) -4 -22 22 -0,06 -25 -40 -14 QB (Pre) 118 (16%) 85 (12%) 108 (15%) 92 (13%) 150 (21%) 169 (23%) 722 QB (Post) 99 (18%) 81 (14.6%) 79 (14%) 58 (10%) 105 (19%) 132 (24%) 554 Gain and/or Loss - QB (%) -16 -5 -27 -37 -30 -22 -23 Voluntary 3 VC (Pre) 211 (10%) 179 (8%) 220 (10.6%) 195 (9%) 624 (30%) 631 (30.6%) 2060 VC (Post) 177 (9%) 192 (10%) 214 (11%) 180 (9.2%) 594 (30%)9 598 (30.5%) 1955 Gain and/or Loss - VC (%) -16 7 -27 -8 -5 -5 -5 IC (Pre) 237 (14%) 219 (12%) 223 (13%) 189 (11%) 409 (24%) 441 (26%) 1718 IC (Post) 154 (10%) 184 (12%) 190 (12.6%) 141 (9%) 412 (27%) 425 (28%) 1506 Gain and/or Loss - IC (%) -35 -16 -15 -25 0,7 -4 -12 QB (Pre) 124 (13%) 131 (14%) 121 (13%) 112 (12%) 211 (23%) 229 (24.5%) 928 QB (Post) 24 (5%) 34 (8%) 37 (9%) 41(10%) 146 (35.5%) 133 (32%) 412 Gain and/or Loss - QB (%) -81 -74 -69 -63 -31 -42 -55 Voluntary 4 VC (Pre) 724 (21%) 764 (22%) 351 (10%) 377 (11%) 582 (17%) 625 (18%) 3423 VC (Post) 779 (21%) 856 (23%) 459 (12%) 479 (13%) 514 (14%) 646 (17%) 3733 Gain and/or Loss - VC (%) 8 12 31 27 -11 3 9 IC (Pre) 552 (28%) 503 (26%) 258 (13%) 229 (12%) 179 (9%) 205 (10%) 1926 IC (Post) 662 (33%) 488 (24%) 359 (18%) 279 (14%) 70 (3%) 115 (5%) 1973 Gain and/or Loss - IC (%) 20 -3 40 22 -61 -44 2 QB (Pre) 76 (12%) 82 (13%) 34 (5%) 32 (5%) 186 (30.5%) 198 (32.5%) 608 QB (Post) 61 (12%) 64 (12,6%) 26 (5%) 24 (4%) 156 (31%) 173 (34%) 504 Gain and/or Loss - QB (%) -20 -22 -24 -25 -16 -13 -17 Voluntary 5 VC (Pre) 1027 (23%) 873 (19%) 313 (7%) 347 (8%) 1014 (22%) 857 (19%) 4431 VC (Post) 831 (24%) 919 (26%) 391 (11%) 396 (11.4%) 429 (12%) 505 (14.5%) 3471 Gain and/or Loss - VC (%) -19 0.052692 25 14 -58 -41 -21 IC (Pre) 65 (24.5%) 630 (23%) 252 (9%) 220 (8%) 460 (17%) 437 (16%) 2650 IC (Post) 520 (26%) 597 (29.5%) 271 (13%) 258 (12.8%) 136 (6%) 226 (11%) 2008 Gain and/or Loss - IC (%) -20 -5 7 17 71 48 -24 QB (Pre) 475 (21%) 561 (24.6%) 117 (5%) 205 (9%) 440 (19%) 480 (21%) 2278 QB (Post) 84 (18%) 110 (23%) 72 (15%) 52 (11%) 73 (15%) 80 (17%) 471 Gain and/or Loss - QB (%) -82 -80 -38 -75 -84 -83 -79 Rev Bras Neurol 56(3):11-14, 2020.

Conhecimento da população sobre Acidente Vascular Cerebral em Torres RS Knowledge of stroke in Torres-RS population

Valmir Soares Machado1, Lidiane de Medeiros Hahn2, Maria Isabel Morgan Martins3, Luiz Carlos Porcello Marrone4

RESUMO ABSTRACT

Introdução: O Acidente Vascular Cerebral (AVC) é uma das Introduction: Stroke is a leading cause of mortality and disability principais causas de morbi-mortalidade na América Latina, in Latin America and few paper evaluate the knowledge of poucos estudos avaliam o conhecimento da população brasileira brazilian population about this subject. Objective: Evaluate the sobre o mesmo. Objetivo: Avaliar o conhecimento da população knowledge of Torres/RS about stroke risk factors and de Torres/RS sobre fatores de risco e sinais/sintomas de AVC signs/symptoms of stroke. Methods: It was performed a Método:Foi realizado um estudo do tipo transversal de caráter transversal study with 375 inhabitants of Torres, in which they descritivo e exploratório, com entrevista a 375 habitantes, no answered two questionnaires, a sociodemographic and the other qual responderam a dois questionários, um sociodemográfico e related to the knowledge of the signs and symptoms of stroke. outro relativo ao conhecimento dos sinais e sintomas do The Chi-square test (χ2) and the T-student test were performed Acidente vascular cerebral. O teste de Qui-Quadrado (χ2) e teste to assess the association between qualitative variables and to T-student foram realizados para avaliar a associação existente verify differences in the absolute frequency and percentage of entre as variáveis qualitativas e para verificar diferenças na variables. Results: The mean age of the participants was 39.7 frequência absoluta e percentual das variáveis. Resultados: A (+/-14.6) years, (230 were women). The average of correct idade média dos participantes foi de 39,7 (+/- 14,6) anos, sendo answers about risk factors was 3.7/11 (34.4%) and recognition of 230 mulheres, a média de acertos sobre fatores de risco foi de signs/symptoms was 3.2/10 (32.5%). Systemic Arterial 3.7/11 (34.4%) e de reconhecimento de sinais/sintomas foi de Hypertension was the risk factor most recognized by the 3,2/10 (32,5%). Em relação aos fatores de risco, Hipertensão population (229 individuals). Facial Paralysis reported by 197 Arterial Sistêmica foi descrita por 229 (17,4%) indivíduos e individuals was the signs/symptoms most recognized by the quanto aos sinais/sintomas, o mais descrito foi a Paralisia Facial Torres population. In addition, it was observed that individuals Central, relatada por 197 (17,2%). Além disso, foi observado que with a lower level of education, male and under 39 years old, indivíduos com menor nível de instrução, do sexo masculino e presented a worse performance in relation to knowledge about com menos de 39 anos, apresentaram um pior desempenho em stroke. Conclusion: The implementation of public policies that relação ao conhecimento sobre AVC. Conclusão:Dessa forma, provide the population information about the importance of the sugere-se a necessidade de implementar políticas públicas que early recognization of stroke signs and symptoms is fundamental levem à população informações sobre a importância do for a better result in the care of this disease. reconhecimento destes sinais e sintomas com a necessidade do socorro rápido a este paciente. Keywords: Stroke, Risk Factors, Signs and Symptoms.

Palavras chaves: Acidente Vascular Cerebral, Fatores de Risco, Sinais e Sintomas.

______1-Professor Especialista. Coordenador do Curso de Enfermagem na Universidade Luterana do Brasil – ULBRA Campus Torres. Mestrando no Programa de Pós-Graduação em Promoção da Saúde, Desenvolvimento Humano e Sociedade; 2-Enfermeira do Programa de Residência Multiprofissional em Saúde do Adulto e Idoso da Universidade Luterana do Brasil – ULBRA; 3-Doutora em Ciências Biológicas ênfase em Fisiologia - UFRGS. Profa. Adjunta do Programa de Pós-Graduação em Promoção da Saúde, Desenvolvimento Humano e Sociedade na Universidade Luterana do Brasil - ULBRA; 4-Neurologista do Hospital São Lucas da PUCRS e Professor da Faculdade de Medicina e do Programa de Pós-Graduação em Promoção da Saúde, Desenvolvimento Humano e Sociedade na Universidade Luterana do Brasil - ULBRA.

Autor correspondente: Valmir Soares Machado, Rua Joaquim Hoffmaister n 204 Getúlio Vargas Torres RS CEP 95560000, e-mail: [email protected]

Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 11 Machado et al. Conhecimento popular sobre AVC satisfatórias quando o indivíduo respondeu ou referenciou (com terminologia popular) algo semelhante: 1-Hipertensão Arterial INTRODUÇÃO Sistêmica, 2-Diabetes, 3- Dislipidemia, 4-Tabagismo, 5- Obesidade, 6- Idade elevada, 7- Sexo Masculino, 8- História O Acidente Vascular Cerebral (AVC) é uma das familiar de AVC ou infarto do miocárdio, 9- Arritmia e 10- principais causas de morbi-mortalidade da população brasileira. Consumo de Drogas/Álcool, 11- Outro (desde que de acordo Trata-se de uma doença extremamente prevalente, dados da com literatura). Os participantes também foram questionados se OMS sugerem que um em cada seis indivíduos terá um AVC saberiam reconhecer um AVC em curso pela pergunta: “O durante sua vida. O AVC é dividido em isquêmico e senhor (a) sabe reconhecer sinal/sintoma de AVC? ” Foram hemorrágico, sendo o primeiro mais prevalente (responsável por consideradas respostas satisfatórias quando o indivíduo 85% dos casos) e o segundo com maior letalidade1,2,3,4. respondeu ou referenciou (com terminologia popular) algo São múltiplos os fatores de risco cardiovasculares semelhante: 1- Fraqueza unilateral, 2- Alteração na fala, 3- relacionados a essa doença, como hipertensão arterial sistêmica, Alteração da visão, 4- Paralisia facial, 5-Cefaleia/dor de cabeça, diabetes mellitus, dislipidemia, tabagismo, arritmias (fibrilação 6- Alteração de sensibilidade, 7-Perda da consciência, 8- atrial), idade avançada e sedentarismo. Sobre o reconhecimento Alteração de memória, 9-Vertigem, 10- Outro (desde que de dessa doença,muitas vezes pode ser difícil, uma vez que, o AVC acordo com literatura). Para cada item respondido corretamente pode apresentar muitos sinais e sintomas neurológicos que foi dado um ponto. O total de acertos de cada indivíduo gerou dependem da região cerebral e artéria acometida pela lesão1,2,4,5. uma nota de 0 a 11 na primeira pergunta e de 0 a 10 na segunda. O reconhecimento precoce do AVC isquêmico Os dados extraídos dos documentos foram tabulados possibilita que muitos pacientes consigam receber um tratamento em planilhas elaboradas no programa Excel versão 2016 e agudo com capacidade de reverter o quadro. Na década de 90, posteriormente, analisados no programa Statistical Package For com a publicação do estudo NINDS, o tempo passou a ser Social Science For Windows (SPSS) versão 22 com nível de fundamental para o tratamento do AVC6,7. Atualmente, com o significância de 5%. Inicialmente foi realizada a estatística advento das técnicas de trombectomia, no AVC agudo, esse descritiva a fim de caracterizar a amostra quanto às processo passa por uma importante transformação8-12. Entretanto, características sócio-demográficas e socioeconômicas. O teste de ampla parcela da população brasileira não sabe o que é essa Qui-Quadrado (χ2) e teste T-student foram realizados para doença e como reconhecê-la de maneira ágil e, com isso, avaliar a associação existente entre as variáveis qualitativas e encaminhar o paciente para o atendimento rápido13. para verificar diferenças na frequência absoluta e percentual O objetivo desse trabalho é avaliar o conhecimento da entre: sexos masculino e feminino, a faixa etária, analfabetos e população de Torres/RS sobre fatores de risco e sinais/sintomas alfabetizados. Além disso, comparou-se a frequência absoluta e de AVC. percentual das respostas de cada questão do questionário do grau de conhecimento do AVC.

MATERIAL E MÉTODOS RESULTADOS Foi realizado um estudo do tipo transversal de caráter descritivo e exploratório, com entrevista a 375 habitantes do Avaliando 375 moradores de Torres-RS, com idade município de Torres–RS, com idade igual ou superior a 18 anos, média de 39,7 (+/-14,6) anos (sendo 230 mulheres), a média de a fim de avaliar o conhecimento dessa população sobre fatores acertos sobre fatores de risco foi de 3,7/11 (34.4%) e de de risco e sinais/sintomas de alerta de AVC, no período de reconhecimento de sinais/sintomas foi de 3,2/10 (32,5%). O março a junho de 2019. fator de risco mais descrito foi hipertensão arterial sistêmica em Torres-RS é uma cidade no litoral norte do Rio Grande 229 (17,4%), seguido de sedentarismo/obesidade que foi descrito do Sul, com uma população de aproximadamente 37.000 mil em 202 (15,4%). O sinal/sintoma mais descrito foi paralisia habitantes. Indivíduos que estavam em viagem/turismo no facial em 197 (17,2%), seguido de alteração na fala que foi município foram excluídos da amostra, assim como indivíduos descrito em 165 (14,4%). que apresentavam um déficit cognitivo ou afasia e não pudessem Observamos algumas diferenças importantes quando completar a entrevista. avaliamos diferenças entre subgrupos. Mulheres apresentaram A coleta de dados teve início após a aprovação do um maior conhecimento sobre os sinais/sintomas do AVC Comitê de Ética e Pesquisa da Universidade de Caxias do Sul conforme descrito na Tabela 1. UCS – RS com parecer número 3.203.515. Para publicação dos resultados da pesquisa, assegurou-se o anonimato, o respeito Tabela 1. Distribuição da frequência percentual das respostas pela dignidade humana, segue os requisitos da bioética de dos participantes em relação ao conhecimento dos acordo com a Resolução 196/96 e foi conduzida dentro dos Sinais/Sintomas, fatores de risco sobre AVC relacionados entre padrões éticos exigidos pela Resolução Nº 466/12. o sexo. A participação dos entrevistados foi de caráter Homens (n:145) Mulheres (n:230) P voluntário, sem fins lucrativos, foi explicado o objetivo da Fatores de Risco 35,7% 33,6% 0,155 pesquisa e esclarecido as dúvidas. Após entregue o Termo de Sinais/Sintomas 31,3% 33,3% <0,001 Consentimento Livre e Esclarecido (TCLE) e assinado foi Fonte: Da própria pesquisa. aplicado o questionário em forma de entrevista contendo perguntas sociodemográficas, socioeconômicas e o Em relação ao desempenho do conhecimento de conhecimento do acidente vascular cerebral. indivíduos com ensino médio ficou evidenciado na tabela 2 Na aplicação do questionário foi realizada a pergunta quando questionados sobre o conhecimento dos fatores de risco “ O Senhor (a)sabe quais são os fatores de risco para identificar e sinais/sintomas sobre AVC, que houve diferença significativa um AVC e o que causa AVC? ”. Foram consideradas respostas nas respostas, mostrando que o fato de ter uma maior formação Falavigna et al.15 no município de Caxias do Sul/RS, há falta de (anos de estudo) traz impacto importante no conhecimento sobre conhecimento dos próprios pacientes em recuperação de AVC. AVC. Esse trabalho indicou que mulheres apresentam um conhecimento maior que homens em relação aos sinais e Tabela 2. Distribuição da frequência percentual das respostas sintomas do AVC, no estudo de Meira, Magalhães, Silva et 16 dos participantes em relação ao conhecimento dos al. ,o conhecimento geral sobre o AVC foi baixo no público Sinais/Sintomas, fatores de risco sobre AVC relacionados ao pesquisado em Minas Gerais, onde reportaram que o grau de conhecimento do AVC relacionada a escolaridade. reconhecimento dos sinais desta patologia também foi maior Ensino Médio (sim) Ensino Médio (não) P entre mulheres e em pessoas com antecedentes familiares. Campos-Souza et al.17 aponta para a necessidade de campanhas Fatores de Risco 40,7% 24,1% <0,001 para a população de Teresina, que possui pouco conhecimento a Sinais/Sintomas 38,3% 23% <0,001 respeito das doenças cerebrovasculares. Fonte: Da própria pesquisa. Dessa forma, é fundamental o desenvolvimento de pesquisas que descrevam o conhecimento da população Outro fator que apresentou uma significativa diferença brasileira sobre o AVC para que se possa desenvolver de conhecimento foi a idade. Essa análise apontou que campanhas que venham conscientizar as pessoas a respeito dos indivíduos com idade superior a 40 anos apresentaram maior sinais e sintomas do AVC e da importância da agilidade no índice de acertos em relação aos mais jovens quando atendimento. entrevistados sobre os sinais e sintomas do AVC (Tabela 3).

Tabela 3. Distribuição da frequência percentual das respostas CONCLUSÃO dos participantes em relação ao conhecimento dos Sinais/Sintomas, fatores de risco sobre AVC relacionados a A partir desse estudo, concluiu-se que a maioria dos idade participantes não tinha conhecimento satisfatório sobre os sinais Idade (18 a 39 Idade >40 anos P e sintomas do AVC. Dessa forma, sugere-se a necessidade de anos) implementar políticas públicas que levem à população Fatores de 35% 32,7% 0,123 informações sobre a importância do reconhecimento destes Risco sinais e sintomas, bem como, da necessidade do socorro rápido Sinais/Sintomas 33,9% 59,2% <0,001 ao paciente. Fonte: Da própria pesquisa. O despreparo e a falta de conhecimento dos participantes sobre sinais e sintomas de alerta do AVC e dos fatores de risco prejudicam a percepção da importância no que DISCUSSÃO diz respeito a mortalidade e morbidade provocada por essa doença. Poucos estudos avaliam o conhecimento e Atualmente o AVC é um grande problema de saúde reconhecimento da população brasileira sobre AVC, pública em países desenvolvidos e em desenvolvimento. A alta principalmente em municípios com menor número de habitantes. morbimortalidade dessa enfermidade gera gastos volumosos aos Estudos que mostrem esse cenário em diferentes sistemas de saúde, sendo uma das doenças que mais onera os regiões do Brasil devem ser estimulados, com o intuito de sistemas. A estratégia de prevenção dos fatores de risco deve ser apresentar um panorama nacional e estabelecer políticas públicas estimulada para precaução dessa situação. Torna-se fundamental para educar a população geral sobre essa enfermidade. Desta o desenvolvimento de campanhas para conscientizar a população forma, se torna necessária a conscientização dos profissionais da geral sobre os cuidados para evitar um AVC.1,2 área de saúde e do poder público para promover trabalhos O reconhecimento dos sinais e sintomas de AVC pode científicos e implementar programas visando melhorar o ser difícil dependendo da artéria acometida e dos sintomas conhecimento populacional a respeito do AVC. iniciais. Eventos que acometem a circulação anterior e que causam déficit corticais são mais facilmente identificados. No DECLARAÇÃO DE FINANCIAMENTO: entanto, muitas vezes, esses ocorrerão e devem ser prontamente Financiamento próprio reconhecidos.2 Na população de estudo foi possível observar um DECLARAÇÃO DE CONFLITO DE INTERESSES: desconhecimento dos mesmos sobre fatores de risco e sinais e Não há conflitos de interesse sintomas de AVC. O pior desempenho foi de pessoas com menor nível de educação, o que já era esperado. 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Guillain-Barré syndrome: advances and future perspectives Síndrome de Guillain-Barré: avanços e perspectivas futuras

Rabello, Francisco de AP Cabral Júnior1; Pupe, Camila Castelo Branco2; Nascimento, Osvaldo JM3.

ABSTRACT RESUMO

The first case of Guillain-Barré syndrome was described in 1916. A síndrome de Guillain-Barré teve seu primeiro caso descrito em Since then, knowledge about the pathophysiology and 1916. Desde então, o conhecimento sobre a fisiopatologia e immunogenesis of this acquired inflammatory imunogênese dessa polirradiculoneuropatia inflamatória polyradiculoneuropathy has been growing steadily, especially adquirida vem crescendo continuamente, especialmente após o after the advent of nerve conduction studies and the discovery of advento dos estudos de condução nervosa e a descoberta de pathogenic autoantibodies. In the present study, we conducted a auto-anticorpos patogênicos. No presente estudo, realizamos review of the main information available in the literature to date uma revisão das principais informações disponíveis na literatura about the syndrome, including its diagnosis and management. até o presente momento sobre a síndrome, incluindo seu diagnóstico e manejo. Keywords: Guillain-Barré syndrome; polyneuropathies; immunology Palavras chave: síndrome de Guillain-Barré; polineuropatias; imunologia.

______1 Mestre em Neurologia pela Universidade Federal Fluminense (UFF). Preceptor da residência de neurologia da Fundação Hospitalar do Estado de Minas Gerais (FHEMIG). Barbacena/MG, Brasil2 Doutora em Neurologia pela UFF. Professora Adjunta de Neurologia da UFF. Niterói/RJ, Brasil.; 3 Doutor em Neurologia pela Universidade Federal do Rio de Janeiro (UFRJ). Professor Titular de Neurologia da UFF. Niterói/RJ, Brasil. [email protected]

Corresponding author: Osvaldo JM Nascimento. Av. N Sa Copacabana, 690/1001, Copacabana, Rio de Janeiro, RJ, Brazil. CEP: 22050-001. [email protected]

Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 15 Rabello et al. Guillain-Barré syndrome GBS has historically been associated with vaccination for A/H1N1 influenza since the epidemic outbreak in the United States in 197615. In the following decades, an effort was made INTRODUCTION to assess whether the event was a mere temporal coincidence or if there was really a causal relationship between GBS and In 1859, the French physician Octave Landry vaccination. A recent systematic review of the literature by reported a series of cases of acute ascending paralysis1, which Dudley and collaborators found that, although the literature is may correspond to the first sample of cases of Guillain-Barré conflicting, the influenza vaccine can rarely cause GBS in Syndrome (GBS). At the time, however, the examination of adults16. Taking the risk-benefit into account, the study intrinsic muscle reflexes was not regularly documented and a concludes that there is an excellent general safety profile in lumbar puncture technique had not yet been described. recommending vaccination in the general population. Willison In 1916, during the period of the First World War, and collaborators advice not to vaccinate patients who have had doctors Georges Guillain, Jean Barré and André Strohl GBS in the last three months or patients who have had post- described the case of two soldiers of the French army who vaccine GBS17. suffered weakness with acute progressive evolution related to Other immunosensitizing factors that have been tingling, diminished reflexes and elevated protein in associated with GBS are metabolic stressors, such as, for cerebrospinal fluid, without cell alteration2. Subsequently, this example, surgery (moderate risk, especially bone and disease became known as Guillain-Barré-Strohl syndrome, and gastrointestinal tract surgery)18; trauma19; gestation; systemic then simply GBS. lupus erythematosus8; as a paraneoplastic syndrome in patients The standardization of the definition of GBS allowed with malignancy, especially in elderly patients with severe the disease to be more frequently diagnosed worldwide in the axonal loss and poor response to treatment with follow decades, being today considered the main cause of immunoglobulin20. acute flaccid paralysis worldwide3. GBS is currently considered an acute or subacute immune-mediated inflammatory polyradiculoneuropathy, PATHOPHYSIOLOGY which classically causes progressive weakness in the limbs associated with hyporeflexia. Sensory changes usually precede GBS is probably a disease with a predominance of or accompany motor symptoms, but they are generally not the 4 lesions due to humoral autoimmunity, rather than cellular most debilitating manifestations . mediated by T lymphocytes21. Although less common, axonal variants have a better understood pathophysiology than the Acute Inflammatory Demyelinating Polyradiculoneuropathy EPIDEMIOLOGY (AIDP) form. In Acute Motor Axonal Neuropathy (AMAN) or Acute Motor and Sensory Axonal Neuropathy (AMSAN) GBS ​ ​ affects approximately 100 thousand people phenotypes, humoral attack (auto-antibodies) to the peripheral every year worldwide, has an annual incidence ranging from nerve axolema predominates, especially the complement 0.4 to 4.0 cases/100.000/year, depending on the methodology fixators of the IgG1 and IgG33 subclasses. of the study and the case definition, with the majority of well- This aberrant immune response is presumably designed prospective studies found an average incidence of 2.0 generated by a mechanism of molecular mimicry between lipo- cases/ 100.000 /year5,6. The disease affects more frequently oligosaccharides (LOS) of infectious agents capable of inducing men (relative risk 1.5 in relation to women), an unusual an immune response (mainly C. jejuni) and gangliosides present characteristic in immune-mediated diseases. It is uncommon in in the axolema (GM1 and GD1a). These ganglioside-linked children under 10 years old (incidence 0.34 to 1.34/100.000/ autoantibodies induce a cascade with complement fixation, year)7, and it becomes more common after 50 years of age8, macrophage recruitment and membrane attack complex with peaks between 50-59 and between 60-69 years9. The deposition. A similar phenomenon occurs in Miller Fisher cumulative risk of developing GBS over the course of a syndrome (MFS), but with anti-GQ1b antibody, which attacks lifetime is on the order of 1 for every 1000 people10. axons that mainly innervate the extraocular muscles, causing In up to 76% (approximately three quarters) of the the typical ophthalmoplegia11,22,23. cases immunosensitizing events can be identified, occurring in The cascade of phenomena that lead to demyelinating general from one to four weeks before the onset of symptoms lesions in patients with AIDP is poorly understood, and appears and mainly include: upper airway infection (35%) and to involve multiple target antigens in the myelin sheath and in gastroenteritis (27%). This pattern is seen in American, Ranvier's nodules, including gliomedine, contactin, TAG-1, European and Asian countries, except Bangladesh, where moesin and neurofascin, related to a broader range of gastroenteritis is more common (36%)9. The etiological agents immunosensitizing agents, including various infectious agents involved in the pathophysiology of GBS have been mainly the and vaccines17. Another hypothesis is that in AIDP there is the bacteria Campylobacter jejuni and Mycoplasma pneumoniae formation of neo-antigens from complexes of glycolipid and the following viruses: Zika, cytomegalovirus, Epstein-Barr, components of the myelin sheath that may be the target of influenza A and enterovirus D6811. GBS after rabies infection heteromeric or multimeric anti-complex antibodies that are is extremely rare11. difficult to be identified by standard techniques24. Since the pandemic by the new coronavirus (COVID- Although not common, rapid recoveries after 19) was officially recognized by the World Health immunoglobulin administration in axonal GBS are in line with Organization12, cases of GBS have been reported within five to the hypothesis that part of the neurological dysfunction can be ten days after flu-like symptoms in patients with viral infection attributed to a reversible electrical stunning in the nodal or by COVID-19 in northern Italy13 and China14, among other paranodal region, before the axonal degeneration itself occurs. countries, leading to the hypothesis that it is another virus In AIDP, part of the clinical improvement may occur due to the implicated in GBS. Despite the preliminary association, the reversal of nerve conduction blockades after treatment10. number of reported cases is still small, therefore, further Diagnose an axonal GBS with or without antibody studies are needed to establish a causal relationship between positivity is a valuable information, because patients generally these two clinical conditions. have slower and more incomplete neurological recovery than weeks in 96% of cases and up to 4 weeks in 99.8% of cases. in purely demyelinating form9. So far, the detection of Relapse is rare, occurring in two to five percent of cases29, and autoantibodies among GBS phenotypes is in the field of when it does, alternative diagnoses should be investigated, such experimental research and does not yet have specific as Chronic Inflammatory Demyelinating repercussions on treatment, although there is a prospect in the Polyradiculoneuropathy (CIDP), which in rare cases may have future for treatments aimed at neutralizing autoantibodies3. an acute presentation (A-CIDP) that simulates GBS30. Recurrence must, however, be distinguished from treatment-related fluctuations, which actually is a new clinical DIAGNOSIS deterioration that occurs after nadir has been achieved or even a clinical improvement. In the first condition (recurrence) there is The diagnostic criteria for GBS were reviewed by no benefit of retreatment, whereas it is common practice to treat Asbury and Cornblath25, with the recent additions to the again the patient with immunoglobulin after a fluctuation, consensus statement published by Leonhard and colleagues in despite little evidence to support this option31. the journal Nature Reviews26 (Table 1). After a variable period of stability, there is usually a gradual recovery of neurological functions, which can be partial or complete. About 80% of patients are able to walk Table 1: Diagnostic criteria for GBS. independently without support or help within 12 months of Characteristics required for the diagnosis of Guillain-Barré Syndrome in symptom onset. Although neurological recovery is more intense clinical practice: in the first year, there is often an accumulative improvement for Progressive weakness in the arms and legs [initially only the legs may be more than five years26. The prognosis of independent gait with involved]. six months of symptoms can be estimated using the Erasmus Areflexia [or decreased tendon reflexes] in the affected limbs. GBS Outcome Score (EGOS). Features that strongly support the diagnosis: The progressive phase lasts from days to four weeks [often two weeks]. Autonomic dysfunction is common and a sign of poor Relative symmetry of signs and symptoms. prognosis, being a manifestation of failure of peripheric Mild sensory symptoms or signs [absent in pure motor variant]. nervous system regulation of blood pressure and cardiac Involvement of cranial nerves, especially bilateral facial paralysis. function causing orthostatic hypotension, labile blood pressure Autonomic dysfunction. and cardiac arrhythmias, among others. Bulbar muscle paralysis Muscle or root pain in the back or limb. can cause diaphragmatic involvement and can lead to acute Increased levels of protein in CSF; normal protein levels do not exclude the respiratory failure and the need for ventilatory support in diagnosis. approximately 20% of cases3. Electrodiagnostic characteristics of motor or sensory-motor neuropathy From a clinical point of view, GBS can be classified (normal ENMG in the early stages does not exclude the diagnosis). into classic GBS, which causes weakness and hyporeflexia in Features that cast doubt on the diagnosis: the four limbs; pharyngocervic-brachial form, in which there is Increase in the number of mononuclear or polymorphonuclear cells in the cerebrospinal fluid (more than 50 cells/mm3). weakness limited to upper, cervical and bulbar regions; Weakness markedly or persistently asymmetric. paraparetic GBS, when the weakness is restricted to the lower Intestinal or bladder dysfunction on presentation or persistent during the limbs; bifacial paralysis with distal paresthesia; MFS, course of the disease. characterized by ophthalmoplegia, ataxia and limb areflexia; Severe respiratory dysfunction and little limb weakness at presentation. Bickerstaff's brainstem encephalitis (BBE), considered a central Sensitive signs with little weakness in presentation. subtype of MFS, because it shares a common pathophysiology Fever at onset of symptoms. of autoantibodies: it is characterized by hypersolence, Nadir in less than 24 hours. ophthalmoplegia and ataxia (Figure 1). The clinical Well-demarcated sensory level indicating spinal cord injury. classification does not depend on complementary exams and Hyper-reflexia or clonus. allows the categorization of the main phenotypes presented by Extensive plantar response [Babinski's sign] the patients, emphasizing the reality observed in clinical Abdominal pain. Slow progression with little weakness without respiratory involvement. practice that there are patients with restricted and incomplete Progression continued for more than four weeks after the onset of symptoms. weakness, as well as additional findings, such as hypersolence Change in consciousness (except for Bickerstaff's brainstem encephalitis). and ataxia. There are several clinical-physiological and clinical- Adapted from Leonhard et al., 2019. serological relationships between these variants, which is compatible with the heterogeneity of the syndrome. MFS can The World Health Organization recommends the occur in isolation or overlap with classic GBS, a phenomenon wide use of the Brighton Criteria (originally created to assess called overlap GBS-MFS32,6,26. the relationship between GBS and vaccination) to determine Mortality from GBS varies from three to ten percent, the diagnostic accuracy in epidemiological studies6,27. with an average of seven percent, and may be higher in some Patients who meet Asbury and Cornblath's diagnostic criteria places, such as Bangladesh, where it reaches 17%. This in general have a classic clinical picture of rapidly progressive difference can be explained by certain factors, such as difficulty bilateral and symmetrical limb weakness (with or without in accessing adequate health care and the presence of an axonal involvement of bulbar and facial muscles, ataxia and variant with a worse prognosis9. ophthalmoplegia) associated with hyporeflexia in the affected The Brazilian National Clinical Protocol and limbs. Although hyporeflexia is a cardinal sign of GBS, in Therapeutic Guidelines for GBS supports professionals to make extremely rare cases hyperreflexia may occur, probably the suspected diagnosis based primarily on the anamnesis and associated with blocking the activity of medullary inhibitory neurological evaluation and the confirmation of the cases with interneurons28. In general, motor involvement is preceded in up complementary exams (CSF and nerve conduction study). Its to 70% of cases by mild sensory symptoms such as diagnostic criteria are very similar to those of Asbury & paresthesias and pain11. Cornblath, except for removing from the criteria suggestive of The natural history of GBS occurs with the GBS the recovery of neurological functions between two to progression of signs and symptoms until nadir (period in which four weeks from the onset of symptoms (related to deterioration stop progressing) which occurs in up to two Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 17 remyelination) and including the presence of pain among those This neurophysiological classification was then criteria of diagnostic support33. revised in order to develop a standardized case definition for the purpose of comparability between studies, with the following neurophysiological variants being established: AIDP, AMAN, AMSAN and inexcitable35,6. The most common form of GBS (in the Americas, Europe and Asia) is AIDP (demyelinating form), present in up to 85% of cases with a motor-sensitive clinical picture. Axonal variants (AMAN / AMSAN) together account for ten percent of cases, affect younger patients (average 31 years old), cause more weakness (MRC classification), less sensitive symptoms, and have a worse prognosis for recovery (only 62% walk without support within six months of presenting symptoms). In addition, axonal variants are more associated with gastrointestinal infection by Campylobacter jejuni. MFS occurs in up to five percent of patients9. Another complementary exam of value in patients with GBS is magnetic resonance imaging of the lumbosacral Adapted from Wakerley et al, 2014. spine, specially to exclude alternative diagnoses such as acute Figure 1. GBS Clinical presentations transverse myelitis and compressive/infiltrative myelopathy. The uptake of gadolinium in the nerve roots is a finding that reinforces the diagnosis of GBS36,37. Support for the diagnosis of GBS is based, among others, on the finding of albumin-cytological dissociation in the cerebrospinal fluid (less than 10 leukocytes/ml and MANAGEMENT hyperproteinorrhachia in general greater than 55mg/dl). This finding is present in more than 68% of cases. Less commonly, The pillars of GBS treatment are general clinical mild pleocytosis (five to 50 leukocytes/ml) may occur (19%). support and immunotherapy. Patients should preferably be The presence of pleocytosis greater than 50 leukocytes/ml, admitted to the Intensive Care Center (ICU), especially if they associated or not with hyperproteinorrhachia is rare in GBS have dysautonomia or have a high risk of progressing to (up to two percent), and when present, it should be a warning mechanical ventilation (Erasmus GBS Respiratory Insufficency sign for the expansion of the differential diagnosis. The score (EGRIS) greater than or equal to five points)26 (Table 2). absence of dissociation can occur early (only 50% of patients with GBS have dissociation in the first three days of Table 2. Erasmus GBS Respiratory Insufficency score (EGRIS) symptoms), so a viable alternative in patients with suspected Measure Category Pontuation GBS and absence of dissociation is to repeat the lumbar Number of days between onset Greater than seven 0 puncture, as up to 84% of patients who collect CSF after seven of weakness and hospital Between four and seven 1 admission days of symptom presentation presents albumin-cytological Equal or less than three 2 dissociation9. Facial and/or bulbar weakness Absent 0 at hospital admission Another complementary exam that reinforces the Present 1 Sum of MRC score* on 51-60 0 diagnosis of GBS is the electroneuromyography (ENMG), hospital admission 41-50 1 whose findings are also highly dependent on the time they are 31-40 2 performed. Initial changes include in the early phase (up to 21-30 3 three days of symptoms) changes in F-wave and H-reflex Equal or less than 20 4 responses (prolonged or absent). Then, with evolution of disease, the distal motor latencies are commonly prolonged. EGRIS score 0 a 7 Temporal dispersion and focal conduction blocks occurs later Adapted from Walgaard et al., 2010. *Sum of the score in the manual (around the third week of symptoms). It has been described assessment of the strength of 6 muscles of the upper and lower limbs using the Medical Research Council (MRC) score: bilateral adduction of the shoulders that the involvement of sensory potentials in the nerves of the (maximum 10 points, five for each shoulder), elbow flexion (up to ten points, upper limbs and preservation of the potential of the sural nerve five for each upper limb); wrist extension (ten points, five for each wrist); thigh is characteristic of GBS, since it is not a length-dependent flexion (ten points, five for each thigh); knee extension (ten points, five for each neuropathy3. knee) and plantar dorsiflexion (ten points, five for each foot), totaling a maximum of 60 points. ENMG can usually be postponed after one week of symptoms, and can optionally be repeated after symptom Regular monitoring of respiratory and autonomic stabilization (ideally between the third and eighth week of function (blood pressure, heart rate and sphincter control), clinical presentation), when there is usually chronic muscle strength and swallowing capacity can be performed denervation with signs of reinnervation, positive sharp waves, more adequately in the ICU than in the infirmary. In addition, fibrillations and decreased recruitment of motor units26. the risk of respiratory failure may be present even in the The electrophysiological classification of GBS was absence of dyspnea, especially if the vital capacity is less than proposed by Hadden and collaborators, dividing the findings 20ml/kg, the maximum inspiratory pressure is less than between five possibilities: normal; primarily demyelinating; 30cmH20 or the maximum expiratory pressure is less than primarily axonal; inexcitable and equivocal34. However, less 40cmH20, situations that demand early intubation and common subtypes of GBS have been described in the last mechanical ventilation to avoid neuromuscular fatigue and thirty years, with the identification of patients with a clinical hypercapnic respiratory failure38. However, if the patient course other than AIDP and the identification of specific evolves stable and improving, without other signs of severity, autoantibodies against axonal gangliosides and glycoproteins32. he can be admitted to the infirmary and the transfer to ICU be reconsidered in case of complications. Early physiotherapy rehabilitation is also part of non- emphasis is still on the importance of early recognition and pharmacological treatment. Psychological support is important, timely treatment of GBS as a way to accelerate clinical as the patient with GBS usually has preserved awareness and recovery. Multiprofessional treatment is essential in the cognition, and functional limitation in a previously rehabilitation of patients with GBS, avoiding secondary independent patient can be a reason for mental suffering. All complications of the disease. procedures must be explained to the patient, who must also be involved in making decisions26. DECLARATIONS OF INTEREST: The Brazilian Clinical Protocol and Therapeutic None. Guidelines for GBS guides the treatment of GBS with human immunoglobulin 0.4g/kg/day for five consecutive days (total FUNDING STATEMENT: cumulative dose of 2g/kg) in the cases established with This research did not receive any specific grant from funding moderate-severe GBS (patients that can´t walk without support agencies in the public, commercial, or not-for-profit sectors. for more than ten meters) within the 14-day period of symptom presentation33. The degree of neurological disability of the patients must be assessed at admission using the GBS disability score (Table 3). REFERENCES 1. Landry O. Note sur la paralysie ascendante aiguë. Gaz Hebd Med Table 3. Score GBS of disability. Chir 1859;6:472-474. Grade Clinical status of the patient 2. Guillain G, Barré JA, Strohl A. 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Síndrome GBS, major scientific advances have occurred in de Guillain-Barré após traumatismo de plexo braquial: relato de caso. understanding the disease, such as studies of nerve conduction, Arq Neuro-Psiquiatr 2006;64(4):1039-1040. which have brought insights into the presence of primarily 20. Hiew FL, Rajabally YA. Malignancy in Guillain-Barré syndrome: A demyelinating or axonal lesions. There was the identification twelve-year single-center study. J Neurol Sci 2017;15(375):275-278. of autoantibodies involved in the immunopathogenesis of some 21. Dalakas MS. Pathogenesis of immune-mediated neuropathies. variants of GBS, which represents an advance in the Biochim Biophys Acta 2015;1852(4):658-666. 22. Susuki K, Yuki N. et al. Dysfunction of nodes of Ranvier: a identification of targets for a future specific mechanism for anti-ganglioside antibody-mediated neuropathies. Exp immunotherapeutic treatment. Despite these advances, the Neurol 2012;233:534-542. Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 19 23. Willison HJ. The translation of the pathological findings described in http://portalsaude.saude.gov.br/images/pdf/2016/fevereiro/24/Guilain- humans to experimental models of acute motor axonal neuropathy. J Barr-----PCDT-Formatado--.pdf>. Acesso em 15 abr 2018. Peripher Nerv Syst 2012;17(suppl 3):3-8. 34. Hadden RDM, Cornblath DR, Hughes RA. et al. Electrophysiological 24. Kaida K, Morita D, Kanzaki M. et al. Ganglioside complexes as new classification of Guillain-Barré syndrome: clinical associations and target antigens in Guillain-Barré-syndrome. Ann Neurol 2004;56:567- outcome. Ann Neurol 1998;44:780-788. 571. 35. Hughes RAC, Cornblath DR. Guillain-Barré syndrome. Lancet 2005; 25. Asbury AK, Cornblath DR. Assessment of current diagnostic criteria 366:1653-1666. for Guillain-Barré syndrome. Ann Neurol 1990; 27(suppl):21-24. 36. Yikilmaz A, Dogonay S, Gumus H. et al. Magnetic resonance 26. Leonhard SE, Mandarakas MR, Gondim FAA et al. Diagnosis and imaging of childhood Guillain-Barré syndrome. Childs Nerv System 2010; management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol 26(8):1103-1108. 2019;15(11):671-683. 37. Zuccoli G, Panigrahy A, Bailey A, Fitz C. Redefining the Guillain- 27. World Health Organization. Media Centre. Guillain-Barré fact sheet. Barré spectrum in children: neuroimaging findings of cranial nerve Disponível em: < involvement. Am J Neuroradiol 2011; 32:639-642. http://apps.who.int/iris/bitstream/handle/10665/204587/WHO_ZIKV_MO 38. Lawn ND, Fletcher DD, Henderson RD. et al. Antecipating C_16.4_spa.pdf;jsessionid=EAE37468B08E30D3D4D6B9DA2236F7D mechanical ventilation in Guillain-Barré syndrome. Arch Neurol F?sequence=1 />. Acesso em 15. abr. 2018. 2001;58:893-898. 28. Yuki N, Kokubun N, Kuwabara S. et al. Guillain-Barré syndrome 39. Verboon C, van den Berg B, Cornblath DR. et al. Second IVIg associated with normal or exaggerated tendon reflexes. J Neurol course in Guillain-Barre syndrome with poor prognosis: the non- 2012;259:1181-1190. randomised ISID study. J Neurol Neurosurg Psychiatry 2020;91:113– 29. Kuitwaard K, Bos-Eyssen ME, Blomkwist-Markens PH, van Doorn P. 121. Recurrences, vaccinations and long-term symptoms in GBS and CIDP. 40. Hughes RA, Swan AV, van Doorn PA. Treatment dilemas in Guillain- J Peripher Nerv Syst 2009;14:310-315. Barré syndrome. J Neurol Neurosurg Psychiatry 2017;88:346-352. 30. Allen JA. Chronic demyelinating polyneuropathies. Continuum 41. Hughes RA. Plasma Exchange versus intravenous immunoglobulin (Minneap Minn) 2017;23(5):1310-1331. for Guillain-Barré syndrome. Ther Apher 1997;1(2):129-130. 31. Kleyweg RP, van der Meché FG. Treatment related fluctuations in 42. Chevret S, Hughes RAC, Annane D. Plasma exchange for Guillain- Guillain-Barré syndrome after high-dose immunoglobulins or plasma- Barré syndrome. Cochrane Database Syst 2017;2:1-54. exchange. J Neurol Neurosurg Psychiatry 1991;54:957-960. 32. Wakerley BR, Uncini A, Yuki N. et al. Guillain-Barré and Miller Fisher syndromes – new diagnostic classification. Nat Rev Neurol 2014;10:537-544. 33. Brasil. Portal da Saúde. Protocolos Clínicos e Diretrizes Terapêuticas. Disponível em: < Rev Bras Neurol 56(3):21-24, 2020.

Infarto da artéria de Percheron: relato de caso Percheron´s artery infarction: a case report

João Carlos Cervelin¹, Matheus Muller da Rosa¹, Rafael Frizon², Denise Krieger³

RESUMO ABSTRACT

O infarto da artéria de Percheron é uma apresentação rara de acidente vascular cerebral, caracterizado principalmente por The artery of Percheron infarct is a rare presentation of stroke, isquemia talâmica bilateral. A apresentação clinica desse infarto featured mainly by thalamic bilateral ischemia. The clinical se apresenta de maneira inesperada e variável. Relata-se um presentation of this infarct is unexpected and variable. It's caso de um paciente masculino, idoso, acometido por diversas reported a case of a male patient, elderly, affected with several comorbidades, admitido na emergência em coma e hemiplégico comorbidities, admitted to the emergency in comatose state and a direita, demonstrando acometimento neurológico. A condição right hemiplegic, proving neurological involvement. The patient's clínica do paciente variou durante a hospitalização, clinical condition has fluctuated throughout the hospitalization apresentando melhora do quadro neurológico focal e midríase presenting improvement of the focal neurologic implication and fixa à direita, levando a um diagnóstico tardio. Paciente evoluiu right mydriasis, culminating in a lagged diagnosis. Patient's death ao óbito devido a causas não neurológicas. due to non neurologic causes.

Palavras-chave: Acidente Vascular Encefálico, Tálamo, Keywords: Stroke, Thalamus, Neurology. Neurologia.

______1-Acadêmicos de Medicina da Universidade do Planalto Catarinense (UNIPLAC), Lages, Santa Catarina, Brasil, 2-Neurologista do Hospital Nossa Senhora dos Prazeres- Lages, Santa Catarina, Brasil, 3-Professora Orientadora do curso de medicina da UNIPLAC, Lages, Santa Catarina, Brasil.

Autor correspondente: João Carlos Cervelin. E-mail: [email protected]

Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 21 Cervelin et al. Artéria de Percheron global, paralisia global de língua e palato. Com 15 dias, compreendia e obedecia comandos, afasia global, nistagmo INTRODUÇÃO horizontal, força grau 2 em MMII e grau 3 em MMSS, hiperreflexia global e Babinski bilateralmente. Evoluiu A Artéria de Percheron (AP), é uma variação com oligúria e azotemia, apresentando insuficiência renal anatômica atípica das artérias tálamo-perfurantes. aguda, sendo admitido em unidade de terapia intensiva Caracteriza-se como um tronco vascular que tem origem a para procedimento hemodialítico, posteriormente partir do segmento P1 da artéria cerebral posterior evoluindo a óbito. (ACP)1,2. Sua função consiste no fornecimento sanguíneo arterial para a regiões bilaterais paramedianas do tálamo, assim podendo se associar ou não ao infarto de mesencéfalo rostral.³ Acidentes vasculares encefálicos isquêmicos (AVEi) que cursam com a oclusão da AP são raros, e com maior grau de dificuldade para serem diagnosticados clinicamente,4, 5 devido a algumas semelhanças clinicas compartilhadas com outros AVEs isquêmicos. Nesse viés, relatamos o caso de um paciente idoso, com múltiplas comorbidades, apresentando alterações típicas e atípicas de infarto de AP. Figura 1: Corte Axial Flair demonstrando hipersinal simetricamente em região talâmica bilateral. Possível sequela isquêmica talâmica se estendendo bilateralmente para os RELATO DO CASO pedúnculos cerebrais.

Paciente masculino, caucasiano, 69 anos, admitido na emergência após ser encontrado caído na cama de sua residência,conforme família, ictus de 6 horas. Cursou com rebaixamento do nível de consciência, hemiplegia proporcionada à direita, anisocoria com midríase à direita e liberação esfincteriana completa. Evoluiu para intubação orotraqueal sendo realizado tomografia computadorizada de crânio (TC) e exame de líquor, ambos sem alterações. Previamente hipertenso (HAS) e diabético tipo 2 (DM2) há 10 anos, apneia obstrutiva do sono desde os 28 anos, Figura 2: Corte axial ilustrando áreas de restrição a episódio de infarto agudo do miocárdio há 2 anos, difusão de moléculas de água em regiões talâmicas mesiais. hemorragia digestiva baixa por diverticulite há 1 ano, e Dilatação ventricular compensatória pielonefrite há 1 mês. Dois dias após a internação não respondia a estímulos verbais, com movimentos de retirada à dor, DISCUSSÃO anisocoria à direita, sem abertura ocular mesmo após suspensão da sedação. Passados 4 dias de internação A AP é uma variante anatômica arterial manteve-se intubadoem ventilação mecânica, sem sedação, responsável pelo abastecimento paramediano do tálamo febril, sem abertura ocular, não contactuante, preservava observada em um terço da população. Deriva dos movimentos de retirada à dor, mobilizava os 4 membros. seguimentos da ACP, sendo descritas 4 variantes Transcorrida uma semana, apresentava-se anatômicas por Gerard Percheron em 1973. O AVEi de AP sonolento, com eventual abertura ocular e contactuação. consiste na variante IIb, caracterizada por uma única Mantinha anisocoria. Cursava com força reduzida artéria que bifurca-se para a região talâmica medial e 5,6 globalmente, sem déficit focal e com hiporreflexia. Fora porção rostral do mesencéfalo, bilateralmente. . Infarto realizado ressonância magnética sem contraste (RM) concomitante de mesencéfalo ocorre em 57% dos casos e 7 (figuras 1 e 2). Logo, a partir da avaliação radiológica, de tálamo anterior em 19%. A prevalência do infarto levou-se em consideração a probabilidade de AVEi da AP. dessa variante é de 0,1 a 0,3% dos casos de AVE, podendo 6 Após 10 dias, apresentou-se sonolento, com ser responsável por até 20% dos infartos talâmicos. . A traqueostomia, abertura ocular ao estímulo doloroso, oclusão da AP ocorre, principalmente, por microangiopatia fraqueza motora difusa, sem déficit focal, hiporreflexia e cardioembolismo. Os principais fatores de risco incluem 22 HAS, DM2 e Fibrilação Atrial.7 As duas primeiras suspeitos de acometimento de AP, devem ser considerados condições eram doenças de base no paciente relatado há para trombólise ou intervenção vascular, semelhante ao pelo menos 10 anos. tratamento de rotina para o infarto isquêmico.1,8, 10 Dado as funções neurológicas talâmicas e as diferentes áreas possíveis de acometimento, o infarto pode manifestar-se clinicamente de maneiras inesperadas, CONCLUSÃO variando entre 7 padrões: alterações do estado mental, alteração comportamental e da memória, afasia/disartria, Descreve-se um caso AVEi de AP, incomum entre anormalidades do movimento ocular, déficits motores, as injúrias isquêmicas cerebrais. O quadro clínico deste sinais e sintomas cerebelares e outras apresentações paciente foi compatível com as informações literárias. clínicas inespecíficas como hipersonia, tremores, Nota-se a importância científica de relatar este evento a convulsões e hipertermia. O paciente do relato apresentou fim de alertar para o diagnóstico diferencial dos inúmeros grave alteração do estado mental, variando entre coma e tipos de AVEi. estupor, além de deficits motores e afasia que predizem o acometimento paramediano bilateral de tálamo, sem DECLARAÇÃO CONFLITO DE INTERESSES: comprometimento de mesencéfalo rostral. Casos Nenhum conflito. semelhantes evoluem desfavoravelmente em 75% dos pacientes.2, 4 DECLARAÇÃO DE FINANCIAMENTO: Embora o rebaixamento do nível de consciência Recursos próprios. seja considerado um achado neurológico ''não focal'', os AVEs podem causar coma agudo.8 Coma, disartria, COMITÊ DE ÉTICA hipersonia e desorientação ocorrem pelo acometimento das Trabalho aprovado no Comitê de Ética em fibras do sistema reticular ativador ascendente presentes na Pesquisa da Faculdade de Medicina da Universidade do área medial talâmica, ocasionando desconexão entre Planalto Catarinense – Lages: Instituição preponente: tálamo e córtex.4, 7 Logo, o diagnóstico de AVE de AP Universidade do Planalto Catarinense – UNIPLAC. CAAE: deve ser considerado na emergência em pacientes 25235619.8.0000.5368. comatosos, sem associação a fatores metabólicos ou farmacológicos.8, 9 A tríade clássica da doença inclui hipersonia, paralisia ocular vertical e síndrome amnésica, estando apenas a hipersonia presente no paciente.3, 10 Não foi possível a avaliação da síndrome amnésica pela falta de verbalização do paciente. Apresentou midríase à direita, diferentemente do paciente relatado por Kichloo com midríase à esquerda.4 A perda dos esfíncteres foi singular frente a outros relatos. O paciente apresentou na admissão um quadro clínico com características típicas de AVEi. No entanto, as alterações do nível de consciência, associadas a recuperação parcial da força ao longo da internação levantaram a suspeita de um AVEi de tálamo. Os diagnóticos diferenciais a serem considerados para esta condição incluem: Trombose venosa cerebral, infarto de artéria basilar, encefalopatia de Wernicke, síndrome de Korsakoff, infecções, intoxicações, câncer e mielinólise osmótica. 1, 3, 4, 5, 6, 8Portanto, a presença de artéria basilar pérvia, infarto talâmico bilateral medial na RM, associado à apresentação clínica, estreitaram o diagnóstico para infarto de AP. Análises completas acerca do tratamento para oclusões arteriais foram realizadas amplamente na história médica, mas não para infarto de AP.7 Se identificado dentro das primeiras horas, pacientes com AVEi agudo,

Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 23 REFERÊNCIAS 7.Li X, Agarwal N, Hansberry DR, Prestigiacomo CJ, Gandhi CD. 1.Fagundes-Pereyra WJ, Furtado AN, Barcelos FM, Motta J. Contemporary therapeutic strategies for occlusion of the artery of Bilateral Thalamic Infarction by Percheron Artery. J BRAS Percheron: a review of the literature. Journal of Neurocirurg. 2014., 25 (1): 20-23. NeuroInterventional Surgery. 2014; 7(2): 95–98 2.Xu Z, Sun L, Duan Y, Zhang J, Zhang M, Cai X. Assessment of 8.Wong ML, Edlow JA. Artery of Percheron Stroke.The Journal of Percheron infarction in images and clinical findings. Journal of Emergency Medicine.2018; 55(1); 114–117. the Neurological Sciences.2017; 383: 87–92. 9.Galnares-Olalde JA, León-Mayorga Y, Halabe-Cherem J, 3.Valdivieso EP, Villanueva HA, Almaguer JED, Tapia KA , Pérez Rubalcava-Ortega J, Alegría-Loyola MA. Infarto talámico bilateral YC. Infarto bitalámico en el área de la arteria de Percheron. Rev secundario a oclusión de la arteria de Percherón. Med. Interna. Fac Med. 2018; 61(5): 1. Disponível 2018; 34(1): 1. Disponível em:http://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S em:http://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S 0026-17422018000500021#B5 0186-48662018000100018 4.Kichloo A, Jamal SM., Zain E-A, Wani F, Vipparala N. Artery of 10.Weerasinghe WS, Kularatne SAM, Pathirage LPMMK. Percheron Infarction: A Short Review. Journal of Investigative Paramedian thalamic syndrome due to occlusion of the artery of Medicine High Impact Case Reports. 2019; 7: 1-6. Percheron: A rare case of stroke. Ceylon Medical Journal, 2019; 5.Khanni JL, Casale JA, KoekA , et al. Artery of Percheron Infarct: 64(1): 30-1. An Acute Diagnostic Challenge with a Spectrum of Clinical Presentations. Cureus. 2018; 10(9): 1-9. 6.Quirland HM, González VL. Infarto de la arteria de Percheron: reporte de un caso y revisión de la literatura. Rev Argent Radiol. 2018., 82(04): 184-186.

24 Rev Bras Neurol 56(3):25-28, 2020.

Historical note Creutzfeldt-Jakob disease: one hundred years of participation in the design of the transmissible spongiform encephalopathies Doença de Creutzfeldt-Jakob: cem anos de participação no desenho das encefalopatias espongiformes transmissíveis

M. da Mota Gomes 1

ABSTRACT RESUMO

Creutzfeldt and Jakob's disease (CJD) has its initial A doença de Creutzfeldt e Jakob (CJD) tem seu milestone in the publication issued 100 years ago marco inicial na publicação emitida há 100 anos que that precipitated its better clinical-pathological and precipitou seu melhor entendimento clínico- etiological understanding. Now, it is established that patológico e etiológico. Agora, está estabelecido que it belongs to the group of the prion diseases or pertence ao grupo da família das doenças de príons transmissible spongiform encephalopathies family. ou encefalopatias espongiformes transmissíveis. A CJD is itself divided into several types, the most própria CJD se divide em vários tipos, sendo o mais common being sporadic that is further subdivided comum o esporádico que também se subdivide de according to the anatomoclinical expression, but acordo com a expressão anatomoclínica, mas mainly due to its aetiology regarding prionic protein principalmente devido à sua etiologia em relação à or genotype. proteína priônica ou genótipo.

Key words- Creutzfeldt-Jakob disease, spongiform Palavras-chave- Doença de Creutzfeldt-Jakob, encephalopathies, prion protein encefalopatias espongiformes, proteína prioníca

______Associate Professor, Laboratory of History of Psychiatry, Neurology, and Mental Health. Institute of Psychiatry. Institute of Neurology, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil Orcid: https://orcid.org/0000-0001-8889-2573

Corresponding author: [email protected]

Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 25 M. da Mota Gomes. Creutzfeldt-Jakob disease. disseminated foci of degeneration' because he believed that it resembled multiple sclerosis and amyotrophic lateral sclerosis4. INTRODUCTION However, retrospective postmortem examination by Masters, Gajdusek, and Richardson, apud Pearce8, revealed the cardinal One hundred years ago, a paper was published. It vacuolation of the cerebral cortex and cerebellum in only two inaugurated the process of recognition of the of Jakob's five patients. Consequently, only these cases are now clinicopathological configuration of a new fatal and peculiar considered to be under the label of CJD. Besides, Creutzfeldt's disease, one of the spongiform encephalopathies. That disease, case is not deemed to be a CJD case, as clinical and nowadays known as Creutzfeldt-Jakob disease (CJD), is pathological findings do not represent the transmissible characterized by rapidly progressive dementia, frequently spongiform encephalopathy group6. associated with behavioural and visual disturbances, ataxia, Surprisingly, Jakob gave credit to Creutzfeldt for extrapyramidal features and myoclonus 7. Many years passed describing the syndrome before him, as he concluded that before the definition of its aetiology, and complete Creutzfeldt's case refers to a "nosologically very closely configuration assigned it as one of the most prevalent human connected if not identical affection", apud Katscher6. prion diseases1. Furthermore, Walther Spielmeyer reinforced This article is published to honour the centenary of Creutzfeldt's pioneering when, for the first time, used the term 4 that publication, by Hans Gerhard Creutzfeldt, in 1920, which "Creutzfeldt-Jakob disease", in 1922. In fact, Duckett & Stern details what supposedly would be the first case of sporadic believe that it is not possible to justify the use of the term CJD (sCJD). 'disease' as Spielmeyer did. That would happen because of the neuropathological school rivalries between Spielmeyer and Jakob. Spielmeyer's gesture in creating this 'disease' was unfortunate because of the confusion it ultimately caused. RECOGNITION OF A NEW DISEASE Duckett & Stern4 declare that neither Creutzfeldt nor Jakob used the eponyms Creutzfeldt Jakob disease or Jakob- Hans Gerhard Creutzfeldt took the vanguard of the Creutzfeldt disease. Both concluded that they had described a new disease definition for the reasons that follow. From 1912 neuropathological syndrome associated with features of many to1914, he worked with Alois Alzheimer at the University of disorders, rather than a disease itself, which still appears Breslau, and at World War I (1914–1919), he was a German vindicated4. naval medical officer. Afterwards, from 1919-20, he worked In 1929, Heidenhain, apud Pearce8, reported three with Walther Spielmeyer in the Psychiatry Research Institute patients, two of them with cortical blindness and significant in Munich, but soon he moved to the Charité Hospital in Berlin, spongy changes that became known as the Heidenhain variant where he worked with Karl Bonhoeffer. After 14 years, he of CJD. returned to Kiel in 1938 to become rector of the psychiatry and Over time, it was recognized that the archetypal human neurology clinic at the Christian-Albrechts University (1938– prion disease is the sCJD, as first described by Jakob. 1945). He then worked until retirement in 1955 at the Institute 8,4 Many unfoldings happened in studying this intriguing of Psychiatry, Munich . Despite the environment at the time disease, and today it is known as one of the prion diseases. of Nazi hegemony, Creutzfeldt has never been a member of the Figure 3 presents some landmarks of the acquisitions on National Socialist Party. He did not act in the interest of the spongiform encephalopathies, including mainly CJD matters. regime; on the contrary, he prevented patients considered a burden for the state to be killed in the Nazi euthanasia program4,5,8. In the 1920s, Creutzfeldt (figure 1) and Alfons Jacob (figure 2) independently described a syndrome characterized by progressive dementia, ataxia, tremors, and death. Creutzfeldt, in 19203, reported a detailed case of a 23- year-old woman with an unusual combination of neurological signs and pathological findings under the title "Über eine eigenartige herdförmige Erkrankung des Zentralnervensystems" ("About a peculiar focal disease of the central nervous system"). In that report, Creutzfeldt distinguished the condition from multiple sclerosis and termed it "pseudosclerosis”. He concluded with caution that it was a unique disease process that occurred in a young woman with the following characteristics: 1. Unknown cause (perhaps family predisposition), 2. Episodic course with remissions, 3. Cortical symptoms in the domain of motor and sensory centres (spasms and hyperalgesia), 4. Psychic symptoms of amental nature [dementia] with a predominance of psychomotor phenomena, 5. Progressive course, 6. Non-inflammatory focal destruction of the cerebral cortex with neuronophagy and reparative glial proliferation (sometimes with vascular proliferation), 7. Diffuse non-inflammatory cell disease with cell loss in the domain of almost all grey matter. In 1921, Jakob published three articles, "On peculiar illnesses of the central nervous system with remarkable anatomical findings"6, and also a separate article (1923), where he described five cases of patients who died of rapidly Figure 1. Hans Gerhard Creutzfeldt (June 2, 1885 Harburg upon Elbe, progressive dementia (between a few weeks and a year after Hamburg -December 30, 1964, Munich, aged 79) and his 1920 paper3,4,8. starting more severe symptoms)6. Jakob designated that disease as a 'Spastic pseudosclerosis encephalopathy with 26 2-Genetic (Genetic Creutzfeldt-Jakob disease -gCJD, fatal familial insomnia-FFI, and Gerstmann-Sträussler-Scheinker syndrome-GSS); 3-Acquired (Kuru, iatrogenic CJD-iCJD, variant CJD-vCJD). Regarding these last ones: Kuru (in Papua New Guinea, from the ingestion of infected brain tissue during cannibalistic mortuary rites) or caused by previous medical or surgical treatments - iCJD, or a vCJD, as a zoonosis causally linked to the bovine spongiform encephalopathy 9. This last type, vCJD, occurs in patients with a median duration of illness between 13 to 14 months, and the median age of death is 28 years. vCJD was identified in 1996 as a novel human prion disease, and its appearance in the United Kingdom led to the hypothesis that this disease was linked to the so- called bovine spongiform encephalopathy 9. The archetypal human prionic disease is sCJD. However, various sCJD distinct clinical patterns phenotypes vary based on classification. Individuals who display features of sCJD variant may also have unique genetic or other disease- altering variables. Appleby et al.1 give support to the existence of 5 sCJD variants in addition to the 3 previously reported variants: classic CJD (n=11); Heidenhain (n=15); and Figure 2. Alfons Maria Jakob (July 2 1884, Aschaffenburg/Bavaria-October 17, 1931, Hamburg, aged 47). He had worked at the Friedrichsberg State Hospital Oppenheimer-Brownell (n=8); along with 2 neuropsychiatric in Hamburg, 1911, in the pathological-anatomical laboratory led by Theodor variants, cognitive (n=28) and affective (n=13). Depending on Kaes, and succeeded him. He was trained by Alzheimer in Munich in 1911– 19124,8. genotypes and prion proteins, these phenotypes may be divided into six subtypes largely correlated with variables as age at onset of symptoms 9. CJD is now recognized as a group of diseases that are most often characterized by rapidly progressive dementia with myoclonus, ataxia, and often seizures. sCJD affects middle- aged individuals of both sexes. The time from symptom onset until death is variable, ranging from weeks to years. sCJD, which accounts for the majority of cases, results in the most rapid decline. However, the disease course of sCJD variants as Heidenhain and Oppenheimer-Brownell may arise later than the classical sCJD. The former is characterized by visual symptoms at onset, including diplopia, visual field defects, hallucinations, and/or cortical blindness. The Oppenheimer-Brownell variant presents with ataxia at the onset. They have median survival times that maybe the double of that of a typical sCJD. In general, much has already been discovered about the origins of sCJD. It was connected to the spontaneous misfolding of the normal prion protein (PrPC) into a disease- Sc Figure 3. Marcos das descobertas de doenças por príons e a associated isoform (PrP ), supposedly due to a random CJD incorporada: clinical and pathological characteristics, mutation.This abnormal misfolding leads to brain damage and interspecies transmission and the unusual causal agents 9,10. the characteristic symptoms of the disease. Besides, mutations in the normal prion protein encoded by the PRioN Protein (PRNP) gene are linked to genetically inherited prion diseases, CREUTZFELDT–JAKOB DISEASE including genetically associated CJD, GSS and FFI. PrPC is NOWADAYS encoded by a single gene, which is located in chromosome 20 and is known as the PRNP gene 7. Prion diseases or transmissible spongiform encephalopathies encompass human and animal illnesses. Concerning the human ones, three groups are acknowledged: 1-Sporadic (Sporadic Creutzfeldt-Jakob disease -sCJD, sporadic fatal insomnia, and variably protease-sensitive prionopathy); Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 27 FUNDING STATEMENT: The author did not receive funding for this work.

DECLARATION OF AUTHOR COMPETING INTERESTS: The author declares no competing interests.

REFERENCES 1. Appleby BS, Appleby KK, Crain BJ, Onyike CU, Wallin MT, Rabins PV. Characteristics of established and proposed sporadic Creutzfeldt-Jakob disease variants. Arch Neurol 2009;66(2):208-215. 2. Centers for Disease Control and Prevention. Creutzfeldt-Jakob Disease, Classic (CJD), Diagnostic Criteria. https://www.cdc.gov/prions/cjd/diagnostic-criteria.html 3. Creutzfeldt HG: Über eine eigenartige herdförmige Erkrankung des Zentralnervensystems. Zeitschrift für die gesamte Neurologie und Figure 4. CJ diseases hallmarks and diagnostic criteria: sporadic CJD2. Psychiatrie 1920;57:1–18. 4. Duckett S, Stern J. Origins of the Creutzfeldt and Jakob concept. J Hist Neurosci. 1999;8(1):21-34. 5. Illert M, Schmidt M. Hans Gerhard Creutzfeldt (1885–1964) in the In short, the designation CJD would assume that the Third Reich: A reevaluation. Neurology 2020;95(2):72-76. aetiology or its unmistakable anatomopathological findings 6. Katscher, F. It's Jakob's disease, not Creutzfeldt's. Nature 1998;393, would be known. This definition would represent the highest 11. https://doi.org/10.1038/29862 level of conceptual understanding of the disease. On the 7. Leemans M. Prion diseases. Anaesthesia & Intensive Care contrary, a syndrome, as in fact the precursor authors pointed Medicine 2019;21. 10.1016/j. out, would have been better applied to the series of symptoms 8. Pearce JM. Jakob-Creutzfeldt disease. Eur Neurol. 2004;52(3):129- and signs that occurred together or varied over time. 131. Consequently, at the time of Creutzfeldt and Jakob, when 9. Will RG, Ironside JW. Sporadic and Infectious Human Prion prionic diseases were not yet known, let alone their typical Diseases. Cold Spring Harb Perspect Med 2017;7(1):a024364. 10. anatomopathological patterns, the term, syndrome, would fit Zabel MD, Reid C. A brief history of prions. Pathog Dis 2015;73(9):ftv087. better in what is now known as sCJD4. These pioneers, however, contributed to the paving of this rich route that configured the family of spongiform encephalopathies. Congratulations to them on the 100th anniversary of their first publication on their "pseudosclerosis".

28 Rev Bras Neurol 56(3):29-30, 2020. Historical note Zeus’ pain: did he experience a thunderclap headache?

A dor de Zeus: fora ele acometido por uma cefaleia em trovoada?

Giuliano da Paz Oliveira1,2,3, Thiago Mendes Barbosa4, Giordanno Santana Mazza1; Raimundo Pereira da Silva-Neto1

ABSTRACT RESUMO

Zeus is known as the king of the gods and god of the sky. His Zeus é conhecido como rei dos deuses e deus dos céus. Tem attributes are lightning and thunder and he is often depicted como atributos os raios e os trovões e é frequentemente about to hurl them. According to Greek mythology, Zeus representado prestes a lançá-los. De acordo com a mitologia molested the titan Metis and decided to swallow her when she grega, Zeus molestou a titã Métis e resolveu engoli-la grávida, o was pregnant, which resulted in an excruciating headache, que resultou em uma cefaleia excruciante, apenas aliviada após only relieved after a craniotomy performed using Hephaestus’ uma craniotomia realizada por meio do machado de Hefesto. O axe. The result of this procedure was the birth of Athena, fruto deste procedimento foi Atena, filha de Zeus. Realizamos Zeus’ daughter. We conducted a combined analysis of some uma análise combinada utilizando escritos mitológicos clássicos writings such as the classical mythological poem Theogony by como o poema Teogonia de Hesíodo, além de outros livros Hesiod, and some other books that examine and retell myths sobre mitologia e artigos médicos que tratam deste tema, para and legends of ancient Greece, with medical papers on this tentar caracterizar a cefaleia de Zeus. Seria possível enquadrar topic, trying to characterize Zeus’ headache. Would it be a cefaleia de Zeus no grupo das cefaleias em trovoada? possible to fit Zeus’ headache into the group of thunderclap headaches? Palavras-chave: Cefaleia, Mitologia, Transtornos de cefaleia secundários, Grécia Antiga Keywords: Headache, Mythology, Secondary headache disorders, Ancient Greece

______1-Universidade Federal do Delta do Parnaíba (UFDPar), Parnaíba-PI, Brazil, 2Neurology and Neurosurgery Department, Universidade Federal de São Paulo (UNIFESP), São Paulo-SP, Brazil, 3Faculdade de Ciências Humanas, Exatas e da Saúde do Piauí, Instituto de Educação Superior do Vale do Parnaíba, Parnaíba-PI, Brazil, 4-Hospital de Urgências de Teresina (HUT), Department of Anesthesiology, Teresina-PI, Brazil.

Corresponding author: Giuliano da Paz Oliveira, 2819 São Sebastião Av. Fátima, Parnaíba-PI. 64001-020, Brazil, e-mail: [email protected].

Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 29 Oliveira et al. Zeus’ pain: a thunderclap headache? Pallas. In the work in question, Byron refers to the goddess Athena as Pallas and describes her birth as follows: According to Greek mythology, Zeus assumed the Can tyrants but by tyrants conquered be, governance of Olympus after defeating the Titans, who were And Freedom find no champion and no child led by his father Cronos, in a battle that lasted ten years1. Zeus Such as Columbia saw arise when she sexually molested the Titan Métis, one of his first actions after Sprung forth a Pallas, armed and undefiled? taking over the Olympus. As an oracle announced that the son Or must such minds be nourished in the wild, of this connection would dethrone Zeus, he swallowed Métis Deep in the unpruned forest, midst the roar when she was about to give birth to their daughter Athena, Of cataracts, were nursing nature smiled fulfilling the prophecy that condemned children to imitate their On infant Washington? Has Earth no more parents1,2. The king of the gods then had an excruciating Such seeds within her breast, or Europe no such shore? headache, that was relieved by a craniotomy performed by Hephaestus with his ax (Figure 1). The result of this procedure It is important to mention the possibility that Zeus' was the birth of Athena, goddess of wisdom, born directly headache could be included in the thunderclap headache group, from the brain of Zeus2,3. considering its onset and intensity, secondary to the imminent birth of Athena. In a final allusion to the work Theogony, the relation of Zeus' moments of anger with lightning and thunder is mentioned1: And now Zeus no longer held back his strength. His lungs seethed with anger and he revealed All his power. He charged from the sky, hurtling Down from Olympos in a flurry of lightning, Hurling thunderbolts one after another, right on target, From his massive hand, a whirlwind of holy flame.

Imagining Zeus´ anger due to the discomfort caused by the headache, we assume that is quite possible that Athena´s birth day was filled with thunder and lightning hurled by him. Could Zeus, the king of gods, the god of the sky, lightning and thunder have been affected by a thunderclap headache?

DECLARATIONS OF INTEREST: None. Figure 1: Illustration of Zeus with a severe headache that would only be relieved by a craniotomy performed by Hephaestus with his ax. According to FUNDING STATEMENT: Greek mythology, that would be how Athena, the warrior-goddess, was born. This research did not receive any specific grant. The figure was drawn by Giordanno Santana Mazza. REFERENCES The definition of the characteristics of Zeus’ headache comes up against the interpretation of the authors 1. Athanassakis A. Hesiod Theogony, Works and Days, Shield. 2nd ed. Baltimore, MD: The John Hopkins University Press; 2004. and the difficulties in translating the original mythical writings. 2. Schwanitz D. Cultura geral: tudo o que se deve saber. 2 ed. Brasiliense et al. described Zeus' headache as a terrible Tradução de Rose BS, Sousa EA, Lohbauer IA, Jannini, K. São experience characterized by progressive pain, attributed to an Paulo: WMF Martins Fontes, 2009, 6. 3 3. Brasiliense LB, Safavi-Abbasi S, Crawford NR, Spetzler RF, expansive intracranial process caused by Athena's growth . Theodore N, The legacy of Hephaestus: the first craniotomy. Maranhão-Filho and Vincent described it as a continuous, Neurosurgery. 2010;67(4): 881-4. intense and almost maddening headache4. Since we are not 4. Maranhão-Filho P, Vincent MB. Uncommon headaches: from Zeus to Harry Potter. Rev Bras Neurol. 2010;46(3):5-13. able to perform an anamnesis with Zeus, we conducted a 5. McGann, JJ. Byron: The Complete Poetical Works, ed. with combined analysis of some writings, trying to rescue the Introduction, Apparatus, and Commentaries. Oxford: Clarendon features of Zeus´ headache. Press, The Oxford English Texts series, v.2, 1980–1993. Hesiodo, in Theogony, describes the moment when Zeus swallows Métis in this way1: Zeus, king of gods, made Metis his first wife, most knowledgeable of gods and immortal men. But when she was about to bear Athena of gleaming eyes, then by a cunning he deceived her mind with coaxing words and put her down into his womb, in accord with the advice of Gaia and starry Ouranos. Continuing, describes the birth of Athena1: From his own head he gave birth to owl-eyed Athena, The awesome, battle-rousing, army-leading, untiring Lady, whose pleasure is fighting and the metallic din of war. Lord Byron in his work Childe Harold´s Pilgrimage5, mentions the birth of Athena, also known as Pallas Athena or 30 Rev Bras Neurol. 56(3):31-32, 2020

Imagens em Neurologia

Guido da Vigevano and the first human neuroanatomical figures Guido da Vigevano e as primeiras figuras neuroanatômicas humanas

Eliasz Engelhardt

Guido da Vigevano (1280–1349), his The interest on anatomy has a long history, and the dissection of varied kinds of animals was performed contemporaneous and pupil, followed him. He also by outstanding naturalists and anatomists along the ages. performed human dissections, and provided the first The philosopher and naturalist Aristoteles (4th anatomical book with illustrations, the Anathomia century BC) must be cited for his studies in this field, designata per figuras (1345), with 24 plates, of which 6 followed by many others. However, the name of have been lost, and of the 18 remaining, 6 plates were Claudius Galenus of Pergamon (2nd -3rd century AD) about neuroanatomy.2-4 These illustrations, although stands out as one of the most important anatomist ever, schematic and rudimentary, can be considered to be the with his findings lasting for over one and a half first neuroanatomical drawings in the history of millennium. neuroscience.2 The forbidden dissections of human corpses, Here, one of the illustrations, plate XVI of mostly for religious reasons, was overruled for a short Vigevano’s Anathomia, is displayed (Figure). time in Alexandria (3rd-4th century BC), and the physicians and anatomists Herophilus of Chalcedon and Erasistratus of Chios had the opportunity to perform studies of the human body. Unfortunately, most of their findings were lost, only a few remaining for posterity. Such prohibition lasted until the late Middle Ages, when dissections of human corpses were allowed again.1,2 Mondinus de’ Liuzzi (ca 1270–1326) was the anatomists who produced the first known anatomical book, based on dissection of human cadavers, the Anathomia Mondini (1316), printed much later (1478), without illustrations.2

Figure. Plate XVI from the Anathomia of Guido da Vigevano (1345) showing the back of a dissected human corpse in a standing position, displaying the brain, the spinal cord, and the roots of the spinal nerves.2-4. [License Creative Commons Attribution 4.0 International https://commons.wikimedia.org/wiki/Category:Guido_da_Vige vano,_human_anatomy?uselang=fr]

______Instituto de Neurologia Deolindo Couto, CDA-IPUB, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil

Corresponding author: [email protected]

Revista Brasileira de Neurologia » Volume 56 » Nº 3 » JUL/AGO/SET 2020 31 Engelhardt. Vigevano – first neuroanatomical figures REFERENCES 1. Olry R. Medieval neuroanatomy: the text of Mondino dei Luzzi and the plates of Guido da Vigevano. J Hist Neurosci CONFLICT OF INTEREST 1997;6:113-123.DOI: 10.1080/09647049709525696 2. Di Ieva A, Tschabitscher M, Prada F, Gaetani P, Aimar E, Pisano P, Levi D, Nicassio N, Serra S, Tancioni F, Arosio M, The author declares that there is no conflict of Rodriguez Y Baena R. The neuroanatomical plates of Guido interest. da Vigevano. Neurosurg Focus 23 (1):E15, 2007. DOI: 10.3171/FOC-07/07/E15 3. Rengachary SS, Colen C, Dass K, Guthikonda K. Development of anatomic science in the late Middle ages: FUNDING STATEMENT the roles played by Mondino de Liuzzi and Guido da Vigevano. Neurosurgery 2008;65:787-794. DOI: 10.1227/01.NEU.0000324991.45949.E4 There is no financial support. 4. Vigevano, Guido da. L'Anathomia designata per figuras. 1345. [Retrieved from (08-08-2020): https://bvmm.irht.cnrs.fr/iiif/314/canvas/canvas-185973/view]

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