Frequency of Cranial Nerve Involvement in Patients with Guillain-Barre’ Syndrome … Rao Suhail Y. Khan et al

Original Article

Rao Suhail Y. Khan* Frequency of Cranial Nerve Involvement Sabir Ali ** Shahid Irfan*** in Patients with Guillain-Barre’ Faiza Naseem****

Syndrome

ABSTRACT *Assistant Professor of Objective: To determine the frequency of involvement in patients Neurology, Department of with Guillain-Barre’ Syndrome Neurology, PIMS Islamabad Study design: Cross sectional/observational study **Assistant Professor of Place and duration: This study was done in neurology ward of PIMS Islamabad Neurology, Medical Unit-I, and medical wards of Bahawal Victoria Hospital Bahawalpur from March 2014 to Bahawal Victoria Hospital Bahawalpur April 2015. ***Associate professor of Materials and methods: Patients of either gender presenting with the primary Medicine, Medical Unit-III, diagnosis of Guillain Barre Syndrome having age of 15 years or above and Bahawal Victoria Hospital admitted to neurology ward of Pakistan Institute of Medical Sciences Islamabad Bahawalpur and medical wards of Bahawal Victoria Hospital Bahawalpur were included in the **** Medical officer, Bahawal study. Diagnosis of Guillain Barre Syndrome was made on nerve conduction Victoria Hospital Bahawalpur studies. Facial palsy (unilateral or bilateral facial weakness), bulbar palsy

(, nasal regurgitation, slurring of speech, weakness of the soft palate and/or absent gag reflex) and need of mechanical ventilation within first two weeks of start of disease were recorded on preformed proforma. Results: 106 patients were included in the study. The mean age of patients was 34.75 years with standard deviation of 16.590 years. Out of 106 patients, 75 (70.75%) patients were male and 31 (29.25%) were female. 32 (30.19%) patients had palsy, 42 (39.62%) patients had bulbar palsy and 35 (33.02%) had need of mechanical ventilation. Conclusion: About one third of GBS patients have some degree of cranial nerve dysfunction during their illness. Bulbar palsy is more common followed by facial Address for Correspondence weakness. Dr. Sabir Ali, House no-13, BVH Key words: Guillain-Barre’ Syndrome, GBS, Bulbar palsy, facial palsy, colony, Bahawal Victoria Hospital Bahawalpur. respiratory weakness, mechanical ventilation Email: [email protected]

Introduction between 0.4-1.7 cases per 100,000 persons in one year.1 Guillain-Barre’ Syndrome (GBS) is a common cause of Core clinical feature of GBS is rapidly progressive acute onset weakness. It is an immune-mediated ascending weakness starting from lower limbs in the disease that is usually preceded by gastrointestinal or most of cases. Maximum weakness is reached within 2- respiratory tract infection in most of the cases. Various 4 weeks from the onset of illness.2 After reaching pathogens like Mycoplasma pneumonia, Campylobacter maximum weakness, patients may have a plateau jejuni, Epstein Bar virus and Cytomegalovirus are phase of disease lasting from several days to weeks or involved in its pathogenesis and immune response many months. This phase is followed by slower recovery directed against these organisms may cross react with phase of illness of varying duration of several months to myelin sheath and other neural tissues causing years. Half of patients may have different cranial nerves 1 weakness. GBS occurs in whole world with various involvement. In Europe, about a third of patients with proportions in all seasons. The incidence varies GBS are mildly affected and they keep on walking with mild difficulty.3 Combination of rapidly progressive

Ann. Pak. Inst. Med. Sci. 2015; 11(3): 115-118 115 Frequency of Cranial Nerve Involvement in Patients with Guillain-Barre’ Syndrome … Rao Suhail Y. Khan et al

weakness in legs and arms, absent reflexes and protein pregnancy, systemic illness like diabetes, chronic renal cell dissociation on CSF examination, is a hallmark for failure, hereditary neuropathies and toxin exposure, the diagnosis of GBS.4 hypokalemic periodic paralysis (serum potassium Facial nerve is commonly involved in GBS, occurring in <3.5mEq/L) and presence of pulmonary comorbidity on at least half of patients. Other cranial nerves like bulbar history &/or chest X-ray were excluded from the study. nerves, abducent and oculomotor nerves are less often The final diagnosis of Guillain Barre Syndrome in these affected.5 Miller Fisher Syndrome is a clinical variant of patients was made on the basis of clinical presentation GBS in which patients may have a triad of ataxia, (progressive symmetrical weakness in the arms and areflexia and ophthalmoplegia.6,7 (Bickerstaff) legs with or without sensory disturbances, encephalitis is another overlapping syndrome that hypoflexia/areflexia, in the absence of a CSF cellular presents with sudden onset of weakness having multiple reaction) and nerve conduction studies. Already cranial or peripheral nerve involvement. Later on designed proforma was used to record the detailed disturbances of consciousness and even coma may medical history, facial palsy (unilateral or bilateral facial occur in the course of this disease. Recognition of this weakness), bulbar palsy (dysphagia, nasal regurgitation, disease is important, because this illness may improve slurring of speech, weakness of the soft palate and/or after the start of treatment.8 absent gag reflex) and need of mechanical ventilation Diagnosis of GBS is generally made on history and within first two weeks of start of disease. Data was clinical examination. Nerve conduction studies (NCS) entered and analyzed by using SPSS 15.0 and electromyography (EMG) studies can be useful in Results the diagnosis of GBS. Delayed distal latencies, temporal 106 patients were included in the study. The mean age dispersion of waveforms, slowed nerve conduction of patients was 34.75 years with standard deviation of velocities, prolonged or absent F waves and H-reflexes 16.590 years. The minimum age of patients was 13 are characteristic findings for demyelination GBS. Low years, maximum age was 75 years, median age was 30 amplitude of compound muscle action potential (CMAP) years and mode age was 30 years. Out of 106 patients, may support axonal GBS. Needle EMG examination 75 (70.75%) patients were male and 31 (29.25%) were shows neuropathic pattern and it may normal in acute female. The frequency of facial palsy, bulbar palsy and nerve lesions. Lumbar puncture for cerebrospinal fluid need of mechanical ventilation was shown in figure no: (CSF) studies is recommended in acute phase of GBS 1. 9 and it shows albuminocytologic dissociation. CSF protein concentrations in patients with GBS are often normal in the first week, but may increase in more of the patients at the end of the second week.9 The aim of this study was to determine the frequency of cranial nerve involvement in patients with GBS in our local clinical setting which would help in early management of these patients and reduce the associated morbidity and mortality. Materials and Methods This is a cross sectional/observational study in the patients presenting with the primary diagnosis of Guillain Barre Syndrome (diagnosed on nerve conduction study) having age of 15 years or above and admitted to neurology ward of Pakistan Institute of Medical Sciences Islamabad and medical ward-I of Bahawal Victoria Figure no 1. Frequency of facial palsy, bulbar Hospital Bahawalpur during the period of March 2014 to palsy and mechanical ventilation April 2015. Patients of both genders were included.

Approval from the Hospital Ethics Committees of both Out of 106 patients, 22 (62.9%) male and 13 (37.1%) hospitals was taken prior to conducting the study. female patients had need of mechanical ventilation with Patients having a sensory level on clinical examination,

Ann. Pak. Inst. Med. Sci. 2015; 11(3): 115-118 116 Frequency of Cranial Nerve Involvement in Patients with Guillain-Barre’ Syndrome … Rao Suhail Y. Khan et al

insignificant p value of 0.209. Out of 106 patients, 29 involvement may occur to cause blood pressure (69%) male patients and 13 (31%) female patients had fluctuations and cardiac arrhythmias. With the bulbar weakness with insignificant p value of 0.754. progression of disease, sensory symptoms like Facial palsy in different gender of patients was shown in numbness, burning and tingling sensation may appear, figure no: 2. Frequency of facial palsy, bulbar palsy and but the picture is usually dominated by motor weakness. need of mechanical ventilation in different age groups Although the most common presentation is an was shown in table no: 1 ascending weakness, but the patients can presents with cranial muscles paralysis.10 In our study, the mean age of patients was 34.75 years with standard deviation of 16.590 years. 75 (70.75%) patients were male and 31 (29.25%) were female. Cranial nerve abnormalities were present in about 30- 39% patients. These results were comparable to the results of other studies. In a study conducted by Areeyapinan P et al11 showed that the most common presentation was motor weakness in 87% of patients, sensory symptoms were present in 78% of patients and facial weakness was present in 18% of patients. Electrophysiological studies showed demyelinating process in most of cases as compared to axonopathy. In another study conducted by Dhadke SV et al12 showed that younger age group was commonly affected by GBS. In this study, male to female ratio was high. Motor weakness was present in 39 out of 40 patients. Cranial nerve weakness was present in 25 out of 40 patients. Figure 2. Facial palsy in different gender of Facial and bulbar muscles were equally affected in 30% of patients. Reflexes were absent in all studied patients. patients Some studies were showing higher frequency of cranial

nerve involvement than the results of our study. In a Table no I. Facial palsy, bulbar palsy and study conducted by Netto AB et al13 showed that mechanical ventilation in different age groups respiratory involvement was present in 273 patients Age group of patients (190 patients were men and 83 patients women). Out of Total p- 273 patients with respiratory failure, bulbar palsy, 13-35 36-55 56-75 n=106 value years years years sensory involvement and symptomatic autonomic dysfunction were found in 68.1%, 32.2% and 26.4% Facial 15 12 5 32 palsy 0.113 respectively. 14.2% 11.3% 4.7% 30.2% 14 n=106 In another study conducted by Azim A et al showed Bulbar that the mean age of patients was 32.4±18.12 years. 24 12 6 42 palsy 0.720 Majority of patients were male. Autonomic dysfunction 22.6% 11.3% 5.7% 39.6% n=106 was present in 35% of patients and 64% patients Mechanical needed mechanical support. Almost similar results were 19 10 6 35 ventilation 0.733 15 17.9% 9.4% 5.7% 33% found in a study conducted by Yakoob MY et al with n=106 cranial nerve abnormalities in 88.2% patients, Discussion autonomic dysfunction in 61.8% patients and respiratory GBS is an acute demyelinating polyneuropathy with failure requiring mechanical ventilation in 56% of rapid onset (hours to days) of weakness. This usually patients. produces symmetrical weakness of lower and upper Durand MC et al16 included one hundred and fifty four limbs and axial musculature, which may progress to patients with GBS in the study and 22% patients were respiratory failure and swallowing problems. Autonomic subsequently required ventilation. Demyelinating GBS

Ann. Pak. Inst. Med. Sci. 2015; 11(3): 115-118 117 Frequency of Cranial Nerve Involvement in Patients with Guillain-Barre’ Syndrome … Rao Suhail Y. Khan et al was more common as compared to axonal GBS in 3. Korinthenberg R, Schessl J, Kirschner J. Clinical presentation and patients who required mechanical ventilation. Facial course of childhood Guillain-Barré syndrome: a prospective multicentre palsy and bulbar palsy were presumed clinical factors study. Neuropediatrics. 2007;38:10–17. causing the need of mechanical ventilation in patients of 4. van Doorn PA1, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. Lancet Neurol. Guillain-Barre’ Syndrome.17 2008;7(10):939-50. However, some studies were showing minimal cranial 5. Ito M, Kuwabara S, Odaka M. Bickerstaff ’s brainstem encephalitis and nerves involvement like a study conducted by Yuan CL Fisher syndrome form a continuous spectrum: clinical analysis of 581 18 et al who studied 49 cases of GBS, with non-specific cases. J Neurol. 2008;255:674–82. age distribution. Motor weakness was the most common 6. Yuki N. Infectious origins of, and molecular mimicry in, Guillain-Barré presentation. Cranial nerve abnormalities, autonomic and Fisher syndromes. Lancet Infect Dis. 2001;1:29–37. dysfunction and respiratory failure were minimal in their 7. Willison HJ. The immunobiology of Guillain-Barré syndromes. J patients. Peripher Nerv Syst. 2005;10: 94–112. 8. Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EF. Acute multiple cranial neuropathies without limb Anticipating mechanical ventilation in Guillain-Barre´ syndrome. Arch weakness may be considered as variant of Guillain- Neurol. 2001;58:893–8. Barre syndrome, which are immune-mediated 9. van der Meché FG, van Doorn PA. Guillain-Barré syndrome and processes that are triggered by various pathogens. chronic inflammatory demyelinating polyneuropathy: immune Isolated Bell's palsy may be present as a variant of mechanisms and update on current therapies. Ann Neurol. 1995;37 Guillain-Barré syndrome with recognized cell-mediated (suppl 1): S14–31. immunity against peripheral nerve myelin antigens. In 10. Newswanger DL, Warren CR. Guillain-Barré syndrome. Am Fam Bell's palsy and GBS, a viral infection or the reactivation Physician. 2004;69(10):2405-10. of a latent virus may provoke an immune reaction 11. Areeyapinan P, Phanthumchinda K. Guillain-Barre syndrome: a clinical study in King Chulalongkorn Memorial Hospital. J Med Assoc Thai. against peripheral nerve myelin sheath, leading to the 2010;93(10):1150-5. demyelination of cranial nerves, especially the facial 12. Dhadke SV, Dhadke VN, Bangar SS, Korade MB. Clinical profile of nerve. The prognosis of GBS is generally favorable with Guillain Barre syndrome. J Assoc Physicians India. 2013;61(3):168-72. a mortality of ten percent and significant number of 13. Netto AB, Taly AB, Kulkarni GB, Rao UG, Rao S. Mortality in patients is left with severe disability. mechanically ventilated patients of Guillain Barré Syndrome. Ann Conclusion Indian Acad Neurol. 2011;14(4):262-6. In conclusion, GBS is seen in all age groups but more 14. Azim A, Singhal S, Baronia AK, Gurjar M, Poddar B. Outcome of mechanical ventilation in patients of Guillain-Barre syndrome: An audit common in younger age group. About one third of GBS from a tertiary care centre. Sahel M J. 2013;16:48-51. patients have some degree of cranial nerve dysfunction 15. Yakoob MY, Rahman A, Jamil B, Syed NA. Characteristics of patients during their illness. Bulbar palsy is more common with Guillain Barre Syndrome at a tertiary care centre in Pakistan, followed by facial weakness. Mechanical ventilation, 1995-2003. J Pak Med Assoc. 2005 ;55(11):493-6 which is the final consequence of several clinical factors 16. Durand MC, Porcher R, Orlikowski D. Clinical and electrophysiological is required in many patients of Guillain-Barre’ predictors of respiratory failure in Guillain-Barre´ syndrome: a Syndrome. prospective study. Lancet Neurol. 2006;5:1021–8. 17. Doorn PAV, Ruts L, Jacobs BC. Clinical features, pathogenesis and References treatment of Guillain-Barre Syndrome. Lancet. Neurol. 2008;7:939-50. 1. Gonzalez-Suarez I, Sanz-Gallego I, Rodriguez de Rivera FJ, Arpa J. 18. Yuan CL, Tsou HK, Wang YJ, Tsai CP. Guillain-Barré syndrome: a Guillain-Barré syndrome: natural history and prognostic factors: a retrospective, hospital-based study. Zhonghua Yi Xue Za Zhi (Taipei). retrospective review of 106 cases. BMC Neurol. 2013;13:95. 2002;65(11):540-7. 2. Doorn PAV, Ruts L, Jacobs BC. Clinical features, pathogenesis and treatment of Guillain-Barre Syndrome. Lancet Neurol. 2008;7:939-50. .

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