DOCSLIB.ORG

Para-Infectious and Post-Vaccinal Encephalomyelitis

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Para-Infectious and Post-Vaccinal Encephalomyelitis Postgrad Med J: first published as 10.1136/pgmj.45.524.392 on 1 June 1969. Downloaded from Postgrad. med. J. (June 1969) 45, 392-400. Para-infectious and post-vaccinal encephalomyelitis P. B. CROFT The Tottenham Group ofHospitals, London, N.15 Summary of neurological disorders following vaccinations of The incidence of encephalomyelitis in association all kinds (de Vries, 1960). with acute specific fevers and prophylactic inocula- The incidence of para-infectious and post-vaccinal tions is discussed. Available statistics are inaccurate, encephalomyelitis in Great Britain is difficult to but these conditions are of considerable importance estimate. It is certain that many cases are not -it is likely that there are about 400 cases ofmeasles notified to the Registrar General, whose published encephalitis in England and Wales in an epidemic figures must be an underestimate. In addition there year. is a lack of precise diagnostic criteria and this aspect The pathology of these neurological complications will be considered later. In the years 1964-66 the is discussed and emphasis placed on the distinction mean number of deaths registered annually in between typical perivenous demyelinating encepha- England and Wales as due to acute infectious litis, and the toxic type of encephalopathy which encephalitis was ninety-seven (Registrar General, occurs mainly in young children. 1967). During the same period the mean annual The clinical syndromes occurring in association number of deaths registered as due to the late effects with measles, chickenpox and German measles are of acute infectious encephalitis was seventy-four- considered. Although encephalitis is the most fre- this presumably includes patients with post-encepha-copyright. quent complication, myelitis and polyradiculitis also litic Parkinsonism. During this 3-year period there occur. There is evidence that 'uncomplicated were five deaths from post-vaccinial encephalitis, measles' is associated with brain involvement in the while the mean number of deaths registered annually prodromal phase. Similar para-infectious disorders as due to 'encephalitis, myelitis and encephalomye- occur in association with other infections including litis, excluding acute infectious encephalitis' was 110. smallpox and influenza. To put these figures into perspective, in 1966 The post-vaccinal types of encephalomyelitis are 612 deaths were registered as due to motor neurone also considered with particular reference to post- disease, 836 to disseminated sclerosis, 7363 to motor http://pmj.bmj.com/ vaccinial encephalitis and rabies post-vaccinal vehicle traffic accidents and 18,554 to primary encephalitis. carcinoma of the lung. The pathogenesis of these disorders is discussed During 1966 the number of deaths from encepha- and it is concluded that there is direct viral invasion litis certified as secondary to infectious diseases was of the nervous system, followed by an antigen- fifteen for measles encephalitis (out of a total of antibody reaction. At times virus may remain latent eighty deaths attributed to measles) and two for within the nerve cell without destroying it. chickenpox encephalitis (from a total of seventeen on September 29, 2021 by guest. Protected deaths due to chickenpox). Introduction Notifications of infectious diseases in 1966 in- Although the majority of cases of encephalitis and cluded 343,642 cases ofmeasles, eighty-three cases of other neurological disorders which occur in associ- acute infectious encephalitis and 114 cases of post- ation with acute viral infections such as measles, infectious encephalitis. Miller (1964) estimated the develop some days after the onset of the exanthem, incidence of measles encephalitis as 1 per 1000 and in a few patients the neurological disorder is either this agrees with other figures (Miller, Stanton & simultaneous with or precedes the development Gibbons, 1956). On this basis one would have of the rash. It is, thus, more logical to describe these expected over 340 cases of measles encephalitis in conditions as para-infectious rather than post- 1966, and of course not all cases of measles are infectious. Similarly, although the encephalitis notified. The figure of 114 cases of post-infectious following vaccination against smallpox (post- encephalitis of all kinds in 1966 is certainly much vaccinial encephalitis) is the typical example of below the true incidence. encephalitis as a sequel to prophylactic inoculations, The death rate in measles encephalitis has varied the tern post-vaccinal should be used for the range but has often been estimated at about 20 % (McNair Postgrad Med J: first published as 10.1136/pgmj.45.524.392 on 1 June 1969. Downloaded from Para-infectious andpost-vaccinal encephalomyelitis 393 Scott, 1967). If, making allowance for non-notifica- pathy oftoxic type characterized by cerebral oedema, tion, there were up to 400 cases of measles encepha- vacuolization of neurones and neuronal degenera- litis in 1966, deaths from this disorder should have tion, especially in the cortex. This syndrome occurs been about eighty. This contrasts with the fifteen mainly in children under 2 years, but may occur in patients actually registered as having died from older children. Acute encephalopathy is also found measles encephalitis. in fatal cases of para-infectious neurological illness However inaccurate the statistics may be, it is in young children. Lyon, Dodge & Adams (1961) clear that post-infectious encephalitis and encephalo- described similar cases in infants and children, myelitis is a significant problem in the United without obvious precipitating cause, though in most Kingdom, as it is in the United States, where about cases there was a febrile illness of variable and 30 % of the total cases of encephalitis are in the post- unidentified type. The lesions in post-vaccinal and infectious group (McNair Scott, 1967). para-infectious encephalopathy resemble those fol- lowing status epilepticus, anoxic episodes or severe Pathology hypoglycaemia. For many years a distinction has been drawn between primary polioclastic encephalitis, involving Clinical aspects predominantly neurones of the grey matter, and due There are many reviews of the neurological to direct invasion by virus (e.g. rabies and arbovirus), syndromes occurring after vaccination and in and secondary myelinoclastic leuco-encephalitis. association with acute viral infections. Miller et al. Here the myelin sheaths of the white matter are the (1956) made a detailed study of the literature at that main site of damage with axis cylinders less affected, time, and discussed their own experience, while the and neurones largely spared (Miller, 1961). The review of McNair Scott (1967) included more recent second group is often, but not always, a sequel to a work. viral infection. Miller et al. (1956) emphasized that although the The early work of Tumbull & McIntosh (1926) majority of patients under consideration had showed that in post-vaccinial encephalitis the usual encephalitis, in some the spinal cord was the main underlying process is one of perivenous demyelina- site of the disease. Polyradiculitis was less common copyright. tion, mainly in relation to small veins in the white than these forms of central nervous system involve- matter. Similar changes were found in encephalitis ment. associated with measles, chickenpox and rubella In the para-infectious groups of disorders, the (Miller et al., 1956). distribution of the various syndromes was as follows Further studies led de Vries (1960) to consider (Table 1): that the classical change in post-vaccinial encepha- TABLE 1. Distribution of the various syndromes litis is one of perivenous demyelination, witlh an associated microglial reaction-'microglia encepha- German http://pmj.bmj.com/ litis'. In this type of encephalitis, which commonly Measles Chickenpox measles occurs 5-15 days after vaccination, the illness runs Encephalitis (%) 96 90 92 an acute course, and if recovery occurs there are Myelitis (Y.) 3 3 4 often few sequelae, though permanent neurological Polyradiculitis (%) 1 7 4 deficit is more common when the disorder is pri- latent marily a myelitis. Similar changes are found in the The average period between the acute infection and the neurological illness was shortest for para-infectious group of neurological disorders, and on September 29, 2021 by guest. Protected also in sporadically occurring acute disseminated rubella and longest for varicella, while measles held encephalomyelitis. an intermediate position. With each infectious In some patients acute haemorrhagic leuco- disease the latent period was shortest for cases of encephalitis occurs and Russell (1955) argued that encephalitis and longest for those of polyradiculitis this, and acute disseminated encephalomyelitis, are (Table 2). variants of a single pathological process. She referred TABLE 2. Latent period (days) to the literature on experimental allergic encephalitis German and discussed the possibility that the naturally Measles Chickenpox measles occurring acute disseminated encephalomyelitis and Encephalitis 4-7 6-3 3-8 acute haemorrhagic leuco-encephalitis are due to a Myelitis 5.5 8-3 6-7 process based on an allergic response to an antigenic Polyradiculitis 9 0 11-3 8-7 stimulus. de Vries (1960) emphasized that some children with There was a wide range of latent periods, and post-vaccinial encephalitis do not show the typical mention has already been made of the occasional demyelinating changes, but instead an encephalo- patient in whom the
Load more

Recommended publications
  • Chasing the Dragon, a Case of Leukoencephalopathy Associated with Heroin Inhalation
    Chasing the dragon, a case of leukoencephalopathy associated with heroin inhalation Daniel Cho MD1, Hani Nazha MD2, Kalin Fisher BS2 Author Affiliations: 1. Charleston Area Medical Center, Charleston, West Virginia 2. West Virginia University, Morgantown, West Virginia The authors have no financial disclosures to declare and no conflicts of interest to report. Corresponding Author: Hani Nazha MD West Virginia University Morgantown, West Virginia Email: [email protected] Abstract Although rare, toxic leukoencephalopathy (TLE) associated with heroin inhalation has been reported. “Chasing the dragon” may lead to progressive spongiform degeneration of the brain and presents with a large range of neuropsychological sequelae. This case is an example of TLE in a middle-aged white male with a history of polysubstance abuse. He presented with a three week history of progressive neuropsychological symptoms, including abulia, bradyphrenia, hyperreflexia, and visual hallucinations. He was initially suspected to have progressive multifocal leukoencephalopathy, however, JCV PCR was negative. MRI showed diffuse abnormal signal in the white matter, extending into the thalami and cerebral peduncles. Brain biopsy was performed, which revealed spongiform degeneration, and a diagnosis of TLE was made. The patient was then transferred to a skilled nursing facility. Clinical suspicion based on a thorough history and clinical exam findings is paramount in recognition of heroin-associated TLE. Although rare, heroin-inhalation TLE continues to be reported. As ‘chasing the dragon’ is gaining popularity among drug users, it is important for clinicians to be able to recognize this disease process. Keywords Opioid, Addiction, Heroin, Leukoencephalopathy Introduction Toxic leukoencephalopathy (TLE) associated with heroin abuse was first described in 1982.1 “Chasing the dragon" is a method of heroin vapor inhalation in which a small amount of heroin powder is placed on aluminum foil, which is then heated by placing a match or lighter underneath.
    [Show full text]
  • Balo's Concentric Lesions with Concurrent Features of Schilder's
    Article / Autopsy Case Report Balo’s concentric lesions with concurrent features of Schilder’s disease in relapsing multiple sclerosis: neuropathological findings Maher Kurdia,b, David Ramsaya Kurdi M, Ramsay D. Balo’s concentric lesions with concurrent features of Schilder’s disease in relapsing multiple sclerosis: neuropathological findings. Autopsy Case Rep [Internet]. 2016;6(4):21-26. http://dx.doi.org/10.4322/acr.2016.058 ABSTRACT Atypical inflammatory demyelinating syndromes are rare neurological diseases that differ from multiple sclerosis (MS), owing to unusual clinicoradiological and pathological findings, and poor responses to treatment. The distinction between them and the criteria for their diagnoses are poorly defined due to the lack of advanced research studies. Balo’s concentric sclerosis (BCS) and Schilder’s disease (SD) are two of these syndromes and can present as monophasic or in association with chronic MS. Both variants are difficult to distinguish when they present in acute stages. We describe an autopsy case of middle-aged female with a chronic history of MS newly relapsed with atypical demyelinating lesions, which showed concurrent features of BCS and SD. We also describe the neuropathological findings, and discuss the overlapping features between these two variants. Keywords Multiple Sclerosis; Atypical Inflammatory Demyelination; Balo’s Concentric Sclerosis; Schilder’s Disease; Pathology CASE REPORT A 45-year-old woman presented in another treatment. Brain magnetic resonance imaging (MRI) hospital with a history of generalized tonic clonic with gadolinium contrast showed space-occupying seizure and acute confusion. She was admitted to lesions in the right and left frontal white matter, with the intensive care unit for close observation.
    [Show full text]
  • MRI Brain in Monohalomethane Toxic Encephalopathy
    Published online: 2021-07-30 NEURORADIOLOOGY MRI brain in monohalomethane toxic encephalopathy: A case report Yogeshwari S Deshmukh, Ashish Atre, Darshan Shah, Sudhir Kothari1 Departments of Radiology, Star Imaging and Research Centre, Opp. Kamala Nehru Park, Joshi Hospital Campus, Erandwane, 1Neurology OPD, Poona Hospital and Research Centre, 27, Sadashiv Peth, Pune, Maharashtra, India Correspondence: Dr. Yogeshwari S. Deshmukh, Department of Radiology, Star Imaging and Research Centre, Opp. Kamala Nehru Park, Joshi Hospital Campus, Erandwane, Pune - 411 004, Maharashtra, India. E-mail: [email protected] Abstract Monohalomethanes are alkylating agents that have been used as methylating agents, laboratory reagents, refrigerants, aerosol propellants, pesticides, fumigants, fire‑extinguishing agents, anesthetics, degreasers, blowing agents for plastic foams, and chemical intermediates. Compounds in this group are methyl chloride, methyl bromide, methyl iodide (MI), and methyl fluoride. MI is a colorless volatile liquid used as a methylating agent to manufacture a few pharmaceuticals and is also used as a fumigative insecticide. It is a rare intoxicant. Neurotoxicity is known with both acute and chronic exposure to MI. We present the characteristic magnetic resonance imaging (MRI) brain findings in a patient who developed neuropsychiatric symptoms weeks after occupational exposure to excessive doses of MI. Key words: Methyl iodide; MRI brain; toxic encephalopathy Introduction can cause cranial neuropathy, pyramidal and cerebellar dysfunction, polyneuropathies, and behavioral changes. Methyl iodide (MI) is a monohalothane used as an organic chemical agent/methylating agent in the synthesis Few case reports have been described, however.[1‑5] As far of various pharmaceutical drugs. It is also used as a as we know, none of them mention abnormal magnetic fumigative pesticide commonly in greenhouses and resonance imaging (MRI) findings except one.[1] strawberry farming.
    [Show full text]
  • Neurological Disorders in Liver Transplant Candidates: Pathophysiology ☆ and Clinical Assessment
    Transplantation Reviews 31 (2017) 193–206 Contents lists available at ScienceDirect Transplantation Reviews journal homepage: www.elsevier.com/locate/trre Neurological disorders in liver transplant candidates: Pathophysiology ☆ and clinical assessment Paolo Feltracco a,⁎, Annachiara Cagnin b, Cristiana Carollo a, Stefania Barbieri a, Carlo Ori a a Department of Medicine UO Anesthesia and Intensive Care, Padova University Hospital, Padova, Italy b Department of Neurosciences (DNS), University of Padova, Padova, Italy abstract Compromised liver function, as a consequence of acute liver insufficiency or severe chronic liver disease may be associated with various neurological syndromes, which involve both central and peripheral nervous system. Acute and severe hyperammoniemia inducing cellular metabolic alterations, prolonged state of “neuroinflamma- tion”, activation of brain microglia, accumulation of manganese and ammonia, and systemic inflammation are the main causative factors of brain damage in liver failure. The most widely recognized neurological complications of serious hepatocellular failure include hepatic encephalopathy, diffuse cerebral edema, Wilson disease, hepatic myelopathy, acquired hepatocerebral degeneration, cirrhosis-related Parkinsonism and osmotic demyelination syndrome. Neurological disorders affecting liver transplant candidates while in the waiting list may not only sig- nificantly influence preoperative morbidity and even mortality, but also represent important predictive factors for post-transplant neurological manifestations.
    [Show full text]
  • Post-COVID-19 Acute Disseminated Encephalomyelitis in a 17-Month-Old
    Prepublication Release Post-COVID-19 Acute Disseminated Encephalomyelitis in a 17-Month-Old Loren A. McLendon, MD, Chethan K. Rao, DO, MS, Cintia Carla Da Hora, MD, Florinda Islamovic, MD, Fernando N. Galan, MD DOI: 10.1542/peds.2020-049678 Journal: Pediatrics Article Type: Case Report Citation: McLendon LA, Rao CK, Da Hora CC, Islamovic F, Galan FN. Post-COVID-19 acute disseminated encephalomyelitis in a 17-month-old. Pediatrics. 2021; doi: 10.1542/peds.2020- 049678 This is a prepublication version of an article that has undergone peer review and been accepted for publication but is not the final version of record. This paper may be cited using the DOI and date of access. This paper may contain information that has errors in facts, figures, and statements, and will be corrected in the final published version. The journal is providing an early version of this article to expedite access to this information. The American Academy of Pediatrics, the editors, and authors are not responsible for inaccurate information and data described in this version. Downloaded from©202 www.aappublications.org/news1 American Academy by of guest Pediatrics on September 27, 2021 Prepublication Release Post-COVID-19 Acute Disseminated Encephalomyelitis in a 17-month-old a,b Loren A. McLendon, MD, a,b Chethan K. Rao, DO, MS, c Cintia Carla Da Hora, MD, c Florinda Islamovic, MD, b Fernando N. Galan, MD Affiliations: a Mayo Clinic College of Medical Science Florida, Division of Child and Adolescent Neurology, Jacksonville, Florida b Nemours Children Specialty Clinic, Division of Neurology, Jacksonville, Florida c University of Florida College of Medicine Jacksonville, Division of Pediatrics, Jacksonville, Florida Corresponding Author: Fernando N.
    [Show full text]
  • Update on Viral Infections Involving the Central Nervous System in Pediatric Patients
    children Review Update on Viral Infections Involving the Central Nervous System in Pediatric Patients Giovanni Autore 1, Luca Bernardi 1, Serafina Perrone 2 and Susanna Esposito 1,* 1 Pediatric Clinic, Pietro Barilla Children’s Hospital, Department of Medicine and Surgery, University of Parma, Via Gramsci 14, 43126 Parma, Italy; [email protected] (G.A.); [email protected] (L.B.) 2 Neonatology Unit, Pietro Barilla Children’s Hospital, Department of Medicine and Surgery, University of Parma, Via Gramsci 14, 43126 Parma, Italy; serafi[email protected] * Correspondence: [email protected]; Tel.: +39-0521-704790 Abstract: Infections of the central nervous system (CNS) are mainly caused by viruses, and these infections can be life-threatening in pediatric patients. Although the prognosis of CNS infections is often favorable, mortality and long-term sequelae can occur. The aims of this narrative review were to describe the specific microbiological and clinical features of the most frequent pathogens and to provide an update on the diagnostic approaches and treatment strategies for viral CNS infections in children. A literature analysis showed that the most common pathogens worldwide are enteroviruses, arboviruses, parechoviruses, and herpesviruses, with variable prevalence rates in different countries. Lumbar puncture (LP) should be performed as soon as possible when CNS infection is suspected, and cerebrospinal fluid (CSF) samples should always be sent for polymerase chain reaction (PCR) analysis. Due to the lack of specific therapies, the management of viral CNS infections is mainly based on supportive care, and empiric treatment against herpes simplex virus (HSV) infection should be started as soon as possible.
    [Show full text]
  • Acute Encephalopathy with Bilateral Thalamotegmental Involvement in Infants and Children: Imaging and Pathology Findings
    Acute Encephalopathy with Bilateral Thalamotegmental Involvement in Infants and Children: Imaging and Pathology Findings Akira Yagishita, Imaharu Nakano, Takakazu Ushioda, Noriyuki Otsuki, and Akio Hasegawa PURPOSE: To investigate the imaging and pathologic characteristics of acute encephalopathy with bilateral thalamotegmental involvement in infants and children. METHODS: Five Japanese children ranging in age from 11 to 29 months were studied. We performed CT imaging in all patients, 10 MR examinations in four patients, and an autopsy in one patient. RESULTS: The encephalopathy affected the thalami, brain stem tegmenta, and cerebral and cerebellar white matter. The brain of the autopsied case showed fresh necrosis and brain edema without inflam- matory cell infiltration. Petechiae and congestion were demonstrated mainly in the thalamus. CT and MR images showed symmetric focal lesions in the same areas in the early phase. These lesions became more demarcated and smaller in the intermediate phase. The ventricles and cortical sulci enlarged. MR images demonstrated T1 shortening in the thalami. The prognosis was generally poor; one patient died, three patients were left with severe sequelae, and only one patient im- proved. CONCLUSIONS: The encephalopathy might be a postviral or postinfectious brain disor- der. T1 shortening in the thalami indicated the presence of petechiae. Index terms: Brain, diseases; Brain, magnetic resonance; Infants, diseases AJNR Am J Neuroradiol 16:439–447, March 1995 A strange type of acute encephalopathy has and cerebellar white matter. All patients in been reported in Japan (1–12). The acute en- whom this condition has been diagnosed thus cephalopathy occurs in infants and young chil- far have been Japanese infants and children, as dren without sexual predilection and is pre- far as we know.
    [Show full text]
  • Acute Leucoencephalopathy with Restriction of Diffusion-A Case Report
    Eastern Journal of Medicine 17 (2012) 149-152 V. Singh et al / Dwi in acute leucoencephalopathy Case Report Acute leucoencephalopathy with restriction of diffusion-a case report Vivek Singh, Vaishali Tomar, Alok Kumar, Rajendra V. Phadke* Department of Radiodiagnosis, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India Abstract. Acute white matter insult in a non traumatic, non ischemic background has been recently identified in certain clinical situations. Named toxic leukoencephalopathy, it may be caused by exposure to a wide variety of agents, including cranial irradiation, therapeutic agents, drugs of abuse, and environmental toxins. Extensive central white matter restriction of diffusion in the absence of significant T2 or Fluid-attenuated inversion recovery (FLAIR) abnormality is the imaging hallmark on Magnetic Resonance Imaging (MRI). Reversibility of the symptoms after the withdrawal of toxin has been shown in a few reports. We present one such case with reversal of changes on MRI and clinical improvement. Key words: ADEM, intramyelinic edema, PRES, toxic leukoencephalopathy 1. Introduction 2. Case report Leukoencephalopathy is a structural alteration A 7 year-old male child presented elsewhere of cerebral white matter in which myelin suffers with a short history of mild fever and had two the most damage (1). Toxic leukoencephalopathy episodes of vomiting. He received multiple is a recently identified entity seen in patients medications including antibiotics empirically. He exposed to a wide variety of agents like cranial went into altered sensorium on the 3rd day. There irradiation, therapeutic agents, drugs of abuse and was no history of seizures or past history of any environmental toxins (1).
    [Show full text]
  • Overview of the Neurotoxic Effects in Solvent-Exposed Workers
    Arch Public Health 2002, 60, 217-232 Overview of the neurotoxic effects in solvent-exposed workers by Viaene M.K. 1 Abstract Workers exposed to solvents are at risk to develop a chronic toxic encephalopathy, although effects (promoting or etiological) on other cen- tral and peripheral nervous system diseases may be possible. Careful monitoring of exposure (environmental monitoring and bio-monitoring) but also bio-effect monitoring is strictly needed. A review of the literature is given. This text is the summary of the report made for the Fund for Occupational Diseases (Fonds voor de Beroepsziekten), Belgium, 1998. Keywords Neurotoxicity syndromes, organic solvents, chronic toxic encephalopathy. Correspondence: M.K. Viaene, MD, PhD, Neuropsycho-Toxicological Expertise Centre, Governmental Psychiatric Hospital (OPZ), Pas 200, 2440 Geel, Tel: 014/ 57 91 11, E-mail: [email protected] 1 Department of Occupational Medicine, Catholic University of Leuven, UZ. St. Rafaël, Kapucijnenvoer 35, 3000 Leuven, Tel: 016/ 33 70 80. 218 Viaene MK. Introduction Organic solvents represent a group of aliphatic and aromatic organic compounds which are usually lipophilic and more or less volatile. Historically, some solvents (e.g. trichloroethylene and chloroform) were used in medi- cine as anesthetics because of their narcotic properties. Propofol (2,6-di- isopropylphenol) is still widely used in this respect. However, in industry numerous chemical or technical processes rely on specific properties of organic solvents which may cause substantial exposure to these substances in the work force. Although the acute neurotoxic potentials of most solvents were known for a long time, it was not earlier than the second half of the 20th century that chronic or delayed neurotoxicity due to occupational sol- vent exposure became a scientific issue.
    [Show full text]
  • Balo`'S Concentric Sclerosis: Still to Be Considered As a Variant of Multiple Sclerosis?
    Neurol Sci (2015) 36:2277–2280 DOI 10.1007/s10072-015-2297-8 BRIEF COMMUNICATION Balo`’s concentric sclerosis: still to be considered as a variant of multiple sclerosis? Anna M. Pietroboni1 • Andrea Arighi1 • Milena A. De Riz1 • Laura Ghezzi1 • Alberto Calvi1 • Sabrina Avignone2 • Elisa Scola2 • Daniela Galimberti1 • Fabio Triulzi2 • Elio Scarpini1 Received: 26 March 2015 / Accepted: 16 June 2015 / Published online: 25 June 2015 Ó Springer-Verlag Italia 2015 Abstract Balo`’s concentric sclerosis (BCS) is considered a presentation of BCS. Moreover, these case reports suggest that rare demyelinating disease and regarded as an aggressive BCS may neither be rapidly progressive nor fatal and may be variant of multiple sclerosis (MS). We describe three cases considered part of the MS spectrum. In line with this hypoth- (one male and two females) with neuroimaging features sug- esis, current treatments for MS were effective in our patients. gestive of BCS and heterogeneous symptoms, with benign long-term clinical course upon treatment with natalizumab and Keywords Balo`’s concentric sclerosis (BCS) Á Multiple fingolimod. Neurological examination, blood and cere- sclerosis (MS) Á Clinically isolated syndrome (CIS) brospinal fluid analyses, brain and spinal cord magnetic reso- nance imaging (MRI) and brain proton magnetic resonance spectroscopy were performed. At onset, patient #1 showed Introduction predominant cognitive impairment with consciousness distur- bances; patient #2 presented with left hemiparesis; patient #3 Balo`’s concentric sclerosis (BCS) is a rare demyelinating demonstrated hesitance in speech and in written word pro- disease and it is regarded as one of the atypical forms of duction, along with right central facial palsy.
    [Show full text]
  • ADEM) After Autologous Peripheral Blood Stem Cell Transplant for Non-Hodgkin’S Lymphoma
    Bone Marrow Transplantation, (1999) 24, 1351–1354 1999 Stockton Press All rights reserved 0268–3369/99 $15.00 http://www.stockton-press.co.uk/bmt Case report Acute disseminated encephalomyelitis (ADEM) after autologous peripheral blood stem cell transplant for non-Hodgkin’s lymphoma A Re and R Giachetti Department of Hematology, University of Parma, Italy Summary: High-dose chemotherapy followed by autologous periph- eral blood stem cell transplantation (PBSCT) is a thera- Acute disseminated encephalomyelitis (ADEM) is a peutic intervention performed with increasing frequency for demyelinating disorder of the central nervous system hematologic and solid malignancies.4 The widespread use with an acute clinical onset and a wide variability in of this procedure depends on its safety and easy feasibility. severity and outcome. It usually follows a viral infection Mild organ toxicities and low incidence of life-threatening or an immunization and is thought to be immuno- complications are usually reported. Neurologic events are mediated. We report a case of ADEM with a dramatic frequently mild and reversible and usually secondary to clinical onset in an autologous peripheral blood stem injury to other organ systems.5 cell transplant (PBSCT) recipient for non-Hodgkin’s We report a case of ADEM in an autologous PBSCT lymphoma who developed the neurologic syndrome 12 recipient with non-Hodgkin’s lymphoma (NHL) who days after PBSC reinfusion. This is the first report of developed the syndrome on day ϩ12 after PBSC ADEM in the setting of autologous PBSCT, a thera- reinfusion, without any recognizable etiology. To our peutic procedure performed with increasing frequency knowledge, this is the first report of ADEM developing in a wide variety of hematologic and solid malignancies.
    [Show full text]
  • Electroencephalography and Brain Imaging Patterns in Children With
    logy & N ro eu u r e o N p h f y Arhan et al., J Neurol Neurophysiol 2016, 7:3 o s l i a o l n o r g u DOI: 10.4172/2155-9562.1000378 y o J Journal of Neurology & Neurophysiology ISSN: 2155-9562 Research Article Article OpenOpen Access Access Electroencephalography and Brain Imaging Patterns in Children with Acute Encephalopathy Ebru Arhan, Yılmaz Akbas*, Kürşad Aydın, Tuğba Hırfanoglu, Ayse Serdaroglu and Zeynep Ozturk Gazi University, Faculty of Medicine, Pediatric Neurology Department, Turkey Abstract Background: The electroencephalographic patterns can be associated with brain imaging and outcome in children with acute encephalopathy. A rigorous association of electroencephalography and imaging findings and outcome of pediatric encephalopathy is lacking. Materıal and method: We performed a retrospective review of clinical, electroencephalography and imaging data of forty-nine children with electroencephalography pattern of diffuse encephalopathy. Results: The most prevalent etiological group was the metabolic group. The most common electroencephalography finding was isolated continuous slowing of background activity. Theta pattern was significantly associated with brain atrophy and with toxic encephalopathy, theta/delta pattern with white matter abnormalities and viral encephalitis. Patients with a theta/delta pattern was more likely require intensive care others, whereas those with FIRDA didn't need intensive care. Conclusıon: This study highlights that electroencephalography-imaging when added to the clinical data may help clinicians in the diagnosis, and hence appropiate treatment of pediatric patients with encephalopathy. Keywords: Encephalopathy; Pediatric; EEG patterns; Brain imaging University Epilepsy Center. After the identification of EEG’s, the clinical data of the patients were retrospectively collected and analyzed from Introductıon hospital medical records.
    [Show full text]