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Toxocariasis of the CNS simulating acute disseminated encephalomyelitis.
Toxocariasis of the CNS simulating acute disseminated encephalomyelitis. Neurology. 2007 Aug 21;69(8):806-7 Authors: Marx C, Lin J, Masruha MR, Rodrigues MG, da Rocha AJ, Vilanova LC, Gabbai AA PMID:- ADEM Research 17709716 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis after mastoid surgery in a child. J Laryngol Otol. 2007 Oct;121(10):1003-5 Authors: Khan NA, Bhalla RK, de Goede CG, Rothera MP, Ismayl O We present a case of acute disseminated encephalomyelitis after mastoid surgery in an 11-year-old child. The aim of this paper is to increase awareness about a previously unreported and an unusual neurological complication of cholesteatoma and mastoid surgery. PMID:- ADEM Research 17517165 [PubMed - indexed for MEDLINE:- ADEM Research]
Multiple myeloma presenting with acute disseminated encephalomyelitis: causal or chance link? Neurology. 2007 May 22;68(21):1873-4; author reply 1874 Authors: Vokaer M, Zegers de Beyl D, Bier JC PMID:- ADEM Research 17515558 [PubMed - indexed for MEDLINE:- ADEM Research]
Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology. 2007 Apr 17;68(16 Suppl 2):S7-12 Authors: Krupp LB, Banwell B, Tenembaum S, BACKGROUND: The CNS inflammatory demyelinating disorders of childhood include both self-limited and lifelong conditions, which can be indistinguishable at the time of initial presentation. Clinical, biologic, and radiographic delineation of the various monophasic and chronic childhood demyelinating disorders requires an operational classification system to facilitate prospective research studies. METHODS: The National Multiple Sclerosis Society (NMSS) organized an International Pediatric MS Study Group (Study Group) composed of adult and pediatric neurologists and experts in genetics, epidemiology, neuropsychology, nursing, and immunology. The group met several times to develop consensus definitions regarding the major CNS inflammatory demyelinating disorders of children and adolescents. RESULTS: Clinical definitions are proposed for pediatric multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), recurrent ADEM, multiphasic ADEM, neuromyelitis optica, and clinically isolated syndrome. These definitions are considered operational and need to be tested in future research and modified accordingly. CONCLUSION: CNS inflammatory demyelinating disorders presenting in children and adolescents can be defined and distinguished. However, prospective research is necessary to determine the validity and utility of the proposed diagnostic categories. PMID:- ADEM Research 17438241 [PubMed - indexed for MEDLINE:- ADEM Research]
MRI features of pediatric multiple sclerosis. Neurology. 2007 Apr 17;68(16 Suppl 2):S46-53 Authors: Banwell B, Shroff M, Ness JM, Jeffery D, Schwid S, Weinstock-Guttman B, BACKGROUND: MRI has revolutionized the diagnostic accuracy of multiple sclerosis (MS) in adults, and is now used extensively to evaluate efficacy of immunomodulatory therapies. Although MRI has also been used to aid in the diagnosis and care of children with MS, the MRI features of MS in children are less well understood. METHODS: The present review summarizes the available literature on MRI in pediatric MS, outlines the specific features of other disorders affecting the CNS white matter in children, compares the MRI appearance of MS in children to seminal neuroimaging studies in adult-onset MS, and discusses the potential role of advanced MRI technologies in delineating the underlying pathobiology of acquired demyelinating disease in children. RESULTS: Although the MRI features of MS in children have similarity to adult-onset MS, children tend to have fewer lesions and a lower propensity for lesions to enhance with gadolinium. The MRI findings in children presenting with a clinical phenotype of acute disseminated encephalomyelitis may be indistinguishable from the first attack of MS. CONCLUSIONS: MRI criteria specific for pediatric-onset multiple sclerosis (MS) and criteria predictive of MS outcome in children experiencing a first demyelinating event will be challenged by the overlap in MRI features between acute monophasic demyelinating syndromes and MS, particularly in younger children. Emergence of new clinically silent lesions on MRI scans separated by at least 3 months is characteristic of MS. Newer MRI techniques evaluating white matter biochemistry and integrity in the youngest MS patients may provide new insights into the relative contributions of inflammation and neurodegeneration in MS. PMID:- ADEM Research 17438238 [PubMed - indexed for MEDLINE:- ADEM Research]
Clinical features of children and adolescents with multiple sclerosis. Neurology. 2007 Apr 17;68(16 Suppl 2):S37-45 Authors: Ness JM, Chabas D, Sadovnick AD, Pohl D, Banwell B, Weinstock-Guttman B, There is increasing appreciation that multiple sclerosis (MS) can begin in childhood or adolescence, but pediatric MS continues to be a rare entity, with an estimated 2 to 5% of patients with MS experiencing their first clinical symptoms before age 16. A prompt diagnosis of pediatric MS is important to optimize overall management of both the physical and social impact of the disease. The widespread use of disease-modifying therapies (DMT) for MS in adults, as early as following an initial isolated episode, has led to the use of DMT in children and adolescents with MS. However, it is imperative to distinguish pediatric MS from other childhood CNS inflammatory demyelinating disorders such as acute disseminated encephalomyelitis. Although increasing evidence suggests a slower disease course in children with MS compared to adults, significant disability can still accumulate by early adulthood. Furthermore, associated neurocognitive deficits can impair both academic and psychosocial function at a critical juncture in a young person's life. This article reviews the clinical characteristics, neuroimaging, paraclinical findings, disease course, epidemiology, genetics, and pathophysiology of pediatric MS vis-à-vis adult MS. Further research of pediatric MS may advance our understanding of MS pathophysiology in general, as well as improve the long-term health care outcomes of children and adolescents diagnosed with MS. PMID:- ADEM Research 17438237 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis. Neurology. 2007 Apr 17;68(16 Suppl 2):S23-36 Authors: Tenembaum S, Chitnis T, Ness J, Hahn JS, Acute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory disorder of the CNS characterized by a widespread demyelination that predominantly involves the white matter of the brain and spinal cord. The condition is usually precipitated by a viral infection or vaccination. The presenting features include an acute encephalopathy with multifocal neurologic signs and deficits. Children are preferentially affected. In the absence of specific biologic markers, the diagnosis of ADEM is still based on the clinical and radiologic features. Although ADEM usually has a monophasic course, recurrent or multiphasic forms have been reported, raising diagnostic difficulties in distinguishing these cases from multiple sclerosis (MS). The International Pediatric MS Study Group proposes uniform definitions for ADEM and its variants. We discuss some of the difficulties in the interpretation of available literature due to the different terms and definitions used. In addition, this review summarizes current knowledge of the main aspects of ADEM, including its clinical and radiologic diagnostic features, epidemiology, pathogenesis, and outcome. An overview of ADEM treatment in children is provided. Finally, the controversies surrounding pediatric MS and ADEM are addressed. PMID:- ADEM Research 17438235 [PubMed - indexed for MEDLINE:- ADEM Research]
Alexia without agraphia in a child with acute disseminated encephalomyelitis. Neurology. 2007 Mar 20;68(12):965-6; author reply 966 Authors: Frank Y PMID:- ADEM Research 17372144 [PubMed - indexed for MEDLINE:- ADEM Research]
Anti-viral T-cell immunity+anti-CNS autoantibody=a model for human acute disseminated encephalomyelitis or multiple sclerosis relapse? Am J Pathol. 2007 Feb;170(2):436-8 Authors: Sobel RA PMID:- ADEM Research 17255311 [PubMed - indexed for MEDLINE:- ADEM Research]
Multiple myeloma presenting with acute disseminated encephalomyelitis: causal or chance link?
Neurology. 2006 Nov 28;67(10):1893-4 Authors: Sheng B, Mak VW, Lee HK, Li HL, Lee IP, Wong S PMID:- ADEM Research 17130436 [PubMed - indexed for MEDLINE:- ADEM Research]
Demyelinating diseases.
J Clin Pathol. 2006 Nov;59(11):1151-9 Authors: Love S A diagnosis of demyelination carries important therapeutic and prognostic implications. In most cases the diagnosis is made clinically, and involvement of the histopathologist is largely confined to postmortem confirmation and clinicopathological correlation. However, every now and then, accurate diagnosis of the presence or cause of demyelination before death hinges on the histopathological assessment. Recognition of demyelination depends on an awareness of this as a diagnostic possibility, and on the use of appropriate tinctorial and immunohistochemical stains to identify myelin, axons and inflammatory cells. In biopsy specimens, the critical distinction is usually from ischaemic or neoplastic disease, and the types of demyelinating disease most likely to be encountered are multiple sclerosis, acute-disseminated encephalomyelitis, progressive multifocal leucoencephalopathy and extrapontine myelinolysis. Interpretation of the pathology has to be made in the context of the clinical, radiological and biochemical findings. Freezing of a small amount of fresh tissue allows for later virological studies, and electron microscopy is occasionally helpful for demonstration of viral particles. PMID:- ADEM Research 17071802 [PubMed - indexed for MEDLINE:- ADEM Research]
Leukoencephalopathy induced by levamisole alone for the treatment of recurrent aphthous ulcers.
Neurology. 2006 Sep 26;67(6):1065-7 Authors: Liu HM, Hsieh WJ, Yang CC, Wu VC, Wu KD We report imaging and clinical findings of leukoencephalopathy occurring after levamisole. The lesions were hypoattenuating on CT and appeared as multifocal oval or elliptical foci in the white matter or along the subependymal veins on MRI. Most lesions resolve after prompt withdrawal of levamisole. Detailed history taking is important for diagnosing levamisole-induced leukoencephalopathy because multiple sclerosis and acute disseminated encephalomyelitis cannot be differentiated by neuroimaging findings alone. PMID:- ADEM Research 17000980 [PubMed - indexed for MEDLINE:- ADEM Research]
Central brain herniation secondary to fulminant acute disseminated encephalomyelitis: implications for neurosurgical management. Case report.
J Neurosurg. 2006 Sep;105(3):472-4 Authors: Sekula RF, Marchan EM, Baghai P, Jannetta PJ, Quigley MR Acute disseminated encephalomyelitis (ADEM), also known as postinfectious encephalomyelitis, is an immunologically mediated demyelinating disorder affecting the central nervous system that typically occurs after infection or vaccination. The prognosis of ADEM is generally favorable. In a small subset of patients with ADEM, however, fulminant cerebral edema requiring neurosurgical intervention will develop. Few recommendations are available to help the neurosurgeon in dealing with such cases. In this report, the authors present the case of a patient with ADEM in whom central brain herniation developed secondary to medically intractable cerebral edema. The authors review the salient features of the disease and suggest a role for neurosurgeons in cases of fulminant ADEM. PMID:- ADEM Research 16961146 [PubMed - indexed for MEDLINE:- ADEM Research]
Alexia without agraphia in a child with acute disseminated encephalomyelitis.
Neurology. 2006 Aug 22;67(4):725 Authors: Little RD, Goldstein JL PMID:- ADEM Research 16924040 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis: an update.
Arch Neurol. 2005 Nov;62(11):1673-80 Authors: Menge T, Hemmer B, Nessler S, Wiendl H, Neuhaus O, Hartung HP, Kieseier BC, Stüve O Acute disseminated encephalomyelitis (ADEM) is a monophasic autoimmune demyelinating disease of the central nervous system that typically follows a febrile infection or a vaccination. Children are predominantly affected. A plethora of viral and bacterial pathogens and a number of vaccinations have been associated with ADEM. Experimental animal studies indicate that both primary and secondary autoimmune responses contribute to central nervous system inflammation and subsequent demyelination. The clinical diagnosis of ADEM is strongly suggested by a close temporal relationship between an infectious incident or an immunization and the onset of leukoencephalopathic neurological symptoms. Paraclinical tests may support the diagnosis. Particularly helpful are acute signs of newly developed extensive, multifocal, subcortical white matter abnormalities on magnetic resonance images of the brain. The cerebrospinal fluid may disclose a mild lymphocytic pleocytosis and elevated albumin levels. Oligoclonal bands are not always present in ADEM and, if so, may be transient. The major differential diagnosis of ADEM is multiple sclerosis. Treatment options for ADEM consist of anti-inflammatory and immunosuppressive agents. In general, the disease is self-limiting and the prognostic outcome favorable. In the absence of widely accepted clinical or paraclinical diagnostic guidelines, a number of recently conducted observational case series have substantially broadened our understanding about the clinical phenotype, diagnosis, and prognosis of ADEM. PMID:- ADEM Research 16286539 [PubMed - indexed for MEDLINE:- ADEM Research]
Postinfectious inflammatory disorders: subgroups based on prospective follow-up.
Neurology. 2005 Oct 11;65(7):1057-65 Authors: Marchioni E, Ravaglia S, Piccolo G, Furione M, Zardini E, Franciotta D, Alfonsi E, Minoli L, Romani A, Todeschini A, Uggetti C, Tavazzi E, Ceroni M BACKGROUND: Acute disseminated encephalomyelitis (ADEM) refers to a monophasic acute multifocal inflammatory CNS disease. However, both relapsing and site-restricted variants, possibly associated with peripheral nervous system (PNS) involvement, are also observed, and a systematic classification is lacking. OBJECTIVE: To describe a cohort of postinfectious ADEM patients, to propose a classification based on clinical and instrumental features, and to identify subgroups of patients with different prognostic factors. METHODS: Inpatients of a Neurologic and Infectious Disease Clinic affected by postinfectious CNS syndrome consecutively admitted over 5 years were studied. RESULTS: Of 75 patients enrolled, 60 fulfilled criteria for ADEM after follow-up lasting from 24 months to 7 years. Based on lesion distribution, patients were classified as encephalitis (20%), myelitis (23.3%), encephalomyelitis (13.3%), encephalomyeloradiculoneuritis (26.7%), and myeloradiculoneuritis (16.7%). Thirty patients (50%) had a favorable outcome. Fifteen patients (25%) showed a relapsing course. Poor outcome was related with older age at onset, female gender, elevated CSF proteins, and spinal cord and PNS involvement. All but two patients received high-dose steroids as first-line treatment, with a positive response in 39 (67%). Ten of 19 nonresponders (53%) benefited from high-dose IV immunoglobulin; 9 of 10 had PNS involvement. The data were not controlled. CONCLUSIONS: A high prevalence of "atypical variants" was found in this series, with site-restricted damage or additional peripheral nervous system (PNS) involvement. Prognosis and response to steroids were generally good, except for some patient subgroups. In patients with PNS involvement and steroid failure, a favorable effect of IV immunoglobulin was observed. PMID:- ADEM Research 16217059 [PubMed - indexed for MEDLINE:- ADEM Research]
The transformation of pediatric gliomatosis cerebri to cerebellar glioblastoma multiforme presenting as supra- and infratentorial acute disseminated encephalomyelitis. Case report.
J Neurosurg. 2005 Jan;102(1 Suppl):72-7 Authors: Senatus PB, McClelland S, Tanji K, Khandji A, Huang J, Feldstein N Cerebellar glioblastoma multiforme (GBM) is a rare entity in adults and an extremely rare entity in children. Approximately 30 cases have been reported in the literature. The authors report the case of a histologically confirmed cerebellar GBM presenting initially as supra- and infratentorial gliomatosis cerebri. Acute disseminated encephalomyelitis had been diagnosed in the patient and that diagnosis remained until near the end of his treatment. This case underscores the need for recognizing the clinical presentation of gliomatosis cerebri and multifocal GBM in the pediatric subpopulation thought to harbor demyelinating disease. PMID:- ADEM Research 16206737 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis in children: discordant neurologic and neuroimaging abnormalities and response to plasmapheresis.
Pediatrics. 2005 Aug;116(2):431-6 Authors: Khurana DS, Melvin JJ, Kothare SV, Valencia I, Hardison HH, Yum S, Faerber EN, Legido A OBJECTIVES: To describe our experience with acute disseminated encephalomyelitis (ADEM), focusing on (1) the relationship between clinical course and MRI findings and (2) the response to plasmapheresis in a subgroup of patients. METHODS: A retrospective record review was conducted of 13 children who were admitted as inpatients with the diagnosis of ADEM during the period 1998-2003. RESULTS: Diagnosis was established by clinical signs and symptoms, cerebrospinal fluid changes and multifocal involvement of deep gray and white matter based on MRI. Initial therapy was high-dose methylprednisolone and intravenous immunoglobulin in 12 patients. One child improved spontaneously. Six of 12 children did not improve with corticosteroid treatment. All 6 had an acute progressive course neurologically, and 5 of them also showed a delay in the onset of neuroimaging changes, eventually developing lesions in the deep gray matter and brainstem. This latter group received 5 sessions of plasmapheresis and recovered over the course of several months with varying degrees of residual neurologic deficits. CONCLUSIONS: Presentation of ADEM with delayed development of MRI lesions in deep gray matter and brainstem may herald a prolonged clinical course and lack of response to glucocorticoid therapy. Plasmapheresis might be an effective therapeutic intervention in these patients. The role of plasmapheresis versus corticosteroids and intravenous immunoglobulin as a primary treatment of ADEM needs to be investigated further. PMID:- ADEM Research 16061599 [PubMed - indexed for MEDLINE:- ADEM Research]
Pediatric central nervous system infections and inflammatory white matter disease.
Pediatr Clin North Am. 2005 Aug;52(4):1107-26, ix Authors: Silvia MT, Licht DJ This article reviews the immunology of the central nervous system and the clinical presentation, diagnosis, and treatment of children with viral or parainfectious encephalitis. The emphasis is on the early recognition of treatable causes of viral encephalitis (herpes simplex virus), and the diagnosis and treatment of acute disseminated encephalomyelitis are described in detail. Laboratory and imaging findings in the two conditions also are described. PMID:- ADEM Research 16009259 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis or multiple sclerosis: can the initial presentation help in establishing a correct diagnosis?
Arch Dis Child. 2005 Jun;90(6):636-9 Authors: Dale RC, Branson JA The differential diagnosis of CNS white matter disease is broad, and can be divided into vascular, metabolic, infective, or inflammatory aetiologies. Isolated inflammatory disorders of the CNS are often associated with demyelination, and the two terms (inflammatory and demyelinating) are often used in conjunction. When the disease is monophasic, the term acute disseminated encephalomyelitis (ADEM) is used. ADEM typically occurs as a post-infectious phenomenon, and by definition, must be an isolated (monophasic) episode. If a relapse occurs shortly after the ADEM presentation in association with a further infection or steroid withdrawal, the term MDEM (multiphasic disseminated encephalomyelitis) is used. When there are relapses or progressive disease, the term multiple sclerosis (MS) is used (for full recommended diagnostic criteria for multiple sclerosis refer to McDonald and colleagues). PMID:- ADEM Research 15908633 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis following Plasmodium falciparum malaria caused by varicella zoster virus reactivation.
Am J Trop Med Hyg. 2005 Apr;72(4):478-80 Authors: Lademann M, Gabelin P, Lafrenz M, Wernitz C, Ehmke H, Schmitz H, Reisinger EC Neurologic complications in the course of Plasmodium falciparum infections are commonly diagnosed as cerebral malaria, but bacterial or viral meningitis may exhibit similar symptoms. One to three weeks after P. falciparum malaria, acute disseminated encephalomyelitis (ADEM) can also mimick the symptoms of cerebral malaria. We describe a 31-year-old woman with life-threatening ADEM five days after successful treatment of P. falciparum malaria. The detection of IgG and IgM antibodies in serum and cerebrospinal fluid (CSF) against multiple viruses and bacteria reflected a non-specific polyclonal B cell activation and was more confusing than helpful for diagnostic decisions. Varicella zoster virus was identified with a reverse transcriptase multiplex polymerase chain reaction in the initially obtained and frozen CSF. This case and findings from the literature indicate that P. falciparum-associated ADEM might not be immune mediated, but of infectious origin. With unclear cerebral complications during or after P. falciparum malaria, prompt initiation of empirical antiviral and antibacterial treatment in addition to antimalarials may reduce mortality. PMID:- ADEM Research 15827291 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis after liver transplantation.
Arch Neurol. 2005 Apr;62(4):650-2 Authors: Lindzen E, Gilani A, Markovic-Plese S, Mann D BACKGROUND: During the past 10 years, acute disseminated encephalomyelitis has been reported a few times after organ transplantation. OBJECTIVE: To report a case of acute disseminated encephalomyelitis as a complication of liver transplantation. DESIGN: Case report. SETTING: The University of North Carolina Hospital and Medical Center, Chapel Hill.Patient A 49-year-old woman admitted because of acute onset of paresthesias, sensory loss, and weakness after liver transplantation. Acute clinical presentation, results of imaging studies, and comprehensive laboratory evaluation were consistent with acute disseminated encephalomyelitis. INTERVENTIONS: High-dose intravenous corticosteroid therapy followed by maintenance oral dosing. MAIN OUTCOME MEASURES: Clinical and magnetic resonance imaging improvement. RESULTS: Corticosteroid therapy halted clinical progression, with partial resolution of lesions on magnetic resonance images of the brain and spinal cord. CONCLUSIONS: This is, to our knowledge, the first report of acute disseminated encephalomyelitis after liver transplantation. Possible pathogenic mechanisms include a cross-reactive immune response to foreign antigens present within the transplanted organ, or an inflammatory response triggered by viral infection in an immunocompromised host. PMID:- ADEM Research 15824267 [PubMed - indexed for MEDLINE:- ADEM Research]
Microangiopathy of brain, retina, and inner ear (Susac's syndrome) in an adolescent female presenting as acute disseminated encephalomyelitis.
Pediatrics. 2004 Jul;114(1):276-81 Authors: Hahn JS, Lannin WC, Sarwal MM Susac's syndrome is a rare disorder that consists of microangiopathy of the brain, retina, and inner ear and usually affects young women in young adulthood. The triad of clinical manifestations consists of acute encephalopathy with neurologic signs, branch retinal artery occlusion (BRAO), and sensorineural hearing loss. We present a case of an adolescent female who presented at age 16 years with clinical and neuroimaging features of acute disseminated encephalomyelitis (ADEM). The full triad did not develop until 2.5 years after the initial neurologic presentation. PMID:- ADEM Research 15231946 [PubMed - indexed for MEDLINE:- ADEM Research]
Narcolepsy caused by acute disseminated encephalomyelitis.
Arch Neurol. 2004 May;61(5):758-60 Authors: Gledhill RF, Bartel PR, Yoshida Y, Nishino S, Scammell TE BACKGROUND: Narcolepsy with cataplexy is caused by a selective loss of hypocretin-producing neurons, but narcolepsy can also result from hypothalamic and rostral brainstem lesions. PATIENT: We describe a 38-year-old woman with severe daytime sleepiness, internuclear ophthalmoplegia, and bilateral delayed visual evoked potentials. Her multiple sleep latency test results demonstrated short sleep latencies and 4 sleep-onset rapid eye movement sleep periods, and her cerebrospinal fluid contained a low concentration of hypocretin. Magnetic resonance imaging showed T2 and fluid-attenuated inversion recovery hyperintensity along the walls of the third ventricle and aqueduct, which are consistent with acute disseminated encephalomyelitis. RESULTS: After treatment with steroids, this patient's subjective sleepiness, hypersomnia, and hypocretin deficiency partially improved. CONCLUSIONS: Autoimmune diseases such as acute disseminated encephalomyelitis can produce narcolepsy. Most likely, this narcolepsy is a consequence of demyelination and dysfunction of hypocretin pathways, but direct injury to the hypocretin neurons may also occur. PMID:- ADEM Research 15148155 [PubMed - indexed for MEDLINE:- ADEM Research]
Monophasic, solitary tumefactive demyelinating lesion: neuroimaging features and neuropathological diagnosis.
Br J Radiol. 2004 Feb;77(914):153-6 Authors: Tan HM, Chan LL, Chuah KL, Goh NS, Tang KK The characteristic clinicoradiological findings of multiple sclerosis and acute disseminated encephalomyelitis (ADEM), demonstrating a recurrent progressive course in the former and monophasicity in the latter associated with multiple discrete white matter lesions with variable enhancement on MRI, are not a diagnostic challenge. On the other hand, the less typical radiological presentation of a solitary tumefactive demyelinating lesion mimics a neoplasm, and often necessitates a biopsy. Nonetheless, histopathological examination is an imperfect gold standard and the recognition of certain imaging features may facilitate the correct diagnosis. PMID:- ADEM Research 15010391 [PubMed - indexed for MEDLINE:- ADEM Research]
First episode of acute CNS inflammatory demyelination in childhood: prognostic factors for multiple sclerosis and disability.
J Pediatr. 2004 Feb;144(2):246-52 Authors: Mikaeloff Y, Suissa S, Vallée L, Lubetzki C, Ponsot G, Confavreux C, Tardieu M, OBJECTIVES: To evaluate prognostic factors for second attack and for disability in children presenting with an initial episode of central nervous system (CNS) demyelination. STUDY DESIGN: A cohort of 296 children having a first episode of acute CNS inflammatory demyelination was studied by survival analysis. RESULTS: The average follow-up was 2.9+/-3 years. At the end of the follow-up, 57% of patients had a diagnosis of multiple sclerosis (MS), 29% had a monophasic acute disseminated encephalomyelitis, and 14% had a single focal episode. The rate of a second attack was (1). higher in patients with age at onset >or=10 years (hazard ratio, 1.67; 95% CI, 1.04-2.67), MS-suggestive initial MRI (1.54; 1.02-2.33), or optic nerve lesion (2.59; 1.27-5.29); and (2). lower in patients with myelitis (0.23; 0.10-0.56) or mental status change (0.59; 0.33-1.07). Of patients with a second attack, 29% had an initial diagnosis of acute disseminated encephalomyelitis. At the end of the follow-up period, 90% of patients had no or minor disability. Occurrence of severe disability was associated with a polysymptomatic onset (3.25; 1.16-11.01), sequelae after the first attack (26.65; 9.42-75.35), further relapses (1.49; 1.16-1.92), and progressive MS (3.57; 1.21-8.72). CONCLUSIONS: Risk of second attack of CNS demyelination is higher in older patients and lower in patients with mental status change. Risk of disability is higher in polysymptomatic and relapsing patients. PMID:- ADEM Research 14760270 [PubMed - indexed for MEDLINE:- ADEM Research]
Detection of coronavirus in the central nervous system of a child with acute disseminated encephalomyelitis.
Pediatrics. 2004 Jan;113(1 Pt 1):e73-6 Authors: Yeh EA, Collins A, Cohen ME, Duffner PK, Faden H We present a case in which human coronavirus was detected in the cerebrospinal fluid of a child presumed to have acute disseminated encephalomyelitis. In murine models, coronavirus has been found to cause a chronic demyelinating condition that resembles multiple sclerosis. Additionally, there is in vitro evidence of human coronavirus's ability to infect neural cells. This case report provides additional support for the hypothesis that coronavirus may be an important etiologic factor in the pathogenesis of demyelinating disease in humans. PMID:- ADEM Research 14702500 [PubMed - indexed for MEDLINE:- ADEM Research]
Hemicraniectomy as a successful treatment of mass effect in acute disseminated encephalomyelitis.
Neurology. 2003 Aug 12;61(3):420-1 Authors: von Stuckrad-Barre S, Klippel E, Foerch C, Lang JM, du Mesnil de Rochemont R, Sitzer M PMID:- ADEM Research 12913218 [PubMed - indexed for MEDLINE:- ADEM Research]
Encephalitis complicating smallpox vaccination.
Arch Neurol. 2003 Jul;60(7):925-8 Authors: Miravalle A, Roos KL A smallpox vaccination program has been initiated. The vaccine is a live virus that was used in the last century. Postvaccinal encephalitis is a complication of this vaccine. The clinical presentation, course, neuroimaging findings, and spinal fluid abnormalities are similar to a disorder that physicians are familiar with, acute disseminated encephalomyelitis. This complication can be prevented with the administration of antivaccinia gamma globulin at the time of vaccination. Antivaccinia gamma globulin is not efficacious once this complication occurs. Intravenous methylprednisolone is the recommended therapy, although intravenous immunoglobulin and plasmapheresis should be investigated in the treatment of postvaccinal encephalitis. PMID:- ADEM Research 12873847 [PubMed - indexed for MEDLINE:- ADEM Research]
20. Immunologic neuromuscular disorders.
J Allergy Clin Immunol. 2003 Feb;111(2 Suppl):S659-68 Authors: Chitnis T, Khoury SJ Immune-mediated disorders of each of the structural subdivisions of the nervous and neuromuscular system have been described. Despite the immune privilege of the central nervous system, and to a lesser extent the peripheral nervous system, immune dysregulation is not uncommon. Environmental, genetic, and immunologic factors have been postulated to be involved in the development of these disorders. Major immune-mediated neurologic diseases of the central nervous system include multiple sclerosis and acute disseminated encephalomyelitis. Immune-mediated diseases of the peripheral nervous system include myasthenia gravis, Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, idiopathic polymyositis, dermatomyositis, and inclusion body myositis. Some of these disorders, such as myasthenia gravis and certain forms of acute inflammatory demyelinating polyneuropathy, are clearly autoimmune in nature, whereas the immune system plays an important role in pathogenesis in others. Understanding the immune mechanisms of disease and uncovering potential therapeutic targets are essential for the design of new treatments. The epidemiology, pathogenesis, diagnostic criteria, and current therapeutic approaches to the major neuroimmunologic diseases are reviewed. PMID:- ADEM Research 12592311 [PubMed - indexed for MEDLINE:- ADEM Research]
Structural similarity between the bee venom peptides and the immunodominant human myelin basic proteins: role for pathogenesis of acute disseminated encephalomyelitis.
J Allergy Clin Immunol. 2003 Feb;111(2):426-8 Authors: Dharmasaroja P PMID:- ADEM Research 12589368 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians.
Arch Dis Child. 2003 Feb;88(2):122-4 Authors: Stonehouse M, Gupte G, Wassmer E, Whitehouse WP Acute disseminated encephalomyelitis will often present to the general paediatrician as an acute polysymptomatic encephalopathy, and initially the diagnosis may not be clear. A brain MRI scan is essential in establishing the diagnosis and so enabling appropriate advice and treatment to be given. Multicentre clinical audit of outcome and controlled therapeutic trials are needed to secure an evidence base for current practice. PMID:- ADEM Research 12538312 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients.
Neurology. 2002 Oct 22;59(8):1224-31 Authors: Tenembaum S, Chamoles N, Fejerman N BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the CNS. Few pediatric series have been published, with retrospective and short-term follow-up studies. OBJECTIVES: To describe a cohort of pediatric patients with ADEM and to determine whether clinical and neuroimaging findings predict outcome. METHODS: A prospective study was conducted between March 1988 and July 2000 on 84 consecutive children with ADEM at the National Pediatric Hospital "Dr. J. P. Garrahan." RESULTS: Mean age at onset was 5.3 +/- 3.9 years, with a significant male predominance. Sixty-two patients (74%) had a preceding viral illness or vaccination. Acute hemiparesis (76%), unilateral or bilateral long tract signs (85%), and changes in mental state (69%) were the most prominent presenting features. Four MRI groups were identified: ADEM with small lesions (62%), with large lesions (24%), with additional bithalamic involvement (12%), and acute hemorrhagic encephalomyelitis (2%). Of the 54 children whose CSF samples were analyzed, none showed intrathecal oligoclonal bands. The use of high-dose corticosteroid treatment, particularly IV methylprednisolone, was associated with good recovery and resolution of MRI lesions. After a mean follow-up of 6.6 +/- 3.8 years, 90% of children showed a monophasic course, and 10% a biphasic disease. Eighty-nine percent of patients show at present Expanded Disability Status Scale scores of 0 to 2.5. Eleven percent have disability scores of 3 to 6.5. CONCLUSIONS: Childhood acute disseminated encephalomyelitis is a benign condition, affecting boys more frequently. No association was found between MRI groups and disability. Disability was related to optic nerve involvement at presentation. Even in relapsing cases, the distinction between acute disseminated encephalomyelitis and MS was possible on the basis of long-term clinical and neuroimaging follow-up and the absence of oligoclonal bands in CSF. PMID:- ADEM Research 12391351 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis in children.
Pediatrics. 2002 Aug;110(2 Pt 1):e21 Authors: Murthy SN, Faden HS, Cohen ME, Bakshi R OBJECTIVE: To describe the epidemiologic, clinical, neuroimaging, and laboratory features; treatment; and outcome in a cohort of children with acute disseminated encephalomyelitis (ADEM). METHODS: A 6-year retrospective chart review of children with the diagnosis of ADEM was conducted. RESULTS: Eighteen cases were identified. Sixteen patients (88%) presented in either winter or spring. Thirteen children (72%) had a recent upper respiratory tract illness. Patients presented most often with motor deficits (77%) and secondly with altered consciousness (45%). Spinal fluid abnormalities occurred in 70%. Despite rigorous microbiologic testing, a definite microbiologic diagnosis was established only in 1 child with Epstein-Barr virus disease and probable or possible diagnoses in 3 children with Bartonella henselae, Mycoplasma pneumoniae, or rotavirus disease. Brain magnetic resonance imaging identified lesions in the cerebral cortex in 80%, in subcortical white matter in 93%, in periventricular white matter in 60%, in deep gray matter in 47%, and in brainstem in 47% of patients. Eleven patients (61%) were treated with corticosteroids, and 2 were treated with intravenous immunoglobulins. All patients survived. Three patients (17%) had long-term neurologic sequelae. CONCLUSIONS: Epidemiologic evidence from this study suggests an infectious cause for ADEM. The agent is most likely a difficult-to-diagnose winter/spring respiratory virus. Magnetic resonance imaging was the neuroimaging study of choice for establishing the diagnosis and for following the course of the disease. Prognosis for survival and outcome was excellent. Recurrent episodes of ADEM must be differentiated from multiple sclerosis. PMID:- ADEM Research 12165620 [PubMed - indexed for MEDLINE:- ADEM Research]
Plasma exchange for severe attacks of CNS demyelination: predictors of response.
Neurology. 2002 Jan 8;58(1):143-6 Authors: Keegan M, Pineda AA, McClelland RL, Darby CH, Rodriguez M, Weinshenker BG The authors reviewed 59 consecutive patients treated with plasma exchange (PE) for acute, severe attacks of CNS demyelination at Mayo Clinic from January 1984 through June 2000. Most patients had relapsing-remitting MS (n = 22, 37.3%), neuromyelitis optica (NMO) (n = 10, 16.9%), and acute disseminated encephalomyelitis (n = 10, 16.9%). PE was followed by moderate or marked functional improvement in 44.1% of treated patients. Male sex (p = 0.021), preserved reflexes (p = 0.019), and early initiation of treatment (p = 0.009) were associated with moderate or marked improvement. Successfully treated patients improved rapidly following PE, and improvement was sustained. PMID:- ADEM Research 11781423 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis associated with hepatitis C virus infection.
Arch Neurol. 2001 Oct;58(10):1679-81 Authors: Sacconi S, Salviati L, Merelli E BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is an autoimmune demyelinating disease of the central nervous system that is frequently preceded by an acute viral infection. This is the first reported case of ADEM associated with hepatitis C virus (HCV) infection. CASE DESCRIPTION: A 46-year-old woman underwent a surgical procedure and received multiple blood transfusions, at which time serologic testing for HCV was negative. Fifty days later, she suddenly developed seizures, alteration of consciousness, right hemiparesis, hemianopsia, and urinary retention. Magnetic resonance imaging revealed symmetric multifocal changes on T2-weighted images in the cerebral gray and white matter and in the cerebellar white matter with some lesion enhancement after gadolinium administration. Blood testing showed a recent HCV infection with high titer of IgM early antigens and a strongly positive reaction for HCV RNA. All other microbiological and virological test results were negative both in serum and in cerebrospinal fluid. Treatment with high-dose dexamethasone was followed by a dramatic improvement of the clinical and magnetic resonance picture. Within a few months the patient recovered completely and there were no relapses during 2 years of follow-up. CONCLUSIONS: Infection with HCV is associated with several autoimmune neurological manifestations. It is recommended the patients with ADEM be screened for HCV. PMID:- ADEM Research 11594929 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis as manifestation of primary HIV infection.
Neurology. 2001 Oct 23;57(8):1493-6 Authors: Narciso P, Galgani S, Del Grosso B, De Marco M, De Santis A, Balestra P, Ciapparoni V, Tozzi V The authors report a 27-year-old woman with clinical, MRI, virologic, and CSF findings consistent with acute disseminated encephalomyelitis as a manifestation of primary HIV infection. Improvements in the clinical and MRI findings and a reduction in HIV RNA levels, both in plasma and in the CSF, were observed during highly active antiretroviral therapy. PMID:- ADEM Research 11673598 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients.
Neurology. 2001 May 22;56(10):1313-8 Authors: Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B OBJECTIVES: To describe the clinical, CSF, and radiologic findings and long-term follow-up in a cohort of patients with acute disseminated encephalomyelitis (ADEM), and to determine possible prognostic factors for progression to MS. METHODS: Forty adults (28 women, mean age 33.5 years) diagnosed with ADEM were analyzed. Clinical symptoms, cranial MRI and CSF findings, and the response to a standardized treatment during the acute phase of the disease were analyzed by chart review. The final diagnosis of ADEM or clinically definite MS was established upon follow-up examination after 8 to 137 months. The patients with ADEM and MS were compared to detect differences between the two groups. RESULTS: Fifteen patients had a preceding infection (n = 14) or immunization (n = 1). The most frequent clinical signs were motor deficit (80%), followed by sensory deficits, brainstem signs, and ataxia. CSF findings were highly variable; normal results were present in 20% of patients. Oligoclonal bands were positive in 65% of patients. Ninety-five percent of all patients improved during the acute phase of the disease. Upon follow-up, 14 patients had developed clinically definite MS. Of the 26 patients with the final diagnosis of ADEM, two patients had died, nine had minor deficits, three had moderate deficits, and 12 patients had no remaining symptoms. Patients with the final diagnosis of ADEM were older, and more often had a preceding infection, clinical signs of brainstem involvement, a higher CSF albumin fraction, and infratentorial lesions. CONCLUSIONS: Many patients initially diagnosed with ADEM develop clinically definite MS upon long-term follow-up. The authors found no useful diagnostic criteria for the differentiation of a first episode of MS from monophasic ADEM. The term ADEM may still be employed as a description of a clinical syndrome, but should not be used as a distinct entity until reliable diagnostic criteria have been developed. PMID:- ADEM Research 11376180 [PubMed - indexed for MEDLINE:- ADEM Research]
Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children.
Neurology. 2001 May 22;56(10):1308-12 Authors: Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ OBJECTIVE: To identify the clinical and neuroradiologic features of acute disseminated encephalomyelitis (ADEM) in childhood. METHODS: A retrospective review was conducted of the medical records and MRI of children who presented to the Royal Children's Hospital in Melbourne with ADEM between January 1993 and December 1998. RESULTS: Of the 31 patients included in this study, 22 (71%) experienced a prodromal illness. Two patients (6%) had received hepatitis B vaccine 3 to 6 weeks before developing their illness. Symptoms and signs typically evolved over several days. Ataxia was the most common presenting feature, occurring in 20 patients (65%). MRI findings were variable, but lesions were most commonly seen bilaterally and asymmetrically in the frontal and parietal lobes. The authors found a high incidence of the corpus callosal and periventricular changes more typically associated with MS, but they also found a high rate of deep gray matter involvement (61% of patients). The use of high-dose IV methylprednisolone was usually associated with rapid recovery. Eighty-one percent of patients recovered completely, with only mild sequelae recorded in the remaining children. CONCLUSION: In the absence of a biological marker, the distinction between ADEM and MS cannot be made with certainty at the time of first presentation, but the authors suggest that a viral prodrome, early-onset ataxia, high lesion load on MRI, involvement of the deep gray matter, and absence of oligoclonal bands are more indicative of ADEM. PMID:- ADEM Research 11376179 [PubMed - indexed for MEDLINE:- ADEM Research]
Recurrence of acute disseminated encephalomyelitis at the previously affected brain site.
Arch Neurol. 2001 May;58(5):797-801 Authors: Cohen O, Steiner-Birmanns B, Biran I, Abramsky O, Honigman S, Steiner I BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a usually monophasic demyelinating disorder of the central nervous system. Recurrences pose a diagnostic challenge because they can be overlooked or suggest an alternative diagnosis. OBJECTIVE: To examine the frequency, nature, and outcome of recurrent ADEM. DESIGN: Review of the medical records of patients diagnosed in our institution as having ADEM between January 1, 1983, and May 31, 1998. Recurrences were defined as appearance of new symptoms and signs at least 1 month after the previous episode. RESULTS: Five (24%) of 21 patients with ADEM developed recurrent disease episodes. In all, diagnosis was confirmed by brain biopsy. One patient had 4 disease episodes, 2 had 3, and the other 2 each had 2. Recurrence appeared 1.5 to 32 months after initial presentation and involved the same brain territory in 6 of 9 recurrences in 3 of 5 patients. In 2 patients, recurrences included neuropsychiatric signs. A good response to corticosteroid therapy was observed in 10 of 13 of treated ADEM attacks: in 3 of the 4 treated initial events and in 7 of 9 recurrences. CONCLUSIONS: Recurrent ADEM may be more prevalent than previously recognized. Patients who relapse tend to have more than 1 recurrence that usually involves, clinically and radiologically, a brain territory that was affected before and can simulate a space-occupying lesion that requires histologic diagnosis. Neuropsychiatric features may be the main presentation of a relapse. Since recurrent ADEM is a corticosteroid-responsive condition, awareness and early diagnosis are mandatory. PMID:- ADEM Research 11346375 [PubMed - indexed for MEDLINE:- ADEM Research]
Special feature: radiological case of the month. Denouement and discussion: acute disseminated encephalomyelitis.
Arch Pediatr Adolesc Med. 2000 Dec;154(12):1269-70 Authors: Semnani H, Reminajes AV, Tavakoli Z PMID:- ADEM Research 11115316 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children.
Brain. 2000 Dec;123 Pt 12:2407-22 Authors: Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG Forty-eight children with disseminated demyelination of the CNS, 28 with acute disseminated encephalomyelitis (ADEM), seven with multiphasic disseminated encephalomyelitis (MDEM) and 13 with multiple sclerosis were studied for a mean follow-up period of 5.64 years. The presentation findings of the ADEM/MDEM group were compared with those of the multiple sclerosis group. The following findings were more commonly seen in ADEM/MDEM presentation compared with the multiple sclerosis presentations: predemyelinating infectious disease (74 versus 38%, P: < 0.05); polysymptomatic presentation (91 versus 38%, P: < 0.002); pyramidal signs (71 versus 23%, P: < 0.01); encephalopathy (69 versus 15%, P: < 0.002); and bilateral optic neuritis (23 versus 8%, not significant). Seizures occurred only in the ADEM/MDEM group (17 versus 0%, not significant). Unilateral optic neuritis occurred only in the multiple sclerosis patients (23 versus 0%, P: < 0.01). There were no differences in the frequencies of transverse myelitis, brainstem involvement, cerebellar signs and sensory disturbance between the two groups. ADEM/MDEM patients were more likely to have blood leucocytosis (64 versus 22%, P: < 0.05), CSF lymphocytosis (64 versus 42%, not significant) and CSF protein elevation (60 versus 33%, not significant). Patients presenting with multiple sclerosis were more likely to have intrathecal synthesis of oligoclonal bands on presentation (64 versus 29%, not significant). MRI showed that subcortical white matter lesions were almost universal in both groups, though periventricular lesions were more common in multiple sclerosis (92 versus 44%, P: < 0.01). By contrast, in ADEM/MDEM there was absolute and relative periventricular sparing in 56 and 78% of patients, respectively. Follow-up MRI revealed complete or partial lesion resolution in 90% and no new lesions in the ADEM/MDEM group. All of the multiple sclerosis patients had new lesions on repeat MRI (five during relapse and six during asymptomatic convalescent phases). The outcome in the ADEM patients was mixed; 57% of patients made a complete recovery. The mean follow-up for the 35 ADEM/MDEM patients was 5.78 years (range 1.0-15.4 years). Eight of the 13 multiple sclerosis patients relapsed within the first year; 11 had a relapsing-remitting course, one a primary progressive course and one a secondary progressive course. These differences in the presentation of ADEM/MDEM compared with multiple sclerosis may help in the prognosis given to families regarding the possibility of later development of multiple sclerosis.
PMID:- ADEM Research 11099444 [PubMed - indexed for MEDLINE:- ADEM Research]
Severe acute disseminated encephalomyelitis with normal MRI at presentation. Neurology. 2000 Oct 24;55(8):1237-8 Authors: Murray BJ, Apetauerova D, Scammell TE
PMID:- ADEM Research 11071514 [PubMed - indexed for MEDLINE:- ADEM Research]
Early diagnostic magnetic resonance imaging in acute disseminated encephalomyelitis. South Med J. 2000 Aug;93(8):793-6 Authors: Okun MS, Millar B, Watson R We report two cases and review the literature concerning the importance of early magnetic resonance imaging (MRI) of the brain as a guide for the early diagnosis and treatment of acute disseminated encephalomyelitis (ADEM). A nonspecific term, ADEM refers to an acute disease that is postinfectious, parainfectious, postvaccinal, or of an unknown precipitating factor. Often when there is clinical suspicion of ADEM, MRI is not done before significant morbidity and mortality occur, despite the existence of adequate treatments. Primary care physicians should be aware of the importance of early MRI in ADEM.
PMID:- ADEM Research 10963511 [PubMed - indexed for MEDLINE:- ADEM Research]
Susac syndrome. Am J Ophthalmol. 2000 May;129(5):682-4 Authors: Murata Y, Inada K, Negi A PURPOSE: To report the first Japanese case of the Susac syndrome characterized by microangiopathy of the brain, retina, and cochlea. METHODS: Case report. A 36-year-old Japanese man underwent extensive clinical and laboratory examination for encephalopathy, bilateral recurrent branch retinal arterial occlusion, and hearing loss. RESULTS: This case was initially diagnosed as acute disseminated encephalomyelitis. Despite exhaustive clinical and laboratory examination of the patient, no evidence of a known systemic disease was found. The bilateral recurrent branch retinal arterial occlusions were widely disseminated. Remission occurred 1 year after symptom onset. This patient demonstrated all of the clinical features associated with the Susac syndrome. CONCLUSION: Susac syndrome has been considered rare, but it is probably more common than previously thought and seems to occur in any race. This disorder usually occurs in young women, but it can affect men.
PMID:- ADEM Research 10844072 [PubMed - indexed for MEDLINE:- ADEM Research]
Encephalomyelitis-associated antimyelin autoreactivity induced by streptococcal exotoxins. Neurology. 2000 Apr 11;54(7):1433-41 Authors: Jorens PG, VanderBorght A, Ceulemans B, Van Bever HP, Bossaert LL, Ieven M, Goossens H, Parizel PM, Van Dijk H, Raus J, Stinissen P OBJECTIVE: After implicating Streptococcus pyogenes as causing acute disseminated encephalomyelitis (ADEM) in a child, we wanted to prove that in vivo activation of autoreactive T lymphocytes by superantigens of this Streptococcus contributed to the dramatic demyelination. BACKGROUND: ADEM is a demyelinating disorder of the CNS sharing many similarities with MS. Demyelination in MS is considered to be the result of an autoimmune process mediated by autoreactive T lymphocytes with specificity for myelin antigens. METHODS: Phenotypic analysis and proliferation assays on blood monocytes, as well as isolation of myelin basic protein (MBP)-reactive T-cell lines/clones; and TCR repertorium analysis by PCR-ELISA and cytokine production. RESULTS: 1) The blood T-cell receptor (TCR) repertoire was compatible with in vivo expansion induced by S. pyogenes exotoxins. 2) TCR expression analysis indicated clonal expansion of CD8+ MBP-reactive T cells, suggesting in vivo activation. MBP-reactive T cells showed crossreactivity to S. pyogenes supernatant and exotoxins. 3) Cytokine mRNA quantification of the mononuclear cells revealed a Th2-biased profile. CONCLUSION: In vivo exposure to S. pyogenes may have induced activation of pathogenic myelin reactive T cells, contributing to the dramatic inflammatory demyelination.
PMID:- ADEM Research 10751252 [PubMed - indexed for MEDLINE:- ADEM Research]
Treatment of acute disseminated encephalomyelitis with intravenous immunoglobulin. Neurology. 2000 Mar 28;54(6):1370-2 Authors: Sahlas DJ, Miller SP, Guerin M, Veilleux M, Francis G Acute disseminated encephalomyelitis (ADEM) is a presumed immune-mediated, demyelinating disease of the CNS for which the standard treatment is high-dose steroids. We describe two patients with ADEM in whom treatment with IV methylprednisolone coincided with deterioration in their clinical status. They were subsequently treated with IV immunoglobulin and exhibited dramatic clinical improvement, with return to their previous level of functioning.
PMID:- ADEM Research 10746613 [PubMed - indexed for MEDLINE:- ADEM Research]
Acute disseminated encephalomyelitis: MR imaging features. AJR Am J Roentgenol. 1999 Oct;173(4):1101-7 Authors: Singh S, Alexander M, Korah IP PMID:- ADEM Research 10511187 [PubMed - indexed for MEDLINE:- ADEM Research]
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