Brass, S. D. et al. Multiple sclerosis vs acute disseminated encephalomyelitis in childhood. Pediatr. Neurol. 29, 227-231

Department of Neurology and Neurosurgery, McGill University, Montreal, Quebec, Canada
Pediatric Neurology (Impact Factor: 1.7). 10/2003; 29(3):227-31. DOI: 10.1016/S0887-8994(03)00235-2
Source: PubMed


The initial presenting clinical and laboratory findings of either acute disseminated encephalomyelitis or the first attack of multiple sclerosis in the pediatric population were compared and contrasted. A retrospective review of the medical records was conducted of all children younger than 17 years who presented with either the diagnosis of acute disseminated encephalomyelitis or multiple sclerosis between 1987 and 2001. Seventeen cases of clinically definite multiple sclerosis (seven female, mean age 12.4 +/- 4.5 years) and seven cases of acute disseminated encephalomyelitis (three female; mean age 8.7 +/- 3.8 years) were reviewed. Systemic and nonfocal neurologic symptoms were more commonly evident in acute disseminated encephalomyelitis than in multiple sclerosis: fever (43% vs 6%), headache (57% vs 24%), fatigue (71% vs 29%), vomiting (57% vs 0%), and encephalopathy (71% vs 6%). In multiple sclerosis patients, T(2)-weighted white matter magnetic resonance imaging lesions were more commonly located in the corpus callosum (64% vs 17%) and the periventricular area (91% vs 50%) compared with those in patients with acute disseminated encephalomyelitis. These results suggest that acute disseminated encephalomyelitis and multiple sclerosis can be differentiated to some degree according to clinical and radiologic data at initial presentation, which is important because the long-term prognosis for childhood multiple sclerosis appears to be less favorable.

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    • "Distinction between ADEM and MS at initial presentation cannot be made, although some clinical, radiologic, and even biochemical features may point toward one or the other diagnosis.[1116] Establishing the diagnosis of MS may require prolonged follow-up as the second attack of MS may occurs over a period of months to several years.[17] So, clinical and radiological (MRI scan) follow-up of children with ADEM beyond the first few months may help in the early diagnosis of MS as the early treatment of MS has been shown to prevent the progression of the disease.[18] "
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    ABSTRACT: ADEM, although relatively uncommon, is probably under-recognized. To spotlight the clinical profile and therapeutic outcome of children with ADEM. This is a prospective study of patients with ADEM who were admitted to the Pediatric Departments in Aladan and Alfarawanya Hospitals in Kuwait, from January 2009 to January 2011. Clinical, microbiological and radiological data were analyzed. Of 48 patients presented with acute neurological symptoms and signs, 21 patients fulfilled criteria for ADEM. 80.95% of cases were presenting in winter and spring, 57% of patients had a history of upper respiratory tract illness. The commonest presentations were motor deficits, convulsions and altered consciousness. CSF virology studies showed herpes simplex virus (HSV) and Epstein-Barr virus (EBV) (3 patients) whereas nasal and nasopharyngeal swab showed evidence of influenza H1N1 virus (1 patient). Brain MRI was performed in all patients and revealed multiple hyperintense supratentorial brain lesions on T2/FLAIR images. 85.7% of patients had cortical and/or subcortical white matter lesions which were bilateral and asymmetric in location and size. ADEM although rare must be considered in children with acute onset of neurological signs and symptoms and must be distinguished from any acute neurological insult.
    No preview · Article · Mar 2013 · Journal of Pediatric Neurosciences
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    • "In addition to encephalopathy, the most common neurologic features of ADEM include long tract (pyramidal tract) signs, acute hemiparesis, cerebellar ataxia, cranial neuropathies, including optic neuritis, and spinal cord dysfunction (transverse myelitis)2-4,18,19). Symptoms of optic neuritis include vision loss, pain with eye movement, and an afferent pupillary defect. "
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    ABSTRACT: Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalopathy. ADEM is considered an autoimmune disorder that is triggered by an environmental stimulus in genetically susceptible individuals. The diagnosis of ADEM is based on clinical and radiological features. Most children with ADEM initially present with fever, meningeal signs, and acute encephalopathy. The level of consciousness ranges from lethargy to frank coma. Deep and subcortical white-matter lesions and gray-matter lesions such as thalami and basal ganglia on magnetic resonance imaging (MRI) are associated with ADEM. In a child who presents with signs of encephalitis, bacterial and viral meningitis or encephalitis must be ruled out. Sequential MRI is required to confirm the diagnosis of ADEM, as relapses with the appearance of new lesions on MRI may suggest either multiphasic ADEM or multiple sclerosis (MS). Pediatric MS, defined as onset of MS before the age of 16, is being increasingly recognized. MS is characterized by recurrent episodes of demyelination in the CNS separated in space and time. The McDonald criteria for diagnosis of MS include evidence from MRI and allow the clinician to make a diagnosis of clinically definite MS on the basis of the interval preceding the development of new white matter lesions, even in the absence of new clinical findings. The most important alternative diagnosis to MS is ADEM. At the initial presentation, the 2 disorders cannot be distinguished with certainty. Therefore, prolonged follow-up is needed to establish a diagnosis.
    Full-text · Article · Jun 2011 · Korean Journal of Pediatrics
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    • "In the absence of a biological marker, the distinction between ADEM and MS cannot be made with certainty at the time of initial manifestation and a preceding or concurrent viral illness, high lesion load on MRI, involvement of the deep gray matter, or absence of oligoclonal bands may be more indicative of ADEM (5, 17). It will be very interesting if we were able to clarify whether these conditions have their own specific biological markers or share the markers. "
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    ABSTRACT: The work was done to study immunogenetic peculiarities of neuroinflammatory diseases among Korean children. A total of 13 children with neuroinflammatory diseases (8 males and 5 females; mean age 4.6±2.6 yr) were consecutively recruited. Genomic typing was performed on their HLA DRB/HLA DQB genes using PCR-SSOP/SSP techniques with gel immunoelectrophoresis. The frequencies of HLA-DR1*15 in children with acute disseminated encephalomyelitis (ADEM) (31%) and DQB1*06 in other neuroinflammatory diseases (38%) were significantly increased compared with control subjects. The frequencies of HLA-DRB3*0202 (100%), HLA-DRB1*1302 (67%), HLA-DRB3*0301 (67%), and HLA-DQB1*0301 (67%) were significantly increased in children with multiple sclerosis and the frequencies of HLA-DRB1*1501 (40%) and HLA-DRB5*0101 (40%) were significantly increased in children with ADEM. HLA-DRB1*1401, HLA-DRB3*0202, and HLA-DQB1*0502 were found in children with acute necrotizing encephalopathy. In conclusion, HLA-DR1*15 and DQB1*06 may be involved in susceptibility to inflammation in Korean children. The frequencies of HLA-DRB1*1501, HLA-DRB5*0101, HLA-DRB3*0301, and HLA-DQB1*0602 were not as high in Korean children with multiple sclerosis as in western children. However, HLA-DRB3*0202 was seen in all children with multiple sclerosis. Our data may provide further evidence that the immunogenetic background of neuroinflammatory diseases in Korean is distinctly different from the ones in western countries. Further studies are necessary to confirm this finding.
    Full-text · Article · Jun 2004 · Journal of Korean Medical Science
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