![]() Previously reported infections have included mycoplasma, Epstein–Bar's virus, coronavirus, coxsackie virus, hepatitis, herpes simplex virus, human immunodeficiency virus, human herpesvirus, parainfluenza virus, influenzas A and B, as well as measles, mumps, and rubella viruses.Ī minority of cases (5%) are associated with vaccination, including but not limited to: hepatitis B, influenza, diphtheria/pertussis/tetanus, measles/mumps/rubella, pneumococcus, and polio. The presence of area postrema syndrome is very rare and may indicate an alternative diagnosis such as neuromyelitis optica spectrum disorders (NMOSDs).Īs with other neuroimmunologic disorders, up to 72% of patients will have a non-life-threatening febrile illness days prior to up to 3 weeks prior to neurologic symptom onset. Associated nonneurologic findings include fever, meningismus, nausea/vomiting, and generalized weakness. In children, the degree of encephalopathy varies and patients may have more than depressed mental status, in fact, younger children may often present with irritability, aggression, and other active behavioral changes.Īdditional neurologic features include cerebellar ataxia, cranial neuropathy, hemiparesis/weakness, optic neuritis (ON) (which can include vision loss, pain with eye movement, and/or afferent pupillary defect), pyramidal dysfunction, and spinal cord dysfunction, which may or may not be consistent with a transverse myelitis (TM).ĭisturbances of language function, movement disorders, and alterations in sensorium are appreciated with less frequency. Encephalopathy (defined as any alteration in mental state or behavior ranging from coma to irritability) is required for the diagnosis. The clinical diagnosis of ADEM can be difficult in that symptoms are often variable and are frequently polyfocal at onset. In the latter phase, production of cytokines and chemokines by activated T cells recruits additional immune cells to the CNS leading to primary injury of white matter.Īlthough more nuanced than described, for the scope of this review, the authors will focus on the clinical aspects of the disease and its interface with emerging literature on myelin oligodendrocyte glycoprotein (MOG) antibody.Ībbreviations: ADEM, acute disseminated encephalomyelitis MRI, magnetic resonance imaging. The former phase is defined by activation and expansion of T cells with spread to the CNS. Historically, this process has been divided into two phases: the priming/activation phase and the recruitment phase. The most consistent proposed mechanism of disease is that of autoimmune priming by an environmental antigen (such as a virus or bacteria) which triggers the production of myelin autoantigens that share antigenic determinates with the causative pathogen. The pathogenesis and pathophysiology of ADEM are not fully understood. The majority of cases occur in children of age 9 years or younger and, interestingly, the rates of hospitalization for this disorder have increased over the past decade. Studies have identified marginally higher rates of ADEM in males. The estimated incidence of ADEM in children ranges between 0.2 and 0.4/100,000 and is without ethnic or racial predilection. Acute disseminated encephalomyelitis (ADEM) is an immune-mediated disorder of the central nervous system (CNS), affecting both children and adults.
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