Acute disseminated encephalomyelitis
Definition
Up to three-quaters of cases may be regarded as postinfectious or postimmunization encephalomyelitis. Acute demyelinating encephalomyelitis has been historically recognised as distinct from MS based on its monophasic course and encephalopathy or coma in combination with multifocal symptoms-cerebellar signs, cerebral motor or sensory features, optic neuritis or myelitis. Typically the latency between a febrile event and the onset of neurologcial symptoms is 7-14 days. Criteria for diagnosis of acute disseminated encephalomyelitis (ADEM) 1. Subacute encephalopathy (altered level of consciousness, behaviour, or cognitive function). 2. Evolution over 1 week to 3 months; new symptoms including focal/multifocal demyelinating syndromes, such as optic neuritis or myelitis within the first 3 months from onset are allowed, as long as they are not separated by a period of complete remission from the initial symptoms (in which case the diagnosis is MS). 3. Accompanied by improvement or recovery although residual neurological deficits may be present. 4. MRI shows predominantly symptomatic white matter lesions that: Are acute (remote lesions accompanied by encephalomalacia cast doubt on the diagnosis if there is no previous explanation for them other than remote demyelinating disease). Are multiple but rarely a single large lesion. Are supra- or infra-tentorial or both Generally include at least one large (1-2cm diameter) lesion Variable enhancement with gadolinium (gadolinium enhancement is not required) May be accompanied by basal ganglia lesions, but their presence is not required.
3 subordinate codes
Nearby in Multiple sclerosis or other white matter disorders
8A40Multiple sclerosis8A41Isolated demyelinating syndromes of the central nervous system8A43Neuromyelitis optica8A44Leukodystrophies8A45Secondary white matter disorders8A46Central demyelination of corpus callosum4B4YOther specified diseases of the immune system4B4ZDiseases of the immune system, unspecified