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A 27 year-old woman presented with the acute onset of rapidly progressive quadriplegia and confusion associated with fever and headache. She had a mumps, rubella and measles vaccination ten days earlier. |
Acute Disseminated Encephalomyelitis (ADEM):
T2-weighted sagittal images of the spine: (Left) Cervical and upper
thoracic spine; (Right) Close up view of the lower thoracic spine. Note that
nearly the entire spinal cord has intramedullary demyelinating lesions (bright on T2). In
this patient, nearly all of the clinical deficits (other than her
confusion) were the result of
her spinal cord involvement.
ADEM is a monophasic demyelinating syndrome that occurs as an autoimmune phenomenon typically following a viral infection or immunization. It is characterized clinically by rapid development of focal or multifocal neurological dysfunction. Clinical features include encephalopathy ranging from lethargy to coma, seizures, and focal or multifocal signs reflecting involvement of the cerebrum (hemiparesis), brainstem (cranial nerve palsies), and spinal cord (paraparesis). ADEM presents acutely, with features similar to encephalitis (fever, headache, seizures and coma). In some cases, it may not be possible to distinguish ADEM from the first episode of multiple sclerosis (MS). The mortality rate varies between 10% and 30%, with complete recovery in 50%. |
Revised
11/29/06
Copyrighted 2006. David C Preston