Acute disseminated encephalomyelitis (ADEM) : report of a clinical case
Locatelli, C.; Ciambra, R.; Pasini, M.; Suprani, T.; Cataldi, M.Lia.; Pocecco, M.
La Pediatria Medica e Chirurgica Medical and Surgical Pediatrics 27(1-2): 109-114
2005
ISSN/ISBN: 0391-5387 PMID: 16922056 Document Number: 594589
Acute disseminated encephalomyelitis (ADEM) is a rare acute inflammatory demyelinating disorder of central nervous system characterized by multifocal white matter involvement. Children and young adults are more commonly affected. The onset of ADEM usually follows a viral infection or immunization after a mean period of 7-14 days. The pathogenesis is not clear but several evidences support the autommune aetiology. ADEM is characterized by multifocal neurological signs and occasionally it rapidly progresses to coma. Magnetic resonance imaging (MRI) is useful to confirm the diagnosis. Treatment is based on intravenous high dose methylprednisolone, which usually leads a rapid improvement. Recently the use of i.v. immunoglobulins has also been suggested. We report a case of a 2-year-old girl with sudden onset of neurological symptoms (irritability, drowsiness, hemiparesis, ataxia, strabismus) after an upper respiratory tract infection. MRI showed the presence of multiple high signal areas in the brain and in the spinal cord. High doses of methylprednisolone (10 mg/Kg) i.v. determined a rapid and persistent improvement of neurological signs and symptoms.