Acute disseminated encephalomyelitis in a five-year-old girl: a case report

Abstract

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating central nervous system disorder with a predilection to early childhood. ADEM is generally considered a monophasic disease. However, recurrence has been described and defined as multiphasic disseminated encephalomyelitis (MDEM). ADEM often occurs after infection or immunization and is clinically defined as acute polyfocal neurological deficits, including encephalopathy. Many times, ADEM is a diagnosis of exclusion, and early diagnosis and treatment are the keys to favorable outcomes. Magnetic resonance imaging (MRI) typically demonstrates reversible, large, ill-defined white matter lesions of the brain and often also the spinal cord, along with frequent involvement of thalami and basal ganglia. CSF analysis may reveal mild pleocytosis and elevated protein but is generally negative for intrathecal oligoclonal bands. We report a case of five years six-month- old girl who presented with fever, vomiting, headache, and cough for seven days. She had one episode of involuntary passage of urine. The child was lethargic at presentation and was unable to stand or walk. Clinical features and investigations, including MRI brain and spine, were suggestive of ADEM. She was started on Inj. Methylprednisolone followed by oral prednisolone in tapering dose for four weeks. Clinical improvement was seen in the form of improvement in activity and power in limbs. The child improved with normal neurological function on day three of inj methylprednisolone. On follow-up for six months, the child did not have any relapse and had complete neurological and radiological recovery.