Abstract
This study determined the statewide incidence and prevalence of acute disseminated encephalomyelitis (ADEM) and examined the course of three pediatric patients treated for tumefactive demyelination (TD) at the Blair E. Batson Children's Hospital. Analyses of ICD-9-CM code hospital records and clinical database were conducted. From 2001 through 2007 the incidence in pediatric patients under 20 years was 0.4/100,000/year, with a prevalence of 8.6/100,000 during 2008. Three patients presented with TD. Case 1 had a 3-week history of ataxia and diplopia; case 2 presented with a sudden onset of coma, while the third child had a 4-month history of increasing lethargy and clumsiness in all extremities. Cerebrospinal fluid examinations were nondiagnostic. MRI examinations revealed asymmetric T2/fluid-attenuated inversion recovery hyperintensity within the pons (case 1), a large heterogenously enhancing temporal lobe mass, with extensive edema (case 2), and multiple small brain lesions with occasional ring enhancement (case 3). In case 1, intralesional MR spectroscopy demonstrated changes consistent with ADEM. Case 2 required intracranial monitoring, and medical treatment to control elevated ICP. Cases 2 and 3 underwent cortical biopsies...Continue Reading
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