An uncommon illness with a rare presentation: neurosurgical management of ADEM with tumefactive demyelination in children.

Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery
Matthew VanLandinghamJonathan Fratkin

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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Citations

Jul 3, 2013·Nihon Rinshō Men'eki Gakkai kaishi = Japanese journal of clinical immunology·Yumiko NakazawaHirokazu Sakai
Apr 6, 2012·Current Treatment Options in Neurology·Daniela Pohl, Silvia Tenembaum
Mar 29, 2011·Neurologic Clinics·Tanuja ChitnisEmmanuelle Waubant
Apr 13, 2011·Pediatric Neurology·Claire J HinnellJean K Mah
Sep 7, 2012·Journal of Child Neurology·David Vargas-Lowy, Tanuja Chitnis
Aug 23, 2011·Journal of Neuro-ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society·Michael K YoonTimothy J McCulley
May 20, 2021·AJR. American Journal of Roentgenology·Mariko NakayamaToshio Moritani

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