Abstract Details

Title Adult-Onset Recurrent Fulminant Acute Disseminated Encephalomyelitis (ADEM) Requiring Hemicraniectomy – A Case Report

Topic Multiple Sclerosis

Presentation(s) P13 - Poster Session 13 (5:30 PM-6:30 PM)

Poster/Presentation
Number 9-003

Objective To describe a rare case of adult-onset recurrent fulminant ADEM requiring hemicraniectomy.

Background ADEM is an autoimmune mediated disorder that predominantly affects children and usually has a monophasic course. Although adult-onset has been described, recurrent fulminant ADEM requiring hemicraniectomy is extremely rare.

Design/Methods N/A

Results A 37-year-old female was diagnosed with ADEM at age 31, after presenting with expressive aphasia and right hemiparesis two weeks following mycoplasma pneumonia. Brain MRI showed an extensive T2 hyperintensity involving the left fronto-parietal lobe white matter extending to the left internal capsule and brainstem with subtle enhancement. CSF was unremarkable. She was treated with steroids followed by IVIG with resolution of symptoms and imaging improvement. The patient did well until 6 years later, when she developed severe headache and visual difficulties, three weeks after an upper respiratory infection. Exam showed a left homonymous hemianopsia and mild left hemiparesis. Brain MRI demonstrated a diffuse T2 hyperintense signal involving the right parieto-occipital lobe white matter and corpus callosum with minimal enhancement. CSF showed 2,086 WBC, 71% neutrophils, protein 225 mg/dl, glucose 59mg/dl. Infectious and other inflammatory/autoimmune etiologies were ruled-out. The patient was diagnosed with recurrent ADEM and started on IV steroids. However, she deteriorated neurologically with decreased consciousness and left hemiplegia. Repeat imaging showed marked progression of the T2 hyperintensity in the right hemisphere white matter with extension to the contralateral side and associated subfalcine and transtentorial herniation prompting decompressive right hemicraniectomy. She was kept on steroids and treated with plasma exchange followed by IVIG with gradual improvement. At six-month follow-up, her examination was remarkable only for left homonymous hemianopsia and mild sensory neglect with significantly improved MRI

Conclusions Recurrent ADEM is a rare disease that can affect adults and be fulminant. Prompt recognition and aggressive treatment, including surgical intervention, can lead to a favorable outcome.

Authors/Disclosures
Maria Antonietta Mazzola, MD (Beth Israel Lahey Health) PRESENTER	Dr. Mazzola has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentec.
Nilufer Yalcin, MD	Dr. Yalcin has nothing to disclose.
Fatemeh Khadjevand, MD, MPH (Tufts Medical Center)	Dr. Khadjevand has nothing to disclose.
David P. Lerner, MD (One Brooklyn Health)	Dr. Lerner has received publishing royalties from a publication relating to health care.
Claudia J. Chaves, MD, F�鶹��ýӳ�� (Newton Wellesley Hospital)	No disclosure on file

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