Abstract Details

Title Adult-Onset Fulminant Necrotizing Encephalitis: A Rare Variant of Neurosarcoidosis

Topic Multiple Sclerosis

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-067

Objective To report a case of necrotizing encephalitis resistant to immunotherapy in an adult immunocompetent patient.

Background Neurosarcoidosis is an inflammatory granulomatous disease of the nervous system. Usually it is a manifestation of systemic sarcoidosis, but a small percentage can be isolated to the nervous system. Necrotizing form of neurosarcoidosis is a rare reported subtype, but is not well understood. Acute necrotizing encephalitis has mainly been described as a parainfectious encephalitis in children, but it is rarely reported in adults and not known to be associated with neurosarcoidosis.

Design/Methods Not applicable

Results A 61-year old healthy Caucasian male presented with a 6 week history of transient left facial tingling and weakness, then confusion, personality change, and 65 pound unintentional weight loss, with a viral prodrome. His initial neurologic exam revealed disorientation and transcortical sensory aphasia. MRI brain demonstrated multifocal bilateral small cortical, subcortical and deep gray T2 hyperintensities, some with enhancement and restricted diffusion. CSF showed lymphocytic pleocytosis and mildly elevated protein An extensive infectious, autoimmune, hypercoagulable, and malignancy evaluation was unremarkable. Serum and CSF paraneoplastic profile returned negative. An EEG was negative for epileptic discharges or seizures. He was treated with high dose intravenous methylprednisolone (IVMP) with partial improvement. His immunotherapy was interrupted for a brain biopsy at another institution, which suggested evidence of necrotizing encephalitis with acute inflammation and giant cells and no vasculitis. Upon follow up at our institution, he had worsened cognitive impairment and evidence of radiological progression. Cyclophosphamide was initiated, however despite this he continued to decline. He did not respond to repeat course of IVMP and plasma exchange. He transitioned to comfort measures and passed away. An autopsy was not obtained.

Conclusions We present this biopsy-proven, fulminant case of necrotizing encephalitis as a rare variant of neurosarcoidosis that should be considered in the spectrum of this disease.

Authors/Disclosures
Amanda Thuringer, DO (University of Kansas Medical Center) PRESENTER	Dr. Thuringer has nothing to disclose.
Yasir N. Jassam, MD, FRCP, FAAN (Hoag Specialty clinic)	Dr. Jassam has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Jassam has received research support from Roche, Chugai Pharmaceutical MedImmune LLC.Novartis, MedDay. Actelion, Alexion Pharmaceuticals,TG Therapeutics Alexion Pharmaceuticals, Teva Pharmaceuticals, Genentech phamarcetucal companies..

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