Abstract Details

Title Adult acute hemorrhagic leukoencephalitis: role of susceptibility-weighted imaging in diagnosis and importance of aggressive early immunotherapy.

Topic Autoimmune Neurology

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

Poster/Presentation
Number 15-027

Objective

We present a case of adult acute hemorrhagic leukoencephalitis emphasizing the role of susceptibility-weighted (SWI) MRI in diagnosis and the importance of aggressive early immunotherapy.

Background Acute hemorrhagic leukoencephalitis (AHLE) is a postinfectious condition characterized by extensive demyelination and foci of intracerebral hemorrhage. Although most often a condition of childhood, AHLE can also affect adults, with high morbidity and mortality.

Design/Methods A 44 year old male with history of recent upper respiratory tract infection presented with headache and encephalopathy which progressed to coma within 24 hours. Cerebrospinal fluid analysis was notable for lymphocytic pleocytosis. MRI demonstrated multifocal white matter hyperintensities on T2 and FLAIR sequences, and rim enhancement with gadolinium. Gradient recalled echo (GRE) sequences were normal but SWI sequences showed extensive microhemorrhages. Serological studies were notable for elevated immunoglobulin G antibody titers to influenza A and B and coxsackie B virus.

Results

The patient was diagnosed with AHLE and immediately treated with immunotherapy including five days of methylprednisolone and five days of plasmapheresis used concomitantly, followed by three days of intravenous immunoglobulin. He demonstrated significant clinical improvement during his immunotherapy course and was successfully discharged to inpatient rehabilitation, and later home. At follow up 3 months later, he only reported fatigue as a residual symptom and had no clinical deficits on examination. Follow-up MRI at that time demonstrated complete resolution of the T2 and FLAIR white matter hyperintensities as well as SWI microhemorrhages.

Conclusions

AHLE is a potentially catastrophic condition with high morbidity and mortality. SWI imaging may detect hemorrhagic lesions not detectable by GRE. Aggressive immunotherapy begun emergently may result in substantial recovery.

Authors/Disclosures
Dana A. Sugar, MD (University of New Mexico) PRESENTER	Dr. Sugar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Abbvie.
Jonathan R. Galli, MD (University of Utah)	Dr. Galli has nothing to disclose.
Stacey Clardy, MD, PhD, FAAN (University of Utah)	Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca/Alexion. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen/Horizon. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. Dr. Clardy has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology/AAN Publications. The institution of Dr. Clardy has received research support from NIH/NINDS. The institution of Dr. Clardy has received research support from SRNA. The institution of Dr. Clardy has received research support from Alexion/AstraZeneca. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with U of Iowa, Miami, Stanford, Barrow, Beaumont Health, CCF, Emory, Penn State, Mayo Clinic, Walter Reed.
John E. Greenlee, MD, FAAN (University of Utah)	Dr. Greenlee has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Medlink. Dr. Greenlee has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Zeigler Cohen Roche. Dr. Greenlee has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Sommers Schwartz PC. Dr. Greenlee has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for St Francis Hospital. Dr. Greenlee has received publishing royalties from a publication relating to health care. Dr. Greenlee has received publishing royalties from a publication relating to health care.

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