Abstract Details

Title Chronic Granulomatous Herpes Simplex Virus Encephalitis: A Distinct but Rare Disease

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 1-084

Objective NA

Background Chronic granulomatous herpes simplex encephalitis (CG-HSVE) is a rare complication following primary herpes simplex virus (HSV) encephalitis. Herein, we highlight the extensive diagnostic workup leading to the identification of CG-HSVE, and the unique treatment approach implemented.

Design/Methods NA

Results

Herein, we present a 13-month-old female with intractable seizures and progressive neurologic decline five months after an initial HSV encephalitis diagnosis (confirmed by PCR) and adequate prophylactic oral acyclovir treatment. Imaging demonstrated a unique and striking pattern of confluent parieto-occipital lesions with irregular nodular/gyriform enhancement and vasogenic edema. Extensive investigations culminating in a brain biopsy demonstrated chronic granulomatous encephalitis (with discrete well-formed necrotizing/non-necrotizing granulomas). HSV-1 DNA was identified in parenchymal tissue without viral inclusions or immunoreactive antigens. She was treated with acyclovir, high dose corticosteroids, intravenous immunoglobulin, and mycophenolate mofetil, resulting in marked clinical and radiologic improvement.

Conclusions CG-HSVE remains a rare entity primarily presenting in the pediatric population– with only fifteen reported cases in the literature; this leads to delays in identification and variable management amongst studies. It is characterized by progressive neurological and radiographic deterioration, intractable seizures, and a characteristic MRI. In all cases, including our own, histopathology was necessary to reach a CG-HSVE diagnosis. Prognosis is variable ranging from good recovery to fatality (in four of the fifteen patients). Earlier identification and treatment are necessary given the significantly variable prognosis of this disease. CG-HSVE pathophysiology is not well understood, possibly mediated by type I interferons and inflammasome activation. Given this uncertain we argue that a two-pronged treatment approach aimed at treating both chronic HSV infection and post-infectious granulomatous inflammation is warranted.

Authors/Disclosures
Heather Yong, MD (Alberta Health Services) PRESENTER	Dr. Yong has nothing to disclose.
Cailey Turner, MD	Dr. Turner has nothing to disclose.
Ronak K. Kapadia, MD	Dr. Kapadia has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics/Amgen.
Tajdin Jadavji, MD	Prof. Jadavji has nothing to disclose.
Luis Murguía-Favela, MD	Dr. Murguía-Favela has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Encoded Therapeutics. Dr. Murguía-Favela has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Regenxbio. Dr. Murguía-Favela has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Dr. Murguía-Favela has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for BioCryst. Dr. Murguía-Favela has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Takeda. Dr. Murguía-Favela has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Grifols. Dr. Murguía-Favela has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Valeo Pharma. The institution of Dr. Murguía-Favela has received research support from Jeffrey Modell Foundation. An immediate family member of Dr. Murguía-Favela has received personal compensation in the range of $500-$4,999 for serving as a Member of Clinical Panel with Canada Drug's Agency.

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