Abstract Details

Title Varicella Zoster Encephalomyelitis in an Immunocompetent Patient without Rash

Topic Infections/AIDS/Prion Disease

Presentation(s) P03 - (-)

Poster/Presentation
Number 248

Objective

Background BACKGROUND: VZV can present with varying neurological manifestations, the most common being post-herpetic neuralgia. A rash may or may not present with VZV infection, however typically immunocompetent patients present with simply a rash and distant, minute areas of small-vessel vasculitis. Conversely, immunocompromised patients display more disseminated disease of small vessels including vasculopathy, myelopathy, Ramsay-Hunt syndrome, cerebellitis, and rarely, encephalomyelitis. VZV encephalomyelitis is a unique entity, with the proposed underlying pathophysiological process that of small vessel vasculitis, inflammation and subsequent demyelination. It was traditionally thought that VZV encephalomyelitis was a disease of the immunocompromised, however there have been increasing numbers involving immunocompetent patients as more clinicians utilize cerebrospinal (CSF) PCR and antibody studies to diagnose VZV as a cause for neurologic disease. To date, there have been fewer than 3 case reports of VZV encephalomyelitis involving immunocompetent patients.

Design/Methods DESIGN/METHODS: The patient's history, exam, clinical and laboratory data and MRI imaging at presentation, as well as longer term follow up investigations were performed and reviewed, including an extensive literature search on previous case reports and publications in this area.

Results RESULTS: CSF, PCR and viral studies as well as correlates on brain and spinal cord imaging and improvement with anti-virals, prove varicella zoster encephalomyelitis in this patient presenting with a progressive paraparesis and myelopathy, encephalitis and cranial neuropathies.

Conclusions CONCLUSIONS: This is the first report of a patient with extensive VZV encephalomyelitis involving the spinal cord, brain, meninges, and cranial nerves without an identifiable rash, and highlights the importance of early consideration of this diagnosis in order to prevent the often aggressive neurologic deterioration seen in these cases.

Authors/Disclosures
Karen M. Lynch, MD PRESENTER	Dr. Lynch has received personal compensation for serving as an employee of Sanofi. Dr. Lynch has or had stock in Sanofi.
	No disclosure on file
Maria A. Rocca (Neuroimaging Research Unit)	Maria Assunta Rocca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen, Bristol Myers Squibb, Eli Lilly, Janssen, Roche. Maria Assunta Rocca has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for AstraZaneca, Biogen, Bristol Myers Squibb, Bromatech, Celgene, Genzyme, Horizon Therapeutics Italy, Merck Serono SpA, Novartis, Roche, Sanofi and Teva. The institution of Maria Assunta Rocca has received research support from MS Society of Canada, the Italian Ministry of Health, the Italian Ministry of University and Research, and Fondazione Italiana Sclerosi Multipla.
	No disclosure on file
Mithila Vullaganti, MD (Tufts Medical Center)	Dr. Vullaganti has nothing to disclose.

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