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Abstract Details

Oculomeningoencephalomyeloradiculitis: A case of fulminant varicella zoster infection in the setting of systemic lupus erythematosus
Infectious Disease
P4 - Poster Session 4 (5:30 PM-6:30 PM)
4-036
The goal of this case is to describe the extent to which varicella zoster virus (VZV) can manifest in the central nervous system (CNS) and to emphasize the importance of early detection in an immunocompromised patient.
A 54-year-old woman with systemic lupus erythematosus (SLE) on prednisone, mycophenolate mofetil, and quinacrine presented with two months of myalgias, two weeks of severe back pain and paraparesis, and three days of rapidly progressive bulbar symptoms. Her exam was notable for complete loss of vision, ophthalmoparesis, left facial palsy, right-sided hearing loss, dysarthria, lower extremity plegia, and a T10 sensory level.

While a flare of SLE was considered, the multifocal nature and bulbar symptoms supported a broader differential including malignancy and infections. The rapid evolution of symptoms favored an aggressive infectious process in the setting of immunosuppression. MRI and CSF sampling were pursued.

MRI of the neuro-axis revealed multifocal non-enhancing parenchymal lesions, several enhancing leptomeningeal lesions, abnormal signal throughout the visual pathways, enhancement of multiple cranial nerves, and cord expansion with enhancement from T2 to the cauda equina. CSF demonstrated xanthochromia, lymphocytic pleocytosis, and elevated protein. Several viral studies in the CSF were performed, and VZV PCR returned positive. Ophthalmic evaluation was notable for progressive outer retinal necrosis. She completed two weeks of IV acyclovir. While vision did not recover, her multifocal deficits and MRI findings improved remarkably over six months.

This case highlights the fulminant nature of CNS varicella zoster infection in the setting of immune suppression. While autoimmune conditions are often characterized by flares of disease activity, the rapid and multifocal involvement seen in this case was atypical and prompted an infectious evaluation. With the neurotropism of VZV, early diagnostic testing in an immunocompromised patient with CNS symptoms is critical and empiric antiviral coverage should be considered early in the treatment course.
Authors/Disclosures
Christopher Perrone, MD (University of Pennsylvania, Department of Neurology)
PRESENTER 		Dr. Perrone has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono.
Etsegenet F. Tizazu, MD (ChristianaCare) No disclosure on file
Dina Jacobs, MD (Hosp of Univ of PA/Dept of Neruo) Dr. Jacobs has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genentech. Dr. Jacobs has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Merck/EMD Serono. Dr. Jacobs has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cycle Pharmaceuticals. Dr. Jacobs has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for TG Therapeutics. Dr. Jacobs has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi . Dr. Jacobs has received personal compensation in the range of $500-$4,999 for serving as a Consultant for William Blair &Company . Dr. Jacobs has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Jacobs has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Jacobs has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Jacobs has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi . The institution of Dr. Jacobs has received research support from Roche/Genentech .