Abstract Details

Title Progressive Multifocal Leukoencephalopathy in an Immunocompetent Patient: A Rare Case with Radiologic-Pathologic Correlation

Topic Infectious Disease

Presentation(s) P4 - Poster Session 4 (5:30 PM-6:30 PM)

Poster/Presentation
Number 4-026

Objective To report a rare case of progressive multifocal leukoencephalopathy in an immunocompetent patient.

Background Progressive multifocal leukoencephalopathy (PML) is a rapidly progressive and usually fatal demyelinating disease that primarily affects immunocompromised patients. Rarely, it can affect immunocompetent patients, in which case it can pose a diagnostic and therapeutic dilemma.

Design/Methods Case report and literature review.

Results We report a 67-year-old woman with a history of idiopathic immune complex mediated membranoproliferative glomerulonephritis with stage III chronic kidney disease (not requiring immunosuppression) who presented with rapid cognitive decline and left-sided hemiparesis over a one month period. Brain MRI showed multiple white matter T2 hyperintense lesions with incomplete peripheral enhancement but no mass effect. Cerebrospinal fluid (CSF) showed mild elevation in total protein but JC virus (JCV) PCR was negative at initial evaluation leading to diagnostic uncertainty. Repeat MRI showed interval increase in size of the lesions. Brain biopsy was pursued and lumbar puncture was repeated while awaiting histopathological studies. Histopathology was consistent with demyelination with sheets of foamy macrophages, relative axonal preservation, bizarre astrocytes, and SV40-positive viral inclusions. JCV in situ hybridization was strongly positive in scattered nuclei. Repeat CSF JCV PCR also returned positive. Detailed immunodeficiency workup including HIV testing, immunoglobulin and complement levels, and blood flow cytometry was unrevealing. The patient was started on mirtazapine and mefloquine. Her clinical course had stabilized at the time of writing this report, and she was discharged to a rehabilitation facility with plans for close outpatient monitoring.

Conclusions PML rarely occurs in the setting of immunocompetence and initial JCV testing can be negative. This can lead to inaccurate diagnosis unless there is a high index of clinical suspicion. While there is limited data regarding the disease course, prognosis and management of PML in the setting of immunosuppression, only isolated case reports exist about PML in immunocompetent patients.

Authors/Disclosures
Faisal Fecto, MD, PhD (Advocate Medical Group) PRESENTER	The institution of Dr. Fecto has received research support from NINDS.
Jason Maljaars (St. Francis Medical Center)	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Tracey Cho, MD, FAAN	Dr. Cho has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Kyverna. Dr. Cho has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Delve Bio. Dr. Cho has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for NIH. The institution of an immediate family member of Dr. Cho has received research support from NIH. Dr. Cho has received publishing royalties from a publication relating to health care.
Andrea J. Swenson, MD (University of Iowa Hospitals and Clinics)	An immediate family member of Dr. Swenson has received personal compensation for serving as an employee of IDT. Dr. Swenson has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. An immediate family member of Dr. Swenson has received stock or an ownership interest from IDT.

��ɫ��

��ɫ��