好色先生

好色先生

Explore the latest content from across our publications
Join The AAN

Log In

Forgot Password?
Create New Account

Loading... please wait

Abstract Details

“String of Pearls” Sign in Progressive Multifocal Leukoencephalopathy — A Radiologic Hallmark Beyond Susac’s Syndrome
Infectious Disease
P9 - Poster Session 9 (5:00 PM-6:00 PM)
3-006
NA
"String of pearls" sign is a radiologic hallmark for Susac’s syndrome representing microinfarcts along the internal capsules due to autoimmune vasculitis.
NA
A 64-year-old woman with history of rheumatoid arthritis and immunosuppressive medication use presented with dysarthria, right-sided ataxia, and gait imbalance. Her brain MRI showed T2-weighted fluid-attenuated inversion recovery (T2-FLAIR) hyperintensities involving the right cerebellum and pons. Her cerebrospinal fluid (CSF) analysis showed positive JCV PCR confirming the diagnosis of progressive multifocal leukoencephalopathy (PML). PML continued to progress despite experimental immunotherapy. Her follow-up MRI 6 months after the diagnosis showed punctate hyperintense lesions on diffusion-weighted imaging (DWI) that appeared isointense on apparent diffusion coefficient (ADC) and T2-FLAIR hyperintensities associated with hypointense signal on T1-weighted images extending from the brainstem to the internal capsules along the corticospinal tracts as well as in bilateral posterior limbs of the internal capsules in "string of pearls" appearance on axial slices.

Although "string of pearls" sign is classically associated with Susac’s syndrome, representing microinfarcts in the internal capsules, we describe its appearance in progressive multifocal leukoencephalopathy as a result of lesion extension along the corticospinal tracts.
Authors/Disclosures
Busranur Agac, MD (National Institutes of Health)
PRESENTER 		Dr. Agac has nothing to disclose.
Govind Bhagavatheeshwaran (National Institutes of Health) Mr. Bhagavatheeshwaran has received research support from National Institutes of Health.
Serhat V. Okar, MD Mr. Okar has nothing to disclose.
Yair Mina, MD (NIH) Dr. Mina has nothing to disclose.
Daniel Reich, MD, PhD (National Institutes of Health, Neuroimmunology Branch, NINDS) Dr. Reich has received research support from NIH. The institution of Dr. Reich has received research support from Adelson Medical Research Foundation. The institution of Dr. Reich has received research support from Sanofi. The institution of Dr. Reich has received research support from Abata Therapeutics. The institution of Dr. Reich has received research support from National Multiple Sclerosis Society. The institution of Dr. Reich has received research support from Cure Alzheimer's Fund. Dr. Reich has received intellectual property interests from a discovery or technology relating to health care. Dr. Reich has received personal compensation in the range of $5,000-$9,999 for serving as a CME Faculty with Integrity. Dr. Reich has received personal compensation in the range of $500-$4,999 for serving as a CME Faculty with Letters and Sciences. Dr. Reich has received personal compensation in the range of $500-$4,999 for serving as a CME Faculty with Academic CME. Dr. Reich has a non-compensated relationship as a Advisor with Sanofi that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Board of Directors with ACTRIMS that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Advisor with Abata Therapeutics that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Advisor with University of Basel RC2NB that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Advisor with Multiple Sclerosis Society of Canada that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Advisor with Tuscan Doctorate in Neuroscience that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Editorial Board with Multiple Sclerosis Journal that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Collaborator, Advisor with Hyperfine that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Collaborator with Imaginab that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Collaborator with Annexon that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Collaborator, Advisor with Philips that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Collaborator with Siemens that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Advisor with Calico Life Sciences that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Advisor with Cognito Therapeutics that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Advisor with Sudo that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Advisor with Allumis that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Advisor with BioCentury that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Collaborator with Regeneron that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Collaborator with Eli Lilly that is relevant to AAN interests or activities. Dr. Reich has a non-compensated relationship as a Advisor with SetPoint that is relevant to AAN interests or activities.
Irene Cortese, MD Dr. Cortese has stock in Keires, AG. Dr. Cortese has stock in Nouscom, AG. Dr. Cortese has stock in PDC*line Pharma.