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Abstract Details

Broadening the Radiographic Spectrum of PML: PML with Homogeneously DWI-Hyperintense and ADC-Hypointense Lesions
Infections/AIDS/Prion Disease
P06 - (-)
183
BACKGROUND: PML is a central nervous system demyelinating disease that most often occurs in the setting of immunosuppression and in particular HIV infection with low CD4 counts.The spectrum of clinical and radiographic findings is broad. MRI lesions typically have low signal on T1,and high signal on T2 and FLAIR sequences. Typical diffusion imaging in PML shows heterogeneity with a mix of high DWI/low ADC signal at the lesions periphery,with high DWI/high ADC at the lesions center.Areas of high DWI/low ADC correlate with regions of active infection and oligodendrocyte swelling on pathology,whereas areas of high DWI/high ADC correlate with gliosis.
DESIGN/METHODS: Case report.
RESULTS: We present a 46-year-old hepatitis C- and HIV-positive man (HIV diagnosed 23 years ago) with no identified prior AIDS-defining illnesses. The patient had a CD4 count of 734 cells/[micro]L with a stably undetectable viral load. He was treated with efavirenz, emtricitabine and tenofovir for 6 years.The patient was referred to the emergency department with insidious onset multifocal,irregular,hyperkinetic movements most consistent with chorea. Brain MRI showed multifocal FLAIR high-signal lesions with the classic appearance of PML. However, there was strikingly homogenous and dense high-signal DWI lesions with concurrent low-ADC signal - a combination of findings most frequently seen with acute cerebral infarction. After testing to rule out alternative diagnoses,a diagnosis of probable PML was made. The patient remained on HAART but chorea persisted.
CONCLUSIONS: The distinct and homogeneous high DWI/low ADC lesions extend the known radiographic spectrum of PML. We hypothesize that the patient's relatively preserved immune system led to a vigorous inflammatory response with associated cytotoxic edema throughout the active lesion, thus leading to this atypical picture.
Authors/Disclosures
Victor Ferastraoaru, MD (Montefiore Medical Ctr-Neurology Dept)
PRESENTER
Dr. Ferastraoaru has nothing to disclose.
David Bearden, MD (University of Rochester School of Medicine) Dr. Bearden has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Bearden has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Praxis. Dr. Bearden has received personal compensation in the range of $100,000-$499,999 for serving as an Expert Witness for law firms.
Constantine Farmakidis, MD, FAAN Dr. Farmakidis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Farmakidis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for J&J. Dr. Farmakidis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Muscular Dystrophy Association.
Clifford R. Jack, Jr., MD (Mayo Clinic) The institution of Dr. Jack has received research support from NIH. The institution of Dr. Jack has received research support from Alexander Family Alzheimer's Disease Research Professorship of the Mayo Clinic.