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Progressive multifocal leukoencephalopathy presenting with acute sensorineural hearing loss in an intestinal transplant recipient
Author(s) -
Lippa Andrew M.,
Ocwieja Karen E.,
Iglesias Julie,
Fawaz Rima,
Elisofon Scott,
Lee Christine,
Sharma Tanvi S.
Publication year - 2020
Publication title -
transplant infectious disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.69
H-Index - 67
eISSN - 1399-3062
pISSN - 1398-2273
DOI - 10.1111/tid.13304
Subject(s) - medicine , progressive multifocal leukoencephalopathy , sensorineural hearing loss , immunosuppression , leukoencephalopathy , hearing loss , lumbar puncture , pathology , audiology , multiple sclerosis , immunology , radiology , cerebrospinal fluid , magnetic resonance imaging
Abstract A 20‐year‐old male presented 3.5 years after intestinal transplantation with rapidly progressive sensorineural hearing loss. Initial brain imaging was consistent with inflammation and/or demyelination. Lumbar puncture was initially non‐diagnostic and a broad infectious workup was unrevealing. Three months after presentation, a repeat LP detected JC virus for which tests had not earlier been conducted. He continued to deteriorate despite withdrawal of prior immunosuppression and addition of mirtazapine, maraviroc, and steroids. He died of progressive neurologic decompensation 5 months after his initial presentation. This case highlights progressive multifocal leukoencephalopathy (PML) as a rare complication after solid organ transplantation and acute sensorineural hearing loss as an unusual first presenting symptom of PML. JC virus should be considered in the differential diagnosis of acute sensorineural hearing loss in any immunocompromised patient.

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