What lies beneath the tent? JC-virus cerebellar granule cell neuronopathy complicating sarcoidosis.
A 49-year-old, HIV-negative woman with sarcoidosis presented with a subacute unilateral cerebellar syndrome. A brain MRI revealed a hyperintense lesion without mass effect in the left cerebellar hemisphere, but no pathology above the tentorium. Steroid therapy for presumed neurosarcoidosis was ineffective and the patient deteriorated progressively. Cerebellar biopsy showed abnormal granule cells and demyelination. Immunocytochemistry confirmed the diagnosis of progressive multifocal leucoencephalopathy (PML) with JC (John Cunningham) virus granule cell neuronopathy. The patient succumbed to progressive brainstem dysfunction despite treatment with cidofovir. Although rare, PML should be considered in all patients with impaired cell-mediated immunity and unexplained neurological dysfunction, even in the absence of HIV infection.
Granot
Ron
R
Department of Neurology, St George Hospital, Kogarah, New South Wales, Australia.
Lawrence
Richard
R
Barnett
Michael
M
Masters
Lynette
L
Rodriguez
Michael
M
Theocharous
Con
C
Pamphlett
Roger
R
Hersch
Mark
M
D000998:Antiviral Agents / Q000627:therapeutic use; D002526:Cerebellar Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D002531:Cerebellum / Q000473:pathology*; D000077404:Cidofovir; D003596:Cytosine / Q000031:analogs & derivatives / Q000627:therapeutic use; D003711:Demyelinating Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007577:JC Virus; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D063065:Organophosphonates / Q000627:therapeutic use; D012507:Sarcoidosis / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D013256:Steroids / Q000627:therapeutic use
D000998:Antiviral Agents; D063065:Organophosphonates; D013256:Steroids; D003596:Cytosine; D000077404:Cidofovir