Atypical PML leading to a diagnosis of common variable immunodeficiency
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Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the brain caused by JC virus (JCV) and occurs almost exclusively in immunocompromised individuals with AIDS or chronic malignancies or in organ transplant patients on immunosuppressive therapy.1 We report a case of PML leading to a diagnosis of common variable immunodeficiency (CVID).
Case report.
A 21-year-old Caucasian man was transferred to our hospital for subacute progressive encephalopathy of 2 weeks’ duration. The patient gradually developed progressive cognitive deficits over this period, including confusion, disorientation, and word-finding difficulties. Past medical history revealed that since early childhood, he had acquired frequent bacterial infections, including otitis media, sinusitis, bronchitis, and pneumonia, requiring antibiotic therapy. One year previously, he required IV acyclovir for herpes simplex virus (HSV) esophagitis. He also had a history of squamous cell carcinoma excised from his left eyelid.
Initial neurologic examination was notable for an encephalopathy with mental status testing demonstrating disorientation and impaired attention and concentration. Language deficits included markedly impaired …
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