NA

Progressive Multifocal Leukoencephalopathy (PML) is a rare neurological disease that damages the myelin sheath of the central nervous system, causing a wide variety of clinical manifestations. PML occurs due to reactivation of the John Cunningham (JC) virus in an immunocompromised patient.   

NA

A 58-year-old man with chronic alcohol abuse was found inebriated and wandering the streets. He was encephalopathic with restricted lateral gaze bilaterally, dysarthria and bilateral upper extremity dysmetria on admission. Contrasted Magnetic Resonance Imaging (MRI) of the brain showed extensive and diffuse white matter changes with no contrast enhancement. Cerebrospinal fluid (CSF) analysis showed lymphocytic predominant white cell elevation, elevated protein (113 mg/dl) and JC virus DNA (8500 copies). He also had lymphopenia (7% relative lymphocytes) and secondary hemochromatosis that were thought to be secondary to excessive alcoholism. Despite extensive workup, there was no evidence of immunocompromised state including malignancy, HIV infection, autoimmune disorders. There was no history of organ transplantation or use of immunomodulator therapy. Lymphopenia was thought to have led to secondary immunodeficiency and resultant JC virus reactivation and infection, leading to PML. Lymphocyte counts continue to improve with cessation of alcohol. Most recent MRI brain (7 months after initial presentation) showed moderate resolution of white matter changes.  

PML in patients with no clear evidence of a compromised immune system is rare.  Occult immune deficiency states like chronic excessive alcoholism and lymphopenia can cause JC virus reactivation.