Ann. Pak. Inst. Med. Sci. 2013; 9(3): 167-169

Abstract

Progressive multifocal leucoencephalopathy (PML) is a rarely occurring demyelinating disease of the central nervous system caused by a neurotropic papovirus named JC virus (JCV). The most frequently affected areas are the cerebral hemispheres, especially the parieto-occipital region, followed by the cerebellum and brain stem. The disease occurs predominantly in individuals with an immunocompromised state and impaired cellular mediated immunity (CMI) due to other underlying illness. The course of the disease is still most often rapidly progressive and fatal. Suspicion of PML should lead to an extensive immunological investigation before considering of brain biopsy, which is still the only specific test.
Key words; Progressive multifocal leucoencephalopathy, JC virus, Cell mediated immunity.

Case Report

A 56 years old male, non-smoker, non-alcoholic, non-diabetic presented with complaints of forgetfulness especially of more recent memory than remote memory since last 4 months. It was associated with headache and recurrent cough which was not associated with sputum for which he was taking treatment from local practitioner but symptoms were not relieved. On examination he was conscious and oriented with normal cardiovascular, respiratory and abdominal examination. Neurological examination revealed mini mental scoring of 19/30 with deficit of recall and rest of the neurological examination was normal. Laboratory investigations revealed pancytopenia, but liver, renal and lipid profiles were within normal limits. HIV serology was negative. MRI brain revealed demylination in parietal and occipital areas (Figure-1) suggestive of progressive multifocal leucoencephalopathy (PML). He was treated with steroids as prednisolone at 1 mg/kg/day with gradual improvement in the symptoms as well as correction of pancytopenia.

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