PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY IN A PATIENT WITH HYPOGAMMAGLOBULINEMIA

We describe a child with congenital hypogammaglobulinemia that was dia gnosed at 13 months of age. When he was 4 years old, gait disturbances began. The main neurological manifestations were progressive spastic tetraparesis and intellectual and speech deterioration. No infectious agent was identified. A magnetic resonance imaging scan of the central nervous system revealed periventricular demyelinating areas in the fr ontal, temporal, and parietal lobes with cortical atrophy. Stereotacti c brain biopsy confirmed the diagnosis of progressive multifocal leuko encephalopathy caused by JC virus. He was treated with intravenous and intraventricular cytarabine and interferon-alpha, and there was clini cal improvement. We emphasize the need for brain biopsy as soon as a n eurological complication is suspected in patients with congenital hypo gammaglobulinemia for whom cerebrospinal cultures or polymerase chain reaction analyses are negative.