Cerebellar syndrome and peripheral neuropathy as manifestations of infection by HTLV-I human T-cell lymphotropic virus

Introduction. Type I human T-cell lymphotropic virus (HTLV-I) is a retrovirus with affinity for CD4 cells. This infection may give rise to a broad spectrum of disorders including T-cell leucemia/lymphoma, the myelopathy/tropical spastic paraparesis complex (M/TSP), and to a lesser extent, uveitis, arthritis, polymyositis and peripheral neuropathy. M/TSP is a progressive, chronic myelopathy characterized by spasticity hypereflexia, muscle weakness and sphincter disorders. Much less frequently it may precede, or give rise to, a cerebellar syndrome with ataxia and intention tremor. Clinical case. We describe the case of a 13 year old adolescent girl who presented with a neurological syndrome which had started with tremor of the head and limbs, ataxia, dysmetria, frequent falls and sphincter disorders. During the two and a half years that she had had this illness she had developed spastic paraparesis of the legs and had repeated urinary infections. Serology of blood and CSF was positive for HTLV-1 using the ELISA technique and confirmed by Western-blot. EMG showed predominantly axonal sensomotor neuropathy. A neurogenic bladder was detected on functional urodynamic studies. On MR there was moderate atrophy of the thoracic spinal cord and slight alterations of the subcortical white matter. Conclusions. The presence of a cerebellar syndrome or neuropathy of uncertain origin, in endemic areas, should lead to the inclusion of HTLV-1 infection in the differential diagnosis, even in the absence of pyramidal symptoms or defined M/TSP. Maternal seropositivity supports the hypothesis of mother-daughter transmission during lactation.