Expanding the etiologic spectrum of spastic ataxia syndrome: chronic infection with human T lymphotropic virus type 1

Abstract

Human T-lymphotropic virus type-1 (HTLV-1) is a neglected infection most often associated with an indolent process. However, a subset of HTLV-1 seropositive patients face the risk to develop life-threatening T-cell lymphoma/leukemia, or the highly disabling and incurable HTLV1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). Over the years, other complications to HTLV-1 have been proposed and debated intensely. One of these, although rare, associations include cerebellar ataxia occurring most often in Japanese patients with manifest HAM/TSP. Here we present a HTLV-1 seropositive patient from the Middle East featuring a slowly progressive cerebellar syndrome with cerebellar atrophy but not evidence of spastic paraparesis. In addition, this patient suffered from autoimmune conditions such as Sjögren’s syndrome and vitiligo which are putatively associated with HTLV-1.

Keywords: Ataxia; HTLV-1; Proviral load.

Fig. 1

Neuroimaging in a HTLV-1-positive patient with ataxia. Brain magnetic resonance imaging of the reported patient at age 62 years of age showing diffuse subtle atrophy of the cerebellar hemispheres and vermis. Top row: axial T2-weighted (left and middle) and T2-weighted fluid-attenuated inversion recovery (right). Bottom row: coronal T2-weighted (left and middle) and sagittal T1-weighted (right) images