title: Temporal lesions and widespread involvement of white matter associated with multi-organ inflammatory disease in human T-lymphotropic virus type 1-associated myelopathy/tropical spastic paraparesis (HAM/TSP).
abstract: Human T-lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic inflammatory disease of the spinal cord, characterized by spastic paraparesis, back pain, and sphincter disorders. Involvement of multiple organs and encephalopathy are uncommon in HAM/TSP. Nonspecific small white matter lesions of unknown etiology, mainly in the periventricular and subcortical regions, have been found on brain magnetic resonance imaging of HAM/TSP patients. Bitemporal lesions have rarely been described. We report the case of a 54-year-old woman diagnosed with HAM/TSP who presented subclinical cognitive deficits associated with bitemporal and widespread white matter lesions. The cerebrospinal fluid (CSF) was inflammatory (blood-CSF barrier dysfunction, intrathecal synthesis of total and HTLV-1 IgG). The proviral load was higher in cerebrospinal fluid than in peripheral blood mononuclear cells. The neurological picture was complicated by multi-organ inflammatory disease (Hashimoto's thyroiditis, uveitis, anemia, and chronic renal failure). This case highlights the potential multisystem inflammatory nature of HTLV-1 infection, with a wide spectrum of manifestations. In cases of HAM/TSP with multi-organ inflammatory disease, encephalic involvement should be investigated, even in the absence of clinical manifestations. Also bitemporal lesions can be the consequence of intense and diffuse inflammation associated with HTLV-1 infection.
authors.affiliation: Neuroinfection Clinic, Hospital Universitário Gaffré e Guinle/Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Gustavo B
authors.initials: GB
authors.lastname: Mendes
authors.affiliation: Neuroinfection Clinic, Hospital Universitário Gaffré e Guinle/Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Rosangela S
authors.initials: RS
authors.lastname: Kalil
authors.affiliation: CSF Laboratory, Clinical Pathology Service, Hospital Universitário Clementino Fraga Filho/Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Carolina
authors.initials: C
authors.lastname: Rosadas
authors.affiliation: Neurology Service, Hospital Universitário Antônio Pedro/Universidade Federal Fluminense, Rio de Janeiro, Brazil.
authors.forename: Marcos R G
authors.initials: MR
authors.lastname: de Freitas
authors.affiliation: Neuroinfection Clinic, Hospital Universitário Gaffré e Guinle/Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, Brazil; CSF Laboratory, Clinical Pathology Service, Hospital Universitário Clementino Fraga Filho/Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil. Electronic address: mpuccioni@hucff.ufrj.br.
authors.forename: Marzia
authors.initials: M
authors.lastname: Puccioni-Sohler
mesh_terms: D005260:Female; D015490:HTLV-I Infections / Q000150:complications*; D015368:Human T-lymphotropic virus 1; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D015493:Paraparesis, Tropical Spastic / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology*; D013702:Temporal Lobe / Q000473:pathology*; D066127:White Matter / Q000473:pathology*
keywords: Encephalopathy; HAM/TSP; HTLV-1