Early Juvenile Human T-cell Lymphotropic Virus Type-1-Associated Myelopathy/Tropical Spastic Paraparesis: Study of 25 Patients.

Background: Human T-cell lymphotropic virus type-1 (HTLV-1) may cause severe diseases such as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and infective dermatitis associated with HTLV-1 (IDH). The clinical characteristics and progression of 25 early onset HAM/TSP associated or not to IDH were described. Methods: Following-up 37 IDH patients with neurological examinations, 54% developed HAM/TSP. To these cases were added 5 cases of juvenile HAM/TSP. The patients were HTLV-1+ and were submitted to dermatological and neurological examinations. Diagnosis of HAM/TSP was performed according to Osame et al (1990) and Castro-Costa et al (2006) criteria. Results: Twenty-one patients were classified as definite HAM/TSP by both criteria, 3 as probable HAM/TSP by Osame et al, and another as probable HAM/TSP according to Castro-Costa et al Median age at onset of neurological manifestations was 9 years for the IDH/HAM/TSP group and 16 years for the HAM/TSP group (P = .045). In 12 patients, the onset of neurological manifestations occurred when they were less than 10 years of age. In the group IDH/HAM/TSP, the neurological symptoms always begun during the period of activity of IDH. The progression of HAM/TSP evaluated in 17 cases was heterogeneous, and 3 had rapid progressive course. Conclusions: The juvenile HAM/TSP may occur very early and also presents marked female predominance. Progression of IDH to HAM/TSP before 19 years of age is frequent (54%). Rapid progressive form may also occur in early HAM/TSP. As juvenile IDH and HAM/TSP are due to vertical transmission through breastfeeding, it is very important to avoid this pathway of infection.

HTLV-I associated myelopathy/tropical spastic paraparesis in a 7-year-old boy associated with infective dermatitis.

HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic myelopathy characterized by a slowly progressive spastic paraparesis and sphincter disturbances beginning in adulthood. Only eight well-documented cases occurring in childhood and adolescence have been described. Infective dermatitis associated to the HTLV-I (IDH) is a chronic eczema of childhood occurring in vertically infected carriers. Here we describe a 7-year-old boy with HAM/TSP and IDH. The neurological manifestations were spastic gait, hyperreflexia of lower limbs, clonus and bilateral Babinski's sign. High levels of HTLV-I antibodies in the serum and in the cerebrospinal fluid were observed. The association of these two diseases and the early onset of HAM/TSP are probably related to a strong humoral anti-HTLV-I response.

Clustering of HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP) and infective dermatitis associated with HTLV-1 (IDH) in Salvador, Bahia, Brazil.

Fifteen families with clustering of infective dermatitis associated with HTLV-1 (IDH) and/or HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP) were observed among 28 families of IDH index cases, 93% of them occurring in two generations. With the exception of two mothers of children with IDH, all the mothers with HAM/TSP had at least one child with HAM/TSP. This is the first report of such clustering involving many families.