Proposal for diagnostic criteria of tropical spastic paraparesis/HTLV-I-associated myelopathy (TSP/HAM).

After the first description of TSP/HAM in 1985 and the elaboration of WHO's diagnostic criteria in 1988, the experience of the professionals in this field has increased so that a critical reappraisal of these diagnostic guidelines was considered timely. Brazilian neurologists and observers from other countries met recently to discuss and propose a modified model for diagnosing TSP/HAM with levels of ascertainment as definite, probable, and possible, according to myelopathic symptoms, serological findings, and/or detection of HTLV-I DNA and exclusion of other disorders.

The McGill Pain Questionnaire constructed for the Dutch language (MPQ-DV). Preliminary data concerning reliability and validity.

The Dutch version of the McGill Pain Questionnaire was composed mainly by following R. Melzack's methodological design. The recommendations of the Finnish research team were considered and integrated. In the first phase as wide an inventory of pain descriptions as possible was drawn up. In the second phase, these pain descriptions were categorized by pain experts and students according to quality aspects. In the third phase the pain descriptions were judged according to intensity aspect as well by pain experts, by students and by pain patients. Finally a fourth phase was set up to obtain insight into the reliability and validity of the McGill Pain Questionnaire - Dutch Version (MPQ-DV).

New candidate loci for multiple sclerosis susceptibility revealed by a whole genome association screen in a Belgian population.

We have completed a whole genome screen for association with multiple sclerosis (MS) in a Belgian population. The 6000 microsatellite markers provided through the Genetic Association of Multiple Sclerosis in EuropeanS (GAMES) collaborative were genotyped in case-control and family-based samples. The 20 most promising markers included three markers (D6S1615, D6S2444 and TNFa) from the classically established HLA class II cluster and one (D6S265) from the recently re-emphasized HLA class I cluster. In other highlighted regions, preliminary candidate genes from the immune system have been identified: e.g. the integrin ligand EDIL3, the high-mobility group box protein TOX, neutral sphingomyelinase activating factor (NSMAF) and the B-cell specific transcription factor POU2AF1.

Neuropathology of human and experimental TSP/HAM: a critical review.

Tropical Spastic Paraparesis/HTLV-1 Associated Myelopathy (TSP/HAM) is clinically characterized by chronic insidious spastic paraparesis associated with variable sensory impairment and sphincter symptoms. Neuropathological studies of this condition are based on a few autopsied cases, and on experimental animal models. However, divergent aspects exist between human and experimental animal neuropathology of TSP/HAM, namely, the site of lesions in the spinal cord, the involvement of peripheral nerves and roots, the nature of histological abnormalities, and the cellular reactions. Moreover, unanswered questions as to the preferential site of spinal affection, the temporal inflammatory picture, the selective damage of the corticospinal tract, the sparing of lower motor neurons, the inconsistent affection of sensory tracts, and the involvement of the brain, are outlined. A long-term, chronological, correlated clinical and neuropathological study in HAM experimental animals is suggested.

Neuropathology of two Brazilian autopsied cases of tropical spastic paraparesis / HTLV-I associated myelopathy (TSP/HAM) of long evolution.

We report on a neuropathological analysis of two cases of TSP/HAM originating from Brazil. These two cases had, respectively, an evolution of 13 and 40 years. The main neuropathological findings consisted of spinal cord atrophy, mainly the lower thoracic cord, diffuse degeneration of the white and grey matter, rare foci of mononuclear and perivascular cuffs, and hyaline hardening of arteriolae. The supraspinal structures were normal, excepting for a slight gliosis in the cerebellum. An analysis on the long evolutive cases as described in the literature is outlined in this study.

Improvement of health-related quality of life in relapsing remitting multiple sclerosis patients after 2 years of treatment with intramuscular interferon-beta-1a.

In patients with relapsing remitting multiple sclerosis (RRMS), the effect of interferon-beta (INFb) on health-related quality of life (HR-QoL) is not firmly documented. The objective of this study is to assess HR-QoL during 2 years of treatment with intramuscular INFb and its correlation with disability. In 36 neurological practices in the Netherlands (17), Belgium (16), United Kingdom (2) and Luxemburg (1), 284 RRMS patients were treated with intramuscular INFb-1a. Physical and mental domains of HR-QoL were measured by the MS54 Quality of Life (MS54QoL) questionnaire, and disability was assessed by the Multiple Sclerosis Functional Composite (MSFC) (Timed 25-Foot Walk Test [Timed 25-FWT], 9 Hole Peg Test [9-HPT], Paced Auditory Serial Addition Test [PASAT]) at baseline and at months 3, 6, 12, 18 and 24. Expanded Disability Status Scale (EDSS) score was assessed at baseline and month 24. Pearson's correlation coefficients were determined and predefined factors were analyzed for relation to HR-QoL after baseline by stepwise regression analyses on physical and mental scores. 204 patients (71.8%) completed 2 years of treatment. Mean values for MS54QoL increased from 56.6 to 61.0 for physical (p < 0.05) and from 57.2 to 61.1 for mental domain (p = 0.07). Correlations between physical domain and MSFC was -0.40 (p < 0.05), and between mental domain and MSFC -0.24 (p < 0.05). MSFC and EDSS did not change. Increase of physical MS54QoL was associated with lower age, lower EDSS, less time for Timed 25-FWT, and higher PASAT score at baseline. Increase of mental MS54QoL was associated with higher PASAT and lower EDSS. Patients who discontinued INFb had lower physical or mental HR-QoL at baseline. In RRMS patients, 2 years of treatment with intramuscular INFb-1a is associated with an increase in HR-QoL, especially in younger patients with low disability.

HTLV-I negative tropical spastic paraparesis: a scientific challenge

We reviewed the historical, clinical and etiological aspects of the progressive chronic spastic myelopathies of unknown etiology, disserting on the clinical similarities between HTLV-I seropositive and seronegative tropical spastic paraparesis (TSP), as well as focusing on the PCR studies of the seronegative TSP

Paraparesia espástica tropical nos trópicos e Brasil: análise histórica / Tropical spastic paraparesis in Tropics and Brazil: historical analysis

A paraparesia espástica tropical (PET)é mielopatia crônica, observada predominantemente nos trópicos recentemente descoberta ser de origem retroviral (HTLV-I). O objetivo deste estudo foi delinear a evoluçäo histórica de sua descriçäo, denominaçöes e referências etiológicas. A análise histórica revelou que essa condiçäo teve diferentes denominaçöes e a descoberta de sua etiologia retroviral em parte dos casos abriu diversas linhas de investigaçöes e interesse epidemiológico, nos trópicos e no Brasil

Neuropathology of two Brazilian autopsied cases of tropical spastic paraparesis / HTLV-I associated myelopathy (TSP/HAM) of long evolution

We report on a neuropathological analysis of two cases of TSP/HAM originating from Brazil. These two cases had, respectively, an evolution of 13 and 40 years. The main neuropathological findings consisted of spinal cord atrophy, mainly the lower thoracic cord, diffuse degeneration of the white and grey matter, rare foci of mononuclear and perivascular cuffs, and hyaline hardening of arteriolae. The supraspinal structures were normal, excepting for a slight gliosis in the cerebellum. An analysis on the long evolutive cases as described in the literature is outlined in this study