A 15 year prospective longitudinal study of disease progression in patients with HTLV-1 associated myelopathy in the UK.
Résumé
Background: The natural history of HTLV-1-associated myelopathy (HAM) has been mainly described in HTLV-1 endemic countries such as Japan, Brazil and Martinique. Objectives: We describe the natural history of the largest cohort of patients with HAM living in the UK from 1993 to 2007. Methods: Prospective, longitudinal study comparing clinical and virological outcome between first and last clinical visit. Incidence and cause of death were documented and the mortality rate calculated. Results: 48 patients were included: 79.2% were female, 79.2% of Afro-Caribbean origin, 83.3% acquired HTLV-1 through breastfeeding or unprotected heterosexual intercourse. Mean age of onset was 46 years. Median durations from onset of symptoms to diagnosis and to last follow up were 2 and 11.6 years. Median time of follow up was 3.8 years. The most common first recalled symptom was unilateral leg weakness. The median times from onset to unilateral, bilateral walking aid and frame or a wheelchair were 11, 11.2, 11.3 and 18 years. The overall average deterioration in timed walk in patients whose need for aid did not change was 2 second/10 meter/year. Three patients progressed rapidly and were unable to walk within two years. Six patients were slow/non-preogressors. The mortality rate was 2.4/ 100 person year follow up. Median HTLV-1 viral load remained unchanged at 14%. Conclusions: HAM is a slowly progressing chronic disease. Timed walk deteriorates by 2 sec/10m/y and patients remain ambulant for 10 years but become wheelchair dependent a decade later. HTLV-1 viral load remains high and unchanged over time regardless of clinical progression.
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