Natural history of the visual impairment of relapsing neuromyelitis optica.

PURPOSE: To describe the clinical features and natural history of the visual impairment of relapsing neuromyelitis optica. DESIGN: Prospective observational case series. PARTICIPANTS: Thirty patients of Afro-Caribbean origin with neuromyelitis optica and 47 patients with multiple sclerosis. METHODS: A complete ophthalmologic examination was performed with each ocular attack and during a remission period at least 6 months after the last attack. MAIN OUTCOME MEASURES: Incidence of unilateral and bilateral optic neuritis and incidence of unilateral and bilateral severe visual loss, defined as visual acuity (VA) < or = 20/200. RESULTS: Twenty-eight patients (93%) with neuromyelitis optica were female and 2 (7%) were male. The mean age of onset was 30+/-10.5 years. Mean disease duration was 9.5+/-5.4 years. Neuromyelitis optica first manifested by an episode of optic neuritis in 23 cases (76.6%), bilateral in 4. The average number of ocular attacks per patient was 2.7+/-1.6. Twenty-one patients (70%) showed impairment in both eyes. Median times from onset to unilateral optic neuritis, bilateral optic neuritis, mono-ocular severe visual loss, and binocular severe visual loss were 0+/-0.08, 1+/-1.6, 2+/-0.8, and 13+/-3 years, respectively. Fifteen patients (50%) experienced severe visual loss in both eyes and 6 (20%) in one eye. Severe visual loss occurred in as few as 2 attacks. CONCLUSION: This study is the largest series of relapsing neuromyelitis optica in a population of African descent. Neuromyelitis optica's visual impairment is very severe; it contrasts drastically with that typically observed in multiple sclerosis. We confirmed that the prognosis for optic neuritis in patients with neuromyelitis optica is worse than the prognosis in patients with multiple sclerosis.

Natural history of human T-lymphotropic virus 1-associated myelopathy: a 14-year follow-up study.

BACKGROUND: The progression of neurological disability in human T-lymphotropic virus 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) remains undefined. OBJECTIVES: To determine the time course of disability scores and to identify predictors of outcome among patients with HAM/TSP. DESIGN: Clinical 14-year follow-up study. SETTING: University hospital. Patients One hundred twenty-three patients with HAM/TSP. MAIN OUTCOME MEASURES: We determined time from onset to the following 4 Kurtzke Disability Status Scale (DSS) end points: scores of 6 (unilateral aid required), 6.5 (bilateral aid required), 8 (wheelchair confinement), and 10 (death related to the disease). Times to reach selected DSS scores were estimated using the Kaplan-Meier method. Univariate and multivariate analyses identified variables related to the rate of progression to DSS 8. The HTLV-1 proviral loads were also assessed. RESULTS: The disability of the cohort progressed throughout the follow-up period. The median times from onset to DSS 6, 6.5, and 8 were 6, 13, and 21 years, respectively. The median time from DSS 6 to DSS 8 was 8 years; DSS 10 was reached by one fourth of the patients within 20 years. Age at onset of 50 years or older and high HTLV-1 proviral load were associated with a shorter time to DSS 8 (P = .01 and P = .02, respectively). A shorter time to DSS 6 significantly adversely affected the time to progression from DSS 6 to DSS 8. CONCLUSIONS: Human T-lymphotropic virus 1-associated myelopathy/tropical spastic paraparesis is a rapidly disabling disease. Monitoring for HTLV-1 proviral load is recommended in future therapeutic trials.