Monomelic amyotrophy: clinical profile and natural history of 279 cases seen over 35 years (1976-2010).

Our objective was to study the clinical characteristics and natural history of monomelic amyotrophy (MMA). We used a retrospective study of 279 patients diagnosed to have either upper (Hirayama disease) or lower limb MMA. Results showed that brachial MMA (BMMA) occurred in 224 patients (male:female, 9:1). Mean age of onset was 19.5 ± 4.18 years. Progression occurred over less than five years in the majority (95.9%) of patients. Duration at the last follow-up was: up to five years in 61.4%, 5-10 in 21.3%, 10-15 in 7.2%, > 15 years in 10.1%. MRI showed asymmetrical lower cervical cord atrophy in 44.6% of patients. Crural MMA (CMMA) occurred in 55 patients (male:female, 13:1). Mean age of onset was 21.38 ± 5.3 years. Similar to BMMA, most cases (65.5%) had onset between 15 and 25 years of age. Total duration of illness at the last follow-up was up to five years in 52.7%, 10 and beyond in 47.3%. In conclusion, a large cohort of patients with monomelic amyotrophy seen over 35 years (1976-2010) is described. Study data support the clinical findings and its natural history with long term follow-up, and the findings emphasize that monomelic amyotrophy is a 'benign' condition with a self-limiting course.