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.
Department of Neurology, National Institute of Mental Health and Neurosciences , Bangalore , India.
Atchayaram
A
Nalini
Mandavilli
M
Gourie-Devi
Kandavel
K
Thennarasu
Aravinda Hanumanthapura
AH
Ramalingaiah
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D004185:Disability Evaluation; D004576:Electromyography; D005121:Extremities / Q000503:physiopathology; D005260:Female; D007839:Functional Laterality; D006801:Humans; D007194:India; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D013116:Spinal Cord / Q000473:pathology; D014897:Spinal Muscular Atrophies of Childhood / Q000175:diagnosis* / Q000503:physiopathology*; D055815:Young Adult
Hirayama disease; India; Monomelic amyotrophy; brachial; crural; natural history