Pan-American Consortium of Multiple System Atrophy. Preliminary Report (P06.079)

Abstract

Objective: To describe the clinical and epidemiological characteristics of a series of patients diagnosed with MSA from 5 different centers from North America and Latin America. Background Multiple system atrophy (MSA) is a rare, sporadic and rapidly progressive neurodegenerative disorder that presents with parkinsonism, cerebellar ataxia and autonomic failure. Clinical, demographic and epidemiological data from different centers have provided valuable information concerning the natural history of MSA and have contributed to improved diagnostic criteria. Design/Methods: We retrospectively analyzed the clinical data of 78 patients with probable MSA from 5 different movement disorders centers from Argentina, Chile, Mexico and the United States. Evaluations were performed according to the clinical and imagenologic criteria recently established. No patients with probable MSA were confirmed by autopsy. Results: Forty women and 38 men were included, with a mean age of 65,5 ±10 years. Family history was negative in 64 patients and unknown in 14. Seventy five percent corresponded to MSA-P variant. Dysautonomic symptoms were observed in 90,8 % of individuals, including mictional urgency 84,6%, orthostatism 65,3%, “cold hands” 41,9%, and severe hypophonia 86%. Dysphagia, falls and cranial cervical dystonia were present in the majority of cases. Response to levodopa was poor and transitory. MR imaging showed putaminal atrophy in 16 patients and hyperintense putaminal rims in 13 and the “hot cross bun” sign in 6. The mean estimate survival was 6 years (range 1-14). Chile and Mexico were countries with greater “mestizo” populations. Conclusions: The present series presents preliminary data about MSA in centers from North America and Latin America. In spite of the great ancestry variability observed in this population, no differences were observed regarding the other Western series. Disclosure: Dr. Gatto has nothing to disclose. Dr. Chana has nothing to disclose. Dr. Etcheverry has nothing to disclose. Dr. Gallin has nothing to disclose. Dr. Miranda has nothing to disclose. Dr. Parisi has nothing to disclose. Dr. Sanguinetti has nothing to disclose. Dr. Rodriguez Violante has nothing to disclose. Dr. Litvan has nothing to disclose.