Assessment of scales ICARS (International Cooperative Ataxia Rating Scale) and SARA (Scale for the Assessment of Ataxia Rating) and their correlation with magnetic resonance imaging in Machado-Joseph disease.v

Abstract

Machado Joseph disease or spinal-cerebelar ataxia type 3 is an autossomical dominant disease and is the most common type of ataxia in Brasil. The main symptoms are ataxia, ocular movement alterations, dystonia, spasticity and pyramidal dysfunction and fasciculation, and also dysfagia and dysarthria.These usually begin in adulthood, between 30-40 years. Despite being frequent there are few biomarkers to asses the disease progression.The objective of this study is to asses the efficacy of two ataxia scales, the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment of Rating Ataxia (SARA), and its relations with magnetic resonance imaging.Patients with molecular confirmation of Machado Joseph disease will be selected. The subjects will be submitted to a neurologic exam, application of the ICARS and SARA scales, and magnetic resonance imaging scans.Patients will be monitored for at lease 12 months, and the data obtained in the assessments will be stored for statistical analysis.