Progression of Idiopathic Late Onset Cerebellar Ataxia (S12.006)

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Objective: To analyze the follow-up of idiopathic late onset cerebellar ataxia (ILOCA) patients. Background ILOCA patients represent a group of neurodegenerative diseases of unknown cause affecting the cerebellum and its afferent and efferent connections. Design/Methods: Clinical and laboratory observations of the course of 50 adult patients initially evaluated in an cerebellar ataxia outpatient clinic over 20 years, with a diagnosis of ILOCA, and followed up regularly. The presence of REM sleep behavior disorder (RBD) was evaluated as probable RBD, based on clinical validated criteria, or definite RBD, based on polysomnographic confirmation. Results: From a total of 50 patients with ILOCA (excluding all patients with acquired or genetically defined cerebellar ataxia), 24 subsequently developed criteria for MSA type-C. 26 patients remained with the diagnosis of ILOCA, among them 8 with a probable diagnosis of late cerebellar atrophy of Marie-Foix-Alajouanine syndrome (MFAS). The group of patients with late cerebellar atrophy of MFAS had an older mean age (72.8 years) than MSA-C patients (59.5 years). Neurological examination, including a thorough eye movement assessment did not differentiate patients with MSA-C and ILOCA. Clinically or definite RBD was diagnosed in 46 % of patients. However, patients who developed MSA type C had a highly percentage of probable and definite previously diagnosis of RBD (83.4%). On the other hand, among patients with ILOCA, the percentage of RBD was significantly lower (11.5%, p Conclusions: In this Brazilian series of ILOCA patients, MSA-C was clinically diagnosed in 48 % of cases and 52 % remained with the diagnosis of ILOCA, including a 12 % with the diagnosis of late cerebellar atrophy of MFAS. RBD diagnosis, either clinical or polysomnographic should included as an important tool in the differentiation of MSA type C and ILOCA. Disclosure: Dr. Teive has nothing to disclose. Dr. Munhoz has nothing to disclose. Dr. Arruda has nothing to disclose. Dr. Werneck has nothing to disclose.