Objective: To describe, through a case report, the range of abnormal sleep patterns of Spinocerebellar Ataxia type 3 (SCA3).

Background: SCA3 is a progressive neurodegenerative condition caused by a CAG repeat. Although it is clinically diagnosed by a characteristic spinocerebellar ataxia, there are commonly recognized extra-cerebellar features which can be suitable to symptomatic treatment. One of these features is sleep pattern disruption with Rapid Eye Movement (REM)-Sleep Behaviour Disorder (RSBD), Restless Legs Syndrome (RLS), Periodic Limb Movement during Sleep (PLMS), Obstructive Sleep Apnoea Syndrome (OSAS) and Excessive Daytime Sleepiness (EDS). Even that, sleep disorders are not thought to be related with clinical severity, triplet repetition length or any other clinical variability.

Method: Case Report.

Results: We present a 63-year-old man with an adult-onset SCA3 now evolving for 20 years. He also had a treated arterial hypertension and he is brother of two SCA3 women patients. While he has been stable in terms of motor impairment, with a Scale for the assessment and rating of ataxia (SARA) score of 11, he recently started complaining about vivid dreams, some abrupt awakenings during the night and abnormal movements during sleep. He also complained about EDS. Polysomnography revealed sleep structure disruption with fragmented sleep. REM phases were compatible with RSBD and short. He had around 60 PLMS per hour. Finally, respiratory evaluation was compatible with a severe SAOS with a respiratory disturbance index of 35 per hour. The patient will be treated with Continuous Positive Airway Pressure (CPAP) and Clonazepam.

Conclusion: We reported an extensive range of SCA3 sleep disorder patterns in only one patient.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/polysomnographic-features-of-spinocerebellar-ataxia-type-3-a-case-report/