Avaliação da função respiratória em portadores de ataxia espinocerebelar tipo 2

Abstract

Spinocerebellar ataxia (SCA) 2 is one of the 48 types of dominant autosomal spinocerebellar ataxias classified to date. Signs and symptoms correspond to progressive cerebellar syndrome with different degrees of dysarthria, dysmetria, nystagmus, tremor of intention and gait ataxia, and extrapyramidal signs such as Parkinson's may also appear. The observed postural changes lead to deficits in balance, gait and functionality in relation to work and daily life activities. They may present respiratory restrictive pattern, since the thoracic expansion in respiratory cycles is altered. Objective: To evaluate respiratory function in patients with SCA 2 by measuring peak expiratory flow and maximal inspiratory pressure and verify its relation with the severity and duration of disease, balance and functionality evolution. Methodology: Cross-sectional, quantitative and descriptive research in 36 patients with spinocerebellar ataxia 2, of both sexes, aged between 18 and 70 years. The evaluative period occurred from September 2017 to August 2018. Patients initially underwent an anamnesis and then underwent the Lawton functionality scale upon examination of peak expiratory flow, maximal inspiratory pressure, and Berg Balance Scale. Results: Of the 36 evaluated, mean age was 42.5 years (dp ± 12.4), the mean time onset of symptoms was 7.6 years (dp ± 8.2). The mean score of the SARA scale was 9.9 points (dp ± 10.2), in the functionality by Lawton and Brody scale the score was 18.4 (dp ± 4.2) and finally in the balance by the Berg Balance scale 42.3 with dp ± 18.2 points. All correlations revealed a significant statistical relationship. Conclusion: Patients with spinocerebellar ataxia type 2 present respiratory dysfunction with an intimate relationship with the SARA scale, functionality and balance. It may present a subclinical form requiring monitoring and evaluation of respiratory function in order to prevent unfavorable respiratory events.