Movement Disorders Unit, Neurology Service, Hospital de Clínicas, Federal University of Parana, Rua General Carneiro, 181 - Alto da Glória, Curitiba, 80060-900, Brazil.
Neurology Service, Hospital Universitário Regional dos Campos Gerais, State University of Ponta Grossa, Ponta Grossa, Brazil.
Cerebellum. 2019 Apr;18(2):196-202. doi: 10.1007/s12311-018-0982-x.
Spinocerebellar ataxia type 2 (SCA2) is characterized by a progressive cerebellar syndrome, and additionally saccadic slowing, cognitive dysfunction, and sleep disorders. The aim of this study was to assess the frequency of abnormal findings in sleep recordings of patients with SCA2. Seventeen patients with genetically confirmed SCA2 from the Movement Disorders Outpatient group of the Hospital de Clínicas da UFPR were evaluated with a structured medical interview and the Scale for the Assessment and Rating of Ataxia (SARA). Polysomnographic recordings were performed and sleep stages were scored according to standard criteria. There were 10 male subjects and 7 females, aged 24-66 years (mean 47.44). A sex- and age-matched control group of healthy subjects was used for comparison. There was a reduction of rapid eye movement (REM) sleep in 12 (70.58%), increased REM latency in 9 (52.94%), increased obstructive sleep apnea-index in 14 (82.35%), absent REM density (REM density was calculated as the total number of 3-s miniepochs of REM sleep with at least 1 REM per minute) in 13 (76.47%), and markedly reduced REM density in 4 (23.52%). There was an indirect correlation according to the SARA scale and the REM density decrease (r = - 0.6; P = < 0.001); and with a disease progression correlating with a reduction in the REM density (r = - 0.52, P = 0.03). In SCA2, changes occur mainly REM sleep. The absence/decrease of REM sleep density, even in oligosymptomatic patients, and the correlation of this finding with disease time and with the SARA scale were the main findings of the study.
脊髓小脑性共济失调 2 型（SCA2）的特征是进行性小脑综合征，此外还伴有眼球运动迟缓、认知功能障碍和睡眠障碍。本研究旨在评估 SCA2 患者睡眠记录中异常发现的频率。来自 UFPR 临床医院运动障碍门诊组的 17 名经基因证实的 SCA2 患者接受了结构化的医学访谈和共济失调评估量表（SARA）评估。进行了多导睡眠图记录，并根据标准标准对睡眠阶段进行评分。有 10 名男性和 7 名女性，年龄 24-66 岁（平均 47.44 岁）。使用性别和年龄匹配的健康对照组进行比较。有 12 例（70.58%）快速眼动（REM）睡眠减少，9 例（52.94%）REM 潜伏期增加，14 例（82.35%）阻塞性睡眠呼吸暂停指数增加，13 例（76.47%）REM 密度缺失（REM 密度计算为每分钟至少 REM 1 次的 REM 睡眠的总 3-s miniepochs 数），4 例（23.52%）REM 密度明显降低。根据 SARA 量表，REM 密度降低与间接相关（r = -0.6；P < 0.001）；与疾病进展相关的 REM 密度降低呈负相关（r = -0.52，P = 0.03）。在 SCA2 中，变化主要发生在 REM 睡眠中。即使在症状较轻的患者中，REM 睡眠密度的缺失/减少，以及该发现与疾病时间和 SARA 量表的相关性是本研究的主要发现。
