Godeiro-Junior Clecio, Felício Andre C, Barsottini Orlando G P, Aguiar Patricia M de Carvalho, Silva Sonia M A, Borges Vanderci, Ferraz Henrique B
Movement Disorders Unit, Department of Neurology and Neurosurgery, Federal University of São Paulo, São Paulo SP, Brazil.
Arq Neuropsiquiatr. 2008 Dec;66(4):800-4. doi: 10.1590/s0004-282x2008000600004.
The association between Dystonia and Parkinson's disease (PD) has been well described especially for foot and hand dystonia. There is however few data on dystonic postures in patients with atypical parkinsonism.
To evaluate the frequency and pattern of dystonia in a group of patients with atypical parkinsonism (multiple system atrophy - MSA, progressive supranuclear palsy - PSP, and corticobasal degeneration - CBD) and to investigate whether dystonia could be the first presenting symptom at disease onset in those patients.
A total of 38 medical charts were reviewed (n=23/MSA group; n=7/CBD group; n=8/PSP group) and data values were described as means/standard deviations. The variables evaluated were sex, age at onset, disease duration, first symptom, clinical features of dystonia and other neurological signs, response to levodopatherapy, Hoehn &Yahr -scale >3 after three years of disease, and magnetic resonance imaging findings.
The overall frequency of dystonia in our sample was 50% with 30.4% (n=7) in the MSA group, 62.5% (n=5) in the PSP group, and 100% (n=8) in the CBD group. In none of these patients, dystonia was the first complaint. Several types of dystonia were found: camptocormia, retrocollis, anterocollis, blepharoespasm, oromandibular, and foot/hand dystonia.
In our series, dystonia was a common feature in atypical parkinsonism (overall frequency of 50%) and it was part of the natural history although not the first symptom at disease onset. Neuroimaging abnormalities are not necessarily related to focal dystonia, and levodopa therapy did not influence the pattern of dystonia in our group of patients.
肌张力障碍与帕金森病(PD)之间的关联已有详尽描述,尤其是足部和手部肌张力障碍。然而,关于非典型帕金森综合征患者肌张力障碍姿势的数据却很少。
评估一组非典型帕金森综合征(多系统萎缩 - MSA、进行性核上性麻痹 - PSP和皮质基底节变性 - CBD)患者中肌张力障碍的频率和模式,并调查肌张力障碍是否可能是这些患者疾病发作时的首发症状。
共查阅了38份病历(MSA组n = 23;CBD组n = 7;PSP组n = 8),数据值以均值/标准差表示。评估的变量包括性别、发病年龄、病程、首发症状、肌张力障碍的临床特征和其他神经体征、对左旋多巴治疗的反应、疾病三年后Hoehn & Yahr分级>3以及磁共振成像结果。
我们样本中肌张力障碍的总体发生率为50%,其中MSA组为30.4%(n = 7),PSP组为62.5%(n = 5),CBD组为100%(n = 8)。在这些患者中,肌张力障碍均不是首发症状。发现了几种类型的肌张力障碍:脊柱前弯症、颈后伸、颈前屈、眼睑痉挛、口下颌肌张力障碍以及足部/手部肌张力障碍。
在我们的系列研究中,肌张力障碍是非典型帕金森综合征的常见特征(总体发生率为50%),它是疾病自然史的一部分,尽管不是疾病发作时的首发症状。神经影像学异常不一定与局灶性肌张力障碍相关,左旋多巴治疗也未影响我们这组患者的肌张力障碍模式。