Teive Hélio A G, Moscovich Mariana, Moro Adriana, Farah Marina, Arruda Walter O, Munhoz Renato P
Departamento de Medicina Interna, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brazil.
Movement Disorders Centre, Toronto Western Hospital, University of Toronto, Toronto, ON, Canada.
Arq Neuropsiquiatr. 2015 Nov;73(11):903-5. doi: 10.1590/0004-282X20150139.
The authors present a Brazilian case series of eight patients with idiopathic very-late onset (mean 75.5 years old) cerebellar ataxia, featuring predominantly gait ataxia, associated with cerebellar atrophy.
26 adult patients with a diagnosis of idiopathic late onset cerebellar ataxia were analyzed in a Brazilian ataxia outpatient clinic and followed regularly over 20 years. Among them, 8 elderly patients were diagnosed as probable very late onset cerebellar ataxia. These patients were evaluated with neurological, ophthalmologic and Mini-Mental Status examinations, brain MRI, and EMG.
62.5% of patients were males, mean age was 81.9 years-old, and mean age of onset was 75.5 years. Gait cerebellar ataxia was observed in all patients, as well as, cerebellar atrophy on brain MRI. Mild cognitive impairment and visual loss, due to macular degeneration, were observed in 50% of cases. Chorea was concomitantly found in 3 patients.
We believe that this condition is similar the one described by Marie-Foix-Alajouanine presenting with mild dysarthria, associated with gait ataxia, and some patients had cognitive dysfunction and chorea.
作者展示了一组巴西的病例系列，包括8例特发性极晚发性（平均75.5岁）小脑共济失调患者，主要表现为步态共济失调，并伴有小脑萎缩。
在巴西一家共济失调门诊对26例诊断为特发性晚发性小脑共济失调的成年患者进行分析，并在20多年里定期随访。其中，8例老年患者被诊断为可能的极晚发性小脑共济失调。这些患者接受了神经学、眼科和简易精神状态检查、脑部MRI和肌电图检查。
62.5%的患者为男性，平均年龄81.9岁，平均发病年龄75.5岁。所有患者均观察到步态小脑共济失调，以及脑部MRI显示的小脑萎缩。50%的病例观察到轻度认知障碍和因黄斑变性导致的视力丧失。3例患者同时出现舞蹈症。
我们认为这种情况与Marie-Foix-Alajouanine所描述的情况相似，表现为轻度构音障碍，伴有步态共济失调，部分患者有认知功能障碍和舞蹈症。
