Burk K, Globas C, Wahl T, Bühring U, Dietz K, Zuhlke C, Luft A, Schulz J B, Voigt K, Dichgans J
Department of Neurology, University of Tübingen, Hoppe-Seyler-Str. 3, D-72076 Tübingen, Germany.
Brain. 2004 Jan;127(Pt 1):175-81. doi: 10.1093/brain/awh013. Epub 2003 Oct 21.
The term idiopathic cerebellar ataxia (IDCA) designates a variety of cerebellar syndromes that may present with a purely cerebellar syndrome (IDCA-C) or with additional extracerebellar features (IDCA-P). Multiple system atrophy is also a sporadic neurodegenerative disorder of unknown origin that may cause prominent cerebellar symptoms (MSA-C). The final neuropathological answer to the question whether IDCA-P and MSA-C represent different varieties of one disease or two distinct entities is still lacking. Three-dimensional MRI-based volumetry allows morphological investigations intra vitam. Volumetric analysis of cerebellum, brainstem and basal ganglia was therefore performed in 46 patients with sporadic cerebellar ataxia and 16 age-matched healthy controls. Patients with dementia were excluded from the study since cognitive impairment is an exclusion criterion for the diagnosis of MSA. Cerebellar patients were clinically divided into two groups: 33 patients with multiple system atrophy with prominent cerebellar symptoms (MSA-C) and 13 patients with extracerebellar features not corresponding to MSA-C (IDCA-P). There was evidence for substantial cerebellar atrophy in both cerebellar groups while additional brainstem atrophy was significantly more pronounced in MSA-C patients. Absolute caudate and putamen atrophy was found to be restricted to single MSA-C individuals while group comparisons of mean volumes did not yield significant differences from controls. Based on the volumetric data, diagnosis could be correctly predicted in 94% of control, 82% of MSA-C and 100% of IDCA-P individuals. The finding of specific imaging characteristics strengthens (i) the value of MRI volumetry in separating MSA-C from other types of sporadic cerebellar ataxia, and (ii) the hypothesis of two independent neurodegenerative disorders in MSA-C and IDCA-P.
术语特发性小脑共济失调（IDCA）指的是多种小脑综合征，这些综合征可能表现为单纯的小脑综合征（IDCA-C）或伴有额外的小脑外特征（IDCA-P）。多系统萎缩也是一种病因不明的散发性神经退行性疾病，可能导致明显的小脑症状（MSA-C）。IDCA-P和MSA-C是代表一种疾病的不同变体还是两种不同的实体，这一问题的最终神经病理学答案仍未明确。基于三维MRI的容积测量法可在活体状态下进行形态学研究。因此，对46例散发性小脑共济失调患者和16名年龄匹配的健康对照者进行了小脑、脑干和基底节的容积分析。患有痴呆症的患者被排除在研究之外，因为认知障碍是MSA诊断的排除标准。小脑疾病患者在临床上分为两组：33例有明显小脑症状的多系统萎缩患者（MSA-C）和13例小脑外特征不符合MSA-C的患者（IDCA-P）。两组小脑疾病患者均有明显的小脑萎缩证据，而MSA-C患者的脑干萎缩更为明显。发现尾状核和壳核的绝对萎缩仅限于个别MSA-C患者，而平均体积的组间比较与对照组无显著差异。根据容积数据，在94%的对照者、82%的MSA-C患者和100%的IDCA-P患者中可正确预测诊断。特定成像特征的发现强化了以下两点：（i）MRI容积测量法在区分MSA-C与其他类型散发性小脑共济失调方面的价值；（ii）MSA-C和IDCA-P是两种独立神经退行性疾病的假说。
