Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia

被引：31

作者：

Naeije, Gilles ^{[1
,2
]}

Rai, Myriam ^{[3
]}

Allaerts, Nick ^{[2
]}

Sjogard, Martin ^{[1
]}

De Tiege, Xavier ^{[1
]}

Pandolfo, Massimo ^{[2
,3
]}

机构：

[1] Univ Libre Bruxelles, ULB Neurosci Inst, Lab Cartog Fonct Cerveau, 808 Lennik St, B-1070 Brussels, Belgium

[2] Univ Libre Bruxelles, Dept Neurol, CUB Hop Erasme, Brussels, Belgium

[3] Univ Libre Bruxelles, ULB Neurosci Inst, Lab Neurol Expt, Brussels, Belgium

来源：

ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY | 2020年 / 7卷 / 06期

关键词：

Friedreich ataxia; cerebellar cognitive affective syndrome; Dentate nuclei; PROGRESSION;

D O I：

10.1002/acn3.51079

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Dentate nuclei (DN) are involved in cerebellar modulation of motor and cognitive functions, whose impairment causes ataxia and cerebellar cognitive affective syndrome (CCAS). Friedreich ataxia (FRDA) disease progression relates to degeneration of the dentate nucleus and dentato-thalamic pathways, causing cerebellar ataxia. Volumetric MRI also shows mild loss in the cerebellar cortex, brainstem, and motor cortex. Cognitive deficits occur in FRDA, but their relationship with ataxia progression is not fully characterized. We found a significant positive correlation between severity of patients' ataxia and more marked CCAS as assessed with the CCAS-Scale. This relation could be related to progressive DN impairment.

引用

页码：1050 / 1054

页数：5

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