Schmahmann鈥檚 syndrome - identification of the third cornerstone of clinical ataxiology

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• 作者：Mario Manto ; Peter Mari毛n
• 关键词：Cerebellar cognitive affective syndrome ; Posterior fossa syndrome ; Cognition ; Executive functions ; Spatial cognition ; Language ; Affect ; Emotion
• 刊名：Cerebellum & Ataxias
• 出版年：2015
• 出版时间：December 2015
• 年：2015
• 卷：2
• 期：1
• 全文大小：562KB
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• 作者单位：Mario Manto (1)
  Peter Mari毛n (2) (3)
  
  1. Unit茅 d鈥橢tude du Mouvement, FNRS-ULB Erasme, 808 Route de Lennik, 1070, Bruxelles, Belgium
  2. Clinical and Experimental Neurolinguistics, Vrije Universiteit Brussel, Pleinlaan 2, 1050, Brussels, Belgium
  3. Department of Neurology and Memory Clinic, ZNA General Hospital Middelheim, Lindendreef 1, 2020, Antwerp, Belgium
  
• 刊物类别：Neurobiology; Neurosciences; Neurology; Neurosurgery;
• 刊物主题：Neurobiology; Neurosciences; Neurology; Neurosurgery;
• 出版者：BioMed Central
• ISSN：2053-8871

文摘

Schmahmann鈥檚 syndrome represents a novel clinical condition consisting of a constellation of cognitive and affective deficits following cerebellar disease. The complex was first described in 1998 as cerebellar cognitive affective syndrome (CCAS) on the basis of a careful neurological examination, detailed bedside mental state tests, neuropsychological investigations and anatomical neuroimaging of a group of 20 patients with focal cerebellar disorders. The syndrome was characterized by four clusters of symptoms including: (a) impairment of executive functions such as planning, set-shifting, verbal fluency, abstract reasoning and working memory, (b) impaired visuo-spatial cognition, (c) personality changes with blunting of affect or abnormal behaviour, and (d) language deficits including agrammatism, wordfinding disturbances, disruption of language dynamics and dysprosodia. This complex of neurocognitive and behavioural-affective symptoms was ascribed to a functional disruption of the reciprocal pathways that connect the cerebellum with the limbic circuitry and the prefrontal, temporal and parietal association cortices. With the introduction of Schmahmann鈥檚 syndrome, clinical ataxiology has found its third cornerstone, the two others being the cerebellar motor syndrome (CMS) mainly delineated by the pioneer French and English neurologists of the 19th and early 20th century, and the vestibulo-cerebellar syndrome (VCS) consisting of ocular instability, deficits of oculomotor movements and ocular misalignment. Keywords Cerebellar cognitive affective syndrome Posterior fossa syndrome Cognition Executive functions Spatial cognition Language Affect Emotion