Motorische und kognitive Störungen bei zerebellären Erkrankungen

Zusammenfassung

Die zerebelläre Ataxie kann sich als Stand-, Gang- und Rumpfataxie, als Extremitätenataxie, Dysarthrie und in einer gestörten Okulomotorik manifestieren. Läsionen des L. flocculonodularis und des kaudalen Vermis gehen mit einer Rumpfataxie und komplex gestörten Okulomotorik einher, des rostralen Vermis mit einer Stand- und Gangataxie und der Kleinhirnhemisphären mit einer ipsilateralen Extremitätenataxie. Dysarthrie tritt bei Läsionen oberer paravermaler Kleinhirnanteile auf. Die zerebelläre Ataxie ist Ausdruck einer gestörten Koordination von Bewegungen, die auf einer fehlerhaften Zeitabstimmung (Timing) und Kontrolle der Kräfte oder Drehmomente an und zwischen den Gelenken beruht. Vermutlich wird das klinische Bild zusätzlich durch gestörte motorische Lernvorgänge bestimmt, insbesondere einer gestörten Adaptation und Automatisierung von Bewegungen. Neuere anatomische Befunde, Untersuchungen mit bildgebenden Verfahren und Läsionsstudien haben wesentlich zu der Hypothese beigetragen, dass das Kleinhirn auch eine Rolle bei kognitiven Funktionen spielen könnte. Die rechte posterolaterale Hemisphäre soll für Sprachaufgaben, die linke für räumlich-visuelle Aufgaben und der Vermis für Affekt und Verhalten von Bedeutung sein. Es bleibt abzuwarten, inwieweit kognitive Dysfunktionen ins klinische Spektrum von Kleinhirnerkrankungen aufzunehmen sind. Deutliche kognitive Defizite gehören jedenfalls nicht zum typischen klinischen Bild.

Abstract

Clinical signs of cerebellar ataxia include ataxia of stance and gait, truncal and limb ataxia as well as dysarthria and oculomotor signs. Lesions of the flocculonodular lobe and caudal vermis are followed by truncal ataxia and oculomotor signs, lesions of the rostral vermis by ataxia of stance and gait, and lesions of cerebellar hemisphere by ipsilateral limb ataxia. Lesions of the superior paravermal cerebellum are accompanied by dysarthria. Clinical signs of ataxia are a consequence of cerebellar incoordination of movement which is caused by disordered timing and control of forces or torques at and across joints. Part of the clinical picture of cerebellar ataxia is likely explained also by disorders in motor learning, in particular in adaptation and lack of automatization of movement. More recent anatomical data and findings in human lesion and brain imaging studies suggest a possible additional role of the cerebellum in non-motor functions, i. e. cognition. The right posterolateral cerebellar hemisphere is thought to be involved in language, the left posterolateral hemisphere in visuomotor tasks and the vermis in affect and behavior. At present, it is unclear whether cognitive dysfunction should be considered part of the clinical picture of cerebellar disease. Cerebellar disorders, in any case, do not present with prominent cognitive dysfunction.

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