title: Postural responses to multidirectional stance perturbations in cerebellar ataxia.
abstract: Previous studies of patients with focal cerebellar damage underscored the importance of the cerebellum for balance control. These studies were restricted to postural control in the pitch plane, and focused mainly on leg muscle responses. Here, we examined the effect of degenerative cerebellar lesions on postural control in multiple directions, and studied how such lesions affect intersegmental coordination of the legs, trunk and arms. We formulated two main questions. (a) Do patients with cerebellar ataxia predominantly have balance problems in the sagittal or frontal planes? (b) Is instability in cerebellar ataxia associated with increased joint motion or with reduced joint motion? We selected nine patients with autosomal dominant spinocerebellar ataxia (SCA)--three with pure ataxia and six with mild extra-cerebellar features--and 12 matched controls. Upright standing subjects received support surface rotations (7.5 degrees at 60 degrees /s) that were randomly delivered in eight different directions of pitch or roll. We used full body kinematics to determine displacements of the center of mass (COM) and of individual body segments. We also collected surface EMG from 10 leg, trunk and arm muscles. Primary variables of interest were COM displacement and trunk control (angles and muscle responses). Secondary analyses focused on angles and muscle responses of the legs and arms. COM analysis demonstrated that SCA patients had greatest instability following backward and laterally directed perturbations. Major factors in causing this instability were, first, a marked reduction of stimulus-induced knee flexion and, second, excessive "hypermetric" motion of the pelvis (in roll) and trunk (in pitch). Muscle responses of SCA patients were characterized by increased late balance correcting activity. Responses of patients with pure ataxia were comparable to those of patients with mild extra-cerebellar features. A main underlying cause of postural instability in SCA patients appears to be "locking" of the knees, which may reflect compensation (by reducing interaction between body links) or reduced vestibulocerebellar control over leg muscles. The observed pathophysiology is very different from that seen in other patient populations.
authors.affiliation: Department of ORL, University Hospital, Basel, Switzerland.
authors.forename: Maaike
authors.initials: M
authors.lastname: Bakker
authors.forename: John H J
authors.initials: JH
authors.lastname: Allum
authors.forename: Jasper E
authors.initials: JE
authors.lastname: Visser
authors.forename: Christian
authors.initials: C
authors.lastname: Grüneberg
authors.forename: Bart P
authors.initials: BP
authors.lastname: van de Warrenburg
authors.forename: Berry H P
authors.initials: BH
authors.lastname: Kremer
authors.forename: Bastiaan R
authors.initials: BR
authors.lastname: Bloem
mesh_terms: D000328:Adult; D000704:Analysis of Variance; D001696:Biomechanical Phenomena; D002524:Cerebellar Ataxia / Q000503:physiopathology*; D004576:Electromyography / Q000379:methods; D005246:Feedback / Q000502:physiology; D005260:Female; D006801:Humans; D007866:Leg / Q000294:innervation / Q000502:physiology; D008297:Male; D008875:Middle Aged; D009068:Movement / Q000502:physiology*; D018482:Muscle, Skeletal / Q000503:physiopathology; D009949:Orientation / Q000502:physiology; D004856:Postural Balance / Q000502:physiology*; D011187:Posture / Q000502:physiology*; D011434:Proprioception / Q000502:physiology*; D013997:Time Factors