3-Hz Postural Tremor in MSA-C and SCA: Revisiting an Old but Underestimated Cerebellar Sign by Posturography.

Abstract

The aim of this study is to evaluate the prevalence and electrophysiological features of 3-Hz postural tremor in multiple system atrophy-cerebellar type and spinocerebellar ataxia. A static posturography examination was administered to 37 persons with spinocerebellar ataxia, 58 others with the cerebellar type of multiple system atrophy, and 53 healthy controls. During the sensory organization tests of 5 multiple system atrophy patients, surface electromyograms were recorded from bilateral tibialis anterior and medial gastrocnemius muscles. The patients with multiple system atrophy had, on average, significantly higher scores on the International Cooperative Ataxia Rating Scale and significantly greater prevalence of cross sign. Almost 80% of them fell during the posturography testing compared with two-thirds of the spinocerebellar ataxia patients. Twenty-seven percent of the spinocerebellar ataxia patients and 82.8% of those with multiple system atrophy displayed postural tremor with a frequency of approximately 3Hz. The tremor's frequency tended to be lower in the spinocerebellar ataxia patients. The surface electromyography revealed highly coherent tremor activity at about 3Hz in the patients' bilateral tibialis anterior and alternating firing in the bilateral antagonist muscles. Combining cross sign with a subject's static score of the International Cooperative Ataxia Rating Scale and occurrence of the tremor produced an indicator able to differentiate the two conditions with a sensitivity of 87.9% and a specificity of 89.2%. The area under the receiver operating characteristics curve for the indicator was 0.942. Three-hertz postural tremor is relatively characteristic of cerebellar type of multiple system atrophy and appears at an early stage of the disease. Identification of the tremor by posturography will facilitate its diagnosis.