Topographic brain mapping of the international cooperative ataxia rating scale

Abstract

The International Cooperative Ataxia Rating Scale (ICARS) is a 100-point semiquantitative scale designed primarily to assess cerebellar dysfunction. However, little is known of the metric properties of this scale. We assessed the ICARS by rating the severity of cerebellar dysfunction in 27 patients with spinocerebellar ataxias (SCA), three patients with sporadic olivopontocerebellar ataxia and 24 healthy control subjects. [(18)F]-fluorodeoxyglucose (FDG) positron emission tomography (PET) study was also performed on each subject. The statistical parametric mapping analyses revealed a significant correlation between the ICARS scores and functional impairment of the frontal regions within SCA patients. The glucose metabolism in the cerebellum, thalamus and caudate nucleus had significant differences between SCA patients and healthy control subjects. The results suggested that the clinical severity of SCA patients correlated with the functional impairment in the frontal regions, the targets of cerebellar efferent projections.

Characterization of Cerebellar Ataxia in Chronic Alcoholics Using the International Cooperative Ataxia Rating Scale (ICARS)

“…The mean total ICARS score in our alcoholic group of 19.67 (range 1 to 43) was relatively lower than the means observed in other groups, for example 30.7 in a group of patients with degenerative cerebellar disease (Richter et al., ) and 52.5 in patients with Friedrich's ataxia (Cano et al., ). However, the range of scores reported in these studies is quite variable (Wang et al., ). In the present study, two‐thirds of our sample of chronic alcoholics scored 14 and above on the ICARS.…”

Consensus Paper: Radiological Biomarkers of Cerebellar Diseases

“…However, the more important clinical application of [ 18 F]-FDG in ataxias is the whole-body PET examination in search of occult primary malignancy in patients with suspected paraneoplastic cerebellar degeneration [14]. Head [ 18 F]-FDG-PET has also been extensively used for physiopathological characterization of chronic inherited or sporadic ataxias [1, 15]. In particular, it documented early hypometabolic areas in presymptomatic carriers of SCA2, SCA3, and SCA17 mutation [16–18] and showed variable correlation with severity of neurological deficit in cross-sectional studies [1].…”

Clinical, genetic, molecular, and pathophysiological insights into spinocerebellar ataxia type 1

“…No abnormalities are identified in T2weighted images. Interestingly, the cerebellar metabolism did not correlate with the severity of ataxia in SCA patients (13). A recent MR functional study reported correlation between the brainstem changes and the neurological deficits in SCA1 (12).…”