FIXATION INSTABILITY AND OCULOMOTOR ABNORMALITIES IN FRIEDREICHS ATAXIA

Eye movements were studied in 13 patients with Friedreich's ataxia and correlated with MRI findings to investigate whether oculomotor abnorm alities can be traced to cerebellar disturbances in this disease. One of the most prominent eye signs was fixation instability (square-wave jerks, SWJ.). Besides SWJ the patients showed various combinations of cerebellar, vestibular and brain-stem oculomotor signs. Our patients d id not comprise a homogeneous group with regard to their oculomotor fi ndings. There was no correlation between the severity of any of the so -called cerebellar oculomotor disturbances and the number of SWJ. We t ried to correlate the extent of oculomotor disturbances with floccular atrophy and atrophy of the dorsal vermis on MRI in seven of the patie nts. None of the oculomotor features (including SWJ) correlated with f locculus or dorsal vermis size. Furthermore, floccular and vermal meas urements on MRI were normal. Accordingly, we think it unlikely that th e oculomotor disturbances, including SWJ, are attributable to cerebell ar pathology per se.