Oculostatic and visual phenomena occurring in association with Wallenberg's syndrome.
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We report the bilateral absence of response to tests of vestibular function in 5 patients with familial dysautonomia.
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Six patients are described who experienced difficulty in driving a motor car. Four had illusions that the car was turning, which occurred particularly on open, featureless roads or the brows of hills and caused the driver to stop. All patients had peripheral or central neurootological abnormalities, but the only finding consistent with the directionality of the symptoms was an unpleasantly increased sense of circularvection during optokinetic stimulation in the direction of the illusion. These problems occur because of a false sense of orientation arising either from inappropriate signals from disordered vestibular canal and otolith organs or from a disordered central interpretation of vestibular information, and become manifest in the absence of adequate visual stabilisation. The other two patients with lateralised vestibular disease made inappropriate steering adjustments in the direction of the imbalance of vestibular tone.
Three patients with spasmodic torticollis following VIII nerve lesions (VIII-ST) underwent quantitative assessment of their sternomastoid EMG during vestibular (otolith and semicircular canal) stimulation. The results were compared with a normal control group and with six patients with idiopathic spasmodic torticollis (ST). Backwards tilt of the VIII-ST patients resulted in a marked increase in the EMG, especially in the more affected sternomastoid, whereas this manoeuvre did not have a significant effect in normal subjects, or had a variable effect in the ST group. These results suggest that those with torticollis following VIII nerve lesions are a distinct group. Since there was no relationship between the side of the VIII nerve lesion and the direction of the torticollis a direct aetiological link between the two is, however, unlikely. The unusual EMG/tilt responses are explained on the basis of peripheral imbalance of utricular signals (maximal in the supine position) in the presence of central deranged processing of information concerning head posture.
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