前庭刺激による感覚認知障害を認めたAdrenoleukodystrophy症例

抄録

The central pathways for the subjective sensation of “turning” and nystagmus are different even though both rely on the same basic input. Thus a lesion of the primary vestibular projection area of the cerebral cortex could induce the phenomenon of the loss of subjective vestibular sensation during vestibular stimulation. This report describes a right handed 35-year-old male with adrenoleukodystrophy, who initially complained of hearing and visual disturbance and showed the vestibular agnosia. MRI showed sub-cortical lesions in the parietal, temporal and occipital areas. The main lesion was in the white matter, especially on the right side (non-dominant hemisphere).
Neurological examination showed auditory agnosia and slight visual agnosia. Deep sensation was also impaired in both upper and lower exthemities, presumably because of cortical lesions. Neurotological examination revealed impaired smooth pursuit, optokinetic nystagmus and voluntary saccades. These abnormal eye movements suggested a parietal or occipital cortex disorder on the non-dominant side. Because a brain stem lesion could be ruled out “nystagmus-sensation dissociation”, which was observed during caloric tests in this case, was considered to be caused by a pure cortical lesion. This phenomenon appeared to be part of the “vestibular agnosia” caused by a lesion of the primary sensory cortex of vestibular sensation.