Department of Neurology and IFB (LMU) Institute for Clinical Neurosciences, University Hospital Munich, Munich, Germany.
Ann N Y Acad Sci. 2011 Sep;1233:127-38. doi: 10.1111/j.1749-6632.2011.06175.x.
A significant number of patients with bilateral vestibulopathy suffer from cerebellar ataxia and central vestibular symptoms and vice versa. We examined 31 patients presenting with the combination of gait and stance ataxia, cerebellar ocular motor signs, and a bilaterally pathological head-impulse test (HIT). Tests included neuro-orthoptical examination, electromyography and neurography, caloric irrigation, pure-tone audiogram, vestibular-evoked myogenic potentials, and volumetric magnetic resonance imaging (MRI). Only 17 of 31 patients had a pathological caloric irrigation. Twenty-three patients had evidence of polyneuropathy (predominantly mixed sensorimotor involving axonal loss and demyelination) and twenty of hypoacusis (1 unilateral and 19 bilateral). Voxel-based morphometry comparing local gray matter brain volume between patients and controls revealed cerebellar atrophy involving both the vermis and the hemispheres. We conclude that there is a clinically relevant combination of cerebellar ataxia with cerebellar atrophy, bilaterally pathological HIT, polyneuropathy, and hypoacusis. This multisensory syndrome is most likely caused by a neurodegenerative disorder affecting different systems, leading to severe impairment of affected patients.
相当数量的双侧前庭病患者同时患有小脑性共济失调和中枢性前庭症状，反之亦然。我们检查了 31 名同时出现步态和站位共济失调、小脑眼动体征和双侧病理性头脉冲试验（HIT）的患者。检查包括神经眼科学检查、肌电图和神经电图、冷热试验、纯音听力图、前庭诱发肌源性电位和容积磁共振成像（MRI）。只有 31 名患者中的 17 名冷热试验异常。23 名患者存在多发性神经病（主要是混合感觉运动性神经病，涉及轴索丢失和脱髓鞘），20 名患者存在听力减退（单侧 1 名，双侧 19 名）。将患者和对照组之间局部灰质脑体积进行基于体素的形态计量学比较，结果显示小脑蚓部和半球均有萎缩。我们得出结论，存在一种具有临床相关性的组合，即小脑性共济失调伴小脑萎缩、双侧病理性 HIT、多发性神经病和听力减退。这种多感觉综合征最有可能由影响不同系统的神经退行性疾病引起，导致受影响患者严重受损。
