悪性発作性頭位眩暉(Bruns症候群,急性小脳虫部症候)の病態生理補遺

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It is well known that paroxysmal positional vertigo is induced by change of head position and subsides immediately when the head is returned to the original position. There are two types of paroxysmal poaitional vertigo. One is what Dix and Hallpike call the benign type, which is thought to be caused by otolith lesions. Another is a malignant type in contrast to the former. It is related to lesion in the central nervous system. Otologists are usually familiar with the former, but the latter is not known even among neurologists or neurosurgeons.We have experienced 37 cases of the malignant type of paroxysmal positional vertigo. They included three cases of metastatic tumor of the cerebellar vermis, six cases of subacute late cerebellar cortical atrophy, five cases of olivo-ponto-cerebellar atrophy, three cases of alcoholic cerebellar atrophy, two cases of post-traumatic cerebellar atrophy, five hematomas of the cerebellar vermis, eight hematomas of the cerebellar hemisphere, two abscesses of the cerebellar hemisphere, two medulloblastomata and one meningioma of the cerebellar vermis.Bruns emphasized two important aspects of the syndrome: 1) vertigo, vomiting, headache and visual disturbance on changing the posture of the head; and 2) freedom from symptoms between episodes. Alpers et al. added a third feature: 3) a constant anterior flexion of the head in most cases, usually at the midline but at times with lateral flexion and rotation. To the above three features of the symptom-complex described by Bruns and Alpers et al., we propose to add a fourth feature: 4) patients usually take a head posture with the affected side down, and if they change the head posture to having the non-affected side down the symptoms tend to be intensified greatly.This symptom-complex has been called "Bruns syndrome", "Das akute Unterwurm-Syndrom" or "retarded type of benign paroxysmal positional vertigo", and confusion and misunderstanding exist about this syndrome. We propose to apply to this syndrome a uniform name of "paroxysmal positional vertigo of malignant type" in contrast to the benign type.The mechanism of the malignant type is usually understood to be intermittent hydrocephalus through blockage of the ventricular system (Bruns, Oppenheim), or derangement of the normal vestibular mechanisms (Alpers et al.). In contrast to these traditional speculations, we consider it to be attributed to the disturbance of the function of the vestibulo-cerebellum (i. e. nodulus and flocculus), which plays a role of inhibiting the vestibulo-ocular system. The characteristic feature of astasia should be emphasized, and we named it "Spinnenkriechen-Syndrom."The lesion of the cerebellar vermis usually presents poor neurological signs except truncal ataxia; therefore, topographical diagnosis to locate this region is sometimes very difficult. To understand and know this symptom-complex mentioned above may lead to the topographically correct diagnosis of the lesion in the cerebellar vermis.

悪性発作性頭位眩暉(Bruns症候群,急性小脳虫部症候)の病態生理補遺

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