ベーチェット病の神経耳科学的検討

Abstract

It seems acceptable that pathological brain stem lesions exist in the patient with Neuro-Behçet disease (NB). However, only few reports have been published in this field. We report the neuro-otological findings in 33 patients who fulfilled the diagnostic criteria of Behçet disease completely or incompletely. The patients were classified into 2 groups: one is group of NB and the other is a group of Behçet disease without NB (non-NB). Furthermore, the latter was divided into 2 subgroups of patients with dysequilibrium and patients with normal equilibrium. They underwent equilibrium function tests, such as body balance test, gaze test, spontaneous and positional nystagmus test, eye tracking test (ETT) and optokinetic pattern test (OKP) by using electro-nystagmography (ENG). The following results were obtained. 1. Cerebellar and brain stem lesions were confirmed in all cases of NB group. 2. Most cases (6 of 8 cases) in the subgroup of dysequilibrium of the non-NB group showed peripheral vestibular disturbance. The other two patients were diagnosed to be vertebro-basilar insufficiency (VBI) and vascular Behçet disease with CNS disorder, respectively. 3. Nine of 13 patients in the normal equilibrium subgroup of the non-NB group were diagnosed to be neuro-otologically normal. However, we found two patients with peripheral vestibular disturbance and CNS disorder associated with hearing disturbance of the retrocochlear origin, respectively. 4. The neuro-otological examination is assumed to be useful to diagnose neuro-Behçet disease, to reveal the lesion site and the state of dysequilibrium in patients with Behçet disease.