[Neuro-Behçet disease with bilateral cheiro-oral syndrome following simultaneous multiple brain hemorrhage].

A 34-year-old man with neuro-Behçet disease showed bilateral cheiro-oral syndrome following simultaneous multiple brain hemorrhage. The patient suddenly developed fever, headache, right-sided heminumbness, and gait disturbance after ciclosporin therapy was instituted. Bilateral uveitis, oral aphthous ulcers and follicular skin eruptions were observed. Neurologically, apathetic state, horizontal gaze palsy, spastic paraparesis with right Babinski sign, gait difficulty, and painful dysesthesia of perioral regions, right upper and lower extremities and left hand were revealed as main symptoms. Erythrocyte sedimentation rate was elevated and C-reactive protein was positive. Cerebrospinal fluid examination showed lymphocytic pleocytosis. A brain CT scan demonstrated focal high density areas with slight contrast enhancement in the median region of the tegmentum of pons, and in the subcortex of right superior parietal lobe. T1, T2 and proton density images of MRI taken 50 days after the onset disclosed small irregular low signal intensities in the lesions that correspond to the CT findings. Corticosteroid was administered immediately after admission, which resulted in a rapid improvement of clinical symptoms and a complete disappearance of laboratory abnormalities and CT changes. As dysesthesia was attenuated, bilateral typical symptoms of cheiro-oral syndrome were found. These results suggest that the intracerebral hemorrhage was caused by angitis related to Behçet disease. MRI findings implicate that the involvement of bilateral paramedian areas including medial parts of both the medial leminiscus and the ventral secondary ascending tract of the trigeminal nerve is responsible for bilateral cheiro-oral syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)