Case of Moyamoya Disease Aggravated during the Treatment of Behçet’s Disease

Behçet’s disease (BD) is a multi-organ involved inflammatory disorder described by recurrent oral ulcers and other systemic manifestations. Almost all the clinical manifestations of BD are believed to be due to vasculitis. On the other hand, the cerebral arteries are rarely involved. Moyamoya disease (MMD) is an unusual chronic cerebrovascular disorder that is described by bilateral progressive stenosis or occlusion of the internal carotid artery and an abnormal collateral vascular network. A 32-year-old woman with MMD was referred for fever, oral pain, and diarrhea, and was diagnosed with BD. Her MMD was aggravated during treatment with high dose steroids to control the intestinal BD and a reduction in the MMD medication due to gastrointestinal bleeding. This is the first reported case of intestinal BD in a patient previously diagnosed with MMD, who experienced aggravation of her MMD after the cessation of MMD medication due to aggravated intestinal BD.

A case of Behcet's bisease with pulmonary infarction / 대한내과학회지

Behcet's disease is a nonspecific vasculitis characterized by exacerbations and remissions of unpredictictable duration. The commom clinical features in most patients are orogenital ulcers, uveitis and skin lesions-especially erythema nodosum-like lesions or positive pathergy test. Among the systemic manifestations of Behcet's disease, pulmonary involvement is very rare and only a few cases have been documented. The main event of pulmonary involvement is vasculitis forming multilocular aneurysms and thrombosis of the pulmonary artery. The commom pulmonary manifestations are hemoptysis, dyspnea, pleuritic chest pain, cough and fever. The prognosis of pulmonary vasculitis is very poor. A 30-year-old male patient with Behcet's disease presented with fever and dyspnea for 10 days. In the past years, his vision of the left eye was lost due to chorioretinitis. He took right hemicolectomy because of the perforation of colonic ulcer. Also he has been suffered from left hemiparesis due to multiple cerebral infarction. We describe a case with Behcet's disease with pulmonary infarction improved with prednisolone and cyclosporine.

Isolated Unilateral Lateral Rectus Muscle Palsy in Neuro-Beh-ret Syndrom

Behcet syndrom is a chronic systemic disease developing recurrent oral and genital ulceration, ocular inflammation, arthritis and neurological manifestations. Anterior uveitis and occlusive retinal vasculitis are the most commom ocular manifestation of Behcet syndrome. A 29 years old Korean male with abducens palsy (6th cranial nerve palsy) was found to have oral aphthae, genital ulcer, skin lesion and other neurological manifestations (weakness and sensory loss of lower extremeties, ataxic gait, speech disturbance and urinary difficulty), but he had neither ocular manifestation such as uveitis nor retinal vasculitis.