Neuro-Behcet 's Disease-A Report of An Autopsy-confirmed Case and Brief Review of the Literature / 第三军医大学学报

ABSTRACT

An autopsy-confirmed case of neuro-Behcet's disease was reported with a review of the incidence, etiology, pathological changes, clinical manifestations, diagnosis, treatment and prognosis of the condition.The lesions of this case were widely distributed, involving the skin, eyes, mouth, genital organ, peripheral blood vessels, liver and central nervous system. The lesions of the central nervous system were mostly located in the cerebrum. A correct clinical diagnosis of neuro-Behcet's disease may be missed or delayed when its neurological symptoms and signs are the first or main manifestations and the other symptoms appear rather late or mild. On the basis of a definite diagnosis of Behcet's disease, the possibility of neuro-Behcet's disease should always be considered especially in patients with disturbances of the nervous system. The pathological diagnosis of neuro-Behcet's disease must be correlated with the clinical manifestasions. And in making the diagnosis, other nervous system disease, especially demyelinating disease euch as multiple sclerosis, acute disseminated ence-phalomyelitis and neuromyelitis optica should be ruled out.No autopsy-confirmed case of neuro-Behcet's disease has ever been reported in China and thie is likely to be the first case