Miyakawa M, Ohkubo O, Fuchigami T, Fujita Y, Moriuchi R, Hiyoshi K, Ejiri K, Harada K
Department of Pediatrics, Nihon University School of Medicine, Tokyo.
No To Hattatsu. 1995 May;27(3):191-6.
Chorea minor is the most curious manifestation of rheumatic fever, first described by Sydenham in 1686. Subsequent evidence showed that chorea could be a late manifestation of rheumatic fever, often occurring several months after a streptococcal infection in contrast to other major manifestations. During the ten-year period between 1984 and 1993, 11 children with rheumatic fever were seen at our hospital, two cases of these being accompanied with chorea minor. Case 1, a male aged 12, presented with involuntary movements. He was diagnosed as having rheumatic fever because of chorea and systolic ejection murmur at the apex of the heart. Plain cranial CT was normal. However, positron emission computed tomography revealed an increased 11C-glucose uptake in the caudate nucleus as compared with the cerebral cortex. Case 2, a female aged 14, presented with involuntary movements. Plain cranial CT was normal, but single photon emission CT showed a difference between the right and left brain. These two patients were given penicillin G (PCG), predonisolone (PSL) and haloperidol. Haloperidol was administered, because PCG and PSL had no effect to improve the clinical manifestations. After administer decreased to a half with a clinical improvement, but the level of GABA did not change. Haloperidol seemed to be an effective and useful agent for motor manifestations of the disease.
小舞蹈病是风湿热最奇特的表现形式，1686年由西德纳姆首次描述。随后的证据表明，舞蹈病可能是风湿热的晚期表现，与其他主要表现不同，它常发生在链球菌感染后的几个月。在1984年至1993年的十年间，我院共诊治了11例风湿热患儿，其中2例伴有小舞蹈病。病例1，一名12岁男性，出现不自主运动。因舞蹈病及心尖部收缩期喷射性杂音，被诊断为风湿热。头颅平扫CT正常。然而，正电子发射计算机断层扫描显示，与大脑皮质相比，尾状核的11C-葡萄糖摄取增加。病例2，一名14岁女性，出现不自主运动。头颅平扫CT正常，但单光子发射CT显示左右脑有差异。这两名患者均接受了青霉素G（PCG）、泼尼松龙（PSL）和氟哌啶醇治疗。给予氟哌啶醇是因为PCG和PSL对改善临床表现无效。用药量减半后临床症状改善，但γ-氨基丁酸水平未改变。氟哌啶醇似乎是治疗该病运动表现的一种有效且有用的药物。
