Departamento de Neuropsiquiatria, Universidade Federal de Santa Maria, Santa Maria, Rio Grande do Sul, Brasil.
Departamento de Neuropsiquiatria; Programa de Pós-graduação em Ciências biológicas: Bioquímica Toxicológica; Laboratório de Bioquímica do Exercício, Universidade Federal de Santa Maria, Santa Maria, Rio Grande do Sul; Programa de Pós-Graduação em Farmacologia, Universidade Federal de Santa Maria, Santa Maria, Rio Grande do Sul, Brasil.
Neurol India. 2023 Jul-Aug;71(4):767-771. doi: 10.4103/0028-3886.383870.
We report a 24-year-old male with blepharophimosis, psychomotor retardation, brachycephaly, microstomia, immobile face, high arched palate, single palmar crease, kyphoscoliosis, talipes equinovarus, inguinal hernia, pyloric stenosis, recurrent infections, bilateral camptodactyly, wide-set eyes, decreased muscle mass, hypotonia, exotropia, and ptosis in the left eye, growth retardation, multiple congenital contractures, and hyporreflexia. Contractures improved with aging, but intellectual disability and blepharophimosis remained. He also presented epilepsy, outbursts of laughter, and predisposition to drug adverse effects (skin lesions with carbamazepine and secondary parkinsonism).
我们报告了一例 24 岁男性患者,其具有眼睑痉挛、精神运动发育迟缓、短头畸形、小口畸形、面容呆板、高拱形腭、单一掌褶、脊柱后凸侧凸、马蹄内翻足、腹股沟疝、幽门狭窄、反复感染、双侧手指屈肌挛缩、眼睛宽距、肌肉量减少、张力减退、外斜视和左眼上睑下垂、生长迟缓、多发先天性挛缩和腱反射减弱。挛缩随年龄增长而改善,但智力残疾和眼睑痉挛仍然存在。他还患有癫痫、大笑发作和易发生药物不良反应(卡马西平引起的皮肤损伤和继发帕金森病)。