From the Department of Radiology, Leicester Royal Infirmary, Infirmary Square, Leicester LE1 5WW, England.
Radiology. 2017 Mar;282(3):916-921. doi: 10.1148/radiol.2016150707.
History A 63-year-old man with learning difficulties presented to the Accident and Emergency Department with right ankle pain after an inversion injury and underwent plain radiography. The patient had developed normally until his teenage years, at which point he experienced cognitive regression. He experienced swallowing difficulties, tinnitus, and fecal incontinence, and he had undergone cataract surgery at the age of 20 years. He also had a small nodule on the volar surface of his right ring finger. Magnetic resonance (MR) imaging of the brain and the right ankle had been performed 3 years previously. Routine biochemistry (full blood count and renal function) results were normal. Total cholesterol level was 3.6 mmol/L (normal, <5.0 mmol/L). The patient had three siblings who had the same condition, with one having died in childhood.
病史 一位 63 岁有学习障碍的男性,在踝关节扭伤后因疼痛就诊于急症室,并接受了 X 线平片检查。患者在青少年时期前发育正常,此后出现认知能力倒退。他有吞咽困难、耳鸣和大便失禁,20 岁时接受了白内障手术。他的右环指掌面还有一个小结节。3 年前曾行脑部和右踝关节磁共振成像检查。常规生化检查(全血细胞计数和肾功能)结果正常。总胆固醇水平为 3.6mmol/L(正常值<5.0mmol/L)。患者有 3 个兄弟姐妹,均有相同的情况,其中 1 人在儿童期死亡。