CAJ | 학술논문

目的：分析肝豆状核变性(HLD)广泛大脑皮质病变的MRI特征。方法回顾性分析该院在2010年9月—2013年9月经确诊的15例HLD患者的临床资料。结果①该组15例患者均以言语不清为首发症状，反应迟钝6例，认知功能障碍8例，精神症状(比如兴奋躁动、脾气暴躁等)5例，继发痫性发作6例；②双侧皮质以及皮质下白质呈广泛对称性分布，存在大片状长T1、长T2异常信号。其中，脑萎缩发生率为66.67％(10/15)，主要表现为脑室系统异常扩大；轻度5例，中度3例，重度2例；③经治疗后，患者的椎体外系症状均控制良好。结论在HLD患者的MRI表现中，除了比较典型的基底节区病变外，同样存在脑部多个部位受累现象，以椎体外系症状为主临床表现，采用相应的治疗干预措施，可有效缓解患者的椎体外系症状。
목적：분석간두상핵변성(HLD)엄범대뇌피질병변적MRI특정。방법회고성분석해원재2010년9월—2013년9월경학진적15례HLD환자적림상자료。결과①해조15례환자균이언어불청위수발증상，반응지둔6례，인지공능장애8례，정신증상(비여흥강조동、비기폭조등)5례，계발간성발작6례；②쌍측피질이급피질하백질정엄범대칭성분포，존재대편상장T1、장T2이상신호。기중，뇌위축발생솔위66.67％(10/15)，주요표현위뇌실계통이상확대；경도5례，중도3례，중도2례；③경치료후，환자적추체외계증상균공제량호。결론재HLD환자적MRI표현중，제료비교전형적기저절구병변외，동양존재뇌부다개부위수루현상，이추체외계증상위주림상표현，채용상응적치료간예조시，가유효완해환자적추체외계증상。
Objective?To?analyze?the?liver?degeneration?(HLD)?widely?MRI?features?of?cerebral?cortex?lesions.?Methods?Retrospective?analysis?in?September?2010?to?September?2013?of?the?clinical?data?of?15?patients?with?HLD?confirmed.?Results?①In?15?patients?who?take?glossolalia?as?starting?symptoms,?slow?6?cases,?8?cases?of?impaired?cognitive?function?and?mental?symptoms?(such?as?excitement?agitation,?temper,?etc.)?in?5?cases,?seizures?secondary?6?cases; ② The?bilateral?cortical?and?subcortical?white?matter?are?widely?distribution?of?symmetry,?there?are?big?shape?abnormal?long?T1,?long?T2?signal.?Among?them,?the?incidence?of?brain?atrophy?was?66.67%?(10/15),?mainly?characterized?by?abnormal?ventricular?system?expanded;?Mild?in?5?cases,?moderate?in?3?patients,?severe?in?2?cases.?③After?treatment,?patients?with?vertebral?body?symptoms?were?well?controlled.?Conclusion?In?the?MRI?manifestation?of?HLD?patients,?in?addition?to?the?typical?of?basal?ganglia?lesion,?parts?of?the?brain?involved?phenomenon?also?exists,?with?the?vertebral?body?is?the?symptom?clinical?manifestations,?adopt?corresponding?treatment?interventions,?can?effectively?alleviate?symptoms?of?patients?with?vertebral?body?system.