CAJ | 학술논문

本文分析了1例经MRI证实的老年型Hallervorden-Spatz病(HSD)，该病在临床上曾误诊为帕金森病。HSD与帕金森病相比较有如下特点：(1)肌强直在病初就同时影响全身，尤以下肢明显；(2)肌强直明显而震颤相对较轻；(3)伴有其他的不自主运动或锥体束征；(4)智能损害出现相对较早较重；(5)服用抗帕金森病药物疗效差；(6)病程进展迅速；(7)MRIT1、T2加权和质子像在苍白球、黑质显示斑点状低密度信号影，特别是脑实质内广泛分布的斑点状低密度信号影和“虎眼征”出现时。根据脑MRI的特点提出了HSD的可能分型。根据HSD临床表现和MRI的特点，在排除其他变性病的基础上，生前诊断HSD是可能的。
본문분석료1례경MRI증실적노년형Hallervorden-Spatz병(HSD)，해병재림상상증오진위파금삼병。HSD여파금삼병상비교유여하특점：(1)기강직재병초취동시영향전신，우이하지명현；(2)기강직명현이진전상대교경；(3)반유기타적불자주운동혹추체속정；(4)지능손해출현상대교조교중；(5)복용항파금삼병약물료효차；(6)병정진전신속；(7)MRIT1、T2가권화질자상재창백구、흑질현시반점상저밀도신호영，특별시뇌실질내엄범분포적반점상저밀도신호영화“호안정”출현시。근거뇌MRI적특점제출료HSD적가능분형。근거HSD림상표현화MRI적특점，재배제기타변성병적기출상，생전진단HSD시가능적。
Objective To explore clinical and brain MRI findings of senile type of Hallervorden-Spatz disese(HSD) in life.Method One case of the senile type of HSD diagnosed by clinical and MRI was analysed,The patient had been diagnosed as Parkinson disease(PD).Result There were the following features on HSD as compared with PD(1) The rigidity was involved in all the extremities simultaneously with the onset,especially on the lower extremities.(2)The rigidity was more seriously than the tremor.(3)Other involuntary movments or signs of pyramidal signs were also impaired.(4)Mental deterioration was early and severely.(5)There were poor effects about the treatments with Madopar.(6)The course of the illness proceeded rapidly.(7)MRI showed macula areas of decreased signal intensity in T1、 T2 weighted and proton images in the globs pallidus and substantial nigra or especially macula areas of decreased signal intensity and the“ eye of the tiger” sign appeared.2.The disense could be divided into several types according to the results of MRI.Conclusion The combination of these neurological with these MRI findings could be considered as higlly snggestive of a diagnosis of HSD in living patient,but other neurodegeneration disorders should be ruled out.