Hemichorea-Hemiballism 症候群：十三個臨床案例分析

Hemichorea-Hemiballism: Clinical Study of 13 Patients

目的：Hemichorea-hemiballism(HCHB)是個不常見的非自主運動，以不同幅度單側肢體揮動表現，這篇文章目的在於探討造成HCHB之原因及相關的神經影像學。方法：我們回朔分析近兩年十三位HCHB病人的病歷，共五位男性，八位女性，年齡分有從40～89歲（平均年齡69±11.36歲），七位病人有進行腦部電腦斷層掃描(CT)，四位病人有腦部核磁共振攝影(MRI)，兩位同時接受CT及MRI檢查。結果：七位病患是糖尿病續發高血糖症，六位有腦中風，在高血糖病患中，發病平均血糖爲392.21 mg/dl，兩位病患在CT上有發現基底核處有高訊號，三位於MRI上蒼白球、尾狀核、豆狀核有高訊號，兩位在CT上並無異常發現，腦中風病患中，兩位病人在MRI上有基底核梗塞，一位有視丘梗塞，一位有前額葉及輻射狀冠處有梗塞，一位有尾狀核梗塞，另一位在CT上並無發現，在預後方面，十三位病患中，有一位自然痊癒，四位在接受治療幾天至一個月內痊癒，一位病患發病十個月後復發，其餘病患仍有間斷性的發作，在門診接受定期治療。結論：糖尿病續發高血糖症和腦中風仍是造成HCHB首要原因，七位病患其病灶處基底核，兩位處基底核外，HCHB預後屬良好，但復原的時間不一，大多數病患需要幾天至幾個月的藥物治療時間來達到痊癒。

Purpose Hemichorea-hemiballism(HCHB) is an uncommon involuntary movement that designates a unilateral uncontrollable, poorly patterned flinging movement of limb at variable amplitude. Our aim of this review is for further etiology recognition and its correlated neuroimaging study in patients with HCHB. Methods We retrospectively studied the records of thirteen patients with HCHB obtained during a period of 2 years (five men and eight women) with ages ranged from 40 to 89 years (mean age 69 ± 11.36 years). Seven patients underwent only brain CT, four patients only with brain MR images and two patients underwent both brain CT and MR images. Results Seven patients had hyperglycemia secondary to diabetes mellitus and six patients had stroke. Among the patients with hyperglycemia, the average blood sugar at the onset was 392.21mg/dl, two patients were found with increase attenuation change at basal ganglia via brain CT, three of them revealed hyperintense lesion at putamen, caudate nucleus and globus pallidus on T1-weighted brain MR images, two showed no evidence of brain lesion on brain CT. Two patients of stroke had basal ganglia infarction, one had thalamus infarction, one had multiple infarction at frontal and corona radiata on brain MR image; one had caudate nucleus infarction, and one with no lesion on brain CT. One of thirteen patients had spontaneous remission in days, four of them had resolution after neuroleptic drugs treatment in days to a month, one patient had symptoms recurrence 10 months after the first attack, the remaining patients had intermittent hemiballistic movement with treatment at out-patient clinic. Conclusion Hyperglycemia secondary to diabetes mellitus and stroke remain as the major causes of HCHB. Seven patients had lesions of basal ganglia and two patients had lesion outside the basal ganglia. The prognosis of HCHB is benign but variable in resolution time. Most of the patients required days to months for symptoms resolution under the neuroleptic agent.