Depressive and Cognitive Symptoms and Psychiatric Comorbidity of Taiwanese Patients with Machado-Joseph Disease

第三型小腦脊髓運動失調症候群（馬查多－約瑟夫病）之相關認知功能與情緒症狀

黃智婉(Chih-Wan Huang)；劉嘉逸(Chia-Yih Liu)；陸清松(Chin-Song Lu)；莊永毓(Yeong-Yuh Juang)

第三型小腦脊髓運動失調症候群 ； 小腦認知情感綜合症 ； 精神相關症狀 ； spinocerebellar ataxia type 3 ； cerebellar cognitive affective syndrome ； Symptoms Checklist-90-R ； Zung's Depression Scale

台灣精神醫學

28卷2期（2014 / 06 / 01）

ii+103 - 108

英文

目的：Machado-Joseph Disease（MJD）為一種脊髓小腦退化之疾患。小腦病灶已被證實可能造成認知功能及情緒障礙（cognitive and mood disorders）。經查閱，MJD精神或情緒症狀之相關研究相當少見，經由精神科醫師診斷性會談評估的則未有發現。本研究乃對MJD個案之精神症狀及認知功能作系統性之評估。方法：研究MJD個案14名，另取性別年齡配對之正常對照組8名。評量方式包括（A）精神科診斷：由精神科專科醫師以標準結構式會談（Mini Neuropsychiatric Interview，MINI）作精神科診斷；（B）Symptoms Checklist（SCL-90-R）；（C）Zung’sDepression Scale；（D）Wechsler Adult Intelligence Scale；（E）Life Quality Scale。結果：共收案MJD組14名，對照組8名。MJD組中精神科診斷major depression 2名、major depression + dysthymia disorder 2名、generalized anxiety disorder 3名、generalized anxiety disorder + OCD1名、精神分裂症1名。兩組SCL各分項評分個案組皆明顯高於對照組（p ＜ 0.001，Mann-Whitney U test）。Zung’s Depression Scale平均45.9 ± 7.7分，明顯高於對照組33.9 ± 5.6（p = 0.001）。Life Quality Scale平均16.8 ± 6.3分，明顯較對照組差26.4 ± 6.0（p ＜ 0.001）。VIQ，PIQ，Full IQ個案組亦明顯低於對照組（VIQ: p ＜ 0.05，PIQ: p ＜ 0.001，FIQ:p ＜ 0.01）。結論：與對照組比較，MJD個案智力及認知功能較差，較多情緒精神症狀，生活品質方面亦較差。MJD個案近七成有精神科診斷，以憂鬱症為多，顯示MJD個案極需精神醫療之協助。

Objectives: The objectives of this study were to investigate the psychiatric and cognitive symptoms as well as and psychiatric comorbidity in Taiwanese patients with Machado-Joseph disease (MJD). Methods: Fourteen patients with genetically confirmed Machado-Joseph disease and eight sex- and age-matched healthy volunteers were recruited. A board-certified psychiatrist conducted structured diagnostic interview. The Wechsler Adult Intelligence Scale was assessed by clinical psychologists. Both groups received assessment with Symptoms Checklist (SCL-90-R) and Zung’s Depression Scale and GHQ/QL-12. Results: Nine of 14 MJD patients (64.3%), had depressive or anxiety disorders. MJD patients showed significant severity of psychiatric symptoms including somatization (p ＜ 0.05), obsession (p ＜ 0.001), depression (p ＜ 0.05), phobia (p ＜ 0.05), hostility (p ＜ 0.01), paranoia (p ＜ 0.01) and psychotism (p ＜ 0.01). They showed significantly lower scores in verbal (p ＜ 0.05), performance (p ＜ 0.001), and full IQ test (p ＜ 0.01). The life quality was also significantly poorer in patients with MJD compared to normal control (p ＜ 0.001). Conclusion: The Taiwanese patients with MJD have more psychiatric and cognitive disturbance than controls. The disturbances influence their life quality. This condition needs more attention and treatment.

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