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

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

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’s Depression 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、general- ized 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 as-sessed by clinical psychologists. Both groups received assessment with Symp-toms 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 pa-tients 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.