MENTAL DISORDERS OF Del22q11.2 SYNDROME (CATCH 22) : CROSS-SECTIONAL AND LONGITUDINAL OBSERVATION OF 8 CASES
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DiGeorge症候群,円錐動脈幹異常顔貌症候群,軟口蓋心臓顔貌症候群に共通して,22番染色体長腕の微小欠失がみつかり,1993年以来"CATCH22"(Wilson)としてまとめられている.しかし,上記の三症候群にあげられた症状は,CATCH22に提示された五主微にとどまらぬため,22q11.2欠失症候群という別の名称も提案されている.この症候群には精神分裂病などの内因性精神病像をはじめ,精神障害が好発することが報告されてきた.近年は精神分裂病ではなく,双極感情病との関係も指摘されている。本研究では,主に東京女子医大病院精神科で診療した8例について報告する.5例が精神分裂病,1例が分裂感情病(躁型),1例が身体化障害兼心気症,1例が境界性人格障害であった(DSM-IV).精神科初診年齢は,平均約23.8歳であった.7例に精神遅滞が認められた.また精神障害発症前からすでに進路に関する葛藤がみられた.6例はいわゆる内因性精神病像を呈したが,6例すべては分裂病症状を呈していた.本例の経過は慢性で転帰は良好ではない.残遺症状がおこりやすく,気分の不安定さが持続した.大多数の症例で社会的職業的水準が低下した.もっとも,社会的な退却は精神障害の発症前からみられた.現在,本症候群に親縁なのは精神分裂病か双極性感情障害かが議論されている.本論はこの疑問に答えようとした.診断的には精神分裂病にはるかに密接だが,横断面像にみられる感情病の要素についても,また波状型の経過をとることにおいても,感情病にも明らかに関係していた.A common characteristic found in most patients affected with DiGeorge syndrome, conotruncal anomaly face syndrome and velo-cardio-facial syndrome, is the microdeletion of chromosome 22g11.2. Wilson integrated these syndromes into a group designate as CATCH 22. However, because the range of symptoms presented in the three aforementioned syndromes are broader than the five symptoms declared as CATCH 22, an alternative nomination was also proposed. This is known as "del22g11.2 Syndrome". It has already been reported that frequent co-occurrence of major psychoses exist in each syndrome, including schizophrenia. Recently the association to the bipolar affective disorder is also mentioned. We report 8 cases of mental disorder in del-22g11.2 Syndrome. Five cases were diagnosed as schizophrenia, one case as schizoaffective disorder, one case as somatization disorder and hypochondriasis, and one case as borderline personality disorder by DSM-IV criteria. The average age of the patients was 23.8 years at the first examination, and mental retardation was found in 7 out of 8 cases. Prior to the onset of the mental disorder the patients had already been in a distressed state about their future life. Six patients have presented clinical pictures of so-called endogenous psychoses. All of these 6 patients showed schizophrenic symptoms. The clinical courses went into slight remission; residual symptoms remained; mood instability continued. The social and occupational standard deteriorated in most patients. However, prior to the mental disorder the patients had already shown social withdrawal. In this study, controversy of diagnosis, schizophrenia or bipolar disorder, was discussed. The diagnosis of psychiatric illnesses of del22g11.2 syndrome was far more associated with schizophrenia, but their clinical pictures and courses were also significantly related to affective disorder.
- 1998-07-25
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