COGNITIVE AND PERSONALITY FUNCTION IN MYOTONIC MUSCULAR-DYSTROPHY
COGNITIVE AND PERSONALITY FUNCTION IN MYOTONIC MUSCULAR-DYSTROPHY
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DOI：
10.1136/jnnp.46.11.971
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发表时间：
1983-01-01
影响因子：
11
通讯作者：
GRIEP, E
中科院分区：
文献类型：
--
作者：
BIRD, TD;FOLLETT, C;GRIEP, E
来自14个家族的强直性肌营养不良患者（29例）进行了Wechsler和Shipley认知功能测试。41%的受试者几乎没有或没有身体残疾。大约1/3的人的韦氏评分较低，而7%的人的评分相对较高。受影响的女性认知功能比男性差。有限的认知能力与母亲遗传的基因和严重的身体残疾，但也有个别例外。最强的认知能力是语言和信息，而最弱的是即时回忆，抽象和空间操作和方向。没有证据表明智力随着时间的推移而下降。CT（计算机断层扫描）扫描显示的脑萎缩体征并不常见，19名受试者中只有1人出现。人格概况也构建了25肌强直受试者使用访谈和MMPI [明尼苏达多相人格调查表]技术。44%的受试者有不显著的人格特征，24%有轻度人格困难，32%有明显的人格异常。严重的人格障碍多见于认知能力低下和重度肢体残疾的患者。没有代表整个群体的典型人格模式。许多人格问题可能是资源有限的人试图科普身体变形和衰弱的神经肌肉疾病的结果。
Patients (29) with myotonic dystrophy from 14 families were tested with the Wechsler and Shipley measures of cognitive function. Of the subjects 41% had little or no physical handicap. Approximately 1/3 had low Wechsler scores, whereas 7% had relatively high scores. Affected females had poorer cognitive function than males. Limited cognitive ability correlated with maternal inheritance of the gene and severe physical handicap, but there were individual exceptions. Strongest cognitive abilities were verbal and informational, whereas the weakest were immediate recall, abstraction and spatial manipulation and orientation. There was no evidence of intellectual decline with time. Signs of cerebral atrophy on CT [computer tomography] scans were uncommon, occurring for certain in only one of 19 subjects. Personality profiles were also constructed for 25 myotonic subjects using interview and MMPI [Minnesota Multiphasic Personality Inventory] techniques. Of the subjects 44% had unremarkable personality profiles, 24% had mild personality difficulties and 32% had prominent personality abnormalities. Serious personality difficulty was most common in patients with low cognitive ability and advanced physical handicap. There was no typical personality pattern representative of the entire group. Many personality problems may be the result of individuals with limited resources attempting to cope with their physically deforming and debilitating neuromuscular disorder.