Cognitive and personality function in myotonic muscular dystrophy.

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Cognitive and personality function in myotonic muscular dystrophy.

Twenty-nine patients with myotonic dystrophy from 14 families were tested with the Wechsler and Shipley measures of cognitive function. Forty-one per cent of the subjects had little or no physical handicap. Approximately one-third had low Wechsler scores, whereas 7% had relatively high scores. There was a trend for affected females to have poorer cognitive function than males. Limited cognitive...

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Noninvasive assessment of left ventricular function in myotonic muscular dystrophy.

In order to assess left ventricular function, measurements of left ventricular internal dimension and its rate of change have been made by echocardiography in 7 patients with myotonic dystrophy and the three children of one of them, who were clinically normal but had abnormal muscle biopsies. Electrocardiograms and systolic time intervals were also recorded in all. Only one patient had signs of...

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Neuropsychological deficits in myotonic muscular dystrophy.

Twenty patients with myotonic muscular dystrophy (MMD) were compared with twenty controls on a battery of standardised neuropsychiological tests measuring motor and cognitive functions. The MMD patients performed significantly poorer on both motor and cognitive tests, particularly those assessing spatial functions. Although both motor and cognitive scores were correlated with age, significant d...

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Personality patterns in patients with myotonic dystrophy.

BACKGROUND Myotonic dystrophy (DM) is a multisystemic disease. The central nervous system is affected by cognitive, affective, and personality disturbances. A characteristic behavior was noted from the first clinical descriptions, but no definitive conclusions have been drawn despite extensive debate. As DM is a genetic disease of well-defined abnormality, it may be a good model for understandi...

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ژورنال

عنوان ژورنال: Journal of Neurology, Neurosurgery & Psychiatry

سال: 1984

ISSN: 0022-3050

DOI: 10.1136/jnnp.47.8.888