强直性营养不良
额叶
心理学
神经心理学
脑血流
执行功能障碍
肌强直
听力学
认知
发射计算机断层扫描
神经心理学测验
内科学
正电子发射断层摄影术
心脏病学
医学
神经科学
作者
G. Meola,Valeria Sansone,Daniela Perani,Silvio Scarone,Stefano F. Cappa,C. Dragoni,Elena Cattaneo,Maria Cotelli,C. Gobbo,F. Fazio,Gabriele Siciliano,Michelangelo Mancuso,Eugenio Vitelli,S Zhang,Ralf Krahe,Richard T. Moxley
标识
DOI:10.1016/s0960-8966(03)00137-8
摘要
A previous study in proximal myotonic myopathy (PROMM/DM-2) and myotonic dystrophy type 1 (DM-1) using brain positron emission tomography demonstrated a reduced cerebral blood flow in the frontal and temporal regions associated with cognitive impairment. The objective was to investigate further cognitive and behavioural aspects in a new series of patients with DM-1 and PROMM/DM-2. Nineteen patients with genetically determined PROMM/DM-2 and 21 patients with moderately severe DM-1 underwent neuropsychological testing and neuropsychiatric interviews. DM-1 and PROMM/DM-2 patients had significantly lower scores on tests of frontal lobe function compared to controls. Neuropsychiatric interviews demonstrated an avoidant trait personality disorder in both patient groups. Brain single photon emission computed tomography showed frontal and parieto-occipital hypoperfusion. The results suggest that there is a specific cognitive and behavioural profile in PROMM/DM-2 and in DM-1, and that this profile is associated with hypoperfusion in frontal and parieto-occipital regions of the brain.
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