Hereditary sensory neuropathy with deafness and dementia: a clinical and neuroimaging study
Hereditary sensory neuropathy with deafness and dementia: a clinical and neuroimaging study
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DOI：
10.1046/j.1468-1331.1999.630357.x
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发表时间：
1999-05-01
影响因子：
5.1
通讯作者：
Mori, E
中科院分区：
文献类型：
--
作者：
Hojo, K;Imamura, T;Mori, E
我们描述了三个兄弟姐妹患者常染色体显性遗传感觉神经病变，感音神经性听力损失和痴呆。患者的认知行为缺陷特征，包括执行功能障碍、冷漠、冷漠和注意力不集中，与额叶功能障碍一致。磁共振成像显示弥漫性脑萎缩，一名患者的氟脱氧葡萄糖正电子发射断层扫描和另一名患者的单光子发射计算机断层扫描显示，额叶内侧和丘脑区域葡萄糖代谢低下或灌注不足。原发性额叶受损伤或继发于丘脑病变的额叶功能障碍可能与痴呆的性质有关。中华神经科杂志，6:357-361 (C) 1999。
We describe three sibling patients with autosomal dominantly inherited sensory neuropathy, sensorineural hearing loss and dementia. The features of cognitive-behavioral deficits in the patients, including executive dysfunction, apathy, indifference and inattention, were consistent with a frontal lobe dysfunction. Magnetic resonance imaging showed a diffuse brain atrophy A fluorodeoxyglucose positron emission tomography in one patient and a single photon emission computed tomography in another demonstrated a glucose hypometabolism or a hypoperfusion in the medial frontal and thalamic regions. Primary frontal involvement or frontal dysfunction secondary to thalamic lesions may contribute to the nature of dementia in these patients, Eur J Neurol 6:357-361 (C) 1999 Lippincott Williams & Wilkins.