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轻度中央桥脑髓鞘溶解症:一种常未被发现的综合征。

Mild central pontine myelinolysis: a frequently undetected syndrome.

作者信息

Pfister H W, Einhäupl K M, Brandt T

出版信息

Eur Arch Psychiatry Neurol Sci. 1985;235(3):134-9. doi: 10.1007/BF00380982.

Abstract

Over a period of 1 year we diagnosed central pontine myelinolysis (CPM) in five patients all of whom survived, two of them with complete functional recovery despite extensive lesions on cranial computerized tomography and magnetic resonance imaging. Diagnosis was based upon the combination of an acute brainstem dysfunction with typical neuroradiological features; a history of chronic alcoholism or a preceding hyponatremia may serve as a diagnostic hint. The spectrum of symptoms ranged from severe tetraplegia and cranial nerve palsies to latent signs of pyramidal tract lesions and discrete ocular motor abnormalities. In two patients pontine and extrapontine manifestations of demyelination were confirmed neuroradiologically; in one patient a solely extrapontine manifestation was present. Thus it is reasonable that: (1) the incidence of comparatively mild forms of CPM as well as extrapontine manifestations are more frequent than hitherto assumed, (2) the clinical outcome of the syndrome is better than expected from earlier fatal case reports and is quite independent of the extent of the lesion as it appears with brain imaging methods.

摘要

在1年的时间里,我们诊断出5例中央桥脑髓鞘溶解症(CPM)患者,所有患者均存活,其中2例尽管头颅计算机断层扫描和磁共振成像显示有广泛病变,但功能完全恢复。诊断基于急性脑干功能障碍与典型神经放射学特征的结合;慢性酒精中毒史或先前的低钠血症病史可作为诊断线索。症状范围从严重的四肢瘫痪和颅神经麻痹到锥体束病变的潜在体征和离散的眼球运动异常。在2例患者中,神经放射学证实了桥脑和脑桥外脱髓鞘表现;在1例患者中,仅出现脑桥外表现。因此,有理由认为:(1)相对轻度形式的CPM以及脑桥外表现的发生率比迄今所认为的更为频繁,(2)该综合征的临床结果比早期致命病例报告所预期的要好,并且与脑成像方法所显示的病变范围完全无关。

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