SYMPTOMATIC OCCULT HYDROCEPHALUS WITH NORMAL CEREBROSPINAL-FLUID PRESSURE - A TREATABLE SYNDROME
SYMPTOMATIC OCCULT HYDROCEPHALUS WITH NORMAL CEREBROSPINAL-FLUID PRESSURE - A TREATABLE SYNDROME
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DOI：
10.1056/nejm196507152730301
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发表时间：
1965-01-01
影响因子：
158.5
通讯作者：
SWEET, WH
中科院分区：
文献类型：
--
作者：
ADAMS, RD;FISHER, CM;SWEET, WH
本文报告3例脑室扩大的常压脑积水的临床资料。所有3例患者均通过外科分流术成功治疗。该综合征包括一种进行性痴呆，其特征是健忘、精神运动迟缓和步态不稳。在晚期，可能出现运动不能性缄默症和严重的双侧锥体束和锥体外系征。脑脊液压力低于200毫米汞柱，气脑造影显示部分或全部脑室系统扩张，空气不能上升到大脑蛛网膜下腔。病因不明的脑干周围蛛网膜下腔阻塞2例，第三脑室囊肿1例。提出了正常脑脊液压力下脑积水的液压机制，并作了简要讨论。据推测，这一物理原理将适用于其他神经系统疾病。这些患者没有并发的内在脑疾病，这使得有可能清楚地描绘慢性脑积水对大脑的临床影响。证据表明，它们的起源在增加脊髓液力施加到心室壁。这些病例的识别和治疗是非常重要的，因为它将导致相当于“治愈”与早老性痴呆或老年性痴呆非常相似的临床病症。在大量的老年痴呆症患者中，必须寻找更多的病例。
The clinical details of 3 cases of "normal-pressure hydrocephalus" with greatly enlarged ventricles are presented. All 3 patients were successfully treated by a surgical shunt procedure. The syndrome consists of a progressive dementia characterized by forget-fulness, psychomotor retardation and unsteady gait. At an advanced stage akinetic mutism and severe bilateral pyramidal and extrapyra-midal signs may be present. The cerebrospinal-fluid pressure is less than 200 mm, and a pneumoencephalogram shows dilatation of part or all of the ventricular system and failure of air to ascend into the cerebral subarachnoid space. The cause was obstruction of the subarach-noid space around the brainstem, of undetermined origin, in 2 of our cases and a cyst of the 3rd ventricle in the other. A hydraulic-press mechanism of hydrocephalus with normal cerebrospinal-fluid pressure is postulated and is briefly discussed. It is surmised that this physical principle will apply to other neurologic disorders. The absence of complicating intrinsic cerebral disease in these patients made it possible to delineate clearly the clinical effects of chronic hydrocephalus on the brain. Evidence of their origin in increased cerebrospinal-fluid force applied to the walls of the ventricles is presented. Recognition and treatment of these cases is of great importance since it will result in what amounts to a "cure" of a clinical condition that closely resembles presenile or senile dementia. It is in the large group of patients with late-life dementia that further cases must be sought.