Shunting effects in patients with idiopathic normal pressure hydrocephalus; correlation with cerebral and leptomeningeal biopsy findings

Shunting effects in patients with idiopathic normal pressure hydrocephalus; correlation with cerebral and leptomeningeal biopsy findings
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DOI:
10.1007/s007010050353
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发表时间:
1999-01-01
影响因子:
2.4
通讯作者:
Juhler, M
Juhler, M
中科院分区:
医学3区
文献类型:
--
作者:
Bech, RA;Waldemar, G;Juhler, M

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正常压力脑积水(NPH)是一个潜在的治疗综合征异常脑脊液动力学。脑膜纤维化和/或蛛网膜下腔闭塞已被认为是病理解剖基质之一。然而,其他类型的成人痴呆,主要是阿尔茨海默病和血管性痴呆可能模仿临床NPH特征。本研究的目的是在一组前瞻性特发性NPH (INPH)患者中,将脑实质和脑膜活检结果与脑脊液分流后的临床结果联系起来。本研究包括27例INPH患者,根据普遍接受的临床、影像学和流体动力学标准诊断和分流。所有患者在插入分流器前均行额脑膜和脑活检。27例活组织检查中有14例出现脑退行性改变,最常见的是阿尔茨海默病(6例)或血管改变(7例)。18例蛛网膜组织活检中有9例蛛网膜纤维化。总体而言,9名患者(33%)改善,其中6名在活检中表现为阿尔茨海默病或血管改变。临床结果与是否存在退行性脑改变和/或蛛网膜纤维化之间没有相关性。然而,在出现退行性脑改变或蛛网膜纤维化的亚组中,有更高的改善率的趋势。结果表明,在INPH综合征中不存在固定的形态学因素。发病机制可能涉及多种病因,在某些情况下可能共存:尽管存在退行性脑实质改变,但患者也可能通过分流改善。
Normal Pressure Hydrocephalus (NPH) is a potentially treatable syndrome with abnormal cerebrospinal fluid dynamics. Meningeal fibrosis and/or obliteration of the subarachnoid space have been suggested as one of the patho-anatomical substrates. However, other types of adult onset dementia, predominantly Alzheimer's disease and Vascular Demential may mimic the clinical NPH characteristics.The purpose of the present study was to correlate cerebral parenchymal and leptomeningeal biopsy findings to the clinical outcome after CSF shunting in a prospective group of idiopathic NPH (INPH) patients. The study comprises 27 patients with INPH, diagnosed and shunted according to generally accepted clinical, imaging and hydrodynamic criteria. In all patients a frontal leptomeningeal and brain biopsy was obtained prior to the shunt insertion.Degenerative cerebral changes, most often Alzheimer (6 cases) or vascular changes (7 cases) were described in 14 out of 27 biopsies. Arachnoid fibrosis was found in 9 of the 18 biopsies containing arachnoid tissue. Overall, nine patients (33%) improved, of whom 6 presented Alzheimer or vascular changes in their biopsies. No correlation was found between clinical outcome and the presence or absence of degenerative cerebral changes and/or arachnoid fibrosis. However, a tendency towards higher improvement rates was noted in the subgroups presenting degenerative cerebral changes or arachnoid fibrosis. The results suggest that no constant morphological element exists in the syndrome of INPH. Various aetiologies may be involved in the pathogenesis and possibly in some cases co-existing: Patients may also improve by shunting despite the presence of degenerative cerebral parenchymal changes.