Westhout Franklin D, Linskey Mark E
Department of Neurological Surgery, University of California Irvine School of Medicine, Orange, CA 92868, USA.
Surg Neurol. 2008 Mar;69(3):288-92; discussion 292. doi: 10.1016/j.surneu.2007.01.068. Epub 2007 Oct 31.
Neurosarcoidosis presents with meningitis, cranial nerve involvement, and parenchymal masses. Usually, abnormal MR enhancement and/or structural lesion(s) are present. Communicating hydrocephalus arises from meningeal arachnoid granulation involvement. Reported cases of obstructive hydrocephalus have all involved obstructing ventricular lesions.
A 40-year-old African American man presented with papilledema, diplopia, and headache. Magnetic resonance imaging revealed "aqueductal stenosis" without abnormal enhancement or obstructive lesion. Although symptoms resolved with shunting, he soon developed progressive psychotic symptoms. Serial MR scans remained free of abnormal enhancement or structural mass lesion(s) but revealed long repetition time and proton density signal changes within the medial temporal lobe structures and deep white matter that remained stable over 8 months despite clinical progression. Serial CSF studies were nondiagnostic. Open temporal lobe brain and meningeal biopsy revealed noncaseating granulomas within the parenchyma and meninges consistent with sarcoid. Total-body computed tomography scans ruled out systemic sarcoid. The patient steadily improved on steroid therapy.
Neurosarcoid psychiatric symptoms are usually associated with diffuse meningeal enhancement from meningitis. Our case reveals that absence of abnormal enhancement or structural mass lesion on MR and normal CSF ACE levels do not rule out neurosarcoid. Based on a patent cerebral aqueduct and the T2 MR and pathology findings, we postulate that altered brain tissue compliance with impairment of normal pulsatile augmentation of aqueduct CSF flow was the likely cause of obstructive hydrocephalus. This represents a very rare psychiatric presentation and reports a new potential mechanism for the development of hydrocephalus with neurosarcoid.
神经结节病表现为脑膜炎、脑神经受累及实质肿块。通常存在异常的磁共振增强和/或结构性病变。交通性脑积水源于脑膜蛛网膜颗粒受累。报道的梗阻性脑积水病例均涉及阻塞性脑室病变。
一名40岁非裔美国男性出现视乳头水肿、复视和头痛。磁共振成像显示“导水管狭窄”，无异常强化或阻塞性病变。尽管分流术后症状缓解，但他很快出现进行性精神症状。系列磁共振扫描始终未发现异常强化或结构性肿块病变，但显示内侧颞叶结构和深部白质内的长重复时间和质子密度信号改变，尽管临床病情进展，但在8个月内保持稳定。系列脑脊液检查未得出诊断结果。开放性颞叶脑和脑膜活检显示实质和脑膜内有非干酪样肉芽肿，符合结节病表现。全身计算机断层扫描排除了系统性结节病。患者接受类固醇治疗后病情稳步改善。
神经结节病的精神症状通常与脑膜炎导致的弥漫性脑膜强化有关。我们的病例表明，磁共振成像上无异常强化或结构性肿块病变以及脑脊液血管紧张素转换酶水平正常并不能排除神经结节病。基于大脑导水管通畅以及磁共振T2加权像和病理结果，我们推测脑组织顺应性改变以及导水管脑脊液流动正常搏动性增强受损可能是梗阻性脑积水的原因。这代表了一种非常罕见的精神症状表现，并报告了神经结节病导致脑积水的一种新的潜在机制。
