The natural history and long-term outcome of 57 limb sarcoidosis neuropathy cases
The natural history and long-term outcome of 57 limb sarcoidosis neuropathy cases
复制标题
DOI：
10.1016/j.jns.2006.01.014
复制
发表时间：
2006-05-15
影响因子：
4.4
通讯作者：
Dyck, P. J.
中科院分区：
文献类型：
--
作者：
Burns, T. M.;Dyck, P. J. B.;Dyck, P. J.
回顾性分析了57例经活检证实的结节病引起肢体神经病变的患者，以描述肢体结节病神经病变的特征性症状、损害、残疾、病程、结局和对皮质类固醇治疗的反应。通常，神经病变有明确的症状发作日期。突出的是阳性神经病性感觉症状（P-NSS），特别是疼痛，掩盖虚弱和感觉丧失。P-NSS是致残的主要原因。几乎总是模式是不对称的，而不是长度依赖性（不像远端多发性神经病）。我们推断（从症状，体征和电生理和其他测试结果的种类和分布），病理过程是局灶性或多灶性的，涉及大多数类别的神经纤维和可变水平的近端到远端水平的根和周围神经。辅助诊断的其他特征包括：全身症状，如疲劳、不适、关节痛、发热和体重减轻;累及多个组织（即皮肤、淋巴结和眼睛）;神经病变模式; MRI特征;以及最终的组织诊断。轴突变性占主导地位，虽然在3例患者中观察到获得性脱髓鞘过程。在大多数情况下，这种疾病有一个慢性，单相过程。在以后的几年里，对受影响的神经结构进行MRI研究有助于识别软脑膜增厚、肺门腺病、神经根、神经丛和肢体神经的增大和T-2增强。糖皮质激素治疗似乎改善症状超过损害。有几个变量与神经病变的改善相关：CSF细胞增多，症状发作和治疗之间的持续时间短，首次评估时残疾程度较高-这可能是未来早期诊断和治疗的理由。(c)2006 Elsevier B.V.保留所有权利。
Fifty-seven patients with biopsy-proven sarcoidosis causing limb neuropathy were reviewed in order to delineate the characteristic symptoms, impairments, disability, course, outcome and response to corticosteroid treatment of limb sarcoid neuropathy. Typically the neuropathy had a definite date of symptomatic onset. Prominent were positive neuropathic sensory symptoms (P-NSS), especially pain, overshadowing weakness and sensory loss. P-NSS were the main cause of disability. Almost always the pattern was asymmetric and not length-dependent (unlike distal polyneuropathy). We inferred (from kind and distribution of symptoms, signs and electrophysiologic and other test results) that the pathologic process was focal or multifocal, involving most classes of nerve fibers and variable levels of proximal to distal levels of roots and peripheral nerves. Additional features aiding in diagnosis were: systemic symptoms such as fatigue, malaise, arthralgia, fever and weight loss; involvement of multiple tissues (i.e. skin, lymph nodes and eye); the patterns of neuropathy; MRI features; and ultimately tissue diagnosis. Axonal degeneration predominated, although an acquired demyelinating process was observed in 3 patients. For most cases, the disease had a chronic, monophasic course. MRI studies done in later years of affected neural structures were helpful in identifying leptomeningeal thickening, hilar adenopathy; and enlargement and T-2 enhancement of nerve roots, plexuses, and limb nerves. Corticosteroid treatment appeared to ameliorate symptoms more than impairments. Several variables were associated with neuropathic improvement: CSF pleocytosis, short duration between symptom onset and treatment, and a higher grade of disability at first evaluation-a possible rationale for future earlier diagnosis and treatment. (c) 2006 Elsevier B.V. All rights reserved.