Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Semin Neurol. 2010 Feb;30(1):38-43. doi: 10.1055/s-0029-1244993. Epub 2010 Feb 1.
Both general neurologists and neurologists with a broad spectrum of subspecialty interests are often asked to evaluate patients with disorders of the spinal cord. Over the past decade, there have been significant advances in our understanding of a wide spectrum of immune-mediated, infectious, metabolic, hereditary, paraneoplastic, and compressive myelopathies. Advances have been made in the classification and management of spinal vascular malformations. Aortic reconstruction surgery has led to an increased incidence of spinal cord stroke. It is important to recognize a dural arteriovenous fistula as a cause of progressive myelopathy. In the past, noninfectious inflammatory myelopathies have frequently been categorized as idiopathic transverse myelitis. Advances in neuroimaging and discovery of a serum antibody marker, neuromyelitis optica-immunoglobulin G (NMO-IgG), have allowed more specific diagnoses, such as multiple sclerosis and neuromyelitis optica. Abnormalities suggestive of demyelinating disease on brain magnetic resonance imaging (MRI) are known to be highly predictive of conversion to multiple sclerosis in a patient who presents with a transverse myelitis ("clinically isolated syndrome"). Acquired copper deficiency can cause a clinical picture that mimics the subacute combined degeneration seen with vitamin B (12) deficiency. A history of bariatric surgery is commonly noted in patients with copper deficiency myelopathy. Genetics has advanced our understanding of the complex field of hereditary myelopathies. Three hereditary myelopathy phenotypes are recognized: predominantly cerebellar (e.g., Friedreich's ataxia), predominantly motor (e.g., hereditary spastic paraparesis), and a leukodystrophy phenotype (e.g., adrenomyeloneuropathy). Evaluation of myelopathies when no abnormalities are seen on spinal cord imaging is a commonly encountered diagnostic challenge. This article presents some "clinical pearls" in the evaluation and management of spinal cord diseases in context of these recent developments.
无论是普通神经科医生还是具有广泛亚专科兴趣的神经科医生，经常都会被要求评估脊髓疾病患者。在过去的十年中，我们对广泛的免疫介导、感染、代谢、遗传、副肿瘤和压迫性脊髓病有了重大的认识进展。脊髓血管畸形的分类和管理也取得了进展。主动脉重建手术导致脊髓卒中的发病率增加。认识到硬脑膜动静脉瘘是进行性脊髓病的一个原因很重要。过去，非传染性炎症性脊髓病经常被归类为特发性横贯性脊髓炎。神经影像学的进步和血清抗体标志物——视神经脊髓炎免疫球蛋白 G（NMO-IgG）的发现，使得更具体的诊断成为可能，如多发性硬化和视神经脊髓炎。磁共振成像（MRI）上提示脱髓鞘疾病的异常，已知在出现横贯性脊髓炎（“临床孤立综合征”）的患者中，对多发性硬化的转化具有高度预测性。获得性铜缺乏可引起类似于维生素 B（12）缺乏引起的亚急性联合变性的临床表现。获得性铜缺乏性脊髓病患者常有减肥手术史。遗传学使我们对遗传性脊髓病这一复杂领域的认识有了进步。三种遗传性脊髓病表型已被识别：主要是小脑（如弗里德里希共济失调）、主要是运动（如遗传性痉挛性截瘫）和白质营养不良表型（如肾上腺脑白质营养不良）。在脊髓成像未见异常的情况下评估脊髓病是一种常见的诊断挑战。本文介绍了在这些新进展背景下评估和管理脊髓疾病的一些“临床要点”。
