副肿瘤性/自身免疫性骨髓病。

Mayra Montalvo, Eoin P Flanagan
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引用次数: 0

摘要

副肿瘤性骨髓病是一类罕见但重要的骨髓病。它们通常表现为隐匿性或亚急性进行性神经系统综合征。危险因素包括吸烟和癌症家族史。脑脊液分析通常显示淋巴细胞增多,蛋白质升高。核磁共振成像结果表明,副肿瘤性骨髓病包括纵向广泛的T2高密度,具有束特异性并伴有增强,但脊髓核磁共振成像也可正常。最常见的相关神经抗体包括虹膜蛋白抗体和塌缩素-反应介质-蛋白-5(CRMP5/抗-CV2)抗体,肺癌和乳腺癌是最常见的肿瘤伴随物。副肿瘤性骨髓病的鉴别诊断包括营养缺乏性骨髓病(B12、铜)以及自身免疫/炎症性疾病,如原发性进行性多发性硬化症或脊髓肉样瘤病。接受免疫检查点抑制剂治疗的癌症患者可能会患上骨髓炎,这可被视为副肿瘤性骨髓病的一种。副肿瘤性骨髓病的治疗包括肿瘤治疗和免疫治疗。尽管采取了这些治疗方法,但预后不良,大多数患者最终会依赖轮椅。
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Paraneoplastic/autoimmune myelopathies.

Paraneoplastic myelopathies are a rare but important category of myelopathy. They usually present with an insidious or subacute progressive neurologic syndrome. Risk factors include tobacco use and family history of cancer. Cerebrospinal fluid analysis usually shows lymphocytic pleocytosis with elevated protein. MRI findings suggest that paraneoplastic myelopathies include longitudinally extensive T2 hyperintensities that are tract-specific and accompanied by enhancement, but spinal MRIs can also be normal. The most commonly associated neural antibodies include amphiphysin and collapsin-response-mediator-protein-5 (CRMP5/anti-CV2) antibodies with lung and breast cancers being the most frequent oncologic accompaniments. The differential diagnosis of paraneoplastic myelopathies includes nutritional deficiency myelopathy (B12, copper) as well as autoimmune/inflammatory conditions such as primary progressive multiple sclerosis or spinal cord sarcoidosis. Patients treated with immune checkpoint inhibitors for cancer may develop myelitis, that can be considered along the spectrum of paraneoplastic myelopathies. Management of paraneoplastic myelopathy includes oncologic treatment and immunotherapy. Despite these treatments, the prognosis is poor and the majority of patients eventually become wheelchair-dependent.

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来源期刊
Handbook of clinical neurology
Handbook of clinical neurology Medicine-Neurology (clinical)
CiteScore
4.10
自引率
0.00%
发文量
302
期刊介绍: The Handbook of Clinical Neurology (HCN) was originally conceived and edited by Pierre Vinken and George Bruyn as a prestigious, multivolume reference work that would cover all the disorders encountered by clinicians and researchers engaged in neurology and allied fields. The first series of the Handbook (Volumes 1-44) was published between 1968 and 1982 and was followed by a second series (Volumes 45-78), guided by the same editors, which concluded in 2002. By that time, the Handbook had come to represent one of the largest scientific works ever published. In 2002, Professors Michael J. Aminoff, François Boller, and Dick F. Swaab took on the responsibility of supervising the third (current) series, the first volumes of which published in 2003. They have designed this series to encompass both clinical neurology and also the basic and clinical neurosciences that are its underpinning. Given the enormity and complexity of the accumulating literature, it is almost impossible to keep abreast of developments in the field, thus providing the raison d''être for the series. The series will thus appeal to clinicians and investigators alike, providing to each an added dimension. Now, more than 140 volumes after it began, the Handbook of Clinical Neurology series has an unparalleled reputation for providing the latest information on fundamental research on the operation of the nervous system in health and disease, comprehensive clinical information on neurological and related disorders, and up-to-date treatment protocols.
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