吉兰-巴雷综合征样亚急性脊髓联合变性及与胃肠道神经内分泌肿瘤的关系

D. Menon, H. Abhilash, V. Vivek
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摘要

亚急性合并变性(SCD)是指逐渐进行性脊髓病或髓神经病变表现为维生素B12缺乏。虽然多神经病变已被广泛认为与维生素B12缺乏症有关,但神经病变是否可以在没有脊髓病的情况下单独表现一直存在争议。SCD很少表现为格林-巴利综合征(GBS)的临床或电生理模拟。我们描述的情况下,一位中年绅士谁提出了两周的快速进展的感觉为主神经病综合征与传导结果一致脱髓鞘过程。鉴于某些临床电差异,患者进一步MRI成像显示SCD的典型特征,维生素B12水平几乎无法检测到。作为评估的一部分,上消化道内窥镜检查显示胃内息肉,组织病理学诊断为WHO II型神经胃内分泌肿瘤(类癌肿瘤)并慢性萎缩性胃炎。据我们所知,类胃癌与SCD之间的关系尚未见报道。我们还讨论了SCD这种gbs样表现的相关临床要点以及与类癌相关的可能病理机制。经肠外补充维生素B12后,患者症状几乎完全缓解,传导参数改善。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Guillain Barre Syndrome-like Presentation of Subacute Combined Degeneration of Spinal Cord and Association with Neuroendocrine Tumour of GIT
Subacute combined degeneration (SCD) refers to the gradually progressive myelopathic or myeloneuropathic presentation of vitamin B12 deficiency. While polyneuropathy has been well recognised with vitamin B12 deficiency, it has been debated whether neuropathy can manifest in isolation without myelopathy. SCD would seldom pose as a clinical or electrophysiological mimicker of Guillain-Barre syndrome (GBS). We describe the case of a middle age gentleman who presented with two weeks of rapidly progressive sensory predominant neuropathic syndrome with conduction findings consistent with a demyelinating process. In view of certain clinical-electrical discrepancies patient was further imaged with MRI revealing features classical of SCD with nearly undetectable Vitamin B12 levels. Upper gastrointestinal endoscopy as part of evaluation revealed a polyp in body of stomach with histopathological diagnosis of WHO II neurogastroendocrine tumour (carcinoid tumour) along with chronic atrophic gastritis. As far as to our knowledge, the association between gastric carcinoid and SCD have not been reported. We also discuss the relevant clinical points of this GBS-like presentation of SCD and possible pathomechanisms of the association with carcinoid. With parenteral Vitamin B12 supplementation patient had near complete resolution of symptoms and improvement in conduction parameters on follow-up.
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