Patel Ghanshyam, Sakiri Ahmet, Brown Abby, Pasha Arfa, Bansal Vibhav
Mercyhealth, Internal Medicine Residency, Rockford, IL, United States.
Department of Medicine, University of Illinois College of Medicine, Rockford, IL, United States.
Front Neurol. 2024 Mar 27;15:1355437. doi: 10.3389/fneur.2024.1355437. eCollection 2024.
Voltage gated calcium channels (VGCCs) play a critical role in neural transmission. Antibodies that target these ion channels can disrupt cellular signal transmission resulting in various clinical presentations. VGCC antibodies are most commonly associated with paraneoplastic syndromes such as Lambert-Eatons myasthenic syndrome. Here, we report a 47-year-old female with Stage IV appendiceal adenocarcinoma status post appendectomy and right hemicolectomy, who presented with progressive memory impairment, aphasia, ataxia, weakness, and headache. Neurologic exam was notable for right-sided parietal drift, decreased right arm swing, and ataxia of the bilateral upper extremities, more prominent on the right side. MRI of the brain with and without contrast was unremarkable. Cerebrospinal fluid (CSF) was notable for an elevated myelin basic protein (4.9 ng/mL, normal reference 0.0-3.7 ng/mL) with normal cell count, flow cytometry, and cytology. An extensive serum autoimmune neurology antibody evaluation revealed elevated VGCC autoantibodies (observed value: 96.1 pmol/L, normal range 0.0-30.0 pmol/L). A diagnosis of paraneoplastic voltage gated calcium channel antibodies secondary to appendiceal adenocarcinoma was made. The patient was treated with five exchanges with plasmapheresis over 10 days with significant clinical improvement in her symptoms. Upon literature review, this would be the first reported case of VGCC antibodies associated with appendiceal adenocarcinoma.
电压门控钙通道（VGCCs）在神经传递中起关键作用。靶向这些离子通道的抗体可破坏细胞信号传递，导致各种临床表现。VGCC抗体最常与副肿瘤综合征相关，如兰伯特 - 伊顿肌无力综合征。在此，我们报告一名47岁女性，她在阑尾切除术后和右半结肠切除术后处于阑尾腺癌IV期，出现进行性记忆障碍、失语、共济失调、虚弱和头痛。神经系统检查发现右侧顶叶漂移、右臂摆动减少以及双侧上肢共济失调，右侧更明显。脑部增强和未增强MRI均无异常。脑脊液（CSF）中髓鞘碱性蛋白升高（4.9 ng/mL，正常参考值0.0 - 3.7 ng/mL），细胞计数、流式细胞术和细胞学检查正常。广泛的血清自身免疫性神经病抗体评估显示VGCC自身抗体升高（观察值：96.1 pmol/L，正常范围0.0 - 30.0 pmol/L）。诊断为阑尾腺癌继发的副肿瘤性电压门控钙通道抗体。患者在10天内接受了5次血浆置换治疗，症状有显著临床改善。经文献检索，这将是首例报道的与阑尾腺癌相关的VGCC抗体病例。
