Department of Neurology, Hospital Universitario Virgen de La Victoria, Malaga, Spain.
Instituto de Investigación Biomédica de Málaga (IBIMA), University of Málaga, Malaga, Spain.
Neurol Sci. 2022 Aug;43(8):4841-4845. doi: 10.1007/s10072-022-06075-y. Epub 2022 Apr 28.
Autoimmune autonomic ganglionopathy is a rare, immune-mediated disorder associated with anti-ganglionic α3-subunit nicotinic acetylcholine receptor (anti-α3gAChR) antibodies, which bind to acetylcholine receptor in autonomic ganglia (parasympathetic and sympathetic) leading to autonomic failure. This disorder is mostly associated with viral infections, but it can also be associated with systemic malignancies. Here, we report the case of a paraneoplastic autonomic ganglionopathy as the first symptom of bladder cancer.
Case report.
A 47-year-old man, without medical history of interest, stated to the emergency department for progressive blurry vision with eye and mouth dryness, constipation, and dizziness upon standing for the last 2 weeks. Orthostatic hypotension was demonstrated by a drop in 13.3 mmHg mean blood pressure (BP) from supine (100/60 mmHg) to 45° reclining sitting position (80/50 mmHg). Blood tests, chest X-ray, brain MRI, and electroneuronography were unremarkable. Electrochemical skin conductance was reduced. Serological examination was positive for anti-α3gAChR antibodies. A full-body CT scan revealed a bladder tumor, which was treated by transurethral bladder resection. The pathologic study demonstrated a low-grade non-muscle-invasive bladder urothelial carcinoma. After tumor resection, and treatment with intravenous immunoglobulins and corticoids, a gradually improvement was observed. Today, the patient remains asymptomatic.
Subacute panautonomic failure can be the first symptom for systemic malignancies. This case reports a paraneoplastic autonomic ganglionopathy as the first symptom of bladder cancer. This case highlights the importance of a systemic study to rule out the presence of cancer when autoimmune autonomic ganglionopathy is present.
自身免疫性自主神经节病是一种罕见的免疫介导疾病,与抗神经节α3 亚单位烟碱型乙酰胆碱受体(抗-α3gAChR)抗体有关,该抗体与自主神经节(副交感和交感)中的乙酰胆碱受体结合,导致自主神经衰竭。这种疾病主要与病毒感染有关,但也可能与全身恶性肿瘤有关。在这里,我们报告了一例副肿瘤性自主神经节病作为膀胱癌的首发症状。
病例报告。
一名 47 岁男性,无相关病史,因渐进性视力模糊伴口干、眼干、便秘和直立时头晕 2 周来就诊于急诊科。体位性低血压表现为从仰卧位(100/60mmHg)到 45°斜卧位(80/50mmHg)平均血压下降 13.3mmHg。血液检查、胸部 X 线、脑部 MRI 和电神经图无明显异常。电化学皮肤传导降低。血清学检查抗-α3gAChR 抗体阳性。全身 CT 扫描发现膀胱肿瘤,经经尿道膀胱切除术治疗。病理研究显示低级别非肌肉浸润性膀胱尿路上皮癌。肿瘤切除后,静脉注射免疫球蛋白和皮质激素治疗后,病情逐渐好转。目前,患者无症状。
亚急性全自主神经衰竭可能是全身恶性肿瘤的首发症状。本病例报告了一例副肿瘤性自主神经节病作为膀胱癌的首发症状。该病例强调了在自身免疫性自主神经节病存在时,进行全身研究以排除癌症的重要性。