[Paralysis of digestive tract with lesions of myenteric plexuses. A new paraneoplastic syndrome (author's transl)].
[Paralysis of digestive tract with lesions of myenteric plexuses. A new paraneoplastic syndrome (author's transl)].
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[消化道麻痹伴肌间神经丛损伤。
DOI：
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发表时间：
1980
期刊：
影响因子：
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通讯作者：
P. Bernard
中科院分区：
文献类型：
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作者：
F. Lhermitte;F. Gray;O. Lyon‐Caen;B. Pertuiset;P. Bernard
一名 67 岁男子于 1979 年 7 月因恶心、吞咽困难和直肠疼痛入院。 64 岁时，他因表皮样癌接受了下唇放射治疗。那时他仍然健康。除慢性支气管炎外，他的病史均为阴性。他从未接触过有毒物质或毒品，也从未离开过欧洲。入院几天后，他出现急性肠梗阻，但剖腹手术未发现病因。同时，患者主诉四肢疼痛，并被发现有弥漫性肌肉萎缩、反射消失和远端感觉丧失。没有出现自主神经功能障碍的迹象。除颈部淋巴结肿大外，体格检查呈阴性。淋巴结活检显示未分化转移癌。阴性检查包括：血细胞计数；血清电离图和免疫电泳；甲状腺功能检查；查加斯病的血清学检测。发现以下异常：ESR：55-105；脑脊液蛋白：145mg/100ml，1细胞mm3；全血叶酸：1.7 mg/ml；血液中存在 Hbs 抗原；肌电图显示去神经支配的证据，但运动传导速度正常。到了九月，患者的虚弱程度加剧，完全性肠梗阻持续存在。食管、胃和十二指肠纤维镜检查未见收缩，活检结果呈阴性。患者于1979年10月5日死于腹膜炎。尸检发现继发于大肠多发性穿孔的腹膜炎。未发现唇癌复发或转移或原发癌的证据。光学显微镜检查没有显示淀粉样变性或硬皮病的证据。消化道检查显示异常仅限于肌间神经丛，从一个层面到另一层面都有所不同。在小肠中，平滑肌增生，并且施万细胞显着增殖，导致肌间神经丛扩大。还注意到严重的神经元损失和 Nageotte 结节。雪旺细胞增殖在胃和大肠中不太明显。食道、胃、大肠和直肠中存在严格局限于肌间神经丛区域的淋巴组织细胞浸润。脊髓前根、后根及半月神经节也可见轻度慢性炎性病变。该病例的显着特征是未分化癌和多发性神经根神经炎与快速发作的完全消化道麻痹相关，继发于肌间丛病变。叶酸水平低不足以解释神经病变。调查显示没有证据表明假性肠梗阻的常见原因：肌肉性、自主神经功能障碍、中毒性、神经丛性（特发性、家族性、炎症性）、南美锥虫病。临床病程、消化道和脊髓根的病理图片以及与癌症的关联表明我们的病例可能代表副肿瘤综合征......
A 67 years old man was admitted on July 1979 for nausea, dysphagia and rectal pain. At age 64 he had undergone radiotherapy on the lower lip for an epidermoid carcinoma. He remained then healthy. His medical history was negative with the exception of chronic bronchitis. He had never been exposed to toxic agents or drugs and had never left Europe. A few days after admission he suffered acute intestinal obstruction but at laparostomy no etiology was found. At the same time the patient complained of pain in all four limbs and he was found to have diffuse wasting of muscles, areflexia and distal sensory loss. No sign of dysautonomia was present. Physical examination was negative with the exception of a cervical lymphadenopathy. The lymph node biopsy showed an undifferentiated metastatic carcinoma. Negative investigations included: blood cells count; serum ionogram and immunoelectrophoresis; thyroid function tests; serological test for Chagas' disease. The following abnormalities were found: ESR: 55-105; CSF protein: 145 mg/100 ml and 1 cell mm3; whole blood folic acid: 1,7 mg/ml; Hbs antigen was present in blood; EMG showed evidence of denervation but motor conduction velocities were normal. By September the patient's weakness had increased and complete intestinal obstruction persisted. At oesophageal, gastric and duodenal fibroscopy no contraction was visible, and biopsies were negative. The patient died of peritonitis on October 5th, 1979. At necropsy peritonitis secondary to multiple perforation of the large bowel was found. No recurrence of the lip carcinoma or metastase or evidence of a primary carcinoma was found. Light microscopy showed no evidence of amyloidosis or scleroderma. Examination of the alimentary tract showed abnormalities restricted to the myenteric plexuses which varied from one level to another. In the small bowel there was hyperplasia of the smooth muscle and the myenteric plexuses were enlarged by marked proliferation of Schwann cells. Severe neuronal loss and nodules of Nageotte were also noted. Schwann cells proliferation was less marked in the stomach and large bowel. Lympho-histiocytic infiltration strictly confined to the region of the myenteric plexuses was present in oesophagus, stomach, large bowel and rectum. Mild chronic inflammatory lesions were also found in anterior and posterior spinal roots and semi-lunar ganglia. The striking feature of this case is the association of an undifferentiated carcinoma and a polyradiculoneuritis with a complete alimentary tract palsy of rapid onset, secondary to lesions restricted to the myenteric plexuses. The low folate level was insufficient to explain the neuropathy. Investigations showed no evidence of the usual causes of intestinal pseudo-obstruction: muscular, dysautonomic, toxic, plexic (idiopathic, familial, inflammatory), Chagas' disease). The clinical course, the pathological pictures of the alimentary tract and spinal roots and the association with a carcinoma suggest that our case may represent a paraneoplastic syndrome...