Immunohistochemical study on gastroenteric nervous system in trisomy 16 mice:an animal model of Down syndrome

AIM To study the development of gastroentericnervous system in trisomy 16 mouse embryos.The gastroenteric nervous system in trisomy 16mice and their normal littermates,serving ascontrols from embryonic days 13 to 18(ED13-18)was identified by using primary antibody againstprotein gene product(PGP)9.5.METHODS Trisomy 16 mouse breeding andtrisomy 16 mouse embryos were identified fromtheir normal littermates by chromosomeexamination;PGP 9.5 immunohistochemicalstainning.CONCLUSION Trisomy 16 mice, as an animal model for Down syndrome, has abnormality not only in several systems and organs but also in gastroenteric innervation. This report describes for the first time that the development of the gastroenteric nervous system was not only delayed but also pathological.

Two uncommon manifestations of leptospirosis:Sweet's syndrome and central nervous system vasculitis

To leptospirosis is the commonest spirocheatal infection in the tropical and temperate countries of Indian sub-continent and Africa and the most common zoonosis worldwide.The protean manifestation of this infectious disease is a challenge for practising clinicians across the world. In poor developing countries,at most clinical suspicion it is essential in the diagnosis of this disease.In this report,we are able to document two uncommon manifestations of leptospirosis, namely Sweet’s syndrome and central nervous system vasculitis.

Acute pancreatitis with hypercalcemia caused by primary hyperparathyroidism associated with paraneoplastic syndrome:A case report and review of literature

BACKGROUND Although acute pancreatitis associated with hyperparathyroidism has occasionally been reported,acute pancreatitis with metabolic encephalopathy caused by hyperparathyroidism combined with paraneoplastic syndrome is an extremely rare entity and poorly described in the literature.CASE SUMMARY We present a case of a 56-year-old female with upper abdominal discomfort and intermittent nausea and vomiting for 1 wk,without apparent abdominal pain or bloating,no jaundice and decreased blood pressure at the outset.The patient was ultimately diagnosed with moderately severe acute pancreatitis(according to the revised Atlanta classification of acute pancreatitis)combined with metabolic encephalopathy secondary to hypercalcemia caused by primary hyperparathyroidism associated with paraneoplastic syndrome.After active treatment of acute pancreatitis,massive fluid resuscitation,resection of parathyroid and uterine malignant tumors,neoadjuvant chemotherapy and other treatments,her serum calcium eventually returned to the normal level.The patient was successfully discharged from hospital.CONCLUSION This is the first case of acute pancreatitis caused by primary hyperparathyroidism associated with paraneoplastic syndrome.

Paraneoplastic syndromes in cholangiocarcinoma

Paraneoplastic syndromes are the symptoms or signs which result from damage to tissues that are distant from the site of malignancy,due to complex interactions between the body’s immune system and malignant neoplasm.Cholangiocarcinoma(CCA)is an aggressive epithelial malignancy of hepatobiliary tree and it is found to be associated with various paraneoplastic syndromes.These syndromes can present as dermatological,neurological,renal,hematological,or multi-systemic manifestations.Clinical suspicion and timely recognition of these syndromes can lead to early diagnosis of covert malignancies like CCA.The management plan remains the removal of the underlying cause which in this case is CCA.

Refractory diarrhea: A paraneoplastic syndrome of neuroblastoma

Neuroblastoma(NB) is the most common extracranial solid tumor in children. Diarrheal NB is quite rare and is not easy to diagnose in the early stage. Six cases of diarrheal NB in our hospital treated from 1996 to 2006 were retrospectively analyzed, including characteristics such as electrolyte imbalance, pathologic features, vasoactive intestinal peptide(VIP) immunohistochemical staining results, treatment, and prognosis. All patients were boys with 3-8 loose or watery stools each day and routine fecal tests were normal. Abdominal tumors were identified by B-ultrasound. Drugs were ineffective. Three patients underwent surgery, and the remaining three patients received surgery and chemotherapy. Diarrhea stopped after treatment in five patients. Two patients died due to intractable hypokalemia. The tumor was located in the adrenal gland in four patients, in the upper retroperitoneum in one patient, and in the presacral area in one patient. Pathologic findings were NB and ganglioneuroblastoma. Five patients were at clinical stage Ⅰ-Ⅱ, and one was at stage Ⅲ. Four patients survived(followed-up for 6 mo to 4 years). Immunohistochemical staining for VIP was positive. Refractory diarrhea is a paraneoplastic syndrome of NB and is rare. Patients aged 1-3 years who present with chronic intractable diarrhea should be followed closely. Intractable diarrhea, hypokalemia, and dysplasia are the initial clinical manifestations. Increased VIP is characteristic of this disease. Potassium supplementation plays a vital role in the treatment procedure, especially preoperatively. The prognosis of diarrheal NB is good following appropriate treatment.

Paraneoplastic syndromes associated with lung cancer

Paraneoplastic syndromes are signs or symptoms that occur as a result of organ or tissue damage at locations remote from the site of the primary tumor or metastases. Paraneoplastic syndromes associated with lung cancer can impair various organ functions and include neurologic, endocrine, dermatologic, rheumatologic, hematologic, and ophthalmological syndromes, as well as glomerulopathy and coagulopathy(Trousseau's syndrome). The histological type of lung cancer is generally dependent on the associated syndrome, the two most common of which are humoral hypercalcemia of malignancy in squamous cell carcinoma and the syndrome of inappropriate antidiuretic hormone secretion in small cell lung cancer. The symptoms often precede the diagnosis of the associated lung cancer, especially when the symptoms are neurologic or dermatologic. The proposed mechanisms of paraneoplastic processes include the aberrant release of humoral mediators, such as hormones and hormone-like peptides, cyto-kines, and antibodies. Treating the underlying cancer is generally the most effective therapy for paraneoplastic syndromes, and treatment soon after symptom onset appears to offer the best potential for symptom improvement. In this article, we review the diagnosis, potential mechanisms, and treatments of a wide variety of paraneoplastic syndromes associated with lung cancer.

Recurrent Hypoglycaemia in a Diabetic Patient Presented a Gastric Cancer: Think about Paraneoplastic Syndrome

The hypoglycaemia paraneoplastic is uncommon in diabetic patients. Our aim was to report a case of paraneoplastic hypoglycaemia discovered after the diagnosis of gastric adenocarcinoma in a diabetic patient. A 65 years old man, was hospitalized for the management of acute digestive haemorrhage. In his medical history, he had diabetes since 20 years. He was alcoholic weaned for 3 years and had an unexplored repetitive epigastralgia. During his hospitalization, the exploration of acute digestive haemorrhage found a gastric adenocarcinoma with hepatic metastasis. At the 4th day of his hospitalization, he presented a hypoglycaemic coma with capillary blood glucose at 0.36 g/L. The patient was conscious after the infusion of Hypertonic Glucose Solution (HGS) 10%. After 3 days, the recurrent hypoglycaemia occurred and we intermittently treated by infusion of glucose solution 10%. The measurements of insulin level and C-peptide weren’t performed because they didn’t exist in the city. So, the notion of cancer and the recurrent hypoglycaemia even if we stopped the oral antidiabetic therapy make us think of a paraneoplastic syndrome. The chemotherapy associated with corticosteroid therapy is the standard treatment of the hypoglycaemia paraneoplastic but the patient refused the chemotherapy and we couldn’t use the corticosteroid therapy because of digestive haemorrhage. At the end of his hospitalization, he still had hypoglycaemia treated by infusion of glucose solution 10%. In the absence of means of diagnosis, we should think of paraneoplastic syndrome in front of recurrent hypoglycaemia and cancer in the diabetic patient.

Paraneoplastic Syndromes of Hypercalcemia and Leukocytosis Associated with Colonic Metastases from Squamous Cell Carcinoma of the Lung: a Case Report

Lung cancer is the most common cancer-related death in both men and women in the world. Approximately 25% of all cancer deaths are attributable to lung carcinoma. Moreover, about one-half of patients with lung cancer have metastases at the time of initial diagnosis, most frequently of lymph nodes, adrenals, liver, bone and brain.

An overview on CV2/CRMP5 antibody-associated paraneoplastic neurological syndromes

Paraneoplastic neurological syndrome refers to certain malignant tumors that have affected the distant nervous system and caused corresponding dysfunction in the absence of tumor metastasis.Patients with this syndrome produce multiple antibodies,each targeting a different antigen and causing different symptoms and signs.The CV2/collapsin response mediator protein 5(CRMP5)antibody is a major antibody of this type.It damages the nervous system,which often manifests as limbic encephalitis,chorea,ocular manifestation,cerebellar ataxia,myelopathy,and peripheral neuropathy.Detecting CV2/CRMP5 antibody is crucial for the clinical diagnosis of paraneoplastic neurological syndrome,and anti-tumor and immunological therapies can help to alleviate symptoms and improve prognosis.However,because of the low incidence of this disease,few repo rts and no reviews have been published about it so far.This article intends to review the research on CV2/CRMP5antibody-associated paraneoplastic neurological syndrome and summarize its clinical features to help clinicians comprehensively understand the disease.Additionally,this review discusses the curre nt challenges that this disease poses,and the application prospects of new detection and diagnostic techniques in the field of paraneoplastic neurological syndrom e,including CV2/CRMP5-associated paraneoplastic neurological syndrome,in recent years.

Metastatic gastric cancer from breast carcinoma presenting with paraneoplastic rheumatic syndrome:A case report

BACKGROUND Breast cancer is the most frequently diagnosed cancer worldwide.It is the leading cause of death by malignant disease in women.CASE SUMMARY A female patient,73 years of age,sought care due to weakness,mild abdominal pain,arthralgia,and weight loss.She was taking anastrazole as maintenance therapy for localized breast cancer and had moderate anemia and elevated acutephase markers.Upper digestive endoscopy showed isolated erosion in the gastric corpus.This lesion was compatible with signet-ring cell adenocarcinoma in anatomopathological study and was confirmed as metastasis of a breast carcinoma in immunohistochemistry,which was positive for estrogen antibody.Further imaging studies determined numerous proximal bone metastases.The patient was treated with prednisone for paraneoplastic syndrome,which improved the anemia and rheumatic disease,and with chemotherapy,which greatly improved the symptoms.She has been followed-up for 6 mo,and her anemia,arthralgias,and acute phase markers have normalized.CONCLUSION Systemic treatment strategies seem to be the best choice for gastric metastasis from breast cancer,resulting in disease control and relapse-free survival.Prospective studies with longer follow-up are needed to better understand the biological,pathological,and clinicopathological characteristics and outcomes of the endoscopic features associated with metastatic gastric cancer from breast carcinoma.

Analysis of metabolic characteristics of metabolic syndrome in elderly patients with gastric cancer by non-targeted metabolomics

BACKGROUND The relationship between metabolic syndrome(MetS)and gastric cancer(GC),which is a common metabolic disease,has attracted much attention.However,the specific metabolic characteristics of MetS in elderly patients with GC remain unclear.AIM To investigate the differentially abundant metabolites and metabolic pathways between preoperative frailty and MetS in elderly patients with GC based on nontargeted metabolomics techniques.METHODS In this study,125 patients with nonfrail nonmeal GC were selected as the control group,and 50 patients with GC in the frail group were selected as the frail group.Sixty-five patients with GC combined with MetS alone were included in the MetS group,and 50 patients with GC combined with MetS were included in the MetS group.Nontargeted metabolomics techniques were used to measure plasma metabolite levels by ultrahigh-performance liquid chromatography-mass spectrometry.Multivariate statistical analysis was performed by principal component analysis,orthogonal partial least squares,pattern recognition analysis,cluster analysis,and metabolic pathway annotation.RESULTS A total of 125 different metabolites,including amino acids,glycerophospholipids,sphingolipids,fatty acids,sugars,nucleosides and nucleotides,and acidic compounds,were identified via nontargeted metabolomics techniques.Compared with those in the control group,there were 41,32,and 52 different metabolites in the MetS group,the debilitated group,and the combined group,respectively.Lipid metabolites were significantly increased in the MetS group.In the weak group,amino acids and most glycerol phospholipid metabolites decreased significantly,and fatty acids and sphingosine increased significantly.The combined group was characterized by significantly increased levels of nucleotide metabolites and acidic compounds.The alanine,aspartic acid,and glutamate metabolic pathways were obviously enriched in the asthenic group,and the glycerol and phospholipid metabolic pathways were obviously enriched in the combined group.CONCLUSION Elderly GC patients with simple frailty,simple combined MetS,and frailty combined with MetS have different metabolic characteristics,among which amino acid and glycerophospholipid metabolite levels are significantly lower in frail elderly GC patients,and comprehensive supplementation of fat and protein should be considered.Many kinds of metabolites,such as amino acids,lipids,nucleotides,and acidic compounds,are abnormally abundant in patients with MetS combined with fthenia,which may be related to tumor-related metabolic disorders.

Studies on Plasma Norepinephrine and EpinephrineContents and Vegetative Nervous SystemFunction in Various Liver Syndrome

Objective: To study the characteristics of plasma norepinephrine (NE) and epinephrine (E) contents alternation and vegetative nervous function disturbance in patients with various Liver Syndrome of TCM.Methods: Subjects were selected on the principle of combination of disease diagnosis and Syndrome Differentiation. Plasma NE and E contents were determined by high performance liquid chromatography (HPLC) and electrochemical detector. The vegetative nervous function were assessed by comprehensive analysis of sympathetic and parasympathetic hyperfunction symptoms, physiologic indexes and physical loading tests.Results: Patients of Liver Excess Syndrome had an elevated plasma NE and E contents and those with the vegetative nervous system disturbance mainly hypersympathetic. On the contrary, patients of Liver Deficiency Syndrome had lowered plasma NE and E contents and those with the vegetative nervous system disturbance mainly hyper-parasympathetic.Conclusion: Plasma NE and E contents and vegetative nervous function disturbance can be taken as the auxiliary laboratory diagnostic indexes for Syndrome Differentiation of Liver Excess and Liver Deficiency.

Sweet syndrome as a paraneoplastic manifestation of cholangiocarcinoma:A case report

BACKGROUND Sweet’s syndrome,also known as acute febrile neutrophilic dermatosis,is a rare skin disorder that may be associated with cancer.CASE SUMMARY A 58-year-old female presented with a cholestatic syndrome and significant weight loss three months before admission.Five months earlier,she had abruptly developed skin lesions with erythematous papules that evolved to erythematous blisters.Clinical evaluation and laboratory tests confirmed hepatic cholangiocarcinoma.Skin lesions histopathological findings showed neutrophilic dermatosis,massive edema,fibrin,necrosis,and elastosis.These results,in association with the macroscopic aspects of the findings,led to the diagnosis of paraneoplastic Sweet’s syndrome due to cholangiocarcinoma.As staging was consistent with an advanced tumor without a cure perspective,we opted to perform percutaneous biliary drainage,and subsequently,palliative care.Eventually,after a few weeks,the patient died.CONCLUSION In conclusion,the diagnosis of the underlying disease-causing Sweet’s syndrome must be accurate,and patients need to be followed-up,as neoplasia such as cholangiocarcinoma may be a later manifestation.

Intracranial Hypertension Syndrome in Systemic Lupus Erythematosus: Clinical Analysis and Review of the Literature

In order to better understand the clinical manifestation of systemic lupus erythematosus （SLE） with intracranial hypertension syndrome （IHS）, we analyzed the clinical features and treatment of a typical SLE patient with IHS. SLE is one of the most unpredictable autoimmune diseases involving multiple organ systems that is defined clinically and associated with antibodies directed against cell nuclei. IHS is an uncommon manifestation of neuropsychiatric SLE （NPSLE） and is characterized by an elevated intracranial pressure, papilledema, and headache with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. IHS has been reported in a few sporadic cases in patients with SLE worldwide, but rarely has been reported in China. In this study, a 34-year-old female SLE patient with IHS was. reported and pertinent literature reviewed. The clinical presentation, image logical features, and investigatory findings were discussed.

Paraneoplastic neurological syndrome with positive anti-Hu and anti-Yo antibodies:A case report

BACKGROUND Paraneoplastic neurological syndrome(PNS)is a rare complication in patients with cancer.PNS can affect the central,peripheral,autonomic nervous system,neuromuscular junction,or muscles and cause various neurological symptoms.Anti-Yo antibody-positive neurological paraneoplasms and anti-Hu antibodypositive neurological paraneoplasms are common,but coexistence of both types has not been described in the literature.CASE SUMMARY Here we present a rare case of paraneoplastic neuropathy occurring in both breast and lung cancers.A 55-year-old woman was admitted to our hospital with unsteadiness while walking.The patient had a history of breast cancer two years previously.Chest computed tomography revealed a 4.6 cm×3.6 cm mass in the right lung,which was diagnosed as small-cell lung cancer(SCLC).Blood test was positive for anti-Yo antibodies,and the cerebrospinal fluid was positive for both anti-Yo and anti-Hu antibodies,and the neurological symptoms were considered to be related to the paraneoplasm.The patient was treated with a course of intravenous immunoglobulin,without noticeable improvement.After being discharged from hospital,the patient underwent regular chemotherapy for SCLC and periodic reviews.The patient’s neurological symptoms continued to deteriorate at the follow-up visit in April 2021.CONCLUSION This case suggests the possibility of two types of tumors appearing simultaneously with two paraneoplastic antibodies.The clinical appearance of two or more paraneoplastic tumors requires additional attention.

Carcinoid syndrome caused by a pulmonary carcinoid mimics intestinal manifestations of ulcerative colitis:A case report

BACKGROUND Pulmonary carcinoids are rare,low-grade malignant tumors characterized by neuroendocrine differentiation and relatively indolent clinical behavior.Most cases present as a slow-growing polypoidal mass in the major bronchi leading to hemoptysis and pulmonary infection due to blockage of the distal bronchi.Carcinoid syndrome is a paraneoplastic syndrome caused by the systemic release of vasoactive substances that presents in 5%of patients with neuroendocrine tumors.Due to such nonspecific presentation,most patients are misdiagnosed or diagnosed late and may receive several courses of antibiotics to treat recurrent pneumonia before the tumor is diagnosed.CASE SUMMARY We report the case of a 48-year-old male who presented with cough,dyspnea,a history of recurrent pneumonitis,and therapy-refractory ulcerative colitis that completely subsided after the resection of a pulmonary carcinoid.CONCLUSION We report and emphasize pulmonary carcinoid as a differential diagnosis in patients with nonresponding inflammatory bowel diseases and recurrent pneumonia.

Paraneoplastic neurological syndrome caused by cystitis glandularis: A case report and literature review

BACKGROUND Paraneoplastic neurological syndrome(PNS)is an unusual event.PNS caused by cystitis glandularis(CG)or a bladder tumor is extremely rare;hence,missed diagnosis or misdiagnosis can easily occur.To date,approximately 21 cases have been reported in PubMed.CASE SUMMARY We report a case of PNS caused by CG and describe the clinical and imaging features.The main clinical feature was advanced cognitive impairment,and early clinical features were memory impairment,decreased computational ability,and abnormal behavior.Later clinical features were dementia,vomiting,inability to eat and walk,urinary incontinence,and hematuria.Imaging features on cranial magnetic resonance imaging were diffuse white matter lesions.Paraneoplastic tumor markers were normal.A total abdominal computed tomography scan showed multiple thickened areas on the bladder wall with local prominence.Cystoscopy revealed a volcanic protuberance on the posterior wall of the bladder with a diameter of 6 cm and no pedicle.The postoperative pathological diagnosis was CG.The patient recovered well following resection of CG.PNS cases caused by previous bladder tumors can be retrieved from PubMed to describe the clinical signs and prognosis of PNS.CONCLUSION The main clinical feature of PNS caused by CG was dementia,and the imaging features were diffuse cerebral white matter lesions.Resection of CG lesions is the fundamental treatment for PNS induced by CG.This case highlights the importance of etiological treatment.

Uncharted Territory: Frequent Relapsing, Steroid Sensitive Secondary Minimal Change Nephrotic Syndrome Cause by Solid Tumor of the Gastro-Esophageal Junction —(Case Presentation and Review of the Literature)

We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. Each time the dose of steroid is tapered, a relapse of the nephrotic syndrome occurred. Eventually, the patient was complaining of dysphagia and difficulty swallowing. Hospital work-up with barium swallow, endoscopy, and CT of the chest, abdomen and pelvis, revealed a focal stenotic lesion with mild to moderate esophageal dysmotility 7/15/2022. A diagnosis of an ulcerating lesion with biopsy confirmed a neuro-endocrine carcinoma of the gastro-esophageal junction was entertained. The CT of the chest/abdomen/pelvis, 7/19/2022, has shown, an esophageal mass of 5.1 × 5.6 × 7 cm of the gastro-esophageal junction with ulceration. No evidence of spread beyond the esophagus and stomach. The histology revealed a poorly differentiated neuroendocrine tumor of the gastro-esophageal junction. The patient underwent several rounds of chemotherapy, radiation, and surgery culminating in tumor control. His nephrotic syndrome was resolved after the tumor has been controlled by surgery and chemotherapy.

胸腺瘤相关重症肌无力合并炎性肌病三例报道并文献复习

目的分析重症肌无力(myasthenia gravis,MG)合并炎性肌病(inflammatory myopathy,IM)患者的临床特点及其与胸腺瘤的相关性。方法分析北京医院2010-3-1—2020-3-31收治的3例MG合并IM患者的临床特点,并结合文献进行复习。结果3例MG-IM中1例合并谷氨酸脱羧酶抗体阳性的僵肢综合征(stiff limb syndrome,SLS)。3例患者均有胸腺瘤,血清肌酸激酶和肌酸激酶同工酶升高,横纹肌抗体和心肌抗体阳性,而肌炎特异性抗体(myositis specific antibodies,MSA)和肌炎相关抗体(myositis associated antibodies,MAA)均阴性;肌电图提示肌源性损害和神经肌肉接头受累;心电图和超声心动图提示心肌受累;肌肉病理诊断多发性肌炎。联合46篇文献中的93例患者,共96例MG-IM患者纳入汇总分析。MG与IM同时发生者占42.7%,以MG症状首发者占34.4%,以IM症状首发者占22.9%。EMG检查提示肌源性损害和神经肌肉接头突触后膜疾病。乙酰胆碱受体(acetylcholine receptor,AChR)抗体阳性者占91.3%(84/92),MSA抗体阳性者仅占3.0%(2/66),MAA抗体阳性者仅占6.3%(2/32),横纹肌抗体阳性者占95.2%(20/21),6例行心肌抗体检测者均呈阳性。CT检查发现胸腺瘤者占64.4%(58/90)。53例行胸腺瘤手术的患者中,47例描述了胸腺瘤病理分型,以胸腺瘤B2型最常见(19例,40.4%),其次为B1型10例(21.3%),B3型9例(19.1%),AB型8例(17.0%),C型1例(2.1%)。89例行骨骼肌病理检查,其中以多发性肌炎最常见(62例,69.7%),其次为皮肌炎13例(14.6%),肉芽肿性肌炎9例(10.1%),免疫坏死性肌病2例(2.2%),嗜酸性粒细胞肌炎2例(2.2%),包涵体肌炎1例(1.1%)。7例尸体解剖患者行心肌病理检查,均诊断巨细胞性心肌炎。结论MG-IM多见于胸腺瘤患者,部分MG-IM患者同时合并心肌炎。大多数MG-IM患者血MSA和MAA抗体阴性。血横纹肌抗体和心肌抗体阳性提示骨骼肌和心肌受累,巨细胞性心肌炎是MG-IM患者死亡的主要原因之一。MG、IM和SLS全面准确的诊断依赖于临床、电生理、免疫学和肌肉病理的综合评估。

抗SOX1抗体相关神经系统副肿瘤综合征的临床异质性分析

目的总结抗Y染色体性别决定区相关高迁移率超家族1(SRY⁃like high⁃mobility group superfamily of developmental transcription factors 1,SOX1)抗体相关神经系统副肿瘤综合征(paraneoplastic neurological syndrome,PNS)的临床表现、影像学特征和预后。方法选取2019年1月至2023年1月首都医科大学附属北京友谊医院抗SOX1抗体相关PNS患者6例,回顾性分析患者的相关资料。结果6例患者中,男5例、女1例,年龄42~76岁。6例患者中,感觉运动周围神经病合并小细胞肺癌(small cell lung cancer,SCLC)2例、边缘叶脑炎2例、副肿瘤小脑变性1例和Lam⁃bert-Eaton肌无力综合征合并SCLC 1例。6例患者血清抗SOX1抗体均阳性,其中合并其他抗体阳性1例、合并脑脊液抗SOX1抗体阳性2例。6例患者神经系统症状均早于肿瘤发现前,均于发现抗SOX1抗体后行肿瘤筛查,其中3例SCLC患者进行治疗后病情较稳定;截至随访时间,余3例患者经检查未发现肿瘤(其中病例5随访>2年,病例2和病例4随访<2年),进行治疗后,症状未见明显进展。结论抗SOX1抗体相关PNS患者存在高度临床异质性,部分患者伴发肿瘤。可增加副肿瘤抗体的检测,以提高早期诊断潜在肿瘤的可能性。