神经系统副肿瘤综合征的临床特征(附1例报告)

目的探讨神经系统副肿瘤综合征(PNS)的临床特征。方法回顾性分析1例临床表现为头痛的神经系统PNS患者的临床资料。结果本例患者表现为反复头痛发作3年。腰穿CSF检查示细胞数正常,蛋白轻度升高;头颅MRI示双侧丘脑异常信号,增强示双侧丘脑低信号。胸部CT示左肺上叶少许胸膜下肺大泡;PET-CT示左肺上叶(主动脉后方)高代谢结节。肿瘤标志物CA19-9、CA242、癌胚抗原水平升高。病理检查确诊为肺腺癌。本研究回顾性总结PNS相关文献报道,其常见临床表现为眩晕、复视、共济失调、斜视性眼阵挛-肌阵挛及脊髓炎。结论 PNS常见表现为眩晕、复视、共济失调、斜视性眼阵挛-肌阵挛及脊髓炎,以反复头痛为临床表现少见,可疑患者应行全身检查排除PNS。     

边缘系统受累神经梅毒的MRI特点

目的探讨边缘系统受累的神经梅毒的MRI特点,提高临床医师对该影像表现的认识。方法对2014年1月1日至2017年12月31日于重庆医科大学附属第一医院住院诊断为神经梅毒患者的头颅MRI进行回顾性研究,纳入边缘系统受累的神经梅毒3例,收集MRI资料并分析其特点。结果 3例患者头颅MRI均表现为双侧海马异常信号,呈T_1低信号、T_2及FLAIR序列高信号,DWI序列等信号改变,增强扫描无强化,并伴有侧脑室颞角扩大,明显海马萎缩。结论 神经梅毒累及边缘系统时,以双侧海马为主,同时伴有明显海马萎缩,FLAIR序列识别病灶最为敏感。     

多发性硬化中的周围神经异常

目的　探讨多发性硬化合并周围神经损害的临床特点和发病机制。方法　对 2例多发性硬化患者进行临床观察及电生理检查 ,并作文献复习。结果　 2例患者均有下运动神经元异常表现 ,膝、跟腱反射消失 ,神经传导速度减慢 ,电生理检查提示病变既没有损害前角细胞 ,也没有累及周围神经的髓内根 ,可能是周围神经受影响。结论　中枢神经系统和周围神经系统联合受累的机制可能在于自身免疫反应 ,有一种类似髓鞘碱性蛋白的抗原 ,由于它在中枢神经系统占优势 ,免疫反应造成神经系统受损程度不同 ,或者中枢神经系统损害严重 ,导致血脑屏障及血神经屏障受损 ,最后导致周围神经脱髓鞘和轴索变性。     

卟啉代谢异常并发神经系统损害5例临床分析

目的 探讨卟啉代谢异常并发神经系统损害的表现。方法 总结3例血卟啉病2例铅中毒继发卟啉代谢异常患者的临床资料。结果 5例均有消化系统及神经系统受累的表现，包括精神异常，意识障碍，周围神经损害，自主神经损害，尿卟胆原（PBG）阳性2例，尿δ－所基酮戊酸（δ－ALA）增高4例，铅中毒继发卟啉代谢异常也出现同样的症状。结论 卟啉代谢异常可造成中枢神经，下丘脑，周围神经，自主神经等神经系统损害，尿中卟啉及其前体物的检验可确诊，并应常规做尿铅检查。应用静脉注射葡萄糖治疗效果较好。     

边缘性脑炎与相关抗体的研究现状

20世纪60年代,Brierley等和Corsellis等首先报道了选择性累及边缘性结构（海马、杏仁核、下丘脑等）的一类中枢神经系统炎性疾病,称为边缘性脑炎(limbic encephalitis,LE)。由于最初的病例多伴有小细胞肺癌、乳腺癌以及淋巴瘤等,通常认为LE与肿瘤相关,或称之为副肿瘤性边缘性脑炎（paraneoplastic limbic encephalitis,PLE）。其临床主要表现为进行性的短时记忆缺失甚至发展为痴呆,伴有癫痫发作,并且有不同程度的小脑、脑干等边缘系统外组织受累;脑电图表现为单侧或双侧颞叶的痫性活动病灶,或者出现局灶性或广泛性的慢波;磁共振成像(MRI)T2或FLAIR像显示在单侧或双侧颞叶内侧面有高信号异常病灶,强化少见,多数患者可发展为颞叶萎缩,部分患者可发现脑干、丘脑等边缘系统外病灶;脑脊液检查呈炎性改变,淋巴细胞轻到中度增高,蛋白含量升高,葡萄糖含量正常,常有IgG指数的升高及寡克隆区带的出现[1-2]。     

有机磷中毒后迟发性周围神经病的临床和神经病理四例

目的探讨有机磷中毒后迟发性周围神经病(OPIDP)的临床及神经病理改变特点。方法对4例口服有机磷后出现周围神经损害症状的患者进行电生理和腓肠神经活检检查。结果 4例患者于急性中毒后平均20.5(10～24)天出现以下肢受累为主的逆行性运动感觉周围神经病,其中2例患者存在锥体束征。电生理检查提示以运动神经受累为主的轴索性周围神经损害。腓肠神经活检主要表现为与病程相关的轴索损害及再生现象,可见急性期的炎性反应、小纤维损害和继发的髓鞘改变。结论有机磷中毒后迟发性周围神经病表现为以运动障碍为主的逆行性神经病,中枢神经系统亦可能受累及;周围神经病理表现为轴索损害为主,同时存在小纤维损害及继发的髓鞘改变。     

干燥综合征合并抗GQlb抗体综合征1例报告

<正>干燥综合征(Sjogren syndrome,SS)是一种累及多系统的免疫介导的慢性炎性疾病,神经系统是其所累及系统之一,包括中枢神经系统及周围神经系统。吉兰-巴雷综合征(Guillain-Barre syndrome,GBS)是一种免疫介导的炎性周围神经病,常呈急性起病,表现为多发神经根及周围神经受累。Miller-Fisher综合征(Miller-Fisher syndrome,MFS)是     

儿童流行性乙型脑炎MRI及扩散加权成像早期诊断的优势

目的探讨儿童乙脑MRI影像特点及早期扩散加权成像价值,以提高对乙脑影像征象认识及早期检出。方法回顾性分析2006-2013年经我院临床诊治的27例乙脑患者,全部病例均为血清IgM乙脑抗体阳性。磁共振扫描仪采用GE 1.5T超导磁共振成像扫描仪。其中20例在发病至神经系统出现症状7d内应用DWI。结果 27例儿童乙脑中24例累及丘脑,13例累及中脑黑质,6例累及皮层或皮层下白质,4例累及基底节,2例累及海马,2例累及胼胝体压部。20例在急性期(7d内)应用DWI检查:8例为细胞毒性水肿,DWI显示病灶范围、信号强度均优于T2WI、FLAIR,其中DWI单独显示3例。7例血管源性水肿,T2WI、FLAIR优于DWI。3例脑内多发病灶内两种水肿共存,DWI与T2WI、FLAIR在显示病灶能力中互有优势。结论儿童乙脑好累及双侧丘脑、中脑黑质,影像学具有一定特征,早期DWI比T2WI、FLAIR更易发现病灶。     

原发性干燥综合征中枢神经系统损害八例临床分析

目的探讨原发性干燥综合征的中枢神经系统损害的临床表现、MRI特点、治疗方法。 方法 回顾性分析漳州市福建医科大学附属漳州市医院确诊的8例原发性干燥综合征合并中枢神经系统损害的临床表现、MRI特点。 结果临床可表现为延髓背外侧综合征、肢体无力、智能改变,可类似多发性硬化、视神经脊髓炎,视神经较经常累及;MRI检查示5例出现脊髓损害：病变部位以颈胸髓为主,颈髓2例,胸髓2例,1例同时累及颈髓和胸髓,病灶可大片状、散在斑片状,可轻度、明显强化或不强化,5例出现颅内损害：病变部位可见于延髓背外侧、丘脑、海马、中脑导水管、第四脑室旁、侧脑室旁,可表现为皮质下及脑室旁白质多发小缺血灶,可类似韦尼克脑病,l例出现颈动脉夹层,7例使用了激素后病情有一定改善。 结论原发性干燥综合征中枢神经系统损害的症状、体征及影像学表现多种多样。     

腓骨肌萎缩症X型伴有可逆性中枢神统系统受累

目的探讨的腓骨肌萎缩症X型的临床特点和引起中枢神经系统受累可能的发病机制。方法报道1例国内未报道过的CMTX可引起中枢神经系统受累Cx32基因突变,分析该例患者的临床、电生理及病理改变特点。结果该患者为基因检查确诊Arg424Trp(R142W)突变的CMTX。临床存在可逆性眼震,电生理提示感觉运动神经受累均受累,轴索髓鞘均损害。肌肉活检提示慢性神经源性病理改变。周围神经活检示周围神经髓鞘损害为主。BAEP提示可逆性中枢性损害。结论 R142W突变的CMTX可以在应激条件下出现可逆性中枢神经系统损害。     

An autopsied case of paraneoplastic neurologic syndrome (limbic encephalitis, cerebellar degeneration, and pseudohypertrophy in the inferior olivary nuclei) associated with T cell lymphoma]

We describe a patient with paraneoplastic neurologic syndrome confirmed on postmortem examination. This 42-year-old man was admitted due to general fatigue, emaciation and acute-onset disorientation. Neurological examination revealed disorientation, moderate cognitive impairment, cerebellar ataxia, bilateral limitations and nystagmus in all directions during external ocular movement, swallowing disorder, bilateral Babinski sign, sensory disturbance in the distal parts of all extremities, and Romberg's sign. T-cell lymphoma was diagnosed following biopsy of the cervical lymph node. Neurological condition improved slightly after chemotherapy, but subsequently deteriorated. At about 6 years after the disease onset, brain magnetic resonance imaging (MRI) revealed atrophy of bilateral hippocampi and the upper vermis of the cerebellum. The patient died of pneumonia after a clinical course of about 6 years and 6 months. Pathologically, neuronal loss, reactive gliosis and perivascular lymphocytic infiltration were observed in the hippocampi, cerebellum, and inferior olivary nuclei. Lymphocytes around the vessels were positive for LCA and UCHL-1, but negative for CD8 and L26, and thus were considered to be T cells. No lymphoma cell was observed in the central nervous system or lymphatic organs. Based on the pathological findings, paraneoplastic neurologic syndrome (limbic encephalitis, cerebellar degeneration and olivary pseudohypertrophy) associated with T-cell type malignant lymphoma was diagnosed. Only three other cases of paraneoplastic neurologic syndrome associated with T-cell lymphoma have been reported. In those cases, death occurred due to the deterioration of malignant lymphoma, whereas the present patient died about 6 years after the remission of malignant lymphoma. Prognosis may thus depend on the course of the malignant lymphoma. In the present patient, neurological symptoms deteriorated after remission of malignant lymphoma, and no pathological lesion were found in the lymphatic organs. Lesions in the central nervous system in paraneoplastic neurological syndromes may follow a course independent of the original malignant disease.     

椎管内硬脊膜外海绵状血管瘤的诊治

目的总结单纯椎管内硬脊膜外海绵状血管瘤的诊治经验。方法回顾性分析2006年2月至2009年3月经病理证实的7例单纯椎管内硬脊膜外海绵状血管瘤患者的临床表现、MRI特征和手术结果。结果 7例均表现为慢性双下肢麻木及无力,1例伴有神经根性症状;脊柱MRI显示6例病变位于胸椎,1例位于颈胸交界区;所有病变均达到手术全切,术后所有患者神经功能症状明显改善。结论单纯椎管内硬脊膜外海绵状血管瘤临床少见;主要表现为脊髓及神经根慢性受压而出现神经功能缺损症状及体征;脊柱MRI示病变位于硬脊膜外脊髓背侧或一侧,且有经相应椎间孔向外生长的趋势;手术切除病变较为容易,术后患者神经功能改善明显。     

Paraneoplastic syndrome in the course of lung adenocarcinoma: morphological picture and immunohistochemical analysis of the inflammatory infiltrates and PECAM-1 expression

We examined sections of brain, spinal cord, spinal roots, and peripheral nerves from a patient with paraneoplastic syndrome in the course of lung adenocarcinoma. Morphological examination showed marked loss of myelin fibers in peripheral nerves, severe brain tissue edema, and paraneoplastic degeneration involving cerebrum and cerebellum with inflammatory components. Inflammatory infiltrates examined immunohistochemically using antibodies against antigens CD 3, CD 4, CD 8, and CD 20 turned out to be composed of cytotoxic T lymphocytes. The expression of platelet-endothelial cell adhesion molecule-1 (PECAM-1) in blood vessels was increased in comparison with control material, which may facilitate transendothelial lymphocyte migration triggering a cascade of biochemical and morphological reactions observed in paraneoplastic syndrome.     

Multiple paraneoplastic syndromes in a patient with antibodies to neuronal nucleoproteins (anti-Hu)

We report the clinical and autopsy studies of a patient with an unusual combination of multiple paraneoplastic neurological syndromes in association with antibodies to a 35–40 kDa neuronal nucleoprotein (anti-Hu). Neurological disease preceded the detection of a small cell carcinoma of the lung. The patient had combined sensory and motor neuronopathy or neuropathy, cerebellar degeneration, brain-stem and limbic encephalitis, and clinical evidence of the Lambert-Eaton myasthenic syndrome and gastrointestinal pseudo-obstruction of paraneoplastic origin.     

Chorea-athetosis in the anti-Hu syndrome

INTRODUCTION: Paraneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-athetosis. CASE REPORT: A 48-year-old woman developed a small-cell lung carcinoma revealed by an anti-Hu syndrome. The neurological features included choreo-athetosis predominating in the upper limbs, chronic sensorimotor axonal polyneuropathy, and opsoclonus. The cerebrospinal fluid was acellular and contained several oligoclonal IgG bands, not found in the corresponding serum. Magnetic resonance imaging revealed bilateral high-intensity lesions on T2/FLAIR sequence in the corona radiata. Moderate transitory improvement of the paraneoplastic neurological syndrome was observed after several carboplatin-etoposid cycles. CONCLUSION: A paraneoplastic origin must be considered in all cases of unexplained choreo-athetosis. Paraneoplastic choreo-athetosis is most often associated with other neurological symptoms. The most frequent associated tumor is a small-cell lung carcinoma with anti-CRMP5 and/or anti-Hu antibodies. Our patient developed paraneoplastic choreo-athetosis related to an anti-Hu syndrome in the absence of anti-CRMP5/CV2 antibodies. Paraneoplastic choreo-athetosis might result from a central lesion, and/or from proprioceptive deafferentation subsequent to peripheral neuropathy.     

Antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors

BACKGROUND: Antiamphiphysin antibodies react with a 128-kd protein found in synaptic vesicles.They were first described in patients with paraneoplastic stiff-man syndrome and breast cancer, but studies suggest that they can also occur in patients with other tumors and neurological disorders. OBJECTIVE: To determine if antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors. PATIENTS AND METHODS: Of 2800 serum samples tested by routine immunohistochemical procedures on sections of paraformaldehyde-fixed rat brain for the detection of autoantibodies associated with paraneoplastic neurological syndromes, 5 were selected because of labeling suggestive of antiamphiphysin antibodies and subsequently confirmed by the results of Western blot analysis using recombinant amphiphysin protein. Controls consisted of 40 patients with various nonparaneoplastic neurological diseases; 101 patients with cancer but without paraneoplastic neurological syndrome; 9 patients with small cell lung cancer, anti-Hu antibodies, and paraneoplastic neurological syndrome; 3 patients with M2-type antimitochondrial antibodies but no neurological disorder; and 30 normal subjects. RESULTS: Of the 5 patients with antiamphiphysin antibodies, patient 1 had sensory neuronopathy, encephalomyelitis, and breast cancer; patient 2 had limbic encephalitis, and small cell lung cancer was detected in the mediastinum after 24 months of follow-up; patient 3 had encephalomyelitis and ovarian carcinoma; and patients 4 and 5 had Lambert-Eaton myasthenic syndrome and small cell lung cancer (patient 4 subsequently developed cerebellar degeneration). None of the 5 had stiffness. Two patients (Nos. 2 and 4) had antimitochondrial antibodies. The two patients (Nos. 4 and 5) with Lambert-Eaton myasthenic syndrome had antibodies directed against the voltage-gated calcium channel, and patient 2 subsequently developed anti-Hu antibodies. In the controls, antiamphiphysin antibodies were detected by Western blot analysis in 3 of 8 patients with anti-Hu antibodies, but in none of the other groups. CONCLUSIONS: These data indicate that antiamphiphysin antibodies are not specific for one type of tumor or one neurological syndrome and can be associated with other neural and nonneural antibodies. The simultaneous association of several antibodies in some patients suggests multimodal autoantibody production.     

A case report of the compression syndrome due to hypertrophic neuropathy

We reported a case of hypertrophic neuropathy of adult onset. The pathological change in the sural nerve was decreased axonal population with onion-bulb formation. On examination, there were enlarged nerve on palpation and she was found to have distal muscle atrophy, weakness and sensory loss. The deep tendon reflexes of extremities were weak. The first clinical feature of this patient was mainly polyneuropathy. The lower limbs were slightly spastic and plantar responses were extensor bilaterally. There was also sensory level at C6 level. After 1.5 years from first examination, she had shown myelopathy. Magnetic resonance imaging (MRI) of the spine showed marked thickening of the nerve roots and it revealed the compression of the spinal cord by enlarged nerve roots from C2 to C6 level. The compression syndrome of the patients with hypertrophic neuropathy was unclear at the onset in this case. MRI study of the spinal cord would be very beneficial to disclose subclinical myelopathy associated with hypertrophic neuropathy, as indicated in this report.     

A patient of sensorimotor neuropathy with small cell lung carcinoma and anti-GM1 antibody]

We reported a 62-year-old woman had sensorimotor neuropathy with small cell lung carcinoma (SCLC) and anti-GM1 antibody. She was admitted with several months history of progressive numbness, walking disturbance and anorexia. Neurologic examination revealed severe numbness and deep sensory disturbance of extremities and body, and mild weakness of distal extremities. Deep tendon reflexes were absent. Her limbs were ataxic. Nerve conduction studies showed no sensory evoked responses. CSF protein was elevated. Sural nerve biopsy revealed severe loss of myelinated fibers and perivascular mononuclear cells surrounding the perineurial vessel. Vasculitic neuropathy was diagnosed, and prednisolone was started, with no benefit. In the clinical course, she developed cough attacks and was found the lymphnode swelling in the mediastinum and supraclavicular fossa, which was diagnosed SCLC. Although anti-Hu antibody were not detected, anti-GM1 antibody was positive. She was treated with intravenous immunoglobulin, with transient improvement. The rare case of the paraneoplastic peripheral neuropathy with SCLC and anti-GM1 antibody was reported.     

Spinal cord stimulation with percutaneous leads after loss of coverage with implanted surgical lead

Objective: This case report presents an application of percutaneous spinal cord stimulation to a patient with complex regional pain syndrome type 1 involving the bilateral lower extremities with loss of coverage with surgical lead. Measurements: The patient underwent an uneventful spinal cord stimulator trial with percutaneous placement of two temporal eight‐electrode epidural leads (Medtronic Inc, Minneapolis, MN, USA) to level T11. Results: Upon experiencing excellent pain relief over the next three days, the patient was implanted with permanent leads and rechargeable generator four weeks later and reported sustained pain relief. Conclusion: Percutaneous spinal cord stimulation offers an alternative treatment option for the patient with loss of coverage with surgical lead.     

Acute motor and sensory neuronopathy associated with small‐cell lung cancer: A clinicopathological study

A 48‐year‐old Chinese woman developed ascending motor paralysis while visiting Japan, leading to tetraplegia and respiratory failure over 2 weeks. The patient’s course was complicated by anoxic encephalopathy. Nerve conduction studies revealed a severely decreased amplitude of compound muscle action potentials and a sural nerve biopsy specimen showed findings consistent with axonal‐form Guillain–Barré syndrome. An autopsy, excluding the brain, demonstrated small‐cell lung cancer that was not detected clinically, axonal‐dominant degeneration in the nerve roots and distal peripheral nerves, and the loss of both myelin and axons in the dorsal spinal column. The spinal anterior horn cells were severely decreased and were accompanied by astrocytic reaction in all spinal segments with lymphocytic infiltration. A limited examination of the dorsal root ganglia did not show Nageotte nodules, but the infiltration of T cells was observed. Although the clinical course mimicked axonal‐form Guillain–Barré syndrome, the autopsy demonstrated both sensory and motor neuronal involvement, as well as small‐cell lung cancer. Although anti‐Hu and antiganglioside antibodies were negative in the patient’s serum, the paraneoplastic mechanism might have damaged the anterior horn and dorsal root ganglia cells, which subsequently led to secondary axonal degeneration. There has been a report on a case of paraneoplastic subacute motor neuronopathy, but the acute course described here has not been reported before.