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1.
Reported paraneoplastic neurological syndromes (PNS) are rare disabling neurological diseases with supposed autoimmune pathogenesis. The aims of this study were to evaluate frequency, clinical course and therapeutic response in the cohort of PNS positive patients (n=10) in the Czech Republic for the first time. Second, we determined the presence and distribution of oligoclonal IgG bands (OB IgG) in PNS and compared the clinical and laboratory features of OB IgG positive and negative patients. A total of 2355 suspicious serum and/or CSF samples were screened by immunofluorescence and immunohistochemistry with definite confirmation by Western blot. OB IgG were detected by isoelectric focusing and immunoenzymatic staining and clinical status was scored according to modified Rankin scale (RS). Four patients had anti-Yo antibody, ovarian cancer and the score in range (2-5) on RS. Five patients had anti-Hu antibody, small cell lung cancer (SCLC), prostate cancer and the score between 1-4 grade on RS. One patient with SCLC and anti-Ri antibody had grade 2. Five of 10 patients with PNS had positive OB IgG and average value 4.2 on RS comparing with negative OB IgG patients with average value 2.6. Finally, we add well-defined cohort of PNS patients to emerging European profile of PNS and conclude that the presence of OB IgG in PNS seems to reflect enhanced immune response with more severe neurological damage and clinical course.  相似文献   

2.
ObjectivesTo evaluate the association of polysomnographic parameters with clinical symptom severity in Robin sequence (RS) patients.MethodsAll patients diagnosed as presenting with RS at Hospital de Clínicas de Porto Alegre from October 2012 to June 2016 were enrolled. They were classified as isolated RS, RS-plus, and syndromic RS. Polysomnography (PSG) was performed, except for those patients in need of respiratory support. Symptom severity was evaluated as defined by the Cole et al. classification. Ordinal OR (for the chance of increase in one grade on the clinical severity scale) and R2 (determination coefficient from ordinal logistic regression) were computed from data analysis.ResultsA total of 80 participants were enrolled in the study. Fifty-five of these were able to undergo polysomnography. Worsening of the studied PSG parameters was associated with increase in clinical severity grading, as follows: desaturation index (OR 1.27; 95% CI; 1.07–1.51; R2 = 19.8%; p = 0.006); apnea/hypopnea Index (OR 1.13; 95% CI; 1.01–1.26; R2 = 12.5%; p = 0.02); sleep mean oxygen saturation (OR 0.16; 95% CI; 0.05–0.52; R2 = 22.6%; p = 0.002); oxygen saturation nadir (OR 0.73; 95% CI; 0.56–0.96; R2 = 10.0%; p = 0.02); percentage of time with oxygen saturation <90% (OR 9.49; 95% CI; 1.63–55.31, R2 = 37.6%; p = 0.012); and percentage of time presenting with obstruction (OR 2.5; 95% CI; 1.31–4.76; R2 = 25.1%; p = 0.006).ConclusionsPolysomnography parameters were associated with severity of clinical manifestations in patients with RS. Oxyhemoglobin saturation-based parameters had surprisingly significant R2 values. Therefore, those parameters, which have traditionally been undervalued in other clinical settings, should also be assessed in the polysomnographic evaluation of RS patients.  相似文献   

3.
《Neuromodulation》2023,26(3):694-699
ObjectivesPatients with spinal lesions or vertebral compression fractures from multiple myeloma often present with back pain that restricts their ability to lie flat and prevents them from undergoing cancer treatment. Temporary, percutaneous peripheral nerve stimulation (PNS) has been described for cancer pain secondary to oncologic surgery or neuropathy/radiculopathy from tumor invasion. The purpose of this case series is to show the use of PNS as an analgesic bridge therapy to treat myeloma-related back pain and allow patients to complete their course of radiation.Materials and MethodsTemporary, percutaneous PNS was placed under fluoroscopic guidance for four patients with unremitting low back pain secondary to myelomatous spinal lesions. Before PNS, the patients had pain refractory to medical management and were unable to tolerate radiation mapping and treatment owing to low back pain while supine. Patients were followed with routine clinic visits to monitor pain and progression through cancer therapy. PNS was removed after approximately 60 days or after completion of radiation.ResultsThis case series presents four successful cases of PNS to treat low back pain from myelomatous spinal lesions and associated vertebral compression fractures. PNS targeted the medial branch nerves to treat both nociceptive and neuropathic low back pain. All four patients successfully completed radiation therapy with PNS in place.ConclusionsPNS can effectively treat low back pain secondary to myeloma-related spinal lesions as a bridge therapy to radiation. The use of PNS is a promising option for back pain from other primary or metastatic tumors. Further research is needed into the use of PNS for cancer-related back pain.  相似文献   

4.
Objectives – SOX1 antibodies have been described in patients with Lambert–Eaton myasthenic syndrome (LEMS) in association with voltage‐gated calcium channel antibodies as serological markers of small cell lung cancer (SCLC). This study was aimed to screen for additional SOX1 autoimmunity in onconeural antibody‐positive sera from patients with paraneoplastic neurological syndromes (PNS) other than LEMS and to identify the clinical–immunological profile and associated tumours of patients with coexisting SOX1 antibodies. Methods– We retrospectively analysed sera from 55 patients with different PNS positive for well‐characterized antineuronal antibodies for the presence of SOX1 antibodies by recombinant ELISA and immunoblot. Results– Eight (14.5%) patients showed additional SOX1 antibodies in the ELISA and the recombinant immunoblot. Five patients had coexisting Hu antibodies, while the other three showed coexisting CV2/CRMP5, amphiphysin, and coexisting CV2/CRMP5 and Hu antibodies, respectively. PNS included (partially overlapping) subacute sensory neuropathy, subacute sensorimotor neuropathy, cerebellar degeneration, brainstem encephalitis, encephalomyelitis and limbic encephalitis. No tumour was detected in two patients, while the others had lung cancer (four SCLC and two non‐SCLC). One patient showed SOX1‐specific intrathecal antibody synthesis. Conclusions– We describe SOX1 reactivity for the first time overlapping with CV2/CRMP5 and amphiphysin antibodies. SOX1 reactivity is predominantly associated with Hu antibodies and SCLC, but can occur also in other types of lung cancer. Neurological manifestations present in patients with coexisting SOX1 antibodies and well‐characterized antineuronal antibodies do not differ from those previously described in patients positive for antineuronal antibodies but no SOX1‐specific anti‐glial antibodies.  相似文献   

5.
《Neuromodulation》2023,26(3):690-693
ObjectivesThe primary objective of this study was to determine the current rate of lead fracture during temporary percutaneous peripheral nerve stimulator (PNS) lead removal at the Mayo Clinic Rochester Division of Pain Medicine.Materials and MethodsA retrospective review of electronic medical records was performed for patients implanted with a temporary percutaneous PNS device between January 1, 2018, and December 31, 2020. Patients were included if they underwent temporary percutaneous PNS system implant, with planned lead removal at 60 days. Data collection included date of implant, diagnosis, peripheral nerve target(s), number of leads, and lead tip status at the time of removal (intact vs fractured).ResultsFifty patients underwent a total of 80 temporary percutaneous PNS leads placed during the time frame analyzed. Of the 80 temporary percutaneous PNS leads implanted, there were five lead fractures at the time of intentional lead removal.ConclusionsThis retrospective review of 50 patients with 80 temporary percutaneous PNS leads implanted for chronic peripheral neuropathic pain resulted in a 6.25% rate of retained lead fragment at the time of lead removal by the provider at the end of 60-day treatment. This fracture and retention rate is consistent with previous published retrospective data on PNS, which has shown a 3% to 21% lead fracture rate during intentional lead removal.  相似文献   

6.
Background

Paraneoplastic Cerebellar degeneration (PCD) is one of the classical paraneoplastic syndromes (PNS) which is characterised by subacute onset, progressive cerebellar ataxia and is usually associated with small cell lung carcinoma, adeno carcinoma of breast and ovary followed by Hodgkin’s lymphoma.

Objective

We herein report a case of subacute onset, progressive cerebellar ataxia in a 37-year-old female, who on evaluation was found to have non-Hodgkin’s lymphoma and experienced good clinical response to treatment.

Discussion

As compared to solid tumours, chances of association of PNS with Lymphomas is quite low and there are only few case reports in the literature showing association of PCD with non-Hodgkin’s lymphoma. As PCD is one of the classical PNS, it is very important to identify subtle cerebellar manifestations in an otherwise apparently normal individual, as early diagnosis and aggressive treatment can immensely improve the mortality and morbidity associated with this syndrome.

Conclusion

This case signifies the importance of suspecting PNS as an important differential diagnosis in a young patient presenting with subacute onset progressive cerebellar ataxia and evaluating her extensively for malignancy in spite of no paraneoplastic antibody been detected as early diagnosis and treatment can lead to gratifying response. We do agree that 2 weeks follow up is a short time interval to determine whether the response was sustained or not, for which a long term follow up is required.

  相似文献   

7.

Objectives

Rituximab treatment has shown clinical improvement in anti-myelin associated glycoprotein (MAG) polyneuropathy. We analyzed scores of clinical scales and the most sensitive electrophysiological parameters before and after immunomodulating treatment with rituximab in a group of patients affected by anti-MAG demyelinating polyneuropathy.

Methods

Clinical scores, the percentage of CD20 B-lymphocytes, anti-MAG antibody titers and electrophysiological data in 7 patients with anti-MAG polyneuropathy were analyzed. The patients were examined before a cycle with rituximab, 6, 12 and 24 months after the end of the treatment. Two patients were treated with rituximab additional cycles and re-evaluated 48 months after the first treatment.

Results

There were no evident correlation between anti-MAG serum antibody titers or clinical scales and electrodiagnostic data. Significant decrease in the proportion of CD20 B-lymphocytes was observed. Significant anti-MAG antibodies titers reduction was detected after re-treatment. At follow-up, pinprik sensation and two point discrimination presented a significant improvement compared with the score before treatment.

Conclusions

In our patients, rituximab did not improve any electrophysiological data. No correlation with anti-MAG serum antibodies course was found. With rituximab only pin sensibility improved.

Significance

Rituximab re-treatment significantly reduces anti-MAG serum antibodies titers but improves only small fibers sensibility.  相似文献   

8.
Autoantibodies in patients with paraneoplastic neurological syndromes (PNS) have been reported to be predominantly IgG1 and IgG3 isotypes. However, no data are available about the IgG subclass distribution of the total serum IgG in these patients. Therefore, we investigated the IgG subclass distribution (given as percentage of total IgG) in 15 anti-Hu positive PNS patients, 15 patients with small cell lung cancer (SCLC) without PNS and 23 healthy controls using a commercial enzyme-linked immunosorbant assay test. Although IgG1 (and to a lower extent IgG3) are the predominant subclasses of the anti-Hu antibodies, PNS and SCLC showed a significant decrease in IgG1 and a concomitant increase in IgG2 compared with healthy controls (P < 0.05, respectively). In contrast, only SCLC patients, but not PNS patients, had higher IgG3 and IgG4 values compared with controls (P < 0.05, respectively). There was no correlation between IgG subclass levels and the titre or the predominant isotype of the antineuronal antibodies. PNS patients with autonomic disturbances had lower IgG4 levels than PNS patients without autonomic disturbances (P < 0.05). Our study demonstrates a disturbance in the IgG subclass distribution in PNS patients which is partly different from SCLC patients. The isotype regulation of the anti-Hu antibody seems to be independent from this phenomenon.  相似文献   

9.
10.
Introduction: Rituximab appears to be beneficial in treatment‐refractory myasthenia gravis (MG); however, prospective, long‐term durability data are lacking. Methods: In this prospective, open‐label study of rituximab in refractory MG, 22 patients (10 nicotinic acetylcholine receptor, 9 muscle‐specific tyrosine kinase, 3 seronegative) received rituximab at baseline, with repeat cycles driven by clinical worsening. Manual muscle testing (MMT) scores and CD19/CD20+ B‐cell counts were serially monitored. Results: At mean follow‐up of 28.8 ± 19.0 months (range, 6–66), mean MMT scores declined from 10.6 ± 5.4 to 3.3 ± 3.1 (P < 0.0001). Mean prednisone dosage declined from 25.2 ± 15.1 to 7.3 ± 7.1 mg/d (P = 0.002). Ten relapses occurred, with average time to first relapse of 17.1 ± 5.5 months (range, 9–23). CD19/CD20+ count recovery did not predict relapse. Three patients experienced prolonged B‐cell depletion (range, 24–45 months) after 1 cycle. Discussion: Sustained clinical improvement was associated with rituximab after 1 cycle, with prolonged time to relapse and reduction in steroid dosage. Muscle Nerve 58 : 453–456, 2018  相似文献   

11.
《Neuromodulation》2021,24(6):1059-1066
ObjectivesWith the development of percutaneously inserted devices, peripheral nerve stimulation (PNS) has been gaining attention within chronic pain literature as a less invasive neurostimulation alternative to spinal column and dorsal root ganglion stimulation. A majority of current PNS literature focuses on targeting individual distal nerves to treat individual peripheral mononeuropathies, limiting its applications. This article discusses our experience treating dermatomal pain with neurostimulation without needing to access the epidural space by targeting the proximal spinal nerve with peripheral nerve stimulation under ultrasound-guidance.Materials and MethodsA temporary, percutaneous PNS was used to target the proximal spinal nerve in 11 patients to treat various dermatomal pain syndromes in patients seen in an outpatient chronic pain clinic. Four patients received stimulation targeting the lumbar spinal nerves and seven patient received stimulation targeting the cervical or thoracic spinal nerves.ResultsThe case series presents 11 cases of PNS of the proximal spinal nerve. Seven patients, including a majority of the patients with lumbar radiculopathy, had analgesia during PNS. Four patients, all of whom targeted the cervical or thoracic spinal nerves, did not receive analgesia from PNS.ConclusionPNS of the proximal spinal nerve may be an effective modality to treat dermatomal pain in patients who are not candidates for other therapies that require access to the epidural space. This technique was used to successfully treat lumbar radiculopathy, post-herpetic neuralgia, and complex regional pain syndrome.  相似文献   

12.
Abstract Background Peripheral nervous system (PNS) affection is an uncommon, sometimes life-threatening manifestation of giant cell arteritis (GCA). Objective To describe characteristics of neurological abnormalities of the PNS in GCA patients. Methods Eighty consecutive cases of biopsy proven GCA were studied. Results Three patients presented with subacute sensorimotor deficits abnormalities in the distribution of the arm plexus. In all cases PNS affection was the leading clinical symptom in addition to a typical clinical syndrome of cranial arteriitis. In one case MRI demonstrated diffuse signal abnormalities surrounding the brachial nerve plexus. In another patient, who died from pulmonary embolism 10 weeks after beginning of therapy, autopsy demonstrated residual arteritis in an artery supplying the brachial nerve plexus. Conclusions Involvement of the PNS is more uncommon than cerebral ischemia and neuroophthalmological complications in patients suffering from GCA. Severe PNS involvement has an affinity to the midcervical nerve roots and the brachial nerve plexus.  相似文献   

13.
BackgroundThe Positive and Negative Syndrome Scale (PANSS) is widely used in schizophrenia and has been divided into distinct factors (5-factor models) and subfactors. Network analyses are newer in psychiatry and can help to better understand the relationships and interactions between the symptoms of a psychiatric disorder. The aim of this study was threefold: (a) to evaluate connections between schizophrenia symptoms in two populations of patients (patients in the acutely exacerbated phase of schizophrenia and patients with predominant negative symptoms [PNS]), (b) to test whether network analyses support the Mohr 5 factor model of the PANSS and the Kahn 2 factor model of negative symptoms, and finally (c) to identify the most central symptoms in the two populations.MethodsUsing pooled baseline data from four cariprazine clinical trials in patients with acute exacerbation of schizophrenia (n = 2193) and the cariprazine–risperidone study in patients with PNS (n = 460), separate network analyses were performed. Network structures were estimated for all 30 items of the PANSS.ResultsWhile negative symptoms in patients with an acute exacerbation of schizophrenia are correlated with other PANSS symptoms, these negative symptoms are not correlated with other PANSS symptoms in patients with PNS. The Mohr factors were partially reflected in the network analyses. The two most central symptoms (largest node strength) were delusions and uncooperativeness in acute phase patients and hostility and delusions in patients with PNS.ConclusionsThis network analysis suggests that symptoms of schizophrenia are differently structured in acute and PNS patients. While in the former, negative symptoms are mainly secondary, in patients with PNS, they are mainly primary. Further, primary negative symptoms are better conceptualized as distinct negative symptom dimensions of the PANSS.  相似文献   

14.
Background and PurposeNeuromyelitis optica spectrum disorder (NMOSD) is a rare demyelinating disease of the central nervous system (CNS). We investigated the medical behaviors of experts in Korea when they are diagnosing and treating NMOSD.MethodsAn anonymous questionnaire on the diagnosis and treatment of NMOSD was distributed to experts in CNS demyelinating diseases.ResultsMost respondents used the 2015 diagnostic criteria for NMOSD and applied a cerebrospinal fluid examination, magnetic resonance imaging (MRI) of the brain and spine, and anti-aquaporin-4 antibody testing to all suspected cases of NMOSD. All respondents prescribed steroid pulse therapy as an first-line therapy in the acute phase of NMOSD, and 67% prescribed azathioprine for maintenance therapy in NMOSD. However, details regarding monitoring, the tapering period of oral steroids, second-line therapy use in refractory cases, management during pregnancy, and schedule of follow-up MRI differed according to the circumstances of individual patients. We analyzed the differences in response rates between two groups of respondents according to the annual number of NMOSD patients that they treated. The group that had been treating ≥10 NMOSD patients annually preferred rituximab more often as the second-line therapy (p=0.011) and had more experience with rituximab treatment (p=0.015) compared with the group that had been treating <10 NMOSD patients.ConclusionsThis study has revealed that NMOSD experts in Korea principally follow the available treatment guidelines. However, the differences in specific clinical practices applied to uncertain cases that have been revealed will need to be investigated further in order to formulate suitable recommendations.  相似文献   

15.
ObjectiveThe cerebral network subserving repetition suppression (RS) of the P50 auditory evoked response as observed using paired-identical-stimulus (S1–S2) paradigms is not well-described.MethodsWe analyzed S1–S2 data from electrodes placed on the cortices of 64 epilepsy patients. We identified regions with maximal amplitude responses to S1 (i.e., stimulus registration), regions with maximal suppression of responses to S2 relative to S1 (i.e., RS), and regions with no or minimal RS 30–80 ms post stimulation.ResultsSeveral temporal, parietal and cingulate area regions were shown to have significant initial registration activity (i.e., strong P50 response to S1). Moreover, prefrontal, cingulate, and parietal lobe regions not previously proposed to be part of the P50 habituation neural circuitry were found to exhibit significant RS.ConclusionsThe data suggest that the neural network underlying the initial phases of the RS process may include regions not previously thought to be involved like the parietal and cingulate cortexes. In addition, a significant role for the frontal lobe in mediating this function is supported.SignificanceA number of regions of interest are identified through invasive recording that will allow further probing of the RS function using less invasive technology.  相似文献   

16.
17.
We evaluated the efficacy and safety of rituximab in an open-label, uncontrolled study of 13 patients with polyneuropathy associated with antibodies to myelin-associated glycoprotein (MAG) and correlated the response to therapy with clinical and laboratory features. One year after rituximab therapy, anti-MAG immunoglobulin M (IgM) titers were significantly reduced. At that time, eight patients (62%) had improved in both the inflammatory neuropathy cause and treatment (INCAT) sensory sumscore and the Medical Research Council sumscore for muscle strength and seven of them also in the INCAT disability score. The improvement in the mean INCAT sensory sumscore was significant at 12 months and correlated with lower anti-MAG antibody at entry and at follow-up. This study suggests that rituximab may be efficacious in patients with anti-MAG associated neuropathy and particularly on sensory impairment and in those with moderately elevated antibody titers. These findings suggest that antibody reduction below a critical level may be necessary to achieve clinical improvement.  相似文献   

18.
ObjectiveTo observe the combination effects of Panax notoginseng saponins (PNS)and dual antiplatelet drugs (DAPT), and to explore the mechanism via cyclooxygenase /prostaglandin pathway.MethodsRight carotid artery thrombosis was induced in Wistar rats by infiltration with 70% FeCl3, and the animals were randomly divided into sham group, model group, DAPT group and PNS + DAPT group, intragastrically treated for 4 weeks. The cerebral pia mater microcirculation was observed in vivo after anesthetizing by anatomical microscope. The wet weight of carotid artery thrombosis was measured. Gastric mucosal injury was observed by hematoxylin and eosin staining. Platelet aggregation rate was detected with adenosine diphosphate -induced turbidimetry. Platelet CD62p expression was detected by flow cytometry. Concentrations of 6-Ketoprostaglandin F1 alpha, prostaglandin E2 in gastric mucosa and thromboxane B2, 6-Ketoprostaglandin F1 alpha, tissue plasminogen activator, plasminogen activator inhibitor, and fibrin fragment D in the plasma were measured by radioimmunoassay.ResultsPNS and DAPT increased the blood flow volume of cerebral pia mater and decreased erythrocyte aggregation and leukocyte adhesion of model rats. Compared to DAPT, PNS and DAPT further reduced the weight of carotid artery thrombosis with enhanced inhibition of platelet aggregation, increased tissue plasminogen activator levels and decreased fibrin fragment D levels. PNS and DAPT alleviated gastric injury induced by dual antiplatelet drugs and upregulated the expression of 6-Ketoprostaglandin F1 alpha in the gastric mucosa compared with DAPT.ConclusionsPNS combined with DAPT increased anti-thrombosis effects of DAPT and mitigated DAPT-related gastric injury. The underlying mechanisms may be associated with enhanced antiplatelet aggregation and activation of the fibrinolytic system and up-regulation of 6-Ketoprostaglandin F1 alpha expression in gastric mucosa.  相似文献   

19.
ObjectiveThe aim of this study is to investigate diagnostic value of electrical lumbar root stimulation (RS) at the laminar level in the early stage of Guillain–Barré syndrome (GBS).MethodsFifteen patients (30 sides) and nine controls (17 sides) were included in the study. Conventional nerve conduction studies, needle electromyography, F responses and electrical lumbar RS were obtained from both groups. The needle electrical stimulation was performed at the L2–3 intervertebral level. Vastus lateralis, tibialis anterior and soleus muscles were investigated bilaterally and simultaneously in the first and fourth weeks.ResultsIn all patients, the amplitudes elicited by lumbar RS were significantly attenuated while the conventional electrophysiological findings were normal and/or not diagnostic in 6 of 15 patients (40%) within the first week. Motor latencies by the lumbar RS were prolonged in the patients, compared to the controls, but the results were not statistically significant.ConclusionsM-responses elicited by lumbar RS appear to be helpful in disclosing proximal conduction abnormalities of GBS early in the course.SignificanceLumbar RS seems to be a useful method in making the diagnosis of GBS early and there is no considerable side effect of this particular method.  相似文献   

20.
《Clinical neurophysiology》2020,131(10):2383-2392
ObjectiveTo examine the peripheral nervous system (PNS) in spinal cord injured (SCI) patients using two novel methods: (1) MScanFit MUNE; a motor unit number estimation method detecting motor unit loss and (2) muscle velocity recovery cycles (MVRCs) measuring muscle membrane properties which has previously shown depolarization of the muscle membrane in denervated muscles.MethodsThirty chronic SCI patients (lesion above Th10) and twenty-five gender –and age matched healthy controls (HC) were examined. MScanFit was recorded from peroneal nerve to anterior tibial muscle (TA) and tibial nerve to abductor hallucis muscle after excluding localized mononeuropathies. MVRCs were recorded from TA.ResultsNerve conduction studies showed mononeuropathy in 8 patients (27%) (sciatic (2), -or peroneal nerve (6)). SCI patients had in average reduced motor unit number compared with HC and prolonged muscle refractory period and reduced supernormality.SignificanceA high prevalence of nerve lesion and a diffuse affection of the PNS following SCI are highly relevant findings that should be accounted for when planning neurorehabilitation for persons living with SCI.  相似文献   

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