Autoimmune paranodopathy (APN) has emerged as an independent rare disease,which is medicated by autoimmune antibodies against the essential complex of paranodal region of Ranvier. The antibodies include anti-neurofascin 155 antibody, anti-contactin-1 antibody and anti-contactin-associated protein 1 antibody. Although there are many similarities between APN and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), patients with APN have relatively unique clinical features, pathogenesis, histopathological results and responses to intravenous immune globulin, distinguishing from typical CIDP. The predominant subclass of IgG among pathogenic antibodies is IgG4, meanwhile, other subclasses have been rarely reported. Early detecting the APN related antibodies and their subclasses not only helps to clarify the diagnosis, but also provides valuable clinical information for the selection of precise treatment and prognosis.

Chronic inflammatory demyelinating polyneuropathy (CIDP) with positive anti-contactin-associated protein-1 (Caspr1) antibody is a rare autoimmune antibody mediated peripheral neuropathy. A 62-year-old male patient was reported in this article, whose clinical manifestations were subacute onset, abnormal distal limb motor sensation, and increased cerebrospinal fluid protein level. The patient had a good response to plasma exchange. Electromyography of lower limbs showed that motor involvement was dominant, motor conduction velocity slowed down, compound motor active potential (CMAP) and sensory nerve active potential amplitude decreased, and F wave was not elicited; electromyography of upper limbs without symptoms showed that CMAP amplitude of median nerve decreased, and conduction velocity was normal. There are few reports of anti-Caspr1 positive CIDP in the world. The article summarized the characteristics of the patient and reviewed the relevant literature, in order to improve clinicians′ understanding and diagnosis and treatment ability of the disease.

Anti-contactin-1(CNTN1) IgG4 antibody is a reliable biomarker of a specific subset of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients with distinct clinical features, including advanced age, aggressive symptom, predominantly motor involvement, sensory ataxia, and poor response to intravenous immunoglobulin. Nerve conduction study showed decrease of nerve conduction velocity, reduction of compound motor active potential amplitude without temporal dispersion at early stage of the disease. The article reported an anti-CNTN1 IgG4 antibody positive case, reviewed the current relevant literature, and then summarized the clinical characteristics of anti-CNTN1 IgG4 antibody associated CIDP.

Objective To investigate the correlation between plasma homocysteine level and impaired glucose tolerance(IGT) patients in peripheral neuropathy.Methods 80 patients with IGT were selected according to the results of routine nerve conduction test,including 40 patients associated with peripheral neuropathy (IGT-PN),and 40 patients without peripheral neuropathy (IGT-NPN).Besides,40 healthy subjects were selected as control.Plasma homocysteine levels were measured in the three groups by enzyme rate method.The severity of neuropathy was scored and graded by the Toronto Clinical Scoring System (TCSS).Results Plasma homocysteine levels were significantly higher in the all IGT groups than those in the control group.The plasma homocysteine level in the IGT-PN group (14.2±2.7) μmol/L was significantly higher than that in the IGT-NPN group (12.3±2.6) μmol/L (P＜0.05).Regression analysis showed that plasma homocysteine level had independent effects on IGT with peripheral neuropathy.Plasma homocysteine level was positively correlated with TCSS score.Conclusions Plasma homocysteine may play an important role in the pathogenesis of peripheral neuropathy in patients with IGT,and their level may be associated with the severity of peripheral neuropathy.

This study retrospectively reviewed 75 patients with impaired glucose tolerance(IGT)admitted in our hospital from March 2015 to October 2015.All patients underwent Toronto clinical scoring system(TCSS) evaluation.Patients with IGT were further divided into normal score group(TCSS-N, n=50)and abnormal score group(TCSS-A, n=25)according to their scoring results, and 30 healthy volunteers were served as control group.All patients and controls underwent motor and sensory nerve conduction studies, as well as sympathetic skin response(SSR)test using the Keypoint.Net(Medoc Ltd)electromyogram device.The results showed that the SSR amplitude of lower limbs was reduced [(0.61±0.44 vs 1.00±0.33)mv, P<0.05]andlatencyoflowerlimbswas extended [(1 880±282 vs 1 642±256)ms, P<0.05]in IGT group compared with control group.But median, ulnar, tibial, and peroneal nerve sensory and motor conduction revealed no difference between two groups.In TCSS-A group, the SSR amplitude of lower limbs was reduced [(0.47±0.39)mv, P<0.05], latency of lower limbs was extended [(2 062±291)ms,P<0.05]and the sensory nerve action potential(SNAP)amplitude of the tibial nerve was significantly lower compared with control group [(1.83±0.37 vs 2.07±0.30)μv, P<0.05].Compared to TCSS-N group, latency of lower limbs was extended [(2 062±291 vs 1 808±246)ms, P<0.05]in TCSS-A group.The SSR amplitude of lower limbs were reduced[(0.66±0.44)mv,P<0.05]and latency were prolonged(P<0.05)in TCSS-N group compared with control group.Pearson correlation analysis showed that the SSR amplitude and latency of the lower limbs were correlated with the postprandial blood glucose, blood glucose fluctuation, body weight, as well as body mass index.These results suggest that there exists peripheral nerve damage in the patients with IGT, mainly involving the small fiber nerve of the lower limbs.Large fibers may also be mildly affected as the disease progresses.

Objective To use nerve conduction study (NCS) to evaluate the function of large fibers,skin sympathetic response (SSR) and contact heat evoked potential (CHEP) to evaluate the function of small fibers in patients with impaired glucose regulation (IGR),and to analyze the occurrence of peripheral neuropathy and damage characteristics.Methods According to the classification criteria of glucose metabolism proposed by WHO in 2006,we selected 120 patients with IGR from January 2015 to December 2016 in our hospital,including 37 impaired fasting glucose (IFG) patients,83 impaired glucose tolerance (IGT) patients,and 60 normal subjects served as control group.All subjects received median,ulnar,tibial,peroneal,sural NCS,SSR and CHEP using the Keypoint.net (Medoc Ltd) electromyogram device.IGR patients were evaluated using the Michigan Neuropathy Screening Instrument (MNSI).Results The abnormal rate of MNSI score in IGR patients was 18.3％ (22/120);the abnormal rate of NCS was 22.5％ (27/120),and the abnormal rate of SSR was 39.2％ (47/120).In IFG group,the abovementioned abnormal rates were 8.1％ (3/37),13.5％ (5/37),29.7％ (11/37) respectively,and 22.9％ (19/83),26.5％ (22/83),43.4％ (36/83) in IGT group.Compared with control group,the tibial,sural nerve sensory nerve action potential amplitude decreased in IGT group (1.3 (0.1,1.9) μV vs 1.4(1.1,3.2) μV,Z=-3.05,P=0.002;(10.5±2.0)μV vs (7.6 ± 1.9)μV,t=0.60,P=0.001);and there was no significant difference between IFG patients and control group.Compared with control group,IFG patients' SSR amplitude reduced in lower limbs (0.7 (0.4,0.8) mV vs 0.8 (0.6,1.0) mV,Z =-2.95,P =0.003),CHEP amplitude decreased in dorsum hand and peroneal area stimuli ((63.0 ±10.0)μV vs(52.4 ±15.3)μV,t=0.61,P=0.003;(44.7 ±12.5)μV vs (28.2 ± 10.6)μV,t=0.31,P =0.000);and in IGR group,SSR amplitude reduced in upper and lower limbs (1.1 (0.5,2.2) mV vs 1.3(0.7,2.6)mV,Z=-2.12,P=0.030;0.4(0.2,0.8)mV vs 0.8(0.6,1.0) mV,Z=-5.96,P=0.000),CHEP amplitude decreased in dorsum hand and peroneal area stimuli ((63.0 ± 10.0) μV vs (38.7± 13.5)μV,t =0.37,P=0.000;(44.7 ±12.5)μV vs(21.9 ± 13.6)μV,t =0.35,P=0.000).Conclusions There is peripheral neuropathy in IGR patients,and the incidence of neuropathy in patients with IGT is higher than those with IFG.Neurophysiological methods are earlier than clinical scores to detect neuropathy.There are only small fiber damages in IFG patients,and IGT patients present by large and small fibrous lesions,mainly in small fibers and lower sensory nerve fibers,characterized by axonal damage and length dependence.

Objective To investigate the relationship between blood pressure variability (BPV) and neurological deteri?oration (ND) during the acute phase in patients with hypertensive minor ischemic stroke. Methods A total of 200 hyperten?sive patients with acute minor ischemic stroke were recruited in this study. Patients were divided into two groups: stable group (n=182) and deterioration group (n=18) according to the neurological prognosis. Values of BPV in 24 h ambulatory blood pressure, 24 h systolic blood pressure variation coefficient (24 h CVSBP), 24 h diastolic blood pressure variation coeffi?cient (24 h CVDBP), day time systolic blood pressure variation coefficient (dCVSBP), day time diastolic blood pressure variation coefficient (dCVDBP), night time systolic blood pressure variability (nCVSBP) and night time diastolic blood pressure variability (nCVDBP) were compared between two groups. The related factors of BPV were analyzed by binary logistic method in the acute phase of patients with hypertensive minor ischemic stroke. Results There were significantly higher levels of 24 h CVSBP [17.75%(17.54%,19.26%) vs 12.78% (10.67%,14.39%)], 24 h CVDBP [25.48%(20.77%,27.87%) vs 17.95% (14.88%, 21.46%)], dCVSBP [18.61%(17.65%,20.65%) vs 12.30%(10.10%,14.75%)], dCVDBP [25.65%(21.25%,29.78%) vs 17.76%(14.89%,22.19%)] in deterioration group than those of stable group (P<0.01). Results of binary logistic regression analysis showed that values of 24 h CVSBP and dCVSBP were risk factors for neurological deterioration in the acute phase of patients with hypertensive minor ischemic stroke. Conclusion The increased 24 h BPV and day time BPV are correlated with neurologi?cal deterioration during the acute phase in hypertensive minor ischemic stroke patients. BPV should be concerned in the acute phase and secondary prevention in patients with ischemic stroke.

Objective To investigate motor nerve function status in rats with diabetes mellitus by motor unit number estimation (MUNE), and discuss it′s early diagnostic value in diabetic peripheral neuropathy (DPN). Methods Diabetic rat model (DM group) was induced by streptozotocin. The MUNE of gastrocnemius muscle and motor nerve conduction (MCV, CMAP) of the sciatic nerve were measured at the 4th, 8th and 12th week after onset of hyperglycemia in the DM group and the control group (normal SD rats). The ultrastructure of sciatic nerve was observed by electron microscope. Results At the 4th week, MUNE of gastrocnemius muscle was significantly decreased in DM group compared to that of the control group (275.88 ± 87.87 vs 369.71 ± 75.64,P<0.05). There were no significant differences in MCV and CMAP of sciatic nerve be?tween two groups. The electron microscopy observation showed that most nerve fibers were normal;a small amount of axonal atrophy, and myelin lamellar structure was separated in DM group. At the 8th week, compared with the control group, MUNE were reduced in gastrocnemius muscle in DM group (357.49±72.68 vs 221.26±92.41, P<0.01). There were no significant dif?ferences in MCV and CMAP of the sciatic nerve between DM group and control group. The electron microscope observation showed that part of nerve fibers were normal, the myelin focal plate layer was loose and separated, axonal atrophy, the axonal membrane and myelin sheath inner layer was separated with big gap. At the 12th week, MUNE of gastrocnemius muscle (127.87±19.80 vs 366.85±51.25), sciatic nerve MCV [(35.06±4.43) m/s vs (50.47±6.07) m/s] and CMAP [(2.91±1.37) mV vs (5.98±2.14) mV] were significantly decreased in DM group than those of control group (P<0.01). The electron microscopy observation showed severely damaged myelin flex and axonal squeeze. Conclusion MUNE is much earlier in detecting ear?ly motor nerve dysfunction in DM than conventional motor nerve conduction test.

Objective To assess the function of motor nerve fiber in patients with diabetic peripheral neuropathy (DPN) by single-fiber conduction studies.Methods According to the diagnostic standard of DPN issued in Toronto meeting in 2009,on the basis of the result of peroneal nerve conventional conduction study,a total of 65 patients with DPN in the Department of Endocrinology and the Department of Neurology of Tianjin Third Central Hospital from October 2012 to October 2013 were enrolled into the study,from whom 33 had abnormal sensory conduction (sensory-diabetic peripheral neuropathy group,S-DPN group),32 had abnormal sensory motor conduction (sensory motor-diabetic peripheral neuropathy group,SM-DPN group).Single-fiber conduction velocity (SF-CV) and single-fiber distal motor latency (SF-DML)were detected in all subjects.The obtained results were compared with the data from 34 healthy volunteers (control group).The relationship of SF-CV,SF-DML and the duration of diabetes mellitus,fasting glucose,HbA1 c was also studied in DPN patients.Results The SF-CV ((43.1 ± 3.6) m/s) was decreased in S-DPN group compared with control group ((47.5 ± 3.3) m/s,t =5.077,P ＜ 0.01).There were no significant differences in SF-DML ((3.6 ± 0.7) ms),motor nerve conduction velocity (MCV (49.5 ± 2.6)m/s) and DML ((3.4 ± 0.6) ms) in S-DPN group compared with that of control group ((3.4 ± 0.5) ms,(50.9 ± 3.5) m/s,(3.2 ± 0.5) ms,respectively).SM-DPN group had lower SF-CV ((35.2 ± 3.6)m/s,t =9.119,14.219),MCV ((40.9 ± 3.2) m/s,t =11.131,13.025) and increased SF-DML ((4.5±0.7) ms,t=5.692,7.231),DML ((4.2 ±0.7) ms,t=5.561,6.975) compared with the other two groups (P ＜0.01).SF-CV in DPN patients was negatively related to the diabetic duration (r =-0.340,P =0.006),while SF-DML had no correlation with duration of DM,fasting blood glucose and HbAlc.Conclusions Detection of SF-CV is easy to find early motor nerve dysfunction in DPN patients.SF-CV is decreased with the increasing duration of diabetes.

Objective To investigate the relationship between total homocysteine (tHcy) and carotid intima-media thickness (CIMT) in brain infarction patients. Methods Sixty patients with fasting plasma tHcy levels ≤10μmol/L (non-Hhcy group), 60 patients with fasting plasma tHcy levels>10μmol/L and≤15μmol/L (H1 group), and 60 patients with fast-ing plasma tHcy levels>15μmol/L (H2 group) were chosen in hospitalized patients with acute cerebral infarction. Values of CIMT were detected in three groups of patients. The clinical biochemical indicators including triglyceride (TG), total choles-terol (TC), high density lipoprotein cholesterol (HDL-C), low density lipoprotein cholesterol (LDL-C), fasting blood sugar (FBS), folic acid (FA), Vitamin B12 (VitB12) and glycated hemoglobin (HbA1c) were also detected. Results There was signifi-cant difference in CIMT between three groups (P<0.01). The value of CIMT increased in H2 group [0.98(0.90, 1.05)mm] com-pared with that of non-Hhcy group [0.85(0.80, 0.95)mm]. The value of CIMT increased in H2 group compared with H1 group [0.98(0.90, 1.05)mm vs 0.85(0.85, 0.95)mm], P<0.05). There were significant differences in tHcy, FA and VitB12 between three groups. Based on the log-transformed values of CIMT as the dependent variable, multiple stepwise linear regression showed significant associations of the following variables with increased CIMT: increasing age, the history of smoking, the history of diabetes, higher LDL-C and tHcy levels. Conclusion Brain infarction in patients with higher tHcy level often have lower levels of FA and VitB12, and increased CIMT. When the level of tHcy >15 μmol/L, there is more significantly higher level of CIMT. The increased CIMT level was associated with some cerebrovascular risk factors in patients with brain infarction.