Objective:To explore the split-face phenomenon in patients with bulbar-involved amyotrophic lateral sclerosis (ALS) through clinical and electrophysiological studies.Methods:A total of 52 clinically definite and clinically probable cases of bulbar-involved ALS, diagnosed according to the World Federation of Neurology El Escorial criteria, were retrospectively collected in the Third Central Hospital of Tianjin from September 2019 to November 2022. And 58 patients with idiopathic facial nerve paralysis with onset time≤7 days who visited the Department of Neurology of the Third Central Hospital of Tianjin during the same period were collected as control group. The firm eye closure (FC) score and cheek bulge (CB) score were used to assess the clinical involvement of facial muscles (dividing into facial muscle involvement group and non-facial muscle involvement group) and the presence of the split-face phenomenon (strong eye closure and weak cheek bulging) in ALS patients. The compound muscle action potential (CMAP) amplitudes of the bilateral orbicularis oculi and orbicularis oris muscles were measured using the Nicolet EDX Viking electromyography/evoked potential system. The CMAP amplitude ratio was calculated. The facial nerve electrophysiological differences were compared between ALS patients with bulbar involvement and patients with idiopathic facial nerve paralysis. The analysis of electrophysiological data across various groups was carried out utilizing the Kruskal-Wallis H test, while pairwise comparisons between groups were executed employing the Bonferroni correction method. Additionally, a stepwise binary Logistic regression analysis was implemented to ascertain the factors associated with facial muscle involvement in patients with bulbar-involved ALS. The receiver operating characteristic (ROC) curve was used to assess the diagnostic accuracy of facial nerve electrophysiological testing in diagnosing ALS in the presence of symptoms of facial muscle involvement. Results:Among the 52 ALS patients with bulbar involvement, there were 20 cases (38.5%) with facial muscle involvements, all of which were bilateral; 16 patients (30.8%) exhibited weakness solely in the ability to puff their cheeks, 1 patient (1.9%) presented with weakness exclusively in closing the eyes, and 3 patients (5.8%) experienced weakness in both closing the eyes and puffing the cheeks. The Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) score of the facial muscle involvement group was lower compared to the non-facial muscle involvement group (36.90±9.20 vs 40.75±5.21, t=2.419, P=0.019), while the FC score and CB score were higher in the facial muscle involvement group [FC score: 0(0, 1) vs 0(0, 0), U=5.854, P<0.001; CB score: 4(3, 4) vs 0(0, 0), U=9.069, P<0.001], showing statistically significant differences. There was no statistically significant difference in the CMAP amplitude of the orbicularis oculi muscle between the facial muscle involvement group and the healthy side of the idiopathic facial nerve paralysis group, the affected side of the idiopathic facial nerve paralysis group, and the non-facial muscle involvement group (all P>0.05). The CMAP amplitude of the orbicularis oris muscle in the facial muscle involvement group [1 100.00 (775.00, 1 375.00) μV] was lower than that in the healthy side of the idiopathic facial nerve paralysis group [1 800.00 (1 400.00, 2 300.00) μV] and the non-facial muscle involvement group [1 555.00 (1 202.50, 1 980.00) μV], with statistically significant differences ( H=5.884, P<0.001; H=4.114, P<0.001). There was no statistically significant difference in the CMAP amplitude of the orbicularis oris muscle between the facial muscle involvement group and the affected side of the idiopathic facial nerve paralysis group ( P>0.05). The CMAP amplitude ratio of the orbicularis oculi/orbicularis oris muscles in the facial muscle involvement group [0.83(0.51, 1.14)] was higher than that in the healthy side of the idiopathic facial nerve paralysis group [0.55(0.39, 0.73)], the affected side of the idiopathic facial nerve paralysis group [0.57(0.40, 0.73)], and the non-facial muscle involvement group [0.60(0.42, 0.71)], with statistically significant differences ( H=-3.440, P=0.003; H=-3.433, P=0.004; H=-3.225, P=0.008). Logistic regression analysis revealed that the CMAP amplitude of orbicularis oris muscle ( OR=0.998,95% CI 0.997-0.999, P<0.001) and ALSFRS-R score ( OR=0.916,95% CI 0.857-0.979, P=0.010) were factors associated with facial muscle involvement in ALS patients with bulbar involvement. The ROC curve analysis results showed that the area under the curve (AUC) of the orbicularis oculi muscle CMAP was 0.629, the AUC of the orbicularis oris muscle CMAP was 0.838, and the AUC of the CMAP amplitude ratio of the orbicularis oculi/orbicularis oris muscles was 0.690 in the facial muscle involvement group. Conclusions:Patients with bulbar-involved ALS have split-face phenomenon characterized by strong eye closure and weak cheek bulging. When bulbar-involved ALS patients have symptoms of facial muscle involvement, the CMAP amplitude of the orbicularis oris muscle decreases significantly, whereas the CMAP amplitude of the orbicularis oculi muscle remains relatively stable, further illustrating the split phenomenon.

Objective:To analyze the clinical and electrophysiological characteristics of patients with sensory neuronopathies (SNN), and to evaluate the significance of electrophysiological markers in the diagnosis and assessment of disease progression.Methods:A retrospective analysis was performed to evaluate the clinical manifestations, electrophysiological characteristics, and spinal cord magnetic resonance imaging (MRI) features of 8 cases diagnosed with SNN at the Third Central Hospital of Tianjin between 2015 and 2023. The neurophysiological examination mainly included limb nerve conduction study (NCS), same core needle electrode electromyography, somatosensory evoked potential (SEP), skin sympathetic reflex (SSR), and contact heat evoked potential (CHEP).Results:Among the 8 cases with SNN, 7 cases exhibited asymmetrical onset and a non-length-dependent pattern. All the 8 cases presented with severe deep sensory ataxia, accompanied by superficial sensory abnormalities and tendon areflexia. Paraneoplastic SNN were the most prevalent etiological subtype (4 cases), all of whom presented peripheral neuropathy as the initial symptom. Among these 4 cases, malignancies were identified in 3 cases and 3 cases presented with anti-Hu antibodies. Among the remaining 4 patients, 2 cases were autoimmune and the other 2 cases were idiopathic. NCS results of the 8 cases revealed decrease or absence of sensory nerve action potential (SNAP) amplitudes, with normal sensory conduction velocities. Six cases showed abnormal SEP, including 2 cases of central damage and 4 cases of peripheral damage, 5 cases had abnormal SSR, and 2 cases exhibited abnormal CHEP. Motor nerve conduction studies were normal in all 8 cases. Six patients underwent spinal MRI, and 4 exhibited abnormal signals in dorsal columns.Conclusions:The hallmark clinical manifestation of SNN is sensory ataxia, characterized by substantial impairment of superficial sensation, typically manifesting in a non-length-dependent distribution. Beyond the widespread and significant reduction in SNAP amplitudes, SNN may also exhibit additional electrophysiological impairments, such as those observed in SEP, SSR and CHEP.SEP combined with spinal cord MRI can improve the detection rate for damages in the central sensory conduction pathway.

Objective:To explore the split-face phenomenon in patients with bulbar-involved amyotrophic lateral sclerosis (ALS) through clinical and electrophysiological studies.Methods:A total of 52 clinically definite and clinically probable cases of bulbar-involved ALS, diagnosed according to the World Federation of Neurology El Escorial criteria, were retrospectively collected in the Third Central Hospital of Tianjin from September 2019 to November 2022. And 58 patients with idiopathic facial nerve paralysis with onset time≤7 days who visited the Department of Neurology of the Third Central Hospital of Tianjin during the same period were collected as control group. The firm eye closure (FC) score and cheek bulge (CB) score were used to assess the clinical involvement of facial muscles (dividing into facial muscle involvement group and non-facial muscle involvement group) and the presence of the split-face phenomenon (strong eye closure and weak cheek bulging) in ALS patients. The compound muscle action potential (CMAP) amplitudes of the bilateral orbicularis oculi and orbicularis oris muscles were measured using the Nicolet EDX Viking electromyography/evoked potential system. The CMAP amplitude ratio was calculated. The facial nerve electrophysiological differences were compared between ALS patients with bulbar involvement and patients with idiopathic facial nerve paralysis. The analysis of electrophysiological data across various groups was carried out utilizing the Kruskal-Wallis H test, while pairwise comparisons between groups were executed employing the Bonferroni correction method. Additionally, a stepwise binary Logistic regression analysis was implemented to ascertain the factors associated with facial muscle involvement in patients with bulbar-involved ALS. The receiver operating characteristic (ROC) curve was used to assess the diagnostic accuracy of facial nerve electrophysiological testing in diagnosing ALS in the presence of symptoms of facial muscle involvement. Results:Among the 52 ALS patients with bulbar involvement, there were 20 cases (38.5%) with facial muscle involvements, all of which were bilateral; 16 patients (30.8%) exhibited weakness solely in the ability to puff their cheeks, 1 patient (1.9%) presented with weakness exclusively in closing the eyes, and 3 patients (5.8%) experienced weakness in both closing the eyes and puffing the cheeks. The Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) score of the facial muscle involvement group was lower compared to the non-facial muscle involvement group (36.90±9.20 vs 40.75±5.21, t=2.419, P=0.019), while the FC score and CB score were higher in the facial muscle involvement group [FC score: 0(0, 1) vs 0(0, 0), U=5.854, P<0.001; CB score: 4(3, 4) vs 0(0, 0), U=9.069, P<0.001], showing statistically significant differences. There was no statistically significant difference in the CMAP amplitude of the orbicularis oculi muscle between the facial muscle involvement group and the healthy side of the idiopathic facial nerve paralysis group, the affected side of the idiopathic facial nerve paralysis group, and the non-facial muscle involvement group (all P>0.05). The CMAP amplitude of the orbicularis oris muscle in the facial muscle involvement group [1 100.00 (775.00, 1 375.00) μV] was lower than that in the healthy side of the idiopathic facial nerve paralysis group [1 800.00 (1 400.00, 2 300.00) μV] and the non-facial muscle involvement group [1 555.00 (1 202.50, 1 980.00) μV], with statistically significant differences ( H=5.884, P<0.001; H=4.114, P<0.001). There was no statistically significant difference in the CMAP amplitude of the orbicularis oris muscle between the facial muscle involvement group and the affected side of the idiopathic facial nerve paralysis group ( P>0.05). The CMAP amplitude ratio of the orbicularis oculi/orbicularis oris muscles in the facial muscle involvement group [0.83(0.51, 1.14)] was higher than that in the healthy side of the idiopathic facial nerve paralysis group [0.55(0.39, 0.73)], the affected side of the idiopathic facial nerve paralysis group [0.57(0.40, 0.73)], and the non-facial muscle involvement group [0.60(0.42, 0.71)], with statistically significant differences ( H=-3.440, P=0.003; H=-3.433, P=0.004; H=-3.225, P=0.008). Logistic regression analysis revealed that the CMAP amplitude of orbicularis oris muscle ( OR=0.998,95% CI 0.997-0.999, P<0.001) and ALSFRS-R score ( OR=0.916,95% CI 0.857-0.979, P=0.010) were factors associated with facial muscle involvement in ALS patients with bulbar involvement. The ROC curve analysis results showed that the area under the curve (AUC) of the orbicularis oculi muscle CMAP was 0.629, the AUC of the orbicularis oris muscle CMAP was 0.838, and the AUC of the CMAP amplitude ratio of the orbicularis oculi/orbicularis oris muscles was 0.690 in the facial muscle involvement group. Conclusions:Patients with bulbar-involved ALS have split-face phenomenon characterized by strong eye closure and weak cheek bulging. When bulbar-involved ALS patients have symptoms of facial muscle involvement, the CMAP amplitude of the orbicularis oris muscle decreases significantly, whereas the CMAP amplitude of the orbicularis oculi muscle remains relatively stable, further illustrating the split phenomenon.

Objective:To analyze the clinical and electrophysiological characteristics of patients with sensory neuronopathies (SNN), and to evaluate the significance of electrophysiological markers in the diagnosis and assessment of disease progression.Methods:A retrospective analysis was performed to evaluate the clinical manifestations, electrophysiological characteristics, and spinal cord magnetic resonance imaging (MRI) features of 8 cases diagnosed with SNN at the Third Central Hospital of Tianjin between 2015 and 2023. The neurophysiological examination mainly included limb nerve conduction study (NCS), same core needle electrode electromyography, somatosensory evoked potential (SEP), skin sympathetic reflex (SSR), and contact heat evoked potential (CHEP).Results:Among the 8 cases with SNN, 7 cases exhibited asymmetrical onset and a non-length-dependent pattern. All the 8 cases presented with severe deep sensory ataxia, accompanied by superficial sensory abnormalities and tendon areflexia. Paraneoplastic SNN were the most prevalent etiological subtype (4 cases), all of whom presented peripheral neuropathy as the initial symptom. Among these 4 cases, malignancies were identified in 3 cases and 3 cases presented with anti-Hu antibodies. Among the remaining 4 patients, 2 cases were autoimmune and the other 2 cases were idiopathic. NCS results of the 8 cases revealed decrease or absence of sensory nerve action potential (SNAP) amplitudes, with normal sensory conduction velocities. Six cases showed abnormal SEP, including 2 cases of central damage and 4 cases of peripheral damage, 5 cases had abnormal SSR, and 2 cases exhibited abnormal CHEP. Motor nerve conduction studies were normal in all 8 cases. Six patients underwent spinal MRI, and 4 exhibited abnormal signals in dorsal columns.Conclusions:The hallmark clinical manifestation of SNN is sensory ataxia, characterized by substantial impairment of superficial sensation, typically manifesting in a non-length-dependent distribution. Beyond the widespread and significant reduction in SNAP amplitudes, SNN may also exhibit additional electrophysiological impairments, such as those observed in SEP, SSR and CHEP.SEP combined with spinal cord MRI can improve the detection rate for damages in the central sensory conduction pathway.

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.

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 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.