In recent years,the results of epidemiological research findings on chronic inflammatory demyelinating polyneuropathy(CIDP)in patients with diabetes mellitus(DM)are quite different,and its correlation is still controversial.Nevertheless,due to the high incidence and treatability of CIDP in DM patients,accurate diagnosis and differentiation of DM-CIDP are of great value for guiding clinical drug use.In terms of clinical manifestations,it can be distinguished by age of onset,disease duration,blood glucose control level and main symptom characteristics.Supplementary diagnostic methods frequently involve evaluating cerebrospinal fluid protein levels,demyelination patterns in electrophysiological tests and neuroimaging studies.Additionally,neuropathological examination and specific serum antibodies can provide more diagnostic support for distinguishing DM-CIDP.Furthermore,a positive response to immunomodulatory and/or immunosuppressive therapies supports the diagnosis of DM-CIDP.By comprehensively evaluating clinical manifestations,electrophysiological characteristics,laboratory tests,neuropathology,imaging,and treatment responses,the accuracy of the differential diagnosis for DM-CIDP can be improved.

There are many causes of peripheral neuropathy, one of which is that caused by various therapeutic drugs, namely drug-induced peripheral neuropathy (DIPN). With the increasing number of cancer patients, chemotherapy-induced peripheral neuropathy (CIPN) is prominent. Most of DIPN showed chronic, sensory and axonal polyneuropathy, and numbness and neuropathic pain were the main symptoms. The medical history of drug exposure and related risk factors are the key to clinical diagnosis. DIPN caused by non-chemotherapy drugs must be stopped in time to prevent further dysfunction. For CIPN, no agents are recommended for the prevention; the appropriateness of dose delaying and reduction, substitutions or stopping chemotherapy should be evaluated; duloxetine is the only agent that has appropriate evidence to support its use, whereas the amount of benefit is limited. It is urgent to perform the multidisciplinary randomized clinical trial for the treatment of CIPN.

Background: and Purpose We aimed to determine the clinical features of Miller Fisher syndrome (MFS) in southern China and compare them with those presenting in other countries. Methods: We collected the medical records of patients diagnosed with MFS during 2013–2016.We analyzed the age, sex, onset season, precursor events, clinical symptoms and signs, findings of nerve conduction studies (NCS), cerebrospinal fluid (CSF), therapeutic remedies, nadir time, and length of hospital stay of patients with MFS in southern China. We concurrently compared the differences between urban and rural areas and between patients with incomplete ophthalmoplegia (IO) and complete ophthalmoplegia (CO). Results: The study enrolled 72 patients: 36 from rural areas and 36 from urban areas, and 50 males and 22 females. The mean age at onset was 47.72 years, and 30 (41.7%) and 21 (29.2%) patients developed MFS in spring and winter, respectively. The typical triad of ophthalmoplegia, ataxia, and areflexia was observed in 50 (69.4%) patients. A history of upper respiratory tract infection 1 week before onset was found in 52.8% of the patients, while 5.6% experienced gastrointestinal infections and 48 (73.8%) exhibited albuminocytological dissociation in the CSF study. Only 26 (36.1%) patients presented abnormalities in NCS. Moreover, restricted outward eyeball movement presented in 83.5% of the patients with classic MFS and acute ophthalmoplegia, and bilateral symmetrical ophthalmoplegia presented in 64.2%. With the exception of the higher proportion of NCS abnormalities in urban areas (47.2% vs. 25.0%), urban and rural differences were insignificant regarding sex ratio, age at onset, high-incidence season, precursor events, disease characteristics, and albuminocytological dissociation in the CSF. Furthermore, patients with CO were older than those with IO (64.53±7.69 vs. 43.19±14.40 years [mean±standard deviation], p<0.001). Conclusions The patients with MFS were mostly male and middle-aged, and most presented in winter and (especially) spring. More than half of the patients had clear precursor events, most of which were classic MFS with the typical triad. More than 70% of the patients presented albuminocytological dissociation in the CSF. NCS abnormalities were uncommon in MFS. The age at onset was lower in patients with IO than in patients with CO; bilateral symmetrical extraocular muscle paralysis was the most common symptom, and the external rectus was the most frequently involved muscle.

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a rare and early-onset neurodegenerative disease caused by variants of the SACS gene which maps to chromosome 13q11 and encodes sacsin protein. Sacsin is highly expressed in large motor neurons, in particular cerebellar Purkinje cells. This article has provided a review for the structure and function of sacsin protein and the mechanisms underlying abnormalities of sacsin in ARSACS disease.
Humans ; Spinocerebellar Ataxias/pathology* ; Ataxia/genetics* ; Muscle Spasticity/genetics*

Objective:To investigate the clinical characteristics of Guillain-Barré syndrome (GBS) combined with hyponatremia in Southern China and its risk factors for prognosis.Methods:The retrospective cohort study involved patients who met the diagnostic criteria of GBS from 18 upper first-class hospitals of 6 provinces/cities in southern China (south of Huaihe River) from January 1, 2013 to September 30, 2016. The clinical data of these patients were collected. According to serum sodium levels, they were divided into hyponatremia group (serum sodium concentration<135 mmol/L) and normal serum sodium group (serum sodium concentrations≥135 mmol/L). Based on Medical Research Coucil sum scores at nadir, these patients were divided into mild GBS group (>40), moderate GBS group (30-40), and severe GBS group (<30). Furthermore, according to the Hughes GBS disability scale (H-GBS-DS) scores at discharge, these GBS patients with hyponatremia were divided into favorable prognosis group (H-GBS-DS<3) and poor prognosis group (H-GBS-DS≥3). The incidence of hyponatremia in patients from the mild GBS group, moderate GBS group, and severe GBS group were compared. Multivariate Logistic regression analysis was performed to determine the clinical risk factors for hyponatremia in GBS patients. The clinical data of hyponatremia patients from favorable prognosis group and poor prognosis group were compared; multivariate Logistic regression analysis was used to determine the risk factors for poor prognosis in GBS patients with hyponatremia.Results:(1) Among the 570 patients, 354 had mild GBS, 94 had moderate GBS, and 122 had severe GBS; 134 GBS patients were combined with hyponatremia, 436 GBS patients had normal serum sodium. The hyponatremia incidence in mild, moderate and severe GBS groups increased successively, ( P<0.05). Multivariate Logistic regression analysis showed that facial paralysis ( OR=1.979, 95%CI: 1.172-3.342, P=0.011), respiratory muscle paralysis ( OR=3.218, 95%CI: 1.611-6.428, P=0.001), secondary pulmonary infection ( OR=4.822, 95%CI: 2.835-8.201, P=0.000), severe GBS ( OR=2.611, 95%CI: 1.444-4.721, P=0.001) and length of hospital stay ( OR=1.029, 95%CI: 1.009-1.050, P=0.004) were risk factors for hyponatremia in GBS patients. (2) Among 134 GBS patients with hyponatremia, 80 had poor prognosis and 54 had favorable prognosis. As compared with the favorable group, the poor prognosis group had significantly lower proportion of patients with extraocular muscle paralysis, statistically higher proportions of patients with respiratory muscle paralysis and secondary pulmonary infection, significantly different severities of GBS, signficantly higher proportion of patients accepted intravenous immunoglobulin (IVIG) and hormone treatments, statistically longer length of hospital stay ( P<0.05). Respiratory muscle paralysis ( OR=25.590, 95%CI: 9.433-69.423, P=0.000), moderate GBS ( OR=17.030, 95%CI: 8.441-34.361, P=0.000), and severe GBS ( OR=51.042, 95%CI: 24.596-105.926, P=0.000) were independent risk factors for poor short-term prognosis of GBS patients with hyponatremia. Conclusions:Severe GBS patients with facial paralysis, respiratory muscle palsy, secondary pulmonary infection, and long hospital stay trend to have hyponatremia. Hyponatremia patients with respiratory muscle paralysis and moderate/severe GBS have poor short-term prognosis.

Respiratory failure is the main causes of death in patients with amyotrophic lateral sclerosis (ALS). Detection of phrenic nerve conduction is an important method for objective assessment of respiratory function, which can make up for the limitations of pulmonary function test (PFT), and has a certain clinical value in improving the quality of life of ALS patients by early and effective measures. This article focuses on the neurophysiology and edetection methods of phrenic nerve conduction and its clinical application in ALS to improve the understanding of clinicians and better serve clinic.

Objective:To explore the changes of the auditory event-related potentials P300 and the Montreal Cognitive Assessment (MoCA) in the chronic mild lead poisoning in order to find out the impairment of cognitive function and intervene early.Methods:In February 2020, 50 patients with chronic mild lead poisoning in Wuhan Center for Prevention and Treatment of Occupational Diseases from June 2011 to June 2015 were selected as the case group, and 50 healthy people were selected as the control group. The changes of auditory event-related potential P300 and MOCA of the two groups were analyzed.Results:Compared with the control group, the latency of P300 of auditory event-related potential in the case group was prolonged and the amplitude was decreased ( P<0.05) . Compared with the control group, the total score of MoCA in the case group was decreased, the mean score of language, abstract and delayed memory items decreased, and the differences were statistically significant ( P<0.05) . Conclusion:The combination of auditory event-related potential P300 and MOCA is helpful to detect the early cognitive impairment in chronic lead poisoning population, and auditory event-related potential P300 is an objective and effective early detection method.

Objective:To explore the changes of the auditory event-related potentials P300 and the Montreal Cognitive Assessment (MoCA) in the chronic mild lead poisoning in order to find out the impairment of cognitive function and intervene early.Methods:In February 2020, 50 patients with chronic mild lead poisoning in Wuhan Center for Prevention and Treatment of Occupational Diseases from June 2011 to June 2015 were selected as the case group, and 50 healthy people were selected as the control group. The changes of auditory event-related potential P300 and MOCA of the two groups were analyzed.Results:Compared with the control group, the latency of P300 of auditory event-related potential in the case group was prolonged and the amplitude was decreased ( P<0.05) . Compared with the control group, the total score of MoCA in the case group was decreased, the mean score of language, abstract and delayed memory items decreased, and the differences were statistically significant ( P<0.05) . Conclusion:The combination of auditory event-related potential P300 and MOCA is helpful to detect the early cognitive impairment in chronic lead poisoning population, and auditory event-related potential P300 is an objective and effective early detection method.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a motor-sensory demyelinating neuropathy mediated by immunity. The pathogenesis is still unclear, and the clinical manifestations are complex. Its diagnoses are difficult, and the treatment effect is not good. At present, most studies consider it to be an autoimmune disease, but the pathogenic antigen and its target to the immune damage mechanism of the disease are still unclear. In this paper, we review the literature on the pathogenesis of CIDP in recent years, and introduce the specific mechanisms which cause CIDP from cellular and humoral immunity.

Objective:To explore the short-term prognostic factors for Guillain-Barré syndrome (GBS) in children.Methods:The clinical data of children with GBS from 24 hospitals in 10 provinces/municipalities/autonomous regions in southern China (south of Huaihe River) from January 1, 2013 to September 30, 2016 were retrospectively analyzed. The factors affecting the short-term prognoses of children were explored.Results:In these 78 children (50 males and 28 females), the average age was 9.53±5.44 years, and 19 were under 5 years old. Fifty children had history of prodromal events; 28 children had cranial nerve involvement, and 22 had autonomic nerve involvement. Five children needed assisted respiration, and one died during hospitalization. There was no statistically significant difference in percentage of children having poor short-term prognosis (scores of Hughes GBS disability scale≥3 at discharge) between children with different ages, children having different days from onset to admission, children with different clinical classifications or electrophysiological classifications, children with different treatment plans, children having presence or absence of prodromal events, children having presence or absence of cranial nerve involvement ( P>0.05). The proportion of children having poor short-term prognosis in children with autonomic nerve involvement was significantly higher than that of children without autonomic nerve involvement (31.8% vs. 10.7%, P<0.05). Conclusion:The short-term prognosis of children with autonomic nerve involvement is poor.