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Objective To summarize the clinical characteristics of peripheral nerve hyperexcitability syndrome (PNHS). Methods and Results Ten patients with PNHS were admitted to He'nan Provincial People's Hospital from October 2018 to April 2022, with the clinical phenotypes of Morvan syndrome in 5 cases, Issacs syndrome in 4 cases, and cramp-fasciculation syndrome (CFS) in one case. There were 10 cases with clinical manifestations of muscle twitching, 8 of dysautonomia, 7 of central nervous system symptoms, 7 of limb weakness, 6 of sensory deficits, and 2 of cramping and muscle stiffness. Five cases were positive for autoimmune antibodies. Two cases had MRI abnormalities located in the brainstem, left occipital lobe and right thalamus. Ten cases had EMG abnormalities, including afterdischarges of F-wave in 10 cases, unit potential of spontaneous motion abnormalities in 10 cases, fasciculation potentials in 10 cases, myokymic discharges in 8 cases, and neuromyotonic and myokymic discharges in 3 cases. Three cases underwent only symptomatic therapy, and 7 cases underwent symptomatic therapy and immunomodulatory treatments. Except for one case who lost of follow-up, 5 cases had a good prognosis, 2 cases with poor prognosis, and 2 cases died. Conclusions PNHS is a heterogeneous group of clinical manifestations. Electrophysiological appearance of afterdischarges may be a sensitive indicator for the diagnosis of PNHS. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Clinical Nursing in Practice is the property of Journal of Clinical Nursing in Practice (Editorial Board, Shanghai Jiao Tong University Press) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Functional limb weakness is a common functional motor disorder, and its clinical diagnosis is mainly based on the medical history, neurological examinations and patients' positive signs. It is prone to misdiagnosis in the clinical practice, treatment requires diagnostic interpretation, physical therapy, and psychological therapy in a multi-disciplinary team pattern. This article reviews the progress in clinical manifestations, diagnosis, differential diagnosis, and the treatment of functional limb weakness, to help clinicians diagnose and treat functional limb weakness effectively in clinical practice, improving patients' prognosis. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Clinical Nursing in Practice is the property of Journal of Clinical Nursing in Practice (Editorial Board, Shanghai Jiao Tong University Press) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Clinical Nursing in Practice is the property of Journal of Clinical Nursing in Practice (Editorial Board, Shanghai Jiao Tong University Press) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Clinical Nursing in Practice is the property of Journal of Clinical Nursing in Practice (Editorial Board, Shanghai Jiao Tong University Press) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Clinical Nursing in Practice is the property of Journal of Clinical Nursing in Practice (Editorial Board, Shanghai Jiao Tong University Press) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective To summarize the clinical, muscle pathology and molecular biological features of late-onset glycogen storage disease type II (GSD II). Methods and Results Five patients with late - onset GSD II diagnosed and treated in The Fifth Affiliated Hospital of Zhengzhou University from January 2013 to January 2020 were selected. The main clinical manifestations of 5 patients were weakness of raising the head, weakness of the proximal extremities, decreased muscle tone, fatigue intolerance, and dyspnea. The activity of acid α-glucosidase (GAA) was significantly reduced. HE staining of muscular tissues in 5 patients showed vacuole-like changes of different sizes, different numbers, and irregular shapes in most muscle fibers. Modified Gomori trichrome (MGT) staining showed a large number of blue and purple particles deposited in the vacuole. Periodic acid-Schiff (PAS) staining showed the glycogen content in the vacuoles were increased in 4 cases, and the glycogen content in the vacuoles were lost in one case. GAA gene testing showed that 9 mutations were detected in 5 patients, and 4 cases were compound heterozygous mutations, which were derived from father and mother respectively. The c.1320_1322delGAT (p.Met440del) was a deletion mutation, c.2331G > C (p.Thr777Thr) was a synonymous mutation, c.2237G > A (p.Trp746*) was a nonsense mutation, c.877G > A (p.Gly293Arg) was a missense mutation, c.2238G > C (p. Trp746Cys) was a missense mutation, c.784G > A (p.Glu262Lys) was a missense mutation, c.2014C > T (p. Arg672Trp) was a missense mutation, and c. 2332 - 2A > G was a splicing mutation. One case was homozygous mutation of c. 1432G > A (p. Gly478Arg), which originated from the mother. Among them, c. 2331G > C, c. 1432G > A and c. 2332 - 2A > G were reported for the first time at home and abroad. Conclusions The clinical manifestations of late - onset GSD II are weakness of proximal limbs and dyspnea. The activity of GAA in peripheral serum is decreased significantly. Muscle tissue pathology is characteristic. GAA gene mutations are mainly compound heterozygous mutation, c.2331G > C, c.1432G > A and c.2332-2A > G are new mutations, which extended the mutation spectrum of GAA gene. [ABSTRACT FROM AUTHOR]

This study aims to explore the experience of assisting in caring for premature infants with periventricular leukomalacia after returning home. The care period was from May 7, 2018 to June 15, 2018. The parents participating in this study did not have prior experience of caring for premature infants. Issues with care were identified through data collection (e.g., observation, family interviews, and reviewing patient history records). After systematic physical assessment and evaluating the family functions of individual cases, confirmed care issues include invalid feeding methods towards infants/related to premature birth and uncoordinated oropharyngeal muscles; physical activity/musculoskeletal dysfunctions; and nervousness of the caretaker role/unpredictable care scenarios. Thus, personalized care plans were established to provide comprehensive overall care by guiding parents to learn the care information required once the premature infant return home. In addition, consultation channels for mouth and limb rehabilitation therapy were also established. This increased the ability and confidence of primary caregivers to allow individual cases to be successfully discharged and returns home to receive continued care. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Clinical Nursing in Practice is the property of Journal of Clinical Nursing in Practice (Editorial Board, Shanghai Jiao Tong University Press) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective To investigate the clinical and imaging differences between limb - girdle muscular dystrophy type 2B (LGMD2B) and immune - mediated necrotizing myopathy (IMNM). Methods Forty-five patients with LGMD2B and 45 patients with IMNM from January 2014 to December 2018 were included. Sex, age of onset, age of diagnosis and time of delay in admission were recorded, and serum creatine kinase (CK) level was measured. Cervical flexion, proximal and distal upper limbs, proximal and distal lower limbs were evaluated by Medical Research Council (MRC) Level 6 and muscle strength percentages were calculated. Skeletal muscle fatty infiltration and edema degree on both thighs were evaluated by MRI. Results The onset age (Z = -3.605, P = 0.000) and diagnosis age (Z = -2.757, P = 0.006) of LGMD2B patients were earlier than those of IMNM patients, while the delay time of admission was longer than that of IMNM patients (Z = -4.749, P = 0.000), the serum CK was higher than that of IMNM patients (Z = -2.247, P = 0.025). The muscle strength of neck flexion (Z = -6.320, P = 0.000) and percentage of muscle strength (t = -6.320, P = 0.000), the muscle strengh of proximal upper extremities (Z = -3.528, P = 0.000) and percentage of muscle strength (t = -3.762, P = 0.000) in IMNM patients were lower than those in LGMD2B patients, while the muscle strengh of distal lower extremity (Z = -3.256, P = 0.001) and percentage of muscle strength (t = -2.883, P = 0.004) in LGMD2B patients were lower than those in IMNM patients. LGMD2B patients had higher muscle fatty infiltration degree in anterior group (Z = -3.119, P = 0.002) and posterior group (Z = -2.534, P = 0.011) of thigh muscle than IMNM patients, but IMNM patients had highter thigh muscle edema degree in posterior group than LGMD2B patients (Z = -3.887, P = 0.000). Conclusions Compared with IMNM patients, LGMD2B patients had earlier onset age, longer delay in diagnosis time, worse muscle strength of distal lower extremities, higher serum CK and more obviously thigh muscle fatty infiltration. In IMNM patients, muscle weakness of neck flexion and proximal upper extremities, thigh muscle edema of posterior group were more obvious. These characteristics contribute to the clinical differentiation of the two diseases. [ABSTRACT FROM AUTHOR]

stroke patients. However, the percentage of completion of early limb rehabilitation among stroke patients is only 16%. Purpose: Raise the early rehabilitation intervention rate to 88% for patients with stroke within 24 hours of hospitalization. Resolution: We developed an education course on post-stroke rehabilitation and a related e-Learning course as well as organized an 'alliance for recovery' team. In addition, we established a standard for post-stroke relay rehabilitation and designed rehabilitation relay cards, Xbox rehabilitation games, and nine squares challenge for brain stroke care. Result: The accuracy of the knowledge of nursing staff related to physical rehabilitation improved from 72.4% to 100%; the accuracy of their perceptions regarding early limb rehabilitation increased from 16% to 100%; and patient satisfaction increased from 68% to 98%. Conclusions: We deployed diverse and innovative strategies to assist limb rehabilitation in patients with stroke. Patients and caregivers should be encouraged to participate in early rehabilitation and related programs and should apply the skills and rehabilitation activities learned to daily life. [ABSTRACT FROM AUTHOR]

Objective To summarize the clinical features and electrophysiological characteristics of stiff-person syndrome (SPS). Methods and Results Clinical data of 11 SPS patients [8 were classic and 3 were stiff-person plus syndrome (SPPS)], who were diagnosed and treated from August 2009 to November 2018, were retrospectively collected. They mainly presented paroxysmal muscle stiffness and pain spasm under stimulation or emotional excitement. The first affected sites were at trunk (4 cases) or limb (7 cases) muscles, and by the time of diagnosis, were mainly at truck muscles (8 cases). Anti - glutamic acid decarboxylase (GAD) antibody testing was positive in 6 cases. Electromyography (EMG) of all the patients showed continuous motor unit activity (CMUA) in the affected muscles at rest state. After treated by benzodiazepines, motor unit potential (MUP) obviously decreased or even disappeared in 6 patients. Conclusions Stiff-person syndrome is a rare autoimmune disease with multiple initial sites, atypical initial symptoms and difficulty in early diagnosis. It should be distinguished from many diseases such as familiar hyperekplexia, tetanus, myotonia congenita and multiple sclerosis (MS). [ABSTRACT FROM AUTHOR]

Dorsal root ganglia (DRG) neurons are the primary afferent neurons of algaesthesis of trunk and extremities. DRG transmits and accommodates sensations and accepts and conveys nociception, so it plays an important role in the mechanism of pain. DRG is accessible to clinical interventions for the control of intractable pain and is a target for neuromodulation therapy. This review focuses on sodium ion channels in DRG, glial cells and inflammatory reaction which are mainly involved in the mechanism of pain. [ABSTRACT FROM AUTHOR]

Objective To investigate the clinicopathological features of Li - Fraumeni syndrome (LFS) manifested as glioblastoma. Methods and Results A 33 - year - old female patient presented hyperspasmia of left extremities. Head MRI showed space - occupying lesion on the right fronto - parietal junction, and contrast - enhanced scanning revealed "garland" enhancement of the lesion. The patient underwent surgical resection. During the operation, an oval tumor was visible after the right parietal cortex was cut open for 2.50 cm, which was about 5 cm x 4 cm x 3 cm in size. The tumor was soft and purple, with clear boundary and rich blood supply, and was totally removed through piecemeal resection. Microscopic examination found obvious dysplasia of glial cells, spindle shape in some areas, and a large number of giant tumor cells. Immunohistochemical staining showed that the tumor cells expressed glial fibrillary acidic protein (GFAP) in cytoplasm and P53 protein in nuclei. Ki-67 labeling index was 25%. Reticular fiber staining was negative. Molecular pathological examination did not detect isocitrate dehydrogenase 1/2 (IDH 1/2) gene exon 4 mutation, telomerase reverse transcriptase (TERT) promoter mutation, or methylation of O6-methylguanine-DNA methyltransferase (MGMT). Fluorescence in situ hybridization (FISH) analysis did not reveal codeletion of 1p/19q. The integrated diagnosis was (right parietal) glioblastoma, IDH - wild type (WHO grade IV). The patient was hospitalized for 15 d and died in 2016. Previous family medical history showed her older sister suffered from right breast cancer, her father died of gastic cancer, her grandmother died of breast cancer, her uncles died of lung cancer and hematopoietic system tumor. Samples from the patient's glioblastoma and right mammary gland invasive duct carcinoma were collected for TP53 gene detection, but no mutation was found. Further, whole exome sequencing (WES) on the patient's freezing blood samples showed TP53 gene c.1009C > T (p.Arg337Cys) pathogenic heterozygous mutation (missense mutation). Combined with the family cancer history, a clinical diagnosis of the patient was Li-Fraumeni syndrome, and her family was Li-Fraumeni syndrome pedigree. Conclusions Li-Fraumeni syndrome is a hereditary tumor syndrome with autosomal dominant inheritance, characterized by high risk of breast cancer, bone and soft tissue sarcoma, brain tumor and adrenocortical cancer, and TP53 gene is the most common gene associated with Li-Fraumeni syndrome. [ABSTRACT FROM AUTHOR]

Objective To observe the rehabilitation effect of modified constraint-induced movement therapy (mClMT) on activities of daily living (ADL) in patients with acute ischemic stroke. Methods A total of 60 patients in acute stage of cerebral stroke with limb dysfunction admitted in Ruijin Hospital from December 2012 to May 2013, were randomly divided into routine rehabilitation treatment group (control group, N = 30) and mClMT group (N = 30). Control group was given routine rehabilitation training, 60 min each time, twice per day, 5 d per week; mClMT group received mClMT treatment with the similar frequency. After 2-week training, all patients were transferred to rehabilitation center for 4-week continous training. The total treatment period was 6 weeks. All of those patients received assessment before treatment and 2 weeks, 6 weeks, 12 weeks after treatment on the ability of daily living and motor function, including the modified Barthel Index (mBl), Fugl-Meyer Assessment (FMA) and Berg Balance Scale (BBS). Results Patients in both groups got increased mBl (P = 0.004, 0.000, 0.000), FMA (upper limb: P = 0.000, for all) and BBS scores (P = 0.005, 0.000, 0.000) 2, 6, 12 weeks after treatment. FMA (lower limb) score was increased 6 and 12 weeks after treatment (P = 0.000, for all). Compared to the control group, patients in mClMT group got increased mBl (P = 0.000, for all), FMA (upper limb: P = 0.000, for all; lower limb: P = 0.000, for all) and BBS scores (P = 0.000, for all) 2, 6, 12 weeks after treatment. Conclusions mClMT has positive therapeutic effects on the motor function of limbs in hemiplegic patients with stroke in acute stage, which can improve the balance ability and the ability of daily life, so as to raise the quality of life of patients. The efficacy of mClMT is superior to general routine rehabilitation treatment. [ABSTRACT FROM AUTHOR]

Objective To explore the effects of motor imagery (MI) combined with the third generation functional electrical stimulation (FES) on upper limb motor function in acute ischemic stroke patients with hemiplegia. Methods Forty acute ischemic stroke patients, within 48 h of onset, were randomly divided into FES group (N = 20) and combination group (FES combined with motor imagery, N = 20). All patients received basic routine rehabilitation training, for example, good limb positioning, accepting braces, balance training and training in the activities of daily living (ADL). FES group received the third generation FES therapy and the combination group also received motor imagery for 2 weeks. All of the patients were assessed with Fugl-Meyer Assessment (FMA), Action Research Arm Test (ARAT) and active range of motion (AROM) of wrist dorsiflexion before and after 2 weeks of treatment. Results After 2 weeks of treatment, the 2 groups had significantly higher FMA score, ARAT score and AROM of wrist dorsiflexion than that in pre-treatment (P = 0.000, for all). Besides, the FMA score (t = - 2.528, P = 0.016), ARAT score (t = - 2.562, P = 0.014) and AROM of wrist dorsiflexion (t = - 2.469, P = 0.018) in the combination group were significantly higher than that in the FES group. There were interactions of treatment methods with observation time points (P < 0.05, for all). Conclusions Motor imagery combined with the third generation FES can effectively promote the recovery of upper limb motor function and motion range of wrist dorsiflexion in patients with acute ischemic stroke. [ABSTRACT FROM AUTHOR]

Inspired by the body character of jellyfish and the swimming mode of caudal fin, a new-style efficient autonomous underwater vehicle tends to be developed. A kind of smart material-shape memory alloy wire is adopted as the actuator to imitate the muscle of jellyfish. Based on rebuilding the configuration of jellyfish and analyzing the swimming strategy, a bionic jellyfish robot prototype propelled by 6 bionic tentacle propulsors is developed. The robot is 230 mm in length, 110 mm in diameter, 590 g in weigh and contains power supply. The robot can reach 110 mm/s straight speed and 18°/s turning speed in wireless control. The swimming experiment demonstrates that the bionic swimmer has the merits of simple structure, easy to seal, good maneuverability, silent propulsion and biology friendly. The feasibility of taking shape memory alloy wires as bionic muscle material is verified. [ABSTRACT FROM AUTHOR]

Study age variation of circumference values and its difference of urban and rural adults of Yunnan Han, measure the circumferences values of Yunnan with 709 adults (356 males,353 females) including the 13 circumference values of head, neck, chest, inspiratory chest circumfirence values, expiratory chest circumference values, and the circumference values of waist, abdomen, hip, thigh, calf, biceps, fore-arm, Maximum biceps, the paper gives an analysis of the changes of circumference value with age, and then with cluster analysis draws a comparison of the circumference values between Yunnan han nan nationality, around circumference values of U-test and other 18 nationalities in China. The results show that: (1) The neck, trunk circumference values have significantly linearly increase with the age increasing. Urban males, urban females and rural females upper limbs circumference values also grows along with the age and apparent linear increase. (2) Between the age groups of circumference values differences of males mainly in 20~29 year group compared with other age groups, circumference values of urban females among different age groups of main performance in 20~29, 30~39 year group and other age groups; Circumference values of the rural females have difference in most age groups. (3) Circumference values of males and females is most not statistically significant in urban and rural difference. (4) Circumference values close to the northern population. [ABSTRACT FROM AUTHOR]

Background Limb remote ischemic per-conditioning (LRPC) has been recognized as an applicable strategy in protecting against cerebral ischemia - reperfusion injury. However, widely used invasive limb remote ischemic per-conditioning (LRPC-I) is traumatic, limiting the possibility of long-term application and making it more difficult to achieve the conversion from basic research to clinical practice. Because of this, if non-invasive limb remote ischemic per-conditioning (LRPC-N) has the same effect of brain protection as LRPC-I, it may be more beneficial to clinical practice. Methods The middle cerebral artery occlusion (MCAO) ischemia-reperfusion injury model was established by the suture method in mice. In the beginning of the ischemia, the LRPC-I and LRPC-N were respectively carried out by three cycles of 10 min distal airbag pressurization or clamping the distant limb artery (ischemia) /10 min reperfusion. Neurological functional deficits after procedure were evaluated, and the cerebral infarct volumes and the degree of cerebral edema were quantified by TTC staining. Results Compared with the control group, the neurological functional outcomes of LRPC - N group and LRPC -I group were improved significantly (P = 0.041, 0.035); the edema volumes were much smaller (P = 0.040, 0.028); the infarct volumes reduced significantly (P = 0.001, 0.019). However, there were no significant differences between LRPC-N group and LRPC-I group on the functional neurological outcomes, edema volumes and the infarct volumes (P = 0.754, 0.946, 0.667). Conclusion Both the LRPC-N and LRPC-I have protective effects on animal models of cerebral ischemia-reperfusion injury, and there is no obvious difference between each other. [ABSTRACT FROM AUTHOR]