Electric Stimulation ; Humans ; Polysomnography ; Sleep Apnea, Obstructive ; therapy

ObjectiveTo investigate the clinical and pathological characteristics of dermatomyositis with muscular perifascicular atrophy (PFA).MethodsA series of 104 consecutive patients clinically and pathologically diagnosed as dermatomyositis by muscle biopsy in our laboratory from December,2003 to August,2011,were enrolled in this study. Muscle biopsy of all the enrolled patients had shown PFA of muscle fibers.ResultsAmong the 104 patients,34 were males and 70 were females with a mean age of 45 years old.Among them,8 cases had normal electromyogram;42 had normal serum creatine kinase level;11 were diagnosed as carcinoma;75 were found to be combined with interstitial lung disease (ILD).Based on morphologic changes of muscle biopsy,they were divided into pure PFA group with 54 cases and PFA plus focal damage group with 50 cases.Compared with the pure PFA group,there was prominent mononuclear cell infiltration into perimysial intermediate sized vessels and membrane attack complement (MAC) deposition in the intramuscular capillaries in the PFA plus group.Skin biopsy had been taken in 12 cases together with muscle biopsy and had shown the border effectof both PFA and interface dermatitis in muscle and skin.ConclusionsOur study suggests that chronic immune vascular damage and insufficiency in dermatomyositis may cause ischemia and focal myofiber damage in watershed regions. The incidence of ILD in our dermatomyositis patients with PFA is high.

ObjectiveIn an attempt to clarify the usefulness of combined nerve and muscle biopsy in the diagnosis of neuromuscular disease when compared with traditional sural nerve biopsy.Methods Fifteen biopsies of superficial peroneal nerve (SPN) and peroneus brevis muscle ( PBM ) by one incision performed within one neurological clinic were reviewed.All patients had peripheral neuropathy while 3 of them had myopathy clinically.The diagnostic significance of SPN and PBM biopsies were classified into 3 grade: essential,helpful,no value.ResultsOf 15 SPN and PBM biopsies,7 showed essential pathological findings whichreachedthe etiologicaldiagnosis, including 5definitevasculitis, 1inflammatory demyelinating polyneuropathy and 1 amyloid neuropathy.Five biopsies are helpful for etiological diagnosis,including demyelinating neuropathy,mild inflammation,and microvascular lesion,et al.Three biopsies are of no value for etiological diagnosis which only have nonspecific change such as type 2 fiber atrophy,neurogenic atrophy and axonal degeneration et al. Finally,SPN and PBM biopsies made the definite etiological diagnosis possible in 12 patients.ConclusionsSPN and PBM biopsy improved the yield of specific pathological and etiological diagnosis of neuropathy and myopathy such as vasculitis and amyloidosis with minor trauma and side effect.Further clinical and pathological studies will be necessary for a better practice of combined nerve and muscle biopsy.

Objective To summarize the clinical and pathological features of glycogen storage disease (GSD) type Ⅱ. Methods The clinical and pathological data of the 20 GSD type Ⅱ patients were reviewed. Results One patient with infantile-onset mainly presented hypotonia, muscle weakness, feeding difficulties, pulmonary infection and cardiomyopathy insufficiency and increase of serum creatine kinase (778 IU/L) and echographic evidence of hypertrophic cardiomyopathy were detected. Electromyography studies indicated a definite myopathy. Nineteen cases were late-onset, presenting a slowly progressive proximal myopathy with truncal involvement or with symptoms dominated by respiratory insufficiency. Not all muscles were equally affected. Increase of serum creatine kinase (208-2600 IU/L) was detected in 14 patients and normal level in 1 patient. Electromyography studies indicated a definite myopathy in 9 patients,with abnormal irritability in 1 patient and susceptible in 4 patients and myotonic discharge in 1 patient and no abnormalities in 2 patients. Echographic evidence of thickening of the interventricular septum and pulmonary hypertension were detected in 2 patients respectively. The common light microscopic feature of all case was a vacuolar myopathy with high glycogen content and acid phosphatase activity in the vacuoles. Conclusions GSD type Ⅱ often presents slowly progressive myopathy which often affect the toro and respiratory muscles.In most patients the serum creatine kinase level is elevated slightly. Muscle biopsy is of use to make the definite diagnosis of this disease.

Objective To explore the effects of electroacupuncture on brain derived neurotrophic factor (BDNF) of rats with sciatic nerve injury (SNI); To discuss its biological mechanism for treatment of SNI. Methods Fifty adult male Wistar rats were chosen, and the sciatic nerves of rats were cut off and pulled on both sides of the cut ends into nerve regeneration chamber. The rats were randomly divided into normal group, sham-operation group, model group, and electroacupuncture group. In the electroacupuncture group, the rats were treated by electroacupuncture for 28 days. After the treatment, the nerve regeneration was observed through HE staining. Immunofluorescence was used to analyze the expression changes of BDNF in the nerve tissue and spinal cord. ELISA was used to observe the changes of expression of serum BDNF. Results The amount of axon regeneration in the electroacupuncture group was obviously more than that in the model group, and the outline of the tissue more clear. Electroacupuncture could promote the expression of BDNF in the nerve, spinal cord and serum of SNI of rats compared with model group (P<0.01). Conclusion Electroacupuncture can promote the repairment and regeneration of SNI in rats by upregulating the expression of BDNF.

Objective To find out a reference range of epidermal nerve fiber density in normal humans and compare the concordance between clinical features, electrophysiology and the results of skin biopsy. Methods Fifty-one patients with peripheral neuropathy and 10 normal controls were studied. Skin biopsies were obtained from distal leg and/or proximal leg and nerves identified using immunohistochemistry with antibody against protein gene product (PGP) 9. 5. Forty-one in 51 performed routine nerve conduction vdocity and electromyography, 21 in 51 performed sympathetic skin response(SSR). The concordance of the consequences was compared. Results Intraepidermal nerve fiber density (IENFD) was (21.4 + 2. 7) /mm in thigh and (15.4±2. 2) /mm in the distal part of the leg in normal controls. IENFD was (15.0± 6. 3)/mm and (8. 1±5.9) /mm in patients. The intraepidermal nerve fiber density was significantly lower in the patients than in the normal controls both in proximal (t = 2. 976, P = 0.004) and distal legs (t= 3.191, P=0.002). Forty-eight out of 51 patients showed abnormalities in skin biopsy, among which 33 patients had length-dependent neuropathy. In the group of abnormal skin biopsy, 41 received routine electrophysiology, among which 21 (51.2%) were abnormal and they were performed SSR, turning out that 17 (81.0%) were abnormal. In 29 patients who had only small fiber neuropathy, 27(93. 1%) showed abnormalities in skin biopsy, out of whom 20 were performed routine electromyography, and it identified that 6 (30. 0%) were abnormaL In 14 receiving SSR, 11 were abnormal. Conclusion Skin biopsy is safe and tolerable, which has a higher sensitivity especially in small fiber neuropathy.

Objective To investigate the value of the immunohistochemistry and Western blot in the diagnosis of the benign muscular dystrophy with abnormal dystrophin expression.Methods The medical histories and clinical manifestations of 4 patients were collected.In addition to routine histological and histochemical studies,expression of dystrophin in muscle fibets was observed by immunohistochemical reaction(dys-N,dys-R and dys-C)and Western blot to anti-dystrophin antibody.Results Two patients had muscular weakness while another 2 patients had only muscular pain and elevated creatine kinase blood levels without muscular weakness.Histochemical stains showed atrophy,hypertrophy and fiber splitting in 2 patients,while only variation in fiber size was presented in anothor 2 patients.One patient had no reaction for dys-N,but had immunostains for dys-C and dys-R in the sarcolemma of muscle fibers.Western blot confirmed that the band of dys-C and dys-R was partly deficient,and the band of dys-N was absent compared with control.Three patients had no reaction for dys-R,but had immunostains for dys-C and dys-N.Compared with control,Western blot confirmed that the band of dys-R was absent,and the band of dys-C and dys-N were truncated.Conclusion The immunohistochemistry is stained with three anti-dystrophin antibodies to avoid diagnostic errors.Western blot is essential to further determine the type of dystrophin protein.

Post-traumatic stress disorder ( PTSD) is a serious psychiatric disorder when someone suffered from a major trauma, next followed by sleep disorder, emotional and cognitive disorder and other symptoms. Over the past few decades, many stress rodents models have been developed for searching the potential pathophysiological pathways of PTSD. All models showed PTSD-like symptoms, but none of them could manifestate all the symptoms and biological changes of PTSD completely. Thus, this article makes a brief summary about the PTSD models commonly used in recent years.

ObjectiveTo investigate the effects of modified Chunzetang on urinary retention in rats with spinal cord injury （SCI） and the c-Jun N-terminal kinase/p38 mitogen-activated protein kinase （JNK/p38 MAPK） signaling pathway. MethodBefore modeling， 10 of the 70 female SD rats were randomly selected to assign to the blank group， and 10 to the sham group. The remaining 50 rats were used to prepare a SCI-induced urinary retention model using the spinal cord transection method. The model rats were randomly divided into model group， low-dose modified Chunzetang group， high-dose modified Chunzetang group， and inhibitor group. After modeling， the blank group， sham group， and inhibitor group were given 2 mL of saline by gavage. The high-dose and low-dose groups of modified Chunzetang were given modified Chunzetang at 28.8 g·kg^-1 and 14.4 g·kg^-1 by gavage， respectively. The inhibitor group was injected intraperitoneally with the JNK inhibitor SP600125 twice a week at a dose of 15 mg·kg^-1. All rats were gavaged for a total of 28 days. Urodynamic and bladder muscle tension tests were conducted to evaluate bladder function. Hematoxylin-eosin （HE） staining was performed to observe the morphology of bladder smooth muscle tissue. Enzyme-linked immunosorbent assay （ELISA） was used to measure the levels of JNK， phosphorylated （p）-p38 MAPK， B cell lymphoma-2 （Bcl-2）， and cysteinyl aspartate-specific proteinase-3 （Caspase-3）. Western blot was used to detect the expression levels of p-JNK， p-p38 MAPK， ETS-like protein-1 （ELK-1）， and activator protein-1 （AP1） in the detrusor muscle. Immunofluorescence was used to detect the expression levels of p-JNK， p-p38 MAPK， and AP1. Terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling （TUNEL） assay was conducted to measure cell apoptosis. ResultCompared with blank group and sham group， the model group showed a significant increase in maximum bladder capacity and bladder compliance， and a significant decrease in leak point pressure. The minimum contraction force was increased， and the contraction frequency was significantly decreased （P<0.01）. The structure of bladder smooth muscle was disordered， with a large number of vacuolar cells， tissue edema， mononuclear cell infiltration， obvious hemorrhage， and a trend towards fibrosis in connective tissue. TUNEL positive cells increased significantly. The protein expression levels of p-JNK， p-p38 MAPK， AP1， and ELK-1 were significantly increased （P<0.01）. Compared with model group， all intervention groups showed significant improvement in urodynamic and bladder muscle contraction tests. In the low-dose modified Chunzetang group， the levels of p-p38 MAPK and Caspase-3 was decreased （P<0.05，P<0.01）. The levels of JNK, p-p38 MAPK and Caspase-3 in the high-dose group were significantly decreased （P<0.01）， and the level of Bcl-2 was significantly increased （P<0.01）. The expression levels of p-JNK， p-p38 MAPK， and AP1 proteins were significantly reduced （P<0.01）， and ELK-1 protein expression was decreased （P<0.05）. The positive rate of p-JNK and AP1 receptors was significantly decreased （P<0.01）. The positive cell rate was significantly decreased （P<0.01）. The high-dose modified Chunzetang group was positioned between the low-dose group and the inhibitor group， with no significant difference compared to the inhibitor group. ConclusionModified Chunzetang can improve urinary retention in SCI and enhance the contraction force of bladder smooth muscle. This effect is related to the inhibition of the JNK/p38 MAPK signaling pathway activation， thereby reducing apoptosis of bladder smooth muscle cells.

Chronic migraine is one of neurological disorders with high rate of disability, but sufficient attention has not been paid in this field. A large number of clinical studies have shown traditional Chinese acupuncture is a kind of effective treatment with less side effects. Through the analysis of literature regarding acupuncture and migraine published from 1981 to 2017 in CNKI and PubMed databases, the mechanism of neural plasticity of acupuncture on chronic migraine was explored. It was believed the progress of chronic migraine involved the changes of neural plasticity in neural structure and function, and the neural plasticity related with neural sensitization during the process of chronic migraine was discussed from three aspects of electrophysiology, molecular chemistry and radiography. Acupuncture could treat and prevent chronic migraine via the mechanism of neural plasticity, but there was no related literature, hindering the further spreading and development of acupuncture for chronic migraine.