OBJECTIVETo study causes of ineffectiveness of microvascular decompression (MVD) in treatment of hemifacial spasm (HFS).

METHODSReoperative MVD was performed in 23 HFS patients with previous ineffective MVD. In the patients, the main causes of ineffectiveness included misjudgment of compressing vessels (7 patients), improper insertion of decompressing grafts (9), improper selection of grafts (5) and small grafts (2).

RESULTSSymptoms of HFS disappeared immediately after the second MVD in 21 patients and delayed in 2 patients (after 2 weeks, 6 weeks). No recurrence of HFS was noted during the follow-up period of 1.0 - 6.0 years (mean 3.4 years).

CONCLUSIONSMVD is an effective microsurgical method for treating HFS. Accurate judgement of compressing vessels and proper decompression are the key to surgical effectiveness.

Adult ; Decompression, Surgical ; methods ; Female ; Follow-Up Studies ; Hemifacial Spasm ; surgery ; Humans ; Male ; Medical Futility ; Microsurgery ; methods ; Middle Aged ; Reoperation ; Treatment Outcome ; Vascular Surgical Procedures ; methods ; Young Adult

OBJECTIVEThis study was sought to compare the sensitivity, specificity and accuracy of (1) dual isotope simultaneous acquisition single-photon emission computed tomography (DISA SPECT) myocardial image with (99m)Tc-sestamibi/(18)F-fluorodeoxyglucose ((99m)Tc-MIBI/(18)FDG); (2) low dose dobutamine alone and combined with Isosorbide Dinitrate (ISDN: Isoket) stress two dimensional echocardiography (2DE) to predict regional movement recovery after revascularization (CRV) in patients with old myocardial infarction (OMI) and severe left ventricular dysfunction.

METHODSTwenty-six patients (mean age 51 +/- 8 years, male 25, female 1) with OMI and severe left ventricular dysfunction (mean left ventricular ejection fraction, LVEF (38.6% +/- 4.9%) underwent low dose dobutamine 10 microg x kg(-1) x min(-1) (Dob10 microg) and ISDN (286 +/- 31 microg/min) combined with Dob5 microg (ISDN-Dob 5 microg) 2DE and DISA SPECT within one week. In echocardiogram and DISA SPECT images: the left ventricle (LV) was divided into 16 segments. The semi-quantitative scoring system was used for both images. Myocardial viability was defined as an improvement of at least >or= 1 grade in at least two contiguous segments at rest 2DE after CRV. The viable segments detecting rate with stress 2DE and DISA SPECT were compared. Compared with the results of post-CRV, the sensitivity, specificity and accuracy of detecting viable segments of two methods were calculated.

RESULTSAmong 272 abnormal segments in 26 patients, 156 (57.4%) segments showed contractile improvement after CRV. The viable segments detecting rate with DISA SPECT was 72.4% (134/254), which was significantly higher than the contractile improved rate after CRV (P < 0.001). During Dob10 microg 2DE and ISDN-Dob5 microg 2DE, the detecting rates were 65.5% (163/249) and 65.7% (176/268), respectively, which were both comparable to the improved rate after CRV (both P > 0.05). With DISA SPECT, the sensitivity, specificity and accuracy were 93.7%, 55% and 76.8%, respectively. Compared with DISA SPECT, Dob10 microg 2DE showed similar sensitivity (88.6%), specificity (64.2%) and the accuracy (77.9%). When ISDN combined with Dob5 microg, the sensitivity (91.4%), specificity (68.1%) and accuracy (81.4%)were comparable to those of Dob10 microg 2DE and DISA SPECT (all P > 0.05), while the specificity was even higher than DISA SPECT (P < 0.05).

CONCLUSIONIn identifying myocardial viability in patients with OMI and severe left ventricular dysfunction, DISA SPECT has higher sensitivity, lower specificity and better accuracy. Dob10 microg and ISDN-Dob5 microg 2DE are both equivalent to DISA SPECT in sensitivities, specificities and accuracies, and even higher in specificity in ISDN-Dob5 microg 2DE.

Adult ; Dobutamine ; Echocardiography ; methods ; Female ; Fluorodeoxyglucose F18 ; Humans ; Isosorbide Dinitrate ; Male ; Middle Aged ; Myocardial Infarction ; diagnostic imaging ; Myocardium ; Myocytes, Cardiac ; diagnostic imaging ; Sensitivity and Specificity ; Tomography, Emission-Computed, Single-Photon ; methods

OBJECTIVEThe aim of this study was to assess the accuracy of adenosine (99m)Tc-MIBI myocardial perfusion SPECT for the diagnosis of coronary artery disease.

METHODSA total of 89 patients [mean age (57 +/- 9) years,62 men, 27 women] were included in this study. Adenosine was infused intravenously at a rate of 140 microg.kg(-1).min(-1) for 6 minutes. At the end of 3 minute of adenosine infusion, 925MBq of (99m)Tc-MIBI was injected intravenously. SPECT myocardial imaging acquisition was obtained 1 hour after adenosine infusion. Coronary angiography was performed in all patients.

RESULTSThirty-one patients had significant coronary artery stenosis and 58 had normal coronary angiography. Adenosine myocardial perfusion imaging was abnormal in twenty-two out of the 31 patients with significant coronary artery stenosis (sensitivity, 71%). Fifty-three out of the 58 patients with normal angiography had a normal adenosine myocardial perfusion imaging (specificity, 91%). The positive predictive value and negative predictive value of adenosine (99m)Tc-MIBI myocardial perfusion imaging for detection of coronary artery disease was 81% and 84% respectively.

CONCLUSIONAdenosine myocardial perfusion imaging is probably an accurate method for detecting coronary artery disease.

Adenosine ; Adult ; Aged ; Coronary Artery Disease ; diagnostic imaging ; Female ; Humans ; Male ; Middle Aged ; Predictive Value of Tests ; Radionuclide Ventriculography ; Technetium Tc 99m Sestamibi ; Tomography, Emission-Computed, Single-Photon ; Young Adult

OBJECTIVETo explore the changes of mRNA and protein expressions of glycolytic and fatty acid metabolic enzymes early after acute myocardial ischemia.

METHODSTwelve dogs were randomly divided into 3 groups (sham, 20 min ischemia and 40 min ischemia, n = 4 each). Myocardial samples from ischemic and nonischemic zone were obtained for histology examination, and the mRNA expressions for Phosphofructokinase (PFK), Glyceraldehyde-3-phosphate dehydrogenase (GAPDH), GLUT1, GLUT4, Medium-chain acyl-CoA dehydrogenase (MCAD) and Heart-fatty acid binding protein (H-FABP) were determined by Real Time PCR-SYBR Green RT-PCR. GLUT1 protein expression was determined by immunohistochemistry. The apoptotic cardiomyocytes was evaluated by TUNEL.

RESULTSCompared to sham hearts, H-FABP mRNA was decreased in nonischemic and ischemic zone (P < 0.05) while GLUT1 mRNA expression was significantly increased in nonischemic and ischemic zone (P < 0.05) in dogs underwent 20 and 40 min ischemia. PFK mRNA tended to be higher in ischemic myocardium (P = 0.065) and GAPDH, MCAD as well as GLUT4 remained unchanged post ischemia (all P > 0.05). Positive GLUT1 protein staining was visualized in ischemic myocardium of hearts underwent 20 and 40 min ischemia. The myocardial apoptosis cells was 6.4% +/- 0.9% in sham hearts, 28.0% +/- 3.7% in hearts underwent 20 min ischemia (P < 0.05 vs. sham) and 38.4% +/- 1.9% in hearts underwent 40 min ischemia (P < 0.05 vs. sham).

CONCLUSIONSSignificant down and up-regulated glycolytic and fatty acid metabolic enzymes early after myocardial ischemia suggested that these enzymes might play an important role in acute myocardial ischemia.

Animals ; Disease Models, Animal ; Dogs ; Fatty Acids ; metabolism ; Glycolysis ; Myocardial Ischemia ; enzymology ; Myocardium ; enzymology ; RNA, Messenger ; genetics

Interventions for endometriosis(EMs)include surgical excision of lesions and hormonal therapy,which usually have limited efficacy and adverse drug reactions.Traditional Chinese medicine(TCM)has the multi-component and multi-target characteristics,which can help patients achieve good clinical benefits by intervening in different parts of the disease.In this paper,we briefly discuss the modern pharmacology of Sanlang and Curcuma longa,and deeply summarize the possible mechanisms of action of TCM monomer and classical compound extracts and their active ingredients through signal pathways in inflammation,immune system,angiogenesis,hormone regulation,etc.,so as to provide theoretical bases for the clinical use of TCM monomers,drug-to-drug groups and compounds in the treatment of EMs.

BACKGROUNDX-linked Charcot-Marie-Tooth type 1 (CMT1X) disease is one of the most common forms of inherited neuropathy caused by mutations in the gap junction beta-1 protein (GJB1) gene (also known as connexin 32). This study presented the clinical and genetic features of a series of Chinese patients with GJB1 gene mutations.

METHODSA total of 22 patients from unrelated families, who were referred to Department of Neurology, Peking University First Hospital from January 2005 to January 2016, were identified with GJB1 mutations. Their clinical records and laboratory findings were retrospectively collected and reviewed. Mutations in the GJB1 gene were analyzed by targeted next-generation sequencing (NGS). Nucleotide alternations were confirmed with Sanger sequencing.

RESULTSThe CMT1X patients predominantly showed distal muscle weakness of lower limbs with mild sensory disturbance. The mean age of onset was 15.6 ± 8.7 years (ranging from 1 year to 42 years). The sudden onset of cerebral symptoms appeared in four patients (18.2%); two were initial symptoms. One case had constant central nervous system (CNS) signs. There were 19 different heterozygous mutations, including 15 known mutations and four novel mutations (c.115G>T, c.380T>A, c.263C>A, and c.818_819insGGGCT). Among the 22 Chinese patients with CMT1X, the frequency of the GJB1 mutation was 4.5% in transmembrane domain 1 (TM1), 4.5% in TM2, 22.7% in TM3, 9.1% in TM4, 4.5% in extracellular 1 (EC1), 27.3% in EC2, 9.1% in intracellular loop, 13.6% in the N-terminal domain, and 4.5% in the C-terminal domain. CMT1X with CNS impairment appeared in five (22.7%) of these patients.

CONCLUSIONSThis study indicated that CNS impairment was not rare in Chinese CMT1X patients. Mutations in the EC2 domain of the GJB1 gene were hotspot in Chinese CMT1X patients.

Adolescent ; Adult ; Central Nervous System ; metabolism ; Charcot-Marie-Tooth Disease ; genetics ; pathology ; Child ; Child, Preschool ; Connexins ; genetics ; DNA Mutational Analysis ; Electrophysiology ; Female ; Genotype ; Humans ; Infant ; Male ; Mutation ; Phenotype ; Retrospective Studies ; Young Adult

BackgroundLMNA-related muscular dystrophy can manifest in a wide variety of disorders, including Emery-Dreifuss muscular dystrophy (EDMD), limb-girdle muscular dystrophy (LGMD), and LMNA-associated congenital muscular dystrophy (L-CMD). Muscle magnetic resonance imaging (MRI) has become a useful tool in the diagnostic workup of patients with muscle dystrophies. This study aimed to investigate whether there is a consistent pattern of MRI changes in patients with LMNA mutations in various muscle subtypes.

MethodsTwenty-two patients with LMNA-related muscular dystrophies were enrolled in this study. MRI of the thigh and/or calf muscles was performed in them. The muscle MRI features of the three subtypes were compared by the Mann-Whitney U-test. The relationship between the clinical and MRI findings was also investigated by Spearman's rank analyses.

ResultsThe present study included five EDMD, nine LGMD, and eight L-CMD patients. The thigh muscle MRI revealed that the fatty infiltration of the adductor magnus, semimembranosus, long and short heads of the biceps femoris, and vasti muscles, with relative sparing of the rectus femoris, was the predominant change observed in the EDMD, LGMD, and advanced-stage L-CMD phenotypes, although the involvement of the vasti muscles was not prominent in the early stage of L-CMD. At the level of the calf, six patients (one EDMD, four LGMD, and one L-CMD) also showed a similar pattern, in which the soleus and the medial and lateral gastrocnemius muscles were most frequently observed to have fatty infiltration. The fatty infiltration severity demonstrated higher scores associated with disease progression, with a corresponding rate of 1.483 + 0.075 × disease duration (X) (r = 0.444, P = 0.026). It was noteworthy that in six L-CMD patients with massive inflammatory cell infiltration in muscle pathology, no remarkable edema-like signals were observed in muscle MRI.

ConclusionsEDMD, LGMD and advanced-staged L-CMD subtypes showed similar pattern of muscle MRI changes, while early-staged L-CMD showed somewhat different changes. Muscle MRI of L-CMD with a muscular dystrophy pattern in MRI provided important clues for differentiating it from childhood inflammatory myopathy. The fatty infiltration score could be used as a reliable biomarker for outcome measure of disease progression.

Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Humans ; Magnetic Resonance Imaging ; methods ; Male ; Muscular Dystrophies ; diagnostic imaging ; Muscular Dystrophies, Limb-Girdle ; diagnostic imaging ; Muscular Dystrophy, Emery-Dreifuss ; diagnostic imaging ; Young Adult

BACKGROUNDKearns-Sayre syndrome (KSS) is a mitochondrial DNA (mtDNA) deletion disorder characterized by a triad of onset before 20 years of age, ophthalmoplegia, and pigmentary retinopathy. The heart and central nervous system are commonly involved. We summarized clinical and brain magnetic resonance imaging (MRI) features of a cohort of Chinese KSS patients.

METHODSNineteen patients confirmed by muscle biopsy and mtDNA analysis were enrolled. We examined clinical profiles, mainly focusing on changes in electrocardiogram (ECG) and brain MRI. The correlation between genotype and phenotype was statistically analyzed.

RESULTSThe mean age of onset was 9.6 ± 4.3 years, with all developing the classic triad at the time of diagnosis. Heart conduction block was detected in 63.2%, with four initially presenting as bundle branch block and developing into complete atrioventricular block over 3-72 months. Brain MRI showed symmetric high-T2 signals in 100% of cerebral and cerebellar white matter, as well as brainstem, 46.7% of basal ganglia, and 53.3% of thalamus. There were two patterns of cerebral white matter involvements, one with selective subcortical U-fibers and the other with periventricular white matter. The size of mtDNA deletion did not significantly correlate with age of onset or percentage of ragged blue fibers on muscle pathology.

CONCLUSIONSThe clinical features of KSS evolve dynamically, affecting the cardiac conduction system predominantly, highlighting the significance of ECG monitoring. Brain MRI showed changes involving both the white matter and deep gray nuclei. Clinical presentation or severity of muscle pathological changes is not related to the size of mtDNA deletions.

Adolescent ; Brain ; pathology ; physiology ; Child ; Child, Preschool ; DNA, Mitochondrial ; genetics ; Female ; Genotype ; Heart Block ; diagnosis ; genetics ; physiopathology ; Humans ; Kearns-Sayre Syndrome ; diagnosis ; genetics ; physiopathology ; Magnetic Resonance Imaging ; methods ; Male

Objective:To observe the activation of microglia and the expression of inflammatory factors in hippocampus of mice with depression-like behavior after mother-infant separation （MS） combined with lipopolysaccharide （LPS） stress， and to explore the possible anti-depression mechanisms of Wenyang （WY）， Jieyu （JY）， and Wenyang Jieyu （WYJY） prescriptions from the perspective of warming Yang and relieving depression. Method:Seventy offspring mice were randomly divided into a normal group （n=10）， a LPS stress group （n=10）， and a modeling group （n=50）. After undergoing 8 h·d^-1 mother-infant separation during postnatal day 5 （PD₅）–PD₁₄， mice in the modeling group were further divided into the MS + LPS group， WY group， JY group， WYJY group， and fluoxetine （FLU） group， with 10 in each group. The birth date of the offspring mice was recorded as PD₀. The mice in the normal， LPS， and MS + LPS groups were fed a normal diet during PD₂₁–PD₉₀， while those in the other groups were treated with the mixtures of corresponding drugs and feed， followed by seven-day intraperitoneal injection of LPS since PD₉₁ for inducing depression. The depression-like behavior of mice in each group was detected in the open-field， O-maze， and social interaction tests. The protein expression of microglia-specific ionized calcium-binding adaptor molecule 1 （Iba-1） in the hippocampus was assayed by immunohistochemistry， and the mRNA expression of interleukin-1β （IL-1β）， interleukin-6 （IL-6）， tumor necrosis factor-α （TNF-α）， Iba-1， and glucocorticoid receptor （GR） by real-time fluorescence quantitative PCR （Real-time PCR）. Result:Compared with the normal group， the LPS group exhibited significantly reduced residence time at the central area within 5 min （P<0.01） and shortened total exercise distance （P<0.01）. In the MS + LPS group， the open-arm activity time and the total activity distance decreased significantly （P<0.01， P<0.05）， whereas the training， discrimination and exploration time increased significantly （P<0.01）. The expression of Iba-1 in hippocampal CA1 region of mice in the LPS and MS + LPS groups was remarkably elevated （P<0.01）. Compared with the LPS group， the MS + LPS group displayed significantly prolonged distance of 5-min exercise （P<0.05）， increased training， discrimination and exploration time （P<0.05， P<0.01）， and up-regulated Iba-1 expression in hippocampal CA1 area （P<0.01）. As revealed by comparison with the MS + LPS group， both the total 5-min exercise distance （P<0.01） and the training and discrimination time （P<0.01， P<0.05） of mice in each administration group was significantly shortened. The discrimination and exploration time of mice in the JY， WYJY， and FLU groups was significantly reduced （P<0.01）， and the expression of Iba-1 in hippocampal CA1 region of mice in each administration group was significantly down-regulated （P<0.01）. Conclusion:The warming Yang and relieving depression method helps to inhibit the occurrence and development of depression due to its efficacy in activating microglia in hippocampus of depression mice and lowering the expression of IL-1β， IL-6， and TNF-α.

Objective:To observe the activation of microglia in hippocampus of depressed and anxious mice induced by maternal separation with acute restraint stress and the expression of interleukin-1β（IL-1β），interleukin-6（IL-6），tumor necrosis factor-α（TNF-α）， investigating the mechanism of Wenyang Jieyu prescription in treating anxiety and depression. Method:Eighty four male C57BL offspring were randomly divided into control group， acute restraint stress group and model group on postnatal day 0（PD0）. Maternal separation combined with acute restraint stress was used to prepare anxious and depressed model mice， dividing the model mice into model group， Wenyang， Jieyu， Wenyang Jieyu and fluoxetine group according to random number table method. During the period of PD21-PD90， the control， acute restraint stress and model mice were fed with normal diet， with the other groups fed with corresponding medicine mixed diet. The Wenyang， Jieyu and Wenyang Jieyu groups were given 5.85， 12.03 and 16.71 g·kg^-1·d^-1 respectively. The fluoxetine group was given 2.60 mg·kg^-1·d^-1. Open field， zero maze test and social interaction tests were used to evaluate the anxiety and depression of model mice. The expression of Iba-1 in hippocampal microglia was detected by immunohistochemistry（IHC）. The mRNA expression of IL-1β， IL-6， TNF-α， Iba-1 and glucocorticoid receptor（GR）were detected by Real-time fluorescence quantitative polymerase chain reaction （Real-time PCR）. Result:Compared with the control group， total movement distance and time spent in central zone in 5 min of the model mice significantly decreased（P<0.01）， time spent in opened arm and total movement distance decreased significantly（P<0.05，P<0.01）， investigation time during testing and training increased significantly（P<0.01）. The expression of Iba-1 protein and mRNA，IL-1β，IL-6，TNF-α mRNA significantly increased（P<0.01）， the expression levels of GR mRNA significantly decreased（P<0.01）. The result of IHC staining showed that microglia were over activated. Compared with the model group， total movement distance and time spent in central zone in 5 min of mice in the Wenyang Jieyu and fluoxetine group significantly increased（P<0.05，P<0.01）.Time spent in opened arm significantly increased（P<0.01）. Investigation time during testing and training significantly decreased（P<0.05，P<0.01）. The expression of Iba-1 protein and mRNA，IL-1β，IL-6，TNF-α mRNA significantly decreased（P<0.05，P<0.01）. The expression of GR mRNA increased significantly（P<0.05，P<0.01）. IHC staining showed the microglia recovered. Time spent in opened arm of mice in the Wenyang group and Jieyu group significantly increased （P<0.01）， time spent investigating during testing decreased significantly （P<0.05）， the expression levels of Iba-1 protein and mRNA，IL-6 mRNA significantly decreased （P<0.05，P<0.01）. The expression of GR mRNA of mice in the Wenyang group significantly increased （P<0.05）， the expression of TNF-α mRNA significantly decreased （P<0.05）. Total movement distance of mice in the Jieyu group increased significantly （P<0.01）， time spent investigating during training decreased significantly （P<0.05），the expression level of IL-1β mRNA significantly decreased （P<0.05）. IHC staining showed that microglia recovered partly in both groups. Conclusion:The comprehensive curative effect and pharmacological action of Wenyang Jieyu prescription were better than Wenyang prescription and Jieyu prescription. Wenyang Jieyu prescription can treat anxiety and depression in maternal separation and acute restraint stress mice， its possible mechanism may be related to the decreased activation of microglia， down-regulation of IL-1β，IL-6，TNF-α expression and up-regulation of GR expression.