Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemo-lytic disease in which there is a stem cell disorder of clonal nature. Previous studies have demonstrated that the numbers of burst-forming units-erythroid...Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemo-lytic disease in which there is a stem cell disorder of clonal nature. Previous studies have demonstrated that the numbers of burst-forming units-erythroid (BFU-E) and colony-forming units-granulocyte / macrophage (CFU-GM) from bone marrow of PNH patients growing in the medium containing PHA-LCM from the normai donors were more reduced than those of normai bone marrow. The purpose of present study was to investigate if PNH lymphocytes are defective in supporting hematopoiesis in vitro. PHA-LCM from PNH blood was added to the culture medium for the growth of PNH and normai BFU-E and CFU-GM. The numbers of PNH bone marrow BFU-E and CFU-GM in the medium containing PHA-LCM from PNH blood were less than those from normai blood; the numbers of normai bone marrow BFU-E and CFU-GM grown in the medium containing PHA-LCM from PNH blood were more decreased than those from normai blood. The results suggest that diminished numbers of PNH bone marrovv BFU-展开更多
Benign paroxysmal positional vertigo(BPPV) is the most frequent episodic vestibular disorder. It is due to otolith rests that are free into the canals or attached to the cupulas. Well over 90% of patients can be succe...Benign paroxysmal positional vertigo(BPPV) is the most frequent episodic vestibular disorder. It is due to otolith rests that are free into the canals or attached to the cupulas. Well over 90% of patients can be successfully treated with manoeuvres that move the particles back to the utriculus. Among the great variety of procedures that have been described, the manoeuvres that are supported by evidenced-based studies or extensive series are commented in this review. Some topics regarding BPPV treatment, such as controlling the accuracy of the procedures or the utility of post-manoeuvre restrictions are also discussed.展开更多
Objective: To investigate the relationship between basal ganglia cerebral infarction and paroxysmal atrial fibrillation(PAF) caused by abnormal vagus nerve tension.Methods: A total of 1 483 cases of elder patients wit...Objective: To investigate the relationship between basal ganglia cerebral infarction and paroxysmal atrial fibrillation(PAF) caused by abnormal vagus nerve tension.Methods: A total of 1 483 cases of elder patients with cerebral infarction who received head CT or MRI examination during the period were enrolled, including 830 male and613 female, with the average age as 78 years. These cases were divided into basal infarction ganglia group(n = 1 045) and non-basal ganglia infarction group(n = 438)according to the anatomic site of cerebral infarction. The differences of the incidence of PAF, left atrial diameter and heart rate variability were compared between the two groups.Results: In basal ganglia infarction group, the incidence rate of PAF was significantly higher than that of non-basal ganglia infarction group(P < 0.05). The incidence trend of cerebral infarction in basal ganglia was age-related, in the >79 years basal ganglia cerebral infarction group, the incidence of PAF was significantly higher than that of nonbasal ganglia infarction group(P < 0.05). There was no significant difference in the left atrial diameter between the basal ganglia infarction group and non-basal ganglia infarction group. Basal ganglia cerebral infarction patients with high PAF had higher heart rate variability than non-basal ganglia infarction group.Conclusion: Elderly patients with basal ganglia infarction have high incidence of PAF.Sympathetic nerve damage in cerebral basal ganglia, increased vagal tension and cardiac vagal tension are the direct causes of PAF. The results indicates that the increased central vagal nerve tension mediated PAF probably is an indication of supplying sympathetic neurotransmitter or cardiac vagal denervation treatment.展开更多
Benign paroxysmal positional vertigo(BPPV) is probably the most common diagnosis at vertigo clinics.Seasonal cycles of several human illnesses could be attributed variously to changes in atmospheric or weather conditi...Benign paroxysmal positional vertigo(BPPV) is probably the most common diagnosis at vertigo clinics.Seasonal cycles of several human illnesses could be attributed variously to changes in atmospheric or weather conditions.In this retrospective study,patients with BPPV from January 2010 to December 2012 were studied,and their charts were reviewed.Statistical analysis revealed a statistically significant difference in patients’ numbers among different months of the year.Also there is a significant statistical correlation between the numbers of patients with climatic variations especially the temperature.The present paper discusses the possible explanations for these results which confirms the seasonal variations in BPPV,together with a review of literature to view the possible associations with other disorders that causes such seasonality.展开更多
Background: Probable benign paroxysmal positional vertigo, spontaneously resolved (pBPPVsr), is a variant of benign paroxysmal positional vertigo (BPPV) in which there is no observable nystagmus and no vertigo with an...Background: Probable benign paroxysmal positional vertigo, spontaneously resolved (pBPPVsr), is a variant of benign paroxysmal positional vertigo (BPPV) in which there is no observable nystagmus and no vertigo with any positional maneuver. Objectives: To calculate the incidence pBPPVsr, compare the characteristics of the patients with pBPPVsr and BPPV not spontaneously resolved and describe the spontaneous resolution in the natural course of BPPV. Methods: Multicenter prospective descriptive study. During a one-year period, all patients with suspected BPPV that presented to the Neurotology Units of five participating centers were recruited. The incidence of pBPPVsr was calculated as a percentage of the total number of patients with BPPV. The prevalence of several variables was compared between pBPPVsr and BPPV not spontaneously resolved. The timing of spontaneous resolution was estimated using Kaplan-Meier curves. Results: 457 patients met the inclusion criteria. The incidence of pBPPVsr was 33.5%. It was significantly higher in males, in patients with normal bone mass and in patients who were not taking sulpiride. A rate of 18% of spontaneous resolution after the first month and 51% after the first year was found. This percentage did not change in a significant way after this moment. The curves for males, patients under 50 and patients with normal blood pressure decreased significantly faster. Conclusions: In our serie, BPPV spontaneously resolved in half of the patients with BPPV during the first year. This seemed to occur more commonly in males and could have been hindered by sulpiride intake, osteoporosis, advanced age and high blood pressure.展开更多
Paroxysmal kinesigenic dyskinesia（PKD） and myotonia congenita（MC） are independent disorders that share some clinical features. We aimed to investigate the sequences of PRRT2 and CLCN1 in a proband diagnosed with P...Paroxysmal kinesigenic dyskinesia（PKD） and myotonia congenita（MC） are independent disorders that share some clinical features. We aimed to investigate the sequences of PRRT2 and CLCN1 in a proband diagnosed with PKD and suspected MC. Clinical evaluation and auxiliary examinations were performed. Direct sequencing of the entire coding regions of the PRRT2 and CLCN1 genes was conducted. Haplotype analysis confirmed the relationships among the family members. The proband suffered choreoathetosis attacks triggered by sudden movements, and lower-limb weakness a n d s t i ff n e s s t h a t w o r s e n e d i n c o l d w e a t h e r. Carbamazepine monotherapy completely controlled his choreoathetosis and significantly relieved his limb weakness and stiffness. His father, when young, had similar limb stiffness, while his mother and brother were asymptomatic. Genetic analysis revealed that the proband and his father harbored a PRRT2 c.649 dup C mutation, and CLCN1 c.1723C〉T and c.2492A〉G mutations. His brother carried only the two CLCN1 mutations. None of these mutations were identified in his mother and 150 unrelated controls. This is the first report showing the coexistence ofPRRT2 and CLCN1 mutations. Our results also indicate that both the PRRT2 and CLCN1 genes need to be screened if we fail to identify PRRT2 mutations in PKD patients or CLCN1 mutations in MC patients.展开更多
The genesis of the Benign Paroxysmal Positional Vertigo(BPPV)seems to be related to some metabolic factors.These factors,such as vitamin D,glucocorticoids,and even thyroid and growth hormones,can affect bone metabolis...The genesis of the Benign Paroxysmal Positional Vertigo(BPPV)seems to be related to some metabolic factors.These factors,such as vitamin D,glucocorticoids,and even thyroid and growth hormones,can affect bone metabolism and the mineralization of otoconia.It also seems to link to factors related to aging or nutritional habits.Besides,since the incidence of BPPV is quantitatively higher in women than in men,female sex steroids could be associated with this process.It could be useful to understand how these factors act in otoconial mineralization if we want to develop treatments aimed at preventing or delaying BPPV recurrences.In this review,we will analyze the role of these metabolic and hormonal factors in otoconial mineralization and in the treatment of BPPV.展开更多
A three-dimensional numerical model of the membranous labyrinth of the semicircular canal of the inner ear was established to investigate the effects of canalithiasis of BPPV on the balance function of the inner ear.T...A three-dimensional numerical model of the membranous labyrinth of the semicircular canal of the inner ear was established to investigate the effects of canalithiasis of BPPV on the balance function of the inner ear.The movement of otolith particles in the membranous labyrinth was simulated when a person turns his head to a specific position.The effects of otolith movements on the balance function of the inner ear were simulated for different numbers,diameters,and initial positions of otoliths.The simulation results show that the otolith diameter affects the movement duration of otoliths in the membranous labyrinth.The number and diameter of otoliths,the diameter of the membranous labyrinth,and the initial position of the otoliths can cause changes in the pressure difference on both sides of the cupula of the crista ampullaris(cupula).The latent period and onset period are related to the diameter of otolith particles and the position of the head.There is also a correlation between the severity of the disease and the diameter and number of otolith particles.展开更多
One of the most common causes of vertigo is Benign Paroxysmal Positional Vertigo(BPPV),a sensation of spinning that is caused by a sudden change in head position.This type of vertigo was first described by Robert Bara...One of the most common causes of vertigo is Benign Paroxysmal Positional Vertigo(BPPV),a sensation of spinning that is caused by a sudden change in head position.This type of vertigo was first described by Robert Barany in the early 1920s[1].He suggested that BPPV was caused by abnormal otoliths,also called statoconium or otoconius,a structure in the saccule or utricle in展开更多
Paroxysmal kinesigenic dyskinesia(PKD),the most common type of paroxysmal movement disorder,is characterized by sudden and brief attacks of choreoathetosis or dystonia triggered by sudden voluntary movements.PKD is ma...Paroxysmal kinesigenic dyskinesia(PKD),the most common type of paroxysmal movement disorder,is characterized by sudden and brief attacks of choreoathetosis or dystonia triggered by sudden voluntary movements.PKD is mainly caused by mutations in the PRRT2 or TMEM151A gene.The exact pathophysiological mechanisms of PKD remain unclear,although the function of PRRT2 protein has been well characterized in the last decade.Based on abnormal ion channels and disturbed synaptic transmission in the absence of PRRT2,PKD may be channelopathy or synaptopathy,or both.In addition,the cerebellum is regarded as the key pathogenic area.Spreading depolarization in the cerebellum is tightly associated with dyskinetic episodes.Whereas,in PKD,other than the cerebellum,the role of the cerebrum including the cortex and thalamus needs to be further investigated.展开更多
Background:Benign paroxysmal positional vertigo(BPPV)is characterized by vertigo lasting from seconds to minutes,induced by head movements.Objectives:Our study aimed to investigate the clinical significance of the cal...Background:Benign paroxysmal positional vertigo(BPPV)is characterized by vertigo lasting from seconds to minutes,induced by head movements.Objectives:Our study aimed to investigate the clinical significance of the caloric vestibular and video head-impulse tests(vHIT)diagnosing the disorder.Methods:68 patients suffering from posterior canal BPPV(25 male,43 females,mean age±SD,54.5±13.2 years)and 56 patients with a normal functioning vestibular system as control were investigated.Bithermal caloric test and vHIT was performed during the same medical check-up.Canal paresis(CP%),gain(GA)and asymmetry(GA%)parameters were calculated.Results:The Dix-Hallpike manoeuvre was only positive in 4%of this population.The CP%parameter was only pathologic in two patients,and there was no significant difference between control and BPPV patients(p=0.76).The GA value was never under 0.8 in this population,but GA%was abnormal in 63.2%.A significant difference comparing the GA%values to the control group was seen(p=0.034).There was no correlation detected between the CP%and GA%values in BPPV.Regarding the GA%value,61%sensitivity and 76%specificity was seen.Conclusion:The Dix-Hallpike manoeuvre was not often positive in the non-acute phase of BPPV;therefore,objective testing is essential.The caloric test does not have clinical significance in BPPV,but vHIT can be helpful based on the GA%parameter。展开更多
BACKGROUND A modified Valsalva maneuver(VM)has been suggested to be superior to the standard VM for conversion of paroxysmal supraventricular tachycardia(PSVT).AIM To evaluate the efficacy and economic benefits of a m...BACKGROUND A modified Valsalva maneuver(VM)has been suggested to be superior to the standard VM for conversion of paroxysmal supraventricular tachycardia(PSVT).AIM To evaluate the efficacy and economic benefits of a modified VM in Chinese patients.METHODS Patients with PSVT admitted to our center between October 2017 and September 2019 were randomly assigned to the modified and standard VM groups.Conversion via VM was performed up to three times.The primary outcome of the study was the success rate of PSVT conversion to sinus rhythm.The secondary outcomes included the incidence of adverse events,economic cost during the visit,and the degree of patient acceptance of the treatment.RESULTS Overall,361 patients were enrolled,with 180 allocated to the modified VM group and 181 to the standard VM group.Baseline characteristics were well matched in the groups.Overall,the modified VM group had higher success rates of PSVT conversion after single(47.78%vs 15.38%,P<0.001)and multiple(62.22%vs 19.78%,P<0.001)VM sessions.No significant differences in the incidences of adverse events and rates of patient acceptance were detected between the two groups(both P>0.05).Moreover,the economic cost of the clinic visit was significantly lower for the modified VM group than for the standard VM group(P<0.05).CONCLUSION The modified VM may confer both therapeutic and economic benefits as compared with the standard VM for conversion of PSVT.展开更多
BACKGROUND Cryoballoon ablation(CBA)is recommended for patients with paroxysmal atrial fibrillation(AF)refractory to antiarrhythmic drugs.However,only 80%of patients benefit from initial CBA.There is growing evidence ...BACKGROUND Cryoballoon ablation(CBA)is recommended for patients with paroxysmal atrial fibrillation(AF)refractory to antiarrhythmic drugs.However,only 80%of patients benefit from initial CBA.There is growing evidence that pretreatment with angiotensin-converting enzyme inhibitors(ACEIs)and angiotensin receptor blockers(ARBs)decreases the recurrence of AF postablation,particularly in nonparoxysmal AF undergoing radiofrequency ablation.The role of ACEIs and ARBs in patients with paroxysmal AF in CBA remains unknown.We decided to investigate the role of ACEIs and ARBs in preventing the recurrence of atrial arrhythmia(AA)following CBA for paroxysmal AF.AIM To investigate the role of ACEIs and ARBs in preventing recurrence of AA following CBA for paroxysmal AF.METHODS We followed 103 patients(age 60.6±9.1 years,29%women)with paroxysmal AF undergoing CBA 1-year post procedure.Recurrence was assessed by documented AA on electrocardiogram or any form of long-term cardiac rhythm monitoring.A multivariable Cox proportional hazard model was used to assess if ACEI or ARB treatment predicted the risk of AA recurrence.RESULTS After a 1-year follow-up,19(18.4%)participants developed recurrence of AA.Use of ACEI or ARB therapy was noted in the study population.Patients on ACEI/ARB had a greater prevalence of hypertension and coronary artery disease.On a multivariate model adjusted for baseline demographics and risk factors for AF,ACEI or ARB therapy did not prevent recurrence of AA following CBA(P=0.72).Similarly,on Kaplan–Meier analysis pretreatment with ACEI/ARB did not predict the time to first recurrence of AA(P=0.2173).CONCLUSION In our study population,preablation treatment with an ACEI or ARB had no influence on the recurrence of AA following CBA for paroxysmal AF.展开更多
Introduction: Posterior canal benign paroxysmal positional vertigo (PC-BPPV) is considered the mostcommon cause of peripheral vertigo in the emergency department (ED). Although the canalith repositioning maneuver (CRM...Introduction: Posterior canal benign paroxysmal positional vertigo (PC-BPPV) is considered the mostcommon cause of peripheral vertigo in the emergency department (ED). Although the canalith repositioning maneuver (CRM) is the standard of care, the most effective method to deliver it in the ED hasbeen poorly studied.Objective: To compare two protocols of the Epley maneuver for the treatment of PC-BPPV.Patients and methods: We prospectively recruited 101 patients with unilateral PC-BPPV on physical examination, randomizing them to either a single Epley maneuver (EM) (n ¼ 46) or multiple maneuvers(n ¼ 55) on the same visit. Measured outcomes included presence/absence of positional nystagmus,resolution of vertigo, and score on the dizziness handicap inventory (DHI) at follow-up evaluations. TheDHI was stratified into mild ( 30) and moderate-severe (>30).Results: Normalization of the Dix-Hallpike maneuver at day 5 was observed in 38% of the single EMgroup and 44.4% in the multiple EM group (p ¼ 0.62). The DHI showed reduction from 42.2 (SD 18.4) to31.9 (SD 23.7) in the single EM group and from 43.7 (SD 22.9) to 33.5 (SD 21.5) in the multiple EM group(p ¼ 0.06). A higher number of patients improved from moderate-severe to mild DHI (p ¼ 0.03) in thesingle EM group compared to the multi-EM group (p ¼ 0.23).Conclusion: There was no statistically significant difference between performing a single EM versusmultiple EMs for treatment of PC-BPPV in the emergency department. The single EM approach isassociated with shorter physical contact between patients and examiner, which is logically safer in apandemic context.展开更多
Objective:To study the potential role of subjective visual vertical(SVV)as a prognostic marker for canalith repositioning maneuver(CRM)in patients with posterior canal benign paroxysmal positional vertigo(PC-BPPV)for ...Objective:To study the potential role of subjective visual vertical(SVV)as a prognostic marker for canalith repositioning maneuver(CRM)in patients with posterior canal benign paroxysmal positional vertigo(PC-BPPV)for the Indian population.Methods:SVV was examined in 30 patients with PC-BPPV before and after canalith repositioning maneuver and after complete resolution of PC-BPPV.Study parameters included the mean of 10 angular tilt readings and direction of deviation,which were compared before and after CRM and following complete resolution of PC-BPPV.Results:The angle of SVV tilt was greater and deviated towards the affected ear before CRM in all patients,which decreased significantly shortly after CRM and continued to decrease after complete resolution of PC-BPPV(p<0.0001).Conclusions:SVV can be used to test utricular dysfunction in PC-BPPV.The angle of tilt improves in response to CRM,which may be used as a prognostic marker in patients with PC-BPPV receiving CRM.展开更多
KCNA1 is the coding gene for Kv1.1 voltage-gated potassium-channelαsubunit.Three variants of KCNA1 have been reported to manifest as paroxysmal kinesigenic dyskinesia(PKD),but the correlation between them remains unc...KCNA1 is the coding gene for Kv1.1 voltage-gated potassium-channelαsubunit.Three variants of KCNA1 have been reported to manifest as paroxysmal kinesigenic dyskinesia(PKD),but the correlation between them remains unclear due to the phenotypic complexity of KCNA1 variants as well as the rarity of PKD cases.Using the whole exome sequencing followed by Sanger sequencing,we screen for potential pathogenic KCNA1 variants in patients clinically diagnosed with paroxysmal movement disorders and identify three previously unreported missense variants of KCNA1 in three unrelated Chinese families.The proband of one family(c.496G>A,p.A166T)manifests as episodic ataxia type 1,and the other two(c.877G>A,p.V293I and c.1112C>A,p.T371A)manifest as PKD.The pathogenicity of these variants is confirmed by functional studies,suggesting that p.A166T and p.T371A cause a loss-of-function of the channel,while p.V293I leads to a gain-of-function with the property of voltage-dependent gating and activation kinetic affected.By reviewing the locations of PKD-manifested KCNA1 variants in Kv1.1 protein,we find that these variants tend to cluster around the pore domain,which is similar to epilepsy.Thus,our study strengthens the correlation between KCNA1 variants and PKD and provides more information on genotype–phenotype correlations of KCNA1 channelopathy.展开更多
BACKGROUND Idiopathic non-cirrhotic portal hypertension(INCPH)is mainly associated with thrombophilia in Western countries.Paroxysmal nocturnal hemoglobinuria(PNH)is a rare hematologic disease that manifests with hemo...BACKGROUND Idiopathic non-cirrhotic portal hypertension(INCPH)is mainly associated with thrombophilia in Western countries.Paroxysmal nocturnal hemoglobinuria(PNH)is a rare hematologic disease that manifests with hemolytic anemia,thrombosis,and peripheral blood cytopenias.Portal and hepatic venous thrombosis were reported in PNH.A rare case of INCPH complicating PNH is described.CASE SUMMARY A 63-year old woman with a 2-year past medical history of PNH without treatment was admitted because of jaundice and refractory ascites requiring large volume paracentesis.Liver histology revealed portal venopathy with portal fibrosis and sclerosis,nodular regenerative hyperplasia,parenchymal ischemic changes,and focal sinusoidal and perivenular fibrosis without bridging fibrosis or cirrhosis,all indicative of INCPH.The flow cytometry confirmed PNH diagnosis and eculizumab treatment was initiated.Her condition was improved gradually,bilirubin was normalized 6 months following initiation of eculizumab,and 1 year later diuretics were stopped.CONCLUSION Eculizumab improved intravascular hemolysis and reversed clinical manifestations of INCPH in a patient with paroxysmal nocturnal hemoglobinuria.展开更多
BACKGROUND Catheter-based pulmonary vein isolation(PVI) is an effective and well-established intervention for symptomatic paroxysmal atrial fibrillation(PAF). Nevertheless, late recurrences of atrial fibrillation(LRAF...BACKGROUND Catheter-based pulmonary vein isolation(PVI) is an effective and well-established intervention for symptomatic paroxysmal atrial fibrillation(PAF). Nevertheless, late recurrences of atrial fibrillation(LRAF) occurring during 3 to 12months are common, and the underlying mechanisms remain elusive. Circular RNAs(circ RNAs) in atrial tissue have been linked to the pathophysiological mechanisms and progression of PAF in a few studies. However, their expression patterns in peripheral blood and regulatory function in LRAF are not clear.METHODS In the present study, the expression profile of circulating circ RNAs in three paired nonvalvular PAF patients with or without LRAF was investigated by high-throughput sequencing and validated by quantitative real-time polymerase chain reaction(q RT-PCR). Bioinformatics analyses, including Gene Ontology(GO), Kyoto Encyclopedia of Genes and Genomes(KEGG)pathway analysis, and circ RNA/mi RNA regulatory network, were performed to predict the functions and potential regulatory roles of differentially expressed(DE) circ RNAs.RESULTS A total of 12,834 circ RNAs, comprising 5,491 down-regulated and 7,343 up-regulated circ RNAs, were found to be DE in blood smaples from the two groups in peripheral blood between LRAF and non-recurrence control individuals. The most enriched GO categories in terms of molecular function, biological process, and cellular component features were catalytic activity,cellular metabolic process, and intracellular part, respectively. The KEGG enrichment study revealed that the most important metabolic process controlled by DE circ RNAs is endocytosis. In the circ RNA/micro RNAs interaction network, four up-regulated circ RNAs(hsa_circ_0002665, hsa_circ_0001953, hsa_circ_0003831, and hsa_circ_0040533) and one down-regulated circ RNA(hsa_circ_0041103) were predicted to play potential regulatory roles in the pathogenesis of LRAF.CONCLUSIONS This investigation discovered the expression pattern of circulating circ RNAs that is indicative of PAF late recurrence, which may serve as risk markers or therapeutic targets for LRAF after PVI.展开更多
This study was aimed to analyze the clinical features of paroxysmal kinesigenic dyskinesia (PKD) and extend the understanding of this disease. From August, 2008 to October, 2010, 9 patients were diagnosed with PKD in ...This study was aimed to analyze the clinical features of paroxysmal kinesigenic dyskinesia (PKD) and extend the understanding of this disease. From August, 2008 to October, 2010, 9 patients were diagnosed with PKD in the Department of Neurology of the First Affiliated Hospital of Zhejiang University, China. The data involving clinical demographic characteristics, somatosensory evoked potentials, results of electromyography, video electroencephalography (EEG), brain magnetic resonance imaging (MRI) and computerized tomography (CT) were collected. All PKD patients exhibited unilat-eral or bilateral recurrent episodic dyskinetic attacks triggered by sudden voluntary movements. The duration of the attacks ranged from several seconds to one minute. The attack frequency ranged from approximately once in several months to more than 10 times in a day. Patients suffered from no conscious disorders during the attack, and no neurological signs were found during the period between attacks. No abnormal somatosensory evoked potentials were found. Routine EEG, video EEG monitoring or brain imaging showed normal findings. Classical treatment for anti-epilepsy, including car-bamazepine and topiramate, was administered to the patients and proved to be effective. It was concluded that PKD is characteristically triggered by sudden voluntary movement; no abnormal elec-troneurophysiological findings are observed in PKD, and antiepileptic drugs are effective in treating the disorder.展开更多
Radioimmunoassays were used to measure the concentration changes of plasma endothelin(ET) and atrial natriuretic peptide(ANP) during the onset and after termination of paroxysmal supraventricular tachycardia(SVT). 30 ...Radioimmunoassays were used to measure the concentration changes of plasma endothelin(ET) and atrial natriuretic peptide(ANP) during the onset and after termination of paroxysmal supraventricular tachycardia(SVT). 30 cases were reviewed and compansons with 42 normal subjects were made. There are very significant differences(P<0.0001) in the concentration changes of both plasma ET and ANP during the onset and 30 minutes after the termination of SVT. During the onset period of SVT. the plasma ET and ANP were markedly elevated and 30 minutes after its termination they were lowered significantly, but their concentrations were still 2-fold higher than ihose of the control group. As the biological effects of ANP and ET are antagonistic to each other. their parallel elevation and lowering of plasma concentrations during and.after the termination of SVT reveal that these 2 hormones parucipate in the pathophysiological process of SVT. This phenomenon is possibly one of the homeostatic regulatory functions in the organism.展开更多
文摘Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemo-lytic disease in which there is a stem cell disorder of clonal nature. Previous studies have demonstrated that the numbers of burst-forming units-erythroid (BFU-E) and colony-forming units-granulocyte / macrophage (CFU-GM) from bone marrow of PNH patients growing in the medium containing PHA-LCM from the normai donors were more reduced than those of normai bone marrow. The purpose of present study was to investigate if PNH lymphocytes are defective in supporting hematopoiesis in vitro. PHA-LCM from PNH blood was added to the culture medium for the growth of PNH and normai BFU-E and CFU-GM. The numbers of PNH bone marrow BFU-E and CFU-GM in the medium containing PHA-LCM from PNH blood were less than those from normai blood; the numbers of normai bone marrow BFU-E and CFU-GM grown in the medium containing PHA-LCM from PNH blood were more decreased than those from normai blood. The results suggest that diminished numbers of PNH bone marrovv BFU-
文摘Benign paroxysmal positional vertigo(BPPV) is the most frequent episodic vestibular disorder. It is due to otolith rests that are free into the canals or attached to the cupulas. Well over 90% of patients can be successfully treated with manoeuvres that move the particles back to the utriculus. Among the great variety of procedures that have been described, the manoeuvres that are supported by evidenced-based studies or extensive series are commented in this review. Some topics regarding BPPV treatment, such as controlling the accuracy of the procedures or the utility of post-manoeuvre restrictions are also discussed.
基金funded by Shanghai Health and Family Planning Commission Youth Found to Wenbo Cheng(20144Y0116)
文摘Objective: To investigate the relationship between basal ganglia cerebral infarction and paroxysmal atrial fibrillation(PAF) caused by abnormal vagus nerve tension.Methods: A total of 1 483 cases of elder patients with cerebral infarction who received head CT or MRI examination during the period were enrolled, including 830 male and613 female, with the average age as 78 years. These cases were divided into basal infarction ganglia group(n = 1 045) and non-basal ganglia infarction group(n = 438)according to the anatomic site of cerebral infarction. The differences of the incidence of PAF, left atrial diameter and heart rate variability were compared between the two groups.Results: In basal ganglia infarction group, the incidence rate of PAF was significantly higher than that of non-basal ganglia infarction group(P < 0.05). The incidence trend of cerebral infarction in basal ganglia was age-related, in the >79 years basal ganglia cerebral infarction group, the incidence of PAF was significantly higher than that of nonbasal ganglia infarction group(P < 0.05). There was no significant difference in the left atrial diameter between the basal ganglia infarction group and non-basal ganglia infarction group. Basal ganglia cerebral infarction patients with high PAF had higher heart rate variability than non-basal ganglia infarction group.Conclusion: Elderly patients with basal ganglia infarction have high incidence of PAF.Sympathetic nerve damage in cerebral basal ganglia, increased vagal tension and cardiac vagal tension are the direct causes of PAF. The results indicates that the increased central vagal nerve tension mediated PAF probably is an indication of supplying sympathetic neurotransmitter or cardiac vagal denervation treatment.
文摘Benign paroxysmal positional vertigo(BPPV) is probably the most common diagnosis at vertigo clinics.Seasonal cycles of several human illnesses could be attributed variously to changes in atmospheric or weather conditions.In this retrospective study,patients with BPPV from January 2010 to December 2012 were studied,and their charts were reviewed.Statistical analysis revealed a statistically significant difference in patients’ numbers among different months of the year.Also there is a significant statistical correlation between the numbers of patients with climatic variations especially the temperature.The present paper discusses the possible explanations for these results which confirms the seasonal variations in BPPV,together with a review of literature to view the possible associations with other disorders that causes such seasonality.
文摘Background: Probable benign paroxysmal positional vertigo, spontaneously resolved (pBPPVsr), is a variant of benign paroxysmal positional vertigo (BPPV) in which there is no observable nystagmus and no vertigo with any positional maneuver. Objectives: To calculate the incidence pBPPVsr, compare the characteristics of the patients with pBPPVsr and BPPV not spontaneously resolved and describe the spontaneous resolution in the natural course of BPPV. Methods: Multicenter prospective descriptive study. During a one-year period, all patients with suspected BPPV that presented to the Neurotology Units of five participating centers were recruited. The incidence of pBPPVsr was calculated as a percentage of the total number of patients with BPPV. The prevalence of several variables was compared between pBPPVsr and BPPV not spontaneously resolved. The timing of spontaneous resolution was estimated using Kaplan-Meier curves. Results: 457 patients met the inclusion criteria. The incidence of pBPPVsr was 33.5%. It was significantly higher in males, in patients with normal bone mass and in patients who were not taking sulpiride. A rate of 18% of spontaneous resolution after the first month and 51% after the first year was found. This percentage did not change in a significant way after this moment. The curves for males, patients under 50 and patients with normal blood pressure decreased significantly faster. Conclusions: In our serie, BPPV spontaneously resolved in half of the patients with BPPV during the first year. This seemed to occur more commonly in males and could have been hindered by sulpiride intake, osteoporosis, advanced age and high blood pressure.
基金supported by grants from the National Natural Science Foundation of China (81330025 and 81125009)
文摘Paroxysmal kinesigenic dyskinesia（PKD） and myotonia congenita（MC） are independent disorders that share some clinical features. We aimed to investigate the sequences of PRRT2 and CLCN1 in a proband diagnosed with PKD and suspected MC. Clinical evaluation and auxiliary examinations were performed. Direct sequencing of the entire coding regions of the PRRT2 and CLCN1 genes was conducted. Haplotype analysis confirmed the relationships among the family members. The proband suffered choreoathetosis attacks triggered by sudden movements, and lower-limb weakness a n d s t i ff n e s s t h a t w o r s e n e d i n c o l d w e a t h e r. Carbamazepine monotherapy completely controlled his choreoathetosis and significantly relieved his limb weakness and stiffness. His father, when young, had similar limb stiffness, while his mother and brother were asymptomatic. Genetic analysis revealed that the proband and his father harbored a PRRT2 c.649 dup C mutation, and CLCN1 c.1723C〉T and c.2492A〉G mutations. His brother carried only the two CLCN1 mutations. None of these mutations were identified in his mother and 150 unrelated controls. This is the first report showing the coexistence ofPRRT2 and CLCN1 mutations. Our results also indicate that both the PRRT2 and CLCN1 genes need to be screened if we fail to identify PRRT2 mutations in PKD patients or CLCN1 mutations in MC patients.
文摘The genesis of the Benign Paroxysmal Positional Vertigo(BPPV)seems to be related to some metabolic factors.These factors,such as vitamin D,glucocorticoids,and even thyroid and growth hormones,can affect bone metabolism and the mineralization of otoconia.It also seems to link to factors related to aging or nutritional habits.Besides,since the incidence of BPPV is quantitatively higher in women than in men,female sex steroids could be associated with this process.It could be useful to understand how these factors act in otoconial mineralization if we want to develop treatments aimed at preventing or delaying BPPV recurrences.In this review,we will analyze the role of these metabolic and hormonal factors in otoconial mineralization and in the treatment of BPPV.
基金This study was founded by the National Nature Science Foundation of China(11572079,11772087,31500765,11472074,11372069)。
文摘A three-dimensional numerical model of the membranous labyrinth of the semicircular canal of the inner ear was established to investigate the effects of canalithiasis of BPPV on the balance function of the inner ear.The movement of otolith particles in the membranous labyrinth was simulated when a person turns his head to a specific position.The effects of otolith movements on the balance function of the inner ear were simulated for different numbers,diameters,and initial positions of otoliths.The simulation results show that the otolith diameter affects the movement duration of otoliths in the membranous labyrinth.The number and diameter of otoliths,the diameter of the membranous labyrinth,and the initial position of the otoliths can cause changes in the pressure difference on both sides of the cupula of the crista ampullaris(cupula).The latent period and onset period are related to the diameter of otolith particles and the position of the head.There is also a correlation between the severity of the disease and the diameter and number of otolith particles.
文摘One of the most common causes of vertigo is Benign Paroxysmal Positional Vertigo(BPPV),a sensation of spinning that is caused by a sudden change in head position.This type of vertigo was first described by Robert Barany in the early 1920s[1].He suggested that BPPV was caused by abnormal otoliths,also called statoconium or otoconius,a structure in the saccule or utricle in
基金supported by grants from the National Natural Science Foundation(81330025).
文摘Paroxysmal kinesigenic dyskinesia(PKD),the most common type of paroxysmal movement disorder,is characterized by sudden and brief attacks of choreoathetosis or dystonia triggered by sudden voluntary movements.PKD is mainly caused by mutations in the PRRT2 or TMEM151A gene.The exact pathophysiological mechanisms of PKD remain unclear,although the function of PRRT2 protein has been well characterized in the last decade.Based on abnormal ion channels and disturbed synaptic transmission in the absence of PRRT2,PKD may be channelopathy or synaptopathy,or both.In addition,the cerebellum is regarded as the key pathogenic area.Spreading depolarization in the cerebellum is tightly associated with dyskinetic episodes.Whereas,in PKD,other than the cerebellum,the role of the cerebrum including the cortex and thalamus needs to be further investigated.
基金supported by EFOP-3.6.3-VEKOP-16-2017-00009 Project and by theÚNKP-20-4-I New National Excellence Program of The Ministry for Innovation and Technology from the Source of The National Research,Development and Innovation Fund.
文摘Background:Benign paroxysmal positional vertigo(BPPV)is characterized by vertigo lasting from seconds to minutes,induced by head movements.Objectives:Our study aimed to investigate the clinical significance of the caloric vestibular and video head-impulse tests(vHIT)diagnosing the disorder.Methods:68 patients suffering from posterior canal BPPV(25 male,43 females,mean age±SD,54.5±13.2 years)and 56 patients with a normal functioning vestibular system as control were investigated.Bithermal caloric test and vHIT was performed during the same medical check-up.Canal paresis(CP%),gain(GA)and asymmetry(GA%)parameters were calculated.Results:The Dix-Hallpike manoeuvre was only positive in 4%of this population.The CP%parameter was only pathologic in two patients,and there was no significant difference between control and BPPV patients(p=0.76).The GA value was never under 0.8 in this population,but GA%was abnormal in 63.2%.A significant difference comparing the GA%values to the control group was seen(p=0.034).There was no correlation detected between the CP%and GA%values in BPPV.Regarding the GA%value,61%sensitivity and 76%specificity was seen.Conclusion:The Dix-Hallpike manoeuvre was not often positive in the non-acute phase of BPPV;therefore,objective testing is essential.The caloric test does not have clinical significance in BPPV,but vHIT can be helpful based on the GA%parameter。
基金Supported by Key Research and Development Project of Shandong Province,No.2016GST201224and Jinan Clinical Medicine Science and Technology Innovation Plan,No.201907056.
文摘BACKGROUND A modified Valsalva maneuver(VM)has been suggested to be superior to the standard VM for conversion of paroxysmal supraventricular tachycardia(PSVT).AIM To evaluate the efficacy and economic benefits of a modified VM in Chinese patients.METHODS Patients with PSVT admitted to our center between October 2017 and September 2019 were randomly assigned to the modified and standard VM groups.Conversion via VM was performed up to three times.The primary outcome of the study was the success rate of PSVT conversion to sinus rhythm.The secondary outcomes included the incidence of adverse events,economic cost during the visit,and the degree of patient acceptance of the treatment.RESULTS Overall,361 patients were enrolled,with 180 allocated to the modified VM group and 181 to the standard VM group.Baseline characteristics were well matched in the groups.Overall,the modified VM group had higher success rates of PSVT conversion after single(47.78%vs 15.38%,P<0.001)and multiple(62.22%vs 19.78%,P<0.001)VM sessions.No significant differences in the incidences of adverse events and rates of patient acceptance were detected between the two groups(both P>0.05).Moreover,the economic cost of the clinic visit was significantly lower for the modified VM group than for the standard VM group(P<0.05).CONCLUSION The modified VM may confer both therapeutic and economic benefits as compared with the standard VM for conversion of PSVT.
文摘BACKGROUND Cryoballoon ablation(CBA)is recommended for patients with paroxysmal atrial fibrillation(AF)refractory to antiarrhythmic drugs.However,only 80%of patients benefit from initial CBA.There is growing evidence that pretreatment with angiotensin-converting enzyme inhibitors(ACEIs)and angiotensin receptor blockers(ARBs)decreases the recurrence of AF postablation,particularly in nonparoxysmal AF undergoing radiofrequency ablation.The role of ACEIs and ARBs in patients with paroxysmal AF in CBA remains unknown.We decided to investigate the role of ACEIs and ARBs in preventing the recurrence of atrial arrhythmia(AA)following CBA for paroxysmal AF.AIM To investigate the role of ACEIs and ARBs in preventing recurrence of AA following CBA for paroxysmal AF.METHODS We followed 103 patients(age 60.6±9.1 years,29%women)with paroxysmal AF undergoing CBA 1-year post procedure.Recurrence was assessed by documented AA on electrocardiogram or any form of long-term cardiac rhythm monitoring.A multivariable Cox proportional hazard model was used to assess if ACEI or ARB treatment predicted the risk of AA recurrence.RESULTS After a 1-year follow-up,19(18.4%)participants developed recurrence of AA.Use of ACEI or ARB therapy was noted in the study population.Patients on ACEI/ARB had a greater prevalence of hypertension and coronary artery disease.On a multivariate model adjusted for baseline demographics and risk factors for AF,ACEI or ARB therapy did not prevent recurrence of AA following CBA(P=0.72).Similarly,on Kaplan–Meier analysis pretreatment with ACEI/ARB did not predict the time to first recurrence of AA(P=0.2173).CONCLUSION In our study population,preablation treatment with an ACEI or ARB had no influence on the recurrence of AA following CBA for paroxysmal AF.
文摘Introduction: Posterior canal benign paroxysmal positional vertigo (PC-BPPV) is considered the mostcommon cause of peripheral vertigo in the emergency department (ED). Although the canalith repositioning maneuver (CRM) is the standard of care, the most effective method to deliver it in the ED hasbeen poorly studied.Objective: To compare two protocols of the Epley maneuver for the treatment of PC-BPPV.Patients and methods: We prospectively recruited 101 patients with unilateral PC-BPPV on physical examination, randomizing them to either a single Epley maneuver (EM) (n ¼ 46) or multiple maneuvers(n ¼ 55) on the same visit. Measured outcomes included presence/absence of positional nystagmus,resolution of vertigo, and score on the dizziness handicap inventory (DHI) at follow-up evaluations. TheDHI was stratified into mild ( 30) and moderate-severe (>30).Results: Normalization of the Dix-Hallpike maneuver at day 5 was observed in 38% of the single EMgroup and 44.4% in the multiple EM group (p ¼ 0.62). The DHI showed reduction from 42.2 (SD 18.4) to31.9 (SD 23.7) in the single EM group and from 43.7 (SD 22.9) to 33.5 (SD 21.5) in the multiple EM group(p ¼ 0.06). A higher number of patients improved from moderate-severe to mild DHI (p ¼ 0.03) in thesingle EM group compared to the multi-EM group (p ¼ 0.23).Conclusion: There was no statistically significant difference between performing a single EM versusmultiple EMs for treatment of PC-BPPV in the emergency department. The single EM approach isassociated with shorter physical contact between patients and examiner, which is logically safer in apandemic context.
文摘Objective:To study the potential role of subjective visual vertical(SVV)as a prognostic marker for canalith repositioning maneuver(CRM)in patients with posterior canal benign paroxysmal positional vertigo(PC-BPPV)for the Indian population.Methods:SVV was examined in 30 patients with PC-BPPV before and after canalith repositioning maneuver and after complete resolution of PC-BPPV.Study parameters included the mean of 10 angular tilt readings and direction of deviation,which were compared before and after CRM and following complete resolution of PC-BPPV.Results:The angle of SVV tilt was greater and deviated towards the affected ear before CRM in all patients,which decreased significantly shortly after CRM and continued to decrease after complete resolution of PC-BPPV(p<0.0001).Conclusions:SVV can be used to test utricular dysfunction in PC-BPPV.The angle of tilt improves in response to CRM,which may be used as a prognostic marker in patients with PC-BPPV receiving CRM.
基金supported by grants from the National Natural Science Foundation of China(82101526,82171238,and 81330025)。
文摘KCNA1 is the coding gene for Kv1.1 voltage-gated potassium-channelαsubunit.Three variants of KCNA1 have been reported to manifest as paroxysmal kinesigenic dyskinesia(PKD),but the correlation between them remains unclear due to the phenotypic complexity of KCNA1 variants as well as the rarity of PKD cases.Using the whole exome sequencing followed by Sanger sequencing,we screen for potential pathogenic KCNA1 variants in patients clinically diagnosed with paroxysmal movement disorders and identify three previously unreported missense variants of KCNA1 in three unrelated Chinese families.The proband of one family(c.496G>A,p.A166T)manifests as episodic ataxia type 1,and the other two(c.877G>A,p.V293I and c.1112C>A,p.T371A)manifest as PKD.The pathogenicity of these variants is confirmed by functional studies,suggesting that p.A166T and p.T371A cause a loss-of-function of the channel,while p.V293I leads to a gain-of-function with the property of voltage-dependent gating and activation kinetic affected.By reviewing the locations of PKD-manifested KCNA1 variants in Kv1.1 protein,we find that these variants tend to cluster around the pore domain,which is similar to epilepsy.Thus,our study strengthens the correlation between KCNA1 variants and PKD and provides more information on genotype–phenotype correlations of KCNA1 channelopathy.
文摘BACKGROUND Idiopathic non-cirrhotic portal hypertension(INCPH)is mainly associated with thrombophilia in Western countries.Paroxysmal nocturnal hemoglobinuria(PNH)is a rare hematologic disease that manifests with hemolytic anemia,thrombosis,and peripheral blood cytopenias.Portal and hepatic venous thrombosis were reported in PNH.A rare case of INCPH complicating PNH is described.CASE SUMMARY A 63-year old woman with a 2-year past medical history of PNH without treatment was admitted because of jaundice and refractory ascites requiring large volume paracentesis.Liver histology revealed portal venopathy with portal fibrosis and sclerosis,nodular regenerative hyperplasia,parenchymal ischemic changes,and focal sinusoidal and perivenular fibrosis without bridging fibrosis or cirrhosis,all indicative of INCPH.The flow cytometry confirmed PNH diagnosis and eculizumab treatment was initiated.Her condition was improved gradually,bilirubin was normalized 6 months following initiation of eculizumab,and 1 year later diuretics were stopped.CONCLUSION Eculizumab improved intravascular hemolysis and reversed clinical manifestations of INCPH in a patient with paroxysmal nocturnal hemoglobinuria.
基金supported by Project of National Ministry of Industry and Information Technology(No.2020-0103-3-1-2)National Natural Science Foundation of China(No.81670217).
文摘BACKGROUND Catheter-based pulmonary vein isolation(PVI) is an effective and well-established intervention for symptomatic paroxysmal atrial fibrillation(PAF). Nevertheless, late recurrences of atrial fibrillation(LRAF) occurring during 3 to 12months are common, and the underlying mechanisms remain elusive. Circular RNAs(circ RNAs) in atrial tissue have been linked to the pathophysiological mechanisms and progression of PAF in a few studies. However, their expression patterns in peripheral blood and regulatory function in LRAF are not clear.METHODS In the present study, the expression profile of circulating circ RNAs in three paired nonvalvular PAF patients with or without LRAF was investigated by high-throughput sequencing and validated by quantitative real-time polymerase chain reaction(q RT-PCR). Bioinformatics analyses, including Gene Ontology(GO), Kyoto Encyclopedia of Genes and Genomes(KEGG)pathway analysis, and circ RNA/mi RNA regulatory network, were performed to predict the functions and potential regulatory roles of differentially expressed(DE) circ RNAs.RESULTS A total of 12,834 circ RNAs, comprising 5,491 down-regulated and 7,343 up-regulated circ RNAs, were found to be DE in blood smaples from the two groups in peripheral blood between LRAF and non-recurrence control individuals. The most enriched GO categories in terms of molecular function, biological process, and cellular component features were catalytic activity,cellular metabolic process, and intracellular part, respectively. The KEGG enrichment study revealed that the most important metabolic process controlled by DE circ RNAs is endocytosis. In the circ RNA/micro RNAs interaction network, four up-regulated circ RNAs(hsa_circ_0002665, hsa_circ_0001953, hsa_circ_0003831, and hsa_circ_0040533) and one down-regulated circ RNA(hsa_circ_0041103) were predicted to play potential regulatory roles in the pathogenesis of LRAF.CONCLUSIONS This investigation discovered the expression pattern of circulating circ RNAs that is indicative of PAF late recurrence, which may serve as risk markers or therapeutic targets for LRAF after PVI.
文摘This study was aimed to analyze the clinical features of paroxysmal kinesigenic dyskinesia (PKD) and extend the understanding of this disease. From August, 2008 to October, 2010, 9 patients were diagnosed with PKD in the Department of Neurology of the First Affiliated Hospital of Zhejiang University, China. The data involving clinical demographic characteristics, somatosensory evoked potentials, results of electromyography, video electroencephalography (EEG), brain magnetic resonance imaging (MRI) and computerized tomography (CT) were collected. All PKD patients exhibited unilat-eral or bilateral recurrent episodic dyskinetic attacks triggered by sudden voluntary movements. The duration of the attacks ranged from several seconds to one minute. The attack frequency ranged from approximately once in several months to more than 10 times in a day. Patients suffered from no conscious disorders during the attack, and no neurological signs were found during the period between attacks. No abnormal somatosensory evoked potentials were found. Routine EEG, video EEG monitoring or brain imaging showed normal findings. Classical treatment for anti-epilepsy, including car-bamazepine and topiramate, was administered to the patients and proved to be effective. It was concluded that PKD is characteristically triggered by sudden voluntary movement; no abnormal elec-troneurophysiological findings are observed in PKD, and antiepileptic drugs are effective in treating the disorder.
文摘Radioimmunoassays were used to measure the concentration changes of plasma endothelin(ET) and atrial natriuretic peptide(ANP) during the onset and after termination of paroxysmal supraventricular tachycardia(SVT). 30 cases were reviewed and compansons with 42 normal subjects were made. There are very significant differences(P<0.0001) in the concentration changes of both plasma ET and ANP during the onset and 30 minutes after the termination of SVT. During the onset period of SVT. the plasma ET and ANP were markedly elevated and 30 minutes after its termination they were lowered significantly, but their concentrations were still 2-fold higher than ihose of the control group. As the biological effects of ANP and ET are antagonistic to each other. their parallel elevation and lowering of plasma concentrations during and.after the termination of SVT reveal that these 2 hormones parucipate in the pathophysiological process of SVT. This phenomenon is possibly one of the homeostatic regulatory functions in the organism.
