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Objective:To explore the related factors affecting the prognosis of patients with tardive dyskinesia, in order to find the risk factors of poor prognosis.Methods:A retrospective cohort study was implemented to collect the data of 113 patients with tardive dyskinesia from the movement disorders clinic of the Department of Neurology, Peking Union Medical College Hospital by telephone follow-up. The main variables studied included gender, age, educational level, living environment, job, cigarette smoking, alcohol drinking, hypertension, diabetes, medication type, interval from taking medicine to dyskinesia, course of dyskinesia and intervention measures. The data were analyzed by the statistical software SPSS 25.0. The quantitative data analysis was done by normality test and descriptive statistics, and the qualitative data analysis was done by χ 2 test or Fisher exact probability test. The variables with P≤0.01 in univariate analysis were included in multivariate Logistic regression analysis. Results:Among the 113 patients, 16 patients (14.16%) were cured, 27 patients (23.89%) had obvious improvement, 25 patients (22.12%) had slight improvement, 26 patients (23.01%) had no obvious change, 15 patients (13.27%) had slight deterioration, and 4 patients (3.54%) had obvious deterioration. The good prognosis rate was 60.18%, and the poor prognosis rate was 39.82%. In single factor analysis, the related factors for poor prognosis included age>52 years (χ 2=15.07, P<0.001), educational level in secondary schools and below (χ 2=8.58, P=0.003), physical labor (χ 2=4.66, P=0.031), hypertension (χ 2 = 16.38, P<0.001), diabetes mellitus (χ 2=6.06, P=0.011), dyskinesia caused by first-generation antipsychotics, calcium channel blocker or flupentixol/melitracen tablets ( P<0.001), and the duration of dyskinesia more than 2 years (χ 2 =7.05, P=0.008). Multivariate Logistic regression analysis showed that the independent risk factors for poor prognosis of tardive dyskinesia included hypertension ( OR=3.60, 95% CI 1.17-11.05, P=0.025) and dyskinesia caused by first-generation antipsychotics, calcium channel blocker or flupentixol/melitracen tablets ( OR=3.14, 95% CI 1.21-8.16, P=0.019). Conclusions:Most patients with tardive dyskinesia have a good prognosis. Hypertension and dyskinesia caused by first-generation antipsychotics, calcium channel blocker or flupentixol/melitracen tablets are independent risk factors for poor prognosis.
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Objective:To summarize the clinical manifestation and imaging of superficial siderosis of the central nervous system and explore the potential etiology.Methods:The clinical and imaging data of 7 patients diagnosed as superficial siderosis of the central nervous system in Peking Union Medical College Hospital from May 2013 to November 2019 were retrospectively reviewed. The etiology and follow-up prognosis through phone call were analyzed.Results:There were 7 patients included (3 male and 4 female) with an average age of 53 years (41-58 years). The cardinal manifestations were sensorineural deafness (all 7 cases), cerebellar ataxia (all 7 cases) and pyramidal signs (all 7 cases). Dizziness (6 cases), bladder disturbance (5 cases), headache (3 cases), double vision (2 cases) and congnitive impairment (1 case) could also happen. Magnetic resonance imaging showed symmetrical well-defined curvilinear homogeneous low signal on T 2 or blood-sensitive sequences (T 2* gradient echo or susceptibility-weighted imaging) over the superficial surface of cerebellar, brain stem, and spinal cord or cranio-cervical junction. All the 7 patients showed cerebellar atrophy especially the upper vermis. The potential causes included trauma history in 3 cases, intraspinal fluid-filled collection which indicated dural defect or duropathologies in 3 cases, intraspinal mass in 1 case and vertebral and disc degeneration in all 7 patients. The 5 patients who successsfully got follow-up showed exacerbation of variable degree. Conclusions:Classical superficial siderosis of the central nervous system is a rare disease with cardinal manifestation of progressive ataxia, sensorineural deafness and pyramidal signs. T 2WI of magnetic resonance imaging showing low signal over the superficial surface of cerebellar, brain stem, and spinal cord could indicate the diagnosis, and blood-sensitive sequences such as T 2* gradient echo or susceptibility-weighted imaging were more sensitive. Duropathologies or dural defect may be the most probable causes of the disease and should be examined and treated carefully.
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Dystonia is a highly heterogeneous disorder both in clinical and genetical manifestation. It is sometimes associated with poor outcomes in clinical practice. Understanding of the definition, classification, mechanism of dystonia is helpful for optimizing management and improving the implementation. Genetic testing for dystonia is important as an accurate molecular diagnosis in certain genes which can influence therapeutic decisions. Referring to the current situation of clinical practice and expert views in China, this article shares the principles of clinical diagnosis and treatment and the application of gene testing, hoping to provide reference to the diagnosis and treatment decisions of dystonia for the ordinary neurologists in the process of medical practice.
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Objective:To investigate the characteristics of gastrointestinal (GI) symptom spectrum in patients with Parkinson′s disease (PD), and to help the early identification of gastrointestinal symptoms and management of PD.Methods:One hundred PD patients in the Department of Neurology, Peking Union Medical College Hospital from January 2017 to August 2017 were enrolled in this study. They were assessed by face-to-face GI dysfunction questionnaire, including eight common symptoms involved in oropharynx, upper and lower digestive tract. The Spearman correlation analysis was performed.Results:The age of PD patients was (61.9±10.5) years, the ratio of male to female was 53∶47 and the disease duration was 4.0 (2.0, 6.0) years. There were 42 cases of Hoehn-Yahr (H-Y) stage 1, 30 cases of H-Y stage 2 and 28 cases of H-Y stage 3 and above (24 cases of H-Y stage 3, three cases of H-Y stage 4 and one case of H-Y stage 5). Totally 58% (58/100) of PD patients had one or more GI symptoms. Constipation (42%, 42/100), dysdefecation (38%, 38/100) and salivation (28%, 28/100) were the top three of most common GI symptoms. Lower GI symptoms were the most common (57%, 57/100), followed by oropharyngeal symptoms (33%, 33/100), and upper GI symptoms (27%, 27/100). GI symptoms could appear in H-Y stage 1 patients, 26.1% (11/42) of which had 1-2 kinds of GI symptoms and over 20% of which had more than three kinds of GI symptoms. A total of 39.3% (11/28) of PD patients with H-Y stage ≥3 had more than three kinds of GI symptoms. The Gastroparesis Cardinal Symptom Index (GCSI) score in patients with upper GI symptoms was 3.0 (2.0,6.5). The constipation symptom score in patients with constipation and dysdefecation was 19.0 (12.0,27.3). As for the clinical type of constipation, 66.7% (38/57) of them were mixed, 21.0% (12/57) were slow transit and 12.3% (7/57) were dysdefecation. In 38.6% (22/57) of the constipated patients, constipation symptoms occurred earlier than PD motor symptoms. Correlation analysis showed that H-Y stage was positively correlated with the course of PD, the number of GI symptoms, salivation, constipation, dysdefecation and constipation symptom scores.Conclusions:Constipation, dysdefecation and salivation were the most common GI symptoms in PD patients. PD patients had at least one GI symptom in the early stage (H-Y stage 1). Lower GI symptoms were more common than oropharyngeal symptoms and upper GI symptoms. With the development of PD, the number of GI symptoms, salivation, constipation and dysdefecation were aggravated, which were important for early symptomatic identification and disease management.
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In 2013,consensus on the phenomenology and classification of dystonia was updated and dystonia was classified into isolated dystonia and combined dystonia according to whether combined with other movement disorders.Up to now,the genes for isolated dystonia include TOR1A,THAP1,ANO3,GNAL,TUBB4A,and newly reported HPCA and COL6A3.As for the combined dystonia,genes for dystonia plus myoclonus include SGCE,CACNA1B,KCTD17,guanosine triphosphate cyclohydrolase 1 and tyrosine hydroxylase;genes for dystonia plus parkinsonism include TAF 1,GCH 1,TH,ATP1A3,PRKRA.Based on the clinical features of dystonia,a comprehensive review of related genes is helpful for its accurate diagnosis.
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As a local and effective denervation method,botulinum toxin has been applied in more and more fields.Practitioners who use botulinum toxin should consider the general situation of patients based on the view of effectors,weigh the pros and cons and set reasonable goals before injection,referring to the Chinese expert consensus.
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Objective To evaluate the safety and efficacy of botulinum toxin type A for injection in the treatment of post-stroke upper limb spasticity (dosage was 200 U,or 240 U if combined with thumb spasticity).Methods The study was a multi-center,stratified block randomized,double-blind,placebocontrolled trial.All the qualificd subjects were from 15 clinical centers from September 2014 to February 2016.They were randomized (2∶1) to injections of botulinum toxin type A made in China (200-240 U;n =118) or placebo (n =60) in pivotal phase after informed consent signed.The study was divided into two stages.The pivotal trial phase included a one-week screening,12-week double-blind treatment,followed by an expanded phase which included six-week open-label treatment.The tone of the wrist,finger,thumb flexors was assessed at baseline and at weeks 0,1,4,6,8,12,16 and 18 using Modified Ashworth Scale (MAS),disability in activities of daily living was rated using the Disability Assessment Scale and impaction on pain,muscle tone and deformity was assessed using the Global Assessment Scale.The primary endpoint was the score difference between botulinum toxin type A and placebo groups in the tone of the wrist flexor using MAS at six weeks compared to baseline.Results Muscle tone MAS score in the wrist flexor of botulinum toxin type A and placebo groups at six weeks changed-1.00 (-2.00,-1.00) and 0.00 (-0.50,0.00) respectively from baseline.Botulinum toxin type A was significantly superior to placebo for the primary endpoint (Z =6.618,P ＜ 0.01).The safety measurement showed 10 subjects who received botulinum toxin type A had 13 adverse reactions,with an incidence of 8.47％ (10/118),and three subjects who received placebo had three adverse reactions,with an incidence of 5.00％ (3/60) during the pivotal trial phase.All adverse reactions were mild to moderate,none serious.There was no significant difference in adverse reactions incidence between the botulinum toxin type A and the placebo groups.During the expanded phase three subjects had four adverse reactions and the incidence was 1.95％.All adverse reactions were mild,none serious.Conclusion Botulinum toxin type A was found to be safe and efficacious for the treatment of post-stroke upper limb spasticity.Clinical Trial Registration:China Drug Trials,CTR20131191
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Objective To assess the classification and characteristics of headaches using the International Classification of Headache Disorders,3rd edition (beta version) criteria in a headache outpatient clinic.Methods In this prospective cross-sectional study,all consecutive patients presenting with headache to a headache outpatient clinic of Peking Union Medical College Hospital between April 1 st,2014 and February 28th,2015 were included.Results The average age of 593 patients was (42.I ± 15.6) years with female-male ratio 1.89∶ 1.Primary headaches were found in 483 (81.5％) patients,of which migraine and tension-type headache were found in 264 (44.5％) patients and 168 (28.3％) patients respectively.Chronic daily headache and new headache were found in 158 (26.6％) patients and 130 (21.9％) patients,while cough headache and thunderclap headache were found in 9 (1.5 ％) patients and 5 (0.8％) patients respectively.Patients with migraine were more likely complicated with medicationoveruse headache than patients with tension-type headache (x2 =4.21,P =0.032).Patients with tensiontype headache were more likely complicated with chronic daily headache than patients with migraine (x2 =18.92,P =0.000).Conclusions In this headache outpatient clinic,most patients were primary headaches.Chronic daily headache and new headache were common,while cough headache and thunderclap headache were uncommon.These headache syndromes should be paid attention to identify the possible underlying malignant etiologies.
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<p><b>BACKGROUND</b>Hemifacial spasm (HFS) is a facial nerve disorder characterized by episodic involuntary ipsilateral facial muscle contraction. Information on Chinese patients with HFS has not been well-characterized. This study aimed to evaluate the clinical feature and the treatment status of HFS across China.</p><p><b>METHODS</b>A cross-sectional study including 1003 primary HFS patients had been carried out in 15 movement disorder clinics in China in 2012. The investigated information was acquired from questionnaires and medical records including demographic data, site of onset, aggravating and relieving factors, treatments prior to the investigation, etc.</p><p><b>RESULTS</b>In this study, the ratio of male to female was 1.0:1.8, the mean age at onset was (46.6 ± 11.5) years. About 1.0% patients were bilaterally affected. The most often site of initial onset was the orbicularis oculi muscle. The most often affected sites were orbicularis oculi, zygomatic, and orbicularis oris muscles. Stress/anxiety and relaxation were most often aggravating and relieving factors, respectively; 2.3% patients had family history, 28.4% cases were combined with hypertension, and 1.4% patients were with trigeminal neuralgia. Botulinum toxin type A (BTX-A) injection was the most commonly used treatment, followed by acupuncture and oral medication. BTX-A maintained the highest repeat treatment ratio (68.7%), while 98.4% patients gave up acupuncture. The mean latency of BTX-A effect was (5.0 ± 4.7) days, the mean total duration of the effect was (19.5 ± 11.7) weeks, and 95.9% patients developed improvements no worse than moderate in both severity and function. The most common side effect was droopy mouth.</p><p><b>CONCLUSIONS</b>The onset age of HFS in China is earlier than that in western countries. The most often used two treatments are BTX-A injection and acupuncture, while the latter kept the poor repeat treatment ratio because of dissatisfactory therapeutic effect.</p>
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Adult , Female , Humans , Male , Middle Aged , Botulinum Toxins, Type A , Therapeutic Uses , China , Cross-Sectional Studies , Hemifacial Spasm , Diagnosis , Drug Therapy , Neuromuscular Agents , Therapeutic UsesABSTRACT
Objective To summarize the clinical and radiological features of DYT6 dystonia with mutations based on the data of our patient cohort as well as the report by others.Methods Clinical data of the 11 patients with DYT6 dystonia in Peking Union Medical College Hospital from June 2009 to May 2012 were retrospectively reviewed and analyzed.Clinical data included gender,onset age,initiative symptom of onset,the sites of involvemet,family history,etc.All patients were examined for brain MRI scan,6 patients were examined for DTI.Results Of the eleven gene-confirmed DYT6 dystonia patients,7 were male and 4 were female,with an onset-age ranged from 5 years to 36 years,the mean age of onset was 19.4years.Eight patients had a family history.There were 10 patients with early onset dystonia and only 1 patient with late onset dystonia.The most common site of onset was the neck (7/11),and the next was the right arm,1-5 body areas were affected at the time of neurological assessment,the average amount was 2.8,and the most frequently affected anatomical site was the neck (10/11),next came lower face,jaw and tongue.Among all the patients,6 patients presented with segmental dystonia,4 patients presented with focal dystonia,only 1 patient presented with generalized dystonia.All the patients with thanatos-associated protein domain-containing apoptosis-associated protein (THAP) domain affected had a family history,but the patients with the same mutant gene varied with clinical manifestation.Only 1 patients with non-THAP domain affected had a family history,but in most families,there were adult asymptomatic mutant gene carriers.Mutations within the THAP domain were associated with an earlier age of onset than non-THAP domain (17.3 and 21.8 years old).Routine MRI of all patients were normal and DTI of 6 patients showed that fractional anisotropy values in the bilateral sensorimotor area in DYT6 dystonia were reduced.A detailed description of a patient with TOR1A and THAP1 gene mutations was given.Conclusions Early onset dystonia is the main manifestation in patients with DYT6 dystonia in China.The most common site of onset is the neck,and the next is the right arm.The most frequently affected anatomical site is the neck,next come lower face,jaw and tongue.Laryngeal dystonia is absent.The patients with same mutant gene show high heterogeneity in the clinical manifestations,mutations within the THAP domain of THAP1 tend to manifest at an earlier age and higher penetration than mutations localized to non-THAP domain.Reduction of fractional anisotropy values indicates that the axonal integrity and coherence in the region of sensorimotor area is damaged in DYT6 dystonia.
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Objective To observe the cerebral activation pattern in patients with cervical dystonia (CD) during finger movements and the changes caused by botulinum toxin injection by functional MRI.The possible etiological mechanism of CD and effect of peripheral botulinum toxin treatment on the level of central nerves system are investigated.Methods A designed functional MRI block with complex finger movements was applied and 11 patients with CD as well as 11 age and gender matched controls were scanned.Compare the activation pattern of CD pre/post treatment groups versus health controls.Evaluate and compare the symtom severity with Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS).Make a correlation analysis between activation pattern alteration and TWSTRS change in CD pre/post treatment groups.Results An reduced extent of activation in patients with CD was demonstrated compared to healthy controls in ipsilateral putamen,prefrontal cortex and contralateral somatosensory cortex to the direction of the head deviation,while an elevated extent of activation in ipsilateral precuneus and fusiform with statistic significance.At the time point of 4 weeks after botulinum toxin treatment patients showed no evident difference with healthy controls except for the decreased activation in contralateral precuneus to the direction of the head deviation.TWSTRS of patients with CD decreased from (20.02 ± 5.52) to (4.11 ± 4.34) with statistic significance (t =11.71,P =0.000) after botulinum toxin treatment.There was a positive correlation between cerebral activation pattern change in some cerebral areas (ipsilateral primary somatosensory cortex,premotor cortex,supplementary motor cortex,insula,fusiform,hippocampus with parahippocampa as well as contralateral middle temporal cortex and hippocampus with parahippocampa to the direction of the head deviation) and TWSTRS score decrease.Conclusions There are widespread abnormalities in cortical and subcortical activation pattern in patients with CD,which might due to dysfunction of sensory-motor integration.We speculate a basic pre-dystonic state is present in affected body parts prior to clinical symptoms appear.Botulinum toxin fulfills its subsequent central effect by reorganizing and normalizing the cerebral cortex in patients with CD.
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Objective To explore the risk factors of benign essential blepharospasm( BEB) and each factor' s intensity.Methods Totally 100 patients with BEB including Meige and cranial-cervical without other neurological abnormalities were recuited from the outpatient clinic of Department of Neurology, Peking Union Medical College Hospital from April 2009 to March 2010 as case group.At the same time, 100 outpatients diagnosed with idiopathic hemifacial spasm ( HFS) and 40 healthy controls without extrapyramidal diseases or other neurological diseases were recuited from the same outpatient clinic.All the cases and controls were investigated by questionnaires about general conditions, social behavioral factors, environmental factors, phychiatrical factors, genetic factors, previous diseases.The data was analysed by Logistic regression model with the statistical software SPSS 11.0.Results Multivariate analysis indicated that eye disease (OR = 5.818, 95% CI 2.510-13.486), anxiety (OR=3.433, 95% CI 1.548-7.610) and depression (OR =2.894, 95% CI 1.091-7.681) were positively related with BEB, whereas coffee drinking (OR = 0.251, 95% CI 0.080-0.784) had a negative relationship with BEB ( all P ＜ 0.05 ).Conclusions The risk factors of BEB are eye diseases, anxiety and depression.Coffee drinking is protective for BEB.The results suggest that local injury and phychiatrical factors may be more important than genetic factor in the aetiology of BEB.
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Objective To evaluate the efficacy of Chinese botulinum toxin A(CBTX-A)injection in the treatment of motor tics in patients with tic disorder.Methods Twenty-five patients(20 male,5 female)were treated with CBTX-A in the sites of their most problematic motor tics.Before treatment the data of Yale Global Tic Severityb Scale(YGTSS)was collected.All patients were assessed at least 3 times after 1 week,4 weeks and within 12-month period.At each visit the following data were collected:YGTSS,Jankovic clinical rating scale,time to response,duration of response,global impression of changes by patients,presence of premonitory sensory tic component and side effects.Results Total number of treatment sessions was 29.The mean response time was 5.0 days.The mean duration of response was 4.6 months.The mean peak effect response in Jankovic clinical rating scale was 3.4.Based on YGTSS,62.1% (18/29)was rated as marked improvement,34.5%(10/29)as moderate improvement and only 3.4% (1/29)as no effect.85.7%(18/21)patients had less premonitory sensation symptoms(mean benefit 72.4%(20%-100%)).Weakness in the site of injection and rash were the main side effect. Conclusion CBTX-A is an effective and safe treatment for motor tics associated with tic disorder.
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Objective To analyze the clinical characteristics of dopa-responsive dystonia (DRD). Methods The clinical characteristics and the causes of misdiagnosis in 15 patients with DRD were reviewed retrospectively.Results 4 male and 11 female patients were investigated in this study. The age at DRD onset was from 3~25 years old,the average age was 11.7 years old.The courses ranged from 2 to 29 years and the average was 12.1 years. The symptoms in female patients often aggravated during gravidity and delivery. Main clinical features included gait irregularity, postural instability and tremor, with marked diurnal fluctuation. The disease may be misdiagnosed as cerebral palsy, idiopathic torsion dystonia, essential tremor, parkinson's disease, neurosis and so on. All the patients in this study improved obviously in 2 weeks after therapy with low dose of Madopar, and recovered in 2~4 years of follow up.Conclusions Clinical diversity and diurnal fluctuation are the characteristics of DRD. Early trial treatment with low dose of Levodopa should be highly recommended for the generalized dystonia in children and untypical adults.