Objective To discuss the clinical significance of percutaneous cervical diskectomy (PCD) in the management of radicular type cervical spondylosis. Methods A retrospective analysis was carried out on clinical records of 106 radicular type cervical spondylosis cases treated by PCD from July 1993 to June 2002. Results Follow-up observations for 6 months～8 years(mean 3.6 years) in 91 cases showed the outcomes were excellent in 36 cases, good in 40, and unchanged in 15, the rate of excellent-or-good results being 83.5%. Conclusions PCD has an excellent therapeutic effect for nerve-root symptoms resulted from cervical disc herniation, such as numbness and pain of the upper extremities or shoulders.

Objective To discuss the clinical value of the treatment of intercondylar fractures of the distal femur using open reduction and internal fi xation. Methods From July 1995 to December 2001, a total of 32 intercondylar fra ctures of the distal femur were treated with open reduction and internal fixatio n with a plate, 95? blade plate, a dynamic condylar screw (DCS) or a condyla r buttress plate. There were 13 cases of type-C1, 10 type-C2 and 9 type-C3 ac cording to the AO/ASIF standard. The outcomes of internal fixation were evaluate d according to preoperative and postoperative radiographs and postoperative func tions of the knee. Results 27 patients were followed-up from 8 months to 8 year s. The results were excellent in 14 cases, good in 9, poor in 4 according to San ders standard. Conclusion Open reduction and internal fixation is an ideal ch oice for intercondylar fractures of the distal femur.

Objective To evaluate the clinical effect of salmeterol xinafoate and fluticasone propionate powder for inhalation combined ipratropium bromide aerosol in patients with stable chronic obstructive pulmonary disease (COPD).Methods One hundred and twenty patients with stable COPD was randomly divided into control group and observation group with 60 patients each.The control group was received ipratropium bromide aerosol inhalation,and the observation group was given salmeterol xinafoate and fluticasone propionate powder for inhalation and ipratropium bromide aerosol for 6 months.BODE index,SGRQ scores,plasma cortisol and bone mineral density were compared between two groups before and after treatment.Results BODE index scores had no significant difference between two groups before treatment (P＞0.05).After treatment,BODE index scores,dyspnea scale,FEV1％,6MWD and BODE index total scores in observation group were superior to those in control group [ (1.9 ± 0.5) grades vs. (2.3 ± 0.5)grades,(58.5 ± 7.3)％ vs.(50.4 ± 6.2)％,(411.1 ± 56.8) m vs.(347.5 ± 60.4) m,(3.3 ± 1.0) scores vs.(3.8 ± 1.3 ) scores ],there were significant differences (P＜0.05 ).SGRQ scores had no significant difference between two groups before treatment (P＞0.05 ).After treatment and in SGRQ scores,respiration symptom,limitation of activity,disease influence scores in observation group were significantly lower than those in control group [ (20.7 ± 10.3 ) scores vs.(37.9 ± 14.4) scores,(20.7 ± 9.5 ) scores vs.(34.8 ± 13.0) scores,(16.3 ± 6.7) scores vs.(27.2 ± 11.8) scores,(17.5 ± 7.0) scores vs. (34.6 ± 12.3) scores],there were signiticant differences (P＜0.01 ).There were no significant difference of plasma cortisol and bone mineral density between two groups (P＞0.05).Conclusions Salmeterol xinafoate and fluticasone propionate powder for inhalation and ipratropium bromide aerosol in patients with stable COPD can greatly improve the lung function and life quality and with less adverse reaction.

Objective To study experienced doctors’ treatment of senile dementia based on association rule. Methods Databases, such as CNKI, CSCD, CBMdisc, CPD, and Wanfang Data were searched for articles about senile dementia. SPSS17.0 and Clementine12.0 software were used for frequency and correlation analysis of prescription. Results In total of 67 articles about TCM therapy for senile dementia were included, containing 112 prescriptions, 178 Chinese herbal medicines, 1589 frequency, and 14.2 entries for each prescription. The first three frequently used herbal medicines were Acori Tatarinowii Rhizoma, Polygala Radix, and Salviae Miltiorrhizae Radix et Rhizoma. Accoring to association rule, the first three medicine pairs were Citrus Reticulatae Pericarpium→Pinelliae Rhizoma, Dioscoreae Rhizoma→Rehmanniae Radix Praeparata, and Codonopsis Radix→Citrus Reticulatae Pericarpium. The first three thriple herbal medicines were Dioscoreae Rhizoma+Lycii Fructus→Corni Fructus, Acori Tatarinowii Rhizoma+Corni Fructus→Rehmanniae Radix Praeparata, and Carthami Flos + Hirudo→Bupleuri Radix. Conclusion The results based on association rule effectively summarized all the doctors’ treatment experience in senile dementia, and provided the beneficial reference for the clinical treatment of senile dementia.

Objective To explore the distribution characteristics of TCM syndrome and clinical relevant factors of benign prostatic hyperplasia (BPH).Methods Totally 781 cases of BPH patients were collected. Chi-square test, analysis of rank and inspection were used to detect the distribution characteristics of TCM syndrome. The multiple classification logistic regression was used to analyze related factors.Results BPH 14 kinds of distribution of syndromes were statistically significant (P<0.01). One of the highest incidence is qi deficiency and blood stasis syndrome (24.97%), and lung heat obstruction is the lowest (1.15%). Composite syndromes (529 cases) were more common than single syndrome (252 cases); syndromes of intermingled deficiency and excess (453 cases) were more common than simple excess (169 cases) or deficiency syndromes (159 cases). BPH single syndrome distribution was different in prostate volume, residual urine volume, maximum urine flow rate, and showed no difference in the serum PSA level. 3 logit model was established successfully. It was found that the patients with the features of 50 to 60 years old, the course < 3 years, I-PSS score for 8 to 19 points showed higher positive incidence of excess syndrome than intermingled deficiency and excess syndromes and deficiency syndrome.Conclusion This study reveals the BPH distribution characteristics and clinical related factors, which has guiding significance to improve TCM clinical level.

OBJECTIVE: To explore the genetic basis for a girl featuring epilepsy, developmental delay and regression. METHODS: Clinical data of the patient was collected. Activities of hexosaminidase A (Hex A) and hexosaminidase A&B (Hex A&B) in blood leukocytes were determined by using a fluorometric assay. Peripheral blood samples were collected from the proband and six members from her pedigree. Following extraction of genomic DNA, whole exome sequencing was carried out. Candidate variants were verified by Sanger sequencing. RESULTS: Enzymatic studies of the proband have shown reduced plasma Hex A and Hex A&B activities. Genetic testing revealed that she has carried c.1260_1263del and c.1601G>C heterozygous compound variants of the HEXB gene. Her mother, brother and sister were heterozygous carriers of c.1260_1263del, while her father, mother, three brothers and sister did not carry the c.1601G>C variant, suggesting that it has a de novo origin. Increased eosinophils were discovered upon cytological examination of peripheral blood and bone marrow samples. CONCLUSION The compound heterozygous variants of c.1260_1263del and c.1601G>C of the HEXB gene probably underlay the Sandhoff disease in this child. Eosinophilia may be noted in infantile Sandhoff disease.
Child ; Eosinophilia/genetics* ; Female ; Genetic Testing ; Hexosaminidase A/genetics* ; Hexosaminidase B/genetics* ; Humans ; Male ; Mutation ; Pedigree ; Sandhoff Disease/genetics*

OBJECTIVE: To explore the genetic basis for a child with myopathy and cerebellar atrophy with ataxia. METHODS: Clinical examinations and laboratory testing were carried out for the patient. The proband and the parents' genomic DNA was extracted from peripheral blood samples and subjected to trio whole-exome sequencing. Candidate variant was validated by Sanger sequencing. RESULTS: The 1-year-and-8-month-old boy manifested motor developmental delay, ataxia, hypomyotonia, increased serum creatine kinase. Cranial MRI showed cerebellar atrophy with progressive aggravation. Genetic testing revealed that the patient has harbored compound heterozygous variants of the MSTO1 gene, namely c.13delG (p.Ala5ProfsTer68) and c.971C>T (p.Thr324Ile), which were respectively inherited from his mother and father. The former was unreported previously and was predicted to be likely pathogenic, whilst the latter has been reported previously and was predicted to be of uncertain significance. CONCLUSION The compound heterozygous c.13delG (p.Ala5ProfsTer68) and c.971C>T (p.Thr324Ile) variants probably underlay the disease in the proband. Above finding has enriched the spectrum of MSTO1 gene variants underlying mitochondrial myopathy and cerebellar atrophy with ataxia.
Ataxia/genetics* ; Atrophy/genetics* ; Cell Cycle Proteins/genetics* ; Child ; Cytoskeletal Proteins/genetics* ; Humans ; Infant ; Male ; Mitochondrial Myopathies ; Mutation ; Neurodegenerative Diseases ; Whole Exome Sequencing

Objective To explore EEG characteristics and the therapeutic effect in children with electrical status epilepticus during slow sleep(ESES).Methods The eligible ESES cases in our center from 2014 to 2020 were included.The age at diagnosis of ESES,the duration of ESES,spike wave index(SWI)during wakefulness and the distribution of spike wave during the period of ESES,age at seizure onset,the clinical syndromes and the outcomes after treatment were analyzed.The ESES cases were divided into 4 groups according to the distribution of spike wave:focal ESES,unilateral ESES,bilateral asymmetric ESES,multiple foci ESES.The SWI during the awake stage were divided into 3 groups based on the different rates:≤20%,21%～49%,≥50%.The therapeutic outcomes were classified into three groups:satisfactory response,seizure control and ineffective.Results 50 cases were included,with 32 males and 18 females.The average onset age of ESES was 6 years and 7 months,and the average duration of ESES was 28 months.A significant correlation between the distribution of ESES and the thera-peutic effects was found,bilateral asymmetric ESES had a good therapeutic effects,while multiple foci ESES showed a poor therapeutic effects.The duration of ESES was significantly correlated with therapeutic effects,and the efficacy was worse when the duration was longer than 1 year.A significant relationship between the SWI during wakefulness of ESES and the therapeutic effects was detected,the patient with SWI≤20%during wakefulness had a good therapeutic effect.There was a negative correlation between the onset age of ESES and the duration of ESES and SWI index during wakefulness.There was a positive correlation between the duration of ESES and SWI index during wakefulness.Conclusion Our results suggest that onset age,distribution,duration and SWI during wake-fulness of ESES were correlated with therapeutic outcomes,The patient with SWI≤20%during wakefulness had a good therapeutic effect and have unfavorable outcomes with ESES last more than 1 year.The earlier onset of ESES,the longer duration of ESES and higher SWI during wakefulness will be showed..

Objective: To investigate the clinical characteristics, treatment and prognosis of relapsed demyeli-nating disease (RDD) associated with myelin oligodendrocyte glycoprotein antibodies (MOG abs) children in southern China. Methods: Children with RDD associated with MOG abs at Department of Neurology in Guangzhou Women and Children′s Medical Center from January 2015 to December 2018 were retrospectively analyzed.The annualized relapse rates (ARRs) and expand disability status scale (EDSS) were used to assess the recurrence frequency and neurological dysfunction respectively. Results: Ten children were included with the age of (6.4±3.6) years old, and male to female ratio was 4∶6.(1)Clinical phenotype: all children had 24 episodes during follow-up, with acute disseminated encephalomyelitis (ADEM)(7/10 cases) and neuromyelitis optica spectrum disorders (NMOSD)(3/10 cases) on the first episode.Among 14 recurrent episodes, ADEM (9/14 times) was the most common, followed by optic neuritis(ON)(3/14 times)and brainstem encephalitis (2/14 times). By the final follow-up, the final diagnosis was multiphasic disseminated encephalomyelitis(MDEM)(6/10 cases), NMOSD(3/10 cases), ADEM-ON(1/10 case), respectively.(2)Laboratory examination: all the children had positive serum MOG abs in the acute stage.The serum MOG abs titer high group(≥1∶640)(6 cases)on the first episode complicated ON (3 cases) and long segment myelitis (3 cases) more common than those of low group(1∶320)(4 cases). (3)Imaging changes: 25 times of bain magnetic resonance imaging (MRI) were performed in the acute stage, MRI changes mostly involved the cortex and subcortical white matter.Four cases had abnormal spinal cord MRI.(4)Treatment and prognosis: intravenous methylprednone (IVMP) combined with intravenous immunoglobulin (IVIG) were administrated in acute stage.Rituximab (2/10 cases), mycophenolate mofetil (4/10 cases), IVIG (2/10 cases) monthly and low dose prednisone orally (2/10 cases) were given respectively in maintains stage.ARRs decreased from 1.4 to 0 and EDSS score improved significantly after these treatments above.Seven cases had residual neurological dysfunction with 3 cases of NMOSD, 3 cases of MDEM and 1 case of ADEM-ON, including motor dysfunction, learning disability and inattention, symptomatic epilepsy and visual impairment. Conclusions ADEM is the most common form of RDD associated with MOG abs in children.Those with high serum MOG abs titer on the first episode are prone to have ON or long segment myelitis.Immunomodification therapy is effective in the relapsed patients, residual neurological sequelae were related to the type of repeated demyelination.

Objective:To investigate the variation of T, B, NK lymphocyte subgroup in children with anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis and their clinical significance.Methods:This was a prospective and control study.Forty children primarily diagnosed with anti-NMDAR encephalitis in the department of neurology in Guangzhou Women and Children′s Medical Center from January 2017 to August 2019 served as patient group, 20 healthy children served as control group.Absolute counts and percentages of T, B and NK lymphocytes in whole blood were detected before and 1 month after treatment in patient group.Serum immunoglobulin G(IgG), IgA and IgM were measured before treatment.The blood levels of T, B, NK lymphocyte subgroup were detected with flow cytometer.NMDAR antibody titers of serum and cerebrospinal fluid were detected in patient group.The differences between patient group at different time points and control group were compared.The patients were divided into two groups according to the response to treatment after 2 weeks and the absolute counts of T, B and NK lymphocytes before treatment were compared between groups.Results:Compared with control group, the blood absolute count of B lymphocyte in patient group were significantly higher before and after treatment( P<0.05). There was no significant difference of B lymphocyte in patient group between before and after treatment.After treatment, T cells(including T inhibitory cells and T helper cells)were significantly increased compared with those before treatment and those in control group( P<0.05), but there was no significant difference between patient group and control group before treatment.These with poor response to treatment after 2 weeks had higher level of B, T lymphocyte subgroup compared to those with good response( P<0.05). The level of IgG, IgA, IgM in patient group showed no significant difference with control group.There was no significant correlation between B lymphocyte count in blood and NMDAR antibody titer in cerebrospinal fluid( r=0.282, P>0.05). Conclusion:B lymphocytes increase greatly in children with anti-NMDAR encephalitis, and the level of B lymphocyte subgroup before treatment are associated with treatment response, and T lymphocytes increase greatly after treatment.There is no significant correlation between the titer of NMDAR antibody in cerebrospinal fluid and B lymphocyte level.