To compare the diagnostic value of high-resolution uhrasonography (HR-US) with nerve conduction study (NCS) in patients with clinically defined carpal tunnel syndrome (CTS),a prospective study was conducted on 37 consecutive patients investigated for sensory hand symptoms. With the clinical diagnosis of CTS as gold standard,NCS showed higher diagnostic sensitivity (80％)than ultrasound (61％) (P =0.047 ).The positive predictive value of HR-US for CTS was 100％.The results indicated that HR-US could be used as a screening method for majority of clinically suspected CTS patients and only for those with negative HR-US results.

Objective To explore the clinical features of Kennedy disease .Methods The clinical data of 3 patients with Kennedy disease was respectively analyzed .Results All three patients were middle-aged male and had a chronic onset .Patients were mainly presented with the muscle weakness and fasciculation in the proximal limb and bulbar , and the symptoms grow progressively .Two patients were hyperplasia of mammary glands , 3 patients had high levels of serum creatine kinase , and 2 patients'serum testosterone level was increased .EMG detected a widespread neuronal damage in all three cases , and the sensory conductions were abnormal in 2 patients.Repeat numbers of CAG in exon 1 of androgen receptor gene of 2 patients were tested, and they were 57, 47 respectively.Conclusions The clinical features of Kennedy disease are muscle weakness and androgen insensitivity syndrome in male patient and EMG presented with motor neuron damaged .Repeat number of CAG in exon 1 of androgen receptor gene is increased.

Objective To study the clinical and electrophysiological features of the patients with hereditary neuropathy with liability to pressure palsy ( HNPP) diagnosed by gene analysis.Methods Seven patients from two HNPP families were assessed on medical history, physical examination, electrophysiology findings and gene analysis.Results A clinical manifestation of acute, painless, recurrent peripheral nerve palsies was typical for HNPP.Median, ulnar and peroneal nerves were usually affected.Electrophysiology study revealed that prolonged distal motor latency and slowing nerve conduction velocity were prominent.Gene studies exhibited a deletion of the peripheral myelination protein 22 gene in all the seven patients.Conclusions HNPP usually affects areas where nerves are subject to entrapment, and many episodes are preceded by minor compression on the affected nerve.As a reliable screening tool in detecting HNPP, the electrophysiological study shows that segmental demyelination is most commonly seen at common nerve entrapment sites.

Objective To investigate the expression of glutathione S-transferase π (Glutathione S-transferase π, GST-π) protein in peripheral blood and brain of patients with drug-resistant epilepsy and refractory epilepsy rats. Meth?ods From January 2010 to March 2014, the expression of GST-πin the blood and brain of 32 cases of drug-resistant epi?lepsy underwent neurosurgery and 10 cases of cerebral vascular malformation underwent surgery were studied and com?pared. The expression of GST-πin the blood and brain in refractory epilepsy rats and normal rats were studied and com?pared. Results The specimen from 20 temporal, 6 frontal and 6 occipital lobes were obtained from drug-resistant epilep?sy patients. The expression levels of GST-πin the blood and brain in refractory epilepsy rats and normal rats were higher than those of the control groups (P<0.05). Conclusion GST-πmay be involved in the process of drug-resistant epilepsy. The GST-πexpression in blood may be used as a marker for resistance to anti-epileptic agents.

Objective To study the value of magnetic resonance imaging (MRI)in diagnosis of cubital tunnel syndrome (CuTS). Methods We studied the findings of electrophysiological examination and MRI in 30 elbows of 23 patients with CuTS and 15 elbows of 1 5 controls.We observed the motor conduction velocity (MCV),cross sectional area (CSA)and relative signal intensity (RSI)of the ulnar nerve acrossing the elbow at the point of largest size proximal to the entrapment point (CSA1 ,RSI1 )and the entrapment point (CSA2 ,RSI2 ).Then we calculated the ratio of CSA (CSAR=CSA1/CSA2 ),and the ratio of RSI (RSIR=RSI1/RSI2 ).Results The value of CSA1 and RSI1 was significantly greater than CSA2 ,RSI2 in the patient group (P<0.05).The value of CSA1 ,RSI1 , CSAR and RSIR in patients were significantly larger than that in controls (P<0.05).MCV was negatively correlated with CSA1 and CSAR in the patient group (r=-0.62,r=-0.53).There were no correlation between MCV and RSI1 ,RSIR in the patient group. The area under ROC curve of CSAR was the largest 0.94 (95% CI,0.83-1).The optimum cutoff point of CSAR was 1.83.The CSAR had sensitivity of 93.3% and specificity of 80% in diagnosis of CuTS.Conclusion MRI combined with the electrophysiologi-cal examination shows a high accuracy of locating the entrapment point of ulnar never lesion at the elbow.The CSAR of the ulnar nerve is the best MRI parameters in diagnosis of CuTS.

Objective To study features of the MRI and clinic in a family with pure hereditary spastic paraplegia (PHSPG) type 6.Methods Target loci (SPG3, 4, 6, 8 10 and 12) linkage analysis was performed in a SPG pedigree having 6 affected individuals using microsatellite markers and NIPA1 gene was screened for mutation by PCR-amplification and sequencing. MRI of brain and cervical and thoracic spinal cord were examined in these 6 patients and 6 normal controls matched for age and sex by two independent radiologists blinded to the clinical diagnosis. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord at the levels of C2～3, C7, T1～4, T9 were measured and data was statistically analyzed using the student's t test. Results A missense mutation of 316g→c in NIPA1 was identified in the affected subjects, presumably resulting in substitution of glutamic acid for arginine in residue 106. Evaluation of the brain MRI images revealed non-specific brain abnormalities. All patients presented thinning of cervical and upper thoracic spine with atrophy in both gray and white matter and enlarged subarachnoid cavity. In severe atrophic segments, a distinct boundary between grey and white matter was observed and the lesions in grey matter presented literal high intensity spots or patches with clear boundary on transaxial T2-weighted images (T2WI) and high signal intensity longitudinal strip on the sagittal T2WI. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord at C2～3, C7, T1～4 were significantly smaller in patients than in controls, while at the T9 level only transverse diameter showed significant difference (7.22±0.08 vs 8.17±0.41, t=2.870, P=0.046). Conclusions These findings indicate that the disease process in patients with SPG6 might be confined to the cervical and thoracic spinal cord, with atrophy in both white and grey matter having a distinct boundary.

Objective:To determine the dosage,method and effectiveness of using magnesium sulfate for treating the intractable epilepsy.Methods:72 cases were allocated to treatment group and 36 to control group. For those in the treatment group,either 10 mL of 25% MgSO4 was administered by intravenous drip or 10 mL of 16.5% MgSO4 by oral intake once per day,in addition to the use of the commonly used antiepilepsy drugs. For those in the control group,the commonly used antiepilepsy drugs such as tegretol,sodium phenytoin,luminal or γ-Aminobutyric Acid were employed.Results:The improvement rate in the treatment group was 87.50%,higher than that (44.44%) in the control group (P＜0.01).Conclusion:Using magnesium sulfate in conjunction with the antiepilepsy drugs is a simple yet effective regime for the treatment of the intractable epilepsy.

OBJECTIVE:To evaluate the effects of clinical pharmacists participating in clinical pathway management for chron-ic heart failure(CHF). METHODS:A total of 107 CHF adult inpatients in Linyi People's Hospital during Jan. 2014-Oct. 2015 were divided into control group(56 cases,3 withdrawal,53 in total)and trial group(58 cases,4 withdrawal,54 in total)accord-ing to random number table. Control group received routine clinical pathway management method of CHF;trial group received clin-ical pathway management with the participation of clinical pharmacists. Clinical efficacy,the utilization of heart failure drugs,eco-nomic indexes,medication compliance after discharge,re-hospitalization rate due to heart failure were compared between 2 groups. RESULTS:Total response rate of trial group was significantly higher than control group,with statistical significance(P<0.05). The utilization rate of ACEI/ARB,β-receptor blocker,target dose rate of ACEI/ARB in trial group were significantly higher than control group,with statistical significance(P<0.05);target dose rate of β-receptor blocker was higher than control group,without statistical significance(P>0.05). Hospitalization time,drug cost,total hospitalization cost and drug ratio of trial group were short-er or lower than control group,without statistical significance(P>0.05). One month after discharge,the proportion of medication compliance in trial group was significantly higher than control group,with statistical significance(P<0.05);re-hospitalization rate was lower than control group,without statistical significance(P>0.05). Three months after discharge,the proportion of medica-tion compliance in trial group was higher than control group,while re-hospitalization rate was lower than control group,with statis-tical significance(P<0.05). CONCIUSIONS:The participation of clinical pharmacists in clinical pathway management of CHF can significantly improve the utilization rate of recommended drugs by guideline,clinical efficacy and medication compliance,and reduce re-hospitalization rate.

Humans ; Pedigree ; Tuberous Sclerosis ; pathology

OBJECTIVETo delineate the clinical, electrophysiological and genetics features of a family where 4 members were affected with hereditary neuropathy with liability to pressure palsies (HNPP).

METHODSClinical features of the 4 patients were summarized. Electrophysiological examination and genetic analysis were carried out.

RESULTSAll of the patients showed recurrent motor and sensory disturbances after minor traction or constriction. Electrophysiology study revealed that the prolonged latency and reduced conduction velocity of peripheral nerve were general and with multiple sites of affection. The nerve locations liable to entrapment showed conduction block. A deletion mutation of peripheral myelin protein 22 (PMP22) gene was identified by genetic analysis.

CONCLUSIONHNPP usually affects areas where nerves are liable to entrapment, and presents with motor and sensory disturbances of the innervated areas. Electrophysiological study reveals general nervous demyelination. Genetic analysis can clarify the diagnosis of HNPP.

Adult ; Arthrogryposis ; genetics ; physiopathology ; Hereditary Sensory and Motor Neuropathy ; genetics ; physiopathology ; Humans ; Male ; Myelin Proteins ; genetics ; Neural Conduction