Objective To explore the characteristics of hereditary spastic paraplegia with thin corpus callosum (HSP-TCC). Methods Clinical, electrophysiological, MRI features of 3 patients with HSP-TCC were reported. The genetic characteristics were reviewed. Results 3 patients revealed difficulty in walking, slowly progressive weakness, spasticity of the lower limbs and mental impairment. The electromyogram in 2 cases showed neurogenic damage in lower limbs muscle, and 1 case showed peripheral nerve damage. Cerebral MRI showed an extremely thin corpus callosum on sagittal image. The locus of 15q13-15 has been identified for HSP-TCC, but some HSP-TCC families have not been linked to this locus.Conclusion HSP-TCC is a common subtype of complicated HSP inherited by autosomal recessive mode. Brain MRI showed extremely thin corpus callosum. Electromyogram many reveal neurogenic damage.

Objective To study the characteristics of peripheral nerve conduction (PNC) in advanced-stage amyotrophic lateral sclerosis (ALS) patients undergoing mechanical ventilation.Methods The sensory conduction velocity (SCV),motor conduction velocity(MCV),distal motor latency(DML) and amplitude of compound muscle action potential(CMAP) were determined in three ALS patients undergoing mechanical ventilation.The clinical data and PNC characteristics of the patients were analyzed.Results The 12 motor nerves were abnormal in all the three patients,including absence of responsive wave (66.7%,8/12),decreased amplitude of CMAP(33.3%,4 /12),prolongation of DML(16.7%,2 /12) and reduction of MCV(16.7%,2 /12).The CMAP amplitude of patients gradually decreased with the progression of disease,and finally led to adverse reactions.The SCV and amplitude of sensory nerve action potential were normal in all the three patients.Conclusion ALS patients undergoing mechanical ventilation may have severely damaged motor nerve conduction,and their sensory nerve is generally not affected,with the specific reason remains to be further studied.

Objective To investigate the clinical, imaging and pathological features of Wernicke's encephalopathy (WE).Methods The clinical, imaging and pathologic datas of 10 patients with WE were analyzed retrospectively.Results 10 patients were not ethylism. 9 cases presented various mental and conscious disturbance, 6 cases initially presented vertigo, nausea and vomiting. 5 cases showed ophthalmoparalysis. 3 cases displayed hypotension.2 cases showed ataxia and 1 case showed severe peripheral neuropathy. 3 of the 5 patients with MRI examinations showed symmetric T_1 and T_2 high signal in encephalocoele and periphery of aqueduct of midbrain, the other 2 cases were no positive finding . 4 cases with supplement VitB_1 were cured, 5 cases died.1 case withdrawed. 5 autopsy cases showed congestion, edema and multiple petechial hemorrhages in encephalocoele and periphery of aqueduct of midbrain.Conclusions The clinical manifestation of WE is atypical, and MRI imaging is helpful for early diagnosis of WE. It is the key treatment to supply the thiamine as early as possible.

Objective To promote the understanding and diagnostic acuity of Hirayama disease by studying the features of electromyogram (EMG) and nerve conduction. Methods EMG was performed in 33 patients with Hirayama disease. Surface electrode was applied to examine the median nerve, cubital nerve, musculospiral nerve and part of the popliteal nerve, and the motor nerve conduction velocity (MCV), distal motor latency (DML) and the amplitude of compound muscle action potential (CMAP) were recorded. The sensory nerve conduction velocity (SCV) was also examined in the median nerve, cubital nerve and part of the sural nerve. Results The neurogenic abnormalities were detected in extremity muscles, with exception of brachioradial muscle, of upper limbs. In all the 33 patients damage was found on the illness-side and 97.0% on the contralateral side. One patient was found to have neurogenic abnormality of brachioradial muscle and 4 of proximal muscles, including 1 in whom muscle of lower limbs was involved. EMG abnormality rate of distal muscles (89.9%) was significantly higher than that of the proximal muscles of upper limbs (6.7%) and also muscles of lower limbs (1.7%). EMG also showed that there was delayed distal motor latency (DML) and decreased wave amplitude of compound muscle action potential (CMAP) in the nerves of upper limbs. statistically significant difference was found between the CMAP amplitude of affected limb (2.52?1.83mV) and the contralateral upper limb (9.82?3.57mV) by the detection of ulnar nerve conduction, while no difference and no nerve block were found between the DML of affected limb (4.25?1.33ms) and the contralateral upper limb (3.28?0.36ms). Conclusions Electrophysiological changes may be detected in some patients with subclinical Hirayama disease, of which the electrophysiological test is valuable in establishing early diagnosis.

Objective To evaluate the progress and experience in treatment of burn patients in the past 10 years (1993～2002) so as to bring forth ideas for further researches in burn surgery. Methods The general clinical data, complications, survival rate and causes of death in 5378 burn patients treated in our institute were collected and analyzed. The results were compared with those cases treated in our hospital during the years 1983～1992. Results in the past 10 years, there were no significant differences in the general clinical data including age, sex, causes, location, burn area and depth, incidence of combined injury and inhalation injury compared with those in the years 1983～1992. The survival rate of the cases in past 10 years was higher than that of the cases in the years 1983～1992. The main causes of death were organ dysfunction, inhalation injury and infection, among which the incidence of organ dysfunction stood foremost.Conclusions The results suggest that hypoxemia due to severe burn shock and inhalation injury, and endotoxin derived from burn wound still are the main causes of burn death. Protection of the body from early damage of postburn remains to be the most urgent problem in the burn research at present.

Objective:To explore effect of serum leptin level on nutriture in the patient of chronic renal failure(CRF)and the relation with TCM syndrome types of CRF.Methods:110 cases in CRF group were divided into 5 subtypes and 30 normal persons were used as control group.Serum leptin level,body height,body weight,and body mass index(BMI)were determined in both groups;and serum albumin,serum pre-albumin,transferin,serum creatinine,urea nitrogen,C-reaction protein,blood insulin,and nutrition risk index(NRI)were detected in CRF group.Results:Serum leptin level in CRF group was higher than that in the control group,and leptin level and nutritional index level in both the subtype groups of Qi-and Yang-deficiency of spleen and kidney were significantly different with those in other 3 subtype groups.Conclusion:High blood leptin is possibly one of the reasons inducing malnutriture of CRF patient,and serum leptin is possibly a partial basis of substances of Qi-and Yang-deficiency syndrome of CRF.

Objective To investigate the clinical features of the amyotrophic lateral sclerosis(ALS) which initial located in anterior horn of thoraci spinal cord.Methods The clinical data of 3 patients with ALS which initial located in anterior horn of thoraci spinal cord were analysed retrospectively.Results All of the 3 cases were male.The age of onset was 52,66 and 62 years old,respectively.They all presented as dyspnoea,with obvious atrophy in intercostal muscle and rectus abdominis.However,the muscle weakness and atrophy in the limbs were occurred slight degree and late comparativly.EMG showed the neurogenic damage in upper limbs and vertebra muslce.Conclusions The ALS which initial located in anterior horn in thoraci spinal cord is male dominant,the average age of onset is delay than that in ALS.The dyspnoea obvious.The respiratory muscle atrophy is earlier than the muscle weakness and atrophy in the limb.Definite diagnosis depende on the EMG.

Objective To elucidate the clinical, electrophysiological, neuropathological features of two cases of hereditary neuropathy with liability to pressure palsies (HNPP) in one pedigree, and to review the literature of HNPP, so as to promote the understanding and diagnostic acuity of the disease. Methods Detailed electromyogram, motor and sensory conduction velocity, and distal motor latency were measured for clinically affected and unaffected nerves in the two patients. Sural nerve biopsy was performed for case one and the specimen was observed under light microscope and elcctronmicroscope. The cases reported in China up to the present were collected. Results Case one was an 18 year old male with a 9 year history of recurrent weakness and numbness of limbs precipitated by compression or stretch. Case two was his father. Although he had not experienced clinical episode of limb weakness and numbness, physiological examination revealed signs of peripheral neuropathy. Eletrophysiological study demonstrated diffuse peripheral nerve damage with decreased nerve conduction velocity, delayed distal motor latency, especially a decrease in motor conduction velocity at common entrapment sites, including clinical unaffected nerves. Sural nerve biopsy showed that myelin sheath of most myelinated fibers with normal axons was thickened. Some thickened myelin sheath was seen to invaginate into the axon. No onion bulb was found and unmyelinated fibers were relatively normal. Only 9 cases of HNPP were reported in China, but no DNA analysis was performed for any of them. Conclusions HNPP is a rare disease with autosomal dominant inheritance. Nerve conduction study is an important diagnostic method for screening. Its definite diagnosis relies on the typical pathological findings in nerve biopsy specimen. Sural nerve biopsy could be avoided for diagnosis if the family history were positive and nerve conduction study should show diffuse peripheral nerve damage

Objective To evaluate the efficiency of Flixotide in decreasing the incidence of asthma following bronchiolitis.Methods 23 children with brochiolitis received Flixotide daily while other 29 subjects not intervented as control.The intervention duration was 60 days,then followed up for more then 1 year.Results After following up over 1 year,only 3 cases in 29 patients with brochiolitis receiving Flixotide developed into asthma.However,24 cases in control group developed into asthma.The incidence of asthma was 13.04% and 82.76% in two group,respectively.Conclusion Flixotide may be an effective intervention to prevent the development of asthma after bronchiolitis.

OBJECTIVE:To compare the efficacy and safety of Jinkui shenqi pill versus Shengjing capsule in the treatment of oligoasthenozoosperimia under behavioral intervention. METHODS:98 patients with oligoasthenozoosperimia were randomly divid-ed into Shengjing capsule group(49 cases)and Jinkui shenqi pill(49 cases). All patients received intervention treatment(cognitive intervention,psychological intervention,diet intervention and exercise intervention,etc.). Based on it,Shengjing capsule group re-ceived 1.6 g Shengjing capsules,orally,3 times a day;Jinkui shenqi pill group received 6 g Jinkui shenqi pill,orally,twice a day. They were treated for 3 months. Clinical efficacy,semen quality (semen volume,sperm concentration,sperm motility rate, sperm motility)and sex hormone levels [testosterone(T),follicle stimulating hormone(FSH),luteinizing hormone(HLH),pro-gesterone(P),prolactin(PRL)] before and after treatment,and the incidence of adverse reactions in 2 groups were observed. RE-SULTS:The total effective rate in Shengjing capsule group was significantly higher than Jinkui shenqi pill group,with statistical significance(P<0.05). Before treatment,there were no significant differences in semen quality and sex hormone levels(P>0.05). After treatment,semen quality in 2 groups was significantly higher than before,semen volume and sperm motility in Shengjing cap-sule group were higher than Jinkui shenqi pill group,with statistical significances(P<0.05). T levels in 2 groups were significant-ly higher than before,while Shengjing capsule group was lower than Jinkui shenqi pill group,with statistical significances (P<0.05);and there were no significant differences in FSH,HLH,P and PRL before and after treatment in 2 groups(P>0.05). And there was no significant difference in the incidence of adverse reactions(P>0.05). CONCLUSIONS:Under behavioral interven-tion,Shengjing capsule has better efficacy than Jinkui shenqi pill in the treatment of oligoasthenozoosperimia,it can significantly improve semen quality,while Jinkui shenqi pill is better in terms of improving sex hormone levels;and both show good safety.