Objective: To investigate the value of the trigeminocervical response (TCR) for revealing bulbar involvement in patients with spinal and bulbar muscular atrophy (SBMA). Methods: Thirty patients with SBMA and 30 healthy male controls were included in this study. In all of the normal controls, stimulation of the infraorbital nerve on one side produced bilateral short latency waves consisting of a positive/negative wave, p19/n31, the mean latency of which was measured. The mean square root of the ratio between the amplitude of p19/n31 and the mean rectifi ed surface electromyography (EMG) activity preceding the stimulus, the A value, was estimated. The parameters of the TCR were compared between the two groups. Results: Among the patients with SBMA, 21 (70.0%) had delayed latencies of p19/n31 (P < 0.01) and all (100%) had reduced A values (P < 0.01) relative to the normal controls. Conclusions: All parameters of the TCR were signifi cantly different between the patients with SBMA and the normal controls. T

Objective To investigate the dynamic changes of the spinal cord during neck flexion in Hirayama disease for diagnosis.Methods MRI examinations in neutral neck position and a fully flexed neck position were performed on 18 cases of Hirayama disease and 31 young normal control subjects.We measured an antero-posterior diameter(APD)and transverse diameter(TD)of the cervical cord at the superior margin of the C6 vertebral body for each position,and investigate the dynamic changes.The different in frequency of these findings between the control and patient groups was examined by means of the x~2 test.The group means were compared by independent-sample t-test.Significance was defined as P

Objective To evoke cerebral potentials by stimulating nociceptive fibers with contact heat evoked potentials stimulator (CHEPS)and estimate the nerve conduction velocities of peripheral nerve fibers mediating these responses.Methods Subjects were set in supine position.A heat-foil technology with a rapid rising speed at 70 ℃/s was used to elicit pain and contact heat evoked potentials(CHEP).Contact heat was delivered via one circular thermode (diameter 27 mm,area 573 mm~2).Thermal stimuli were sent at two intensity levels (49.5 ℃ and 54.5 ℃) to three body sites:thenar eminence,the dorsum of hand and proximal volar forarm.Contact heat evoked potentials were recorded from Cz and Pz.A systemic effect between stimulus intensities and pain rating were observed,the main components of this evoked potential were observed.Nerve conduction velocity was calculated from latency difference of CHEP and center to center distance of distal and proximal stimulus arrays.Results The pain intensity rating was 3.2?0.3 and 4.4?0.5 when thenar eminence was stimulated at the temperature of 49.5 ℃ and 54.5 ℃ respectively;the rating was 6.3?0.8 and 7.2?0.5 when the dorsum of hand and proximal volar forarm were stimulated at the temperature of 54.5 ℃ respectively.Three components,Cz/N550,Cz/P750 and Pz/P1000,were found in the evoked potentials.Nerve conduction velocities of the fibers were (12.9?7.5) and (1.7?0.4) m/s respectively,which were corresponding to those of A8 fiber and C fiber.Conclusions CHEPs can be elicited reliably and stably.Velocities of peripheral nerve fibers demonstrate that A8 fiber and C fiber mediate the response.

Objective To establish the method of contact heat evoked potential(CHEP)and to explore the value of this evoked potential in pain testing of patients with cerebral infarction.Methods A total of 100 healthy volunteers and 30 patients were examined.The healthy volunteers were divided into 3 groups according to the length of their arms:(Group A:56.0～65.0 cm ;Group B :65.5～74.0 cm ;Group C :74.5～83.0 cm).A recently de- veloped heat-foil technique with a rapid temperature rising rate at 70℃/s was used to elicit pain and contact heat e- voked potentials.Contact heat was delivered via one circular thermode(diameter 27 mm,area 573 mm~2)and set at two intensity levels(49.5℃and 54.5℃)to three body sites:the thenar eminence,the dorsum of hand and proximal volar forearm.The subjects were asked to rate the pain with numerical rating scale after each stimulus and CHEP was recorded from Cz and Pz.The association between stimulus intensities and pain rating was explored,the main compo- nents of the evuked potential were watched.CHEP,sensory conduction velocity(SCV)and somatosensory evoked potentials(SEP)were performed in patients with hemi-anesthesia caused by cerebral infarction.Results The pain intensity ratings were 3.2?0.3 and 4.4?0.5 at thenar eminence,5.0?0.7 and 6.3?0.8 at the dorsum of hand and 5.3?0.6 and 7.2?0.5 at the proximal volar forearm when the temperature of 49.5℃and 54.5℃was applied, respectively;Three components,Cz/N550,Cz/P750 and Pz/P1000,were identified in the evoked potentials.Cz/ N550 and Cz/P750 appeared when the dorsum of hand and proximal volar forearm were stimulated.In contrast,Pz/ P1000 could be identified when nociceptors of thenar eminence and proximal volar fbrearm were excited.In the pa- tients with cerebral infarction,CHEP disappeared or became abnormal on one side,while SCV and SEP were normal on that side.Conclusion It was suggested that CHEP could be elicited reliably in the controls.CHEP is helpful in the assessment of analgesia in patients with cerebral infarction.

ObjectiveTo explore the characteristics of the metabolite concentrations of the precentral gyrus in healthy Chinese adults using proton magnetic resonance spectroscopy (1H-MRS).Methods 1H-MRS was carried out in 92 healthy Chinese adult volunteers. The metabolite ratios of NAA/Cr, Cho/Cr and NAA/Cho were compared and analysed.ResultsNo significant changes of NAA/Cr, Cho/Cr and NAA/Cho ratios were observed between the left and right precentral gyrus, nor between the male and female. However, the precentral gyrus NAA/Cr and NAA/Cho ratios were decreased gradually with aging and Cho/Cr ratios were increased gradually with aging.ConclusionThe characteristics of the precentral gyrus metabolite concentrations in healthy Chinese adults have been obtained.

ObjectiveTo explore the factors that delayed the diagnostic process and resulted in misdiagnosis of amyotrophic lateral sclerosis (ALS),in order to look for solution. MethodsThe records of 99 cases with ALS from 1999 to 2003 in our hospital were reviewed retrospectively. Clinical characteristics and diagnostic process on the patients were statistically analyzed.ResultsThe time needed to confirm diagnosis was (13.1±7.5) months. There was a positive correlation between the time when EMG was performed and the time the diagnosis was made. 58.6% of patients were initially misdiagnosed in other hospitals. The most common misdiagnosis was cervical spondylosis. The misdiagnosis more likely occured in the patients of 40-59 years old. The misdiagnosis rate in the patients with initial lower extremities symptoms was higher than that with initial bulbar and upper extremities symptoms. The misdiagnosis more likely occured in patients with early cervical MRI.ConclusionThe major causes of misdiagnosis are unfamiliarity of the physician with the disease,misleading findings or misinterpretation of neuro-radiological or neuro-physiological findings.

Objective To diagnose Kennedy's disease (KD) via molecular analysis of the androgen receptor gene with suspected KD.Methods Two patients with suspected KD were reported.We analyzed their clinical features and investigated the number of CAG repeats in the androgen receptor genes. Results Both of the patients were characterized by slow progression of predominant proximal and bulbar muscle weakness.Patient 2 had oligospermatism.Serum creatine kinase and triglyceride levels were found markedly increased.The exact number of CAG was 52 in patient 1 and 48 in patient 2,respectively.These 2 patients were finally diagnosed as Kennedy's disease through the analysis of androgen receptor gene by PCR and direct sequencing.Conclusions The method of molecular analysis for KD had been copied in China.The clinical and molecular biological features of 2 Chinese patients with KD had been discussed.KD is a neurodegenerative disorder by proximal limb muscular atrophy and weakness with lower motor neuron signs,bulbar involvement.Dyscrinism and metabolic abnormalities may also be observed.Gene analysis is the unique and reliable methods to diagnose KD.

Objective To investigate the expression of free fatty acid receptor GPR40 and evaluate the possible function of GPR40 in the adult monkey hippocampus after ischemia. Methods According to the post-ischemic adult monkey model of Yamashima, a total of 24 adult monkeys were randomly divided into 4 groups: sham-operated group (control) and post-ischemic day 4, 9, 15 (d4, d9,d15). The expression of free fatty acid receptor GPR40 was detected at the protein level by immunoblotting and immunohistochemistry in the adult monkey hippocampus. Results Immunoblotting analysis showed the expression of GPR40 was decreased in CA1 and increased in DG after ischemia (posfischemic group vs control, P＞0.05). Immunohistochemistry data revealed that the double stained cells of GPR40 and NeuN were also decreased by 50% (d15) in CA1 and had no significant changes in DG after ischemia. Interestingly, the co-labeled cells of GPR40 and GFAP were increased 2.5 folds (d4) in post-ischemic SGZ. Conclusions There is the different expression of GPR40 in adult monkey hippocampal CA1 and DG regions after ischemia. Co-labeled cells of GPR40 and GFAP are increased in post-ischemic SGZ, which indicates that polyunsaturated free fatty acid such as DHA, a ligand of GPR40, may alleviate neuronal injury in post-ischemic hippocampus.

Objective To search for a method to differentiate the peripherial and central part of spinothalamic tract. Methods The subjects were set on supine position. A heat-foil technology was used to elicit pain and contact heat evoked potentials (CHEP). Thermal stimuli were sent at 54.5 ℃ to three body sites: the dorsum of hand, proximal volar foratm and C7. CHEP was recorded from Cz and Pz. The main components of CHEP were watched. Nerve conduction velocity was calculated. Results Two components, Cz/N550 and Cz/P750, were found in the evoked potentials. The latency of CHEP were different when the dorsum of hand, proximal volar forarm and C, were stimulated respectively. There was no statistical difference between males and females. Nerve conduction velocity of the fiber was (12.9±7.5) m/s which was corresponded to that of AS fiber. Conclusion It is suggested that pain evoked potential could be elicited reliably,the stimulation of C7 would help differentiate the lesion of peripherial and central part of spinothalamic tract.

OBJECTIVETo investigate the effect of N-acetyl-cysteine (NAC) and depakine (DP) on the changes of membrane potential and peroxidate in rat cortex neurons exposed to ferrous chloride (FeCl(2)).

METHODSCultured cortex neurons of newly born SD rats were randomly divided into control group (PBS group), model group (FeCl(2) group), NAC pretreatment group (NAC group), DP pretreatment group (DP group) and NAC+DP pretreatment group (NAC+DP group). In the latter three groups, NAC (0.08 mg/ml) and DP (0.1 mg/ml) were added in the cell culture 2 and 3 h before FeCl(2) (1 mmol/L) exposure, respectively. After exposure to FeCl(2), the membrane potential of the neurons was detected with fluorescent dye DiBAC4(3) (bis-(1,3-dibutylbarbituric acid) trimethine oxonol), and the peroxidate level with 2,7-dichlorofluorescin diacetate (H(2)DCF) by laser confocal scanning microscope (LCSM) and nuclear factor-KappaB (NF-KappaB) level with immunocytochemistry.

RESULTSCompared with FeCl(2) group, the expression of NF-KappaB and peroxidate level in the neurons were decreased significantly in NAC and NAC+DP groups (P<0.01), but not in DP group (P>0.05). FeCl(2) depolarized the membrane potential and increased the expression of NF-KappaB in the neurons. Compared with FeCl(2) group, significant changes in the membrane potential were observed in DP and NAC+DP groups (P<0.01) but not in NAC or PBS group (P>0.05).

CONCLUSIONBoth NAC and DP can protect the neurons from FeCl(2)-induced damage but through different pathways, and their combined use can significantly alleviate neuronal damages due to FeCl(2) exposure. Antioxidants such as NAC in combination with antiepileptic drugs may produce favorable effect in prevention and treatment of posttraumatic epilepsy.

Acetylcysteine ; pharmacology ; Animals ; Animals, Newborn ; Cells, Cultured ; Cerebral Cortex ; cytology ; metabolism ; physiopathology ; Female ; Ferrous Compounds ; pharmacology ; Male ; Membrane Potentials ; drug effects ; Neurons ; cytology ; metabolism ; physiology ; Neuroprotective Agents ; pharmacology ; Peroxides ; metabolism ; Rats ; Rats, Sprague-Dawley ; Valproic Acid ; pharmacology