[Follow-up study of patients with chronic motor axonal neuropathy].

Objective: To study the characteristic of chronic motor axonal neuropathy and the prognosis of it.

Methods: 16 chronic motor axonal neuropathy (CMAN) patients were diagnosed by clinical manifestation and neurophysiologic manifestation. Follow-up study was done including living state, medicine they taken, change of physical sign and motor unit number estimation.

[Basic and clinical researches on amyotrophic lateral sclerosis/motor neuron disease].

This study includes three aspects: (1) we have reported some novel or rare mutations of SOD1 (Cu/Zn superoxide dismutase) gene in Chinese families of ALS/MND, and found quite different features from Western patients in polymorphisms with some candidate genes such as vascular endothelial growth factor (VEGF) in sporadic ALS/MND in China. Meanwhile, we have so for established a complete clinical database with more than 1 200 cases; (2) we have established some neurophysiologic techniques of diagnosis and differential diagnosis at early-stage for ALS/MND, which include trigemino-cervical response, sternocleidomastoid and rectus electromyography, contact heat evoked potentials, and motor unit number estimate; (3) we have attempted some experimental and clinical treatments for ALS/MND, which include gene and stem cell therapies in animal models, and a pilot clinical trial of granulocyte colony stimulating factor (G-CSF) for ALS/MND patients (NCT00397423).

Fully intact contact heat evoked potentials in patients with amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is typically considered to be a disease of motor, not sensory, neurons. However, reports exist of sensory system involvement in ALS. In this study we aimed to study the characteristic of contact heat-evoked potentials (CHEPs) in patients with ALS and to evaluate the nociceptive pathway in these patients.

Stratifying disease stages with different progression rates determined by electrophysiological tests in patients with amyotrophic lateral sclerosis.

By determining the usefulness of motor unit number estimate (MUNE) and compound muscle action potential (CMAP) amplitude in patients with amyotrophic lateral sclerosis (ALS), we tried to find an effective way to stratify the disease stages. In all, 112 consecutive ALS patients were enrolled, among whom 73 were elicited in a longitudinal study. MUNE by the standard incremental technique, the average CMAP amplitude, total Medical Research Council (MRC) score, ALS-functional rating score (ALS-FRS), Appel ALS rating scale (AARS), and forced vital capacity (FVC) were performed at baseline and months 3, 6, and 12 after study entry.

[Test of sensory nerve in patients with Kennedy disease].

Objective: To study the electrophysiological and pathological features of Kennedy disease (KD) and to make sure the functional and anatomical change of the sensory nerve of KD patients.

Methods: Electrophysiological and pathological tests were performed in 14 KD patients, including electromyogram, conduction velocities of median, ulnar, peroneal, tibial, and sural nerves, trigemino-cervical reflex (TCR), contact heat evoked potential (CHEP), and biopsy of the sural nerves. During TCR, the patients lay on the back, holding the heads slightly raised to make the sternocleidomastoid muscles contract slightly.

[Establishment of a method of contact heat evoked potential and its application in carpal tunnel syndrome].

Objective: To establish a method to evoke cerebral potentials by stimulating nociceptive fibers, estimate the conduction velocities of nerve fibers mediating these responses, and assess the value of the potentials in carpal tunnel syndrome.

Methods: One hundred patients with carpal tunnel syndrome and 20 sex-, and aged-matched healthy persons underwent stimulation of contact heat delivered via a circular thermode to excite selectively nociceptors with a rapid rising time at 70 degrees C/s to elicit pain and contact heat evoked potential (CHEP). Thermal stimuli were sent at two intensity levels (49.

[Clinical features and dynamics of cervical magnetic resonance imaging in Hirayama disease].

Objective: to investigate the clinical feature and dynamic changes of the cervical dural sac and spinal cord during neck flexion in Hirayama disease (juvenile muscular atrophy of distal upper extremity).

Methods: Clinical data were taken and MRI in neutral neck position and a fully flexed neck position were performed on 27 cases of Hirayama disease.

Results: (1) All patients were consistent with the diagnostic criteria of Hirayama disease who had asymmetric muscular atrophy and weakness of the hand and forearm.