Objective To introduce a new functional self-assessment scale of amyotrophic lateral sclerosis (ALS). Methods By comparing current different ALS functional scales and combining relative cl inical experience and numeric pain intensity scale, ALS self-assessment scale was set down by International Association of Neural Restoration. Results ALS self-assessment scale included 3 categories with 18 items, adopting 10 points grading system, namely 10 was defined as the normal, 0 as the worst, and the total scores was 180. This scale included: ① Bulbus medullae function: speech, swallowing, sal ivation, and tongue extension. ② Limbs function: left arm movement, left hand movement, right arm movement, right hand movement, left leg movement, right leg movement, trunk movement, head-up, walking, and cl imbing stairs. ③ Others: breathing, muscular tone, pain, and muscle discomfort. Conclusion ALS self-assessment scale is specifically designed for ASL patients. It can evaluate patient’s function comprehensively and is simple and convenient, consuming less time.
Objective To evaluate proton MR spectroscopy (1H-MRS) for detection of the motor cortex and adjacent brain in amyotrophic lateralsclerosis (ALS) patients with apparent upper motor neuron involvement after olfactory ensheathing cells(OECs) transplantation. Methods From December 2004 to February 2005, 7 patients with clinically definite ALS who could safely undergo MRS were admitted into the perspective study. The neurological status, ALS functional rating scale (ALSFRS), EMG, and 1H-MRS taken before and 2 weeks after operationswere carefully analyzed. The NAA/Cr and Cho/Cr ratios were measured in the cerebral peduncle,genu and posterior limb of the internal capsule, corona radiata and precentral gyrus. Results The ALSFRS in 2 cases mproved obviously whose ALSFRS increased from 30 to 33 and from 29 to 34 respectively. And 5 cases remained stable 2 weeks after OECs transplantation. Statistical analyses for all seven cases showed both theNAA/Cr and Cho/Cr ratios decreased, but in the two cases with ALSFRS improvement the NAA/Cr increased in the certain anatomic position which confirmed the neurological and EMG findings. Conclusion The proton MR spectroscopy is a suitablenoninvasive measure for ALS evaluation. The preliminary study suggests that twoof the seven ALS cases improved apparently shortterm after OECs transplantation. More patients are required for the clinical study and longer followup duration is needed for future research.
Objective To determine whether transplanting olfactory ensheathing cells (OECs) is effective in controlling or reversing the deterioration caused by amyotrophic lateral sclerosis (ALS). Methods Between February 2003 and April 2006, 327 patients (241 males and 86 females) with probable or definite ALS (diagnosed according to the El Escorial criteria) received the OECs transplantation. Their ages ranged from 20 to 84 years (51.6±11.1 years). The duration of symptoms before surgical treatment was 4-8 months to 13 years (2.9±2.0 years). OECs were cultured and injected into pathological regions of the spinal cord and/or bilateral corona radiata of the brain; the patients were divided into three groups, group A (cord only,n=29), group B (cord and brain,n=6), and group C (brain only,n=292) based on the transplant sites. Results The patient’s neurological function was assessed both before and at 4 weeks after transplantation by using the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) of the ALS CNTF Treatment Study (ACTS). The scores were increased from 17.2±8.6 preoperation to 20.1±9.7 postoperation in group A (P＜0.05),from 24.2±6.8to 25.7±6.6 (P＞0.05) in group B, and from 20.3±8.6 to 22.0±9.4 (P＜0.001) in group C.There were no significant difference in increased ALSFRS scores among the threegroups (P＞0.05). The total improvement rate of neurological function was 77.1% (252/327). The result of electromyographic examination showed that spontaneouspotential diminished and/or disappeared, the amplitude of the motor unit actionpotential decreased remarkably and the numbers of motor unit action potential greatly increased in 261 cases (79.8%). Sixteen patients (4.9%) experienced thevarious complications including headache, shortterm fever, seizure attack, central nerve system infection, pneumonia, respiratory failure, urinary tract infection, heart failure, and possible pulmonary embolism; of them, there were 4 deaths(1.2%). Conclusion These preliminary results suggest that the OECs transplantation is effective in controlling or reversing the physiological deterioration caused by ALS.