Pathogenesis Reseach And Clinical Study Of Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis is a progressive neurodegenerative disease,which is clinically characterized by muscle weakness, wasting, spacisity andpathologically characterized by selective loss of upper alld lower motorneurons. It is present worldwide, 95% of all cases are sporadic, and the remains5% are an auosomal dominant inheritance. The cause of the disease is stillunknown. There are some major hypotheses of the pathogenesis: Exicitotoxity,oxidative, auoimmunity, deficiency of nerve growth factor and Viral infection.To provide clues to the cause of the disease, improve accuracy of diagnosis andprovide more clinical data for the further study of the pathogenesis and thetherapy, we retrospectively reviewed the records of 105 cases of ALS in ourhospital during recent twenty years. At the same time, we measure theroexcitatory amino cids, glutamate and asparate, in cerebrospinal fluid ofamyotrophic lateral sclerosis and control by reverse-phase, high pefformanceliquid chromatography (HPLC).Objective To study the clinical features of amyotrophic lateral sclerosis(ALS), and investigate the change of the concentration of excitatory aminoacids in cerebrospina fluid of amyotrophic latera1 sclerosis, provide clinicaland experimental data for the further research in pathogenesis and theraPy.Methods 1) We forospectively reviewed the recOrds of l05 cases of ALSin our hospita dUring recent twenty years. Clinical charctedstics \symPtOm' sign and data of laboratory examination on the patiellts weestatistical anayzed. 2) In this Study, we measure the neurexcitatory aminoacids, gfutaxnate and aspanate, in cerebrospinal fluld of aInyotrophic latffelsclerosis and control by reverserphase, high peffonnance liquidchromatograPhy (HPLC), and ana1ysis the correlation betwen amino acidsconcelltration and dUration of disease, initial symPtom, sex, or patiellt age. TheALS diagnosis mainly based on the clinical situation and electrOmyography.We excluded ALSonAnicking disorders by waist PUntw, image analysis andother investigation.Results l) The mean age at onset was 42.7 years, earlier than in the WestCountry. There is no difference betWen male and female. The clhaax of theonset age is 40~60 years old. 2) Sex ratio (male/female) was 2:l. 3) UpperextrCmities is the most common location of onset. Patiellt nd ideal bulbaxsymPtoms tended to be predominan in female and older than those with initialsmptom in uPper or lower eXtremities. 4) ALS develops frOm the initia1location to the near motor neuron vertically or horizontally. 5) Theconcendations of the glutamate and aSPedate, in cerebrospinal fluld ofamyotrophic lateral sclerosis were significanily elevated. 6) There is norelationshiP betWeen drino acids concentration and dUration of disease, initialWtOm, sex, or patiefit age in ALS patienis.Conclusions ALS is an age-related \ male predominan disease. UPPerextremities is the most common location of onset. Patient ed initial bulbarsyInPtoms tended to be predominan in female and older than those with iallialsMtom in uPPer or lower extremities. The deve1opment of the ALS fOllowssome pattems. The concefitrations of the glLltamate and aspedate, incerebrosPinal fluid of amyotrophic lateral sclerosis wee significantiy elevatedthan the control. These results suggest that excitOtoxicity is relevan to thepathOgenesis ofALS.