Study On Diagnosis And Treatment

Our study assessed potential clinical usefulness of proton magnetic resonance spectroscopy(1H-MRS) in amyotrophic lateral sclerosis (ALS) patients, and analyzed levels of plasma and cerebrospinal fluid (CSF) glutamate in ALS patients.In the first section ,clinical features of ALS patients were characterized as follows: (1) Thirty seven cases of definite and probable ALS patients (according to El Escorial criteria) were enrolled. Mean age at first visit was 50.1±9.5 years. With respect to sex predominance, male-to-female ratio was 2.7:1 for the population overal, in contrast to 1:1 in the bulbar-onset ALS subgroup.(2) As to anatomic region of initial involvement, onset symptomatology presented as bulbar in 22%, cervical in 46%, and lumbosacral in 32% of our patient population. (3) Needle electrode examinations demonstrated different frequencies abnormality in bulbar,cervical,thoracic and lumbosacral musculature, the highest frequencies of abnormality generally being found in those muscles innervated by initially affected regions .(4) Serum IgM Anti-GM1 antibody levels were found to be abnormal in 29.2% of our ALS patients .1H-MRS is a new neuroimaging technique which can non-invasively quantify signal changes of certain metabolites in the brain. Our study focused upon the precentral gyrus to determine whether any signal changes could be detected in ALS patients; and to determine whether riluzole can reverse corticomotoneuron damage in patients with ALS. We divided the population into two groups, and treated patients in the first group with riluzole (riluzole 50mg bid), while patients in the second group received immune therapy (cyclophosphamidum combined with dexamethasone). We found that: (1) ALS patients have significantly reduced N-acetylaspartate(NAA) resonance intensity relative to Creatine(Cr)(P<0.01). (2) After one month of therapy, this parameter (NAA/Cr) significantly increased (P<0.05) in the riluzole-treated patients, but remained unchanged in the immune therapy group. (3) The improvement of NAA/Cr was consistent with improvement of bulbar score (Appel Scale) in the riluzole group. The foregoing NAA/Cr appears to be more sensitive than ALS scale with respect to detection of drug effects. We concluded that NAA/Cr could be used as a surrogate corticomotoneuron marker. Furthermore, it could also be used to monitor disease progression or to assess responses to drug therapy.Excitotoxcity of glutamate is one of the major facets in ALS pathogenesis. In the last section of our study, levels of plasma and CSF glutamate in ALS patients were measured via high performance liquid chromatography (HPLC) . It was demonstrated that (1) Plasma glutamate levels are higher in ALS patients than in normal controls (P<0.05). We found no correlation between plasma glutamate levels and age, sex or duration of disease. (2) After the initial month of riluzole therapy, plasma glutamate levels significantly decreased (P<0.05), while no significant change was found in patients treated with cyclophosphamidum. (3) No difference in levels of CSF glutamate was shown between ALS patients and normal controls. The evidence suggests that glutamate plays a significant role in ALS, although it might not be the only significant component in the pathogenetic mechanism of ALS.