Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rabbits immunized with particulate and soluble preparations of rat lymphoid tissue of the HO strain produced antisera which reacted without strain specificity on rat lymphocytes. Absorption of the sera with tissue from the AS strain of rat removed the antibodies reacting with AS tissue leaving activity against HO cells only. Studies with backcross rats showed that the antigens detected by these sera were products of the AgB genes or genes segragating with them. The immunosuppressive activity of rabbit antisera specific for Ag-B5 rat transplantation antigens was tested in a rat renal allograft assay. Some of the antisera markedly prolonged the survival of (AS X HO)F1 kidneys transplanted to AS rats. The prolongation of graft survival was not due to ALS activity since the sera were active in the absence of antibody directed against recipient antigens. There was no correlation between in vivo enhancement and anti-donor lymphocytotoxic titres of the xenoantisera.
Transplantation 1976 Sep
PMID:Immunological enhancement of rat renal allografts using rabbit antisera with specificity for rat transplantation antigens. 6 35

Hemopexin, a serum glycoprotein that binds free heme and transports it to hepatic parenchymal cells, has been measured by radial immunodiffusion. We have confirmed elevation of serum hemopexin concentration in Duchenne's muscular dystrophy patients and carries, and demonstrated elevations in dermatomyositis/polymyositis and myasthenia gravis, but not in amyotrophic lateral sclerosis. In monkeys, elevations of hemopexin levels were specifically induced by hematin injections, muscle-crush, or myoglobin injections. Myoglobin leakage is the likely explanation of hemopexin level elevation in Duchenne's dystrophy patients and carriers and in dermatomyositis/polymyositis. In myasthenia gravis there might be a slight myoglobin leakage not heretofore suspected; or, the elevation of hemopexin levels might be a new reflection of a dysimmune state in myasthenia gravis, and perhaps as such is a further incrementing factor in dermatomyositis/polymyositis. Hemopexin, presumably as a longer-phase reactant, is sometimes an index of neuromuscular disease when other data are negative or equivocal.
Arch Neurol 1978 Sep
PMID:Elevations of hemopexin levels in neuromuscular disease. 9 25

To test the hypothesis that host resistance factors may be abnormal in Guamanians in whom amyotrophic lateral sclerosis and Parkinsonism-dementia develop, cellular immunity was evaluated in both diseases and compared to that of Guamanians with other nervous-system diseases, normal adult Guamanians and non-Guamanians with amyotrophic lateral sclerosis and Parkinsonism. Diminished responses to skin-test antigens, lymphopenia, diminished per cent and total T cells and, less frequently, decreased mitogen responses were seen in Guamanian patients with amytorophic lateral sclerosis and Parkinsonism-dementia but not in the other patient or normal groups. Guamanian patients with amyotrophic lateral sclerosis and diminished cellular immunity had an increased frequency of HLA-Bw35 (P less than 0.005) and shorter mean duration of disease (P less than 0.05) than those with normal cellular immunity. In Parkinsonism dementia diminished cellular immunity was less strongly associated with HLA-BW35 (P less than 0.05) and was not associated with differences in duration of disease. Normal Guamanians and those with other nervous-system diseases showed no association of diminished cellular immunity with HLA-Bw35. The association appeared disease-related, with onset concomitant with the neurologic expression of Guamanian amyotrophic lateral sclerosis and Parkinsonism-dementia.
N Engl J Med 1978 Sep 28
PMID:Cellular immunity in Guamanians with amyotrophic lateral sclerosis and Parkinsonism-dementia. 30 83

Some authors have suggested that amyotrophic lateral sclerosis (ALS) may be precipitated by a "deficiency factor" following gastrectomy. Methods to establish a statistical association of the disease with gastrectomy include a case-control study. Two such studies have been made in Japan, one involving 712 and the other 158 cases of the diseases. No statistically significant association between gastrectomy and the subsequent occurrence of ALS was found, indicating it is unlikely that the operation predisposes to ALS. None of many other factors studied except mechanical injury showed a statistically significant association in either study.
Arch Neurol 1979 Sep
PMID:Does gastrectomy predispose to amyotrophic lateral sclerosis? 47 26

Creatine phosphokinase (CPK) is usually elevated in inflammatory and degenerative muscle disease but is usually reported as normal in neuropathic diseases. A review of the literature indicates, however, that it is elevated in 50 to 75% of patients who have motor neuron diseases, and that these elevations are usually 5 to 6 times normal. The effect of bedrest and moderate exercise on serial CPK values in a patient with motor neuron disease is assessed. Bedrest for 24 hours decreased his CPK to 0.58 of the baseline value; 5 hours following moderate exercise it increased to 1.71 of the basal value. This phenomenon appears to be a response to exercise stimuli rather than to the disease process itself. CPK values up O 1000 IU/L are compatible with the diagnosis of amyotrophic lateral sclerosis (ALS). In patients whose CPK values seem atypical, it is best to have them rest 48 hours before repeating the test.
Arch Phys Med Rehabil 1979 Sep
PMID:Exercise effect on creatine phosphokinase elevation in motor neuron disease. 49 5

Amyotrophic lateral sclerosis (ALS) is a degenerative neurologic disease having both upper and lower motor neuron signs and symptoms. When the speech musculature is involved, a mixed dysarthria and dysphagia usually result. In a 49-year-old man with ALS, dysarthria and dysphagia progressed from mild to severe forms over 17 months. Eleven months after the patient first experienced symptoms, neurologic examination showed fasciculations of the extremities and tongue, limb weakness, and hyperreflexia of the limbs and velopharyngeal mechanism. Tongue strength was one-fourth that of normal. Lingual alternate motions rates for consonant-vowel syllables were also reduced. To enhance lingual strength and swallowing, a tongue-strengthening program was developed for use with articulation training; to augment velopharyngeal function, a palatal lift was fitted; and to increase extremity strength, physical therapy was initiated. Six months after the initial neurologic examination, medical and speech reevaluation showed progressive weakness of the body parts affected initially; continued decline in tongue strength and lingual alternate motion rate; hypoactive reflex activity, indicative of progressive involvement of the lower motor neuron system; and continued deterioration of articulation and phonation owing to the progressive nature of the disease.
Arch Phys Med Rehabil 1979 Sep
PMID:Progressive speech deterioration and dysphagia in amyotrophic lateral sclerosis: case report. 49 10

This paper presents two juvenile cases of familial amyotrophic lateral sclerosis. They are the first and fourth child in a family with seven children from the eastern part of Finland. All seven children, as well as the parents, were examined by our group. In the first case the disease showed a rather mild course, while in the second a noticeable progression was observed even during a period of 10 months. The patients come from a rural area with a stable population and low immigration, which may favor an enrichment of certain genes and therefore support the possible hereditary basis for the disease.
Acta Neurol Scand 1979 Sep
PMID:Juvenile amyotrophic lateral sclerosis. A report of two cases in a single family. 51 16

Thirty-two patients with motor neurone disease were investigated using quantitative electrophysiological techniques. Estimates of the number of surviving motor units in the extensor digitorum brevis muscle and measurements of the electrophysiological parameters of these units are present along with the values for motor nerve conduction velocities. The results indicate that reinnervation in motor neurone disease is sufficient to compensate completely for the loss of up to 50% of the motor neurone pool supplying the muscle. The capacity for reinnervation is greater than we have found in a number of neuropathies but the efficiency of reinnervation decreases as the number of surviving motor units falls. Reinnervation appears to cease when 5% or less of the motor units remain viable. There is no electrophysiological evidence of a preferential loss of fast conducting axons, of pathological slowing of conduction nor of a dying-back process affecting the motor axon. Comparison of the electrophysiological parameters in progressive muscular atrophy and amyotrophic lateral sclerosis shows no significant differences. The underlying pathophysiological mechanisms are discussed in terms of the results.
J Neurol Neurosurg Psychiatry 1978 Sep
PMID:A quantitative electrophysiological study of motor neurone disease. 69 Jun 47

Pecularities attending the fixation of antibodies from the sera of patients with arterial hypertension, amyotrophic lateral sclerosis, multiple sclerosis, hepato-cerebral disease, and myoclonus-epilepsy were studied by the indirect Coons' method. Antibrain antibodies, complimentary to various nervous tissue structures always formed in the patients suffering from the mentioned diseases. The antigenic properties of individual components of the brain tissue of the patients failed to change as much as to differ completely from the antigenic properties of the same formations in normal animals. At the same time various components of different neurons and glial cells, myelin membranes of various conductive systems, and, to a lesser degree, cells of the ependyma and vascular walls located in various portions of the brain, posessed not only common, but also different antigenic properties.
Biull Eksp Biol Med 1976 Sep
PMID:[Fixation of human serum anticerebral antibodies in different regions of the rabbit brain]. 82 60

The prevalence of ALS and its distribution in Finland was investigated on the basis of prevalence numbers by counties. On the prevalence day, 1 January 1973, 168 ALS patients were found alive. They were collected from hospital records and the registers of the National Pension Institute. The prevalence was 3.56 cases per 100,000 populations, 4.25/100,000 for men and 1.92/100,000 for women, which gave a male to female prevalence ration 1.5 to 1. The distribution of the disease was uneven in the country being more frequent in the southeastern counties. The disease was contracted earlier (at 51.9 years of age) and its course was slower (3.7 years up to the prevalence day) in the prevalence material than in cases derived from the mortality statistics (58.0 years at the onset and duration of 2.6 years, respectively). This is explained by the dropping out of some of the younger and/or more benign cases from the mortality statistics. This seems to be true also in other countries.
Acta Neurol Scand 1977 Sep
PMID:Amyotrophic lateral sclerosis in Finland. I: An epidemiologic study. 90 93


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