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)

The clinical characteristics of ALS in Finland were investigated. The study was based on the hospital records of 255 patients. No correlation was found between the disease and other previous illnesses. Muscular weakness was the most frequent complaint of the patients as their first symptom (149/255, i.e. 58 per cent). The duration of the disease was shortest (1.9 years) in male cases with the first symptoms in the bulbar area, and it was longest (3.4 years) in females with a spinal onset of the symptoms. The duration of the disease was in inverse relation to the age when it was contracted. Progressive muscular atrophy with fasciculations was considered the most important clinical sign of ALS. It was most frequently observed in the upper extremities including shoulders. Sensory disturbances were absent, sphincter abnormalities were reported in only three cases, bed sores did not exist, and there were no reports of impotence in the male patients. Two families with two familial cases in each were found, altogether two men and two women. Their disease did not differ from that of the rest of the series. The ALS patients soon became too disabled to work; 56 per cent bacame permanently disabled in less than 1 year's time, and only 9 per cent retained their working capacity for more than 2 years. The average length of stay at hospital was 32 days for ALS and 37 days for MS patients, but the later group was hospitalized 2-3 times more frequently than the former. Gastrostomy or tracheostomy and artificial respiration did not lenghten the lives of the patients.
Acta Neurol Scand 1977 Sep
PMID:Amyotrophic lateral sclerosis in Finland. II: Clinical characteristics. 90 94

The mortality and prevalence of ALS in the various countries of Finland was studied. The work was based on death certificates derived from a 10-year period from 1963 to 1972, and altogether 421 cases were found. The mean duration of the disease was 2.7 years, and the mean age at death was 61.2 years. The average annual mortality rate was 0.91 per 100,000. The male to female ratio was 0.87 to 1, males outnumbered females only in age groups under 65. Some clustering of the cases seems to be taking place in the south-eastern part of the country. The rural to urban distribution of the patients' places of birth and domiciles did not differ markedly from that of thw whole population. After ALS itself pneumonia was the most common direct cause of death. Of other significant conditions coded in the death certificates schizophrenia and cancer did not occur more often than could be expected by chance. No evidence of inheritance of the disease was found.
J Neurol Sci 1976 Sep
PMID:The epidemiology of amyotrophic lateral sclerosis in Finland. A study based on the death certificates of 421 patients. 95 May 75

Amyotrophic lateral sclerosis is usually considered a disease that will have a fatal termination in 1 to 3 years. A prospective study of 100 patients with this disorder revealed that 20 of them were living 5 years after the onset of their disorder. Review of other published series reveals that patients have been reported who lived for longer than 5 years and have then usually been reported as atypical cases, although the only way in which they are described as atypical is the duration of the disease. It is our assumption, based on these data as well as on additional clinical observations, that many patients with amyotrophic lateral sclerosis live for longer than 5 years and, rarely, they may have remissions of their illness. The possible significance of these observations is discussed.
Mayo Clin Proc 1976 Sep
PMID:Patient resistance and prognosis in amyotrophic lateral sclerosis. 95 88

The F-wave velocity in the central segment (axilla to spinal cord) was studied employing the "collison technique" described by Kimura (1974), and compared with the conduction velocity obtained with the usual methods. In 25 normal subjects the F-wave velocity increased proceeding proximally, reaching the maximum values in the central tract (64.86 +/- 2.23 m/sec in ulnar nerve). In 11 patients affected by motor neurone disease and 11 patients affected by amyotrophic lateral sclerosis the F-wave velocity decreased significantly proceeding proximally and the minimum values were found in the central tract (52.51 +/- 2.15 m/sec in MND and 48.64 +/- 5.60 m/sec in ALS). We therefore suggest the use of F-wave velocity as a more complete element for precise localization of the lesion in the central segment when the motoneurone is primarily involved.
Acta Neurol Scand 1976 Sep
PMID:F-wave velocity in motor neurone disease. 96 78

Using radial immunodiffusion, albumin and immunoglobulin G were determined in non-preconcentrated cerebrospinal fluid from 127 controls and from 239 patients. In controls the concentrations of albumin and immunoglobulin G followed normal distribution. The two variables were correlated linearly (r = 0.60). The elliptic bivariate normal range was calculated, and was found to contain 95% of the paired values. As a clinical limit, this range discriminated more effectively between normal and altered pairs than the two one-dimensional normal ranges X+/-2 s, thus improving the evaluation of laboratory findings in the single case. Likewise in clinically defined groups of patients, bivariate evaluation of results provided additional evidence. In many distinct clinical syndromes, e.g. bacterial encephalomeningitis, polyneuropathy, amyotrophic lateral sclerosis, albumin and immunoglobulin G concentrations exhibited an especially close correlation, probably resulting from damage to the blood-cerebrospinal fluid barrier. However, no correlation of these two variables was detected in acute encephalomeningitis due to virus infection, and in multiple sclerosis: in these groups, immunoglobulin G concentrations were elevated independently of albumin. Since evidence is lacking as to the pathogenesis of multiple sclerosis, it seems noteworthy that the same phenomenon was observed in a well-defined group of viral infections.
J Clin Chem Clin Biochem 1976 Sep
PMID:[Bivariate evaluation of laboratory findings: immunoglobulin G and albumin in cerebrospinal fluid (author's transl)]. 96

Anti-lymphocyte sera against human thymus [ALS-(THY)] were absorbed serially with cultured human lymphoblasts (CHL) or thymus and residual antigen-binding activity was tested. The absorbed ALS were used to bind 125I-labeled antigens from lymphocytes labeled by the lactoperoxidase catalyzed iodination technique. Absorption of ALS(THY) with CHL led to the absorbed serum having less than 5 to 10% of its original antigen-binding activity against labeled CHL antigens while maintaining from 20 to 40% of its original activity against labeled THY. Serial absorption of ALS(THY) with THY led to an equal decrease in activity against both THY and CHI. When the immunoprecipitates from these experiments were examined on polyacrylamide gels containing SDS it was found that serial absorption of ALS(THY) with THY first removed activity against a component of m.w. similar to 48,000 leaving relatively greater activity against material of apparent high molecular wieght. In contrast, absorption of ALS-(THY) with CHL removed the antibodies against the high molecular weight material while leaving activity against the component of m.w. 48,000. When these absorbed ALS were used to induce in vitro lymphocyte proliferation, it was found that ALS(THY) absorbed with CHL, did not. The retention or loss of mitogenicity seemed to correlate with retention or loss of binding activity against the component(s) of m.w. similar to 48,000.
J Immunol 1975 Sep
PMID:Lymphocyte plasma membranes. V. Specificity and mitogenicity of absorbed anti-lymphocyte sera. 115 Oct 74

The inter-organ distribution of radioactivity in rats injected with 51Cr-labelled SRBC is altered after treatment with ALS absorbed with this antigen. The alteration is due to the presence of soluble SRBC antigens in the serum and subsequent immunization of the tested animals. The 51Cr distribution does not correspond to the uptake of antigenic material in immunized rats.
Immunology 1975 Sep
PMID:Organ distribution of sheep red blood cells in ALS-treated rats. 116 11

A new system for computer aided EMG-analysis was tested in 3 groups of patients with neurogenic lesions (ALS, ulnar nerve lesions, diabetic polyneuropathy). The system entails automatic segmentation and parametrization of the single MUAP as well as the automatic classification of the MUAP into motor units. Emphasis was placed on its practicability in an everyday clinical setting. All patient groups differed significantly from normal groups in most of the computed parameters of the motor units. Moreover, on the basis of the computer-aided analysis of the MUAP up to 90% of individual patients in the ALS and ulnar nerve lesion groups and up to 40% in the diabetic polyneuropathy group, who did not have any pathological spontaneous activity or paris, could be classified as pathological. It is concluded, that computer-aided EMG-analysis has become a practical tool for routine use in the clinical laboratory simplifying early diagnosis of subtle EMG-changes, and aiding the less experienced examiner.
Electromyogr Clin Neurophysiol 1992 Sep
PMID:Computer-aided EMG-analysis in patients with neurogenic lesions. 139 99

Over the past 15 yr, a marked increase in crude mortality rates from Parkinson's disease (PD) and amyotrophic lateral sclerosis (ALS) has occurred in the U.S. This is often attributed to as yet undefined environmental factors. The deterministic risk of general mortality and mortality due to PD, ALS, ischemic heart disease (IHD), and stroke for the years 1963, 1977, and 1986 in the U.S., as defined by the method of longitudinal Gompertzian analysis, were calculated and compared. When the rise in PD and ALS mortality is viewed from the perspective of deterministic and competitive mortality dynamics, it becomes evident that the major force increasing mortality from these two neurologic diseases is the declining mortality from IHD and stroke. Consequently, there is no need to invoke intrinsic etiologic alterations in the environment to account for the observed increases in PD and ALS mortality. Recognition of the competitive nature of human mortality illustrates the inherent risk of making etiopathogenic conclusions based upon single disease mortality data.
J Clin Epidemiol 1992 Sep
PMID:Rising mortality due to Parkinson's disease and amyotrophic lateral sclerosis: a manifestation of the competitive nature of human mortality. 143 14

It has been assumed that amyotrophic lateral sclerosis (ALS) involves precocious senility as one of its pathogenetic aspects. The authors studied 55 autopsied cases of ALS in relation to age at death, ranging from 42 to 86. The materials consisted of 8 cases in the fifth decade, 8 in the sixth, 20 in the seventh, 12 in the eighth, and 7 in the ninth. The total duration of illness ranged from 6 months to 14 years. The most distinct relationship was observed in the anterior horn lesion of the cervical enlargement which became less severe with advancing age, irrespective of the length of illness. Fifth decade cases showed marked atrophy with severe neuronal loss and fibrillary gliosis in the anterior horn, while those in the ninth decade showed slight changes which were similar to age-matched controls. On the other hand, pyramidal tract degeneration did not show any correlation to age at death or to length of illness. Pyramidal tract degeneration was found in all younger age group cases, being always severe. In the older age groups, however, the degeneration varied extremely in degree from case to case. Some cases showed severe degeneration comparable with that in the younger age groups, while the others had no findings suggesting degeneration. In addition, cases on artificial respirators had a longer duration of illness, and more marked degeneration in the anterior horn, irrespective of age. Our study did not reveal that senile changes including senile plaques and neurofibrillary tangles were more marked in ALS cases. No clinicopathological correlation with dementia was recognized.(ABSTRACT TRUNCATED AT 250 WORDS)
Nihon Ronen Igakkai Zasshi 1992 Sep
PMID:[Neuropathological study of amyotrophic lateral sclerosis in relation to aging]. 143 59


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