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)

A 51-year-old man had primary amyloidosis, with typical amyloid neuropathy and signs of motor neuron disease, including widespread fasciculation in limb muscles, tongue atrophy and fasciculation, swallowing and chewing difficulty, symmetric hyperreflexia, and bilateral Hoffmann's signs. Fasciculations, fibrillations, and positive sharp waves were found in electromyography of all muscles tested. Motor nerve conduction velocities were moderately slow. Lambda chains were detected in serum and CSF. Amyloid was found in sural nerve biopsy. This combination of amyloid neuropathy and features of amyotrophic lateral sclerosis is related to the motor neuron disease of plasma cell dyscrasias.
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PMID:Primary amyloidosis with peripheral neuropathy and signs of motor neuron disease. 301 5

We performed double-blind crossover trials to assess the effects of thyrotropin-releasing hormone (TRH) on amyotrophic lateral sclerosis patients. For acute intravenous trials, 500 mg TRH or placebo with norepinephrine was given at 1-week intervals (16 patients). CSF TRH concentration increased, and clinical side effects appeared with TRH. For chronic studies, 25 mg TRH and a saline placebo were given subcutaneously every day for 3 months (25 patients). CSF TRH level increased 29-fold after a single TRH injection, and mild transient side effects occurred. Vital signs, respiratory function, semiquantitative and quantitative neurologic function, muscle strength by manual and dynamometer testing, and EMG were studied. With daily TRH, 10 patients noted subjective improvement without objective evidence, and 10 patients complained of worsening of the disease with objective decline after TRH was stopped. Statistical analysis, however, showed no beneficial effects from either acute or chronic TRH trials.
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PMID:Amyotrophic lateral sclerosis: effects of acute intravenous and chronic subcutaneous administration of thyrotropin-releasing hormone in controlled trials. 308 Jun 95

In spinal cords from seven amyotrophic lateral sclerosis (ALS) patients and four controls, we found no difference in thyrotropin-releasing hormone (TRH) concentration relative to protein content, but there was a reduction per tissue wet weight in ALS. Immunohistochemical localization of TRH in ALS cord was unaltered. Histidyl proline diketopiperazine (HisPro-DKP), a possible metabolite of TRH, was significantly elevated per protein content in ALS. CSF levels of TRH and HisPro-DKP were unchanged. These findings suggest that TRH neurons are not primarily affected in ALS, but TRH and tissue protein are lost together as the disease progresses.
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PMID:Amyotrophic lateral sclerosis: thyrotropin-releasing hormone and histidyl proline diketopiperazine in the spinal cord and cerebrospinal fluid. 309 30

An investigation of the efficacy of thyrotropin-releasing hormone in amyotrophic lateral sclerosis included study of the intrathecal pharmacokinetics of this neuropeptide. Its mean elimination half-life in CSF was 0.90 hours and was monoexponential. During a 2-hour infusion, 2.75% crossed the CSF/blood-brain barrier. Infusion for 12 months with an implanted pump in three patients at a rate of 3 mg/24 hr resulted in a mean CSF steady state of 2.88, 2.42, and 2.74 micrograms/ml, respectively. Pharmacokinetic data were similar at 6 and 12 months. This is an effective system with potential uses in the treatment of other degenerative diseases.
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PMID:Pharmacokinetics of intrathecal thyrotropin-releasing hormone. 310 19

The concentrations of 23 amino acids (AA) were measured in CSF of patients with Amyotrophic Lateral Sclerosis (ALS). A micro-method with picomole sensitivity was used. Compared with healthy controls no significant alterations of single or total AA concentrations were found. These results contrast with data published in a previous study and will be discussed in detail.
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PMID:Free amino acid pattern of cerebrospinal fluid in amyotrophic lateral sclerosis. 312 52

Prealbumin (transthyretin) quantified in CSF was negatively correlated with degree of dementia in 24 patients with dementia of Alzheimer type (DAT). There was neither a significant correlation between prealbumin in serum and degree of dementia nor between prealbumin in CSF and number of T lymphocytes in serum. There was no such relationship in 7 patients with multi-infarct dementia. Prealbumin was also quantified in 4 non-demented control groups, one consisting of 14 age- and sex-matched neurological patients, 17 with MS, 6 with ALS and 10 patients who had had a subarachnoid hemorrhage. Prealbumin was significantly lower in the last group. The study suggests that there may be a relationship between prealbumin levels in cerebrospinal fluid and degree of dementia. It is suggested that this may be caused by affection of the choroid plexus.
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PMID:Reduced prealbumin (transthyretin) in CSF of severely demented patients with Alzheimer's disease. 322 31

Particular clinical pictures of ALS may occur during the course of some "benign gammapathies". We observed 12 patients (age range 57 to 74 years; 9 men-3 women) with initially benign gammapathy (9 IgG, 1 IgA, 2 IgM) associated to a clinical picture of progressive anterior horn and pyramidal tract involvement. These cases led us to recognize some particularities of gammapathy-associated ALS: Relative frequency of asymmetrical clinical manifestations, rarity of bulbar signs; Decrease of sensory nerve conduction velocities without evidence of sensory clinical symptoms (8 out 9); Increase CSF protein content including the monoclonal component, axonal degeneration and immunostaining evidence of the paraprotein fixation observed on nerve biopsies (5 out 7 cases). Association of ALS and gammapathy is not fortuitous as shown by epidemiology, experiments, pathology and effects of different immunological treatments as related in this study. Demonstration of infra-clinical neuropathy face to a clinical syndrome of ALS should prompt too careful screening for a gammapathy.
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PMID:[Peripheral neuropathies simulating amyotrophic lateral sclerosis in gammopathies]. 323 59

Serum and CSF from 32 patients with idiopathic ALS, 30 age-matched controls and 30 MS patients were investigated regarding immunoglobulin concentration and virus-specific antibodies, the lymphocytes in the peripheral blood and lymphocyte subsets were also investigated. ALS patients' results were compared with findings in MS and controls. The ALS patients had significantly higher IgG concentration in serum than the controls, marked lymphopenia, reduction of CD2, CD8 and Leu 7 positive cells and increase of the CD4/CD8 ratio and of SIg-positive lymphocytes. Compared with the MS patients, the ALS patients showed similarity in T-subset distribution with a lower standard deviation. No HTLV-I and HIV antibodies were found in any group and no significant differences in antibody distribution to Toxoplasma G, herpes simplex, cytomegalovirus, measles and mumps viruses were evident. All ALS patients were investigated at an early disease stage, therefore, our findings seem to support the conclusion that the immune alterations are related to the mechanisms of the disease and not to complications of its evolution.
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PMID:Immunity assessment in the early stages of amyotrophic lateral sclerosis: a study of virus antibodies and lymphocyte subsets. 326 63

Ten patients with amyotrophic lateral sclerosis were given intrathecal injections of natural interferon alpha, 1 million units weekly for 7 to 24 weeks. Six patients completed the trial. Four voluntarily withdrew after 7 to 13 injections. The slopes of deterioration for 40 quantitative tests of neuromuscular function for the control and treatment periods were compared by paired t test in the six patients who completed the trial and in the patient who withdrew after 13 injections. No significant differences were found. The patients tolerated treatment well. The CSF reaction was modest and spontaneously reversible. Indomethacin and ibuprofen blocked interferon side effects.
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PMID:Intrathecal administration of natural human interferon alpha in amyotrophic lateral sclerosis. 352 80

The authors determined FDP levels in the cerebrospinal fluid by the method of Merskey in 214 neurological patients and found raised levels in 58.6% of cases (from 0.1 to 8.0 ug/ml, with normal value range 0-0.5 ug/ml). In the control group the FDP levels in the CSF were normal. No correlation was noted between the FDP levels in the CSF and in blood. Raised CSF FDP level was observed in exacerbations of multiple sclerosis, strokes especially of embolic origin, syringomyelia, bulbar form of amyotrophic lateral sclerosis, epilepsy, migraine, lumbar disc lesions, polyneuropathy, parkinsonism, brain atrophy, after craniocerebral trauma, in Kleine-Levin syndrome. The authors are studying now the course of FDP changes in the CSF in various cases in the aspect of clinical-laboratory correlations.
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PMID:[Fibrin fibrinogen degradation products in the cerebrospinal fluid of neurological patients]. 610 Mar 18


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