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)

This article reports delayed dysneuria 143 cases, 54 cases in male and 89 cases in female, age 8-59 years old. They are treated by atropine. After the cholinesterase inhibited symptoms had vanished or had improved and after the other factors had been eliminated the delayed dysneurias occur after poisoning 5.42 days. They are the peripheral neuritis, the amyotrophic lateral sclerosis, the myasthenic crisis, the peptic neuritis, the encephalitis, the mixed aphasia and the symptoms like Guillain-Barre's syndrome. Their death rates are higher in two months to the types of the myasthenic crisis, the encephalitis and the amyotrophic lateral sclerosis and to the other types of disease and the cases. Poisoned two months later, their prognosis are better and the mechanism are not very clear now.
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PMID:[The report of organophosphorus pesticides cause delayed nervous system diseases (143 cases)]. 166 36

Uneqivocally positive edrophonium tests, both clinically and electrophysiologically, were observed in a classic case of the Lambert-Eaton myasthenic syndrome. A review of the literature revealed that a positive edrophonium response has been reported in a majority of cases of myasthenia gravis (MG) and overlap myasthenic syndrome and in some cases of the Lambert-Eaton myasthenic syndrome, botulism, congenital myasthenic syndrome, drug-induced myasthenic syndrome, the Guillain-Barre syndrome, and amyotrophic lateral sclerosis. From this, we conclude that an unequivocally positive edrophonium test alone is not necessarily diagnostic of MG and that the diagnosis of MG should be based on the clinical features together with edrophonium-responsiveness and other laboratory findings.
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PMID:Edrophonium responsiveness not necessarily diagnostic of myasthenia gravis. 226 95

In the terminological dictionary of the AAEE conduction block is defined as inability of impulse to pass through a certain point in nerve fibre, although conduction is preserved below that point. Conduction block is manifested as a quantitatively corresponding drop of the amplitude and area of motor evoked potential (MEP) in the distal part in relation to the values of these parameters proximal to the block. Excessive desynchronization of conduction in nerve fibres and certain technical factors should be excluded. Multifocal conduction block is found in many neuropathies, particularly purely motor ones. The probable mechanisms responsible for the difference between motor and sensory nerve fibres with respect to their susceptibility to block development have been discussed. The understanding of conduction block could have important clinical implications, especially in the differential diagnosis of chronic demyelinative neuropathies, in the selection of immunological research methods, in the choice of treatment and prognostication of possible improvement (e.g. in the Guillain-Barre syndrome). Purely motor neuropathy with multifocal conduction block may simulate amyotrophic lateral sclerosis. The development of block in neuropathies is associated frequently with increased level of antibodies against GM1 and the interrelation between these phenomena is not known; the development of block may be due to deposition of GM1 antibodies in internodes or Ranvier's nodes with production of demyelination or with damage to ion channels. Although the concept of conduction block has been introduced in electroneurographic diagnosis there are still many doubts as to the correctness of diagnostic criteria, indispensable technical conditions, and also to the basic problems, such as e.g. 1) is conduction block really limited to acquired neuropathies (its possible occurrence in congenital neuropathies is discussed), 2) why is conduction block not reflecting clinical improvement, 3) in what degree is block reflecting the loss of motor units. These questions are discussed by the author in the light of literature reports and own experience.
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PMID:[Conduction bloc in peripheral nerves. Facts and hypotheses]. 804 25

High titers of anti-GD1a antibodies have been found in patients with Guillain-Barre syndrome or motor neuropathy. To determine the possible diagnostic relevance of these antibodies, we measured serum anti-GD1a IgG and IgM antibodies by enzyme-linked immunosorbent assay in 195 patients with different motor syndromes and in 335 control subjects. Moderately high antibody titers (1/1,280-1/5,120) were occasionally found in patients with chronic inflammatory demyelinating polyneuropathy (5%), multifocal motor neuropathy (18%), lower motor neuron disease (3.8%), or amyotrophic lateral sclerosis (1.8%) and in immunological control subjects (1.2%), while titers of 1/20,480 or higher were only found in 2 patients with Guillain-Barre syndrome (IgG in both) and 2 with motor neuropathy and IgM lambda monoclonal gammopathy improving with immunotherapy. In both motor neuropathy patients and the Guillain-Barre syndrome patient who were retested during recovery, anti-GD1a titers decreased concomitantly with clinical improvement. High anti-GD1a antibody titers may be found in several motor syndromes but only markedly increased anti-GD1a titers are strictly associated with potentially treatable dysimmune neuropathies.
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PMID:Anti-GD1a ganglioside antibodies in peripheral motor syndromes. 861 33

We examined serum antibodies to 4 species of fucogangliosides: fucosyl-GM1, fucosyl-GD1b, alpha galactosyl (alpha fucosyl) GM1 and alpha galactosyl (alpha fucosyl) GD1b, in 329 patients with various neurologic diseases, 32 patients with non-neurologic autoimmune diseases and 50 healthy persons. Nine patients with Guillain-Barre syndrome, 2 with amyotrophic lateral sclerosis and 2 with cerebral infarction had IgG anti-fucosyl-GM1 antibody. Five patients with Guillain-Barre syndrome, 1 with cerebral infarction and 1 normal control subject had IgM anti-fucosyl-GM1 antibody, 3 of whom also had IgG anti-fucosyl-GM1 antibody. Sixteen of 17 patients who had IgG or IgM antibody to fucosyl-GM1 showed no sensory dysfunction. Yoshino et al. [J. Neurochem. 1993, 61: 658-663] speculated that anti-fucosyl-GM1 antibody functions in the development of sensory neuropathy, but our results do not support this. Two patients with sensory ataxic neuropathy had high IgM antibody titers to fucosyl-GD1b and alpha galactosyl (alpha fucosyl) GD1b. These fucogangliosides may be the target molecules for serum antibodies in some patients with sensory ataxic neuropathy.
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PMID:Antibodies to fucogangliosides in neurological diseases. 926 Aug 62

Pro and active-matrix metalloproteinase-9 (MMP-9) was measured in sera from patients with amyotrophic lateral sclerosis (ALS), Guillain-Barre syndome (GBS), and healthy subjects. Both forms of MMP-9 were elevated in sera of ALS and GBS patients, compared with healthy controls. It has been postulated that elevated MMP-9 reflects damage to peripheral nerve and muscle. This possibility was investigated in sera, and tissue extracts of sciatic nerves and muscle from mice 5 and 12 days after axotomy of the sciatic nerve. Pro-MMP-9 was elevated in sera and extracts of damaged nerve and muscle, suggesting such damage may be followed by elevated pro-MM9-9 in sera. Active MMP-9 was only elevated in the sera. However, in situ activation of MMP-9 is tightly regulated and localised, and probably difficult to demonstrate by ELISA, resulting in a short half-life active MMP-9, implying any active MMP-9 in the serum may have a more immediate origin than injured muscle or nerve, for example circulating blood cells.
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PMID:The pro and the active form of matrix metalloproteinase-9 is increased in serum of patients with amyotrophic lateral sclerosis. 1565 14

Neuromuscular diseases can affect all respiratory muscles, leading to acute respiratory failure, which is the most common cause of morbidity and mortality in those patients. Two situations must be distinguished. 1) Acute respiratory failure as part of a neuromuscular disorder of acute onset and possibly reversible (Guillain-Barre syndrome, myasthenic crisis...). 2) Acute respiratory failure occurring in a patient with an already advanced neuromuscular disease (amyotrophic lateral sclerosis, Duchenne muscular dystrophy...). This article describes the neuromuscular acute respiratory failure in these different aspects, discusses its initial management in the emergency department and identifies the parameters that have to be monitored.
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PMID:[Acute respiratory failure in neuromuscular disease]. 2661 4

Myasthenia gravis (MG), amyotrophic lateral sclerosis and Guillain-Barre syndrome (GBS) have been classically considered as exceptional or unusual diseases in people with a geriatric profile. Over the past 25 years, several population-based studies have been conducted in the Osona area (Barcelona), which, for the first time, has led to describing the high global incidences in the elderly, especially those over 80 years-old. The results suggest the possibility of underdiagnosis of these neuromuscular diseases in the elderly, a fact that could be especially relevant in the case of MG and GBS, since they are 2potentially reversible entities with high mortality in the event of underdiagnosis and absence of treatment.
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PMID:[Under-diagnosis of neuromuscular diseases in patients of 80 years and older]. 3052 74

Wilting disorder is an abnormal condition characterized by weakness and paralysis of the upper and lower extremities. Pathogenesis and treatment target of the disorder are unclear; hence, allopathic treatment is generally used to relieve the symptoms. To investigate the treatment mechanism and effect of Traditional Korean Medicine (TKM) in patients with wilting disorder, we reviewed in vivo studies that focused on the effect of TKM on the main symptoms of wilting disorder and treatment of the diseases that can cause these symptoms. We electronically searched the PubMed, Cochrane, and CNKI (China National Knowledge Infrastructure) databases using the following search terms: (weakness OR motor function disorder) (myasthenia gravis OR Guillain-Barre syndrome OR amyotrophic lateral sclerosis OR paralysis OR polymyositis OR muscular dystrophy) AND (herbal medicine OR acupuncture OR bee-venom OR pharmacoacupuncture OR electro-acupuncture OR moxibustion). We selected 11 studies that demonstrated the effect of TKM treatment on the main symptoms of wilting disorder. In these studies, inducted models of amyotrophic lateral sclerosis, myasthenia gravis, Duchenne muscular atrophy, polymyositis, and Guillain-Barre syndrome were used. With regard to treatment, herbal medicine was used in five studies, and acupuncture and bee-venom pharmacoacupuncture were used in three studies each. Future research is needed to determine the effectiveness of TKM treatment in patients with diseases that can cause the main symptoms of wilting disorder.
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PMID:Traditional Korean Medicine Treatment for Patients with Wilting Disorder: A Literature Review of In Vivo Studies. 3053 61