Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0152025 (polyneuropathy)
 7,862 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The following parameters have been examined in twenty-one patients suffering from chronic renal failure (creatinine level between 4.5 and 18.8 mg/100 ml serum): maximum motor nerve conduction of the peroneal nerve, amplitude of the compound muscle action potential of the extensor digitorum brevis muscle, serum creatiine, total protein, serum globulins, serum albumins, alkali reserve, time of increase of serum creatinine above 4 mg/100 ml up to time of determination of the maximum motor nerve conduction, daily urinary excretion, mean blood pressure, (p less than 0.01) was found between maximum motor nerve conduction, as well as amplitude of the compound muscle action potential, and the serum albumin level only. Decreased levels of serum albumin, is correlated with diminished nerve conduction and a lower amplitude. The relationship between the electrophysiological data and serum albumin levels maybe explained on the basis of progression of a pre-existing polyneuropathy due to additional dietary malnutrition. A different interpretation is the assumption of an inactivation of neurotoxin on binding by albumins. A decrease in the albumin level would, therefore, result in an increased amount of unbound toxic agent. The values of the maximum motor nerve conduction were between 16 m/sec and 51 m/sec (mean value 42.2 m/sec), pointing to a polyneuropathy of primary axonal type rather that to primary demyelinization. The amplitudes of the compound muscle action potentials were not greatly reduced and thus the uraemic polyneuropathy seems to be of mixed type. In uraemic polyneuropathy different aetiological factors have to assumed. According to the prevalent factor a polyneuropathy of predominantly axonal or predominantly demyelinizing type may result.
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PMID:[Motor nerve conduction velocity in uraemic polyneuropathy: correlation with metabolic factors (author's transl)]. 101 10

Forty-three patients who had sepsis and multiple organ failure (critical illness) were studied prospectively to determine the incidence and severity of peripheral nerve function and to correlate such function with a number of variables. Electrophysiologic studies indicated a primary axonal degeneration of motor and sensory fibers in 30 (70 percent). Fifteen (30 percent) had the clinical signs of difficulty in weaning from assisted ventilation, weakness of limb muscles, and reduced or absent deep tendon reflexes. Full recovery from the polyneuropathy occurred among the 23 (53 percent) who survived, except three who had a very severe polyneuropathy. A peripheral nerve function index, computed from electrophysiologic measurements, showed statistically significant (p less than 0.01) negative correlations with the time in the critical care unit, and the serum glucose value; the serum albumin level showed a positive correlation. Multiple regression analyses indicated all three factors accounted for 47 percent (r2 = 0.4678) of all potential variables. In a separate analysis, the nerve function index correlated with the amplitude of the diaphragm compound muscle action potential (p less than 0.01). The results were consistent with the polyneuropathy being due to the same mechanisms that are currently postulated to cause dysfunction in this syndrome of other organ systems (including the neuromuscular respiratory system).
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PMID:Peripheral nerve function in sepsis and multiple organ failure. 184 61

We studied five patients in the intensive care unit (ICU) with acute polyneuropathy. All had previously presented severe infectious processes, accompanied by diverse organ failure accompanied by the Adult Respiratory Distress Syndrome (ARDS) in all cases. Two patients died and the three survivors suffered severe motor deficiencies. Electromyographic studies revealed axonal damage which predominantly affected motor neurons. Cerebrospinal fluid was normal in all the patients except one, who showed a moderate increase of lymphocytes. The common causes of polyneuropathy were excluded, but in all cases a nutritional disorder was detected, based on laboratory values of proteins, serum albumin and transferrin. We conclude that polyneuropathy is relatively frequent among critically ill patients and must be closely monitored because of diagnostic difficulties and the repercussions on the progress of these patients. In spite of uncertainties about its cause, it appears to be related to severe infectious processes, ARDS, and nutritional disorders.
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PMID:Acute polyneuropathy in critically ill patients. 217 65

Antibodies to gangliosides were detected in sera from three of 19 patients with chronic inflammatory polyneuropathy (CIP) by a thin-layer chromatogram overlay technique. All three of the patients fell into a clinical subset of the group that had multifocal motor neuropathy, and in all three patients the antibodies reacted with GM1 ganglioside. However, the fine specificities of the antibodies differed as demonstrated by cross-reactivity with different gangliosides in each of the three patients. The antibodies in patient 1 reacted with GM1, GD1b, and asialo-GM1 suggesting that the terminal Gal(beta 1-3)GalNAc moiety that is common to these three glycolipids is an important part of the epitope(s). This was confirmed by showing reactivity of the antibodies with Gal(beta 1-3)GalNAc conjugated to bovine serum albumin. Patient 2 had antibodies that did not react with GD1b, but cross-reacted with GM2 ganglioside suggesting that the epitope(s) involved the inner portion of the oligosaccharide moiety that is shared between GM1 and GM2. Patient 3 had antibodies that reacted with GM1 and asialo-GM1, but they did not cross-react with either GD1b or GM2. These results provide further evidence for a relationship between motor nerve syndromes and anti-GM1 antibodies and also suggest that GM1 could be a principal target antigen since other reactive gangliosides differed among the patients. However, the possible pathogenic effects of anti-GM1 antibodies on motor nerves remain to be established.
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PMID:Anti-GM1 ganglioside antibodies with differing fine specificities in patients with multifocal motor neuropathy. 258 93

CSF protein and cellular profiles were studied in 28 HIV-infected patients. Twenty of them had neurological complaints, but only 6 patients had objective neurological deficits such as dementia, ocular motility disorders or polyneuropathy. The serum/CSF HIV antibody ratio was on average lowest in acquired immunodeficiency syndrome (AIDS) (4 patients) and highest or almost normal in lymphadenopathy syndrome (LAS) (11) and asymptomatic seropositivity (ASX) (7), while it varied between these extremes in AIDS-related complex (ARC) (6). However, low values of the ratio were also found in the HIV-infected patients free of neurological symptoms and even in one ASX patient. The CSF IgG index was elevated in all these 4 general stages of HIV infection without any significant differences between them. The CSF/serum albumin ratio was slightly increased in patients with neurological deficits, but this ratio showed no association with any other clinical factor analysed. CSF leucocytes were increased in the early stages of the disease, but later the cellular reaction subsided. HIV was isolated from post mortem brain tissue of two AIDS patients and from the CSF of one of them. The results suggest increased intrathecal virus-specific IgG synthesis, not only in patients with neurological deficits and at advanced stages of infection, but also in neurologically symptom-free subjects and at early infection. The lack of correlation between the increased virus-specific IgG synthesis within the CNS and the presence of neurological symptoms suggests that neurologically "silent" areas of brain white matter are often affected in HIV infection.
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PMID:CSF protein and cellular profiles in various stages of HIV infection related to neurological manifestations. 303 7

Nutritional assessments were measured in the sixteen long-term survivors who had undergone a massive resection of more than two-thirds of the small intestine or less than 170 cm of the remaining small intestine. Prealbumin, retinol binding protein and zinc in serum and arm muscle circumference were significantly lower than the normal range. Serum albumin had a tendency to correlate to the length of the remaining small intestine. Nutritional risk index had a correlation with the length of the remaining small intestine. In this study, nutritional assessments in the patients with a massive resection of the small intestine indicated to be in preclinical malnutritional state. This may support that supplementary nutritional therapy is necessary for such patients. In addition, we reported a patient with sensory polyneuropathy caused by vitamin E deficiency due to short bowel syndrome. The level of vitamin E was low in his serum, 294 micrograms/dl (normal: 1004 +/- 65) and in his red blood cells, 136 micrograms/dl (normal: 176 +/- 9). His symptom was markedly decreased within two weeks after the administration of large doses of vitamin E.
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PMID:[Nutritional assessments in the long-term survivors following massive resection of the small intestine]. 322 94

Chickens fed with the same composition of diet as our low income beriberi polyneuropathic patients, developed clinical symptoms of thiamine deficiency in 22.3 +/- 6.3 days. There appeared to be a body store of thiamine which is utilized during a period of deficient intake. Haemoglobin content and serum albumin did not change appreciably during thiamine deficiency. The blood thiamine content was low and the thiamine pyrophosphate (TPP) effect increased to more than 25 percent during the development of the beriberi polyneuropathy, which resumed after one week on thiamine tetrahydrofurfuryl disulfide (TTFD) treatment. However, the clinical features gradually improved after about one month. Neurophysiological findings including somatosensory evoked potentials (SSEPs) and neuromorphological studies of the peroneal and sciatic nerves were compatible with a major degree of axonal degeneration and secondary minimal segmental demyelination. We may conclude that the experimentally induced beriberi polyneuropathy in chickens seems a good model for studying these forms of neuropathy in view of diagnosis and treatment.
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PMID:Experimentally induced beriberi polyneuropathy in chickens. 773 17

Eighteen Swedish patients with familial amyloidotic polyneuropathy were tested for the met30 mutation of the transthyretin (TTR) (prealbumin) gene by RFLP analysis of genomic DNA using the restriction enzyme NsiI. The results confirmed previous findings that the Swedish variant of familial polyneuropathy has the same valine by methionine substitution at position 30, as seen in patients with FAP from Japan, Portugal or patients of Swedish descent from USA. However, two of the patients were homozygous, totally lacking the wild type allele. Measurable serum values for the variant transthyretin (TTR) was detected with a RIA-method in all the 18 Swedish FAP patients studied with a mean concentration of 12.1 +/- 5.1 (SD) mg/100 ml, (11.0 +/- 4.1 when the two homozygotes were excluded). In 45 Japanese patients the mean was 9.2 +/- 2.7 mg/100 ml. The variant TTR was not detected in the healthy controls. The value for the variant TTR was nearly twice that high in the two homozygous patients, 21.14 and 21.16 mg/100 ml, respectively. There was no correlation between the serum levels of variant TTR and the duration of disease or levels of serum albumin in the FAP-patients.
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PMID:Diagnostic radioimmunoassay and DNA-analysis in Swedish and Japanese patients with familial amyloidotic polyneuropathy. Homozygosity for the TTR met30 gene. 809 20

Neuropathic complications of the burn patient are frequently undiagnosed. A retrospective study was performed looking at neuropathies in patients admitted to a tertiary care burns centre from 1984 to 1991. Nineteen out of a total of 800 patients had signs and symptoms of neuropathy, confirmed on neurophysiological testing. Most patients were severely burned with 11 patients (69%) having a total burn surface area of > 20%. Twenty-eight percent were full thickness burns. Mononeuritis multiplex was the most common finding in these patients, occurring in 11 (69%). This has not been reported before. Three patients (19%) had an isolated mononeuropathy, one (6%) had a radiculopathy and one had a generalized axonal polyneuropathy. Of the patients with mononeuropathy, nine had lesions only in burned areas and four had lesions in burned and unburned areas. Eleven patients had complications of sepsis with five also having renal failure. Age, sex, serum albumin, magnesium, phosphate, creatinine, the presence of sepsis and the number or type of drug did not correlate with the number of affected nerves nor the extent of recovery. The length of hospitalization and severity of the burns were the only two factors which correlated with the number of affected nerves. Vascular occlusion of the vasa nervorum, direct thermal injury or a disseminated neurotoxin are postulated as possible aetiological mechanisms.
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PMID:Neuropathy in burn patients. 838 17

FAP (familial amyloidotic polyneuropathy) is a systemic amyloid disease characterized by the formation of extracellular deposits of transthyretin. More than 80 single point mutations are associated with amyloidogenic behaviour and the onset of this fatal disease. It is believed that mutant forms of transthyretin lead to a decreased stability of the tetramer, which dissociates into monomers that are prone to unfolding and aggregation, later forming beta-fibrils in amyloid deposits. This theory does not explain the formation of beta-fibrils nor why they are toxic to nearby cells. Age at disease onset may vary by decades for patients with the same mutation. Moreover, non-mutated transthyretin also forms the same deposits in SSA (senile systemic amyloidosis), suggesting that mutations may only accelerate this process, but are not the determinant factor in amyloid fibril formation and cell toxicity. We propose that glycation is involved in amyloidogenesis, since amyloid fibrils present several properties common to glycated proteins. It was shown recently that glycation causes the structural transition from the folded soluble form to beta-fibrils in serum albumin. We identified for the first time a methylglyoxal-derived advanced glycation end-product, argpyrimidine [N(delta)-(5-hydroxy-4,6-dimethylpyrimidin-2-yl)-L-ornithine] in amyloid fibrils from FAP patients. Unequivocal argpyrimidine identification was achieved chromatographically by amino acid analysis using dabsyl (4-dimethylaminoazobenzene-4'-sulphonyl) chloride. Argpyrimidine was found at a concentration of 162.40+/-9.05 pmol/mg of protein in FAP patients, and it was not detected in control subjects. The presence of argpyrimidine in amyloid deposits from FAP patients supports the view that protein glycation is an important factor in amyloid diseases.
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PMID:Argpyrimidine, a methylglyoxal-derived advanced glycation end-product in familial amyloidotic polyneuropathy. 1528 12


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