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At least 19 recordsLinked to original sources
PubMed1979

A rational approach to dementia.

Dementia is a common problem facing all medical practitioners and it frequently results in hospitalization and death. This review provides a framework for dealing with dementia in clinical practice that is based on both traditional concepts and recent advances in the understanding of the problem. Distinguishing at the bedside between dementia and other disorders of intellect is emphasized. The main causes of dementia and their clinical characteristics are reviewed and a rational approach to definitive diagnosis is developed. Simple, effective symptomatic forms of therapy are described.

Aged↗
PubMed1976

The neurological complications of cardiac transplantation.

Review of the neurological complications encountered in 83 patients who received cardiac homografts over a seven-year period leads to the following conclusions: (1) Neurological disorders are common in transplant recipients, occurring in over 50 per cent of patients. (2) Infection was the single most frequent cause of the neurological dysfunction, being responsible for one-third of all CNS complications. (3) The infective organisms were typically those considered to be usually of low pathogenicity: fungi, viruses, protozoa and an uncommon bacterial strain. (4) Other clinical neurological syndromes were related to vascular lesions, often apparently from cerebral ischaemia or infarction occurring during the surgical procedure, metabolic encephalopathies, cerebral microglioma, acute psychotic episodes and back pain from vertebral compression fractures. (5) The infectious complications and probably the development of neoplasms de novo, are related to immunosuppressive therapy which impairs virtually all host defence mechanisms and alters the nature of the host's response to infective agents or other foreign antigens. (6) Because neurological symptoms and signs were usually those of behavioural changes or deterioration in intellectual performance, the neurological examination was often of little value in diagnosing the nature or even the anatomical site of the neuropathological process. (7) The possibility of an infectious origin of the neurological manifestations must be aggressively pursued even in the absence of fever and a significantly abnormal spinal fluid examination. The diagnostic error made most frequently was to ascribe neurological symptoms erroneously to metabolic disturbances or to "intensive care unit psychosis" when they were in fact due to unrecognized CNS infection. (8) Maintenance of mean cardiopulmonary bypass pressures above 70 mmHg, particularly in patients with known arteriosclerosis, may reduce operative morbidity. (9) Though increased diagnostic accuracy is possible with routine use of a variety of radiological and laboratory techniques, two further requirements probably must be met before a significant reduction in the frequency of neurological complications will occur: the advent of greater immunospecificity in suppressing rejection of the grafted organ while preserving defences against infection; and a more effective armamentarium of antiviral and antifungal drugs.

Adolescent↗
PubMed1979

Relation of potassium transport to oxidative metabolism in isolated brain capillaries.

1. The uptake of K by a capillary suspension isolated from rat brain was studied with the radioactive analogue (86)Rb.2. Rb uptake was dependent upon the presence of oxygen and could be markedly inhibited with ouabain.3. The ouabain sensitive Rb uptake was measured at varying external concentrations of K. Uptake of K (as (86)Rb) was half-maximal when the K concentration was 3.0 mM. This in vitro affinity of the transport carrier for K is similar to that found in previous in vivo studies of K efflux from brain to blood.4. I propose that the ouabain sensitive K pump is located on the antiluminal plasma membrane of brain capillary endothelial cells and that this pump contributes to the maintenance of a constant concentration (i.e. 3 mM) of K in brain interstitial fluid.5. Glucose and palmitate were tested as possible energy substrates for the support of active Rb uptake by isolated brain capillaries. The rate of Rb uptake increased 40% when 0.25 mM-palmitate was added to a capillary suspension containing 5 mM-glucose. This stimulation of Rb uptake could be blocked by 1 mM-4-pentenoic acid, an inhibitor of fatty acid oxidation. In contrast, the fraction of Rb uptake supported by glucose was not altered by 4-pentenoic acid.6. The rates of [U-(14)C]glucose and [U-(14)C]palmitate oxidation to CO(2) were measured in isolated brain capillaries and compared to their oxidation by brain slices and synaptosomes. Palmitate was the source of 28% of the (14)CO(2) produced by the capillaries but only 0.5% of the (14)CO(2) produced by the brain slices and synaptosomes.7. It is concluded that brain capillaries are similar to renal tubules in their polar distribution of ouabain sensitive K transport carriers, dependence on oxidative metabolism for active ion transport, and use of fatty acids as energy substrates. These features may underlie the vulnerability of brain capillaries in several metabolic diseases that cause brain oedema.

Animals↗
PubMed1978

[Monophylic vacuolisation of promyelocytes in Menke's-syndrome (trichopoliodystrophy) (author's transl)].

In one case of Menkes' Syndrome (Trichopoliodystrophy), a monophylic vacuolisation of myeloic cells (promyelocytes) of the bone marrow was observed. This finding correlates with an identical observation reported in medical literature, as well as with vacuolisations of metabolic active cells of the brain, musculature and skin observed in patients suffering from this disease. In the present paper, this finding is interpreted as an expression of the underlying disease and of the deficiency of oxidative cell ferment systems, and is discussed as a possible diagnostic and therapeutic criterion.

Autopsy↗
PubMed2025

Odd-Chain Dicarboxylic Acid Feeding Produces a Glutaric Aciduria Type 1-Like Metabolic Signature in Mice.

Glutaric aciduria type-1 (GA1) is an inherited mitochondrial neurometabolic disorder with a poorly understood pathogenesis and unmet medical needs. GA1 can be diagnosed via its hallmark biochemical signature consisting of glutaric aciduria, 3-hydroxyglutaric aciduria, and increased plasma glutarylcarnitine. These glutaryl-CoA-derived metabolites are thought to originate solely in the mitochondria. Here, we demonstrate that wild-type mice fed an 11-carbon odd-chain dicarboxylic acid (undecanedioic acid, DC11) recreate the biochemical phenotype of GA1. Odd-chain dicarboxylic acids like DC11 are not present in food but can arise from several endogenous processes, such as lipid peroxidation and fatty acid ω-oxidation. DC11 is chain-shortened in peroxisomes to glutaryl (DC5)-CoA, which then gives rise to the GA1-like pattern of DC5 metabolites in urine, tissues, and blood. Glutaric acid released from peroxisomes during DC11 chain-shortening can enter mitochondria for reactivation by the enzyme succinyl-CoA:glutarate-CoA transferase (SUGCT) and become substrate for glutaryl-CoA dehydrogenase (GCDH), the enzyme that is deficient in GA1. Our data provide proof-of-concept that the generation of dicarboxylic acids by ω-oxidation, which is stimulated during the same catabolic states known to trigger acute encephalopathy in GA1, may exacerbate disease by increasing the glutaryl-CoA substrate load in mitochondria.

Animals↗
PubMed1978

Kinky hair disease. Report of a case.

A confirmed case of kinky hair disease is described. Findings include a defect in copper metabolism, peculiar facies, retrognathia, skeletal open-bite, generalized gingival enlargement, and skeletal radiographic abnormalities.

Brain Diseases, Metabolic↗
PubMed1977

Menkes kinky hair syndrome: Is it a treatable disorder?

A male infant with Menkes Kinky Hair Syndrome was treated with a 3-week course of cupric acetate infusions, which was terminated when he developed aminoaciduria. The lack of improvement seen in this infant is representative of the reported experience with parenteral copper therapy in this condition, and may be attributable to the presence of a clinically significant abnormality in copper metabolism in utero.

Administration, Oral↗
PubMed1979

Plasma amino acids as predictors of the severity and outcome of sepsis.

Sepsis is a major catabolic insult resulting in a peripheral energy deficit which is made up in part by increased breakdown of lean body mass and oxidation of amino acids, principally the branched chain amino acids. The prognosis in any given case of sepsis is difficult to predict, but should theoretically be related to the degree of disturbance in peripheral energy deficit, which may in turn, be related to plasma amino acid pattern. In order to study whether this hypothesis was correct, plasma amino acids and some of their metabolic byproducts, the beta-hydroxyphenylethanolamines, were studied in 25 septic patients, and were used as discriminant variables in a series of computer performed discriminant analyses and multiple regressions. The two functions tested were the degree of metabolic septic encephalopathy as a determinant of the severity of sepsis and the final outcome in the septic patient. Plasma amino acid patterns exhibited elevated levels of the aromatic and sulfur containing amino acids, phenylalanine, tryosine, tryptophan, methionine, cysteine, and taurine, normal concentrations of alanine, and low normal concentrations of the branched chain amino acids, valine, leucine and isoleucine. Arginine levels, as previously noted, were very low. Patients not surviving the septic episode exhibited higher concentrations of aromatic and sulfur containing amino acids, while patients surviving sepsis had higher concentrations of the branched chain amino acids and arginine. When the degree of encephalopathy as a determinant of the severity of sepsis and step wise discriminant analysis with multiple crescent techniques were used, the best discriminant function between patients with and without encephalopathy was found to result from the interaction of cysteine, methionine, phenylalanine, isoleucine, leucine, and valine. These amino acids gave a correct classification in 82% of patients with no encephalopathy, and 80% of patients with septic encephalopathy. When the same amino acids were used for the discriminant analysis for patients dying of sepsis and patients surviving, the best discriminant function was achieved by using plasma concentrations of alanine, cysteine, methionine, isoleucine, arginine, tyrosine and phenylalanine resulting in 91% of the nonsurvivors, and 79% of the survivors correctly classified. The results suggest a close and significant relationship between the deranged energy metabolism and muscle protein breakdown in sepsis, and the outcome. This further suggests a central role for certain amino acids in perhaps predicting the severity of sepsis and its outcome.

2-Hydroxyphenethylamine↗