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At least 19 recordsLinked to original sources

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

Computerized axial tomography and cerebral scintigraphy in leukodystrophy. A study of two boys presumably suffering from lysosomal disease.

Two unrelated boys, 12 and 9 years old, suffered from a diffuse cerebral disease that followed a parallel, subacute course. Mental regression, loss of hearing and vision, spastic-ataxic and pseudobulbar disturbances, and atrophy of the optic nerves occurred in both. Enzyme studies and the liver biopsy of one of the patients suggest a "lysosomal disease." The hallmark of both patients is the striking similarity of the cerebral scintigraphy and the computerized axial tomography (CT). Cerebral scintigraphy showed annular and crescent-shaped areas of increased radioactivity in the parietoccipital region. The CT indicated bilateral, symmetric bands of elevated density after contrast enhancement in the paraventricular white matter in the same region. These findings and the neurologic symptoms are compatible with leukodystrophy. Thus cerebral scintigraphy and CT appear to be useful aids in the diagnosis of metabolic brain disease. Computerized axial tomography is preferred for distinguishing whether lesions are in white or gray matter.

Brain

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

[Occurrence of triphasic waves in two cases of thyrotoxic crisis (author's transl)].

An electroencephalogram taken on two patients suffering from a thyrotoxic crisis, showed the presence of typical triphasic waves. This pattern was first described in 1950 by Foley et al., who believed that it occurred specifically in patients with hepatic encephalopathy. Since then, however, it has been shown that triphasic waves can be identified in other (e.g. renal) metabolic brain diseases and, less commonly, in a variety of either organic or functional cerebral disorders.

Electrocardiography

[The E.E.G. in methylmalonic acidaemia (author's transl)].

The EEG study was carried out on 8 cases of methylmalonic acidaemia, in the neonatal period (3 cases), developing later with a ketotic coma, hyperglycinemia, hyperammonaemia, leucopenia and thrombocytopenia (3 cases) and detected before birth (2 cases). The tracings of the neonates in the first group had a periodic appearance. The 2 children detected pre-natally had essentially normal EEGs bar a slightly faster rhythm. The tracings of the children in ketotic coma were similar to those seen in metabolic coma of other cause. The value of this study was, besides characterizing the EEC pattern which resembled that seen in other metabolic illnesses in the neonatal period, to study the changes in the EEG trace and their improvement during dialysis treatment. In one case recordings were made throughout the duration of dialysis.

Amino Acid Metabolism, Inborn Errors

[Neonatal myoclonic encephalopathy (author's transl)].

The authors studied 5 patients with neonatal myoclonic encephalopathy commencing in the hours immediately after birth and consisting of erratic, asynchronous, non-periodic myoclonus associated with generalized jerks in 4. The EEG showed bursts of pseudo-periodic activity on a low voltage background rhythm. Neuropathological study showed poliodystrophy in one case and a disturbance of cortical lamination in another. The relation of these cases with non-ketotic hyperglycinaemia is discussed.

Amino Acid Metabolism, Inborn Errors

[The E.E.G. in non-ketotic hyperglycinaemia (author's transl)].

12 cases of non-ketotic hyperglycinemia in neonates diagnosed at a time of neurological distress were studied. A characteristic tracing was observed permitting correct diagnosis in 6 cases. It consisted of a burst suppression characterized by high voltage complexes separated by low amplitude sequences. This appearance remained until the 15th day, with no electroclinical changes. In cases followed the trace closely resembled hypsarrhythmia.

Amino Acid Metabolism, Inborn Errors

3-methylglutaconic aciduria: report on a sibship with infantile progressive encephalopathy.

Choreoathetosis, spastic parapareses, dementia and optic atrophy were the main clinical features in a sibship with progressive encephalopathy of late onset. The urine contained constantly elevated amounts of 3-methylglutaric and 3-methylglutaconic acids. The identity of these metabolites was confirmed by synthesis and mass spectrometry. On leucine loading, the excretion of the metabolites was elevated.

Amino Acid Metabolism, Inborn Errors