Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “ENCEPHALOMALACIA”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 613 records · Page 34Linked to original sources
PubMed1982

X-linked Leigh's syndrome.

Two male half siblings developed rapid progression of neurologic symptoms at 11/2 and 21/2 years of age. Neither boy had a metabolic acidosis. Characteristic features of subacute necrotizing encephalomyelopathy, the neuropathologic basis of Leigh's syndrome, were demonstrated at autopsy. X-linkage of the disorder was considered because the boys had different fathers. An X-linked form of Leigh's syndrome was supported by a review of the literature, which showed an unexplained male/female ratio in Leigh's syndrome of 1.83/1, and a significant excess of male-male siblings. An X-linked form of Leigh's syndrome would explain the excess of males, and may account for some of the clinical and biochemical heterogeneity.

Brain↗
PubMed1992

Neuropathologic findings in cortical resections (including hemispherectomies) performed for the treatment of intractable childhood epilepsy.

Despite the use of hemispherectomy in the treatment of medically refractory seizures since the early 1950's, few studies published have documented neuropathologic findings in the resected specimens. This report describes the neuropathologic findings in 38 children who underwent either hemispherectomy or multilobar cortical resection as treatment for medically intractable epilepsy between 1986 and 1990. Examination of the resected specimens revealed a variety of abnormalities which fell into four broad categories. Malformations or hamartomatous lesions were the dominant finding in 15 patients, whereas encephalomalacic lesions were the most prominent abnormality in 16; chronic pathogen-free encephalitits (Rasmussen's encephalitis) was present in 3 and an additional 3 children had Sturge-Weber-Dimitri syndrome. There were no gross or microscopic abnormalities in 1 patient. This report provides the first comprehensive description of the pathologic findings in a series of children with refractory epilepsy of varying types treated by hemispherectomy-multilobar resection.

Brain Ischemia↗
PubMed1994

Computerized electroencephalographic assessment of congenital brain infarction.

We studied 12 children (8 female and 4 male) aged 2.2-14.3 years, whose computed tomographic (CT) examination had shown evidence of malacic and/or porencephalic outcomes of early vascular brain infarction. Topographic spectral electroencephalographic (EEG) analysis was performed in all patients in the awake state. The following spectral EEG variables were studied: topography, absolute and relative power of delta, theta, alpha, beta bands, overall power, and peak alpha frequency asymmetries. The results of topographic spectral EEG analysis were compared with the localization and nature of lesions as detected by CT scans. Depending on the nature of the lesions, we were able to identify two different spectral patterns. Porencephalic cysts were characterized by an increase in delta and theta bands in the areas surrounding the lesion sites, as identified by CT. Spectral EEG patterns of malacic outcomes resulted in a focal increase in theta and delta band power, corresponding to the topography of lesions. Moreover, in 9/12 subjects an asymmetry of alpha rhythm in occipital leads was found homolaterally to the lesion sites, associated with a decrease in power, without any CT evidence of an occipital lesion.

Adolescent↗
PubMed1977

Bilateral symmetrical softening of the thalamus.

The clinical and pathological data of a 48 year old patient who survived 40 days are reported. Her disturbance of consciousness corresponded to an apallic syndrome, which 12 days later bacame akinetic mutism. Symmetrical softening involving the medial thalamic nuclei from the plane of the corpora mamillaria to the red nuclei was found. The ischemic lesion might be explained by transient circulatory collapse combined with hypoplasia of the vertebrobasialr arteries. On the EEG slight irregular alpha activity was recorded (alpha coma) and external stimuli elicited theta-delta waves (paradox activation). A survey of the literature of akinetic mutism is included and the correlation between non-hypnoid unconsciousness and EEG is discussed.

Akinetic Mutism↗
PubMed1983

CT response of brain parenchyma to intravenous contrast injections.

Pre- and postcontrast scans of the same normal brain tissue layer were compared in 20 patients in order to evaluate postcontrast density response. The original purpose of this investigation was to explain a paradoxical phenomenon of density decrease, which has been regularly observed in our patients. Control phantom measurements revealed that the decrease of density values is a function of the time interval between the analysed and the previous scan. Prolonged interscan interval--due to contrast injection--enables a change in the sensitivity of CT detectors to be registered. It is probable that any study of cerebral blood volume could be unfavourably influenced by this phenomenon. Comparison between the postcontrast brain scan density decrease and density decrease of a phantom showed substantially no difference, so that we estimate the contrast response of the brain parenchyma in toto as insignificant. Further, a comparative analysis between contrast response of the white and gray matter has been made. The gray-white matter difference increased from precontrast 4.56 H to postcontrast 7.82 H. The relatively high difference of 3.26 H between the white and gray matter enhancement compared with negligible enhancement of the brain slice as a whole leads us to the hypothesis, that an absolute decrease of white matter density, as a biological reaction to the injected contrast solution, has to be presumed.

Adolescent↗
PubMed1980

Radiological Diagnosis of Periventricular and Subcortical Leukomalacia.

Nine newborn infants with histories of perinatal asphyxia are presented. The pneumoencephalographic findings which led to the diagnosis are typical and constant. They include marked subcortical atrophy with rounded, dilated, and undisplaced lateral ventricles. Cystography with 3 cc of air demonstrated multiple subcortical and pareventricular cavities, without communication with the ventricular system, but with the typical honeycomb appearance of paraventricular and subcortical leukomalacia described in postmortem findings. The CT findings are typical, and provide the location of the cavities as well as their density.

Birth Weight↗
PubMed1978

CT scan appearances in Leigh's disease (subacute necrotizing encephalomyelopathy).

Four cases of Leigh's disease (subacute necrotizing encephalomyelopathy), two confirmed at postmortem examination, have been investigated recently by CT scanning. All four showed the same symmetrical areas of low attenuation in the basal ganglia, especially the putamina. These areas corresponded with the typical areas of necrosis seen in this disease. There was also some evidence of atrophic change in the posterior fossa in some cases. This CT scan appearance may be diagnostic in the appropriate clinical context.

Brain↗
PubMed1991

Concentrations of fumonisin B1 in feeds associated with animal health problems.

Ninety-eight samples of feeds associated with 44 cases of equine leukoencephalomalacia (ELEM) and 83 samples of feed associated with 42 cases of a porcine pulmonary edema syndrome (PPE) were analyzed for fumonisin B1 (FB1). For comparison purposes, 51 feed samples not associated with PPE or ELEM were also analyzed. Feed associated with ELEM contained FB1 ranging from less than 1 microgram/g to 126 micrograms/g with 75% of the cases having at least 1 sample above 10 micrograms/g. Feeds associated with PPE ranged from less than 1 microgram/g to 330 micrograms/g with 71% of the cases having at least 1 sample greater than 10 micrograms/g. Quantitation was by high performance liquid chromatography (HPLC)/fluorescence using the fluorescamine derivative with confirmation by thin layer chromatography (TLC) and/or gas chromatography/mass spectroscopy (GC/MS).

Animal Feed↗
PubMed1989

Dexamethasone in neonatal chronic lung disease: pulmonary effects and intracranial complications.

Eighteen infants were entered into a randomized, placebo-controlled trial of dexamethasone therapy for chronic lung disease. Initial ventilation requirements were similar in the two groups, although all infants were in headbox oxygen on entry to the trial. The dexamethasone-treated infants showed a significantly more rapid improvement during the 1st week of treatment, although the overall duration of oxygen therapy was similar in both groups. Cranial ultrasound examination revealed new periventricular abnormalities in three out of the five dexamethasone-treated infants who had previously normal scans, compared with none of four similar placebo-treated infants. A large trial, focussing on potential complications, is now needed.

Administration, Oral↗
PubMed1978

Congenital lactic acidosis due to pyruvate carboxylase deficiency: absence of an inhibitor of TPP-ATP phosphoryl transferase.

Two children are described who suffered from episodes of metabolic acidosis and progressive mental and motor deterioration. The patients showed periodic elevation of blood lactate, pyruvate and alanine, which was accompanied by vomiting, hypotonia or convulsions. The concentrations of lactate and pyruvate in cerebrospinal fluid were found to be increased. Liver biopsies revealed a decrease in pyruvate carboxylase activity and normal pyruvate decarboxylase activity. No inhibitor of TPP-ATP phosphoryl transferase was detected in urine from the patients. These findings suggest that congenital lactic acidosis due to pyruvate carboxylase deficiency is probably a different disease entity from Leigh's encephalomyelopathy. A possible mechanism of brain damage caused by a defect in pyruvate carboxylase is postulated.

Acidosis↗
PubMed1992

The implications of naturally occurring levels of fumonisins in corn for human and animal health.

Contamination of corn with the fungus Fusarium moniliforme and its secondary metabolites, the fumonisins, has been associated with several human and animal diseases. This paper summarizes present knowledge and presents new data on the levels of fumonisins present in foods and feeds associated with these diseases as well as in commercial corn and corn-based products. The doses of fumonisins to which humans and animals consuming these products would be exposed are compared with those doses known to produce LEM in horses and hepatocarcinogenesis in rats. It is concluded that the known naturally occurring levels of fumonisins present a potential threat to human and animal health and realistic tolerance levels need to be set.

Animal Feed↗
PubMed1992

Fumonisin B1 production and vegetative compatibility of strains from Gibberella fujikuroi mating population 'A' (Fusarium moniliforme).

We examined 25 strains of Fusarium moniliforme from eight states known to be associated with equine leukoencephalomalacia, a disease caused by the mycotoxin fumonisin B1. We determined the mating population, mating type, and vegetative compatibility group to which each of these strains belonged. All 25 strains were in the 'A' mating population; 12 were A+ and 13 were A-. Seventeen of the 25 strains were female fertile; these strains also averaged higher levels of fumonisin B1 production than did the strains that were female sterile. Nitrate non-utilizing (nit) mutants were generated in all 25 strains and each strain was assigned to a unique vegetative compatibility group based on the inability of the derived nit mutants to form a prototrophic heterokaryon with complementary nit mutants derived from any of the other strains examined. From these data, we concluded that the production of fumonisin B1 is a general characteristic of strains from the 'A' mating population of Gibberella fujikuroi associated with equine leukoencephalomalacia, since all 25 of the isolates that we examined were genetically distinct individuals.

Animals↗
PubMed1992

AAL toxins, fumonisins (biology and chemistry) and host-specificity concepts.

The AAL toxins and the fumonisins (FB1 and FB2) are structurally related and produced respectively by Alternaria alternata f.sp. lycopersici and Fusarium moniliforme. AAL toxin is characterized as a host-specific toxin, toxic to tomato, whereas fumonisin B1 causes equine leukoencephalomalacia. FB1 and FB2 were biologically active in susceptible tomato tissue (Earlypak-7) and animal tissue culture (rat hepatoma H4TG and dog kidney MDCK). Conversely, AAL toxin was also active in the rat and dog tissue culture cells. Both fungi produce toxin/s in culture that causes death in rats; these toxins are other than AAL and fumonisin. The peracetylated derivatives of AAL and FB1 are biologically inactive in both the tomato bioassay and the animal tissue culture systems. Acetylation of the amine renders AAL inactive. The hydrolysis product of AAL (phentolamine) is toxic to the susceptible tomato line whereas the phentolamine of fumonisin is not. AAL and FB1 can be analyzed by Continuous Flow Fast Atom Bombardment (CFFAB) and Ionspray Mass Spectrometry (ISM), both sensitive to the picomole range. The N-acetyl of the TFA hydrolysis product of AAL and FB1 is determined by comparing the fragment ions at m/z 86 and 140 for FB1 and 72 and 126 for AAL.

Alternaria↗