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At least 343 records · Page 19Linked to original sources

PubMed1995

Multicystic encephalopathy: review of eight cases with etiologic considerations.

Multicystic encephalomalacia (MCE) is a rare lesion that arises during the perinatal period. Although hypoxic-ischemic insults may be responsible for this lesion, recent evidence suggests that herpesviruses may represent another etiologic agent. To elucidate the pathogenesis of MCE, eight cases collected over a 34-year period were evaluated for destructive lesions in gray and white matter. Immunocytochemical methods, in situ hybridization and polymerase chain reaction (PCR) methodology were employed to search for herpes simplex viruses types 1 and 2 (HSV1 and HSV2), cytomegalovirus (CMV), varicella zoster virus (VZV), Epstein-Barr virus (EBV) and JC variant of papovavirus (JCV). Review of the clinical histories revealed that there had been a complicated labor and delivery in 6/7 cases. Neuropathological lesions consisted of extensive tissue destruction, neuronal loss and gliosis in hemispheric white matter, cerebral cortex, basal ganglia, thalamus, cerebellum and brainstem tegmentum. Only one case showed evidence of latent HSV infection by PCR. CMV, VZV, JCV and EBV were not detected. Arteriopathy was noted in one case. The widespread nature of the lesions and their association with perinatal ischemia suggest that severe hypoxia may be the more common etiology of MCE. Term infants appear especially susceptible to this type of cerebral damage.

Autopsy↗

PubMed1977

[Scope and limitations of cranial computerized tomography (author's transl)].

Cranial computerized tomography (CT) has revolutionized the scope of diagnostic procedures in intracranial diseases. The method is based on the quantitative registration of x-ray absorption and enables many different pathological features within the skull to be directly visualized for the first time on the tomogram. Cerebral haemorrhage and encephalomalacia can be immediatley differentiated on the basis of the totally different appearance on CT. In the case of tumours, the additional administration of the contrast medium intravenously provides enhancement of the diagnostic evidence. Atrophic processes can be diagnosed by means of CT without recourse to additional neuroradiological methods and this facilitation is of particular value in neuropaediatrics. Orbital processes can be easily spotted due to the high definiation matrix of image. The limitations of CT are based on physical conditions.

Atrophy↗

PubMed1976

Diabetes insipidus in hypoxemic encephalopathy.

Diabetes insipidus developed in two patients who experienced severe hypoxemia as a consequence of drug-induced respiratory failure. Post-mortem examination revealed generalized cerebral edema, encephalomalacia, and a profound and diffuse loss of neurons. Hypoxemia-induced diabetes insipidus may indicate that the patient is suffering from severe brain damage.

Adult↗

PubMed1978

Neonatal paroxysmal monorhythmic alpha activity.

Thirteen infants with neonatal seizures showed paroxysmal monorhythmic electrographic activity, predominantly in the alpha range (8 to 13 Hz) and localized to the rolandic cortical areas. This rhythmic discharge, which is commonly lateralized, represents an electrical seizure discharge. Such discharges may exist as the only electrographic seizure activity, but in the majority of cases (8/13) independent epileptiform discharges are observed in other cortical areas. All infants with paroxysmal monorhythmic alpha activity had clinically observed seizures. Computerized tomography performed six or more weeks after observing the electrographic abnormality demonstrated diffuse as well as localized cortical atrophy in a distribution similar to the monorhythmic alpha activity. In other cases, localized monorhythmic alpha activity was correlated on subsequent evaluations with focal neurological abnormalities (eg, hemiparesis and hemiatrophy) and a high incidence of microcephaly (83%). On the basis of these findings, we suggest that encephalomalacia may be important in the pathogenesis of paroxysmal monorhythmic alpha seizures in the neonate.

Alpha Rhythm↗

PubMed1984

Delayed hemorrhagic infarction. A cause of late neonatal germinal matrix and intraventricular hemorrhage.

Although a single perinatal or postnatal event may be directly correlated to intraventricular hemorrhage (IVH) in some infants, in other infants IVH may be related to a series of insults. Asphyxia, hypotension, and a pressure-passive low cerebral blood flow (CBF) may lead to an infarction. Subsequent events known to cause sudden rises in the CBF may then produce a hemorrhage into damaged tissues. We report two cases of this proposed model for delayed hemorrhage into infarcted tissues, or late IVH. Both neonates were severely asphyxiated, and both experienced profound hypotension and a low CBF on the first postnatal day. Late IVH was found in both neonates; at 2 to 3 months of age, one neonate was found to have computed tomographic evidence for diffuse encephalomalacia, and the other neonate was noted to have an occipital porencephalic cyst.

Cerebral Hemorrhage↗

PubMed1992

The role of dominant premotor cortex and grapheme to phoneme transformation in reading epilepsy. A neuroanatomic, neurophysiologic, and neuropsychological study.

We studied a 24-year-old man who had reading epilepsy after removal of a left frontal arteriovenous malformation. Lesion analysis by means of a neuroanatomic template placed a 2-cm region of encephalomalacia anterior to the left central sulcus in premotor cortex (Brodmann's area 6). Lexical and nonlexical reading activation tests demonstrated seizures during reading and increased discharge rates when the patient was reading aloud or silently articulating. Seizures (perceived or actual jaw clicking) were electrographically characterized by brief left frontocentral epileptiform transients. Grapheme to phoneme transformation, not linguistic complexity, appears to be the critical stimulus in some reading epilepsies. The case adds anatomic relevance to the phonologic component of reading and supports the putative role of dominant premotor cortex in activation of precise sequences of motor linguistic output in reading and writing. Reading epilepsy may be a reflex or action myoclonus syndrome localized to Brodmann's area 6 (Exner's area).

Adult↗

PubMed2000

Mental retardation and seizure disorder in Schimke immunoosseous dysplasia.

Schimke immunoosseous dysplasia (SID) is a rare, pleiotropic disorder compromising spondyloepiphyseal dysplasia, nephrotic syndrome, defective T-cell-mediated immunity, and vascular changes which can lead to cerebral infarcts. The cause is unknown but an autosomal recessive inheritance pattern has been suggested. Understanding of the clinical phenotype is evolving; however, the neurologic spectrum is not well known. We report on a 17-year-old woman who presented with behavior changes, developmental regression, and partial complex seizures in early childhood. Computed tomographic scan of the brain was normal at that time. Short stature and cognitive deficits became evident several months later. At 4 1/2 years, she developed nephrotic syndrome and later malignant hypertension. Recent magnetic resonance imaging of the brain showed focal encephalomalacia in the parietal regions and a magnetic resonance angiography documented narrowing of the middle cerebral arteries. A skeletal survey showed evidence of spondyloepiphyseal dysplasia. We have not been able to identify an immune defect. To our knowledge this is the first reported patient with SID, profound mental retardation, and a seizure disorder. This case supports the theory that an intrinsic vascular defect may be more important in the pathogenesis of SID than a T-cell-mediated immune deficit.

Abnormalities, Multiple↗

PubMed1997

Cytotoxic effects of repin, a principal sesquiterpene lactone of Russian knapweed.

Repin is the principal sesquiterpene lactone isolated from Russian knapweed (Centaurea repens), a perennial weed found in many parts of the United States. Ingestion of Centaurea repens by horses has been reported to cause a movement disorder simulating Parkinson's disease (PD) and nigrostriatal degeneration, called equine nigrostriatal encephalomalacia (ENE). To understand the mechanisms whereby ingestion of Centaurea repens induces ENE and a PD-like disorder, repin cytotoxicity was examined to explore its pathogenetic relationship to ENE and to PD. Repin was highly cytotoxic to both PC12 cells and mouse astrocytes in a dose- and time-dependent manner. The cytotoxic effects were accompanied by depletion of glutathione (GSH), a rise in the level of reactive oxygen species (ROS) and damage to cellular membranes. Although repin is a highly reactive electrophile that can readily conjugate GSH, GSH depletion may not be the sole mechanism underlying repin cytotoxicity as shown by our study using buthionine sulfoximine, in which severe GSH depletion did not result in a parallel increase in cell death. However, pre-treatment with GSH-glycoside or with lipoic acid provided significant protection from repin-induced cell death. These data suggest that oxidative stress plays a major role in repin cytotoxicity. Since oxidative stress is considered to play a major role in neuronal degeneration accompanied by depletion of mitochondrial GSH and an increase in lipid peroxides in the substantia nigra of PD, further elucidation of mechanisms of repin neurotoxicity may generate clues regarding not only the mechanisms of neuronal degeneration but also the possible role of environmental factors in the pathogenesis of PD.

Animals↗

PubMed1995

Galloway-Mowat syndrome in Taiwan.

We report on two Chinese female infants with multiple congenital anomalies: microcephaly, apparent porencephaly or encephalomalacia, developmental delay, minor facial anomalies, and contractural arachnodactyly. In the first patient, focal glomerulosclerosis was diagnosed histologically by percutaneous renal biopsy due to proteinuria with hematuria. Congenital hypothyroidism presenting with markedly low T3 and T4 was also noted. She died at age 5 months. The second patient had a very similar condition but less severe brain and kidney malformations. A variant of Galloway-Mowat syndrome is suspected.

Abnormalities, Multiple↗

PubMed1981

Progressive neurological disease associated with chronic cytomegalovirus infection.

Cytomegalovirus (CMV), the most common infectious cause of nervous system damage in the fetus and newborn infant, generally has been thought to exert its teratogenic effect during early pregnancy, and it has been assumed that clinical or postmortem abnormalities reflect static insults incurred earlier. Over the past two years we have identified two infants who presented with various clinical signs suggesting CMV infection. Serial CT head scans showed mild hydrocephalus progressing to extensive loss of brain substance and hydrocephalus in one and a normal scan going on to polycystic encephalomalacia in the other. Both infants had urine or leukocyte cultures that were repeatedly positive for CMV. These data suggest that CMV can cause progressive central nervous system damage after birth. The findings may account for some unexplained brain malformations and for a portion of the neurodegenerative disorders occurring in babies who appear normal at birth.

Brain Diseases↗

PubMed1993

Dopamine agonist treatment of antegrade amnesia from a mediobasal forebrain injury.

We studied the persistent antegrade memory impairment in a woman whose brain had been surgically impaled, leaving a 1-cm-wide mediobasal tract of encephalomalacia that extended just anterior to the septal nuclei and medial to the nucleus accumbens. In a blinded, controlled, alternating repeated-measures protocol, bromocriptine significantly improved her verbal learning, functional memory, and daily recall, perhaps by acting on neurons that had been disconnected from the ventral tegmental tract's dopaminergic inputs.

Adult↗

PubMedDate not supplied

Immediate post-dosing paralysis following severe soman and VX toxicosis in guinea pigs.

There have been numerous studies of the central nervous system (CNS) involvement in organophosphate (OP) poisoning showing status epilepticus and/or 'electrographic seizures'. Brain damage has been demonstrated as 'neuronal necrosis' primarily in the cortex, thalamus and hippocampus. To the authors' knowledge there have been no reports of partial/total paralysis following close upon OP exposure although delayed paralysis has been reported. This report summarizes the immediate, OP induced paralytic events recorded in guinea pigs during development of the Canadian reactive skin decontaminant lotion (RSDL). As part of the development work, supra-lethal cutaneous doses of OP were applied to large numbers of guinea pigs followed by decontamination with the RSDL or predecessor lotions and solvents. Soman (pinacolyl methylphosphonofluoridate; GD) challenges were applied to 1277 animals and S-(2-diisopropyl-aminoethyl) methylphosphorothiolate (VX) challenges to 108. The classic sequence of clinical signs--ptyalism, tremors, fasciculations, convulsions, apnea and flaccid paralysis before death--was seen in the 658 animals that died and in many of the survivors. Eighty-four of 688 survivors of GD and 4 of 39 survivors of VX showed random paralysis of various distal regions following recovery from an insult which produced convulsions and/or flaccid paralysis. Because the experiments were designed to assess the decontamination procedures, there were no apparent relationships between the amounts of OP applied and the sequellae recorded. The observations of paralysis were also incidental to the prime focus of the experiments. Because of this, only ten animals paralysed following GD exposure were examined for histological effects. The pathologist diagnosed 'encephalomalacia' and 'focal necrotic lesions' in the cerebral cortex and 'focal necrotic lesions' in one spinal cord. Of the 84 guinea pigs paralysed after GD challenge, one was not decontaminated and the decontaminants used on the remainder were sufficiently varied that there appeared to be no relationship between the type of decontaminant and the resulting paralysis.

Animals↗

PubMedDate not supplied

Quantitative MRI of the brain in children with sickle cell disease reveals abnormalities unseen by conventional MRI.

Conventional MRI (cMRI) has shown that brain abnormalities without clinical stroke can manifest in patients with sickle cell disease (SCD). We used quantitative MRI (qMRI) and psychometric testing to determine whether brain abnormalities can also be present in patients with SCD who appear normal on cMRI. Patients 4 years of age and older with no clinical evidence of stroke were stratified by cMRI as normal (n = 17) or abnormal (n = 13). Spin-lattice relaxation time (T1) of gray and white matter structures was measured by the precise and accurate inversion recovery (PAIR) qMRI method. Patient cognitive ability was assessed with a standard psychometric instrument (WISC-III or WISC-R). In all 30 patients with SCD, qMRI T1 was lower than in 24 age- and race-matched controls, in cortical gray matter (P < .0006) and caudate (P < .0009), as well as in the ratio of gray-to-white matter T1 (P < .008). In the 17 patients who were shown to be normal by cMRI, qMRI T1 was still lower than in controls, in both cortical gray matter (P < .02) and caudate (P < .004). Histograms of voxel T1 show that the proportion of voxels with T1 values intermediate between gray and white matter (ie, consistent with encephalomalacia) was 9% higher than controls in patients shown to be normal by cMRI (P < .05) and 15% higher than controls in patients shown to be abnormal by cMRI (P < .0005). The full scale intelligence quotient (FSIQ) of all patients with SCD was 75, compared to the FSIQ of 88 in a historical control group of patient siblings (P < .001). The FSIQ of patients shown to be normal by cMRI was 79, significantly lower than the FSIQ of patient siblings (P < .04). The FSIQ of 71 in patients shown to be abnormal by cMRI was significantly lower than both the patient siblings (P < .005) and the patients shown to be normal by cMRI (P < .04). Patients shown to be abnormal by cMRI scored lower than patients shown to be normal by cMRI, specifically on the subtests of vocabulary (P = .003) and information (P = .03). Cognitive impairment is thus significant, even in patients with SCD who were shown to be normal by cMRI, suggesting that cMRI may be insensitive to subtle neurologic damage that can be detected by qMRI. Because cognitive impairment can occur in children normal by cMRI, our findings imply that prophylactic therapy may be needed earlier in the course of SCD to mitigate neurologic damage.

Adolescent↗

PubMed1995

Isolation and identification of two potent neurotoxins, aspartic acid and glutamic acid, from yellow star thistle (Centaurea solstitialis).

Horses grazing for prolonged periods on yellow star thistle (YST), a plant which grows wild in western parts of the United States, develop an extrapyramidal disorder known as nigropallidal encephalomalacia (NPE). Attempts have been made to identify, isolate, and characterize the toxins responsible for the disease in animals. Using the organotypic tissue culture system on mouse cortical explants as a specific assay method for neurotoxicological evaluation, it has been possible to isolate and characterize two potent neuroexcitotoxic compounds, aspartic and glutamic acids, the former being the major toxic component in the alcoholic extract of the plant. There is also evidence that other neurotoxic compounds are present in the extract. The detailed procedure for isolation and characterization of these compounds is given here.

Animals↗

PubMed2001

Fetal magnetic resonance imaging (MRI) of ischemic brain injury.

The aim of the present study was to demonstrate the usefulness of fetal magnetic resonance imaging (MRI) in ischemic brain injury. We report seven cases of fetal brain ischemia prenatally suspected on ultrasound (US) and confirmed by fetal MRI. Sonographic abnormalities included ventricular dilatation (n=3), microcephaly (n=1), twin pregnancy with in utero death of a twin and suspected cerebral lesion in the surviving co-twin (n=3). MRI was performed with a 1.0 T unit using half-Fourier acquisition single-shot turbo spin-echo (HASTE) sequences between 28 and 35 weeks of gestation. US and MRI images were compared with pathologic findings or postnatal imaging. MRI diagnosed hydranencephaly (n=1), porencephaly (n=2), multicystic encephalomalacia (n=2), unilateral capsular ischemia (n=1), corpus callosum and cerebral atrophy (n=1). In comparison with US, visualization of fetal brain anomalies was superior with MRI. The present cases demonstrate that MRI is a valuable complementary means of investigation when a brain pathology is discovered or suspected during prenatal US.

Brain↗

PubMed1997

Variation in virulence in the gnotobiotic pig model of O157:H7 Escherichia coli strains of bovine and human origin.

Escherichia coli strains of serotype O157:H7 have been incriminated in outbreaks and sporadic cases of food-borne illness, including diarrhea, hemorrhagic colitis, hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Food-producing animals, particularly cattle, are believed to be reservoirs of the organism. Whether all strains of bovine origin pose human health risk is unknown and was the impetus for this investigation. We compared the virulence of ten SLT-I, SLT-II, and eae DNA probe-positive O157:H7 strains from cattle to 10 like strains associated with human diarrheal disease outbreaks for virulence in one day-old gnotobiotic pigs. All strains caused diarrhea, and only four pigs inoculated with either of two bovine strains failed to develop that condition. Signs of central nervous system disease, death, debilitation requiring euthanasia before the end of an eight day observation period, and/or encephalomalacia occurred in 32/42 pigs inoculated with the strains isolated from human beings, 13/39 pigs inoculated with strains from cattle, and 7/7 pigs inoculated with a positive control strain. More strains of human origin (9/10) than bovine origin (5/10) caused these effects. The results of this study indicate considerable variability in virulence of O157:H7 strains possessing the same known virulence determinants, and suggest that disease outbreaks tend to be caused by the more virulent of these strains.

Adhesins, Bacterial↗

PubMed1992

Orbital porencephalic cyst following penetrating orbitocranial trauma.

In a 10-year-old boy an orbitocranial penetrating wound produced by an umbrella tip caused an orbital roof bone fragment to penetrate up to the anterior part of the third ventricle behind the left foramen of Monro. Hemorrhages and encephalomalacia developed along the trajectory of the fragment and subsequently a porencephalic cyst was formed at this site. Six months after the trauma, increased pressure developed in the left ventricular system due to obstructive hydrocephalus and consequently the porencephalic cyst herniated into the orbit through the orbital roof fracture, producing intermittent diplopia, left exophthalmos, and palpebral swelling. A ventriculo-peritoneal shunt led to shrinkage of the orbital cyst content and resolution of the symptoms.

Child↗

PubMed1993

Pathologic findings in a patient with a vein of Galen aneurysm treated by staged endovascular embolization.

Vein of Galen aneurysms are uncommon malformations that can be treated with a combination of endovascular embolization and surgery. Since the relevant techniques are new and innovative, their pathologic sequelae are not yet well described. A patient treated with staged embolization over a 15-month period developed evidence of cerebral venous hypertension on angiography. At necropsy, we observed marked myointimal proliferation of the aneurysm wall, dura, and cerebral vessels in addition to severe widespread encephalomalacia, calcinosis, and rare mural thrombi. These vascular changes have not been previously described in cases of vein of Galen aneurysm, and may, in part, be a consequence of embolization therapy.

Cerebral Veins↗