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PubMed1993

Herpesviruses in chronic encephalitis associated with intractable childhood epilepsy.

Herpesviruses (especially cytomegalovirus [CMV] and Epstein-Barr virus [EBV]) have been implicated in the pathogenesis of chronic (Rasmussen) encephalitis associated with epilepsy. To assess the presence of herpesvirus genes in brain tissue from epileptic children with chronic (usually Rasmussen type) encephalitis, DNA was extracted from surgically resected brain tissue and studied by the polymerase chain reaction using primers specific for CMV, varicella zoster virus, herpes simplex virus, EBV, and human herpesvirus 6 genes. By this technique evidence for the presence of low levels of CMV and EBV genes was detected in most brain specimens from encephalitis patients and in several brain specimens from patients without encephalitis (eg, cortical dysplasia, gliosis, or encephalomalacia) who also had intractable epilepsy. Occasionally, both EBV and CMV genes were found in the same brain. Signal strength for both CMV and EBV was much lower in epileptic brains than in the brains of acquired immunodeficiency syndrome patients with CMV encephalitis or brain lymphoma. We found evidence for infection of the brain by human herpesvirus 6 in only one patient with encephalitis. Polymerase chain reaction technology applied to resected brain tissue from epileptic patients may provide evidence for or against viral-mediated pathogenesis of Rasmussen encephalitis or other types of encephalitis. The small amounts of EBV and CMV genes found suggest that herpesvirus infection of the brain does not directly cause Rasmussen encephalitis.

Autoradiography↗

PubMed1996

Fumonisins, mycotoxins of increasing importance: their nature and their effects.

The fumonisins (FBs) are a group of closely related mycotoxins that are prevalent in maize. They were isolated from strains of Fusarium moniliforme (Sheldon), which were implicated in the aetiology of human oesophageal cancer in the Transkei, South Africa. Their discovery explained the cause of equine encephalomalacia, or "hole in the head" syndrome, when it was found by feeding trials in horses that they elicited the disease. Subsequently, they were found to cause hepatic cancer in rats and pulmonary oedema in pigs, with most animal species tested showing liver and kidney damage. FB1 is the most important of the group and, although poorly absorbed from the gastrointestinal tract, its action is at the cellular level, affecting sphingolipid metabolism. Ceramides derived from sphingosine metabolism are cell regulatory factors affecting, among other things, DNA synthesis. Because FB1 has a close molecular resemblance to sphinganine, it interferes with ceramide biosynthesis and, hence, the processes that it regulates, which is thought to explain its carcinogenic properties. Studies on the FBs are still at a relatively early stage, but it is already clear that they play an important role in animal mycotoxicoses and, by implication, in human disease. A more positive aspect is that they will be used in elucidating the role of sphingolipids in cellular regulation.

Animal Diseases↗

PubMed1985

Pathology of nerve agents: perspectives on medical management.

Recent investigations at several institutes have demonstrated extensive acute brain pathology in experimental animals exposed to single subcutaneous doses of the chemical warfare nerve agents soman or sarin. Lesions include neuronal and neurophil degeneration, necrosis, and in animals that survive for several weeks, a degenerative encephalopathy characterized by mineralization, encephalomalacia, atrophy, and hydrocephalus. The cerebral cortex, amygdaloid complex, hippocampus, and multiple thalamic nuclei are consistently affected. This pattern of injury resembles that described for epilepsy and ischemic brain injury. Some animals have cardiac lesions characterized by acute necrosis with subsequent mild inflammation and fibrosis. Anticonvulsants protect experimental animals from lesion development. In rats, this encephalopathy causes long-range behavioral changes characterized by hyperactivity during routine handling and variances in traditional behavioral tests. The histopathologic features and distribution of lesions in nerve agent-poisoned animals support the hypothesis that epileptiform seizure activity is a major factor in nerve agent pathology. Other localized insults such as ischemia and hypoxia probably contribute to the pathogenesis.

Animals↗

PubMed1987

Late neuropathological consequences of strangulation.

A case of a young man who was a victim of strangulation is presented. He arrived at the hospital in refractory status epilepticus, controlled only with intravenous pentobarbital. The initial CT scan showed mild cortical edema. Two days later, a CT scan showed diffuse cortical swelling and bilateral basal ganglia infarcts. Upon discontinuation of pentobarbital therapy, his neurological examination revealed spontaneous ventilation and a gag reflex. A CT scan 4 weeks after the insult demonstrated hypodensities in both cerebral hemispheres and hydrocephalus. EEG was isoelectric throughout his hospitalization. He survived nearly 5 months and succumbed to pneumonia. Neuropathological examination demonstrated severe encephalomalacia, multiple cystic infarcts and generalized compensatory ventriculomegaly. Microscopic examination was particularly remarkable for a pronounced gemistocytic astrocyte proliferation in the white matter. This case illustrates the long-term neuropathological consequences of severe, global hypoxia/ischemia and the paucity of intact brain required to maintain a persistent vegetative state.

Adult↗

PubMed1991

Anomalous origin of the common carotid arteries as a cause of stroke.

A 45-year-old patient presented with symptoms caused by an encephalomalacia in the right medial cerebral artery. Angiography showed the common carotid arteries to originate just above the aortic valves. This unique vascular anomaly can be explained by current views on the development of the aorta and aortic arch arteries. The anomaly may be a risk factor for atherosclerotic obstruction of the carotid arteries.

Aorta, Thoracic↗

PubMed1986

A review of the sheep-multiple sclerosis connection.

This paper reviews a notion that the prevalence of multiple sclerosis is high in global areas where sheep populations are concentrated. Pilot studies are reported to serum antibodies in humans to three sheep diseases; focal symmetrical encephalomalacia (FSE), maedi visna and sarcocystis. In MS patients and controls antibodies were not found to the epsilon neurotoxin of the FSE organism, Clostridium welchii type D and to a caprine retrovirus that is closely related to maedi-visna virus. However, 34% of MS and control patients had antibodies to the protozoan parasite Sarcocystis spp., tissue cysts of which contain a powerful neurotoxin, sarcocystin. It is suggested that epidemiological MS prevalence rates for country areas of southern Australia require further study along with an examination for the prevalence of MS in vegetarians.

Adult↗

PubMedDate not supplied

Computed tomographic analysis of bilateral cingulotomy for intractable mood disturbance and chronic pain.

Forty-two patients out of 300 who had undergone bilateral stereotactic cingulotomies were studied by means of computerized tomography (CT). The appearance showed bilateral encephalomalacia, measuring on the average 5 X 7 mm2, located in the cingulate gyrus. These induced lesions had attenuation values similar to cerebrospinal fluid and did not enhance with contrast. CT is a useful technique for initial evaluation, management, and follow up of these patients.

Affective Disorders, Psychotic↗

PubMedDate not supplied

Neuroradiographic abnormalities in congenital cytomegalovirus infection.

To determine the spectrum of neuroradiographic abnormalities associated with congenital cytomegalovirus (CMV) infection, CT brain scans of 15 infants with symptomatic infection were reviewed. The initial CT scans were abnormal in 13 patients. Abnormalities included intracranial calcifications, cortical atrophy, ventricular enlargement, subdural effusions, porencephaly and polycystic encephalomalacia. Intracranial calcifications were present in 33% of the infants. In addition, three of the 15 infants developed progressive hydrocephalus which required ventriculoperitoneal shunt placement. These cases illustrate that congenital CMV infection causes a variety of structural CNS lesions and suggest that progressive hydrocephalus may be a relatively common consequence of symptomatic congenital CMV infection.

Atrophy↗

PubMedDate not supplied

Bacterial meningitis as an etiology of perinatal cerebral infarction.

Despite significant improvement in mortality rate, survivors of neonatal bacterial meningitis experience a significant incidence of neurodevelopmental sequelae. Neuropathologic studies have demonstrated vasculitis, arachnoiditis, and ventriculitis with secondary edema and encephalomalacia. Areas of cerebral infarction, most commonly thought to be venous in origin, have been reported as well. We performed cranial computed tomographic scans on all eight neonates with bacterial meningitis admitted to our Newborn Special Care Unit within the past 36 months and demonstrated abnormalities in seven. Six of these infants were found to have large areas of infarction related primarily to major arterial vascular distributions. We suggest computed tomographic studies for all neonates with bacterial meningitis and subsequent scans at 4-6 months of age in those with abnormal neonatal scans in order to plan better for early intervention services.

Bacterial Infections↗

PubMedDate not supplied

Brain calcification in hypoxic-ischemic lesions: an autopsy review.

Calcification of ischemic lesions in a child's brain is well recognized by pathologists; however, clinicians and radiologists usually associate cerebral calcification with infections, particularly the TORCH organisms. We illustrate this phenomenon in a 5-month-old infant with extensive, calcified, multicystic encephalomalacia without evidence of a cerebral infection. In order to ascertain the incidence of cerebral calcification in pure hypoxic-ischemic lesions, we retrospectively analyzed 486 consecutive autopsies. Ninety-nine patients had histologic evidence of cerebral hypoxic-ischemic lesions and hypoxia or ischemia. Thirty-nine of these patients displayed microscopic calcification; 23 patients had slight, 12 had minor, and 4 had prominent calcifications. Prominent calcification lesions were large enough to be detected by routine radiologic methods. Correlations between degree of calcification and the underlying disease process and between the gestational age and the length of survival were not statistically significant. This study illustrates the very frequent occurrence of brain calcification in ischemic brain lesions in children. It is necessary to include this diagnosis in the differential diagnosis of cerebral calcification.

Adolescent↗

PubMedDate not supplied

Etiologies and distribution of neonatal leukomalacia.

Neonatal leukomalacia was classified into 4 groups: focal (F), widespread (W), diffuse (D), and multicystic encephalomalacia (MCE) according to the distribution of ischemic necrosis in the cerebral hemisphere. The highest and lowest values of PaCO2, PaO2, and pH and the lowest systolic and diastolic blood pressures were compared among each group and controls. The lowest PaCO2 values were significantly lower in MCE than in the F, W, and D (F + W + D) group or controls. The lowest values of systolic and diastolic blood pressures in the W and F + W + D groups were significantly lower than in the controls; therefore, hypocarbia can be an etiologic factor of MCE rather than periventricular leukomalacia. Hypotension may be closely related to a causal factor of neonatal leukomalacia.

Blood Pressure↗

PubMed1994

Brain lesions of fetal onset in encephalopathic infants with nonimmune hydrops fetalis.

Nonimmune hydrops fetalis (NIHF) comprised 79% (45/57) of all infants with hydrops fetalis at our institution over a 6-year period. Thirty-seven infants with NIHF were liveborn. One or more electroencephalograms were performed on 40% of liveborn infants (15/37); the majority (87%) were moderately to markedly abnormal, including burst suppression, lack of background, multifocal sharp waves, excessive discontinuity, and disorganization reflecting significant neonatal encephalopathies. Postmortem neuropathologic examinations were performed in 86% of infants with NIHF who died or were stillborn, 81% of whom demonstrated intrauterine brain insults including microcalcifications, cerebral and/or cerebellar hypoplasia, microcephaly, encephalomalacia, cavitary lesions, astrocytosis, polymicrogyria, and severe neuronal loss. Cranial ultrasonography failed to document the diverse pathologic lesions that were later noted on postmortem examination. Ten infants survived the neonatal period, but 6 were neurologically abnormal at the time of discharge. Infants with NIHF are at risk for antepartum brain injury, and electroencephalographic abnormalities reflect in part a fetal brain disorder. A prospective clinical study is needed to fully assess the prevalence, incidence, spectrum of central nervous system involvement, contribution of intrapartum and neonatal stress, and long-term outcome in surviving infants with NIHF.

Brain↗

PubMed1994

Monozygous twin with polymicrogyria and normal co-twin.

Monozygous twinning has been associated with brain lesions such as holoprosencephaly, hydranencephaly, multicystic encephalomalacia, and porencephaly. Polymicrogyria is rare but has been reported in autopsied cases. We present a monozygotic twin with complicating twin-twin transfusion syndrome, the donor exhibiting normal psychomotor development and the recipient psychomotor delay associated with the brain anomaly, polymicrogyria. The brain anomaly might be due to blood pressure instability or a circulatory disturbance resulting from increased coagulability due to polycythemia.

Cerebral Cortex↗

PubMed1995

Epilepsy in children with meningomyelocele.

The medical records of 89 children followed at a multidisciplinary Meningomyelocele Clinic at the Children's Clinics for Rehabilitative Services were reviewed. Almost all children in southern Arizona with meningomyelocele are followed at this clinic. Eight children (foreign nationals) were excluded because they were not eligible for neurosurgery/neurology services at the clinic. The remaining 81 children have been followed at the clinic from 0.25 to 21 years. Seventeen children (21%; age: 1.3-17 years, mean: 9.1 +/- 4.4 years; follow-up: 1.3-16 years) manifested seizures at some time during their course. All children with seizures had shunted hydrocephalus. Neonatal seizures occurred in 2 children currently not receiving medication. An additional 3 children had an acute symptomatic seizure associated with an intraventricular hemorrhage during ventriculoperitoneal shunt revision, 2 of whom later developed epilepsy. Fourteen children (17.3%) had epilepsy; 12 were taking antiepileptic drugs. Seizures were controlled on medication in 5 children. EEG abnormalities were present in 12 children (focal slowing 4, focal spikes 8, diffuse slowing 3, generalized or bilaterally synchronous spike-wave 4). Most of these children (12/14) had evidence of additional central nervous system (CNS) pathology (i.e., areas of encephalomalacia or past stroke 7, cerebral malformations 2, CNS calcifications 1, and frequent apneic spells/cardiac arrest 2). We conclude that epilepsy occurs in approximately 17% of children with meningomyelocele, and most have other CNS pathology to account for their seizures.

Adolescent↗

PubMed1995

Patterns of cerebral injury and clinical presentation in the vascular disruptive syndrome of monozygotic twins.

The prenatal histories, clinical courses, and neuroradiographic studies of 8 infants who had survived the in utero demise of a homozygous co-twin were reviewed. Three distinct modes of clinical presentation were found: (1) severe neonatal encephalopathy with seizures; (2) a more benign neonatal course with onset of seizures and profound developmental disabilities within the first 6 months of age; (3) late infantile presentation with seizures. Only the third group had milder outcomes. Neuroradiographic studies demonstrated two pathologic patterns: varying degrees of periventricular white matter infarction with migrational abnormalities observed with earlier demise of the co-twin, and multicystic encephalomalacia observed when demise occurred at or near term. Pathophysiology is uncertain and most likely multifactorial. Exsanguination injury to the survivor can occur acutely following co-twin demise, so urgent delivery may be appropriate at or near term.

Brain Diseases↗

PubMed1995

The pathophysiologic relationships between lesion pathology, intracranial ictal EEG onsets, and hippocampal neuron losses in temporal lobe epilepsy.

In temporal lobe epilepsy (TLE) lesion patients the pathology, location of intracranial ictal EEG onsets, and hippocampal neuron losses were compared. Patients (n = 63) were classified into: (1) Tumors (n = 26, e.g. astrocytomas, gangliogliomas); (2) vascular (n = 9, e.g. cavernous and venous angiomas); (3) developmental (n = 17, e.g. cortical dysplasia, heterotopias); or (4) atrophic (n = 11, e.g. cortical or white matter encephalomalacia). Other variables were; (1) the location of the temporal lesion in the mesial to lateral, and anterior to posterior plane, (2) a clinical history of an initial precipitating injury (IPI) prior to the onset of TLE (e.g. prolonged first seizure, head trauma), (3) hippocampal neuron densities, (4) focal or regional location by intracranial depth EEG of ictal onsets, and (5) seizure outcomes. Results showed that severe hippocampal neuron losses were associated with two statistically significant findings. First, patients with mesial lesions in or adjacent to the body of the hippocampus had greater neuron losses compared to mesial lesions anterior or posterior to the hippocampus (P = 0.04). Second, lesion patients with an IPI history had greater Ammon's horn (AH) neuron losses compared to those without IPI histories (P = 0.0005), and the profile of loss was similar to hippocampal sclerosis (HS). Granule cell losses correlated in a complex manner in that; 1) by regression analysis densities decreased with longer intervals of TLE (P = 0.006), (2) tumor patients with IPIs had less granule cell loss compared to those without IPIs intervals of TLE (P = 0.006), (2) tumor patients with IPIs had less granule cell loss compared to those without IPIs (P = 0.05), and (3) developmental patients with IPIs had greater granule cell loss than patients without IPIs (P = 0.009). Mesial-temporal depth EEG electrodes were the first areas of ictal activity in 15 of 16 patients (94%), and greater hippocampal neuron losses were not associated with focal mesial-temporal EEG onsets. Seizure outcomes were worse in tumor patients compared to HS patients (P = 0.01), and patients with post-resection seizures had incomplete resections of their lesions and/or hippocampi. These results indicate that in TLE lesion patients the amount and pattern of hippocampal neuron loss depends on the location of the lesion, the pathologic classification, and a history of an IPI. Further, despite variable neuron losses, in temporal lesion patients the hippocampus was nearly always involved in the genesis or propagation of the chronic seizures.

Case-Control Studies↗

PubMed2004

Neurological disorders produced by Clostridium perfringens type D epsilon toxin.

Although the epsilon toxin of Clostridium perfringens type D produces disease in many species of domestic livestock, neurological disturbance is more common and better studied in sheep. High levels of circulating toxin, especially in lambs, cause cerebral microvascular endothelial damage with disruption of the blood-brain barrier leading to a severe, diffuse vasogenic oedema and an acute or peracute clinical course to death. With lower toxin levels, or in partially immune sheep, a focal, bilaterally symmetrical encephalomalacia sometimes occurs in selectively vulnerable brain regions after a more protracted clinical course, but the pathogenesis is uncertain.

Journal Article↗

PubMed2004

Clinicopathologic findings in patients with infantile hemiparesis and epilepsy.

Infantile hemiparesis may be associated with significant morbidity and may have a profound impact on a child's physical and social development. There are little published data evaluating the clinicopathologic features of patients with infantile hemiparesis. The present study retrospectively examines these clinicopathologic features in a surgical series of 21 patients with infantile hemiparesis. The study group was comprised of 21 patients, 13 females and 8 males, ranging in age from 5 to 41 years (mean, 20 years) at the time of surgery. Hemiparesis involved the right side in 16 patients and the left side in 5 patients. Imaging studies identified porencephaly in 8 patients (38%), encephalomalacia in 5 patients (24%), focal cerebral atrophy in 9 patients (43%), ventricular dilatation in 6 patients (29%), and white matter hyperintensities in 4 patients (19%). Concomitant neurologic diseases included medically intractable epilepsy in all 21 patients and visual field defects in 11 patients (52%). Significant perinatal history included prematurity in 7 patients (33%) and cesarean section, forceps delivery, placental abruption, fetal distress, and prolonged rupture of membranes each in 1 patient (5%). The remainder of the patients had an uncomplicated perinatal course (43%). Twelve patients underwent functional hemispherectomy (57%), 8 patients underwent lobectomy (38%) and 1 patient underwent "cyst" resection (5%). Histological evaluation demonstrated lesional (corresponding to radiographic findings) tissue in 15 of the 21 cases (71%). Infarction, malformations due to abnormalities of cortical development (cortical dysplasia) and gliosis with microcalcifications were each found in 6 patients (29%). Infarction and a geographically distinct area of cortical dysplasia were found to coexist in 1 case. Histopathologic findings in the 6 cases in which excised tissue was considered nonlesional included gliosis in all 6 of the cases, hippocampal sclerosis in 2 cases (10%), and neuronal heterotopia in 2 cases (10%). An osteoma was identified in 1 patient. The most common pathological findings observed in our series were infarction and cortical dysplasia, although radiographically, infarct-related changes were the most evident. Hippocampal sclerosis was encountered in 2 patients, suggesting that a subset of cortical dysplasias and hippocampal sclerosis may be caused by an in utero ischemic event.

Adolescent↗