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PubMed2004

Characterization of the role of glutathione in repin-induced mitochondrial dysfunction, oxidative stress and dopaminergic neurotoxicity in rat pheochromocytoma (PC12) cells.

Repin, a major constituent in extracts of the plant Centaurea repens is thought to be the active principal responsible for the development of equine nigropallidal encephalomalacia (ENE), a fatal Parkinson-like neurodegenerative disorder in horses. Although the exact mechanism by which ingestion of this weed causes ENE is uncertain, a limited body of experimental evidence suggests a critical role for the glutathione redox system. In the present study, the mechanism of repin neurotoxicity was examined in PC12 cells with a focus on determining the role of glutathione (GSH) in repin-induced mitochondrial dysfunction, oxidative stress and dopaminergic toxicity. The results demonstrate that repin reduced both cellular GSH levels and mitochondrial function in a manner that was time- and concentration-dependent. The repin-induced changes in GSH levels were found to precede the changes in mitochondrial function. Depletion of GSH with a potent GSH depletor (ethacrynic acid (EA)) and a GSH synthesis inhibitor (buthionine sulfoximine (BSO)) prior to repin treatment enhanced the repin-induced mitochondrial change. In addition, repin caused a concentration-dependent decrease in cellular dopamine levels in NGF-differentiated PC12 cells. Increases in intracellular GSH levels induced by pre-treatment with reducing agents (N-acetyl-L-cysteine or reduced glutathione) completely protected the cells from repin-induced mitochondrial and dopaminergic toxicity. Antioxidants, coenzyme-Q and ascorbic acid completely blocked repin-induced dopaminergic toxicity. These data suggest that GSH plays a critical role in repin-induced neurotoxicity and that the maintenance of neuronal redox status may prove to be a useful strategy for the prevention and/or treatment of ENE. The results support the view that GSH depletion, leading to oxidative damage and subsequent mitochondrial dysfunction, may serve as a trigger for neuronal cell death.

Animals↗

PubMed2004

Magnetic resonance imaging in 122 children with spastic cerebral palsy.

The interrelationship between magnetic resonance imaging findings, types of cerebral palsy, and gestation was studied. We analyzed the magnetic resonance imaging of brain in 122 children with spastic cerebral palsy. Forty-three patients had spastic hemiplegia, 61 had spastic diplegia, and 18 had spastic tetraplegia. Magnetic resonance imaging abnormalities were observed in 75% of patients. Periventricular leukomalacia accounted for 66% of abnormalities observed in patients with spastic diplegia; other types of brain lesions were uncommon. In patients with spastic tetraplegia, two types of magnetic resonance imaging abnormalities predominated: congenital brain anomalies and term-type brain injuries, 42% and 33% respectively. Types of magnetic resonance imaging abnormalities were more heterogeneous in patients with spastic hemiplegia. Preterm brain injuries (periventricular leukomalacia and posthemorrhagic porencephaly) were observed often in patients born at preterm but were also observed in patients born at term. Term-type brain injuries (term-type border-zone infarct, basal ganglia-thalamic lesion, subcortical leukomalacia, and multicystic encephalomalacia) were observed only in patients born at or near term. We conclude that magnetic resonance imaging findings for patients with spastic cerebral palsy were closely related to types of cerebral palsy and gestation at birth. Magnetic resonance imaging in patients with perinatal brain injury may reflect pathologic changes and is useful in understanding and evaluating cerebral palsy.

Cerebral Palsy↗

PubMed2003

Resolution of eating disorders after right temporal lesions.

We report two patients whose eating disorder resolved after right temporal lobe lesions. The first case report involves a woman with a history of bulimia nervosa and partial seizures arising from the occipital and right temporal regions. The second case is a woman with a history of anorexia nervosa that resolved after a head injury that resulted in right-sided inferofrontal and temporal encephalomalacia. Not only did both patients' eating disorders resolve, but their moods and libidos improved.

Adult↗

PubMed2003

Elective extracorporeal support for complex tracheal reconstruction in neonates.

BACKGROUND: Congenital obstructive anomalies of the trachea present unique challenges in reconstruction and perioperative airway management. Complications include anastomotic breakdown, leak and granulation formation related to the complexity of the repair, and difficulties with perioperative airway management. We describe our technique of elective intraoperative and postoperative extracorporeal support to improve surgical exposure and postoperative healing. METHODS: We have performed complex tracheal reconstructions in 4 newborns (2.2 to 4.3 kg) for long segment tracheal stenosis and complete tracheal rings, diagnosed with bronchoscopy and computerized tomography. Three of the 4 infants had other significant anomalies including complex congenital heart disease, hydrocephalus, encephalomalacia, left lung agenesis, facial anomalies, vertebral anomalies, and hand and hip anomalies. The repairs were performed through a median sternotomy using an extracorporeal membrane oxygenation circuit for support. Venoarterial support was used for the sliding tracheoplasty reconstruction. Extracorporeal membrane oxygenation was converted to venovenous for postoperative "airway rest." After diuresis, the lungs were reexpanded and the 4 patients were ventilated and removed from extracorporeal membrane oxygenation at 4, 5, 8, and 9 days postoperatively. Bronchoscopy was performed to evaluate the airway. RESULTS: All patients had excellent healing of the trachea without granulation tissue. There were no complications of extracorporeal membrane oxygenation support or bleeding issues. All 4 patients survived the surgery and immediate postoperative period with 2 late deaths. The child with congenital heart disease expired after 8 weeks after having hepatorenal failure develop. The child with Goldenhar's syndrome and a single left lung died after 5 months in the hospital. The other 2 patients survived. Two of the infants required late tracheostomy for facial and laryngeal anomalies. CONCLUSIONS: Extracorporeal membrane oxygenation provides an excellent environment for complex tracheal reconstruction and promotes postoperative healing by minimizing trauma to the reconstructed airway.

Congenital Abnormalities↗

PubMed1993

A clinical-pathological study of nonsurvivors of newborn ECMO.

Extracorporeal membrane oxygenation (ECMO) is an important means of supporting newborns with respiratory failure. While short- and long-term follow-up of ECMO survivors has been thoroughly addressed, there is no systematic study of nonsurvivors. Nineteen nonsurvivors of newborn ECMO with autopsy results are divided into two groups: group 1: 12 patients who had intracranial lesions as the primary cause of death (hemorrhage 8, encephalomalacia 2, infarct 2); and group 2: 7 patients with nonintracranial primary causes of death. Patients in group 1 were significantly more acidotic, hypotensive, and smaller in age and birth weight pre-ECMO. Among group 2 patients, two with diaphragmatic hernia died of primary pulmonary disease (diffuse alveolar damage, pulmonary hypoplasia and necrosis, bronchopneumonia). One of 2 patients with persistent fetal circulation (PFC) was treated with massive doses of tolazoline and suffered fatal gastrointestinal hemorrhage and ischemic necrosis of heart, spleen, testes, and adrenals. The other PFC patient had severe pulmonary interstitial fibrosis. Two patients with meconium aspiration and a patient with streptococcal sepsis had diffuse pulmonary damage and multiple organ failure (renal medullary necrosis, and infarcts of adrenal, spleen, liver). In this series, intracranial pathology was the most common cause of death in ECMO patients, related to gestational age, acidosis, hypoxia, and size, but probably unrelated to carotid ligation.

Acidosis, Lactic↗

PubMed1987

Precocious puberty after traumatic brain injury.

After traumatic brain injuries in 33 prepubertal children, precocious puberty was observed in seven. Precocious puberty developed significantly more frequently in girls than in boys (54.5 versus 4.5%, P less than 0.01). Six children with precocious puberty were in coma for greater than or equal to 2 weeks. Follow-up computed tomography revealed cerebral atrophy or focal encephalomalacia in all children with and 69% of children without precocious puberty. There were no striking differences in incidence of motor or cognitive deficits or posttraumatic epilepsy in children with and without precocious puberty. In four of five children, basal sex steroid levels were elevated, and the response to luteinizing hormone releasing hormone stimulation revealed a pubertal pattern after the appearance of secondary sex characteristics.

Brain Injuries↗

PubMed1993

The high-risk infant.

A large number of infants are born each year with biologic or environmental risk factors that put them at increased risk for developmental disability, although most do not go on to have major disabilities. Some risk factors, for example, intraparenchymal hemorrhage, periventricular cysts, encephalomalacia, and abnormal neurodevelopmental examination, carry a much higher risk of developmental disability than others. There is much overlap among risk factors, and infants with multiple risk factors generally have a greater risk of disability than infants with just a single risk factor. All high-risk infants should receive careful pediatric follow-up that includes developmental screening, but efficient use of so far quite limited resources argues for selection of the highest risk infants for comprehensive developmental follow-up or early intervention programs. A system of tracking and monitoring high-risk infants during infancy and childhood would allow for early identification of developmental delay and appropriate referral for community resources.

Child, Preschool↗

PubMed1997

Giant cell arteritis in association with cerebral amyloid angiopathy: immunohistochemical and molecular studies.

Giant cell arteritis (GCA) usually manifests as a transmural vascular infiltrate of mononuclear and multinucleated giant cells (MNGC). We describe six patients with GCA associated with severe cerebral amyloid angiopathy (CAA), all with cerebral hemorrhage or varying degrees of cerebral infarct, and histological evidence of Alzheimer's disease (cortical CAA often predominating over senile plaques and neurofibrillary tangles). One case showed mostly cortical involvement (with old microhemorrhages), and the others were primarily leptomeningeal (with involvement of the underlying cortex and extensive encephalomalacia of adjacent brain). Many vessels with CAA exhibited a pronounced adventitial and perivascular infiltrate of lymphocytes, histiocytes, and MNGC. Immunohistochemical staining showed deposition of beta/A4 peptide primarily in the thickened media of CAA vessels, and within the cytoplasm of MNGC--suggesting phagocytosis of insoluble peptide. Cystatin C antibody stained vascular amyloid and diffusely highlighted astrocytic and MNGC cytoplasm. HAM56-positive macrophages were frequently seen around amyloid-laden vessels. Anti-smooth muscle actin immunohistochemistry suggests the occurrence of medial destruction by amyloid, with relative preservation of intimal cells. Ultrastructural studies performed in one case confirmed the presence of intracytoplasmic amyloid in MNGC. The GCA seen in these cases of CAA most likely represents a foreign body response to amyloid proteins, causing secondary destruction of the vessel wall. DNA from brain tissues of five affected patients was examined to assess whether mutations were present in exon 17 of the APP gene or exon 2 of the cystatin C gene, a finding that might explain the foreign body giant cell response to amyloid proteins in these cases. However, restriction fragment mapping of amplified gene segments showed that previously described mutations were not present in these cases.

Actins↗

PubMed2002

Histopathology of brain tissue from patients with infantile spasms.

This chapter reviews the patterns of neuropathologic abnormality encountered in cortical resections performed for the treatment of infantile spasms (ISS) in the broader context of the larger "universe" of neuropathology seen in the central nervous system of infants with this syndrome as well as older children with intractable epilepsy. Although destructive lesions (encephalomalacia), Sturge-Weber-Dimitri syndrome, and even neoplasms can cause ISS, its most common neuropathologic substrate is cortical dysplasia (CD), representing the result of aberrant neuronal migration to the neocortex from the germinal matrix. Ways to recognize CD morphologically and assess its severity are reviewed, as are special stains and immunohistochemical methods that may be useful in confirming the diagnosis. The similarity and relationship between (sporadic) CD and tubers of tuberous sclerosis are discussed. Future potentially fruitful research directions aimed at refining the clinicopathologic analysis of the role of CD in ISS and epilepsies of older children are briefly considered.

Brain↗

PubMed1986

Quantification of cerebral oxygenation and haemodynamics in sick newborn infants by near infrared spectrophotometry.

New apparatus was made whereby indices of cerebral oxygenation and haemodynamics in sick newborn infants could be quantified by near infrared (NIR) spectrophotometry and displayed instantaneously at the cotside. The indices included oxygenated haemoglobin, reduced haemoglobin, oxidised cytochrome aa3, and total haemoglobin concentration: cerebral blood volume, mixed cerebral venous saturation, and changes in cerebral blood flow were then derived. Striking changes were observed in response to alterations in arterial oxygen saturation and carbon dioxide tension and to tilting of the infant. Abnormal responses were detected in cerebral oedema following birth asphyxia, patent ductus arteriosus, and cystic encephalomalacia. NIR spectrophotometry provides valuable quantitative data at the cotside for the management of sick infants and for exploring the pathophysiology of damage to the brain.

Blood Volume↗

PubMed1997

Outcome analysis of the transglabellar/subcranial approach for lesions of the anterior cranial fossa: a comparison with the classic craniotomy approach.

The classic approach to anterior skull base lesions uses bifrontal craniotomies together with lateral rhinotomies. This approach requires frontal lobe retraction and is associated with postoperative anosmia and the development of frontal lobe encephalomalacia. The transglabellar/subcranial approach permits removal of anterior skull base lesions without frontal lobe retraction and avoids facial scars. No studies to date, however, have directly compared the two approaches in terms of patient morbidity. The present retrospective study compares the two approaches when used for the removal of anterior skull base lesions in terms of estimated blood loss, number of transfusions, number of days in the hospital and intensive care unit, and postoperative complications. Twenty patients with anterior skull base lesions were examined. The classic approach was used on 10, and the transglabellar/subcranial route was used on 10. When compared with the classic approach, the transglabellar/subcranial approach resulted in a lower estimated blood loss and subsequent transfusion rate, fewer days in the hospital and intensive care unit, and lower numbers and less severe types of complications. Furthermore, visualization of the tumors before resection with the transglabellar/subcranial approach allowed preservation of olfaction in virtually all of these patients. Although this study represents a small sample population, the results are sufficiently impressive to favor the transglabellar/subcranial approach for the removal of a variety of anterior skull base lesions.

Adult↗

PubMed1994

Cardiomyopathy in childhood and adult life, with emphasis on hypertrophic cardiomyopathy.

Over 60 entries in the genetic catalog have cardiomyopathy features--32 autosomal dominant, 35 autosomal recessive and X-linked. Over 40 present in, or can have survival into, adult life. Major clinicopathologic categories of these cardiomyopathic disorders included: sudden death (13 entities); cardiac conduction disturbance important feature; associated myopathy or motor dysfunction; storage diseases with cardiac involvement; cardiac amyloidoses; and, other categories. Genes, abnormality of which can cause hypertrophic cardiomyopathy (HCM), have been identified on chromosomes 1, 14 and 15, the locus on chromosome 14 involving the B-myosin heavy chain gene, but at least one unidentified locus is known to exist and there is a suggestive locus on chromosome 16, so that HCM is not a single disease but a group of disorders with clinicopatholopic similarities. To investigate these aspects of HCM in some detail, sixty-six patients with "sharply demarcated" differential myocardial fiber bundle hypertrophy (DMBH), considered to be of significant degree, from a pediatric autopsy data base of approximately 8,000 cases, were reviewed. Twenty-three of the patients died suddenly, without antecedent significant cardiac dysfunction, seven had clinical congestive heart failure of varying duration, three were stillborn, six showed evidence of aspiration of amniotic sac content (three had history of fetal distress), five had ischemic bowel disease, three (two with clinical cerebral palsy and one with Ondine's curse syndrome) had cerebral atrophy and sclerosis and one had extensive more acute encephalomalacia, and a variety of other major "causes of death" were present. Whether all infants and children with DMBH meeting the criteria used, who do not have congenital heart disease, have dominant hypertrophic cardiomyopathy (HCM) cannot be established by studies of this type, but the "concentration" of a gene or genes for HCM in pediatric autopsy series because the strong effect of HCM on life expectancy is relevant to this possibility. The data raise the question that stillbirth, fetal distress with aspiration of amniotic sac content, ischemic bowel disease and cerebral atrophy and sclerosis may be hitherto underappreciated features of HCM in childhood, and that patients with HCM may be peculiarly liable to die with certain types of septic shock, such as acute meningococcemia. In the material of this study, sudden death was statistically more frequent in females than in males in childhood (p < .029).

Adolescent↗

PubMed1997

Acute confusional migraine and migrainous infarction in childhood.

We report two children with acute confusional migraine (ACM) and another with migrainous infarction (MI), aged 7-12 years. There was a family history of migraine in all patients. The patients, who were all right-handed, all manifested sudden onset of consciousness disturbance and other neurological deficits as the first aura in their life. The symptoms in all cases almost completely resolved spontaneously within 24 h, but transient occipital slowing on EEG with laterality corresponding to the side of migrainous origin lasted more than 24 h. In the cases of ACM in the critical phase, although MRI and MR angiography showed no abnormal findings, IMP-SPECT performed within 48 h of migraine attacks revealed a regional change in cerebral blood flow, which is one particular case demonstrated hypoperfusion in the left posterior cerebral artery (PCA) territory. Therefore, although ACM was diagnosed clinically by exclusion, SPECT was thought helpful for the diagnosis of ACM. We speculated that transient hypoperfusion affecting the dominant-sided PCA territory involving the medial temporal structures was responsible for the confusion with amnesia in ACM, in contrast to the lack of confusion or amnesia in the case of MI showing cystic encephalomalacia in the right thalamic and hippocampal regions.

Acute Disease↗

PubMed1999

A patient with cerebral palsy whose mother had a traffic accident during pregnancy: a diffuse axonal injury?

A 16-year-old girl had spastic cerebral palsy (CP) with triplegia and focal epilepsy. The patient's past history included her mother's lower abdominal trauma caused by a traffic accident at the 7th month of gestation. Brain examination with magnetic resonance imaging (MRI) revealed encephalomalacia at the bilateral parieto-temporal lobes and the left caudate nucleus, segmental narrowing of the splenium of the corpus callosum, dilatation of the left lateral ventricle and an abnormally high intensity at the right posterior portion of the internal capsule. These findings might indicate a diffuse axonal injury (DAI), but not an asphyxic brain damage. In this patient, CP might be caused by an intrauterine DAI when her mother was involved in the accident.

Accidents, Traffic↗

PubMed2001

Carbamyl phosphate synthetase 1 deficiency: a destructive encephalopathy.

Carbamyl phosphate synthetase I is a urea cycle enzyme. Severe deficiency of carbamyl phosphate synthetase I presents in the neonatal period as hyperammonemic encephalopathy with altered consciousness and occasional seizures after feeding begins. Episodes of altered consciousness with or without seizures and focal neurologic deficits are seen later with patients of partial carbamyl phosphate synthetase I deficiency. Fatal cerebral edema with brain herniation may develop on occasion. Three patients presenting with carbamyl phosphate synthetase I deficiency are reported with neuroimaging and pathologic findings illustrating the destructive encephalopathy with acute cerebral edema, followed by diffuse cerebral atrophy and occasional cystic encephalomalacia. The deterioration in carbamyl phosphate synthetase I deficiency occurs during the hyperammonemic crises. This deficiency may be difficult to treat despite the current advances in treatment strategies, especially in neonatal-onset patients with low carbamyl phosphate synthetase I activity.

Atrophy↗

PubMed2001

Cerebral blood flow velocity in two patients with neonatal cerebral infarction.

Cerebral blood flow velocity was measured in the middle cerebral artery of two patients who exhibited unilateral neonatal cerebral infarction during the neonatal period. Doppler studies demonstrated increases in cerebral blood flow velocity but decreases in the resistance index on the affected side of the middle cerebral artery in the neonate who developed hemiplegia with cystic encephalomalacia, although the neonate with normal neurologic outcome exhibited symmetric cerebral blood flow velocity and resistance index. The asymmetry in cerebral blood flow velocity measurements of both middle cerebral arteries may be useful to evaluate the severity of brain damage and predict the neurodevelopmental prognosis of unilateral neonatal cerebral infarction.

Blood Flow Velocity↗

PubMed1998

Neuroleptic malignant syndrome and methylphenidate.

A 1-year-old female presented with neuroleptic malignant syndrome probably caused by methylphenidate. She had defects in the supratentorial brain including the basal ganglia and the striatum (multicystic encephalomalacia) due to severe perinatal hypoxic-ischemic encephalopathy, which was considered to be a possible predisposing factor causing neuroleptic malignant syndrome. A dopaminergic blockade mechanism generally is accepted as the pathogenesis of this syndrome. However, methylphenidate is a dopamine agonist via the inhibition of uptake of dopamine, and therefore dopaminergic systems in the brainstem (mainly the midbrain) and the spinal cord were unlikely to participate in the onset of this syndrome. A relative gamma-aminobutyric acid-ergic deficiency might occur because diazepam, a gamma-aminobutyric acid-mimetic agent, was strikingly effective. This is the first reported patient with neuroleptic malignant syndrome probably caused by methylphenidate.

Brain↗

PubMed1998

Neuroradiologic aspects of West syndrome.

Cranial computed tomography (CT) or magnetic resonance imaging (MRI), or both, of 143 patients with West syndrome were retrospectively reviewed. Twenty-four children had normal CT scans. The most striking findings on pathologic scans were cerebral atrophy, ventricular enlargement, and encephalomalacia. Of 78 children who underwent MRI, 14 had normal scans. Cerebral malformations, cerebral atrophy, and delayed myelination were the most common pathologic findings and were observed in 25, 20, and 16 of the cases, respectively. MRI was found to be more informative in 21 of the 27 patients who underwent both neuroimaging methods. In fact, in two patients with normal CT scans, MRI revealed delayed myelination and hyperintensity of the hippocampal area on T2-weighted images in one and increased intensity of basal ganglia on T1-weighted images in the other. On the basis of all data, four patients were classified as cryptogenic and 126 as symptomatic. The most frequent etiologic factors were perinatal asphyxia, cerebral malformations, and infections, encountered in 34, 25, and 24 of the children, respectively. Twenty-one cases could be classified solely on the basis of the data obtained with neuroimaging modalities. The data confirm and provide additional details of the ways in which neuroimaging, especially MRI, is useful in distinguishing between cryptogenic and symptomatic cases of West syndrome.

Brain↗