Multicystic Encephalomalacia Research Articles

Polymicrogyria in monozygous twins and an elder sibling

Monozygous twin births have been associated with brain lesions such as hydranencephaly, multicystic encephalomalacia, and porencephaly. Prenatal circulatory injury has been considered to be the cause. Polymicrogyria is rare but has been reported in autopsied cases. The sibship in this case report, comprising monozygotic male twins and their elder sister from the same non‐consanguineous parents, all had global developmental delay. Brain MRI showed polymicrogyria. We suggest that, apart from circulatory compromise, genetic etiology must be implicated as the cause of polymicrogyria.

Urologic Pathology, Abstract 211–219, Posters

The aim of this study was to define the risk ratios of the late-infancy magnetic resonance imaging pattern for long-term outcome in term infants with perinatal asphyxia. We evaluated 65 term infants with perinatal asphyxia and performed magnetic resonance imaging examinations between 4–12 months of age. Magnetic resonance imaging scans were classified as follows: (1) periventricular leukomalacia in 21 (32%) infants, (2) marked cortical atrophy in 17 (26%) infants, (3) multicystic encephalomalacia in 10 (15%) infants, (4) deep gray matter involvement in 8 (12%) infants, (5) focal cortical involvement in 6 (9%) infants, (6) myelination delay in 3 (5%) infants. The overall outcome was favorable in 19 (29%) of 65 infants. Infants with diffuse cortical involvement (multicystic encephalomalacia and marked cortical atrophy) are four times (odds ratio: 4.4 and 4.1 respectively) more likely to attain the unfavorable outcome than the infants with other patterns of magnetic resonance imaging. Infants with focal cortical involvement had relatively favorable outcome in 60% of the cases. In conclusion, it appears that the overall outcome of infants with perinatal asphyxia correlated well with the magnetic resonance imaging patterns obtained between 4 and 12 months of age.

Hallazgos por resonancia magnética en la parálisis cerebral espástica

To study the types of lesion found on magnetic resonance (MR) in cerebral palsy (CP) and their correlation with a the type of CP, gestational age at birth and aetiology. We made a retrospective review of all patients over one year in age, diagnosed as having spastic CP, who were seen in a paediatric neurology clinic between 1987 and 1998. The MR were evaluated blindly using data from the clinical history. Seventy two patients fulfilled criteria for inclusion in the study. The following results were obtained (all associations were statistically significant with p<0.01):1. Patterns of lesion: MR findings were classified into one of the following patterns: parasagittal lesion (7), diffuse multicystic encephalomalacia (4), periventricular leukomalacia (PVL) (26), bilateral dilatation of the basal ventricles without alteration of the signal from the periventricular white matter (5), bilateral lesions in the basal ganglia (5), focal multicystic encephalomalacia (limited to one vascular area) (4), unilateral lesions of hypersignal in the white matter (4), unilateral dilation of a lateral ventricle with or without homolateral cortical atrophy (3), malformations/disorders of migration (7), normal (7). 2. Correlation of the findings with the gestational age and type of CP: a) Spastic tetraplegia: 64% of the premature babies showed PVL. In newborn babies at term the lesions were more varied. b) Spastic diplegia: 80% of the premature babies had PVL and 83% of newborn babies at term had normal MR. c) Spastic hemiplegia: 81% had unilateral lesions. 3. Correlation with the aetiology: 62% of the cases of parasagittal lesions, diffuse multicystic encephalomalacia and bilateral lesions of the basal ganglia were due to hypoxic-ischemic encephalopathy. The MR findings in CP show good correlation with gestational age, type of CP and aetiology.

Esquisencefalia acompañada de porencefalia en una niña con infección congénita por citomegalovirus

At the present time it is perfectly clear that schizencephaly is an anomaly of neuronal migration and porencephaly is the result of destruction of the cerebral cortex. Schizencephaly is the final result of a variety of lesions which occur during a critical period of the development of the brain. Imaging studies have shown many cerebral anomalies varying from lissencephaly to multicystic encephalomalacia. We report the case of a girl in whom porencephaly was associated with schizencephaly and in whom angioresonance showed hypoplasia of the right middle cerebral artery. The parents of an eight month old girl consulted us after observing that she had reduced movement of her left arm. On clinical examination there was left hemiparesia. Both CAT and MR showed closed lip schizencephaly of the left hemisphere and an area of right frontoparietal encephalomalacia. Study of the vascular structures showed underdevelopment of the right middle cerebral artery. The cytomegalovirus has been considered to be the principal infectious agent involved in the aetiology of different disorders of neuronal migration. It has been reported that the cytomegalovirus causes vasculitis which leads to reduction in perfusion and lesions due to secondary encephalomalacia. The fact that our patient had a hypoplastic middle cerebral artery, and a normal left middle cerebral artery, may be explained by subsequent recanalization of the middle cerebral artery. This would seem to indicate that the lesion causing schizencephaly is older and there has been time for recanalization.

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 γ-aminobutyric acid-ergic deficiency might occur because diazepam, a γ-aminobutyric acid-mimetic agent, was strikingly effective. This is the first reported patient with neuroleptic malignant syndrome probably caused by methylphenidate.

CT findings in neonatal hypothermia.

Newborn infants are particularly prone to hypothermia, a condition with a high mortality. To study the CT brain patterns in infants with hypothermia and neurological symptoms. We reviewed the brain CT of nine infants with neonatal hypothermia, multiple organ failure, seizures and coma. Two infants had normal CT scans, acutely and at follow-up, and were clinically normal at follow-up. In seven infants, CT showed diffuse cerebral oedema, with reversal of the normal density relationship between grey and white matter and a relative increased density of the thalami, brainstem and cerebellum - the 'reversal sign'. In six surviving infants with severe developmental delay, follow-up CT revealed cerebral atrophy with multicystic encephalomalacia. The 'reversal sign' has been described in the abused child, birth asphyxia and anoxia due to drowning. Neonatal hypothermia is offered as a further cause.

MRI findings in patients with spastic cerebral palsy. I: Correlation with gestational age at birth.

The authors studied MR images of the brain in 152 patients, aged 1 to 19 years (mean 3.3), who had spastic cerebral palsy (CP) and were attending two hospitals in Japan in 1993 and 1994. The relation was studied between the patients' gestational age at birth and their MRI findings, including the severity of periventricular leukomalacia (PVL) seen on MRI. In 119 of these patients, the CP was thought to be due to acquired, destructive brain injury. PVL was seen in 90 and posthemorrhagic porencephaly in 11. These preterm-type brain injuries were observed often in patients who had been born preterm but were also seen in those born at term. Fullterm-type border-zone infarct, bilateral basal ganglia-thalamic lesion, subcortical leukomalacia, and multicystic encephalomalacia were seen in 9, 14, 7, and 3 patients, respectively; these term-type brain injuries were observed only in patients born at or near term. Of the patients with PVL, 90% had been born preterm. Severe PVL was common in the patients whose gestational ages at birth were between 25 and 32 weeks; all patients with PVL who had been born at term had only mild PVL. The authors concluded that MRI findings for patients with spastic CP are closely correlated with gestational age.

Multicystic encephalomalacia in term infants.

The terms "multicystic encephalomalacia" and "subcortical leucomalacia" (SCL) are used to describe the presence of areas of necrosis that develop into cystic lesions inside the brain. These lesions are generally due to severe asphyxia and/or hypotension. The designation SCL can also be used to describe the extent and the seat of the lesion in the brain or to distinguish subcortical lesions from periventricular and/or combined ones. In this study we give an account of our experience with eight newborns admitted to our neonatal intensive care unit, who presented clinical-neurological alterations and encephalomalacic lesions whose presence was documented by ultrasonography.

Three types of multicystic encephalomalacia and the analyses of its risk factors

Three types of multicystic encephalomalacia and the analyses of its risk factors

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 the 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.

Differential involvement of the brain in neonatal asphyxia: a pathogenic explanation.

Multiple cystic lesions in brain parenchyma supplied by the anterior cerebral circulation is a recognized pattern of cerebral injury associated with hypoxic-ischemic encephalopathy in the term infant. This report presents a series of seven infants (gestational age, 39.3 +/- 2.8 weeks; range, 36 to 44 weeks) who developed multicystic encephalomalacia in the distribution of the anterior cerebral circulation after severe neonatal asphyxia. Cerebral imaging and pathologic studies demonstrate relative preservation of the cerebellum, brain stem, and cerebral structures supplied by the vertebrobasilar circulation. Compared to the vertebrobasilar vasculature, the anterior cerebral vessels in the term infant have dense sympathetic innervation. Asphyxia, a potent sympathetic stimulator, may induce vasoconstriction in the anterior circulation and differentially accentuate the effects of hypoxia/ischemia on cerebral tissue.

Bilateral spastic cerebral palsy--MRI pathology and origin. Analysis from a representative series of 56 cases.

MRI of the brain was performed on 56 children with bilateral spastic cerebral palsy (CP) at a mean age of 10.7 years. Specific pathology was found in 91 per cent; periventricular leukomalacia was present in 42 per cent of term- and 87 per cent of preterm-born children. Parasagittal subcorticocortical injury, multicystic encephalomalacia and basal ganglia lesions were identified in 16 per cent, in all but one associated with severe peri-/neonatal events at term or near term. Maldevelopment comprised 9 per cent, all but one found in term-born children. MRI morphology correlated strikingly with outcome. Periventricular leukomalacia was associated with more severe disability in term- than preterm-born children.

Multicystic Encephalopathy

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.

Multivariate Autoregressive Analysis of Carotid Artery Blood Flow Waveform in a Newborn with Multicystic Encephalomalacia

Multivariate Autoregressive Analysis of Carotid Artery Blood Flow Waveform in a Newborn with Multicystic Encephalomalacia

Expectant management of twin pregnancy with single fetal death

To evaluate the course and outcome of expectantly managed twin pregnancies with single fetal death in the second half of gestation. Case-controlled study of 29 consecutive pregnancies from 1973 to 1993, with sonographic evidence of a twin at 20 weeks gestation with antenatal demise later in pregnancy, matched for maternal parity with 58 twin pregnancies without fetal death and delivered in the same year as the index case. Outcome measures included the incidence of complications of pregnancy, gestational age and mode of delivery, placentation, and perinatal outcome. University Hospital Rotterdam. The frequency and severity of pregnancy-induced hypertensive disorders was significantly higher in the study group than in controls. We found no differences between the study group and controls with regard to median gestational age at delivery (33 weeks versus 34 weeks) and median birthweight of liveborn infants (1880 g versus 2160 g). No consumptive coagulopathy was apparent in our 29 patients. The main cause of neonatal death was prematurity; multicystic encephalomalacia was not observed. Our results support expectant management in twin pregnancies complicated by single fetal death.

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.

Neuropathological Findings of Neonatal Mice Following Hypoxic Encephalopathy

One‐day‐old mice were subjected to hypoxia by 8‐hour exposure to a 5% oxygen, 95% nitrogen mixture. The brains from ten surviving mice with moderate brain hemorrhage were microscopically examined at 36 and 60 hours after the hypoxic event. These brains showed the coexistence of parasagittal cerebral injury, periventricular leukomalacia, multicystic encephalomalacia and subependymal germinolysis. Parasagittal cerebral injury is regarded as the principal ischemic lesion of the full‐term human infant, and periventricular leukomalacia as that of the premature infant. These results suggest the important role of hypoxia/ischemia in the pathogenesis of subependymal germinolysis and regional differences in maturation between mouse and human brain.

Three Cases with Cavitary Lesions in a Twin Gestation Complicated by a Single Intrauterine Fetal Demise

Three cases with cavitary lesions on the cerebral convexities in twin gestation complicated by a single intrauterine fetal demise were investigated clinicopathologically. They all exhibited profound mental retardation and severe motor disturbance due to rigidospastic tetraplegia. The co‐twins were macerated.Neuropathologically, malformed lesions and destructive lesions coexisted in varying degrees according to the time in fetal life, at which insults probably occurred. The cavitary lesions were located bilaterally and symmetrically on the central, parietal and occipital region of the cerebral hemispheres, in some cases, including the frontal region. Case 1 showed an incomplete gyral pattern and porencephaly with polymicrogyria bordering the defect, thus allowing us to date the insult before the 5th fetal month. In case 2, multicystic encephalomalacia was disclosed with cortical dysgenesis exhibiting status marmoratus of the cortex. Case 3 displayed sclerotic cavitary lesions with sclerosis of the insula and a few instances of cortical dysgenesis. Based on the clinical data and pathological findings, the cavitary lesions of case3 may be dated in the third trimester of gestation. Case 2 can be considered to have sustained injuries between the times estimated for cases 1 and 3.As the pathogenesis of the congenital cavitary lesions, several concepts have been proposed: intrauterine DIC (Moore et al. 1969;Romero et al. 1984), vascular disruption (Hoyme et al. 1981;Jung et al. 1984), intrauterine infection such as cytomegalic inclusion disease (Friede et al. 1976), and hypoxia due to CO intoxication of the mother (Bank1 et al. 1967) or other cause. Though the pathological observation of our cases revealed no evidence of intrauterine DIC, renal cortic necrosis, intrauterine infection or occlusion of the intracranialvessels, long‐term exposure to the dead twin might have contributed to the lesions of the liveborn co‐twin.

Brain abscesses in neonates — report of three cases

We report three newborns with brain abscesses. Two infants suffered from Serratia marcescens meningitis and one infant had enterococcal sepsis and meningitis. Brain abscesses were detected by cerebral sonography. Outcome in one infant with S. marcescens infection was poor. This patient developed multicystic encephalo-malacia and severe developmental retardation. In the other patient with S. marcescens infection surgical drainage of the abscess was performed. The outcome was good both in this infant and in the patient with enterococcal brain abscess.

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 Pa co 2, Pa o 2, and pH and the lowest systolic and diastolic blood pressures were compared among each group and controls. The lowest Pa co 2 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.