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Neuropathologic study of resected cerebral tissue from patients with infantile spasms.

Studies to date on the neuropathologic substrates of infantile spasms have largely utilized autopsy material of children who die after a long and complicated seizure history. This makes the interpretation of primary versus secondary changes in the cerebral tissue difficult if not impossible. We have recently had the opportunity to review the neuropathologic changes in cortical tissue resected from infants and children with a history of infantile spasms. The major identifiable abnormalities were destructive lesions, sometimes classifiable as cystic-gliotic encephalomalacia, and dysplastic changes of varying degree. The cortical dysplasias had some similarity to cerebral changes described in tuberous sclerosis, including the presence of bizarre gemistocytic "balloon" cells, and secondary cytoskeletal changes within neuronal cell bodies. Such material provides an opportunity to apply immunohistochemical and molecular techniques to epileptic tissue in an attempt to understand the morphologic substrates of infantile spasms and other types of generalized epilepsy.

Brain↗

A neuropathological survey of brains submitted under the Bovine Spongiform Encephalopathy Orders in Scotland.

Bovine spongiform encephalopathy was not confirmed histologically in 225 of 829 bovine brains submitted for diagnosis. Several previously described disorders of the central nervous system were observed in these brains as well as disorders not previously recognised in Britain, including bilateral vacuolation of the substantia nigra, hippocampal sclerosis with brainstem neuronal chromatolysis and necrosis, focal symmetrical encephalomalacia and meningio-angiomatosis. Severe cerebellar dysplasia consistent with pre-natal bovine viral diarrhoea--mucosal disease virus infection or mineralisation of the blood vessels of the basal ganglia were interpreted respectively as congenital changes or changes due to ageing and were considered to be of no clinical significance.

Animals↗

CT of 338 active professional boxers.

Computed tomography (CT) was performed in 338 active professional boxers. CT scans were abnormal in 25 boxers (7%). The most common CT abnormality was brain atrophy (22 cases). Focal lesions of low attenuation consistent with posttraumatic encephalomalacia were noted in only three boxers. Boxers with abnormal CT scans did not differ from those with borderline or normal CT scans in regard to age, win-loss record, number of bouts, or history of an abnormal electroencephalogram. Thirty-seven boxers with borderline CT scans (49%) and 17 with abnormal CT scans (68%) reported a previous technical knockout (TKO) or knockout (KO), compared with only 89 (37%) of the 238 boxers with normal CT scans (P < .01). Brain atrophy was noted more frequently in boxers with a large cavum septum pellucidum (CSP) than in those with a small or no CSP (P < .05). Boxers with abnormal or borderline CT scans who experienced a TKO or KO were slightly older than those with normal CT scans and a history of a TKO or KO (P < .05).

Adolescent↗

Brain damage to the survivor within 30 min of co-twin demise in monochorionic twins.

Single fetal death in a twin pregnancy in the late second or early third trimester is associated with significant morbidity and mortality rate in the surviving co-twin, especially in monochorionic twin pregnancies. The common causes are twin-to-twin transfusion syndrome, chromosomal abnormalities, and congenital anomalies of the fetus or anomalies of the umbilical cord-placenta. Here we report a case of monochorionic twin pregnancy in which one fetus had a single umbilical artery (SUA) while the co-twin had two umbilical arteries. The twin with SUA died in utero at the 30th week of gestation and the other fetus was delivered by cesarean section immediately due to fetal distress diagnosed by cardiotocography. Disseminated intravascular coagulation and multicystic encephalomalacia have been observed in the surviving neonate. This case and review of the literature suggest that neurologic complication rates are also increased in monochorionic twin pregnancies with single fetal demise despite the immediate delivery as in our case.

Adult↗

Fetal encephalopathy after maternal anaphylaxis. Case report.

Fetal hypoxic-ischemic encephalopathy can be diagnosed at birth by means of cerebral ultrasound scanning. The morphological appearance of the lesions depends on the time elapsed between the insult and examination of the brain. We report a case of a neonate affected by multicystic encephalomalacia and corpus callosum atrophy attributable to an episode of maternal anaphylactic shock which occurred at 27 weeks of gestation following intravenous iron injection. The diagnosis was made by means of a cerebral ultrasound scan performed at birth and confirmed by magnetic resonance. This case demonstrates that maternal severe acute hypotension during pregnancy can cause fetal cerebral damage similar to the hypoxicischemic injuries occurring in the perinatal period.

Adult↗

Computerized tomography and magnetic resonance imaging following cranial base surgery.

The computed tomography scans and magnetic resonance imaging films of 57 patients who underwent anterior or anterolateral cranial base surgery from January 1987 to August 1989 were retrospectively reviewed to ascertain the significance of early and late postoperative intracranial imaging changes. Extra-axial changes (air, blood, cerebrospinal fluid collection) were found in 96% of patients; axial changes (brain edema, contusion) were seen in 30% of patients in the first postoperative period (72 hours). Subsequently, extra-axial changes began to resolve but axial changes became more prevalent. After 6 months, only axial changes persisted (encephalomalacia). It was encouraging to find a low correlation of imaging abnormalities with clinically significant findings.

Encephalomalacia↗

Distribution of cells immunopositive for AM-3K, a novel monoclonal antibody recognizing human macrophages, in normal and diseased tissues of dogs, cats, horses, cattle, pigs, and rabbits.

The monoclonal antibody AM-3K, which was developed using human pulmonary macrophages as the immunogen, immunocytochemically labels most human macrophages except for blood monocytes and dendritic cell populations. AM-3K also shows cross-reactivity in some animal species. To evaluate the usefulness of AM-3K, the present study investigated the detailed distribution of AM-3K-immunopositive macrophages in normal and diseased tissues of dogs, cats, horses, cattle, pigs, and rabbits. Zamboni's solution-fixed, paraffin-embedded sections were the most available for the immunocytochemistry with AM-3K. In all animal species examined, AM-3K labeled most macrophages in splenic red pulp, lymph node sinuses and thymus, and tissue macrophages in the interstitium of various organs and sites such as the kidneys, lungs, heart, pancreas, intestines, and skin. Alveolar macrophages and perivascular microglial cells were also immunoreactive for AM-3K. Interestingly, Kupffer cells of dogs, cats, and horses were labeled for AM-3K, but those of cattle, pigs, and rabbits were not. Furthermore, in tumor tissues and inflammatory lesions such as liver fibrosis and encephalomalacia that were obtained from dogs, infiltrating macrophages were stained with AM-3K, but not all infiltrating macrophages reacted to AM-3K. In addition, only 30-50% of pulmonary and peritoneal macrophages obtained from cats and dogs were reactive for AM-3K. AM-3K did not react with blood monocytes, dendritic cell populations, and osteoclasts. These observations indicate that AM-3K specifically labels most exudate and tissue macrophages in the animal species examined. However, the expression of antigens recognized by AM-3K on macrophages may be dependent on differential maturation stages or different functions evoked by some conditions. AM-3K immunoreaction products were seen on the cytoplasmic membrane of macrophages by immunoelectron microscopy. AM-3K would be useful for detection of macrophage populations in the animal species examined here.

Animals↗

Shunt surgery in growing skull fractures: report of two cases.

Growing skull fractures indicate underlying brain damage which is an important aetiological factor in their development. Two cases of growing skull fractures managed successfully with shunt surgery are presented. In one, the growing skull fracture was associated with an underlying tense occipital porencephalic cyst and in the other with cystic encephalomalacia and hydrocephalus. Following shunt surgery, signs of raised intracranial pressure were relieved, improvement in neurological status was noted, regrowth of the bone edges was seen and major surgery including brain debridement was avoided. The role of shunt surgery in growing skull fractures in infants is discussed and relevant details highlighted.

Brain Diseases↗

[Multicystic neonatal encephalopathy after a traffic accident with minor maternal injuries].

We report a case of traffic accident at 30 weeks of gestation. The maternal injuries were minor. The fetal heart rate patterns showed tachycardia and decreased modulation. Ultrasound scanning was normal, without abruptio placentae. Ten days after the accident, a cesarean section was done on altered fetal heart rate with a normal infant at birth. This baby died 17 days latter from an ischemic multicystic encephalomalacia. With the view of the literature, we try to explain the pathophysiological mechanism of this severe fetal outcome despite minor maternal injuries. Hypovolemic collapsus, caval syndrome and maternal stress could be the cause of placental hypoperfusion. Abruptio placenta and feto-placental hemorrhage are others explanations. We propose the management for first aid workers and the specialized unit care.

Accidents, Traffic↗

Cognitive impairment in children with hemoglobin SS sickle cell disease: relationship to MR imaging findings and hematocrit.

BACKGROUND AND PURPOSE: Children with hemoglobin SS sickle cell disease are known to suffer cognitive impairment if they have silent infarct, but recent evidence suggests that patients with hemoglobin SS sickle cell disease may be impaired even if they are free of infarction. We test a hypothesis that cognitive impairment in children with hemoglobin SS sickle cell disease is associated with low hematocrit and MR imaging abnormalities. METHODS: A cohort of 49 patients was examined, all of whom had hemoglobin SS sickle cell disease but no history of clinical stroke. The Wechsler scales, which are standardized and age-adjusted, were used to assess cognitive function. Patients also underwent MR imaging examination of the brain, and hematocrit was measured in a subset of 45 patients. MR images were evaluated by at least two readers, and abnormal imaging findings were evaluated by at least three readers. Any lesion was sufficient to be classified as abnormal, with lesions defined to include lacunar infarction, encephalomalacia, or leukoencephalopathy. Hematocrit data were used if obtained within 3 months of psychometric testing and if there were no confounding events in the patients' charts. Wechsler test scores were then evaluated in relation to imaging findings and hematocrit values. RESULTS: Patients with imaging abnormalities had more cognitive impairment than did patients with normal imaging findings in verbal intelligence quotient (P <.02) and verbal comprehension (P <.01). Patients with low hematocrit had cognitive impairment shown by many performance measures, including full-scale intelligence quotient (P <.006), verbal comprehension (P <.006), and freedom from distractibility (P <.02). Multivariate analysis showed that MR imaging and hematocrit were independent predictors of full-scale intelligence quotient. CONCLUSION: Focal brain injury, revealed by MR imaging, is associated with cognitive impairment, but our data suggest that diffuse brain injury may also contribute to impairment. These findings show that impairment is multifactorial and suggest that chronic brain hypoxia is part of the pathophysiology of sickle cell disease.

Adolescent↗

Prefrontal sonic treatment: MR findings.

The authors describe the use of MR in a patient who underwent prefrontal sonic treatment for relief of intractable headaches. MR demonstrated well-demarcated regions of encephalomalacia within the white matter of the frontal lobe (with relative sparing of cortex) in a characteristic conical volume that corresponded to the insonified regions.

Aged↗

Pyogenic meningitis: sonographic evaluation.

Forty infants with proven pyogenic meningitis were evaluated by real time cranial sonography. A spectrum of sonographic abnormalities was observed which included echogenic sulci, focal or diffuse increase in parenchymal echoes, ventriculitis, ventriculomegaly with or without aqueductal block, subdural collection, parenchymal infarcts, abscess and subdural empyema. There were two infants with normal sonogram while encephalomalacia was seen in another two patients. An excellent correlation was observed between clinical profile, cerebrospinal fluid biochemistry and sonographic findings.

Brain Abscess↗

[Clinico-morphologic correlations in cerebral infarction after recurrent hemorrhage].

Clinical-morphological analysis of cases in which, in the course of vasogenic necrotic brain lesion, hemorrhagic foci appeared within the infarcted area, was performed. The material comprised 355 cases with encephalomalacia (223 women and 123 men). The patients died at age between 39 and 96 years. In the clinical picture a sudden onset of the disease prevailed, with hemiparesis or hemiplegia associated in 216 cases with disturbances of consciousness. The course of the disease was in general progressive. Only in 30 patients improvement of clinical state was observed. The time of survival varied from several hours to several weeks. Most patients (140) died in the first week in deep cerebral coma or state of decerebration. In nine cases clinical symptomatology of herniation and secondary hemorrhages into the brain stem was found. A sudden onset and violent course of the disease resulted in a diagnosis of primary hemorrhage with penetration to the subarachnoid space or ventricular system in 32 patients. Only in six cases was a secondary hemorrhage into the primary infarction diagnosed clinically. Hemorrhagic malacia was most frequently (in 255 cases) located in region vascularized by the middle cerebral artery and very seldom, the basilar artery or cerebellar arteries. Malacia was accompanied by considerable brain edema. This was particularly intense in extensive infarctions and persisted even in cases with considerable long survival. In 45 cases secondary hemorrhagic foci were found within brain stem. The morphology of the infarction foci was variable in most cases. Independently to fresh unreactive diffuse necrosis, sometimes areas with various advancement of resorption and organization processes were observed in the same case. Both in areas of fresh reactionless malacia and of advanced macrophage-glial disintegration, or even in areas of advanced gliomesodermal organization, stand-like or spherical hemorrhages could be seen merging sometimes into extensive pools. The morphology of hemorrhagic foci was largely dependent on their size and localization. The hemorrhagic foci located close to the depth of the cortical sulci very often destroyed the cortex and white matter, and penetrated to the subarachnoid space (141 cases). In was usually almost impossible to detect the source of bleeding within the necrotically changed area. The resorption reaction at the edge of extensive hemorrhagic foci was in general minimal. A lesion of the vessel wall, caused by stasis of long duration, resulting from disorders of the vascular function in the course of defficient blood supply and ischemic brain edema are considered the main causes of bleeding into the pale infarction.

Adult↗

Congenital microcephaly due to vascular disruption: in utero documentation.

Death in utero of one member of a monozygotic twin pair has been associated with vascular disruptive phenomena in the surviving twin. It has been hypothesized that this event initiates clot formation in the surviving twin with consequent necrosis of tissues distal to the occluded vessels. This case report describes onset in utero of multicystic encephalomalacia and microcephaly in a surviving twin whose brain appeared normal on ultrasound scanning before death of the cotwin at 21 weeks' gestation. The case provides further support for the hypothesized pathogenetic sequence and illustrates the importance of reviewing all prenatal ultrasound scans in infants with congenital microcephaly.

Cerebral Infarction↗

[Serum neuraminic acid level in patients with cerebrovascular disorders].

The authors determined the level of neuraminic acid in the serum of 50 patients with encephalomalacia and 25 patients with cerebral haemorrhages on the 1st, 3d and 7th days of the disease. Twenty healthy subjects served as controls. A statistically significant rise in the concentration of this substance was observed in both groups of patients throughout the whole period of observations.

17-Hydroxycorticosteroids↗

[Levels of ammonia in patients with stroke].

The authors determined peripheral-blood ammonia level in patients with cerebral strokes. The determinations were done by the method of Konitzer et al. on the 1st, 3rd and 7th days of the disease. The results of determinations were subjected to statistical analysis with the t test of Student. It was found that the mean serum ammonia level in patients with cerebral stroke on the 1st day of the disease was higher than in the control group. The difference was statistically significant. The level of ammonia in the blood of patients with cerebral haemorrhage was significantly higher on the 1st day of the disease than in patients with encephalomalacia. The difference was statistically significant with error probability of 0.05.

Adult↗

Complications of Citrobacter neonatal meningitis: assessment by real-time cranial sonography correlated with CT.

Real-time cranial sonography via the anterior fontanelle was used serially over a 3- and 6-week period, respectively, to evaluate two infants who developed multicystic encephalomalacia secondary to Citrobacter neonatal meningitis. Sonographic findings included heterogeneous parenchymal echogenicity, gyral prominence, periventricular hypoechoic areas from which cystic spaces evolved, and development of hydrocephalus. Serial cranial computed tomography over the same time period confirmed the sonographic observations in each case.

Citrobacter↗

[The value of computer tomography in the diagnosis of encephalomalacic foci].

The authors report a comparative analysis of the results of angiographic investigations and computer tomography in patients with clinical manifestations of stroke. Attention is called to the accuracy of the information obtained in computer tomography with regard to the extent of focal encephalomalacia and stage of evolution. Signs are described making possible differentiation between vasogenic lesions and other pathological processes of identical or similar features in computer tomography.

Adult↗