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At least 433 records · Page 24Linked to original sources
PubMed2000

Post-herpes encephalitic anterior pituitary insufficiency with hypothermia and hypotension.

A 49-year-old man with herpes simplex encephalitis at age 22 was admitted with hypotension (90/60 mm Hg) and hypothermia (33.7 degrees C). His blood pressure was 80-90/50-60 mm Hg, with temperatures averaging 35 degrees C, for at least 3 years before admission. Evaluation of his hypothermia and hypotension revealed a low free triiodothyronine, low normal thyrotropin, luteinizing hormone < 2 mIU/L, follicle stimulating hormone <3 mIU/L, and low testosterone of 1.39 ng/dL. A baseline cortisol of 13.9 microg/dL was stimulated to 41.8 microg/dL with corticotropin, indicating he had partial anterior hypopituitarism with an intact pituitary-adrenal axis. Posterior pituitary function was normal. MRI revealed a "bright" posterior pituitary on a T1-weighted image, further indicating a normal posterior pituitary. Extensive decreased T1-weighting on MRI in the right and left temporal lobes was consistent with encephalomalacia. With thyroid hormone replacement, his blood pressure increased to 110/70 mm Hg with a temperature of 37 degrees C.

Diagnosis, Differential↗
PubMed1989

Neonatal electroencephalography and neuropathology.

One-hundred-eight EEGs from 47 newborn infants were compared with the postmortem neuropathological findings. The degree of EEG background abnormality had good correlation with the severity of the brain lesion; the more severe the EEG background abnormality, the more extensive and intensive the morphological change. Widespread encephalomalacia was demonstrated in six infants who manifested isoelectric tracings. In particular, cerebral cortex, corpus striatum, thalamus, midbrain, and pons were affected in all patients with this abnormal EEG pattern. Burst-suppression patterns, which were seen in seven infants, also correlated with multifocal severe brain damage, but there was no common structure that was consistently affected for all patients with this pattern. Positive rolandic sharp-wave transients (PRS) appeared highly specific for white matter lesions. All eight infants with PRS had white matter lesions. However, the sensitivity of PRS for white matter lesions was not high (32%), and the white matter lesions of PRS-positive patients were not necessarily composed of periventricular leukomalacia. The sensitivity of EEG asymmetry was also low (40%) for the focality of morphological change, although the specificity was relatively high (85%). The origin of seizure discharges, on the other hand, had poor correlation with the site of the brain lesion.

Brain↗
PubMedDate not supplied

Ischemic complication of a cerebral developmental venous anomaly: case report and review of the literature.

We report a case of a nonhemorrhagic infarct associated with a thrombosed developmental venous anomaly (DVA), with secondary gliosis and Wallerian degeneration. The initial MRI scan showed an acute ischemic infarct in the region of the DVA, seen as a region of restricted diffusion on diffusion-weighted imaging (DWI), with later development of encephalomalacia and Wallerian degeneration on follow-up MRI. No blood products were seen. We believe that thrombosis of the collector vein of a DVA with associated infarction is a rare but possible complication that should be considered within the proper clinical setting and can be easily and confidently diagnosed by means of DWI.

Adult↗
PubMed1987

Magnetic resonance imaging of the brain in childhood herpesvirus infections.

We used magnetic resonance imaging (MRI) to evaluate nine children with neurologic disorders caused by infections with members of the herpesvirus family. MRI studies were abnormal in eight children and demonstrated a wide range of central nervous system lesions, including cystic encephalomalacia, ventricular enlargement, cerebral atrophy and focal parenchymal lesions. When compared with conventional computed tomographic scanning, MRI was more sensitive in detecting abnormalities of white matter and in defining the extent of parenchymal lesions. These studies indicate that MRI scans are highly useful in children with herpesvirus infections involving the central nervous system.

Brain↗
PubMed1988

Severe protein C deficiency in a newborn.

An infant presented at birth with a cutaneous lesion that developed the characteristics of purpura fulminans. He sustained a cerebral infarction during the first 4 days of life, which was initially misinterpreted to be cerebral hemorrhage resulting from thrombocytopenia and hypofibrinogenemia. After the diagnosis of Protein C deficiency was made and effective replacement therapy begun, no further cutaneous lesions occurred. Although there was no evidence of further cerebral infarction, the initial insult progressed to severe encephalomalacia and cerebral atrophy. This disorder should be considered in infants with purpura fulminans or those with cerebral infarction, as prompt institution of transfusion therapy to replace the missing protein may prevent further damage.

Humans↗
PubMed2003

Diffusion tensor magnetic resonance imaging of microstructural abnormalities in children with brain injury.

We present two pediatric cases demonstrating that diffusion tensor imaging is more efficient at revealing microstructural abnormalities of the brain than conventional magnetic resonance imaging because it enables measurements of the directionality and integrity of white matter fiber tracts. One patient suffered from left hemiparesis, and the other had right hemiparesis. However, whereas conventional magnetic resonance imaging showed only the findings of traumatic contusional hemorrhages in the left temporal and parietal lobes of the first patient and focal encephalomalacia in the left anterior thalamus of the second patient, diffusion tensor imaging successfully disclosed microstructural abnormalities in the right cerebral peduncle of the midbrain of the first patient and in the posterior limb of the left internal capsule of the second. Theses two cases demonstrate that diffusion tensor imaging is more capable than magnetic resonance imaging at detecting the microstructural pathologic lesions that are responsible for clinical motor weakness, especially when conventional magnetic resonance imaging has failed to detect subtle structural abnormalities.

Brain↗
PubMedDate not supplied

Basal membrane labyrinths in the healing stages of chick nutritional encephalopathy.

Chicks were fed a diet rich in oxidized oil and deficient in alpha-tocopherol. As soon as nutritional encephalopathy developed the animals were immediately put on to a normal diet, and kept alive for up to 166 days. Examination of the cerebellum showed multiple foci of healed encephalomalacia with EM appearance of basal membrane labyrinths between astroglial cells and around blood vessels. The possible origin of the basement membrane material in this conditions was considered and compared with similar formations in normal animals, in CNS tumour explants, and after portocaval anastomosis as well as after spinal cord lesions.

Animals↗
PubMed2005

Maternal mutation 677C > T in the methylenetetrahydrofolate reductase gene associated with severe brain injury in offspring.

A frequent polymorphism in the gene coding for 5,10-methylenetetrahydrofolate reductase is the substitution 677C > T which produces a thermolabile and inefficient enzyme. Homozygosity for the 677C > T allele is the most important determinant of hyperhomocys-teinemia, when folic acid intake is reduced. Most studies on the relationship between the 677C > T variant in the mother and defects in the offspring have focused on neural-tube defects. This study is a retrospective case-control investigation of hypoxic-ischemic encephalopathy of the newborn (HIEN) with reference to the 677C > T polymorphism as a genetic risk for this condition. The prevalence of the 677C > T allele was studied in 11 children with HIEN, their respective mothers, and 85 healthy individuals. Plasma homocysteine levels after fasting and methionine loading were determined in both mothers and controls. Ten of 11 patients were evaluated using magnetic resonance (MR) imaging, and all showed multicystic encephalomalacia and severe brain vasculopathy. Seven mothers were homozygous and four heterozygous for the 677C > T allele. Five of the children were homozygous and six heterozygous for this polymorphism. The variant allele frequency was higher in the group of mothers with affected children than in the controls and was associated with an increase in plasma homocysteine after methionine loading, in the group of mothers than in controls. The 677C > T mutation in mothers, either in a homozygous or heterozygous state, together with poor nutritional status (probable folate deficiency) may represent a risk factor for irreversible HIEN in the offspring.

Brain Damage, Chronic↗
PubMed1995

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.

Adolescent↗
PubMed1997

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.

Adolescent↗
PubMed1995

Expectant management of twin pregnancy with single fetal death.

OBJECTIVE: To evaluate the course and outcome of expectantly managed twin pregnancies with single fetal death in the second half of gestation. DESIGN: 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. SETTING: University Hospital Rotterdam. RESULTS: 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. CONCLUSIONS: Our results support expectant management in twin pregnancies complicated by single fetal death.

Adult↗
PubMed2000

Temporal lobe epilepsy: when are invasive recordings needed?

Temporal lobe epilepsy (TLE) is the most common type of medically intractable partial epilepsy amenable to surgery. In the majority of cases, the underlying pathology in temporal lobe epilepsy is mesial temporal sclerosis (MTS). Whereas historically invasive recordings were required for most epilepsy surgeries, indications have dramatically changed since the introduction of high-resolution MRI, which uncovers structural lesions in a high percentage of cases. No invasive recordings are required to perform a temporal lobectomy in patients with intractable epilepsy who have structural imaging suggesting unilateral MTS and concordant interictal and ictal surface EEG recordings, functional imaging, and clinical findings. Invasive testing is needed if there is evidence of bitemporal MTS on structural imaging and/or electrophysiologically, and additional information from functional imaging, neuropsychology, and the intracarotid amobarbital (Wada) test also does not help to lateralize the epileptogenic zone. Depth electrodes can be particularly helpful in this setting. However, no surgery is indicated, even without invasive recordings, if bitemporal-independent seizures are recorded by surface EEG and all additional testing is inconclusive. Other etiologies of TLE such as a tumor, vascular malformation, encephalomalacia, or congenital developmental abnormality account for about 30% of all patients who undergo epilepsy surgery. Epilepsy surgery is indicated after limited electrophysiologic investigations if neuroimaging and electrophysiology converge. However, approaches for resection in lesional temporal lobe epilepsy vary among centers. Completeness of resection is crucial and invasive recordings may be needed to guide the resection by mapping eloquent cortex and/or to determine the extent of the non-MRI-visible epileptogenic area. Specific approaches for the different pathologies are discussed because there is evidence that the relationship between the lesions visible on MRI and the epileptogenic zone varies among lesions of different pathologies, and therefore variable surgical strategies must be applied.

Adolescent↗
PubMed2006

Deep brain stimulation of the subthalamic nucleus as adjunct treatment for refractory epilepsy.

PURPOSE: We studied the efficacy and safety of bilateral subthalamic deep brain stimulation (DBS) for refractory partial-onset epilepsy in two cases. METHODS: This was an open treatment pilot study for subjects who had failed numerous medications and had seizure injuries. Seizure counts and adverse events were collected during a 3-4 month baseline, and for 26-32 months after DBS surgery, with AEDs held constant. RESULTS: Case 1, age 45, with bitemporal seizures, had about half the seizure frequency but still fell with injuries. Case 2, age 46, with left frontal encephalomalacia, had a frequency reduction of about one-third, but a more meaningful reduction of seizure severity and injuries. CONCLUSIONS: Subthalamic DBS partly reduced partial-onset seizures, but the quality of life was more affected by seizure-related injuries.

Accidental Falls↗
PubMed2001

Clinical and magnetic resonance imaging regression of progressive multifocal leukoencephalopathy in an AIDS patient after intensive antiretroviral therapy.

A 36-year-old homosexual man with 6 months of visual symptoms and headaches had right homonymous hemianopia, mild new learning impairment, and alexia with agraphia. The initial brain magnetic resonance imaging (MRI) scan was reported consistent with left occipital infarction. Subsequent MRI demonstrated abnormal demyelination in subcortical white matter and deep parieto-occipital white matter bilaterally, but primarily left. Human immunodeficiency virus testing and cerebrospinal fluid polymerase chain reaction for JC virus DNA were both positive, consistent with progressive multifocal leukoencephalopathy (PML) with AIDS. His clinical status steadily deteriorated, and MRI white matter abnormalities worsened despite high-dose antiretroviral therapy. After the antiretroviral regimen was intensified by the addition of a protease inhibitor, rapid clinical and radiographic improvement occurred with subsequent MRI studies revealing only residual left parieto-occipital encephalomalacia. PML in AIDS patients has been associated with a nearly uniformly poor prognosis until recent reports of improved outcomes after highly active antiretroviral therapy. This patient with PML and AIDS similarly showed a robust clinical and MRI response to intensive antiretroviral combination therapy, which has been maintained for more than 3 years.

AIDS-Related Opportunistic Infections↗
PubMed1986

Cerebral vasculitis as presenting symptom of systemic lupus erythematosus.

A 42-year-old woman developed right-sided hemiparesis due to left-sided encephalomalacia revealed by CT scan. Subsequent angiography revealed vasculitis of several intra-cranial arteries. The ESR was 65 mm/h. Further laboratory tests revealed no evidence of systemic disease so that no causal diagnosis could be posed. Treatment with prednisone (3 X 30 mg daily) led to complete cure of the hemiparesis within 6 weeks. Sixteen months later, the patient developed cutaneous lesions in the neck. Histological examination of these lesions indicated the presence of systemic lupus erythematosus (SLE). Neurological presentation of SLE is exceptional, while cerebral vasculitis as initial symptom of SLE has never been described before.

Adult↗
PubMed1995

CPEO and carnitine deficiency overlapping in MELAS syndrome.

Mitochondrial myopathy, encephalopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome is one of the mitochondrial encephalomyopathies that has distinct clinical features including stroke-like episodes with migraine-like headache, nausea, vomiting, encephalopathy and lactic acidosis. We report a 27-year-old woman who presented with partial seizure, stroke-like episodes including hemiparesis, hemianopia and hemihypethesia, sensorineural hearing loss, migraine-like headache, and lactic acidosis. Brain computed tomographic scan showed encephalomalacia in the right parieto-occipital area and recent hypodensity in the left temporoparieto-occipital area with cortical atrophy. Muscle biopsy revealed ragged-red fibers and paracrystaline inclusions in the mitochondria. Genetic study revealed an A to G point mutation at nucleotide position (np) 3243 of mitochondrial DNA. External ophthalmoplegia and ptosis were also found during two exaggerated episodes in this patient. Therefore, the overlapping syndrome of chronic progressive external ophthalmoplegia in the MELAS syndrome is considered in this case. Furthermore, we also found carnitine deficiency in this patient and she was responsive well to steroid therapy. Muscle biopsy also revealed excessive lipid droplets deposits. Therefore, the carnitine deficiency may occur in MELAS syndrome with the A to G point mutation at np 3243. We recommend the steroid or carnitine supplement therapy be applied to the MELAS syndrome with carnitine deficiency.

Adult↗
PubMed1976

Encephalolopathies in cattle in Tasmania.

Five central nervous diseases are described and their relationships to previously recorded conditions are discussed. The conditions are congenital inclusion-body encephalopathy probably caused by a paramyxovirus infection, idiopathic neuraxial oedema of a newborn calf, focal symmetrical encephalomalacia of a young calf, yacca (Xanthorrhoea spp) poisoning of adult cattle leading to spinal demyelination, and focal demyelination and neuropathy in an adult cow.

Animals↗
PubMed1959

Effect of dietary fats on fatty acid composition of human erythrocytes and chick cerebella.

Through gas-liquid chromatography it can be shown that increasing the ingestion of linoleic acid-containing fats increases the deposition of linoleic acid in erythrocytes and in brain tissue. Such changes are probably causally related to the tocopherol requirement, the incidence of chick encephalomalacia, and the peroxide hemolysis test. Whether similar mechanisms are involved in the ability of unsaturated oils to lower serum cholesterol levels remains to be determined.

Animals↗