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At least 19 recordsLinked to original sources

Resection of frontal encephalomalacias for intractable epilepsy: outcome and prognostic factors.

PURPOSE: Because focal encephalomalacia is an important cause of medically intractable partial epilepsy and few studies have evaluated the efficacy and the safety of resecting focal-encephalomalacias to improve seizure control, we studied a cohort of 17 consecutive patients who underwent resection of encephalomalacias in the frontal lobes as a treatment of their intractable epilepsy. METHODS: We evaluated several factors for their value in predicting postsurgical seizure control. Pre- and postsurgical magnetic resonance imaging (MRI) scans were reviewed independently by 2 blinded investigators. RESULTS: At a median of 3 years of follow-up (range 0.6-7.5 years), 12 patients (70%) were seizure-free or had only rare seizures. The presence of a focal fast frequency discharge (focal ictal beta pattern) at the beginning of seizures on scalp EEG was predictive of seizure-free outcome (p = 0.017), even among patients who had complete resection of their encephalomalacias (p = 0.016). There was no significant differences in outcome with regard to age at the time of the injury that caused encephalomalacia, interval between injury and onset of seizures, duration of presurgical seizure history, presurgical seizure frequency, age at surgery, or the completeness of encephalomalacia resection. The analysis regarding completeness of encephalomalacia resection almost reached significance, suggesting that it may also be an important predictive factor (p = 0.051). CONCLUSIONS: We conclude that surgery is a very effective treatment for intractable frontal lobe epilepsy (FLE) secondary to encephalomalacias. Patients are more likely to become seizure-free if they have a focal ictal beta discharge on their scalp EEG. Complete resection of the encephalomalacia should be attempted, since our results suggest that this may be a favorable predictive factor. Moreover, the operative strategy for our patients entailed, whenever possible, complete resection of the encephalomalacias and of the adjacent electrophysiologically abnormal tissues.

Adolescent↗

Neonatal multifocal encephalomalacia and other lesions in the brains of Georgia chicks: 1991-95.

Outbreaks of neonatal multifocal encephalomalacia with sepsis have been reported among flocks of very young chicks in Belgium, Scotland, and the United States. The purposes of the present study were to describe intralesional bacterial cocci in chicks with this type of encephalomalacia and to determine its incidence during 1991-95, and to determine the importance of this lesion with respect to the frequency of all other brain lesions/ diseases during the same time period. All laboratory records of broiler chickens examined at the Georgia Poultry Laboratory from Jan. 1, 1991, through Dec. 31, 1995, where the histopathologic diagnoses included the letter string *encephal* were retrieved for further study. The leading etiology for brain disease was nutritional encephalomalacia (57%), followed by neonatal encephalomalacia (22%), septic meningoencephalitis (16%), and Marek's disease (14%), in turn followed by nonpurulent encephalitis (7%), avian encephalomyelitis (3%), and mycotic meningoencephalitis (3%). Diagnosis of neonatal multifocal encephalomalacia with sepsis in the brains of Georgia chicks is a perennial one. Microscopically, the condition is characterized by mild to maximal multifocal locally extensive fibrin thrombosis of blood capillaries, and necrosis (encephalomalacia, malacia) of surrounding zones of brain stem and/or cerebral hemisphere neuropile. In 44% of cases of neonatal encephalomalacia fibrin thrombosis of blood capillaries was accompanied by intralesional gram-positive coccoid bacteria that were most abundant in medium- and small-sized arterioles or venules. Only gram-positive coccoid bacteria are found in cases of neonatal encephalomalacia (P < 0.05), and only gram-negative rod-shaped bacteria are found in cases of septic meningoencephalitis (P < 0.05). Therefore, bacterial culture and routine light microscopic histopathology are sufficient for diagnosing the condition when the differential diagnosis for neurologic disease in chicks includes neonatal encephalomalacia.

Animals↗

Preventive effect of selenium, methionine and antioxidants against encephalomalacia of chicks induced by dilauryl succinate.

The protective effect of supplemental selenium, methionine, ascorbic acid, menaquinone and five antioxidants against encephalomalacia of chicks fed a diet containing dilauryl succinate was examined. Diauryl succinate induces vitamin E deficiency signs such as fragility of the erythrocytes and encephalomalacia. Supplementation of selenium and methionine with or without simultaneous supplementation of a low level of dl-alpha-tocopheryl acetate had little effect on preventing encephalomalacia. The preventive effect of ascorbic acid, methylene blue, ethoyquine, 2,6-ditertiary-butyl-p-cresol and butylated hydroxyanisole was roughly in proportion to their dietary level, and a high level of any of them could almost completely protect the chicks from encephalomalacia, while diphenyl-p-phenylenediamine was not as effective and the effect was not proportional to the dose. Menaquinone had little effect. No difference was observed in the plasma tocopherol levels and peroxide levels in the adipose tissueof the chick fed eith er dilauryl succinate or cornstarch. The effect of dilauryl succinate appears to be independent of peroxides generated in the chick.

Adipose Tissue↗

Magnetic resonance imaging features of equine nigropallidal encephalomalacia.

Magnetic resonance imaging (MR) was used to make a diagnosis of equine nigropallidal encephalomalacia in a horse. Equine nigropallidal encephalomalacia is a neurodegenerative disease that has many characteristics with Parkinson-like diseases in humans. Historically, horses were euthanized based on clinical signs and exposure to the toxic weed, yellow star thistle (Centaurea solstitialis). Previously, the disease has only been confirmed on necropsy. MR imaging can provide accurate and sensitive visualization of typical lesions seen in the brain of horses affected with equine nigropallidal encephalomalacia. Lesions were seen on T1-weighted, T2-weighted and proton density images. There was no contrast enhancement following Gd-DTPA administration. Lesions seen on MR were confirmed at necropsy. Using MR to confirm a diagnosis of equine nigropallidal encephalomalacia will prevent unnecessary suffering of horses and expense to owners that would otherwise incur, while further diagnostics are performed.

Animals↗

Cerebellar encephalomalacia on magnetic resonance imaging after removal of acoustic tumor.

MRI is widely used for postoperative surveillance of patients undergoing surgery for removal of acoustic neuroma. The purpose of this study was to investigate the frequency and pattern of postoperative changes in the cerebellum and brain stem on MRI after removal of acoustic neuroma. A retrospective study was conducted in 30 consecutive patients who underwent postoperative MRI between 1994 and 1995. The timing of the scans after surgery ranged from 12 months to 10 years. T2 -weighted turbo spin-echo images revealed cerebellar encephalomalacia in 17 of 30 cases. Cerebellar encephalomalacia was found more consistently in patients who had large tumors and was more frequent after the suboccipital approach. Encephalomalacia is largely caused by gliotic changes in the adjacent cerebellar tissues after tumor removal.

Adolescent↗

Hyperprolactinemia presenting with encephalomalacia-associated seizure disorder and infertility: a novel application for bromocriptine therapy in reproductive endocrinology.

OBJECTIVES: To describe successful oral bromocriptine therapy for hyperprolactinemia accompanied by seizure disorder and encephalomalacia identified during infertility evaluation. MATERIALS & METHODS: A 32 year-old male with an unspecified seizure disorder was referred for infertility consultation. The initial seizure was at age 12, and was treated sequentially with phenytoin, phenobarbitol, and carbamazepine monotherapy which was maintained for six years and then discontinued. At age 26, a second seizure was experienced and 150mg lamotrigine b.i.d. was initiated. When the patient married it was discovered that erection was impossible; tadalafil was administered without success. At our center, total testosterone was low at 107ng/dl and semen analysis revealed oligoasthenozoospermia. Serum prolactin was 79.3ng/ml; visual field examination was normal. Brain MR showed no pituitary enlargement. However, encephalomalacia of the right occiptal lobe with porencephalic dilation of the occipital horn of the right lateral ventricle was present. RESULTS: The patient was initially placed on 2.5mg/d bromocriptine but after three months only a nominal reduction of serum prolactin was achieved. Serum prolactin normalized (25.4ng/ml) after bromocriptine was increased to 7.5mg/d. Repeat semen analysis showed overall sperm concentration of 85M/ml, 30% motility and 12% normal forms morphology (Kruger strict criteria). Total testosterone level was 191ng/dl after final bromocriptine dose was attained, which was well tolerated. DISCUSSION: Bromocriptine has an established role in the management of hyperprolactinemia, particularly in women. For males with elevated serum prolactin even when associated with focal encephalomalacia and seizure disorder, bromocriptine therapy can offer safe benefits including improved semen parameters and normal serum testosterone levels.

Adult↗

Computed tomography evolution of multicystic encephalomalacia.

A case of multicystic encephalomalacia is presented, demonstrating postischemic maldevelopment in the cerebrum on serial computed tomography scans. The developmental features of multicystic encephalomalacia are discussed, as well as migration abnormalities such as encephaloclastic porencephaly and agenetic porencephaly. Representative computed tomography scans of porencephaly are included.

Brain↗

Phagocytic astrocytes and neurons in old encephalomalacia.

Cytoplasmic accumulation of hemosiderin was observed within astrocytes and neurons as well as in other phagocytes associated with old encephalomalacia. The first patient was 3 years old when she died with malnutrition and superimposed infection. A cortical infarct had been caused by an old thrombus in a small artery. The second case was a 9-year-old girl who had old encephalomalacia in the pineal region after extirpation of a pineoloma. The third patient was a 22-year-old man who had a cortical infarct from an embolus associated with chronic rheumatic endocarditis. Granules of hemosiderin had accumulated within the perikaryon of many astrocytes and other phagocytes in all cases, and in a few neurons in the second instance. Review of the literature reveals that cytoplasmic accumulation of particulate matter has been described infrequently with regard to neuroepithelial derivatives. Our findings indicate that astrocytes and neurons as well as derivations of mesenchyme may act as phagocytes of old blood.

Adult↗

Bilateral encephalomalacia in a pig related to latent Aujeszky's disease virus infection.

A case of bilateral encephalomalacia in a pig related to latent Aujeszky's disease virus infection is reported. The pig was experimentally inoculated with the NIA-3 strain and survived the infection after showing intense central nervous system disease. Abnormal behaviour was observed up to the date of death. The pig was demonstrated to be latently infected with the virus by polymerase chain reaction (PCR). The main microscopic lesion was a bilateral encephalomalacia which involved structures related to the limbic system. A complete description of lesions observed and their relation to abnormalities shown by the pig are exposed.

Animals↗

Multicystic encephalomalacia in an adult case of Lennox-Gastaut syndrome: a case report.

It is well known that multicystic encephalomalacia results in a severe neurological deficit and psychomotor retardation. The authors reported the case of a 30-year-old man with Lennox-Gastaut syndrome who had multicystic encephalomalacia in CT and MRI but whose neurological deficit was not serious. It is possible that the forceps delivery caused MCE and that a vaccination against Japanese encephalitis at the age of 9 triggered the Lennox-Gastaut syndrome.

Adult↗

Identification of a novel intronic variant in COL4A2 gene associated with fetal severe cerebral encephalomalacia and subdural hemorrhage.

BACKGROUND: Genetic variants in COL4A2 are less common than those of COL4A1 and their fetal clinical phenotype has not been well described to date. We present a fetus from China with an intronic variant in COL4A2 associated with a prenatal diagnosis of severe cerebral encephalomalacia and subdural hemorrhage. METHODS: Whole exome sequencing (WES) was applied to screen potential genetic causes. Bioinformatic analysis was performed to predict the pathogenicity of the variant. In in vitro experiment, the minigene assays were performed to assess the variant's effect. RESULTS: In this proband, we observed ventriculomegaly, subdural hemorrhage, and extensive encephalomalacia that initially suggested cerebral hypoxic-ischemic and/or hemorrhagic lesions. WES identified a de novo heterozygous variant c.549&#x2009;+&#x2009;5G&#x2009;>&#x2009;A in COL4A2 gene. This novel variant leads to the skipping of exon 8, which induces the loss of 24 native amino acids, resulting in a shortened COL4A2 protein (p.Pro161_Gly184del). CONCLUSION: Our study demonstrated that c.549&#x2009;+&#x2009;5G&#x2009;>&#x2009;A in COL4A2 gene is a disease-causing variant by aberrant splicing. This finding enriches the variant spectrum of COL4A2 gene, which not only improves the understanding of the fetal neurological disorders associated with hypoxic-ischemic and hemorrhagic lesions from a clinical perspective but also provides guidance on genetic diagnosis and counseling.

Female↗

Sonographic recognition of multiple cystic encephalomalacia.

Multiple cysts in both hemispheres were detected in four children under 1 year of age by real-time sonographic sector scanning. These cysts, typical of multiple cystic encephalomalacia, followed viral encephalitis in two, bacterial meningitis in one, and bacterial meningitis superimposed on intracerebral hemorrhage in one. The diagnosis of multiple cystic encephalomalacia, which has a grave prognosis, is readily made with high-resolution real-time sonography.

Brain↗

Web-like malformation of the carotid artery and multicystic encephalomalacia.

Multicystic encephalomalacia and hydranencephaly lie within a spectrum of brain lesions linked to ischemic cerebral damage. Causes include vascular malformation, thrombosis, embolism, infection, and toxins. We describe an infant with multicystic encephalomalacia associated with a peculiar web-like malformation of the right common carotid and left subclavian arteries. We postulate that this luminal bridging is a congenital malformation resulting from defective canalization of the medium-sized blood vessels but could represent organized and recanalized thrombi, the etiology of which remains unknown.

Brain Ischemia↗

Dietary factors affecting experimental models of nutritional encephalomalacia.

Various factors affecting the experimental development of nutritional encephalomalacia (NE) were studied in young chicks. The effects of these factors were evaluated by calculation of the age at which one-half were affected (T1/2). The incidence of ataxia and mortality and statistical analysis of the intensity of the disease were also calculated. No differences were found among the safflower oil samples oxidized for periods ranging from 12 to 48 hr in their potency to induce NE, while oil oxidized for 72 hr was less effective. No difference was observed between the effects of oxidized safflower oil and freshly distilled methyl esters of safflower oil on the development of NE. This disorder was more severe in chicks fed a fat-free diet deficient in vitamin E for the first week and then the NE-inducing diet than in chicks fed the NE-inducing diet from hatching. Feeding chicks vitamin E for the first week delayed the development of encephalomalacia but did not prevent it. In order to prevent NE in young chicks fed oxidized safflower oil, a ratio of .3 mg alpha-tocopherol per gram oil was required.

Animals↗

Silent acute pancreatitis with encephalomalacia mimicking hepatic coma.

A very rare case of acute pancreatitis with concurrent encephalomalacia and ascites mimicking hepatic coma is described. The possibliity that the pancreatitis was caused by the administration of chlorothiazide in a diabetic patient is suggested as possible etiology. It is emphasized that when a cirrhotic patient develops coma, the possibility of painless,silent pancreatitis with encephalomalacia as well as hepatic coma should be considered in the differential diagnosis.

Acute Disease↗

[Multicystic encephalomalacia: MR imaging findings and clinical correlation].

PURPOSE: To review the MR imaging findings of multicystic encephalomalacia and to investigate the correlation between MR imaging and clinical findings. MATERIALS AND METHODS: Twenty-one patients who presented with convulsion, mental-motor retardation and microcephaly and had evidence of multicystic encephalomalacia on MR images were included in this study. MR imaging patterns and clinical findings were reviewed. Consequently, we correlated MR imaging findings and clinical outcome. RESULTS: All patients had cortical thinning, white matter destruction, atrophy and gliosis. Tetraplegia was seen in 17 out of 19 patients with mixed type cerebral palsy in two patients with diffuse or symmetric involvement on MR imaging. Both of the patients with mixed type cerebral palsy had basal ganglia involvement on MR imaging. Hemiplegia was seen in two patients with asymmetric involvement on MR imaging. Microcephaly was seen in 17 patients with diffuse or symmetrical, and in one patient with asymmetrical, involvement. Microcephaly and tetraplegia was seen in all patients with cerebellar and basal ganglion involvement. CONCLUSION: Microcephaly and spastic tetraplegia were developed mostly in patients with diffuse involvement, whereas hemiplegia was seen in patients with asymmetric involvement. The clinical outcome was worse in patients with cerebellar and brainstem involvement. Therefore, we supposed that the symmetry of lesions and cerebellar or brainstem involvement might be used as a prognostic indicator.

Encephalomalacia↗

Multiple cystic encephalomalacia of infancy: computed tomographic findings in two cases with associated intracerebral calcification.

Two initially healthy infants developed acute encephalopathic illnesses characterized by stupor, seizures, cerebrospinal fluid (CSF) erythrocytic and monocytic pleocytosis, increased CSF protein, and decreased CSF glucose and progression to chronic decerebration. In one case, herpes simplex virus was recovered from cutaneous lesions. The initial computed tomography (CT) scan revealed widespread subcortical increased attenuation with further increase after contrast medium injection and patchy areas of decreased attenuation in the deep cerebral white matter. Subsequent CT scans demonstrated progressive cortical calcifications and persistence of low attenuation areas. Autopsy revealed multiple cystic encephalomalacia. The second infant had similar clinical, CSF, and CT findings but remains in a chronic decerebrate state at 14 months of age. The CT abnormalities seen in these patients have not been encountered in any of 13 other infants with the clinical diagnosis of meningitis or encephalitis. Multiple cystic encephalomalacia of infancy is a rare condition with a uniformly bleak prognosis. Computed tomography may prove useful in the early diagnosis, thereby aiding clinicians in counseling and in the acute and long-term management of patients with this lesion.

Brain Diseases↗