Cases reported "Encephalomalacia"

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1/8. Cranio-cerebral erosion: delayed diagnosis and treatment.

    Cranio-cerebral erosion is a well-known complication of calvarian fracture with underlying dural tear and cerebral injury in infancy and early childhood. The anatomy, pathogenesis and natural evolution of these lesions remain obscure. The common clinical symptoms are seizures, focal neurological deficits, impairment of consciousness and a soft subgaleal mass. Three patients of cranio-cerebral erosion who underwent delayed surgery in their adult lives are presented to illustrate the common and uncommon features, and their long-term outcome is discussed.
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keywords = seizure
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2/8. Isolated sulphite oxidase deficiency: clinical and biochemical features in an Italian patient.

    A patient with isolated sulphite oxidase deficiency presented with seizures at 12 h of life and followed a severe course, dying at 10 months of age. There was mild facial dysmorphism and the brain showed multiple cystic fibrosis.
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3/8. 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.
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ranking = 481.98994299822
keywords = seizure disorder, seizure
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4/8. Neonatal periventricular leukomalacia: real-time sonographic diagnosis with CT correlation.

    The utility of real-time sonography in the diagnosis of neonatal periventricular leukomalacia (PVL) has been described only recently. Six cases are reported of PVL diagnosed by serial real-time scanning. The sonographic findings were correlated with the computed tomographic findings and the clinical history. In five of six infants in whom scanning was performed, characteristic multiseptated periventricular cavitations developed 2-3 weeks after birth or later. A transition from normal to increased periventricular echogenicity was often observed before the development of the periventricular cavitations in nonhemorrhagic PVL. The parenchymal abnormality demonstrated by sonography correlated well with an abnormal neurologic outcome. It is suggested that serial real-time scanning be performed in neonates whose history suggests the possibility of hypoxic-ischemic brain injury. Nonspecific predictors of PVL include seizures, apnea, disturbed mental status, abnormal muscle tone, and leg weakness.
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keywords = seizure
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5/8. Demonstration of a little known cause of infantile epilepsy, occipital porencephaly, by computerized tomography (CT).

    Among 500 epileptic patients studied by Computerized tomography, 11 patients presented an occipital porencephaly (or a dilation of the occipital horn with a "porencephalic" aspect) sometimes associated with a ventricular dilation. These 11 patients represented 2.2% of our patients and 5% of those less than 20 yr of age which is frequent in relation to lesions of the same type having a different topography. Clinical and EEG studies of these 11 patients revealed hemiplegia or hemiparesis in 8 cases, hemianopsia in 7 cases, a severe partial epilepsy in 6 cases which was temporal in 5 patients, a secondary generalized epilepsy in 3 cases, and an epilepsy unable to be classified in 2 cases. The "porencephalies" responsible for these clinical signs are most likely of encephalomalacic origin, secondary to circulatory troubles in the peri- or postnatal period. Remillard et al. maintain that perinatal occlusion of the posterior cerebral artery is responsible. However, in our personal series, the results obtained from CT, PEG and angiography do not permit us to be as affirmative as to the vascular etiology responsible.
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ranking = 0.5967367294307
keywords = epilepsy
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6/8. 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.
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ranking = 1
keywords = seizure
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7/8. seizures and 'disappearing' brain lesions.

    BACKGROUND--patients with seizures may have abnormal brain imaging. Lesions demonstrated on computerized tomography or magnetic resonance imaging often suggest the underlying cause of the seizures, and may prompt an invasive investigation for diagnosis and treatment. In an increasingly recognized subset of patients however, the imaged lesions spontaneously resolve. While poorly understood, these 'disappearing' lesions may in fact be the consequence of seizures, rather than the cause. Two patients with 'disappearing' lesions are presented: the first patient had a proven cause, the second a probable one. Case 1. A patient with new onset seizures had an abnormal MRI. An angiogram confirmed thrombosis of the straight sinus. On follow-up, she was doing well; repeat imaging was normal with resolution of the lesion. Case 2. A patient with a long seizure history had been well controlled on medications, and had no change in frequency of her seizures. She was found to have a left homonymous hemianopsia. MRI revealed a right parieto-occipital lesion. The patient refused further work-up and was lost to follow-up. She returned almost a year later; repeat imaging was normal. CONCLUSION--patients with seizures and abnormal imaging suggestive of structural lesions may occasionally have lesions that resolve spontaneously. While some diagnoses remain elusive, an important consideration to exclude in these patients is venous thrombosis.
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ranking = 7
keywords = seizure
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8/8. Multicystic encephalomalacia in a surviving monochorionic twin.

    From a recent monochorionic diamniotic twin pregnancy seen at this hospital, one of the twins died in utero at 37 weeks' gestation. The other twin, a male infant, was delivered by cesarean section because of fetal distress, with resuscitation performed in the delivery room. The infant developed generalized tonic seizure shortly after stabilization, and was put on anticonvulsants. The initial brain echography was normal; follow-up echograms and CT scans performed at 8 and 12 days old, respectively, revealed diffuse low density over both side of cerebral hemisphere. At one month old, the infants's brain echogram showed diffuse cystic encephalomalacia. At four months, he was noted to be spastic and significantly delayed in neurodevelopment. disseminated intravascular coagulation caused by fetal-to-fetal transfer of thromboplastic material from the dead fetus was considered as the most possible cause of the neurological complication in this patient. In addition, perinatal hypoxic-ischemic insult may also have been a superimposed, influencing factor.
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ranking = 1
keywords = seizure
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