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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four children had optic nerve hypoplasia with hypopituitarism, and their clinical picture varied with age. The newborn had apnea, hypotonia, seizures, hyopglycemia, and prolong jaundice. The young infant had defective vision, behavioral delay, hypotonia, and seizures. Except for a mildly receding lower jaw and a high-arched palate, the appearance of the patients was not unusual. The fasting blood glucose level was mildly depressed. In two cases the liver was palpable and results of liver function tests were abnormal. The older child, who was blind and mentally retarded, had growth failure. The extent of the pituitary hormone deficiencies was variable, including diabetes insipidus. The septum pellucidum was not invariably absent. Clinical and pathological findings indicate that the brain lesion might be more diffuse than hitherto recognized. Early recognition of this syndrome and timely intervention might diminish serious sequels.
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PMID:Optic nerve hypoplasia with hypopituitarism. Septo-optic dysplasia with hypopituitarism. 111 54

Five cases of incidentally detected pituitary macroadenomas are described. All five had suprasellar extensions, but none had visual field defects as tested by computed perimetry. One patient had sellar changes seen on an X-ray film taken following a head injury, while the others were detected by computed tomography performed for seizures, stroke and meningitis. Four patients underwent trans-sphenoidal surgery for suprasellar extensions with or without hypopituitarism, while one was managed conservatively as he had normal visual fields and pituitary function and there was a spontaneous decrease in size of the adenoma. The management of such incidental pituitary macroadenomas is discussed.
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PMID:Incidental pituitary macroadenomas. 163 22

We report on three infants with hand anomalies and congenital hypopituitarism. In two of the cases, a hypothalamic tumor was found; the third infant died without postmortem brain studies. Family history in the first case suggested possible familial recurrence; the mother's sister had died at 17 hr of age with polydactyly, microglossia, and flat nasal bridge (no autopsy done). Our second case was born by cesarean section after a pregnancy complicated by extremely low maternal estriols. At birth, hypopituitarism was diagnosed, a cranial CT scan was read as normal, and hormonal replacement was begun with thyroxine, hydrocortisone, and growth hormone. At 11.5 mo of age she developed seizures; and a repeat CT scan showed a mass extending beneath the hypothalamus. This tumor was removed surgically at 12 mo, the first successful treatment of this disorder. Our third possible case had a bifid epiglottis, hypopituitarism, and hand anomalies. A CT scan at birth failed to reveal a mass in the hypothalamus. This child died from complications of untreated hypopituitarism, and no neuropathology studies were done. These three cases were conceived between March 10th and April 17th in three different years in three geographically contiguous counties of Vermont. Clustering in time and space and possible familial recurrence, in one of these cases, suggest a possible gene/environment interaction.
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PMID:A cluster of Pallister-Hall syndrome cases, (congenital hypothalamic hamartoblastoma syndrome). 314

Prior to the antibiotic (AB) era, cavernous sinus thrombosis (CST) was almost uniformly fatal. AB therapy has significantly reduced mortality, but additional treatment with anticoagulants (AC) has remained controversial. We reviewed our experience with seven cases, as well as the literature since Lyons' 1941 introduction of AC treatment, to determine effectiveness, complications, and morbidity among survivors. We divided the cases into (1) those treated with AB alone, and (2) those treated with a combination of AB and AC. We found no conclusive evidence for reduction of mortality when AC was used in combination with AB. However, early AC therapy reduced morbidity (blindness, stroke, ophthalmoplegia, hypopituitarism, focal seizures, and vascular steal syndrome), whereas delayed or inadequate use provided no apparent benefit above AB therapy alone. Complications of AC therapy were rare; cerebral venous thrombosis occurred frequently, but in association with dural sinus thrombosis as a direct result of the disease. We conclude that AC therapy is indicated early in the treatment of CST to reduce morbidity among survivors.
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PMID:The role of anticoagulation in cavernous sinus thrombosis. 328 Oct 56

Hypoglycemia of infancy and childhood represents a treatable cause of mental retardation and seizures. Most neonates with hypoglycemia have transient disorders, but with persistent hypoglycemia one must consider hyperinsulinism, hypopituitarism, or hereditary hepatic enzyme deficiencies. Outside of the neonatal period, ketotic hypoglycemia is the most common cause of hypoglycemia in childhood. One cannot overemphasize the value of obtaining certain diagnostic tests at the presentation of spontaneous hypoglycemia, including blood for insulin, cortisol, growth hormone, and urine for ketone bodies. Supportive treatment with intravenous glucose to maintain the blood glucose greater than 50 mg/dl is important until a diagnosis is established allowing specific therapy aimed at the underlying disorder.
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PMID:Hypoglycemia of infancy and childhood. 330 1

Twelve patients presented with evidence of hypopituitarism in the neonatal period, but only four were correctly diagnosed at that time. Craniofacial, optic and neurological features were common. Optic hypoplasia occurred in six cases, large anterior and posterior fontanelles in four, wide sutures in four, depressed nasal bridge in three, facial palsy in three, and one infant had an asymmetrical, crying facies. Seizures occurred in three during the neonatal period and in eight at a later age. All 12 had hyperbilirubinaemia, 11 had hypoglycaemia, and micropenis occurred in five of the seven boys. This survey suggests that the incidence of neonatal hypopituitarism may have increased.
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PMID:Neonatal hypopituitarism: a neurological perspective. 338 98

The syndrome of septo-optic dysplasia with congenital hypopituitarism consists of optic nerve hypoplasia, midline malformations of the prosencephalon and hypothalamic hypopituitarism. There is great variability of these features and clinical manifestation is age-dependent: Newborns present with hypoglycemic seizures, apnea, cyanosis, hypotonia, prolonged jaundice (and micropenis in boys) because of growth hormone and/or ACTH-deficiencies. Wandering eye movements and more or less visual disturbance become evident during infancy and growth retardation even later in some cases. Early recognition is facilitated by the pathognomonic fundoscopic findings, together with normal electroretinogram, absent visually evoked potentials and computer tomography. Early hormone substitution is essential to prevent hypoglycemic damage.
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PMID:[Septo-optic dysplasia with congenital hypopituitarism (author's transl)]. 719 68

Five children had bilateral optic nerve hypoplasia, absent septum pellucidum, and hypopituitarism. Absence of the septum pellucidum was shown by computed axial tomography. All of the children were first-born. One of the children was definitely not mentally retarded and one at age 7 months was developing normally. All of the children had severe visual impairment and nystagmus. Pupillary responses varied. Ocular deviations included one with exotropia and three with esotropia. The extent of pituitary hormone deficiency varied. One child was symptomatic from birth, having severe hypoglycemia and seizures. Two children had prolonged neonatal jaundice. One unusual case had growth hormone deficiency with a normal growth curve. Another child with diabetes insipidus had dehydration and polyuria without polydipsia.
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PMID:Optic nerve hypoplasia associated with absent septum pellucidum and hypopituitarism. 735 76

MELAS syndrome is a form of mitochondrial myopathy with manifestations of seizure, stroke-like syndrome, lactic acidosis, ragged red muscle fibres and mitochondrial encephalopathy. The syndrome has been reported in association with a variety of endocrine and metabolic disorders including diabetes mellitus (DM), hypothalamo-pituitary hypofunction, hypothalamic growth hormone deficiency and delayed puberty. Mitochondrial DNA (mtDNA) point mutation may be the major pathological defect. However, association of MELAS syndrome with hyperthyroidism has not previously been reported. A case is reported from Taiwan of a 32-year-old woman suffering from MELAS syndrome with associated DM and hyperthyroidism. When the latter was diagnosed in April 1988, the patient underwent subtotal thyroidectomy. There was no family history of thyroid disease. Because of repeated seizures, she had computed tomography (CT) and magnetic resonance imaging (MRI) of the brain which showed focal, low-density lesions over the cerebral hemispheres. Both serum and cerebral spinal fluid lactic acid levels were elevated. Mild elevations of serum T4 and T3 and a high titre of TSH receptor antibody were still present. Hyperglycaemia was noted during hospitalization and DM confirmed by oral glucose tolerance test. Muscle biopsy showed ragged red fibres. DNA analysis showed an A-to-G transition at the 3243rd nucleotide position of the tRNA(Leu(UUR)) gene of the mtDNA from the patient. Quantitative polymerase chain reaction (PCR) and restriction analysis revealed that about 60% of the blood mtDNA was of mutant type. The patient received antithyroid drugs for hyperthyroidism, diet control for DM and anti-epileptic drugs for seizure.
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PMID:MELAS syndrome associated with diabetes mellitus and hyperthyroidism: a case report from Taiwan. 755 21

We report on a male epileptic patient, presently 27 years old, who has suffered complex-partial attacks for 19 years. Under treatment with carbamazepine the seizures were completely controlled. In addition, the patient exhibited partial hypopituitarism. CT and MRI revealed the presence of 2 lipomas, one located within the optico-chiasmatic cistern and the other one in the medial temporal lobe. To our knowledge, this combination of the generally rare lesions has not been described yet.
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PMID:Multiple intracranial lipoma: a case report. 945 25


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