11/687. Severe cerebral venous sinus thrombosis and dural arteriovenous fistula in an infant with protein s deficiency.A 12-month-old infant presented with cerebral seizures and neurological deficits. MRI scan of the brain and angiography showed massive cerebral venous sinus thrombosis complicated by a dural arteriovenous fistula. Subsequent clotting analysis revealed a protein s deficiency. Screening for inherited coagulation inhibitor deficiency is recommended in children with unexplained or atypical thrombotic events.- - - - - - - - - - ranking = 1keywords = cerebral, brain (Clic here for more details about this article) |
12/687. Postpartum hypertension and convulsion after oxytocic drugs.An 18-year-old primipara developed acute hypertension leading to cerebral edema and convulsions following the IV injection of a bolus of 10 units of oxytocin with 0.2 mg methylergonovine maleate. oxytocin in a dose of more than 2 units should not be administered IV in a single injection, as severe hypotension may result. If oxytocin is required, it can be injected either IM, or by IV pump or drip. The use of ergot in obstetrics should be limited to the treatment of life-threatening postpartum hemorrhage and be given only by the IM route. Ergot should not be administered to patients with cardiac, renal, or hypertensive disease, or in association with a vasoconstrictor.- - - - - - - - - - ranking = 0.16617458802749keywords = cerebral (Clic here for more details about this article) |
13/687. Instructive case report. A 26-year-old Indian woman with seizures and multiple intracranial mass lesions.We describe the case of a 26-year-old Indian woman who presented to our institution with seizures and papilledema. Her diagnosis was originally thought to be neurocysticercosis, but later confirmed to be intracranial tuberculoma. Antituberculous therapy with isoniazid, rifampin, pyrazinamide and ethambutol was initiated. Improvement in the patient's neurological symptoms with diminution in size of the intracerebral lesions was observed. This case illustrates the difficulty in distinguishing intracranial tuberculoma from neurocysticercosis. Radiological features that distinguish these two diseases are described. Both diseases must be considered in regions endemic for tuberculosis and cysticercosis.- - - - - - - - - - ranking = 0.17342010781686keywords = cerebral, intracerebral (Clic here for more details about this article) |
14/687. Behavioral psychophysiological intervention in a mentally retarded epileptic patient with brain lesion.Behavioral psychophysiological treatment entailing Slow Cortical Potential (SCP) biofeedback training and behavioral self-control training was conducted with a 27-year-old male epileptic patient (seizures for 23 years) with Wechsler IQ 64 who underwent callosotomy. The patient had 12/week secondary generalized tonic-clonic seizures. The treatment, consisting of 43 SCP training sessions and 22 behavioral control sessions, yielded a highly significant reduction of seizure frequency to about 7.5/week; such a decrease had never been observed after administration of new anticonvulsant drugs, nor after the callosotomy. During SCP feedback training, the patient was able to produce highly-significant cortical differentiation of SCPs of about 4 microV. In addition, he developed several new behaviors indicating growing ability of self-perception and self-regulation. These findings suggest that a combination of SCP biofeedback with behavioral treatment of epilepsy can be used even in mentally retarded patients with organic brain disorders.- - - - - - - - - - ranking = 0.014762359175287keywords = brain (Clic here for more details about this article) |
15/687. Genetic factors in human sleep disorders with special reference to Norrie disease, prader-willi syndrome and Moebius syndrome.Sleep-wake problems are common in specific inborn errors of metabolism and structure of the central nervous system. Psychological factors, behavioural difficulties, metabolic disturbances, and widespread rather than focal damage to the nervous system are present in many of these diseases and all influence the sleep-wake cycle. However, a number of conditions cause relatively focal damage to the neuroanatomical substrate of sleeping and waking. These include fatal familial insomnia, with involvement of the prion protein gene on chromosome 20, Norrie disease, the prader-willi syndrome and the Moebius syndrome. The last three important conditions, although rare, are considered in detail in this review. They result in sensory deprivation, hypothalamic and mid-brain damage, and involve the X-chromosome, chromosome 15, and chromosome 13, respectively. These conditions cause a wide variety of sleep disturbance, including parasomnias, daytime sleepiness, and a condition like cataplexy. The place of the relevant gene products in normal sleep regulation needs further exploration.- - - - - - - - - - ranking = 0.0029524718350574keywords = brain (Clic here for more details about this article) |
16/687. X-linked mental retardation syndrome with seizures, hypogammaglobulinemia, and progressive gait disturbance is regionally mapped between xq21.33 and Xq23.We identified a family with three males in two generations with moderate mental retardation. The two oldest were first cousins whose mothers were sisters. The third affected was a grandson through a daughter of one of the sisters, strongly suggesting X- linked inheritance. The affected males had prominent glabella, synophrys, prognathism, generalized hirsutism, and bilateral single palmar creases. All developed seizures in childhood. The two oldest have had a slow deterioration in neurological status with poor gait and balance and progressive weakness. No deterioration in their mental status has been observed. The oldest had cerebellar atrophy confirmed on computed tomography and magnetic resonance imaging scans of the brain and prolonged nerve conduction velocity. Two of the males had hypogammaglobulinemia (IgA deficient). Two-point linkage analysis using 27 microsatellite markers on the x chromosome resulted in a maximum lod score of 2.23 at straight theta = 0 for locus DSX101. Recombination was observed at locus DSX1170 in Xq21.33 and locus DXS8067 in Xq23. We conclude that this family represents an X-linked disorder associated with a recognizable phenotype, progressive neurological deterioration, and variable hypogammaglobulinemia. The gene appears to lie between Xq21.33 and Xq23.- - - - - - - - - - ranking = 0.0029524718350574keywords = brain (Clic here for more details about this article) |
17/687. Transient MRI enhancement in a patient with seizures and previously resected glioma: use of MRS.A 35-year-old man presented with partial seizures 10 years after resection of a left-sided glioblastoma multiforme. At the old operative site MRI demonstrated extensive cortical and white matter gadolinium enhancement, and PET showed hypermetabolism. biopsy of the area was postponed when MRS showed a normal biochemical spectrum. MRI and PET abnormalities resolved after control of the seizures. MRS is noninvasive and can provide essential information in the management of patients with seizures and previously treated cerebral neoplasms.- - - - - - - - - - ranking = 0.16617458802749keywords = cerebral (Clic here for more details about this article) |
18/687. Early rebleeding from intracranial dural arteriovenous fistulas: report of 20 cases and review of the literature.OBJECT: In this study the authors sought to estimate the frequency, seriousness, and delay of rebleeding in a homogeneous series of 20 patients whom they treated between May 1987 and May 1997 for arteriovenous fistulas (AVFs) that were revealed by intracranial hemorrhage (ICH). The natural history of intracranial dural AVFs remains obscure. In many studies attempts have been made to evaluate the risk of spontaneous hemorrhage, especially as a function of the pattern of venous drainage: a higher occurrence of bleeding was reported in AVFs with retrograde cortical venous drainage, with an overall estimated rate of 1.8% per year in the largest series in the literature. However, very few studies have been designed to establish the risk of rebleeding, an omission that the authors seek to remedy. methods: Presenting symptoms in the 20 patients (17 men and three women, mean age 54 years) were acute headache in 12 patients (60%), acute neurological deficit in eight (40%), loss of consciousness in five (25%), and generalized seizures in one (5%). Results of the clinical examination were normal in five patients and demonstrated a neurological deficit in 12 and coma in three. Computerized tomography scanning revealed intracranial bleeding in all cases (15 intraparenchymal hematomas, three subarachnoid hemorrhages, and two subdural hematomas). A diagnosis of AVF was made with the aid of angiographic studies in 19 patients, whereas it was a perioperative discovery in the remaining patient. There were 12 Type III and eight Type IV AVFs according to the revised classification of Djindjian and Merland, which meant that all AVFs in this study had retrograde cortical venous drainage. The mean duration between the first hemorrhage and treatment was 20 days. Seven patients (35%) presented with acute worsening during this delay due to radiologically proven early rebleeding. Treatment consisted of surgery alone in 10 patients, combined embolization and surgery in eight, embolization only in one, and stereotactic radiosurgery in one. Three patients died, one worsened, and in 16 (80%) neurological status improved, with 15 of 16 AVFs totally occluded on repeated angiographic studies (median follow up 10 months). CONCLUSIONS: The authors found that AVFs with retrograde cortical venous drainage present a high risk of early rebleeding (35% within 2 weeks after the first hemorrhage), with graver consequences than the first hemorrhage. They therefore advocate complete and early treatment in all cases of AVF with cortical venous drainage revealed by an ICH.- - - - - - - - - - ranking = 0.00057727319576667keywords = parenchymal (Clic here for more details about this article) |
19/687. Pneumoencephalus and convulsions after ventriculoscopy: a potentially catastrophic complication.A nine-year-old boy with hydrocephalus underwent ventriculoscopy under general anesthesia. After introduction of ventriculoscope the patient had sudden bradycardia, hypotension, and shrinkage of ipsilateral cerebral hemisphere. The ventriculostomy was abandoned. At the end of anesthesia and endotracheal extubation, the patient developed generalized convulsions. Reexploration of wound did not reveal anything significant; however, postoperative CT scan of head showed massive pneumoencephalus. The patients received elective ventilation of lungs for 24 hours and made complete recovery. The authors describe the reasons for these complications and further management.- - - - - - - - - - ranking = 0.16617458802749keywords = cerebral (Clic here for more details about this article) |
20/687. Deletion including the oligophrenin-1 gene associated with enlarged cerebral ventricles, cerebellar hypoplasia, seizures and ataxia.Non-specific X-linked mental retardation is a heterogeneous group of disorders with an incidence of approximately 1 in 500 males. A recently identified gene in Xq12, encoding a Rho-GTPase-activating protein, was found to be mutated in individuals with mental retardation. We describe here two sisters with a 46,XY karyotype and a microdeletion of the oligophrenin-1 gene and 1.1 Mb of flanking dna. We have characterised the molecular interval defining this microdeletion syndrome with the fibre-FISH technique. A visual physical map of 1.2 Mb was constructed which spans the oligophrenin-1 gene and the androgen receptor gene. The analysis of the patients revealed a deletion which extended from the 5' end of the AR gene to a region approximately 80 kb proximal to the EPLG2 gene. The clinical manifestations of the two sisters include psychomotor retardation, seizures, ataxia, hypotonia and complete androgen insensitivity. Cranial MRI scans show enlargement of the cerebral ventricles and cerebellar hypoplasia. Our findings give further support for the involvement of the oligophrenin-1 gene in specific morphological abnormalities of the brain which is of importance in the investigation of male patients presenting with mental retardation. In combination with our results from physical mapping we suggest that a region around the oligophrenin-1 locus is relatively bereft of vital genes.- - - - - - - - - - ranking = 0.83382541197251keywords = cerebral, brain (Clic here for more details about this article) |
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