Language outcome following multiple subpial transection for Landau-Kleffner syndrome.
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Landau-Kleffner syndrome is an acquired epileptic aphasia occurring in normal children who lose previously acquired speech and language abilities. Although some children recover some of these abilities, many children with Landau-Kleffner syndrome have significant language impairments that persist. Multiple subpial transection is a surgical technique that has been proposed as an appropriate treatment for Landau-Kleffner syndrome in that it is designed to eliminate the capacity of cortical tissue to generate seizures or subclinical epileptiform activity, while preserving the cortical functions subserved by that tissue. We report on the speech and language outcome of 14 children who underwent multiple subpial transection for treatment of Landau-Kleffner syndrome. Eleven children demonstrated significant postoperative improvement on measures of receptive or expressive vocabulary. Results indicate that early diagnosis and treatment optimize outcome, and that gains in language function are most likely to be seen years, rather than months, after surgery. Since an appropriate control group was not available, and that the best predictor of postoperative improvements in language function was that of length of time since surgery, these data might best be used as a benchmark against other Landau-Kleffner syndrome outcome studies. We conclude that multiple subpial transection may be useful in allowing for a restoration of speech and language abilities in children diagnosed with Landau-Kleffner syndrome. (+info)
Magnetoencephalography in children with Landau-Kleffner syndrome and acquired epileptic aphasia.
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BACKGROUND AND PURPOSE: Landau-Kleffner syndrome (LKS) is epileptiform aphasia acquired during childhood and occurring in children with previously normal language development. The epileptiform activity in these children is thought to result in a functional ablation of eloquent speech areas. The purpose of this study was to investigate the usefulness of magnetoencephalography (MEG) for localizing the source of epileptiform activity in these patients. METHODS: Nineteen patients with acquired aphasia and a suspected diagnosis of LKS were referred for MEG evaluation. Patients ranged in age from 4 to 14 years. Fourteen MEG studies were performed on a 74-channel system, four on a 148-channel whole-head system, and one on a 37-channel system. RESULTS: Thirteen of the 19 patients had perisylvian MEG spikes. In 10 of the patients, the spikes were bilateral, and in three they were unilateral. Four other patients had non-sylvian spikes, and two patients had no spikes recorded. The results of MR imaging were normal or noncontributory for all 19 patients. CONCLUSIONS: MEG can play a useful role in evaluating children with LKS and acquired epileptiform aphasia, both in diagnosis and in aiding presurgical localization of epileptiform activity when surgery is being considered. (+info)
Cognitive functions and epileptic activity.
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Epilepsy, and its treatment, can affect the development and use of language in a number of different ways. The seizures may be a symptom of a lesion in areas of the brain essential for language function, and the complications of both the seizures and of the drugs can interfere with learning. However, the purpose of this review is to consider the role of epileptic activity as demonstrated by electrical discharges in the electroencephalogram (EEG), whether these are accompanied by overt seizures or not, on cerebral function especially that of language. The Landau-Kleffner, the continuous spike-waves during sleep, and the benign epilepsy of childhood with Rolandic spikes syndromes are considered; as well as the evidence of epileptic discharges affecting language development. If there are doubts that a patient is suffering from epilepsy the diagnosis may be difficult; and anyone involved in the treatment of language disorders should keep this possibility in mind. Particularly during childhood an EEG, awake and asleep, should be an important part of the assessment of both developmental and acquired dysphasia. The control of overt fits may be relatively easy, but drugs are not so effective in controlling the epileptic activity in the EEG. The treatment of such discharges used to be discouraged, but the evidence is now strongly in favour of employing both medical and surgical treatment. The results in restoring language function are sometimes dramatic. (+info)
Landau-Kleffner syndrome: study of four cases.
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We describe four patients with clinical features of Landau-Kleffner syndrome and discuss electroencephalographic features, treatment and prognosis. Anticonvulsants and prednisone were used for treatment with good control of seizures in all cases and a less effect response in acquired aphasia. Further studies are necessary to elucidate the causes and management of this syndrome. (+info)
Landau-Kleffner syndrome: a case report.
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A healthy 5 year old boy developed aphasia, attention disorder and hyperkinesia preceded by transient formed visual hallucinations and emotional outburst, immediately after a stressful event of forced separation from his father. EEG showed generalized epileptiform activity. He was diagnosed as Landau-Kleffner syndrome (LKS). CT and MRI of the brain were normal. SPECT showed left mesial temporal hypoperfusion. He improved on antiepileptics and ACTH. (+info)
Nonconvulsive status epilepticus presenting as a subacute progressive aphasia.
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We report a 62-year-old man with non-convulsive status epilepticus (NCSE) presenting as a progressive aphasia that developed insidiously over 5 weeks. On video-EEG monitoring, aggravation of the aphasia coincided with occurrence of seizure activities arising from the left fronto-temporal area. Brain MRI was noncontributory but a fluorodeoxyglucose-PET scan revealed a hypometabolism in the left anterior temporal area. Following anticonvulsant treatment, aphasia recovered gradually over several weeks despite prompt resolution of epileptic discharges on EEG. Our patient's findings, gradual onset of isolated aphasia with gradual resolution after initiation of treatment, may differ from previously reported cases with aphasic status epilepticus because their aphasia showed abrupt onset and rapid resolution with anticonvulsant medication. (+info)
The pathophysiological mechanisms of cognitive and behavioral disturbances in children with Landau-Kleffner syndrome or epilepsy with continuous spike-and-waves during slow-wave sleep.
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Epilepsy with continuous spike- and -waves during slow-wave sleep and Landau-Kleffner syndrome are two rare childhood epilepsy syndromes. The underlying pathophysiology remains unknown. The current opinions about epidemiologic risk factors, genetic predisposition, EEG and MEG findings, influence of anti-epileptic drugs, neuroradiology, positron emission tomopgraphy, evoked potentials, auto-immunity and subpial transection are summarized. (+info)
Treatment with flunitrazepam of continuous spikes and waves during slow wave sleep (CSWS) in children.
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We describe our treatment of two boys with continuous spikes and waves during slow wave sleep (CSWS). One of the boys was suffering from non-convulsive status epilepticus and the other from conscious disturbance with automatism. Their ictal EEG readings showed continuous diffuse spike and wave complexes, which were considered to show electrical status. The boys were diagnosed as having CSWS, and were later diagnosed with Landau-Kleffner syndrome (LKS). EEG readings returned to normal on intravenous injection of flunitazepam (FZP) at a dose of 0.02 mg/kg, suggesting that FZP is an effective treatment for CSWS. (+info)