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Management of Landau-Kleffner syndrome

Show simple item record Mikati M.A. Shamseddine A.N.
dc.contributor.editor 2005 2017-10-05T16:01:25Z 2017-10-05T16:01:25Z 2005
dc.identifier 10.2165/00148581-200507060-00006
dc.identifier.issn 11745878
dc.description.abstract Landau-Kleffner syndrome (LKS) is an acquired epileptic aphasia disorder in which children, usually 3-8 years of age who have developed age-appropriate speech, experience language regression with verbal auditory agnosia, abnormal epileptiform activity, behavioral disturbances, and sometimes overt seizures. There are no controlled clinical trials investigating the therapeutic options for LKS. Only open-label data are available. Early diagnosis and initiation of prompt medical treatment appear to be important to achieving better long-term prognosis. Several antiepileptic drugs have been reported to be beneficial in treating this syndrome. These include valproic acid (valproate sodium), diazepam, ethosuximide, clobazam, and clonazepam. Reports on the efficacy of lamotrigine, sultiame, felbamate, nicardipine, vigabatrin, levetiracetam, vagal nerve stimulation, and a ketogenic diet are few and more experience is needed. Carbamazepine and possibly phenobarbital and phenytoin have been reported to occasionally exacerbate the syndrome. As initial therapy, valproic acid or diazepam is often empirically chosen. Subsequently, other antiepileptic drugs, corticosteroids, or intravenous immunoglobulin (IVIG) therapy are often used. Corticosteroid therapy should probably not be delayed more than 1-2 months after the initial diagnosis. Various corticosteroid regimens including oral prednisone and, recently, high doses of intravenous pulse corticosteroids, as well as corticotropin (adrenocorticotropic hormone) have been reported to be effective in LKS. Oral corticosteroids are used more often and usually need to be maintained for a long period of time to prevent relapses. The use of IVIG has been associated with an initial dramatic response in only a few patients. In our experience, a long-term worthwhile improvement has been noted in only 2 of 11 patients. These two patients had an immediate response to IVIG initially and after relapses before eventually achieving a long-term sustained remission. Surgical treatment by multiple subpial transection, which is reserved for patients who have not responded to multiple medical therapies, has been followed in selected cases by a marked improvement in language skills and behavior. However, a widely accepted consensus about suitable candidates for this surgery and about its efficacy is still lacking. Speech therapy, including sign language, and a number of classroom and behavioral interventions are helpful in managing LKS, and should be used in all patients. © 2005 Adis Data Information BV. All rights reserved.
dc.format.extent Pages: (377-389)
dc.language English
dc.relation.ispartof Publication Name: Pediatric Drugs; Publication Year: 2005; Volume: 7; no. 6; Pages: (377-389);
dc.source Scopus
dc.title Management of Landau-Kleffner syndrome
dc.type Review
dc.contributor.affiliation Mikati, M.A., Department of Pediatrics, Faculty of Medicine, American University of Beirut, Beirut, Lebanon, American University of Beirut, 3 Dag Hammarskjold Plaza, New York, NY 10017-2303, United States
dc.contributor.affiliation Shamseddine, A.N., Department of Pediatrics, Faculty of Medicine, American University of Beirut, Beirut, Lebanon
dc.contributor.authorAddress Mikati, M.A.; American University of Beirut, 3 Dag Hammarskjold Plaza, New York, NY 10017-2303, United States; email:
dc.contributor.authorCorporate University: American University of Beirut Medical Center; Faculty: Faculty of Medicine; Department: Pediatrics and Adolescent Medicine;
dc.contributor.authorDepartment Pediatrics and Adolescent Medicine
dc.contributor.authorFaculty Faculty of Medicine
dc.contributor.authorUniversity American University of Beirut Medical Center
dc.description.citedCount 39
dc.format.extentCount 13
dc.identifier.coden PTDGF
dc.identifier.pubmedID 16356025
dc.identifier.scopusID 29144451510
dc.relation.ispartOfIssue 6
dc.relation.ispartofPubTitle Pediatric Drugs
dc.relation.ispartofPubTitleAbbr Pediatr. Drugs
dc.relation.ispartOfVolume 7
dc.type.publication Journal
dc.subject.otherChemCAS benzodiazepine, 12794-10-4
dc.subject.otherChemCAS carbamazepine, 298-46-4, 8047-84-5
dc.subject.otherChemCAS clobazam, 22316-47-8
dc.subject.otherChemCAS clonazepam, 1622-61-3
dc.subject.otherChemCAS corticotropin, 11136-52-0, 9002-60-2, 9061-27-2
dc.subject.otherChemCAS dexamethasone, 50-02-2
dc.subject.otherChemCAS diazepam, 439-14-5
dc.subject.otherChemCAS ethosuximide, 77-67-8
dc.subject.otherChemCAS etiracetam, 102767-28-2, 33996-58-6
dc.subject.otherChemCAS felbamate, 25451-15-4
dc.subject.otherChemCAS immunoglobulin, 9007-83-4
dc.subject.otherChemCAS lamotrigine, 84057-84-1
dc.subject.otherChemCAS methylprednisolone, 6923-42-8, 83-43-2
dc.subject.otherChemCAS nicardipine, 54527-84-3, 55985-32-5
dc.subject.otherChemCAS phenobarbital, 50-06-6, 57-30-7, 8028-68-0
dc.subject.otherChemCAS phenytoin, 57-41-0, 630-93-3
dc.subject.otherChemCAS prednisone, 53-03-2
dc.subject.otherChemCAS primidone, 125-33-7
dc.subject.otherChemCAS sultiame, 61-56-3
dc.subject.otherChemCAS topiramate, 97240-79-4
dc.subject.otherChemCAS valproic acid, 1069-66-5, 99-66-1
dc.subject.otherChemCAS vigabatrin, 60643-86-9
dc.subject.otherChemCAS Adrenocorticotropic Hormone, 9002-60-2
dc.subject.otherChemCAS Anticonvulsants
dc.subject.otherChemCAS Glucocorticoids
dc.subject.otherChemCAS Immunoglobulins, Intravenous
dc.subject.otherIndex anticonvulsive agent
dc.subject.otherIndex benzodiazepine
dc.subject.otherIndex carbamazepine
dc.subject.otherIndex clobazam
dc.subject.otherIndex clonazepam
dc.subject.otherIndex corticosteroid
dc.subject.otherIndex corticotropin
dc.subject.otherIndex dexamethasone
dc.subject.otherIndex diazepam
dc.subject.otherIndex ethosuximide
dc.subject.otherIndex etiracetam
dc.subject.otherIndex felbamate
dc.subject.otherIndex immunoglobulin
dc.subject.otherIndex lamotrigine
dc.subject.otherIndex methylprednisolone
dc.subject.otherIndex nicardipine
dc.subject.otherIndex phenobarbital
dc.subject.otherIndex phenytoin
dc.subject.otherIndex prednisone
dc.subject.otherIndex primidone
dc.subject.otherIndex sultiame
dc.subject.otherIndex topiramate
dc.subject.otherIndex valproic acid
dc.subject.otherIndex vigabatrin
dc.subject.otherIndex agnosia
dc.subject.otherIndex aphasia
dc.subject.otherIndex avascular necrosis
dc.subject.otherIndex behavior disorder
dc.subject.otherIndex behavior therapy
dc.subject.otherIndex child
dc.subject.otherIndex corticosteroid therapy
dc.subject.otherIndex drug efficacy
dc.subject.otherIndex drug response
dc.subject.otherIndex epilepsy
dc.subject.otherIndex epileptic discharge
dc.subject.otherIndex human
dc.subject.otherIndex hyperglycemia
dc.subject.otherIndex hypertension
dc.subject.otherIndex immune deficiency
dc.subject.otherIndex ketogenic diet
dc.subject.otherIndex Landau Kleffner syndrome
dc.subject.otherIndex language ability
dc.subject.otherIndex language development
dc.subject.otherIndex priority journal
dc.subject.otherIndex remission
dc.subject.otherIndex review
dc.subject.otherIndex side effect
dc.subject.otherIndex speech
dc.subject.otherIndex speech therapy
dc.subject.otherIndex stomach ulcer
dc.subject.otherIndex treatment outcome
dc.subject.otherIndex vagus nerve stimulation
dc.subject.otherIndex Adrenocorticotropic Hormone
dc.subject.otherIndex Anticonvulsants
dc.subject.otherIndex Behavior Therapy
dc.subject.otherIndex Child
dc.subject.otherIndex Child, Preschool
dc.subject.otherIndex Glucocorticoids
dc.subject.otherIndex Humans
dc.subject.otherIndex Immunoglobulins, Intravenous
dc.subject.otherIndex Landau-Kleffner Syndrome
dc.subject.otherIndex Psychosurgery
dc.subject.otherIndex Speech Therapy

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