title: Landau-Kleffner syndrome: a study of 29 patients.
abstract: OBJECTIVE
  The aim of the study was to retrospectively analyze the electroclinical features, etiology, treatment, and prognosis of 29 patients with Landau-Kleffner syndrome (LKS) with a long-term follow-up.
  
  
  METHODS
  Inclusion criteria were a diagnosis of LKS with: (1) acquired aphasia or verbal auditory aphasia; (2) with or without focal seizures, secondarily generalized tonic-clonic seizures, absences, or atonic seizures.
  
  
  RESULTS
  Mean follow-up was 12 years. All cases except six had seizures. Before the onset of aphasia, developmental language and behavioral disturbances were present in 19 and 14 patients, respectively. All patients had verbal auditory agnosia. Aphasia was severe in 24 patients and moderate in five. Nonlinguistic cognitive dysfunctions were moderate in 14 patients. Behavioral disturbances were found in 16 patients. During the continuous spike-and-wave discharges during slow sleep phase, the spike-wave index was >85% in 15, 50-85% in eight, and 30-50% in four. In two patients, the EEG recording showed occasional bilateral spikes, without continuous spike-and-wave discharges during slow sleep. In this phase, the awake EEG recording showed more frequent interictal abnormalities, predominantly in the temporal regions. Eight patients recovered language completely, but the remaining patients continue to have language deficits of different degrees.
  
  
  CONCLUSIONS
  Landau-Kleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most of the patients associated with continuous or almost continuous spike-and-wave discharges during slow wave sleep. The most commonly used treatments were clobazam, ethosuximide, sulthiame. High-dose steroids were also administered. Adequate and early management may avoid language and cognitive deterioration.
authors: Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina. Electronic address: rhcaraballo@arnet.com.ar.
authors: Roberto Horacio
authors: RH
authors: Caraballo
authors: Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
authors: Natalia
authors: N
authors: Cejas
authors: Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
authors: Noelia
authors: N
authors: Chamorro
authors: Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
authors: María C
authors: MC
authors: Kaltenmeier
authors: Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
authors: Sebastian
authors: S
authors: Fortini
authors: Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
authors: Ana María
authors: AM
authors: Soprano
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use; D001037:Aphasia / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D001921:Brain / Q000187:drug effects / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012640:Seizures / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D012890:Sleep; D013256:Steroids / Q000627:therapeutic use; D013997:Time Factors; D055815:Young Adult
chemical_list: D000927:Anticonvulsants; D013256:Steroids
keywords: Acquired aphasia; CSWS/ESES; Children; Landau–Kleffner syndrome; Treatment; Verbal auditory aphasia