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Landau-Kleffner syndrome: a study of 29 patients.
Seizure. 2014 Feb; 23(2):98-104.S

Abstract

PURPOSE

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.

CONCLUSION

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+Show Affiliations

Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina. Electronic address: rhcaraballo@arnet.com.ar.Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.Department of Neurology, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

24315829

Citation

Caraballo, Roberto Horacio, et al. "Landau-Kleffner Syndrome: a Study of 29 Patients." Seizure, vol. 23, no. 2, 2014, pp. 98-104.
Caraballo RH, Cejas N, Chamorro N, et al. Landau-Kleffner syndrome: a study of 29 patients. Seizure. 2014;23(2):98-104.
Caraballo, R. H., Cejas, N., Chamorro, N., Kaltenmeier, M. C., Fortini, S., & Soprano, A. M. (2014). Landau-Kleffner syndrome: a study of 29 patients. Seizure, 23(2), 98-104. https://doi.org/10.1016/j.seizure.2013.09.016
Caraballo RH, et al. Landau-Kleffner Syndrome: a Study of 29 Patients. Seizure. 2014;23(2):98-104. PubMed PMID: 24315829.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Landau-Kleffner syndrome: a study of 29 patients. AU - Caraballo,Roberto Horacio, AU - Cejas,Natalia, AU - Chamorro,Noelia, AU - Kaltenmeier,María C, AU - Fortini,Sebastian, AU - Soprano,Ana María, Y1 - 2013/10/08/ PY - 2013/07/23/received PY - 2013/09/24/revised PY - 2013/09/30/accepted PY - 2013/12/10/entrez PY - 2013/12/10/pubmed PY - 2014/9/30/medline KW - Acquired aphasia KW - CSWS/ESES KW - Children KW - Landau–Kleffner syndrome KW - Treatment KW - Verbal auditory aphasia SP - 98 EP - 104 JF - Seizure JO - Seizure VL - 23 IS - 2 N2 - PURPOSE: 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. CONCLUSION: 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. SN - 1532-2688 UR - https://www.unboundmedicine.com/medline/citation/24315829/Landau_Kleffner_syndrome:_a_study_of_29_patients_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1059-1311(13)00277-X DB - PRIME DP - Unbound Medicine ER -