Language regression as a manifestation of epilepsy


  • Joana Ferreira Department of Pediatrics, Hospital Senhora da Oliveira - Guimarães
  • Sofia Lopes Department of Pediatrics, Hospital Senhora da Oliveira - Guimarães
  • José Carmona Lopes Department of Neurology, Hospital Senhora da Oliveira - Guimarães
  • Cristina Ferreira Department of Pediatrics, Hospital Senhora da Oliveira - Guimarães
  • Catarina Magalhães Department of Pediatrics, Hospital Senhora da Oliveira - Guimarães



cognition, epilepsy, epileptic syndromes, Landau-Kleffner syndrome


Introduction: Childhood epileptic encephalopathies are age-dependent brain disorders in which ictal and interictal epileptogenic activity is the apparent cause of progressive cognitive and neuro-psychological impairment.

Case report: A previously healthy four-year-old boy presented to the Emergency Department with a history of receptive and expressive language regression with four days of evolution, associated with seizure onset. Clinical features and electroencephalographic findings led to diagnosis of Landau-Kleffner syndrome. The boy was treated with valproate, clobazam, and prednisolone, with language improvement.

Discussion/Conclusion: Landau-Kleffner syndrome is a rare epileptic encephalopathy with pathognomonic sudden aphasia, epilepsy, and paroxysmal electroencephalographic abnormalities. The condition should be suspected in children with normal development who show a deterioration of established language skills. Early diagnosis and treatment are important to improve outcome.


Download data is not yet available.


Landau WM, Kleffner FR. Syndrome of acquired aphasia with convulsive disorder in children. Neurology. 1957; 7:523–30.

Berg AT, Berkovic SF, Brodie MJ, Buchhalter J, Cross JH, van Emde Boas W, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005–2009. Epilepsia. 2010; 51:676–85.

Landau WM, Kleffner FR. The Landau-Kleffner syndrome. Epilepsia. 2009; 50:3.

Wilfong A. Epilepsy syndromes in children [accessed 25 March 2020]. Available at:

Caraballo RH, Veggiotti P, Kaltenmeier MC, Piazza E, Gamboni B, Lopez Avaria MF, et al. Encephalopathy with status epilepticus during sleep or continuous spikes and waves during slow sleep syndrome: a multicenter, long-term follow-up study of 117 patients. Epilepsy Res. 2013; 105:164-73.

Stefanatos G.Changing perspectives on Landau-Kleffner syndrome. Clin Neuropsychol. 2011; 25:963-88.

Motwani N, Afsar S, Dixit N, Sharma N. Landau-Kleffner syndrome: an uncommon dealt with case in Southeast Asia. BMJ Case Rep. 2015.

Pearl PL, Carrazana EJ, Holmes GL. The Landau-Kleffner syndrome. Epilepsy Curr. 2001; 1:39–45.

Ballaban-Gil K. Language disorders and epilepsy. Pedley TA, Meldrum BS eds. Recente advances in epilepsy. 2nd ed. London: Churchill Livingstone, 1998:205-19.

Maquet P, Hirsch E, Dive D, Salmon E, Marescaux C, Franck G. Cerebral glucose utilization during sleep in Landau-Kleffner syndrome: a PET study. Epilepsia. 1990; 31:778.

Takeoka M, Riviello JJ Jr, Duffy FH, Kim F, Kennedy DN, Makris N, et al. Bilateral volume reduction of the superior temporal areas in Landau-Kleffner syndrome. Neurology. 2004; 63:1289.

Bourgeois BF, Landau WM. Landau-Kleffner syndrome and temporal cortical volume reduction: cause or effect? Neurology. 2004; 63:1152.

Tasinari CA, Cantalupo G, Dallabernardina B, Darra F, Bureau M, Cirelli C, et al. Encephalopathy related to status epilepticus during slow sleep (ESES) including Landau-Kleffner syndrome. Epileptic Syndromes in Infancy, Childhood and Adolescence 5th ed John Libbey Eurotext Chapter: 16.

Conroy J, McGettigan PA, McCreary D, Shah N, Collins K, Parry-Fielder B, et al. Towards the identification of a genetic basis for Landau-Kleffner syndrome. Epilepsia. 2014; 55:858-65.

Szepetowski P. Genetics Studies in Idiopathic Focal Epilepsies in Childhood. J Pediatr Epilepsy 2016; 05:139-41.

Hughes Jr. A review of the relationships between Landau-Kleffner syndrome, electrical status epilepticus during sleep, and continuous spike-waves during sleep. Epilepsy Behav. 2011; 20:247-53.

Marescaux C, Hirsch E, Finck S, Maquet P, Schlumberger E, Sellal F, et al. Landau-Kleffner syndrome: a pharmacologic study of five cases. Epilepsia. 1990; 31: 768–77.

Arts WF, Aarsen FK, Scheltens-de Boer M et al. Landau-Kleffner syndrome and CSWS syndrome: treatment with intravenous immunoglobulins. Epilepsia 2009; 50:55-8.

Cross JH, Neville BG. The surgical treatment of Landau-Kleffner syndrome. Epilepsia. 2009; 50:63-7.

Van Bogaert P, Aeby A, De Borchgrave V, De Cocq C, Deprez M, De Tiège X, et al. The epileptic syndromes with continuous spikes and waves during slow sleep: definition and management guidelines. Acta Neurol Belg. 2006; 106:52-60.




How to Cite

Ferreira J, Lopes S, Lopes JC, Ferreira C, Magalhães C. Language regression as a manifestation of epilepsy. REVNEC [Internet]. 2021Jun.30 [cited 2022Aug.7];30(2):99-104. Available from:



Case Reports

Most read articles by the same author(s)

1 2 > >>