Language regression as a manifestation of epilepsy
DOI:
https://doi.org/10.25753/BirthGrowthMJ.v30.i2.17501Keywords:
cognition, epilepsy, epileptic syndromes, Landau-Kleffner syndromeAbstract
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.
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References
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: https://www.uptodate.com.
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.
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