Landau-kleffner syndrome-A rare experience

Year 2012, Volume: 17 Issue: 1, 36 - 39, 24.01.2013

Ramnath Santosh Ramanathan Tina Ahluwalia Ankush Sharma

Abstract

Abstract. Landau Kleffner syndrome (LKS)/Acquired epileptic aphasia (AEA) typically develops in healthy children who acutely or progressively lose receptive and expressive language ability coincident with the appearance of paroxysmal EEG changes. In most cases described in detail, a clearly normal period of motor and language development occurs before LKS symptoms appear. The same was the case with our patient also. LKS should be differentiated from autism with minimal language regression, especially when it is associated with isolated EEG abnormalities. Currently LKS is considered as part of the syndrome of electrical status epilepticus of sleep (ESES) by many, which is also known as continuous spike and wave of slow-wave sleep (CSWS). We also have increasing evidence that the language disorder is directly caused by epileptic discharges in critical language areas which must be viewed as a special kind of epileptic aphasia. We present a case of 5-year-old child admitted with sudden loss of speech along with seizures in the form of staring look and classical EEG abnormalities of Landau Kleffner syndrome. We started our patients on high dose oral steroids following failure of intravenous immunoglobulin trial. There are small numbers of publications reviewing Landau-Kleffner syndrome in the literature hence making it one of those rarer entities.

Key words: Landau Kleffner Syndrome, Acquired epileptic aphasia, seizures, Continuous spike and wave of slow wave sleep, Electrical status epilepticus of sleep.

Keywords

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References

• Deonna TW. Acquired epileptiform aphasia in children (Landau-Kleffner syndrome). J Clin Neurophysiol 1991; 8: 288-298.
• Arts WF, Aarsen FK, Scheltens-de Boer M, Catsman- Berrevoets CE. Landau-Kleffner syndrome and CSWS syndrome: treatment with intravenous immunoglobulins. Epilepsia 2009; 50: 55-58.
• Steinlein OK. Epilepsy-aphasia syndromes. Expert Rev Neurother. 2009; 9: 825-833.
• Billard C, Fluss J, Pinton F. Specific language impairment versus Landau-Kleffner syndrome. Epilepsia 2009; 50: 21-24.
• Bourgeois BF. Clinical aspects of epilepsy including diagnosis, management, pharmacotherapy, and surgery. Curr Opin Neurol Neurosurg 1993; 6: 233- 239.
• Nickels K, Wirrell E. Electrical status epilepticus in sleep. Semin Pediatr Neurol 2008; 15: 50-60.
• Arts WF, Geerts AT. When to start drug treatment for childhood epilepsy: the clinical-epidemiological evidence. Eur J Paediatr Neurol 2009; 13: 93-101.
• Gallagher S, Weiss S, Oram Cardy J, et al. Efficacy of very high dose steroid treatment in a case of Landau-Kleffner syndrome. Dev Med Child Neurol 2006; 48: 766-769.
• Szabó L, Nagy J, Kálmánchey R. Adrenocorticotropic hormone therapy in acquired childhood epileptic aphasia. Ideggyogy Sz 2008; 61: 409-416.