Original articleCorticosteroids for the Treatment of Landau-Kleffner Syndrome and Continuous Spike-Wave Discharge During Sleep
Introduction
Landau-Kleffner syndrome and its variants such as continuous spike-wave discharge during sleep or electrical status epilepticus during sleep are epileptic encephalopathies of childhood. Landau-Kleffner syndrome is an acquired epileptic aphasia presenting as progressive loss of speech in a previously well child with an abnormal and usually continuously epileptic electroencephalogram (with or without seizures) [1], [2], [3]. Continuous spike-wave discharge during sleep is similar to Landau-Kleffner syndrome, although language is preserved in continuous spike-wave discharge during sleep and the patients demonstrate deterioration in cognition or behavior with an epileptic electroencephalogram, rather than speech or language problems [4], [5]. The treatment of this unusual group of patients is controversial and includes antiepileptic drugs, surgery, intravenous immunoglobulin, and corticosteroids [6]. The present report describes our experience in treating patients with Landau-Kleffner syndrome and its variants with corticosteroids.
Section snippets
Methods
Patients were diagnosed with Landau-Kleffner syndrome or continuous spike-wave discharge during sleep after language regression (Landau-Kleffner syndrome) or cognitive/behavioral deterioration with intact language (continuous spike-wave discharge during sleep) with the characteristic epileptic electroencephalogram (Fig 1) usually with continuous spike-wave discharge during sleep. Patients were then admitted to our pediatric epilepsy monitoring unit for 24-hour continuous video
Results
Ten patients, 3 females and 7 males, were studied (Table 1). Age of onset ranged from 2 to 11 years (mean 7.5 years). Eight patients manifested Landau-Kleffner syndrome, and two patients had continuous spike-wave discharge during sleep. Most patients had seizures (8/10); however, two patients did not have clinical seizures, only an abnormal and epileptic electroencephalogram. The electroencephalography overall was abnormal in all 10 patients. Abnormalities included focal epileptic abnormalities
Discussion
Landau and Kleffner in 1957 described six children who developed an acquired receptive aphasia in conjunction with an epileptic disorder. The electroencephalograms demonstrated severe paroxysmal changes, which appeared to parallel the course of language impairment [1]. This syndrome of acquired aphasia, seizures, and an epileptic electroencephalogram has become known as the Landau-Kleffner syndrome. The mechanism is thought to be a verbal auditory agnosia or “word deafness” resulting from a
Conclusion
Corticosteroids are a safe and effective treatment for patients with Landau-Kleffner syndrome and continuous spike-wave discharge during sleep. Many patients manifest improvement in language, cognition, and behavior after treatment. Side effects are few and reversible, and the benefits long lasting. Corticosteroids should be considered as a treatment option in children with Landau-Kleffner syndrome and continuous spike-wave discharge during sleep.
References (25)
- et al.
Landau-Kleffner syndromeSix patients including discordant monozygotic twins
Pediatr Neurol
(1993) - et al.
Effects of high dose intravenous corticosteroid therapy in Landau-Kleffner syndrome
Pediatr Neurol
(2000) - et al.
Case studyCorticosteroid treatment of language regression in pervasive developmental disorder
J Am Acad Child Adolesc Psychiatry
(1995) Prednisone therapy in childhood epilepsy
Pediatr Neurol
(2003)- et al.
Successful use of intravenous immunoglobulins in Landau-Kleffner syndrome
Pediatr Neurol
(1998) - et al.
Syndrome of acquired aphasia with convulsive disorder in children
Neurology
(1957) The Landau-Kleffner syndrome
Acquired epileptiform aphasia in children (Landau-Kleffner syndrome)
J Clin Neurophysiol
(1991)- et al.
Verbal auditory agnosia and seizures in children
Dev Med Child Neurol
(1977) Age of onset and outcome in “acquired aphasia with convulsive disorder” (Landau-Kleffner syndrome)
Dev Med Child Neurol
(1985)