Elsevier

Pediatric Neurology

Volume 32, Issue 5, May 2005, Pages 300-306
Pediatric Neurology

Original article
Corticosteroids for the Treatment of Landau-Kleffner Syndrome and Continuous Spike-Wave Discharge During Sleep

https://doi.org/10.1016/j.pediatrneurol.2004.12.006Get rights and content

Landau-Kleffner syndrome and its variants such as continuous Spike-Wave Discharge during Sleep (CSWS) are progressive epileptic encephalopathies of childhood. The treatment of this unusual group of patients is controversial. We describe our experience in treating patients with Landau-Kleffner syndrome and CSWS with corticosteroids. The patients received Prednisone 1 mg/kg/day for 6 months, 1 year, then yearly. Follow-up was for 1–10 years (mean 4 years). Ten patients, 3 females, 7 males were studied. Age of onset ranged from 2 to 11 years (mean 7.5 years). Eight patients manifested Landau-Kleffner syndrome, and two had CSWS. Most patients had seizures (8/10); however, two patients did not have clinical seizures. MRI was normal in all patients. SPECT scan was abnormal in four patients, normal in three, and not available in three. All but one patient manifested significant improvement in language, cognition, and behaviour, which continued after the corticosteroid trial. Side effects were few (4/10) and transient and consisted of weight gain (2), behavioral change (1), and hypertension (1). Corticosteroids are a safe and effective treatment for patients with Landau-Kleffner syndrome and CSWS. Most patients had improvement in language, cognition, and behaviour after treatment. Side effects are few and reversible, and benefits appear long lasting. Corticosteroids should be considered as a treatment option in children with Landau-Kleffner syndrome and CSWS.

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)

  • J.F. Mantovani et al.

    Acquired aphasia with convulsive disorderCourse and prognosis

    Neurology

    (1980)
  • P.F. Paguier et al.

    The Landau-Kleffner syndrome or acquired aphasia with convulsive disorderLong-term follow up of six children and review of the recent literature

    Arch Neurol

    (1992)
  • Cited by (0)

    View full text