Overview
Landau-Kleffner syndrome (LKS) is an acquired epileptic aphasia characterized by sudden or gradual loss of language abilities in previously normally developing children, often accompanied by abnormal epileptiform activity on EEG and sometimes clinical seizures 5.Diagnosis
Clinical Presentation: Sudden or rapid deterioration in speech and language abilities 1.
EEG Findings: Abnormal epileptiform activity, particularly bitemporal spike and wave discharges during sleep 135.
Objective Tests: Serial auditory-evoked potentials (AEPs) can help assess central nervous system function; behavioral audiometric testing may be non-compliant 1.
MRI: Typically normal in LKS 2.
Family History: Seizure disorders may be present 1.
Speech and Hearing Assessments: Essential for identifying language regression and auditory processing issues 12.Management
First-Line Treatments:
- Antiepileptic Drugs: Valproic acid (valproate sodium) and diazepam are commonly used empirically 4.
- Corticosteroids: High-dose prednisolone has shown efficacy in some cases 3.
Adjunctive Therapies:
- Speech and Language Therapy: Critical for addressing communication difficulties 23.
- Educational Support: Modified educational programs can be beneficial 3.
Other Treatments: Reports exist for lamotrigine, sultiame, felbamate, nicardipine, vigabatrin, levetiracetam, vagal nerve stimulation, and ketogenic diet, but more experience is needed 4.Special Populations
Pediatrics: Early diagnosis and intervention are crucial for better long-term outcomes 4.
Comorbidities: No specific management differences noted for comorbidities; multidisciplinary approach remains key 24.Key Recommendations
Initiate Early Multidisciplinary Assessment: Early diagnosis through EEG, speech assessments, and multidisciplinary evaluation improves prognosis (Evidence: Moderate 12).
Empirical Use of Valproic Acid or Diazepam: Start with valproic acid or diazepam as initial antiepileptic therapy (Evidence: Expert opinion 4).
Integrate Intensive Speech Therapy: Incorporate comprehensive speech and language interventions alongside medical treatments (Evidence: Moderate 23).
Consider High-Dose Corticosteroids: Evaluate high-dose corticosteroids for patients showing significant regression (Evidence: Weak 3).
Monitor with Serial Auditory-Evoked Potentials: Utilize serial AEPs to monitor central nervous system function and treatment response (Evidence: Moderate 1).References
1 Plyler E, Harkrider AW. Serial auditory-evoked potentials in the diagnosis and monitoring of a child with Landau-Kleffner syndrome. Journal of the American Academy of Audiology 2013. link
2 Fandiño M, Connolly M, Usher L, Palm S, Kozak FK. Landau-Kleffner syndrome: a rare auditory processing disorder series of cases and review of the literature. International journal of pediatric otorhinolaryngology 2011. link
3 Gallagher S, Weiss S, Oram Cardy J, Humphries T, Harman KE, Menascu S. Efficacy of very high dose steroid treatment in a case of Landau-Kleffner syndrome. Developmental medicine and child neurology 2006. link
4 Mikati MA, Shamseddine AN. Management of Landau-Kleffner syndrome. Paediatric drugs 2005. link
5 Mouridsen SE. The Landau-Kleffner Syndrome: a review. European child & adolescent psychiatry 1995. link