Landau-Kleffner Syndrome
Landau-Kleffner Syndrome (LKS)
Landau-Kleffner syndrome (LKS), also known as acquired epileptic aphasia, is a rare childhood neurological disorder characterized by the sudden or gradual development of aphasia (language disorder) and an abnormal electroencephalogram (EEG).
Key Points:
- Age of onset: Typically between 3-7 years
- Gender distribution: Affects both males and females equally
- Prevalence: Rare disorder, exact incidence unknown
- First described by William Landau and Frank Kleffner in 1957
Clinical Manifestations
Language Dysfunction
- Acquired aphasia
- Progressive loss of receptive language
- Verbal auditory agnosia
- Expressive language deterioration
- Language regression pattern
- Initially normal language development
- Sudden or gradual loss of language abilities
- May progress to complete mutism
Seizure Activity
- Occurs in 70-80% of cases
- Types:
- Focal seizures
- Generalized tonic-clonic seizures
- Atypical absence seizures
- Predominantly nocturnal seizures
Behavioral Changes
- Hyperactivity
- Attention deficits
- Aggression
- Social withdrawal
- Emotional lability
Diagnostic Criteria and Workup
EEG Findings
- Characteristic spike-wave discharges
- Bilateral temporal regions
- Activation during sleep
- Continuous spike-waves during slow-wave sleep (CSWS)
Required Investigations
- Serial EEG recordings (awake and sleep)
- Brain MRI
- Comprehensive neuropsychological assessment
- Audiological evaluation
- Speech and language assessment
Differential Diagnosis
- Autism spectrum disorders
- Childhood disintegrative disorder
- Hearing impairment
- Other epileptic encephalopathies
- CSWS syndrome
Treatment Approaches
Antiepileptic Medications
- First-line options:
- Valproic acid
- Levetiracetam
- Clobazam
- Second-line options:
- Ethosuximide
- Sulthiame
Immunomodulatory Therapy
- Corticosteroids
- Oral prednisolone
- ACTH
- Pulse methylprednisolone
- Intravenous immunoglobulins (IVIG)
Rehabilitation Services
- Speech and language therapy
- Occupational therapy
- Educational support
- Behavioral intervention
- Family counseling
Outcome and Follow-up
Prognostic Factors
- Favorable factors:
- Early diagnosis and treatment
- Later age of onset
- Shorter duration of active phase
- Poor prognostic factors:
- Early age of onset
- Prolonged CSWS
- Delayed treatment initiation
Long-term Outcomes
- Language recovery:
- Variable recovery patterns
- 20-30% complete recovery
- 40-50% partial recovery
- 20-30% persistent deficits
- Seizure outcomes:
- Generally good seizure control
- Most patients seizure-free by adolescence
