1. Landau-Kleffner
Syndrome
Acquired Aphasia with Epilepsy
By Genevieve V. Walton

2. Objectives
●Define Landau-Kleffner Syndrome (LKS)
●Understand course of LKS
oSymptoms
oTreatment
oPrognosis
●Neurodiagnostic Studies

3. Definition
●Usual Onset Age: 3-7 y/o (18mo-13yrs)
●Children develop normally until onset
●Lose both receptive and expressive
language skills
●Retains general intelligence
●Abnormal EEG activity
o~80% have clinical seizures

4. Symptoms
●Progressive Aphasia
oReceptive (Comprehension)
oExpressive (Communication)
oOnset over weeks or months
●In some: Auditory Agnosia
●Seizures during sleep
oMay progress to ESES
●Behavioral Disorders

5. Differential Diagnosis
●Autism
●Hearing Impairment
●Learning Disability
●Auditory/Verbal Processing Disorder
●Childhood Schizophrenia
●Mental Retardation

6. Treatment
●Diet
oKetogenic
oAtkins
●Medication
oAntiepileptic Therapy
oCorticosteroids
●Surgical
oMultiple Subpial Transection
●Speech Therapy

7. Prognosis
●Varies widely
●Usually outgrown by 15 y/o
●May have residual speech difficulties
●ESES portion may persist
●Improves with early treatment

8. Epidemiology
●True cause unknown
●Male to Female ratio of 1.7:1
●1957-2002: 198 cases
●12% have a family member with epilepsy
●Theorized infection causes

9. Neurodiagnostic Studies
●EEG
oHigh voltage, repetitive spikes or spike-wave
oBackground can be normal, or show diffuse slowing
oSeen mostly during sleep
●Radiographic Imaging
oNormal unless underlying structural issue
 Such as cerebral tumors or polymicrogyria
●BAEP
oNormal, no hearing deficit

10. Case Study
●4 y/o right handed male
●Previously healthy
●Staring spells
●Rapid speech & language regression
●Onset of symptoms in June 2006
●Followed by behavioral problems
●Referred to Mayo in August 2006

11. Case Study
●Family Neurological History
oFather – No seizures or other problems
oMother – Psychomotor seizures starting at 10
y/o. Medicated until college. No seizures
since.
 Mothers aunt & maternal cousin have histories of
grand mal seizures

12. Case Study
●Tests Performed
oEEG
 Routine Wake & Sleep
 EMU - Characterization of Spells
oMRI
 With & without Gadolinium Contrast
oMetabolic
oChromosomal Analysis

13. Case Study
●Test Findings
oEEG
 Dys 3 focal left central & generalized atypical spike and wave
 Bilateral spike and wave discharges throughout most of sleep
 Consistent with Landau-Kleffner Syndrome
oMRI
 Normal
oMetabolic
 Negative
oChromosomal
 Negative

14. Activation During Wakefulness

15. Decreased Activation During Light Sleep

16. Activation of Repetitive Spike and Slow Wave

17. Activation on LAR-TPi Montage

18. Activation on TAL Montage

19. Case Study
●Treatment
oMedication
 Oral Valproic Acid (Depakene)
 Started at 2mL bid, gradually increased to 5mL bid
 Oral Diazepam, pulse therapy
 4 weeks at 12.5mg
 4 weeks off medication
 4 weeks at 10mg
oSpeech Therapy
 1:1 in school
 1:1 private 2x/week

20. Case Study
●Current Status
oSeizures under control
oReceptive language, vocabulary, & articulation skills in
low-average range
oExpressive language skills show moderate delay, age
equivalent of 3:5
oExhibits metathesis, but can correct
oContinued speech therapy sessions
oNext follow-up in approx. 5 months

21. Review
●True Cause Unknown
●Progressive Aphasia
oLose both receptive and expressive language skills
●Abnormal EEG Activity
oSeizure activity during sleep
●Neurodiagnostic tools include EEG, Imaging, & BAEP
●Available treatments include diet, medication, surgery,
and speech therapy
●Prognosis varies

22. Questions?