Landau-Kleffner Syndrome

Acquired Aphasia with Epilepsy

By Genevieve V. Walton

Objectives

●Define Landau-Kleffner Syndrome (LKS)●Understand course of LKSoSymptomsoTreatmentoPrognosis

●Neurodiagnostic Studies

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 activityo~80% have clinical seizures

Symptoms

●Progressive AphasiaoReceptive (Comprehension)oExpressive (Communication)oOnset over weeks or months

●In some: Auditory Agnosia●Seizures during sleep

oMay progress to ESES

●Behavioral Disorders

Differential Diagnosis

●Autism●Hearing Impairment●Learning Disability●Auditory/Verbal Processing Disorder●Childhood Schizophrenia●Mental Retardation

Treatment

●DietoKetogenicoAtkins

●MedicationoAntiepileptic TherapyoCorticosteroids

●SurgicaloMultiple Subpial Transection

●Speech Therapy

Prognosis

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

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

Neurodiagnostic Studies

●EEGoHigh voltage, repetitive spikes or spike-waveoBackground can be normal, or show diffuse slowingoSeen mostly during sleep

●Radiographic ImagingoNormal unless underlying structural issue

Such as cerebral tumors or polymicrogyria

●BAEPoNormal, no hearing deficit

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

Case Study

●Family Neurological HistoryoFather – No seizures or other problemsoMother – Psychomotor seizures starting at 10

y/o. Medicated until college. No seizures since. Mothers aunt & maternal cousin have histories of

grand mal seizures

Case Study

●Tests PerformedoEEG

Routine Wake & Sleep EMU - Characterization of Spells

oMRI With & without Gadolinium Contrast

oMetabolicoChromosomal Analysis

Case Study

●Test FindingsoEEG

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

Activation During Wakefulness

Decreased Activation During Light Sleep

Activation of Repetitive Spike and Slow Wave

Activation on LAR-TPi Montage

Activation on TAL Montage

Case Study

●TreatmentoMedication

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

Case Study

●Current StatusoSeizures under controloReceptive language, vocabulary, & articulation skills in

low-average rangeoExpressive language skills show moderate delay, age

equivalent of 3:5oExhibits metathesis, but can correctoContinued speech therapy sessionsoNext follow-up in approx. 5 months

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

Questions?