lks presentation.ppt
TRANSCRIPT
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?