lks presentation.ppt
TRANSCRIPT
![Page 1: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/1.jpg)
Landau-Kleffner Syndrome
Acquired Aphasia with Epilepsy
By Genevieve V. Walton
![Page 2: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/2.jpg)
Objectives
●Define Landau-Kleffner Syndrome (LKS)●Understand course of LKSoSymptomsoTreatmentoPrognosis
●Neurodiagnostic Studies
![Page 3: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/3.jpg)
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
![Page 4: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/4.jpg)
Symptoms
●Progressive AphasiaoReceptive (Comprehension)oExpressive (Communication)oOnset over weeks or months
●In some: Auditory Agnosia●Seizures during sleep
oMay progress to ESES
●Behavioral Disorders
![Page 5: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/5.jpg)
Differential Diagnosis
●Autism●Hearing Impairment●Learning Disability●Auditory/Verbal Processing Disorder●Childhood Schizophrenia●Mental Retardation
![Page 6: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/6.jpg)
Treatment
●DietoKetogenicoAtkins
●MedicationoAntiepileptic TherapyoCorticosteroids
●SurgicaloMultiple Subpial Transection
●Speech Therapy
![Page 7: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/7.jpg)
Prognosis
●Varies widely●Usually outgrown by 15 y/o●May have residual speech difficulties●ESES portion may persist●Improves with early treatment
![Page 8: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/8.jpg)
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
![Page 9: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/9.jpg)
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
![Page 10: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/10.jpg)
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
![Page 11: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/11.jpg)
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
![Page 12: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/12.jpg)
Case Study
●Tests PerformedoEEG
Routine Wake & Sleep EMU - Characterization of Spells
oMRI With & without Gadolinium Contrast
oMetabolicoChromosomal Analysis
![Page 13: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/13.jpg)
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
![Page 14: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/14.jpg)
Activation During Wakefulness
![Page 15: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/15.jpg)
Decreased Activation During Light Sleep
![Page 16: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/16.jpg)
Activation of Repetitive Spike and Slow Wave
![Page 17: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/17.jpg)
Activation on LAR-TPi Montage
![Page 18: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/18.jpg)
Activation on TAL Montage
![Page 19: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/19.jpg)
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
![Page 20: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/20.jpg)
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
![Page 21: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/21.jpg)
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
![Page 22: LKS presentation.ppt](https://reader036.vdocuments.mx/reader036/viewer/2022081507/589c9f731a28abf4148b5875/html5/thumbnails/22.jpg)
Questions?