Landau-Kleffner syndrome. Treatment with subpial intracortical transection.
Landau-Kleffner syndrome (LKS) is an acquired epileptic aphasia occurring in childhood and associated with a generally poor prognosis for recovery of speech. It is thought to be the result of an epileptogenic lesion arising in speech cortex during a critical period of development. Utilizing a new surgical technique designed to eliminate the capacity of cortical tissue to generate seizures while preserving the normal cortical physiological function, we have treated 14 children with aphasia, seizures and a severely abnormal EEG by multiple subpial transection of the epileptogenic cortex. Seven of the 14 patients (50%) have recovered age-appropriate speech, are in regular classes in school and no longer require speech therapy. Four of the 14 (29%) have shown marked improvement, are speaking and understanding verbal instruction but are still receiving speech therapy. Thus, 11 of the 14 (79%), none of whom had used language to communicate for at least 2 years, are now speaking--a rate of sustained improvement considered unusual in this disorder. This study documents the value of a treatment modality not previously used in LKS. Success depends on selection of cases having severe epileptogenic abnormality that can be demonstrated to be unilateral in origin despite a bilateral electrographic manifestation.
Morrell
F
F
Department of Neurological Science, Rush-Presbyterian-St Luke's Medical Center, Chicago, IL 60612, USA.
Whisler
W W
WW
Smith
M C
MC
Hoeppner
T J
TJ
de Toledo-Morrell
L
L
Pierre-Louis
S J
SJ
Kanner
A M
AM
Buelow
J M
JM
Ristanovic
R
R
Bergen
D
D
D000293:Adolescent; D002540:Cerebral Cortex / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D015225:Magnetoencephalography; D008297:Male; D008723:Methohexital; D009017:Morbidity; D010841:Pia Mater; D011184:Postoperative Period; D013060:Speech; D013997:Time Factors; D017211:Treatment Failure
D008723:Methohexital