TY - JOUR
T1 - Landau - Kleffner syndrome
T2 - Treatment with subpial intracortical transection
AU - Morrell, Frank
AU - Whisler, Walter W.
AU - Smith, Michael C.
AU - Hoeppner, Thomas J.
AU - Toledo-morrell, Leyla De
AU - Pierre-louis, Serge J.C.
AU - Kanner, Andres M.
AU - Buclow, Janice M.
AU - Ristanovic, Ruzica
AU - Bergen, Donna
AU - Chez, Michael
AU - Hasegawa, Hisanori
PY - 1995/12/1
Y1 - 1995/12/1
N2 - Summary: 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.
AB - Summary: 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.
KW - Epiletogenic lesions
KW - Landau-Kleffner syndrome
KW - Multiple subpial transection
KW - Synaptogenesis
UR - http://www.scopus.com/inward/record.url?scp=0029564407&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0029564407&partnerID=8YFLogxK
U2 - 10.1093/brain/118.6.1529
DO - 10.1093/brain/118.6.1529
M3 - Article
C2 - 8595482
AN - SCOPUS:0029564407
VL - 118
SP - 1529
EP - 1546
JO - Brain
JF - Brain
SN - 0006-8950
IS - 6
ER -