Landau-Kleffner syndrome: Treatment with subpial intracortical transection

Frank Morrell, Walter W. Whisler, Michael C. Smith, Thomas J. Hoeppner, Leyla De Toledo-Morrell, Serge J C Pierre-Louis, Andres M Kanner, Janice M. Buelow, Ruzica Ristanovic, Donna Bergen, Michael Chez, Hisanori Hasegawa

Research output: Contribution to journalArticle

297 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1529-1546
Number of pages18
JournalBrain
Volume118
Issue number6
StatePublished - Dec 1995
Externally publishedYes

Fingerprint

Landau-Kleffner Syndrome
Speech Therapy
Seizures
Cortex
seizures
Aphasia
Therapy
cortex
Critical Period
Electroencephalography
Language
Therapeutics
therapeutics
Prognosis
childhood
lesions (animal)
Modality
prognosis
Disorder
Eliminate

Keywords

  • Epileptogenic lesions
  • Landau-Kleffner syndrome
  • Multiple subpial transection
  • Synaptogenesis

ASJC Scopus subject areas

  • Neuroscience(all)
  • Statistics, Probability and Uncertainty
  • Applied Mathematics
  • Mathematics(all)
  • Statistics and Probability
  • Agricultural and Biological Sciences (miscellaneous)
  • Clinical Neurology

Cite this

Morrell, F., Whisler, W. W., Smith, M. C., Hoeppner, T. J., De Toledo-Morrell, L., Pierre-Louis, S. J. C., ... Hasegawa, H. (1995). Landau-Kleffner syndrome: Treatment with subpial intracortical transection. Brain, 118(6), 1529-1546.

Landau-Kleffner syndrome : Treatment with subpial intracortical transection. / Morrell, Frank; Whisler, Walter W.; Smith, Michael C.; Hoeppner, Thomas J.; De Toledo-Morrell, Leyla; Pierre-Louis, Serge J C; Kanner, Andres M; Buelow, Janice M.; Ristanovic, Ruzica; Bergen, Donna; Chez, Michael; Hasegawa, Hisanori.

In: Brain, Vol. 118, No. 6, 12.1995, p. 1529-1546.

Research output: Contribution to journalArticle

Morrell, F, Whisler, WW, Smith, MC, Hoeppner, TJ, De Toledo-Morrell, L, Pierre-Louis, SJC, Kanner, AM, Buelow, JM, Ristanovic, R, Bergen, D, Chez, M & Hasegawa, H 1995, 'Landau-Kleffner syndrome: Treatment with subpial intracortical transection', Brain, vol. 118, no. 6, pp. 1529-1546.
Morrell F, Whisler WW, Smith MC, Hoeppner TJ, De Toledo-Morrell L, Pierre-Louis SJC et al. Landau-Kleffner syndrome: Treatment with subpial intracortical transection. Brain. 1995 Dec;118(6):1529-1546.
Morrell, Frank ; Whisler, Walter W. ; Smith, Michael C. ; Hoeppner, Thomas J. ; De Toledo-Morrell, Leyla ; Pierre-Louis, Serge J C ; Kanner, Andres M ; Buelow, Janice M. ; Ristanovic, Ruzica ; Bergen, Donna ; Chez, Michael ; Hasegawa, Hisanori. / Landau-Kleffner syndrome : Treatment with subpial intracortical transection. In: Brain. 1995 ; Vol. 118, No. 6. pp. 1529-1546.
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abstract = "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.",
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