Episodic epileptic verbal auditory agnosia in Landau Kleffner syndrome treated with combination diazepam and corticosteroids.
We report 2 pediatric patients who presented initially with seizures followed by subacute language regression characterized by a verbal auditory agnosia. These previously normal children had no evidence of expressive aphasia during their symptomatic periods. Further, in both cases, auditory agnosia was associated with sleep-activated electroencephalographic (EEG) epileptiform activity, consistent with Landau-Kleffner syndrome. However, both cases are unique since the episodic auditory agnosia and sleep-activated EEG epileptiform activity rapidly responded to combination therapy with pulse benzodiazepine and corticosteroids. Further, in each case, recurrences were characterized by similar symptoms, EEG findings, and beneficial responses to the pulse benzodiazepine and corticosteroid therapy. These observations suggest that pulse combination high-dose corticosteroid and benzodiazepine therapy may be especially effective in Landau-Kleffner syndrome.
Department of Neurology, NYU Langone School of Medicine, New York, NY, USA Institute of Neurology, Saint Barnabas Medical Center, Livingston, NJ, USA od4@nyu.edu.
Orrin
O
Devinsky
Institute of Neurology, Saint Barnabas Medical Center, Livingston, NJ, USA.
Rina
R
Goldberg
Department of Neurology, NYU Langone School of Medicine, New York, NY, USA.
Daniel
D
Miles
Institute of Neurology, Saint Barnabas Medical Center, Livingston, NJ, USA.
Aviva
A
Bojko
Department of Neurology, NYU Langone School of Medicine, New York, NY, USA.
James
J
Riviello
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000377:Agnosia / Q000150:complications* / Q000188:drug therapy* / Q000503:physiopathology; D000927:Anticonvulsants / Q000627:therapeutic use; D001921:Brain / Q000187:drug effects / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003975:Diazepam / Q000627:therapeutic use; D004569:Electroencephalography; D004827:Epilepsy / Q000150:complications* / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000150:complications* / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D012890:Sleep / Q000502:physiology
D000305:Adrenal Cortex Hormones; D000927:Anticonvulsants; D003975:Diazepam
Landau Kleffner syndrome; auditory agnosia; electrical status epilepticus of sleep