Episodic epileptic verbal auditory agnosia in Landau Kleffner syndrome treated with combination diazepam and corticosteroids
| Autor: | Rina Goldberg, James J. Riviello, Aviva Bojko, Orrin Devinsky, Daniel K. Miles |
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| Rok vydání: | 2014 |
| Předmět: |
Male
medicine.medical_specialty Combination therapy medicine.drug_class Landau–Kleffner syndrome Auditory agnosia Audiology Electroencephalography Adrenal Cortex Hormones medicine Humans Child Benzodiazepine Landau-Kleffner Syndrome Diazepam Epilepsy medicine.diagnostic_test Brain medicine.disease Expressive aphasia Anesthesia Child Preschool Pediatrics Perinatology and Child Health Agnosia Corticosteroid Anticonvulsants Neurology (clinical) Psychology Sleep medicine.drug |
| Zdroj: | Journal of child neurology. 29(10) |
| ISSN: | 1708-8283 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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