A child with refractory complex partial seizures, right temporal ganglioglioma, contralateral continuous electrical status epilepticus, and a secondary Landau-Kleffner autistic syndrome.
A 7-year-old, right-handed girl started to have seizures at age 1 year 4 months. She developed normally until age 4 when she had worsening of seizures with auditory verbal agnosia, complete aphasia, and a behavioral disorder fulfilling the diagnostic criteria of autism. Medical therapy failed. MRI revealed a right temporal tumor. Video/EEG monitoring at age 7 showed contralateral electrical status epilepticus in wakefulness and sleep and ipsilateral onset of seizures. Resection (ganglioglioma with excessive inflammation) resulted in seizure freedom and marked reduction of the autistic features. This case is unique for being, to our knowledge, (1) the first in which a lesion located in the right, rather than left, temporal lobe resulted in secondary falsely localizing left temporal lobe electrical status epilepticus with a clinical picture of Landau-Kleffner syndrome and autism, and (2) the fourth reported patient with lesional Landau-Kleffner syndrome to respond to resective surgery.
Mikati
Mohamad A
MA
Department of Pediatrics and Adolescent Medicine, Adult and Pediatric Epilepsy Program, American University of Beirut, Medical Center, Beirut, Lebanon. mohamad.mikati@duke.edu
El-Bitar
Mohamad K
MK
Najjar
Marwan W
MW
Rbeiz
Jean J
JJ
Barada
Wissam H
WH
Najjar
Vicky F
VF
Yaktin
Umayma
U
Tourjuman
Oulfat
O
D001321:Autistic Disorder / Q000150:complications*; D001932:Brain Neoplasms / Q000150:complications*; D002648:Child; D004569:Electroencephalography; D017029:Epilepsy, Complex Partial / Q000150:complications*; D005260:Female; D018303:Ganglioglioma / Q000150:complications*; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000150:complications*; D008279:Magnetic Resonance Imaging; D013226:Status Epilepticus / Q000150:complications*; D013702:Temporal Lobe / Q000473:pathology*; D014741:Video Recording