Fluctuating Kluver-Bucy syndrome in a child with epilepsy due to bilateral anterior temporal congenital malformations.
An 11-year-old boy with epilepsy due to congenital bilateral anterior temporal lobe malformations presented with fluctuating Kluver-Bucy syndrome (KBS). Since the age of 2, he had experienced clusters of three or four daily complex partial seizures over 2-3 days in a month, followed by a seizure-free interval of 3-4 weeks. During the seizure-free period, the patient exhibited hyperorality, sniffing, irritability alternating with placidity, anxiety, unsolicited sexual gestures, and unusual calmness after eating. KBS features escalated up to the onset of the seizure cluster, and remitted after the seizures. Brain MRI revealed bilateral anterior temporal cortical dysplasia with enlarged and dysmorphic amygdalar-hippocampal complex. Brain [(18)F]fluorodeoxyglucose positron emission tomography PET showed bilateral anterior and mesial temporal hypometabolism. Video/EEG monitoring revealed independent right and left temporal lobe seizures. This is the first reported case of KBS due to congenital bitemporal malformations. Also, KBS behavior phenotype in this patient fluctuated, with escalation during the seizure-free period and remission induced by the monthly seizure cluster. This fluctuating pattern could represent forced normalization.
Pestana
Elia M
EM
Section of Pediatric Epilepsy, Epilepsy Center, Department of Neurology, Cleveland Clinic Foundation, Cleveland, OH 44195, USA.
Gupta
Ajay
A
D001921:Brain / Q000000981:diagnostic imaging; D002648:Child; D004569:Electroencephalography; D017029:Epilepsy, Complex Partial / Q000150:complications* / Q000151:congenital / Q000601:surgery; D004833:Epilepsy, Temporal Lobe / Q000150:complications* / Q000151:congenital / Q000601:surgery; D006801:Humans; D020232:Kluver-Bucy Syndrome / Q000209:etiology / Q000503:physiopathology / Q000523:psychology*; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D019635:Neurosurgical Procedures / Q000009:adverse effects*; D010641:Phenotype; D049268:Positron-Emission Tomography; D011183:Postoperative Complications / Q000503:physiopathology / Q000523:psychology*; D012640:Seizures / Q000503:physiopathology; D013702:Temporal Lobe / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery*