Gelastic seizures and hypothalamic hamartomas: evaluation of patients undergoing chronic intracranial EEG monitoring and outcome of surgical treatment.
We retrospectively studied 12 consecutive patients with gelastic seizures and hypothalamic hamartomas who, because of intractable epilepsy, underwent chronic intracranial EEG monitoring or epilepsy surgery. All patients had medically refractory seizures that included laughter as an ictal behavior (gelastic seizures). The hypothalamic hamartomas were identified with neuroimaging studies (12 of 12) and by pathologic verification (four of 12). Associated clinical features included behavioral disorders (n = 5), developmental delay (n = 4), and precocious puberty (n = 2). Interictal extracranial EEG predominantly showed bi-hemispheric epileptiform changes suggesting a secondary generalized epileptic disorder. Intracranial EEG recordings, performed in eight patients, indicated the apparent focal onset of seizure activity (anterior temporal lobe [n = 7] and frontal lobe [n = 1]). None of the seven patients who underwent a focal cortical resection, however, experienced a significant reduction in seizure tendency. An anterior corpus callosotomy, performed in two patients with symptomatic generalized epilepsy, resulted in a worthwhile reduction in drop attacks. Results of this study may modify the surgical strategies in patients with gelastic seizures and hypothalamic hamartomas.
Cascino
G D
GD
Department of Neurology, Mayo Clinic, Rochester, MN.
Andermann
F
F
Berkovic
S F
SF
Kuzniecky
R I
RI
Sharbrough
F W
FW
Keene
D L
DL
Bladin
P F
PF
Kelly
P J
PJ
Olivier
A
A
Feindel
W
W
D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004828:Epilepsies, Partial / Q000209:etiology*; D005260:Female; D005625:Frontal Lobe / Q000473:pathology / Q000601:surgery; D006222:Hamartoma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D006624:Hippocampus / Q000473:pathology / Q000601:surgery; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D007223:Infant; D008607:Intellectual Disability / Q000150:complications; D007845:Laughter; D008279:Magnetic Resonance Imaging; D008297:Male; D011629:Puberty, Precocious / Q000150:complications; D012189:Retrospective Studies; D013702:Temporal Lobe / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome