Intractable reflex audiogenic epilepsy successfully treated by peri-insular hemispherotomy.
We report a case of an infantile hemiplegia seizure syndrome (IHSS) that presented with intractable reflex audiogenic startle epilepsy which in itself is an uncommon form of seizure disorder. Peri-insular hemispherotomy provided complete seizure control. Also of particular interest was that this syndrome resulted from an iatrogenic brain injury sustained during the course of a caesarian section. We review the different mechanisms of birth injury reported in the literature and, discuss the physiopathogenesis of the hemispheric damage in this patient. We also review the literature on "reflex epilepsy" as it applies to this case. Intractable reflex audiogenic (startle) epilepsy in IHSS submitted to hemispherotomy has not previously been reported.
Department of Neurosurgery, Centre Hospitalier Universitaire Vaudois (CHUV), Rue du Bugnon 46, Lausanne 1011, Switzerland.
Rosa
R
Martinez-Mañas
Roy Thomas
RT
Daniel
Damien
D
Debatisse
Malin
M
Maeder-Ingvar
Kathleen
K
Meagher-Villemure
Jean-Guy
JG
Villemure
Thierry
T
Deonna
D002540:Cerebral Cortex / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D002648:Child; D020195:Epilepsy, Reflex / Q000601:surgery*; D007839:Functional Laterality; D038421:Hemispherectomy / Q000379:methods*; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods; D008297:Male; D016900:Neurofilament Proteins / Q000378:metabolism; D009474:Neurons / Q000378:metabolism; D012196:Review Literature as Topic; D016896:Treatment Outcome
D016900:Neurofilament Proteins