Uğur Işık1, Sema Saltık2, Bahattin Tanrıkulu3, M Memet Özek3. 1. Department of Pediatrics, Division of Pediatric Neurology, Acıbadem University School of Medicine, Istanbul, Turkey. ugur.isik@acibadem.com.tr. 2. Cerrahpaşa School of Medicine, Department of Pediatrics, Division of Pediatric Neurology, Istanbul University, Istanbul, Turkey. 3. Department of Neurosurgery, Division of Pediatric Neurosurgery, Acıbadem University School of Medicine, Istanbul, Turkey.
Abstract
BACKGROUND: Hypothalamic hamartomas (HH) generally present with gelastic seizures. It is very unusual for a pediatric patient with HH to present with infantile spasms (IS). CASE PRESENTATION: Here we present a 6-month-old boy diagnosed with IS whose brain magnetic resonance imaging (MRI) showed an 18 × 18 × 16 mm mass in the hypothalamus. His seizures did not respond to antiepileptic treatment with vigabatrin and valproic acid. He had disconnective surgery for HH. Immediately postoperatively, his seizures subsided and he has now been seizure-free for 2 years. CONCLUSION: Although hypothalamic hamartomas generally present with gelastic seizures, they should also be considered in the differential diagnosis of infantile spasms.
BACKGROUND:Hypothalamic hamartomas (HH) generally present with gelastic seizures. It is very unusual for a pediatric patient with HH to present with infantile spasms (IS). CASE PRESENTATION: Here we present a 6-month-old boy diagnosed with IS whose brain magnetic resonance imaging (MRI) showed an 18 × 18 × 16 mm mass in the hypothalamus. His seizures did not respond to antiepileptic treatment with vigabatrin and valproic acid. He had disconnective surgery for HH. Immediately postoperatively, his seizures subsided and he has now been seizure-free for 2 years. CONCLUSION: Although hypothalamic hamartomas generally present with gelastic seizures, they should also be considered in the differential diagnosis of infantile spasms.
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