European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
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Eur. J. Paediatr. Neurol. · May 2014
Case ReportsHemiconvulsion-Hemiplegia-Epilepsy syndrome associated with inflammatory-degenerative hystopathological findings in child with congenital adrenal hyperplasia.
Hemiconvulsion-Hemiplegia (HH) syndrome represents an uncommon consequence of prolonged unilateral clonic or hemiconvulsive status epilepticus in childhood, usually occurring during a febrile illness, followed by ipsilateral hemiplegia. The subsequent appearance of focal seizures configures the so called Hemiconvulsion-Hemiplegia-Epilepsy (HHE) syndrome. The pathogenesis of HH/HHE syndrome is still unclear. ⋯ Disregulation of the inflammatory cascade has been proposed as one of the possible pathogenetic mechanisms underlying HH/HHE syndrome. To our knowledge however, this is the first report of an association with a histologically documented inflammatory process. The clinical and histopathological findings of our reported case lend support to the possible role of inflammation in the pathogenesis of HH/HHE syndrome.
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Eur. J. Paediatr. Neurol. · May 2014
Outcomes of hypoxic ischaemic encephalopathy treated with therapeutic hypothermia using cool gel packs - experience from Western Australia.
Therapeutic hypothermia is the standard clinical practice for neonates with moderate to severe hypoxic ischaemic encephalopathy (HIE). ⋯ Neonates undergoing therapeutic hypothermia with cool gel packs had both good survival rates and long term neurodevelopmental outcomes and met international benchmarks.