Acute necrotizing encephalopathy of childhood (infantile bilateral thalamic necrosis): two non-Japanese cases

This report describes two Spanish cases of acute necrotizing encephalopathy of childhood, a rare disease first described in Japan by Mizuguchi and col leagues. Similar cases have been reported from Taiwan and other countries o f the Far East. Two cases have been reported from the UK and one from the U SA. The disease affects young children and is characterized by acute enceph alopathy after a viral illness, with seizures and decreased levels of consc iousness. The hallmark of the disease is diffuse and symmetrical CNS lesion s of both thalami, internal capsules, upper brainstem tegmentum, and cerebr al white matter. The aetiology is unknown but an infectious or parainfectio us process seems likely, Because of the predominant involvement of the thal ami, we propose the term 'infantile bilateral thalamic necrosis', a more sp ecific term and one which distinguishes the entity from other basal ganglia diseases in childhood.