[P1-19] Underlying Disorders in Children with Infection-related Acute Encephalopathy
[Introduction] Children with infection-related acute encephalopathy (AE) show neurological symptoms such as impairment of consciousness and seizures. Several factors including infectious agents and genetic polymorphisms may lead to the onset of AE. We experienced patients with pre-existing underlying neurological disorders in our series. To clarify the intrinsic factors, we investigated the underlying diseases and past histories of the patients with AE.
[Methodology] We enrolled patients with AE between 2009 and 2015 from the database of the Tokai Pediatric Neurology Society. We extracted patients with three types of AE based on clinico-radiological findings: acute disseminated encephalomyelitis (ADEM), acute encephalopathy with reduced subcortical diffusion (AED), and mild encephalopathy with reversible splenial lesion (MERS). We assessed past medical histories of the three groups.
[Results] Prevalence of underlying disease or past medical illness was significantly higher in patients with AED(42/120, 35%) than that in patients with ADEM(4/39, 10%) or MERS(19/75, 25%). In patients with AED, 18 patients had seizure disorders, including Dravet syndrome (n=2), West syndrome (n=3), other symptomatic epilepsy (n=7), febrile seizure (n=3), and single afebrile seizure (n=2). Seven patients had histories of status epilepticus. Fifteen patients were born prematurely and five patients had periventricular leukomalacia. In patients with MERS, 6 patients had seizure disorders, two had craniopharyngioma, and two had nephritic syndrome.
[Conclusions] Our study revealed seizure predisposition in patients with AED and MERS. These two types of infection-related AE are supposed to be induced by a combination of an intrinsic factor of seizure susceptibility and an extrinsic factor of infectious illness.
[Methodology] We enrolled patients with AE between 2009 and 2015 from the database of the Tokai Pediatric Neurology Society. We extracted patients with three types of AE based on clinico-radiological findings: acute disseminated encephalomyelitis (ADEM), acute encephalopathy with reduced subcortical diffusion (AED), and mild encephalopathy with reversible splenial lesion (MERS). We assessed past medical histories of the three groups.
[Results] Prevalence of underlying disease or past medical illness was significantly higher in patients with AED(42/120, 35%) than that in patients with ADEM(4/39, 10%) or MERS(19/75, 25%). In patients with AED, 18 patients had seizure disorders, including Dravet syndrome (n=2), West syndrome (n=3), other symptomatic epilepsy (n=7), febrile seizure (n=3), and single afebrile seizure (n=2). Seven patients had histories of status epilepticus. Fifteen patients were born prematurely and five patients had periventricular leukomalacia. In patients with MERS, 6 patients had seizure disorders, two had craniopharyngioma, and two had nephritic syndrome.
[Conclusions] Our study revealed seizure predisposition in patients with AED and MERS. These two types of infection-related AE are supposed to be induced by a combination of an intrinsic factor of seizure susceptibility and an extrinsic factor of infectious illness.