Abstract
BackgroundAcute necrotizing encephalopathy of children (ANEC) is a rare fulminant type of acute encephalopathy that mainly occurs in children with a characteristic clinico-radiological pattern. It is commonly preceded by viral infections. The condition carries a poor prognosis with high morbidity and mortality rates. We highlight the relationship between diagnostic multi-parametric magnetic resonance imaging (MRI) findings and correlate them with the clinical outcome of children with ANEC by enrollment of MRI scoring.ResultsThe thalami were involved in all 30 patients, brain stem in 80%, basal ganglia (13.3%), cerebral white matter (WM) in 73%, and cerebellar WM in 33%. Hemorrhage was present in 86.7% patients, edema 80%, and necrosis in 13.3%. We found that the patients having the highest MRI scores were in the poor outcome category; whereas the patients with lower MR score (1 or 2) had better outcomes. Statistically positive correlation (r= 0.1198) was found between the multi-parametric MR score and the outcome category.ConclusionAn extended multi-parametric MRI should be performed in ANEC, allowing early detection and scoring of the disease for better prognosis. There is a positive correlation between the clinical outcome and the MR scoring.
Highlights
Acute necrotizing encephalopathy of children (ANEC) is a rare fulminant type of acute encephalopathy that mainly occurs in children with a characteristic clinico-radiological pattern
All had prodromal symptoms appear in the first stage; the main symptom was fever presented in 26 cases (87%), signs of upper respiratory tract infections presented in 10 cases (33.3%), and gastroenteritis symptoms presented in 6 cases (20%), and the least was the skin manifestation presented in 2 case (7%) only
With the development of ANEC in the mid stage, rapid and progressive symptoms of brain dysfunctions presented as generalized convulsions and disturbance of consciousness in 18 cases (60%); focal neurological deficits presented in 24 cases (80%) and abnormal movements in 14 cases (46.6%)
Summary
Acute necrotizing encephalopathy of children (ANEC) is a rare fulminant type of acute encephalopathy that mainly occurs in children with a characteristic clinico-radiological pattern. It is commonly preceded by viral infections. The prognosis of ANEC is variable, from a mild form even up to a complete recovery to a severe form with a high mortality [7]. The diagnosis of ANEC was Ibrahim et al Egyptian Journal of Radiology and Nuclear Medicine (2020) 51:113 mainly based on characteristic neuro-radiologic findings [8] characterized by multiple, symmetrical lesions showing T2 prolongation in the thalami, frequently with accompanying lesions in the brain stem tegmentum, putamina, cerebral, and cerebellar hemispheres WM [9]
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