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Acute necrotizing encephalopathy of childhood
1Division of Neurology, Department of Pediatrics, Chang Gung Children's Hospitals, Taipei. wanghs444@cgmh.org.tw
Insights
Acute necrotizing encephalopathy of childhood (ANEC) is a rare brain condition affecting young children, characterized by symmetric thalamic lesions. Diagnosis involves differentiating it from other neurological disorders.
Area of Science:
- Pediatric Neurology
- Neuroimaging
- Infectious Disease Complications
Background:
- Acute necrotizing encephalopathy of childhood (ANEC) is a rare neurological disorder affecting previously healthy children.
- It is characterized by symmetric lesions in the thalami and other brain regions.
- The condition predominantly affects infants and young children in Taiwan and Japan.
Purpose of the Study:
- To describe the clinical, radiological, and pathological features of acute necrotizing encephalopathy of childhood.
- To highlight the diagnostic criteria and differential diagnoses for ANEC.
- To provide insights into the prognosis and sequelae of this rare condition.
Main Methods:
- Review of clinical presentations, neuroimaging findings (ultrasonography, CT, MRI), and pathological data.
- Comparison with other neurological conditions that present with similar symptoms or brain lesions.
- Analysis of patient outcomes and long-term sequelae.
Main Results:
- ANEC typically follows a febrile illness with respiratory or gastrointestinal symptoms.
- Hallmark neuroimaging findings include multifocal, symmetric brain lesions, particularly in the bilateral thalami.
- The prognosis is generally poor, with less than 10% of patients achieving complete recovery; focal neurological deficits are common sequelae.
Conclusions:
- Acute necrotizing encephalopathy of childhood is a distinct clinicoradiological entity.
- Accurate diagnosis requires differentiation from other encephalopathies, vascular events, and metabolic disorders.
- Further research into the pathogenesis of ANEC is needed.
Abstract:
Symmetric change of the entire area of the bilateral thalami, as with panthalamic lesions, plus involvement of other regions in the brain rarely occurs to previously healthy children. The term, acute necrotizing encephalopathy of childhood, has recently been proposed. Its clinical, radiological, and pathological features are described. This disease predominantly affects infants and young children living in Taiwan and Japan, and manifests itself as acute encephalopathy following 2 to 4 days of fever and minor symptoms of the respiratory and/or gastrointestinal systems. The hallmark of this encephalopathy consists of multifocal, symmetric brain lesions affecting the bilateral thalami, and/or cerebral periventricular white matter, brainstem tegmentum, or cerebellar medulla, which can be documented by ultrasonography, computed tomography, and magnetic resonance imaging of the brain. The prognosis is usually poor. Less than 10% of patients recover completely. Cases with good outcome were reported to have reversible imaging changes. Focal neurologic deficits are common sequelae. The pathogenesis of acute necrotizing encephalopathy of childhood is still not known. Some diseases, such as Reye's syndrome, vascular occlusion, tumor, hemorrhage of the thalamus, Sandhoff disease, or Leigh and Wernicke encephalopathies must be differentiated clinically, radiologically, or pathologically. By excluding other disease entities, acute necrotizing encephalopathy of childhood can be diagnosed.
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