1. Department of Pediatric Infectious Diseases, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal
2. Department of Pediatric Neuroradiology, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal
3. Department of Pediatric Neurology, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal
- Publicação em versão integral: Port J Pediatr 2021;52:78-9. DOI: https://doi.org/10.25754/pjp.2021.20545
A previously healthy 2-year-old male toddler presented with high fever, cough, upper limb tremor and gait ataxia, rapidly evolving to neurologic deterioration, drowsiness, and bradycardia and, therefore, requiring intensive care. Complete blood cell count, C reactive protein, aminotransferases, ammonia, and lactate were normal and the toxicology screen was negative. Nasopharyngeal swab polymerase chain reaction (PCR) was positive for rhinovirus. Lumbar puncture revealed pleocytosis of 24 cells/μL with predominant mononuclear cells, normal glucose, and mildly elevated protein levels (61.7 mg/dL), with sterile cerebrospinal fluid cultures and negative PCR for herpes virus and enterovirus. The electroencephalogram showed an encephalopathic pattern with irregular and slow background activity and brain magnetic resonance imaging (MRI) showed multifocal bilateral thalamic lesions.
Acute necrotizing encephalopathy was admitted, and methylprednisolone 30 mg/kg/day initiated. Due to persistent altered mental status, associated with aphasia and dysphagia, and as biotin-thiamine-responsive basal ganglia disease could not be excluded, these vitamins were also initiated. Clinical improvement was noticed after five days, with full recovery of swallowing and speech. Eight months after the diagnosis, he showed lower right-hand dexterity and left-hand dominance. The metabolic evaluation revealed normal amino acids and redox potential and genetic study of biotin is ongoing, despite no family history of acute necrotizing encephalopathy.
Acute necrotizing encephalopathy is a severe neurological disorder characterized by rapid neurologic deterioration secondary to a virus febrile illness. It is imagiological diagnosed by a distinctive feature of multifocal symmetric brain lesions, affecting the bilateral thalamus, brainstem, cerebral periventricular white matter, and cerebellum. Common etiologic agents include influenza virus and other infectious diseases as well as toxic, metabolic, and inflammatory/vascular central nervous system disorders. In this case, we question the pathogenicity of rhinovirus associated with acute necrotizing encephalopathy, as it has not been described before. The genetic result is also important, even without a positive familial history of acute necrotizing encephalopathy, as if positive for the Ran binding protein it may predict new episodes and early management can improve the frequently poor prognosis. In children without brainstem lesions, improvement in the outcome was described particularly when steroids were started within the first 24 hours from the onset.
Keywords: Acute Hemorrhagic/diagnosis; Child, Preschool; Leukoencephalitis, Neurorradiology