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[Acute infantile bilateral thalamic necrosis]
M P Vicente-Gutiérrez1, F J Cambra-Lasaosa, J Campistol
1Unitat Integrada de Pediatría, Hospital Sant Joan de Déu-Clínic. Universidad de Barcelona, Barcelona, España.
Revista De Neurologia
|February 18, 2003
Summary
Acute infantile thalamic necrosis is a rare, severe neurological condition affecting young children after viral infections. This case highlights its rapid progression and poor prognosis, even with intensive care.
Area of Science:
- Neurology
- Pediatrics
- Infectious Diseases
Background:
- Acute necrotizing encephalitis, recently identified in Japanese children, follows viral infections and causes rapid consciousness decline.
- Characterized by bilateral thalamic lesions on neuroimaging, it has been termed infantile bilateral thalamic necrosis.
- The condition has been increasingly reported globally, including isolated cases in Western countries.
Observation:
- A case report details an 11-month-old Spanish infant presenting with altered consciousness and hypertonia after a febrile gastrointestinal illness.
- Initial investigations revealed hemoconcentration and metabolic acidosis, without clear signs of infection.
- Brain CT scan demonstrated significant hypodensity in both thalami, preceding generalized hypotonia and cardiorespiratory failure.
Findings:
- The infant experienced rapid deterioration, leading to brain death within 40 hours of admission.
- Despite mechanical ventilation and inotropic support, the patient's outcome was unfavorable.
- Neuroimaging consistently reveals selective thalamic involvement in affected children.
Implications:
- This case underscores the severe and rapidly progressive nature of acute infantile thalamic necrosis.
- The term acute infantile thalamic necrosis is proposed as more fitting given the thalamic focus and pediatric population.
- Further research is needed to understand the pathogenesis and identify potential therapeutic strategies for this rare condition.