1 - Pediatric Infectious Diseases Unit;
2 - Pediatric Neurology Unit;
3 - Pediatric Intensive Care Unit. Hospital Dona Estefânia CHLC EPE. Lisbon. Portugal
10th Excellence in Pediatrics Conference, Praga, RepúblicaCehca, 2018
Resumo:
Introduction: Acute necrotizing encephalopathy (ANE) is a rare and severe acute encephalopathy and is characterized by rapid neurologic deterioration preceded by a virus-associated febrile illness. Cerebrospinal fluid pleocytosis is absent and the suggestive imaging pattern consists on symmetric multifocal thalamic lesions
Case Description: A previously healthy 4-year-old boy presented with fever, dry cough pharyngitis and drowsiness with a GCS of 14, diminished strength on both arms, gait ataxia, Babinsky sign bilaterally. Head CT and CSF analysis were normal, and he started acyclovir and ceftriaxone. Within hours his condition deteriorated, with a GSC of 11, only responding to noxious stimuli and required intensive care. EEG showed slow and poorly differentiated activity, etiologically nonspecific. Ciprofloxacin and oseltamivir were associated to his therapy and he was also given immunoglobulin 1g/kg for two days and metilprednisolone 30mg/kg/day for 5 days. MRI showed bilateral thalamic lesions suggesting acute necrotizing encephalitis. Because metabolic etiologies such as Biotin-Responsive Basal Ganglia Disease could not be excluded he also was started on biotine and tiamine. PCR search for respiratory virus was positive for adenovirus and parainfluenza 3, and negative for Influenza A and B. The remaining work-up was negative and oseltamivir, ciprofloxacin and acyclovir were suspended. The patient’s mother had a history of acute encephalopathy in chldhood, and her CT scan showed thalamic lesions. The child gradually improved and was discharged after 9 days with no neurologic sequelae.
Conclusion: ANE is an immune-mediated disease with a potentially devastating course. ANE should be included in the differential diagnosis of encephalopathy with a rapid evolution. Its pathogenesis is not completely understood. This case shows that less frequent viral agents may be involved, and family history should also be valued as some forms of ANE seem to be familial.
Palavras Chave: adenovírus, bilateral thalamic necrotizing encephalopathy