Acute necrotizing encephalopathy of childhood: an under-recognized and diverse clinico-radiological syndrome

Abstract

Background: With advances in neuroimaging, acute necrotizing encephalopathy of childhood (ANEC) is being recognized as an acute encephalopathy with a characteristic rapidly deteriorating neurological course with poor neuro-developmental outcomes with high mortality and morbidity rates. Indian data of ANEC is primitive and hence this study was planned. The objective of the study was to evaluate the clinical, radiological characteristics and its outcomes in children with ANEC.Methods: Retrospective review of 10 infants and children with ANEC at Department of Paediatrics, S.N. Medical College and HSK Hospital, Bagalkot from January 2013 to December 2019. ANEC was suspected based on clinical and radiological characteristics and diagnosis was made based on diagnostic criteria proposed by Mizuguchi et al. Clinical and radiological (Magnetic resonance imaging (MRI) brain characteristics) findings and response to early pulse dose steroid therapy and supportive treatment were assessed in all cases. All cases were followed for evaluation of neurodevelopmental outcome and response to physiotherapy was noted in all the cases.Results: Total 10 cases were analysed (age ranged from 6 months to 11 years). Sex distribution male to female was 3:7. All cases had precedent viral illnesses and had fever, coryza, diarrhoea. The initial neurological symptoms included seizures and status epilepticus (n=7), altered sensorium (n=3), focal neurological signs , gait disturbances (n=2) and diplopia (n=1). MRI brain revealed characteristic thalamus involvement with varied involvement of midbrain, pons, medulla (n=10). Other findings were cavitation (n=5), haemorrhage (n=4), minimal residual lesions (n=4), cerebral atrophy (n=1), normal brain study (n=5) on follow-up. 9 out of 10 cases survived, responded to early physiotherapy and rehabilitation. 6 children had complete recovery with minimal disability in 3 cases.Conclusions: ANEC is a clinico-radiological syndrome. Early detection and appropriate treatment improves outcome in ANEC.

Acute necrotizing encephalopathy of childhood from Eastern Africa

“…The survivors may be subjected to serious neurological complications such as muscle spasticity and speech disorders, but less than 10% of cases have recovered without any neurological sequelae 8 . Abnormal laboratory investigation with significant elevated levels of serum aminotransferase and CSF protein makes the prognosis of the disease to be worse 10 . With the aid of physiotherapy, our patient could walk on his own in contrast to his initial presentation of being bedbound with limited neuromotor activities on his both upper and lower limbs.…”

“…Most of the literature recommend the use of intravenous glucocorticoids and immunoglobulins as it has been seen to improve the patient's clinical condition remarkably. 6,10 In our patient, a combination of intravenous immunoglobulin, antiviral medications together with antibiotics was empirically prescribed medications during his hospital stay. Although immunoglobulin injection was not initiated early, its efficacy in this case cannot be predicted.…”

Acute Necrotizing Encephalopathy of Childhood from Eastern Africa

“…Most of the literatures recommend the use of intravenous glucocorticoids and immunoglobulins as it has been seen to improve the patient's clinical condition remarkably. [6,10] In our patient a combination of intravenous immunoglobulin, antiviral medications together with antibiotics were empirically prescribed medications during his hospital stay. Although immunoglobulin injection was not initiated early, its efficacy in this case cannot be predicted.…”

“…[8] Abnormal laboratory investigation with significant elevated levels of serum aminotransferase and CSF protein makes the prognosis of the disease to be worse. [10] With the aid of physiotherapy our patient could walk on his own in contrast with his initial presentation of being bed-bound with limited neuro-motor activities on his both upper and lower limbs.…”

“…[9] The laboratory findings are not specific for the diagnosis as they are mainly supportive, most cases present with elevated white blood cell counts, C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). [10] Serum transaminase, lactate dehydrogenase (LDH), and creatinine kinase (CK) are frequently observed to be elevated as there is direct relation to increased fibrin degradation products. [8] Occurrence of electrolyte imbalance is uncommon.…”