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

Authors

  • Raghavendra Narayan Vanaki Department of Paediatrics, S.N. Medical College and HSK Hospital, Bagalkot, Karnataka http://orcid.org/0000-0001-9904-0923
  • Suchitra Basappa Diwanmal Department of Paediatrics, S.N. Medical College and HSK Hospital, Bagalkot, Karnataka
  • Ramesh Rajesh Pol Department of Paediatrics, S.N. Medical College and HSK Hospital, Bagalkot, Karnataka
  • Bhuvaneshwari Chandrashekhar Yelamali Department of Paediatrics, S.N. Medical College and HSK Hospital, Bagalkot, Karnataka
  • Milind Ramakant Kulkarni Department of Paediatrics, S.N. Medical College and HSK Hospital, Bagalkot, Karnataka
  • Sudheendraswamy Veerannayya Brahmandbher Department of Paediatrics, S.N. Medical College and HSK Hospital, Bagalkot, Karnataka

DOI:

https://doi.org/10.18203/2349-3291.ijcp20204468

Keywords:

ANEC, Encephalitis, Rankin scale, Rehabilitation

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.

Author Biography

Raghavendra Narayan Vanaki, Department of Paediatrics, S.N. Medical College and HSK Hospital, Bagalkot, Karnataka

Consultant Pediatric Intensivist, and Associate Professor, Department of Pediatrics, S.N.Medical College, & HSK Hospital, Bagalkot-587103, Karnataka, India

References

Mizuguchi M, Tomonaga M, Fukusato T, Asano M. Acute necrotizing encephalopathy with widespread edematous lesions of symmetrical distribution. Acta Neuropathol. 1989;78:108-11.

Mizuguchi M, Abe J, Mikkaichi K, Noma S, Yoshida K, Yamanaka T, et al. Acute necrotizing encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions. J Neurol Neurosurg Psychiatry. 1995;58:555-61.

Mizuguchi M. Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain Dev. 1997;19:81-92.

Wang HS, Huang SC. Acute necrotizing encephalopathy of childhood. Chang Gung Med J. 2001;24:1-10.

Barkovich AJ. Toxic and metabolic brain disorders. In: Pediatric Neuroimaging, 4th ed. Philadelphia: Lippincott Willams & Wilkins 2005: 76-189.

Seo HE, Hwang SK, Choe BH, Cho MH, Park SP, Kwon S. Clinical spectrum and prognostic factors of acute necrotizing encephalopathy in children. J Korean Med Sci. 2010;25(3):449-53.

Mizuguchi M, Hayashi M, Nakano I. Concentric structure of thalamic lessions in acute necrotizing encephalopathy. Neuroradiol. 2002;44(6):489-93.

Yoshikawa H, Watanabe T, Abe T, Oda Y. Clinical diversity in acute necrotizing encephalopathy. J Child Neurol. 1999;14:249-55.

Kim JH, Kim IO, Lim MK. Acute necrotizing encephalopathy in Korean infants and children: imaging findings and diverse clinical outcome. Korean J Radiol. 2004;5:171-77.

Ravid S, Topper L, Eviatar L. Acute necrotizing encephalopathy presenting as a basal ganglia syndrome. J Child Neurol. 2001;16:461-2.

Protheroe SM, Mellor DH. Imaging in influenza A encephalitis. Arch Dis Child. 1991;66:702-5.

Tran TD, Kubota M, Takeshita K, Yanagisawa M, Sakakihara Y. Varicella-associated acute necrotizing encephalopathy with a good prognosis. Brain Dev. 2001;23:54-7.

Cusmai R, Bertini E, Capua MD. Bilateral, reversible, selective thalamic involvement demonstrated by brain MR and acute severe neurological dysfuction with favorable outcome. Neuropediatrics. 1994;25:44-7.

San Millan B, Teijeira S, Penin C, Garcia JL, Navarro C. Acute necrotizing encephalopathy of childhood: report of a spanish case. Pediatr Neurol. 2007;37:438-41.

Yagishita A, Nakano I, Ushioda T, Otsuki N, Hasegawa A. Acute encephalopathy with bilateral thalamotegmental involvement in infants and children: imaging and pathology findings. Am J Neuroradiol. 1995;16:439-47.

Mastroyianni S, Gionnis D, Voudris K, Skardoutsou A, Mizuguchi M. Acute necrotizing encephalopathy of childhood in non-Asian patients: report of three cases and literature review. J Child Neurol. 2006;21:872-9.

Olgar S, Ertugrul T, Nisli K, Aydin K, Caliskan M. Influenza A associated acute necrotizing encephalopathy. Neuropediatrics. 2006;37:166-8.

Ohasaka M, Houkin K, Takigami M, Koyanagi I. Acute necrotizing encephalopathy associated with human herpesvirus-6 infection. Pediatr Neurol. 2006;34:160-3.

Wong AM, Simon EM, Zimmerman RA. Acute necrotizing encephalopathy of childhood: correlation of MR findings and clinical outcome. Am J Neuroradiol. 2006;27:1919-23.

Okumura A, Mizuguchi M, Kidokoro H, Tanaka M, Abe S, Hosoya M, et al. Outcome of acute necrotizing encephalopathy in relation to treatment with corticosteroids and gammaglobulin. Brain Dev. 2009;31:221-7.

Quinn TJ, Dawson J, Walters MR, Lees KR. Reliability of the modified Rankin Scale: a systematic review. Stroke. 2009;40(10):3393-5.

Downloads

Published

2020-10-21

Issue

Section

Original Research Articles