Top

Published in:

28-09-2024 | Encephalopathy | Research

Clinical prognostication in acute necrotizing encephalopathy of childhood: the role of magnetic resonance imaging severity assessment

Authors: Kumail Khandwala, Kiran Hilal, Sidra Kaleem Jafri, Syed Musa Mufarrih, Saira Samnani, Ahsun Amin Jiwani, Usman Ali

Published in: Pediatric Radiology | Issue 12/2024

Abstract

Background

Acute necrotizing encephalopathy of childhood is a unique entity with bilateral gray and white matter involvement.

Objective

The aim of this study is to explore whether the severity of findings on imaging scans is indicative of the prognosis and clinical outcomes for pediatric patients with acute necrotizing encephalopathy.

Materials and methods

A retrospective cross-sectional study was conducted on 42 patients diagnosed with acute necrotizing encephalopathy. A severity score based on MR imaging was computed for each patient, utilizing a point system determined by the existence of factors such as hemorrhage, cavitation, enhancement, diffusion restriction, and lesion location. The scoring was categorized into mild, moderate, and severe. Clinical outcomes were determined at the time of discharge and at follow-ups as mild disability, moderate disability, severe disability, and death according to the modified Rankin Scale. Associations were determined by Fisher’s exact test, chi-square test, and one-way ANOVA.

Results

The study included 21 boys and 21 girls with a mean age of 71.5 months. A statistically significant connection (P=0.027) was found between the severity score from MR imaging and the clinical outcome. A statistically significant relationship was also observed between diffusion restriction (P=0.008), cerebellar involvement (P=0.048), and an unfavorable clinical outcome. Additionally, individuals who experienced shock exhibited a correlation with adverse outcomes (P=0.01).

Conclusion

In predicting the outcome of acute necrotizing encephalopathy, cerebellar involvement and presence of diffusion restriction were associated with worse clinical outcomes in our study. Developing a comprehensive MR-based severity score is crucial for improving diagnostic accuracy and patient outcomes. Our findings underscore the importance of including diffusion restriction and cerebellar involvement in the scoring system.

Graphical abstract

Mizuguchi M, Abe J, Mikkaichi K et al (1995) Acute necrotizing encephalopathy of childhood: new syndrome presenting with multifocal, symmetric brain lesions. J Neurol Neurosurg Psychiatry 58:555–561CrossRefPubMedPubMedCentral

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

Kim JH, Kim IO, Lim MK et al (2004) Acute necrotizing encephalopathy in Korean infants and children: imaging findings and diverse clinical outcome. Korean J Radiol 5:171–177CrossRefPubMedPubMedCentral

Jan F, Jafri SK, Ibrahim SH (2019) Acute necrotizing encephalopathy. J Coll Physicians Surg Pak 29:649–653CrossRefPubMed

Wang HS, Huang SC (2004) Acute necrotizing encephalopathy of childhood. Chang Gung Med J 24:1–10

Virhammar J, Kumlien E, Fällmar D et al (2020) Acute necrotizing encephalopathy with SARS-CoV-2 RNA confirmed in cerebrospinal fluid. Neurology 95:445–449. https://doi.org/10.1212/WNL.0000000000010250CrossRefPubMedPubMedCentral

Khandwala K, Mubarak F, Ahmad MN (2021) Imaging patterns of encephalopathy in patients with COVID-19. J Coll Physicians Surg Pak 30:S42–S45. https://doi.org/10.29271/jcpsp.2021.01.S42

Bassuk AG, Burrowes DM, McRae W (2003) Acute necrotizing encephalopathy of childhood with radiographic progression over 10 hours. Neurology 60:1552–1553CrossRefPubMed

Neilson DE (2010) The interplay of infection and genetics in acute necrotizing encephalopathy. Curr Opin Pediatr 22:751–757CrossRefPubMed

10.

Shinohara M, Saitoh M, Takanashi J et al (2011) Carnitine palmitoyl transferase II polymorphism is associated with multiple syndromes of acute encephalopathy with various infectious diseases. Brain Dev 33:512–517CrossRefPubMed

11.

Wong AM, Simon EM, Zimmerman RA et al (2006) Acute necrotizing encephalopathy of childhood: correlation of MR findings and clinical outcome. AJNR Am J Neuroradiol 27:1919–1923PubMedPubMedCentral

12.

Biswas A, Varman M, Gunturi A et al (2018) Teaching neuro-images: acute necrotizing encephalopathy of childhood. Neurology 90:e177–e178CrossRefPubMed

13.

Yagishita A, Nakano I, Ushioda T et al (1995) Acute encephalopathy with bilateral thalamotegmental involvement in infants and children: imaging and pathology findings. AJNR Am J Neuroradiol 16:439–447PubMedPubMedCentral

14.

Yamamoto H, Okumura A, Natsume J et al (2015) A severity score for acute necrotizing encephalopathy. Brain Dev 37:322–327CrossRefPubMed

15.

Wu X, Wu W, Pan W et al (2015) Acute necrotizing encephalopathy: an underrecognized clinicoradiologic disorder. Mediators Inflamm 2015:1–10CrossRef

16.

Ibrahim RSM, Elzayat W, Seif HM et al (2020) Multi-parametric magnetic resonance imaging in acute necrotizing encephalopathy of children: validity and prognostic value. Egypt J Radiol Nucl Med 51:113. https://doi.org/10.1186/s43055-020-00214-1CrossRef

17.

Horváthy-Szőcs A, Liptai Z, Dobner S et al (2021) A closer investigation of the synchronous bilateral pattern of MRI lesions in acute necrotizing encephalopathy type 1. AJNR Am J Neuroradiol 42:2251–2253CrossRefPubMedPubMedCentral

18.

Handryastuti S, Silvana S, Yunus RE et al (2022) Difficulties in diagnosis acute necrotizing encephalopathy of childhood: a case report. Child Neurol Open 9:2329048X221095699. https://doi.org/10.1177/2329048X221095699

19.

Aksoy E, Öztoprak Ü, Çelik H et al (2021) Acute necrotizing encephalopathy of childhood: a single-center experience. Turk J Med Sci 51:706–715. https://doi.org/10.3906/sag-2102-47CrossRefPubMedPubMedCentral

20.

Shokry I, Farid TM (2015) PP08.5 – 2322: Acute necrotizing encephalitis of children (ANEC). Eur J of Paediatr Neurol 19(1):S61. https://doi.org/10.1016/S1090-3798(15)30201-4

21.

Chow CK, Ma CKL (2020) Presentation and outcome of acute necrotizing encephalopathy of childhood: a 10-year single-center retrospective study from Hong Kong. J Child Neurol 35:674–680. https://doi.org/10.1177/0883073820927915CrossRefPubMed

22.

Ormitti F, Ventura E, Summa A et al (2010) Acute necrotizing encephalopathy in a child during the 2009 influenza A(H1N1) pandemia: MR imaging in diagnosis and follow-up. Am J Neuroradiol 31:396–400CrossRefPubMedPubMedCentral

23.

Aydin H, Ozgul E, Agildere AM (2010) Acute necrotizing encephalopathy secondary to diphtheria, tetanus toxoid and whole-cell pertussis vaccination: diffusion-weighted imaging and proton MR spectroscopy findings. Pediatric Radiol 40:1281–1284CrossRef

24.

Goo HW, Choi CG, Yoon CH, Ko TS (2003) Acute necrotizing encephalopathy: diffusion MR imaging and localized proton MR spectroscopic findings in two infants. Korean J Radiol 4:61–65CrossRefPubMedPubMedCentral

25.

Neilson DE, Adams MD, Orr CM et al (2009) Infection-triggered familial or recurrent cases of acute necrotizing encephalopathy caused by mutations in a component of the nuclear pore, RANBP2. Am J Hum Genet 84:44–51. https://doi.org/10.1016/j.ajhg.2008.12.009CrossRefPubMedPubMedCentral

26.

Levine JM, Ahsan N, Ho E, Santoro JD (2020) Genetic acute necrotizing encephalopathy associated with RANBP2: clinical and therapeutic implications in pediatrics. Mult Scler Relat Disord 43:102194. https://doi.org/10.1016/j.msard.2020.102194

27.

Ohashi E, Hayakawa I, Murofushi Y et al (2021) Recurrent acute necrotizing encephalopathy in a boy with RANBP2 mutation and thermolabile CPT2 variant: the first case of ANE1 in Japan. Brain Dev 43:873–878. https://doi.org/10.1016/j.braindev.2021.04.009CrossRefPubMed

28.

Jiang J, Wang YE, Palazzo AF, Shen Q (2022) Roles of nucleoporin RanBP2/Nup358 in acute necrotizing encephalopathy type 1 (ANE1) and viral infection. Int J Mol Sci 23:3548. https://doi.org/10.3390/ijms23073548CrossRefPubMedPubMedCentral

Title: Clinical prognostication in acute necrotizing encephalopathy of childhood: the role of magnetic resonance imaging severity assessment
Authors: Kumail Khandwala
Kiran Hilal
Sidra Kaleem Jafri
Syed Musa Mufarrih
Saira Samnani
Ahsun Amin Jiwani
Usman Ali
Publication date: 28-09-2024
Publisher: Springer Berlin Heidelberg
Keywords: Encephalopathy
Magnetic Resonance Imaging
Magnetic Resonance Imaging
Published in: Pediatric Radiology / Issue 12/2024
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI: https://doi.org/10.1007/s00247-024-06058-5

Prediction of feeding difficulties in neonates with hypoxic-ischemic encephalopathy using magnetic resonance imaging-derived radiomics features

Magnetic Resonance Imaging
Original Article

Pseudo hepatic vein thrombosis in a newborn with infracardiac total anomalous pulmonary venous connection

Total Anomalous Pulmonary Venous Connection
Case Report

Mega-corpus-callosum syndrome with cortical malformations

Genetic Testing
Image

Development of a user-friendly calculator for a pediatric split-bolus polytrauma computed tomography protocol

Open Access
Polytrauma
Methodology

Endemic pediatric fungal infections in India: clues to diagnosis

Topical Collection: Global Pediatric Radiology - Perspective from India

Spleen shear wave elastography measurements do not correlate with histological grading of liver fibrosis in Fontan physiology: a preliminary investigation

Portal Hypertension
Original Article

Keynote webinar | Spotlight on adolescent vaping

Live
Webinar | 29-01-2025 | 18:00 (CET)

Growing numbers of young people are using e-cigarettes, despite warnings of respiratory effects and addiction. How can doctors tackle the epidemic, and what health effects should you prepare to manage in your clinics?

Watch it live: Wednesday 29th January, 18:00-19:30 CET

Prof. Ann McNeill

Dr. Debbie Robson

Benji Horwell

Developed by: Springer Medicine

Join the webinar