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Journal of Pediatric Neurology 2023; 21(02): 085-094
DOI: 10.1055/s-0042-1757166
Original Article

The Clinical, Radiological, and Electrophysiological Profile of Children Presenting with Acute Fulminant Cerebral Edema Due to Suspected Encephalitis in an Eastern Indian Tertiary Care Center

Suman Das 
1   Department of Neurology, Bangur Institute of Neurology, Kolkata, West Bengal, India
,
Biman Kanti Ray
1   Department of Neurology, Bangur Institute of Neurology, Kolkata, West Bengal, India
,
Gobinda Mondal
2   Department of Pediatric Medicine, Dr. B C Roy Post Graduate Institute of Pediatric Sciences, Kolkata, West Bengal, India
,
Dilip Kumar Paul
2   Department of Pediatric Medicine, Dr. B C Roy Post Graduate Institute of Pediatric Sciences, Kolkata, West Bengal, India
,
Kaushani Chatterjee
2   Department of Pediatric Medicine, Dr. B C Roy Post Graduate Institute of Pediatric Sciences, Kolkata, West Bengal, India
,
Lopamudra Mishra
3   Department of Pediatric Medicine, Calcutta National Medical College, Kolkata, West Bengal, India
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Abstract

Objective Our objective was to describe the clinicodemographic, laboratory, and outcome profiles of a rare phenotype of pediatric acute encephalitis syndrome (AES) with acute fulminant cerebral edema (AFCE) and compare them with that of AES without AFCE.

Methods We retrospectively analyzed medical records of a cluster of children hospitalized with encephalitis between June 1, 2021 and December 31, 2021. Their clinical and demographic features, laboratory investigations (hematological, biochemical, serological, microbiological, radiological, and electrophysiological tests), and follow-up data up to 3 months postdischarge were recorded. Patients with AFCE and those without it were divided into groups A and B, respectively, and their characteristics were compared.

Results There were 11 and 15 patients in groups A and B, respectively. There were no significant differences between the two groups in terms of sex, neurological status at admission, hematological and cerebrospinal fluid values, pediatric intensive care unit (PICU) course, and management, etiological identification, and mortality and disabilities at discharge. Patients having reversal or having white cerebellar signs did not significantly differ in their outcomes. However, the patients in group A had significantly lower age, higher incidence of abnormal findings on head computed tomography scans at admission, longer duration of hospitalization, and neurological sequelae at 3 months. The numbers of patients with identified etiologies were zero in group A but five in group B (two Japanese encephalitis, two scrub typhus, and one dengue). Patients of group A had bilateral asymmetric temporal-parieto-occipital T2 hyperintense lesions in magnetic resonance imaging, whereas patients of group B had bifrontal predominant or thalamo-mesencephalic lesions. Multifocal epileptiform discharges were seen in electroencephalogram in both groups, which reverted to normal in 9 and 46% in groups A and B at 3 months, respectively.

Conclusion Younger age is a significant risk factor for the development of AFCE in pediatric AES. AFCE patients have worse outcomes at 3 months, although they do not significantly differ from their non-AFCE counterparts at discharge. When occurring in clusters, AFCE patients exhibit the same radiological and electroencephalographic features.

Keywords

encephalitis - acute fulminant cerebral edema - reversal sign - white cerebellar sign - computed tomography


Publication History

Received: 11 May 2022

Accepted: 01 August 2022

Article published online:
11 October 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Krishnan P, Glenn OA, Samuel MC. et al. Acute fulminant cerebral edema: a newly recognized phenotype in children with suspected encephalitis. J Pediatric Infect Dis Soc 2021; 10 (03) 289-294
    CrossrefPubMedGoogle Scholar
  • 2 Cohen RA, Kaufman RA, Myers PA, Towbin RB. Cranial computed tomography in the abused child with head injury. AJNR Am J Neuroradiol 1985; 6: 883-888
    PubMedGoogle Scholar
  • 3 Glaser CA, Honarmand S, Anderson LJ. et al. Beyond viruses: clinical profiles and etiologies associated with encephalitis. Clin Infect Dis 2006; 43 (12) 1565-1577
    CrossrefPubMedGoogle Scholar
  • 4 Han BK, Towbin RB, De Courten-Myers G, McLaurin RL, Ball Jr WS. Reversal sign on CT: effect of anoxic/ischemic cerebral injury in children. AJNR Am J Neuroradiol 1989; 10 (06) 1191-1198
    PubMedGoogle Scholar
  • 5 Sharawat IK, Kasinathan A, Dawman L. An ominous finding: cerebellar reversal sign. Neuropediatrics 2020; 51 (05) 373-374
    Article in Thieme ConnectPubMedGoogle Scholar
  • 6 Gopalakrishnan S, Uddandam R, Sodhi K. White cerebellum sign. J Nepal Paediatr Soc 2016; 36 (02) 216-217
    CrossrefPubMedGoogle Scholar
  • 7 Malik V, Murthy TVSP, Raj V, Vyas S, Mehar US. ‘White cerebellar sign’ in immediate postpartum period. Med J Armed Forces India 2015; 71 (Suppl. 01) S163-S165
    CrossrefPubMedGoogle Scholar
  • 8 Baby N, Gilvaz P, Kuriakose AM. White cerebellum sign: a poor prognostic sign. Pediatr Neurol 2019; 101: 86-87
    CrossrefPubMedGoogle Scholar
  • 9 Sharawat IK, Kesavan S, Subramani V, Vyas S, Sahu JK, Saini L. Unusual cause of white cerebellum. Indian J Pediatr 2018; 85: 591-592
    CrossrefPubMedGoogle Scholar
  • 10 Mbaba AN, Abam R, Ogolodom MP. White cerebellum sign—an ominous radiological imaging finding: a case report and review of the literature. Biomed J Sci Tech Res 2019; 15 (01) 11159-11161
    PubMedGoogle Scholar
  • 11 Krishnan P, Chowdhury SR. “White cerebellum” sign—a dark prognosticator. J Neurosci Rural Pract 2014; 5 (04) 433
    Article in Thieme ConnectPubMedGoogle Scholar
  • 12 Lan SY, Lin JJ, Hsia SH, Wang HS, Chiu CH, Lin KL. CHEESE Study Group. Analysis of fulminant cerebral edema in acute pediatric encephalitis. Pediatr Neonatol 2016; 57 (05) 402-407
    CrossrefPubMedGoogle Scholar
  • 13 Rao S, Elkon B, Flett KB. et al. Long-term outcomes and risk factors associated with acute encephalitis in children. J Pediatric Infect Dis Soc 2017; 6 (01) 20-27
    CrossrefPubMedGoogle Scholar
  • 14 Bigi S, Fischer U, Wehrli E. et al. Acute ischemic stroke in children versus young adults. Ann Neurol 2011; 70 (02) 245-254
    CrossrefPubMedGoogle Scholar
  • 15 Mishra D, Sharma S, Sankhyan N. et al; Multi-disciplinary Group on Management of Status Epilepticus in Children in India. Consensus guidelines on management of childhood convulsive status epilepticus. Indian Pediatr 2014; 51 (12) 975-990
    CrossrefPubMedGoogle Scholar
  • 16 Sankhyan N, Vykunta Raju KN, Sharma S, Gulati S. Management of raised intracranial pressure. Indian J Pediatr 2010; 77 (12) 1409-1416
    CrossrefPubMedGoogle Scholar
  • 17 LaRovere KL, Riggs BJ, Poussaint TY. et al; Overcoming COVID-19 Investigators. Neurologic involvement in children and adolescents hospitalized in the United States for COVID-19 or multisystem inflammatory syndrome. JAMA Neurol 2021; 78 (05) 536-547
    CrossrefPubMedGoogle Scholar
  • 18 Gentile CP, Hardy D, Agarwal S. Acute fulminant cerebral edema: a profile of 5 pediatric cases from a tertiary care center. Neurology 2020; 94 (15, Supplement): 2152
    PubMedGoogle Scholar
  • 19 Kahle KT, Walcott BP, Nahed BV. et al. Cerebral edema and a transtentorial brain herniation syndrome associated with pandemic swine influenza A (H1N1) virus infection. J Clin Neurosci 2011; 18 (09) 1245-1248
    CrossrefPubMedGoogle Scholar
  • 20 Chalela JA, Rothlisberger J, West B, Hays A. The white cerebellum sign: an under recognized sign of increased intracranial pressure. Neurocrit Care 2013; 18 (03) 398-399
    CrossrefPubMedGoogle Scholar
  • 21 Austin EB, Hobbs H, Crouse BA, Lobos AT. A case report of full recovery from severe cerebral edema secondary to acetaminophen-induced hepatotoxicity in a 13 year old girl. BMC Pediatr 2018; 18 (01) 247
    CrossrefPubMedGoogle Scholar
  • 22 Castro MJI, Gonzalez-Aragon MF. Electroencephalographic findings in patients with viral hermetic and nonherpetic encephalitis. Arch Neurocienc 2009; 14 (01) 3-10
    PubMedGoogle Scholar
  • 23 Maeda K, Chong PF, Akamine S. et al. Case report: acute fulminant cerebral edema with perivascular abnormalities related to Kawasaki disease. Front Pediatr 2021; 9: 732110
    CrossrefPubMedGoogle Scholar
  • 24 Bird CR, Drayer BP, Gilles FH. Pathophysiology of “reverse” edema in global cerebral ischemia. AJNR Am J Neuroradiol 1989; 10 (01) 95-98
    PubMedGoogle Scholar
  • 25 Moose S, Andronikou S. Hypoxic-ischaemic injury—the ‘white cerebellum sign’ versus the true ‘reversal sign’. SA J Radiol 2005; 9 (01) 32-33
    CrossrefPubMedGoogle Scholar
  • 26 Dwarakanath S, Bansal A, Rudrappa S, Gopal S, Venkataramana NK. White cerebellum sign—a case report and review of literature. J Pediatr Neurosci 2006; 1 (Suppl S1): 22-23
    PubMedGoogle Scholar
  • 27 Iyer RS, Thomas B. Teaching neuroimages: reversal sign on CT in a child with hypoxic-ischemic brain injury. Neurology 2011; 76 (06) e27
    CrossrefPubMedGoogle Scholar
  • 28 Singh D, Sharma A. White cerebellum sign: red flag in the emergency department. CJEM 2014; 16 (05) 413
    CrossrefPubMedGoogle Scholar
  • 29 Bhoil S, Bhoil R. Reversal sign: a red-flag in emergency departments. Emerg Nurse 2015; 23 (07) 24-25
    CrossrefPubMedGoogle Scholar
  • 30 Mba SE, Kalangu K, Musara A, Ndlovu D, Zimani NH. White cerebellum sign after ventriculo-peritoneal shunt insertion: a case report and review of the literature, international. J Neurosurg 2018; 2 (02) 31-34
    PubMedGoogle Scholar
  • 31 Sahu AK, Bhoi S. White cerebellum: what do you think?. Emerg Med J 2019; 36 (10) 619-642
    CrossrefPubMedGoogle Scholar
  • 32 Pereira CU, de Carvalho AF, Rabelo NN, Kalkmann GF, Crestani LN, dos Santos LA. White cerebellum sign case series and literature review. Arch Pediatr Neurosurg 2020; 2 (03) e512020
    CrossrefPubMedGoogle Scholar
  • 33 Ninan S, Thompson P, Gershon T. et al. Fatal Pediatric COVID-19 case with seizures and fulminant cerebral edema. Child Neurol Open 2021; 8: X211022532
    CrossrefPubMedGoogle Scholar
  • 34 Kim MG, Stein AA, Overby P. et al. Fatal cerebral edema in a child with COVID-19. Pediatr Neurol 2021; 114: 77-78
    CrossrefPubMedGoogle Scholar