Factors associated with outcomes of severe acute necrotizing encephalopathy: A multicentre experience in Malaysia

Abstract

Acute necrotizing encephalopathy (ANE) in childhood is a rare clinico-radiological syndrome with distinctive bilateral thalamic lesions. The diagnosis is often based on the criteria proposed by Mizuguchi et al. 1 It comprises a spectrum of virus-associated encephalopathy, 2 often but not exclusively associated with influenza infection and a familial and a relapsing form have been reported. The mortality rates are varied, ranging from 0% to 43% and survivors often have severe disabilities. [3][4][5][6][7][8] Whilst there is no clear consensus for ANE treatment, 9 the key therapeutic strategy is the early initiation of firstline immunotherapy, particularly of corticosteroids. 4,9 More C

Acute necrotizing encephalopathy of childhood: Prevention is better than cure especially if the cure remains elusive

“…Their use of an active, case-seeking methodology likely bypassed some difficulties in discussing and documenting the FND diagnosis, such as the fear of increasing stigma for the patient or inciting criticism of the clinician. 1 However, other causes of underdiagnosis -utilizing higher diagnostic thresholds for FND, the perception that FND misdiagnoses are penalized, or the outdated belief that FND is a diagnosis of exclusion 1 -may have led clinicians to underreport in the present study. Their ascertainment method required that physicians opt-in to report FND cases.…”

“…Received: 12 March 2023 | Accepted: 17 March 2023 DOI: 10.1111/dmcn.The recent publication by Lee et al1 highlighting the high mortality and morbidity of acute necrotizing encephalopathy of childhood (ANEC) has caught our attention, shedding light on this rare but serious neuroinflammatory disorder that occurs in association with influenza and other neurotropic viruses 2. By contrast to European cohorts and the recently published Canadian cohort,3 where 23% (7/20) have dominant missense mutations in RanBP2/Nup358 causing a This commentary is on the case series by Lee et al on pages 1256-1263 of this issue.familial…”

Immune‐mediated encephalitis

“…A retrospective multicentre Malaysian study identified 27 children with ANE over a 12 month period with one-third mortality. 25 While there is no consensus as to the optimum treatment strategy, there is emerging evidence that early and multimodal immunotherapy may at least improve mortality. 25 Alongside broad immunomodulatory strategies, the postulated role of excitotoxicity has led to neuroprotective strategies such as therapeutic hypothermia being employed.…”

“…25 While there is no consensus as to the optimum treatment strategy, there is emerging evidence that early and multimodal immunotherapy may at least improve mortality. 25 Alongside broad immunomodulatory strategies, the postulated role of excitotoxicity has led to neuroprotective strategies such as therapeutic hypothermia being employed. 26 Despite best efforts, the mortality and morbidity associated with virus-associated encephalopathy syndromes, and ANE particularly, remain high, and prevention may be crucial.…”

“…In general, virus‐associated encephalopathy syndromes can be associated with significant morbidity and mortality. A retrospective multicentre Malaysian study identified 27 children with ANE over a 12 month period with one‐third mortality 25 . While there is no consensus as to the optimum treatment strategy, there is emerging evidence that early and multimodal immunotherapy may at least improve mortality 25 …”