Pediatric recurrent acute necrotizing encephalomyelitis, RANBP2 genotype and Sars-CoV-2 infection: Diagnosis, pathogenesis and targeted treatments from a case study

Intense work-up is required for pediatric COVID-related acute necrotizing encephalopathy with RANBP2 variants

“…We read with interest the article by Forest et al about a 10yo female with a history of acute disseminated encephalomyelitis (ADEM) at age 2y from which she recovered without sequelae who suffered SARS-CoV-2 related acute necrotizing encephalopathy (ANE) also known as acute necrotizing encephlaomyelitis (ANEM) at age 10 [ 1 ]. The patient required temporary intubation and artificial ventilation [ 1 ]. Methyl-prednisolone was ineffective but plasma exchange (PE) and intravenous immunoglobulins (IVIGs) were beneficial [ 1 ].…”

“…The patient required temporary intubation and artificial ventilation [ 1 ]. Methyl-prednisolone was ineffective but plasma exchange (PE) and intravenous immunoglobulins (IVIGs) were beneficial [ 1 ]. No long-term follow-up data were provided [ 1 ].…”

“…Methyl-prednisolone was ineffective but plasma exchange (PE) and intravenous immunoglobulins (IVIGs) were beneficial [ 1 ]. No long-term follow-up data were provided [ 1 ]. The study is appealing but carries limitations that raise concerns and should be discussed.…”

“…Expansion of the cerebral lesion occurred under therapy with steroids [ 1 ]. Therefore, it cannot be ruled out that deterioration of MRI abnormalities was due to steroids.…”

“…Therefore, it cannot be ruled out that deterioration of MRI abnormalities was due to steroids. The patient had received IVIGs already for the ADEM at age 2 [ 1 ]. Why were IVIGs not immediately given at age 10 again?…”