Immunomodulatory therapy in recurrent acute necrotizing encephalopathy ANE1: Is it useful?

Familial acute necrotizing encephalopathy due to mutation in the RANBP2 gene

“…In addition, rapid deterioration of the neurological condition often requires intensive care. Lastly, some authors suggested that acute necrotizing encephalopathy episodes could be gammaglobulin-and/or steroidresponsive [7,9,10].…”

A severity score for acute necrotizing encephalopathy

“…Neurological outcome of ANE is very poor and the mortality and morbidity rates are high [5,10]. Some immunomodulation therapies and hypothermia have been tried for children with ANE [10][11][12].…”

Familial acute necrotizing encephalopathy without RANBP2 mutation: Poor outcome

“…In contrast, the outcome of ANE1 was better, only two deaths in the first episode. Furthermore, two patients had recovered, even after recurrence …”

“…mapped the locus of ANE1 to chromosome 2q12.1‐2q13, and identified mutations in RANBP2 as the cause. Although ANE occurs as frequently in Asia as in North America or Europe, there have been no cases of ANE1 . Lee et al .…”

“…Most cases are sporadic, but some familial or recurrent cases have been reported from North America and Europe. Heterozygous missense mutations of RAN‐binding protein 2 ( RANBP2 ) are found in approximately a half of these cases; this variant of ANE is called ANE1 . Although ANE predominantly affects children in Asia, no ANE1 patients have been reported .…”