Several names for the syndrom
FIRES syndrome was named in 2009i but was also known as:
• AERRPS – Acute encephalitis with refractory partially repetitive epilepsy
• Acute non-herpetic encephalitis with severe refractory epilepsy
• Idiopathic catastrophic epileptic encephalopathy
• Refractory epileptic encephalopathy fever-induced in children
• DESC Syndrome – (Devastating Epileptic Encephalopathy in School-aged Children).
And even the meaning of the FIRES acronym has changed from Fever Induced Refractory Epileptic Encephalopathy in School-aged children to Febrile Infection-Related Epilepsy Syndrome in 2012.
The variations of these names is a sign of the elusive nature of the disease.
This also explains the difficulty to accurately assess the prevalence of FIRES and the need to centralize medical data.
As research progresses, it is essential to use the same name for this syndrome internationally.
i-Dr Andrea VAN BAALEN –Orphanet2012