An eight-year-old boy survived 200 seizures in a single day due to an extremely rare epilepsy syndrome after doctors utilised a multi-step approach for his stability. The boy suffered an infection-related epilepsy syndrome, which is so rare that only 200 of such cases have been reported worldwide.
Maaz Ansari was admitted to hospital presenting with persistent seizures following what initially appeared to be a routine fever. His condition was so severe that he was having more than 200 seizures over a period of 24 hours. Doctors diagnosed him of suffering from a rare infection-related epilepsy syndrome called FIRES. Realising the urgency of the situation, the medical staff at KJ Somaiya Hospital and Research Centre, including chief pediatric intensivist Dr. Irphan Ali, pediatric neurologist Dr. Ankit Pandey, and a team of pediatricians including Dr. Deepika Shenoy Dr. Santosh B. and Dr. Usman, decided to adopt intensive and specialised care.
He was admitted to the Pediatric Intensive Care Unit (PICU), where he received specialised care including 10 anti-epileptic medications, drug-induced coma, extended ventilator support for 40 days, and nutritional management through a ketogenic diet. Diagnostic tests revealed high inflammation in the fluid around the brain, for which appropriate anti-inflammatory treatment was administered through intravenous and intrathecal routes.
After approximately two months of intensive care, including significant time on respiratory support, his condition stabilised sufficiently for him to be discharged. The medical team continues to monitor his progress through regular follow-up appointments, and they report that his condition remains stable.
Dr. Irphan Ali, paediatrician and neonatologist at the hospital, said, “The triggers for this condition are often a seemingly minor, nonspecific febrile infection, such as a cold or upper respiratory infection. This infection is typically followed by the onset of super-refractory status epilepticus, meaning seizures that are difficult to control with standard treatments. Apart from this, there are several other challenges that come up for treatment, which include diagnostic, therapeutic, and long-term impact on the individual. The high mortality and morbidity rate further adds to several complications.”
What is FIRES?
Febrile Infection-Related Epilepsy Syndrome (FIRES) is such a rare condition that doctors believe that only about 200 cases are reported worldwide, and the estimated incidence is 3.4 to 7.2 per 1 lakh per year. It is characterised by the onset of difficult-to-control, frequent instances of seizures that develop following a febrile illness, despite the absence of a detectable brain infection.
Dr. Manisha Bobade, CEO, K J Somaiya Hospital & Research Centre, said, “In India, the instances of rare diseases and conditions being reported are increasing with each year. Such conditions not only present varied challenges in their diagnosis but in their treatment as well. FIRES is one such rare condition that needs specialised care for each patient, as there are very few cases reported around the world."
