Kurnool KIMS doctors treat rarest NORSE disease of 9-yr boy

KURNOOL: A boy, who was suffering from the rarest NORSE disease, was treated on time successfully by doctors at KIMS hospital, Kurnool. Pediatric Neurologist Dr. Swetha Rampally, Paediatric critical care doctors Dr. Naveen Reddy, Dr. Vasu, and Dr. Ravikiran attended to the boy on time and saved his life. The problem is not known not only to the common public but also to the medical fraternity well had to be treated diligently. Otherwise, it could be fatal.

Dr. Swetha Rampally, consultant Paediatric Neurologist explained the case details.

"Deepak is a 9-year-old, studying 4th class without any medical history. He is a resident of Adoni, Kurnool district. He was brought to KIMS hospital, Kurnool in March 2023, with four episodes of seizures and one day of fever. He was put on a ventilator at admission because of frequent seizures and poor consciousness. The pediatric critical care team & pediatric neurology team took responsibility. He was started on neuroprotection and antibiotics for suspected meningitis (brain infection). However, the tests (CSF & MRI brain) for meningitis were not suggestive of it.

He continued to have multiple frequent seizures for which he was put on anti-seizure medications and medically induced coma to control the seizures. We suspected it could be NORSE (New-Onset Refractory Status Epilepticus) based on EEG pattern and clinical observation. He was started on immunotherapy for the NORSE. Regular EEG monitoring helped in detecting and controlling even brief seizures. He responded to the treatment gradually, and we achieved seizure control after two weeks of admission. Meticulous infection control was challenging and was well taken care of by the critical care team. Excellent nursing care, physiotherapy, and nutrition care were also involved. He remained in the hospital for six weeks. Adequate and timely communication with the family remained fundamental to our management protocol. We explained to them why some tests were done, why some treatments were prescribed, and what future lay ahead of the child to provide clarity into this rare disorder with uncertainty.

NORSE (New-Onset Refractory Status Epilepticus) is a rare devastating condition that can occur abruptly in previously healthy people mostly in children and young adults. Awareness of this rare disease within the general public is minimal and is limited even in the medical community currently. A clear cause generally remains unknown. It has an unpredictable clinical course, requiring prolonged critical care and neurological care, with aggressive therapies and intensive monitoring. It can result in severe life-altering neurological consequences, drug-resistant epilepsy, or death. Almost all survivors need multiple antiseizure medications and they need to be followed up by regular pediatric neurology check-ups for better outcomes. Early recognition and early initiation of appropriate treatment can be lifesaving and minimize complications," explained Dr.Swetha Rampally & Dr. Naveen Reddy.