Abstracts

Rationale: This is a clinical observational study on 8 children who had new-onset refractory status epilepticus (NORSE), a recently described entity. This report highlights the emergence of generalised dyskinesia during the course of the illness and the good response to ACTH in many of the patients with a good eventual outcome. ‘ Methods: Retrospective chart review identified 7 patients from a tertiary care center in Mumbai, India while one active patient was included from a Philadelphia hospital. Our inclusion criteria were 1) Normal patients prior to onset of status epilepticus (SE) 2) Refractory SE defined as no / transient response to first line AEDs with clinical and / or EEG evidence of ongoing seizures 3) Lack of a known etiology despite exhaustive investigations Results: 8 patients (5 boys, 3 girls) were included with a mean age of 4.9 yrs. They were all previously in good health. 6 patients had a transient febrile illness at onset. Between 3-6 classes of AEDs were used to control SE with all patients being on continuous midazolam infusions and 3/8 patients receiving propofol and / or pentobarbital / pentothal infusions. IVIG / methylprednisone was used in 6 patients wile the ketogenic diet was tried in 4. 5 patients required prolonged ventilation. 7 patients developed moderate to severe persistent dyskinesias (despite discontinuation of phenytoin) which started usually as the SE abated and continued for weeks to months. Exhaustive investigations did not yield a definite etiology in any patient. MR imaging was normal at onset and subsequently showed varying degrees of cerebral atrophy. In 4 patients ACTH was followed by seizure cessation within 2-14 days (mean 6.2 days). The US patient relapsed during ACTH taper after a few weeks only to respond again after an ACTH dose increase. In 2 patients SE responded without ACTH though dyskinesias persisted for 2 and 10 months. Dyskinesias responded within 2 and 4 weeks respectively. At 1-4 years follow-up 4 patients are normal, 1 patient has moderate-severe language delay and the US patient has continuing seizures despite ACTH. Of the 2 patients not given ACTH one is vegetative 5 years later while one has autism and mild MR. Conclusions: A series of 8 cryptogenic NORSE patients is described most of whom developed a significant dyskinetic syndrome which has not been described in other series. We postulate a possible role for ACTH in treatment of NORSE and the subsequent dyskinesia.