11. Intensive Care Medicine
                                          
Management of a rare case of new onset refractory status epilepticus in a district general hospital
Author(s):
Katyayani K., Kapoor R.
Institute(s):
East Kent Hospitals University NHS Foundation Trust, Dept of Anaesthesiology & Intensive Care, Canterbury, United Kingdom

Background: New onset refractory status epilepticus(NORSE) is status epilepticus which has continued or recurred despite general anaesthetic for 24 hours or more. The infrequency with which it is encountered and limited evidence base available for treatment strategies make it a formidable management challenge.

Case report: A 29 year old lady developed refractory generalized seizures requiring admission to intensive care and administration of general anaesthetic. EEG was continuously monitored, intravenous infusions of anaesthetic induction agents and benzodiazepines were used in an attempt to maintain a burst suppression pattern or electrographic silence while antiepileptic medications were titrated to therapeutic ranges. CT, MRI, CSF examination and serological test for various infectious, autoimmune and toxic aetiologies were normal. Ketamine infusion, hypothermia, Trans Cranial Electro Magnetic Stimulation, Immunoglobulins and steroids, Ketogenic diet were attempted as seizures remained refractory. Short duration burst suppression was evident with some treatment modalities but none showed sustained effect. Attempts to wean anaesthetic drugs were met with recurrence of electrographic and clinical seizures. After 46 days she was transferred to a tertiary centre where she had further MRI, PET scan, brain and muscle biopsies, mitochondrial and POLG sequencing, enzyme assays but no cause was identified. Plasma exchange was attempted. Her stay was complicated by prolonged Burkholderia Gladioli sepsis. Sedation was gradually weaned off after 63 days(despite periods of worsening EEG with assumption that it was ineffective). Six months later her seizures gradually improved and currently she has around 10-15 brief clinical focal seizures per day, is alert, interacts with environment, haemodynamically stable and is making excellent progress on the rehabilitation ward.
Discussion: In this case NORSE was the presenting feature; no recognisable aetiology was ever identified. A review article published by Simon etal(1) outlines multiple treatment approaches, most of which were attempted at the district general hospital. 

References: 1. Simon shorvon et al: Brain2011:134;2802-2818.

Learning points: This case report emphasises the fact that good recovery can occur after weeks or months of SE especially in cases where no cause has been identified. Hence it is of paramount importance that therapy is continued and premature withdrawal of care is not contemplated.