Neurological Disorders

New-onset super-refractory status epilepticus following fever: more than a case of NORSE

Abstract

Alicia Hernando-Asensio*, María Asunción Martín-Santidr, Jesús Macarrón-Vicente, Ana Isabel Gómez-Menéndez, Beatriz García-López, Mónica Bártulos-Iglesias and Daniel Pascual-Carrascal

We present the case of a healthy 32-year-old woman who came to our hospital due to fever and left otalgia. She subsequently developed dizziness and gait instability, opsoclonus-myoclonus syndrome, and altered level of consciousness. She was admitted to the intensive care unit, developing status epilepticus, which was refractory to third-line treatment with propofol and barbiturates. A thorough assessment including autoimmunity studies, viral testing, and mitochondrial disease testing yielded negative results. A brain magnetic resonance imaging scan revealed signal hyperintensities in both caudate nuclei and putamina, and gadolinium-enhancing small punctiform lesions in the left hemisphere and left cerebellar tonsil. Suspecting an immune-mediated disorder, we started treatment with high-dose steroids and plasmapheresis, in addition to different combinations of antiepileptic drugs. The patient was refractory to these treatments; electroconvulsive therapy improved the EEG tracing and may have helped manage status epilepticus. Subsequent examinations revealed paralysis of left cranial nerves IX, X, and XII, which resolved nearly completely in the final days of hospitalisation, leaving the patient practically asymptomatic. Our findings point to encephalitis with brainstem involvement and manifesting as cryptogenic new-onset refractory status epilepticus.