SOX1-Positive Encephalitis Presenting as New-Onset Refractory Status Epilepticus (NORSE): A Rare Paraneoplastic Manifestation
Neetika Sharma1, Ashish Sharma1, Roopa Sharma2
1AIIMS Bilaspur India, 2Rutgers University New Jersey USA
Objective:
Here we present a case of a female who presented with new onset refractory status epilepticus (NORSE) .On investigation she found out to be suffering from lung carcinoma with no brain metastasis . She had NORSE as a paraneoplastic manifestation secondary to a rare positive SOX1 paraneoplastic antibody whose definitive therapy guidelines are still lacking. We highlight the importance of definitive therapy and need of treating underlying disease for complete recovery .
Background:
A 70-year-old female with no comorbidities presented with 1 hour of generalised tonic clonic seizures . Despite IV lorazepam, levetiracetam, and phenytoin, her seizures persisted and she required sedation, intubation, and ICU monitoring. Her seizures stopped after anaesthetic use and was extubated 72 hours later.
Routine investigations and MRI Brain were normal; CSF analysis revealed 5 cells , 70 mg/dl protein and 54 mg/dl sugar and a paraneoplastic panel was SOX1-positive. She was treated with IV methylprednisolone and IVIG. She again started to have focal seizures and her antiepileptic dose was adjusted and was given IV rituximab . CECT chest showed a right suprahilar mass with mediastinal lymphadenopathy. Due to her age and poor GCS (E2V1M4), her family declined further procedures. EEG was done to rule out non convulsive status epilepticus and showed delta slowing only with no epileptiform discharges. She was seizure-free for the last month of her hospital stay and was discharged in stable condition.
Design/Methods:
Not applicable.
Conclusions:
SOX1-positive antibodies are strongly associated with an underlying malignancy. While immunotherapy can help manage symptoms, full recovery is only achieved after the underlying malignancy is treated.
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