Objective:

To present a case of delayed recurrent LGI-1 encephalitis 10 years after initial presentation and highlight knowledge gaps in long-term management and surveillance of autoimmune neurological disorders. 

Background:

LGI-1 Encephalitis is a rare autoimmune encephalitis that is classically considered to be monophasic, however relapse has been reported. High antibody titer, sleep disorders during the acute phase and delay in immunotherapy have been linked to risk of relapse of LGI-1 encephalitis. Prior retrospective cohort studies have established a recurrence rate of ~28% observed during a median of 18-month follow-up. Little is understood about prolonged follow-up and the role of immunosuppression for encephalitis patients in the long-term.

Design/Methods:

NA

Results:

72 yo M presented for evaluation of sensation of “an electrical jolt” down his left neck and spine which awaken him from sleep in the context of recent ischemic stroke. He has a history of LGI-1 autoimmune encephalitis which presented with faciobrachial dystonic seizures. Of note, this patient was in the original 2010 case series describing LGI-1 as the pathogenic target for autoimmune encephalitis. At initial presentation, he was treated with IV methylprednisolone followed by a 6-week oral prednisone taper resulting in seizure freedom for the interim on lamotrigine and gabapentin. Repeat serum antibody testing for investigation of this second episode was positive for LGI-1 antibodies which were again treated with steroids with resolution of symptoms.

Conclusions:

There are currently no guidelines for long-term term management and surveillance of autoimmune encephalitis, including LGI-1. The advantage of this case being one of the first identified cases of LGI-1 encephalitis also lends itself to informing the long-term natural history of the disease. The differences in clinical presentations years apart are poorly understood. Possible avenues for surveillance include serial sodium monitoring. One survey indicated 74% of practitioners treat acute LGI-1 with steroids with practice varying widely on chronic immunosuppression.