Abstract
Objectives LGI1-antibody encephalitis (LGI1-AbE) is a common form of autoimmune encephalitis. Despite almost universal responses to immunotherapy, patients can have a variety of outcomes and sustained disability. We initiated an international consortium to define the clinical profile of this disorder.
Methods We performed detailed phenotyping of clinical, therapeutic and outcome data in 171 patients with LGI1-AbE from the UK, South Korea, USA, Germany, Switzerland, and Australia.
Results Median age of onset was 62 years (range 22–92). 68% (116/171) were male. The median follow up was four years (range 1–15 years). 23% (39/171) had a history of autoimmunity. 11% (18/171) had an associated malignancy. 35% (59/171) had a relapsing course, with thyroid autoimmune disease being the most common disorder (8%). Patients manifested a mean of two seizure semiologies, including faciobrachial dystonic seizures (57%), focal with loss of awareness (40%), generalised tonic clonic (32%), focal with awareness (19%), thermal seizures (7.5%), and paroxysmal dizzy spells (7.5%). 92% exhibited cognitive disturbance. 59% experienced psychiatric/behavioural features (anxiety 39%, disinhibition 36%, psychosis 36%, depression 27%). 70% experienced sleep disturbance (insomnia 55%, hypersomnolence 32%, sleep wake reversal 18%, parasomnia 11%). Despite seizure resolution in over 90%, 82% experienced late features including emotionality (51%), loss of libido (29%), apathy (28%), anhedonia (22%), obsessive compulsive disorder (21%), sweet food preference (19%), impulse control disorder (12%), and loss of taste/smell (7%).
Conclusions In an international cohort of LGI1-AbE, we identified not only well-recognised acute manifestations, but also only recently-recognised late clinical features which substantially impact quality of life.
