Objective: Describing a rare case of CASPR2 medicated auto immune encephalitis presenting with profound chroea

Background: This is a 69 year old retired engineer who presented to the neurology clinic two months after bowel surgery for diverticular disease with abnormal, animated impulsive movement and personality change as described his family and band members. Neurological examination revealed florid chorea but no other significant focal neurological signs. He had insight into his problems and was not confused. Outpatient investigations MRI and autoimmune encephalitis screen was arranged.

Methods: Case report.

Results: A month later he was admitted to hospital with worsening of his symptoms and aggressive behaviour, which was totally out of character for him. He remained alert but was disinhibited and impulsive with profound chorea. MRI brain revealed no abnormality, CSF study was normal but CASPR 2 antibody test was positive. He was commenced on steroids but had poor response and then received a course of IVIG. At this point a cognitive assessment was carried out on which showed some deficit in visuo-spatial abilities. On discharge a reducing regime of steroids was given and he was more settled. In the meantime a PET CT was arranged, which was unremarkable. On further follow up the chorea continued to worsen, he has become more restless, has had two falls and is getting minimal sleep at night. He has now been started treatment with plasma exchange.

Conclusions: CASPR2 antibody normally causes a peripheral nerve excitability . We present a very rare presentation of profound chorea caused by CASPR 2 medicated auto immune encephalitis.

References: Ballint et al Movement disorders with neuronal antibodies: syndromic approach, genetic parallels and pathophysiology Brain, Volume 141, Issue 1, 1 January 2018, Pages 13–36,

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MDS Abstracts - https://www.mdsabstracts.org/abstract/caspr2-antibody-mediated-autoimmune-encephalitis-presenting-with-profound-chorea/