P-AD003. Autonomic dysfunction in CASPR2 antibody associated neurological disease

Abstract

Introduction . Contactin-associated protein-like 2 (CASPR2) antibody-associated neurological disease is a rare autoimmune illness with central and peripheral manifestations. The core clinical features are peripheral nerve hyperexcitability, autonomic dysfunction, insomnia, and fluctuating encephalopathy. We aim to describe the autonomic manifestations in a cohort of patients with CASPR2 antibody-associated neurological disease. Methods . This study was a retrospective chart review that included eight patients with CASPR2 antibody-associated neurological illness. Their medical records were reviewed and details regarding clinical manifestations of autonomic dysfunction and cardiac autonomic function testing (AFT) were collected. The response of autonomic manifestations to immunotherapy was also noted on follow up. Results . Clinically, autonomic manifestations were present in 7/8 patients. Most commonly, cardiovascular manifestations (palpitations, postural dizziness, tachycardia, fluctuating blood pressure, and postural hypotension) were noted in six patients while others included gastrointestinal (diarrhea or constipation), genitourinary (urinary frequency, urinary retention), and sudomotor (hyperhidrosis) manifestations in three patients each. One patient developed a hypertensive emergency with posterior reversible encephalopathy syndrome. AFT was carried out in five patients and it showed severe cardiac autonomic dysfunction in three and definite autonomic dysfunction in two patients. All patients had a reduction in total power with sympathetic dominance. On follow-up after treatment with immunotherapy (mean duration 19.71 ± 17.61 months; range, 2–52 months), all patients had improvement in autonomic symptoms and one patient had persistent tachycardia but no symptoms. Conclusion . Autonomic dysfunction is a core feature of CASPR2 antibody-associated neurological disease and is usually multi-organ in nature with varied manifestations. Cardiac AFT shows sympathetic dominance. While the clinical response to treatment is good, cardiac autonomic dysfunction in the acute period can lead to dangerous fluctuations in blood pressure and result in complications.