Objective: In the present case report, we aim to describe a patient suffering from a seldom autoimmune encephalitis presenting with isolated progressive hemichorea.

Background: Anti-IgLON5 disease is a novel complex neurological disorder with neuroimmunological and neurodegenerative aspects. The clinical manifestation of anti-IgLON5 disease includes sleep disturbances with non-rapid eye movement (REM) sleep parasomnia and REM behavior disorder besides obstructive sleep apnea syndrome and stridor as well as other heterogeneous neurological symptoms comprising bulbar symptoms, gait abnormalities, movement disorders, autonomic dysfunction, and neurocognitive alterations in different combinations and severity.

Method: Case report summarizing clinical history, diagnostic work-up and treatment of a patient, in whom antibodies against the neuronal surface antigen IgLON5 were detected.

Results: This 87-year-old woman has presented to our clinic with progressive right-sided hemichorea for two years. Apart from the hemichorea the patient did not complain about further neurological symptoms. Extensive routine laboratory blood diagnostics including molecular-genetic testing for Huntington’s disease as well as cerebral magnetic resonance imaging (MRI) and whole-body [18]F-FDG PET/CT scan revealed normal results. As the patient declined lumbar puncture, the testing for onconeural antibodies and antibodies directed towards neuronal surface antigens was solely done from the serum. Antibodies against the neuronal surface antigen IgLON5 could be detected. Moreover, polysomnographic examination was performed and revealed normal sleep architecture without any sleep-related symptoms.

Conclusion: The clinical spectrum of anti-IgLON5 disease continues to expand. Here we report the first case of anti-IgLON5 disease presenting with isolated progressive hemichorea.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/anti-iglon5-mediated-autoimmune-encephalitis-presenting-with-hemichorea/