Objective: To report on the possible association of motor tics and anti-IgLON5 disease

Background: Anti-IgLON5 disease is an antibody-mediated neurodegenerative syndrome characterised by sleep disorders, progressive supranuclear palsy, a bulbar syndrome and cognitive decline [1]. Movement disorders are frequently part of the disease phenotype, with gait disorders and chorea being most common [2]. Many patients experience multiple concurrent movement disorders [2]. Immunotherapy is the mainstay of management, although patients with classical phenotype may be less likely to respond to therapy [3].

Method: A 72 year old lady, with a background of relapsing polychondritis, presented with months history of abnormal, irregular repetitive movements of the right shoulder which suppressed with concentration. She described associated dysphagia and insomnia. She was noted to have a variety of purposeless, slow repetitive movements of the right face and eyes and of the left neck. These movements were associated with mounting tension when suppressing them and generalised motor restlessness. Retrospectively, she described motor tics since childhood with occasional vocalisations consistent with a late diagnosis of Tourette’s syndrome.

Results: In view of new dysphagia, sleep change and worsening involuntary movements an autoimmune panel was performed, which showed a negative ANA and ANCA with positive IgLON5 antibodies, confirmed on HEK293 transfected cells. MRI Brain showed a left parietal meningioma without mass effect and a normal MRI cervical spine. EEG was unremarkable. Barium swallow did not show evidence of oesophageal dysmotility. Brain PET scan showed normal glucose metabolism. She was commenced on an induction course of IVIG (2g/kg) which resulted in an improvement in her involuntary movements.

Conclusion: Suppression of involuntary movements, in the context of anti-IgLON5 positivity, with IVIG raises the possibility of motor tics being part of the phenotype of this disease. Further study is required to determine the true association between anti-IgLON5 disease and motor tics.

References: 1. Gaig C, Graus F, Compta Y, Högl B, Bataller L, Brüggemann N, et al. Clinical manifestations of the anti-IgLON5 disease. Neurology. 2017;88(18):1736-43.
2. Gaig C, Compta Y, Heidbreder A, Marti MJ, Titulaer MJ, Crijnen Y, et al. Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease. Neurology. 2021;97(14):e1367.
3. Cabezudo-García P, Mena-Vázquez N, Estivill Torrús G, Serrano-Castro P. Response to immunotherapy in anti-IgLON5 disease: A systematic review. Acta Neurologica Scandinavica. 2020;141(4):263-70.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/worsening-tic-disorder-expanding-the-phenotype-of-iglon5-disease/