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Abstract Details

First Reported Case of a Patient With Anti-IgLON5 Antibody-positive Corticobasal Syndrome
Autoimmune Neurology
Autoimmune Neurology Posters (7:00 AM-5:00 PM)
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We examined anti-IgLON5 antibody in serum samples of patients with Parkinson’s disease and related disorders. Additionally, we determined whether immunotherapy was effective in patients with anti-IgLON5 antibodies.
Anti-IgLON5 disease is a neurological disorder characterized by disturbed sleep, bulbar dysfunction, movement disorder, and cognitive impairment. Neuropathological findings also reveal the presence of tauopathy.
This retrospective study included 44, 40, 18, and 56 patients with multiple system atrophy, progressive supranuclear palsy, corticobasal syndrome (CBS), and Parkinson’s disease, respectively, which were diagnosed according to disease-specific clinical diagnostic criteria.

In the entire study cohort, only one patient with CBS had anti-IgLON5 antibodies. This patient was a 78-year-old female with a 4-year history of slowly progressive left-sided impairment of dexterity and gait disturbance. Neurological examination showed rigidity, dystonia in left limb, left-sided apraxia, cortical sensory deficit, and gait disturbance. She was diagnosed with probable corticobasal degeneration (CBD) based on the consensus criteria proposed by Armstrong and colleagues. The patient was treated with intravenous methylprednisolone pulse therapy for three days, followed by two courses of intravenous immunoglobulin therapy for five consecutive days; the latter led to gradual improvement of apraxia, gait disturbance, and cortical sensory deficits. Additionally, significant improvement in tracer uptake was observed with single-photon emission computed tomography using 123I-N-isopropyl-p-iodoamphetamine and dopamine transporter.

This is the first report of a patient with anti-IgLON5 disease presenting with CBS that met the diagnostic criteria of probable CBD, although the complete background of the patient’s pathology remains unknown. We also showed the efficacy of immunotherapy in the present patient based on clinical evaluation and radioisotope assessment. The present case highlights the importance of testing for anti-IgLON5 antibodies in patients with CBS who can potentially benefit from immunotherapy.
Authors/Disclosures
Kimiharu Fuseya
PRESENTER
Kimiharu Fuseya has nothing to disclose.
Akio Kimura, MD (Gifu University Graduate School of Medicine) Dr. Kimura has nothing to disclose.
Nobuaki Yoshikura, MD, PhD (Gifu univesity graduate school of medicine department of neurology) Dr. Yoshikura has nothing to disclose.
Akira Takekoshi Akira Takekoshi has nothing to disclose.
Yuichi Hayashi (Department of Neurology, Gifu University Grad) Yuichi Hayashi has nothing to disclose.
Takayoshi Shimohata, MD, FAAN (Department of Neurology, Gifu University) Dr. Shimohata has nothing to disclose.