Objective: To describe the first case of non-paraneoplastic autoimmune encephalitis with antiglycine receptor (anti-GlyR) antibodies manifesting as a Corticobasal Syndrome (CBS) and the response to immunotherapy.

Background: A 55 years old man presented with 1,5 years history of progressive arrhythmic tremor (cephalic onset with subsequent generalization), left sensitive-motor syndrome with rigidity, pain and abnormal posture of left arm, left hemifacial myoclonus, loss of language, visual hallucinations, episodic self-injurious behavior, apathy and no recognition of people and usual objects. Previous personal history of vitiligo.

Methods: Physical examination showed left homonymus hemianopsia, rigidity of the left extremities, left “hand in claw” dystonic posture, left hemisensitive loss with cortical involvement, global aphasia, anterocollis, generalized arrhythmic tremor and impossibility for standing and walking. Blood test, brain and spinal magnetic resonance, single photon emission computed tomography imaging with dopamine transporter (DatScan), body Computed Tomography, possitron emission tomography, neurophysiology test, cerebrospinal fluid (CSF) biochemistry, blood and CSF microbiology, 14.3.3 protein and genetic test were normal. Mirrored oligoclonal bands in CSF and anti-GlyR antibodies in serum were positive. Other autoantibodies in blood and CSF were negatives.

Results: Treatment with intravenous immunoglobulins (IVIg) followed by plasma exchange and Rituximab was effective, with clinical improvement. One year after immunotherapy the patient remains stable. Complementary studies ruled out cancer.

Conclusions: Anti-GlyR antibodies are previously described in association with progressive encephalomyelitis with rigidity and myoclonus (PERM) syndrome which is related with tumors in about 20-30% of cases. Immunotherapy response is positive in most cases. This is the first case of CBS related with anti-GlyR antibodies. Immune-mediated pathology can mimic many neurological diseases, including neurodegenerative and movement disorders. As being potentially treatable, they should be considered in the differential diagnosis.

References: 1. Carvajal González A, Leite MI et al. Glycine receptor antibodies in PERM and related síndromes: characteristics, clinical features and otucomes. Brain 2014 Aug; 137 (Pt 8): 2178-92. 2.Hinson SR, López Chiriboga AS et al. Glycine receptor modulating antibody predicting treateable stiff person spectrum disorder. Nuerology Neuroimmunology and neuroinflammation. 2018 Jan; 23;5(2):e438.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/antiglycine-receptor-antibodies-and-rapidly-progressive-corticobasal-syndrome/