Objective: To describe a complex case of atypical parkinsonism compatible with corticobasal syndrome (CBS), associated with autoimmune findings in serum and cerebrospinal fluid (CSF) and significant improvement achieved after immunotherapy.

Background: CBS is a rare neurodegenerative disorder characterized by a progressive, asymmetrical, akinetic rigid syndrome and early cortical signs. This entity is associated with a heterogeneous spectrum of pathologies, and recently described autoimmune etiology.

Patients with anti-GlyR antibody syndrome are, on average 50 years old and suffer from spasms, stiffness, rigidity, myoclonus, and related walking difficulties. Cognitive impairment is present in half of patients. Limb paresis and pyramidal signs are observed in 60% of cases, bulbar disturbance or abnormal reflexes in 57%.Subacute courses occur in 44% of cases. CSF abnormalities are observed in about half of the cases. Intrathecal anti-GlyR antibody synthesis was observed in the some cases. The response to anti-inflammatory treatment is mostly good, with the modified Rankin scale scores dropping from a median of 5 during disease peak to 1.

Method: We describe the case of a 55-years-old male patient had a severe asymmetric akinetic rigid syndrome, with progressive deterioration of gait, becoming wheelchair-bound, sensory disturbances, subacute cognitive impairment with total loss of speech, and self-injurious behaviors. Complementary tests showed CSF with elevated proteins, oligoclonal bands with mirror pattern, and anti Gly-R autoantibodies in serum.

Results: Due to the analytical results, we strongly suspected an autoimmune etiology. After immunotherapy with plasmaferesis, inmunoglobulines and rituximab, the patient had significant improvement in symptoms, being able to communicate with simple words, improving motor symptoms, and being able to walk with a walking support. Self-aggressive behaviors persisted, although with less frequency and intensity

Conclusion: There are few descriptions in the medical literature about clinically manifested  CBS with autoimmune etiology. This case report reveals the importance of considering autoimmune etiology in certain cases of atypical parkinsonism because of the potential benefit of appropriate treatment. The patient’s significant improvement after immunotherapy suggests that this approach may be beneficial in other similar cases.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/autoimmune-encephalitis-due-to-antiglycine-antibodies-presenting-as-corticobasal-syndrome/