Objective: To report a case of hemichorea due to seronegative autoimmune process.

Background: Autoimmune encephalitis can present with diverse hyperkinetic or hypokinetic phenomenology. Autoimmune movement disorders may be challenging to diagnose and can be misdiagnosed as neurodegenerative or metabolic conditions, leading to delayed or missed treatment opportunities. Thus, recognition of characteristic clinical patterns of an autoimmune movement disorder is critical to improving outcomes.

Method: Case report

Results: A 72-year-old female with history of Crohn’s disease, on adalimumab presented with three months of progressive right-sided hemichorea, orofacial dyskinesias, well-formed visual hallucinations, insomnia, dream enactment, and cognitive decline. Brain magnetic resonance imaging (MRI) showed microhemorrhages in left putamen, left cerebellum and anterior right frontal lobe, as well as mild generalized atrophy. Electroencephalography showed mild diffuse slowing. Cerebrospinal fluid studies (CSF) demonstrated pleocytosis (21 nucleated cells/mm3), positive CSF oligoclonal immunoglobulin G (IgG) bands, and elevated CSF IgG index (2.1). Autoimmune/paraneoplastic panels in serum and CSF were negative. Chest, abdomen and pelvis computed tomography (CT) of the chest, abdomen, and pelvis were negative for malignancy. The patient was initially treated with quetiapine with minimal improvement prior to completion of the above work-up. Haloperidol was subsequently added concurrently with initiation of IV methylprednisolone (IVMP) and IV immunoglobulins (IVIG). Two weeks after completion of IVIG and IVMP and while on a prednisone taper (at 40 mg daily) and haloperidol (4mg daily), hemichorea and orofacial dyskinesias were markedly reduced though circadian rhythm dysregulation and cognitive issues were persistent. She is scheduled to obtain a positron emission tomography CT. Haloperidol is being tapered, and further treatment with tocilizumab is planned.

Conclusion: Autoimmune movement disorders should remain on the differential diagnosis for rapidly progressive hyperkinetic movement disorders as early immunotherapy can provide substantial clinical benefit.

References: –

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MDS Abstracts - https://www.mdsabstracts.org/abstract/seronegative-autoimmune-encephalitis-presenting-as-hemichorea-a-case-report/