Objective: We describe a 93-year-old man with subacute, progressive onset of disabling orofacial dyskinesias (OD), found to have positive lupus anticoagulant (LA) and anti-beta2-glycoprotein IgA (anti-β2GP).

Background: ODs are movement disorders characterized by continuous, orofacial and tongue movements, most commonly associated with tardive dyskinesia (TD) and neuroleptic exposure. OD is also seen with edentulousness, autoimmune/paraneoplastic conditions, and neuroacanthocytosis, among others.

Method: Our patient is a 93-year-old man with coronary artery disease and hypertension, who had 2-3 weeks of progressively worsening, disabling ODs interfering with feeding. He had not eaten in 4 days. He was never exposed to neuroleptics or offending medications. He had well-fitting dentures for many years. Exam notable for severe, continuous ODs interfering with speaking and eating; no chorea elsewhere and no rigidity, ataxia, or tremor. CT head showed moderate small vessel ischemic changes with mild atrophy, without ventriculomegaly or subacute or chronic strokes. MRI was contraindicated. CBC, CMP, thyroid panel, B12, copper, ceruloplasmin, syphilis, vitamin E, ANCA, and ANA were all normal. No acanthocytes noted. Iron studies showed mild anemia. Amantadine and clonazepam improved ODs and he passed a swallow evaluation. Weeks later, testing resulted for positive LA and positive anti-β2GP IgA at 23.12 units. He did not have a history of thrombotic events or strokes. Unfortunately, the patient had a decline in health and family chose comfort care.

Results: The most common cause of ODs is TD, however, occurrence is very rare otherwise. Antiphospholipid antibody syndrome is a rare cause of chorea, occurs in 1.3% of patients; typically women in their 40s and children. It is believed to be due to thrombosis or disruption of the blood brain barrier with direct neural damage to surface antigens with dopaminergic properties. Edentulousness and ill-fitting dentures are linked to ODs, but is mild in 70% of cases and never disabling. Our patient is unique as he was of advanced age, was never exposed to neuroleptics or offending medications, had well-fitting dentures, did not have a history of thrombotic events or strokes, and had no other manifestations outside of disabling ODs, and had positive LA and anti-β2GP.

Conclusion: Antiphospholipid antibody syndrome should be considered in the elderly population and may potentially cause disabling dyskinesias.

References: 1. Panzer, Jessica, and Josep Dalmau. “Movement disorders in paraneoplastic and autoimmune disease.” Current opinion in neurology 24.4 (2011): 346. 2. Gelosa, G., et al. “Narrowing the window for ‘senile chorea’: A case with primary antiphospholipid syndrome.” Journal of the neurological sciences 284.1 (2009): 211-213. 3. Peluso, Silvio, et al. “Antiphospholipid-related chorea.” Frontiers in neurology 3 (2012): 150.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/a-case-of-disabling-orofacial-dyskinesias-caused-by-lupus-anticoagulant/