Recurrent Hemichorea in a Patient with Diabetes and Anti-Phospholipid Syndrome: A Case Report

Recurrent Hemichorea in a Patient with Diabetes and Anti-Phospholipid Syndrome: A Case Report

Chorea is a neurological disorder characterized by involuntary, irregular, and abrupt movements that can affect various parts of the body. It can be caused by a wide range of conditions, including cerebrovascular disease, diabetes mellitus, and systemic autoimmune disorders. Hemichorea, a subtype of chorea, involves unilateral involuntary movements and is often associated with specific underlying pathologies. This case report presents a unique instance of recurrent hemichorea in a patient with both diabetes mellitus and anti-phospholipid syndrome (APS), highlighting the complexities in diagnosis and management.

The patient, a 65-year-old woman, presented with a 3-month history of involuntary movements in her left arm and leg. She had a 10-year history of type 2 diabetes mellitus, which had not been regularly managed. Additionally, she had been diagnosed with secondary APS in June 2018 following episodes of thrombocytopenia and pulmonary embolism. Laboratory tests at the time of APS diagnosis revealed a decreased platelet count of 61 x 10^9/L (normal range: 100–300 x 10^9/L), an increased anti-nuclear antibody (ANA) titer of 185.36 U/mL (normal range: 0–18 U/mL), a lower complement 3 (C3) level of 0.74 g/L (normal range: 0.9–1.8 g/L), an elevated anti-cardiolipin antibody-immunoglobulin G (ACA-IgG) titer of 91.48 GPL-U/mL (normal range: 0–18 GPL-U/mL), positive anti-beta-2 glycoprotein-I antibodies (anti-β2GPI), and prolonged lupus anticoagulant (LAC) tests. These findings, along with imaging evidence of pulmonary embolism, led to the diagnosis of systemic lupus erythematosus (SLE) and secondary APS. The patient was initially treated with prednisone and hydroxychloroquine but declined anticoagulant therapy due to bleeding risks, opting instead for clopidogrel. After three months, her platelet count normalized, and she discontinued all medications without medical advice.

In January 2019, the patient experienced a non-ketotic hyperglycemic episode with a random serum glucose level of 15.22 mmol/L and a hemoglobin A1c (HbA1c) of 16.8% (normal range: 4.0%–6.0%). During this episode, she developed hemichorea affecting her left limbs. A T1-weighted brain MRI revealed high-signal-intensity lesions in the right basal ganglia, consistent with diabetic striatopathy (DS). Her diabetes was managed with subcutaneous insulin injections, leading to significant improvement in her symptoms over the following months. However, three months later, she experienced a recurrence of hemichorea and was admitted for further evaluation.

Upon admission, the patient exhibited persistent involuntary movements in her left limbs. Her fasting blood glucose levels ranged from 4.8 to 6.9 mmol/L, and post-prandial levels ranged from 7.1 to 11.6 mmol/L, with an HbA1c of 7.5%. Repeat cranial MRI showed persistent T1-hyperintensities in the right striatopallidal region, unchanged from the previous scan. Cerebrospinal fluid (CSF) analysis was normal, with no abnormalities in cell counts, glucose, or protein levels. Despite treatment with clonazepam and sulpiride, her symptoms remained poorly controlled.

On the second day of admission, the patient developed acute heart failure (AHF), evidenced by a dramatically elevated pro-B-type natriuretic peptide level of 13,024 pg/mL (normal range: 0–300 pg/mL). Echocardiography revealed reduced left ventricular function with an ejection fraction of 41% (normal range: 50%–70%), and chest radiography confirmed pulmonary edema. Concurrently, her hemichorea worsened. Immunological markers were re-evaluated, showing persistently elevated ACA-IgG (150.1 GPL/mL), positive anti-β2GPI, prolonged LAC tests, increased ANA titer (145.37 U/mL), and low C3 levels (0.88 g/L). These findings indicated that her APS, related to SLE, remained uncontrolled. After excluding common risk factors for heart failure, such as infection, fluid overload, and arrhythmias, it was concluded that the AHF was likely due to lupus activity. Treatment with diuretics, fluid restriction, and methylprednisolone (40 mg/d) was initiated, resulting in complete resolution of the hemichorea within two days.

Diabetic striatopathy (DS) is a syndrome associated with hyperglycemia, characterized by hemichorea, specific imaging findings, and reversibility with glucose control. The exact mechanism of DS is unclear, but it is thought to involve a combination of hyperglycemia and vascular insufficiency leading to transient striatal dysfunction. Hyperglycemia depletes gamma-aminobutyric acid (GABA), the primary inhibitory neurotransmitter, and induces inflammation that disrupts the blood-brain barrier, causing occlusive vasculopathy and transient ischemia in vulnerable striatal neurons. Prompt correction of hyperglycemia typically resolves the symptoms. A meta-analysis of 53 DS cases confirmed the presence of hyperintense basal ganglia lesions on T1-weighted MRI in all patients, consistent with the findings in this case. This suggests that the patient’s first episode of hemichorea was secondary to DS.

Chorea is a rare manifestation of APS, with an estimated prevalence of 1.3%. It is strongly associated with high plasma titers of anti-phospholipid antibodies (aPL). The pathophysiology of APS-related chorea is not fully understood, but it is believed that aPL binds to the endothelium of brain vessels, disrupting the blood-brain barrier and affecting phospholipids in the basal ganglia, leading to choreic movements. Unlike DS, APS-related chorea often lacks characteristic imaging findings, with most patients showing normal brain MRI or age-related small-vessel ischemic changes.

While DS is generally considered reversible, some cases have reported permanent structural lesions and persistent symptoms due to insulin resistance or delayed treatment. In this case, the patient’s diabetes was well-controlled, and her initial hemichorea resolved with glucose management. However, the recurrence of hemichorea coincided with the discontinuation of APS treatment, suggesting that underlying comorbidities played a significant role in the recurrence.

Currently, there are no established diagnostic criteria for APS-related chorea. In this case, the patient had persistently elevated aPL levels, normal cerebral angiography and CSF findings, and well-controlled diabetes. The recurrence of hemichorea, which worsened with AHF and improved dramatically with methylprednisolone, supports the diagnosis of APS-related chorea.

This case highlights the importance of considering both DS and APS in patients presenting with chorea, particularly those with comorbidities such as diabetes and autoimmune disorders. The interplay between these conditions can complicate diagnosis and management, necessitating a thorough evaluation of underlying causes and tailored treatment strategies. The patient’s recurrent hemichorea underscores the need for ongoing management of both diabetes and APS to prevent further episodes.

In conclusion, this case report illustrates the complexities of diagnosing and managing recurrent hemichorea in a patient with diabetes and APS. The first episode was attributed to DS, while the recurrence was linked to uncontrolled APS. The resolution of symptoms with corticosteroid therapy further supports the role of APS in the recurrence of hemichorea. This case emphasizes the importance of a comprehensive approach to patients with chorea, considering both metabolic and autoimmune etiologies to ensure effective treatment and prevent recurrence.

doi.org/10.1097/CM9.0000000000000698

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