From the Guidelines
Extrapyramidal manifestations of CNS lupus primarily include chorea, which is the best documented movement disorder in SLE, associated with antiphospholipid antibodies and/or APS. These manifestations occur due to immune-mediated damage to the basal ganglia. The symptoms can significantly impact quality of life and may indicate active disease requiring aggressive immunosuppression to prevent permanent neurological damage. According to the EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations 1, chorea is characterized by irregular, involuntary, and jerky movements involving any part of the body in random sequence. Some key points to consider in the management of these manifestations include:
- Brain imaging should be considered when other focal neurological signs are present or to exclude secondary causes of chorea.
- Symptomatic therapy with dopamine antagonists is usually effective.
- Glucocorticoids in combination with immunosuppressive agents (azathioprine, cyclophosphamide) may be used to control NPSLE disease activity.
- Antiplatelet and/or anticoagulation therapy is administered in antiphospholipid-positive patients, especially when other antiphospholipid/APS-related manifestations are present. Early recognition and prompt treatment are essential to improve morbidity, mortality, and quality of life outcomes in patients with CNS lupus.
From the Research
Extrapyramidal Manifestations of CNS Lupus
The extrapyramidal manifestations of Central Nervous System (CNS) lupus include:
- Parkinsonism: characterized by symptoms such as mask-like facies, cog-wheel rigidity, and bradykinesia 2, 3, 4
- Myoclonus: a type of movement disorder that can occur early in the disease course 5
- Chorea: a less common manifestation of CNS lupus, but still a recognized extrapyramidal symptom 6, 2
- Akinetic mutism: a rare condition characterized by a lack of movement and speech, which can occur in some cases of CNS lupus 4
Clinical Presentation and Diagnosis
These extrapyramidal manifestations can present in various ways, including:
- Global slowing of movements and slowing of speech 3
- Mask-like facies with a faint malar rash sparing the nasolabial folds, hard palate ulcer, cog-wheel rigidity, and proximal muscle weakness 3
- Bradykinesia, mutism, and shuffling gait 4
- Akinetic mutism and masked face, with abnormal signals in bilateral basal ganglia on MRI brain and hypoperfusion in the same area on SPECT 4
Treatment and Management
Treatment of these extrapyramidal manifestations typically involves immunosuppression, such as: