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Reversible posterior leukoencephalopathy in connective tissue diseases
Liliane Min1, Jessica Zwerling, Lenore Concepcion Ocava
1Division of Rheumatology, Department of Medicine, Albert Einstein College of Medicine, Bronx, NY 10461, USA. minl01@yahoo.com
Seminars in Arthritis and Rheumatism
|June 13, 2006
Summary
Reversible posterior leukoencephalopathy (RPLS) is a rare neurological disorder affecting patients with connective tissue diseases like systemic lupus erythematosus (SLE). Prompt diagnosis and treatment, including plasmapheresis, can lead to full recovery.
Area of Science:
- Neurology
- Rheumatology
- Radiology
Background:
- Connective tissue diseases, such as systemic lupus erythematosus (SLE) and Wegener's granulomatosis (WG), can present with neurological complications.
- Reversible posterior leukoencephalopathy (RPLS) is a rare clinicoradiological entity characterized by reversible white matter edema, primarily affecting the posterior circulation of the central nervous system.
Observation:
- This report details a case of RPLS in a patient with SLE and reviews 12 additional cases in patients with connective tissue diseases.
- Associated risk factors included malignant hypertension, renal failure, and treatments with cyclophosphamide, cyclosporine, or methylprednisolone.
- Neurological symptoms such as seizures and altered mental status presented diagnostic challenges, with differential diagnoses including infection, uremia, hypertension, infarction, thrombosis, demyelinating disorders, and vasculitis.
Findings:
- RPLS was identified in 13 patients with connective tissue disease, including 9 with SLE, 2 with WG, and 1 with SLE and systemic sclerosis.
- Treatment strategies involved blood pressure control, hemodialysis, drug withdrawal, plasmapheresis, and high-dose methylprednisolone.
- The patient in this case fully recovered with plasmapheresis and high-dose methylprednisolone; most patients experience symptom resolution within two weeks.
Implications:
- Accurate diagnosis of RPLS is crucial for optimal outcomes in patients with connective tissue diseases presenting with neurological symptoms.
- Early recognition and differentiation from other central nervous system disorders are essential for managing this rare but potentially life-threatening condition.
- Understanding the epidemiological, clinical, radiological, and therapeutic aspects of RPLS in connective tissue diseases aids in clinical decision-making.
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