Reversible posterior leukoencephalopathy in connective tissue diseases.
OBJECTIVE
To describe a case of reversible posterior leukoencephalopathy (RPLS) involving a patient with systemic lupus erythematosus (SLE) and to review the medical literature to define the epidemiological, clinical, radiological, and therapeutic aspects of this syndrome in various connective tissue diseases.


METHODS
Report of 1 case and review of the English literature using Medline search from 1967 to 2005.


RESULTS
Including our reported case, RPLS has been identified in 13 patients with connective tissue disease. In separate case reports, 9 SLE patients, 2 Wegener's granulomatosis (WG) patients, and 1 patient with SLE and systemic sclerosis presented with RPLS. Associated risk factors included malignant hypertension, acute renal failure, and recent treatment with cyclophosphamide, cyclosporine, or methylprednisolone. Patients were treated with blood pressure control, hemodialysis, or withdrawal of the offending drug. In our patient, plasmapheresis and high-dose methylprednisolone resulted in a full recovery. In most cases, complete resolution of neurological symptoms occur within 2 weeks of presentation, along with improvement or resolution of imaging abnormalities.


CONCLUSIONS
RPLS is a clinicoradiological entity, associated with reversible white matter edema involving most commonly the posterior central nervous system circulation. Seizures and altered mental status in patients with SLE or WG can pose difficult diagnostic and therapeutic challenges. The differential diagnosis is broad and includes infection, uremia, hypertension, infarction, thrombosis, demyelinating disorders, and vasculitis. Accurate diagnosis of RPLS and its differentiation from other, more common causes of the central nervous system is essential to ensure the best possible outcome in this rare but life-threatening neurological disorder.
Min
Liliane
L
Division of Rheumatology, Department of Medicine, Albert Einstein College of Medicine, Bronx, NY 10461, USA. minl01@yahoo.com
Zwerling
Jessica
J
Ocava
Lenore Concepcion
LC
Chen
I-Hweii Amy
IH
Putterman
Chaim
C
D000328:Adult; D003240:Connective Tissue Diseases / Q000150:complications*; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D006801:Humans; D007968:Leukoencephalopathy, Progressive Multifocal / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008181:Lupus Nephritis / Q000628:therapy; D014057:Tomography, X-Ray Computed