Reversible Posterior Leukoencephalopathy in Connective Tissue Diseases

Liliane Min, Jessica L. Zwerling, Lenore Concepcion Ocava, I. Hweii Amy Chen, Chaim Putterman

Research output: Contribution to journalArticle

61 Citations (Scopus)

Abstract

Objectives: 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. Conclusion: 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.

Original languageEnglish (US)
Pages (from-to)388-395
Number of pages8
JournalSeminars in Arthritis and Rheumatism
Volume35
Issue number6
DOIs
StatePublished - Jun 2006

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Leukoencephalopathies
Connective Tissue Diseases
Systemic Lupus Erythematosus
Granulomatosis with Polyangiitis
Methylprednisolone
Central Nervous System
Malignant Hypertension
Plasmapheresis
Systemic Scleroderma
Uremia
Demyelinating Diseases
Vasculitis
Nervous System Diseases
Acute Kidney Injury
Cyclophosphamide
Infarction
Cyclosporine
Renal Dialysis
Edema
Seizures

Keywords

  • reversible posterior leukoencephalopathy
  • systemic lupus erythematosus
  • Wegener's granulomatosis

ASJC Scopus subject areas

  • Anesthesiology and Pain Medicine
  • Orthopedics and Sports Medicine
  • Rheumatology

Cite this

Reversible Posterior Leukoencephalopathy in Connective Tissue Diseases. / Min, Liliane; Zwerling, Jessica L.; Ocava, Lenore Concepcion; Chen, I. Hweii Amy; Putterman, Chaim.

In: Seminars in Arthritis and Rheumatism, Vol. 35, No. 6, 06.2006, p. 388-395.

Research output: Contribution to journalArticle

Min, Liliane ; Zwerling, Jessica L. ; Ocava, Lenore Concepcion ; Chen, I. Hweii Amy ; Putterman, Chaim. / Reversible Posterior Leukoencephalopathy in Connective Tissue Diseases. In: Seminars in Arthritis and Rheumatism. 2006 ; Vol. 35, No. 6. pp. 388-395.
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