Reversible posterior leukoencephalopathy syndrome in p-ANCA-associated vasculitis

Intern Med. 2006;45(20):1169-71. doi: 10.2169/internalmedicine.45.1839. Epub 2006 Nov 15.

Abstract

The perinuclear antineutrophilic cytoplasmic antibody (p-ANCA) is closely associated with rapidly progressing glomerulonephritis, microscopic polyangiitis, and allergic granulomatous angiitis. While mononeuropathy due to vasculitis is a well-known neurological manifestation of these conditions, manifestations involving the central nervous system (CNS) have rarely been reported. Our patient presented the very characteristic CNS lesion of reversible posterior leukoencephalopathy syndrome (RPLS) which has often been associated with hypertension, eclampsia, cyclosporine neurotoxicity, and other diseases. The patient also developed the recently established disease entity, Takotsubo cardiomyopathy.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Anti-Inflammatory Agents / therapeutic use
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Cardiomyopathies / complications*
  • Cardiomyopathies / diagnostic imaging
  • Cerebrovascular Disorders / drug therapy
  • Cerebrovascular Disorders / etiology*
  • Diplopia / etiology
  • Edema / etiology
  • Edema / physiopathology
  • Fatal Outcome
  • Female
  • Fever / etiology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Methylprednisolone / therapeutic use
  • Multiple Organ Failure / etiology
  • Rheumatoid Factor / analysis
  • Ultrasonography
  • Vasculitis / complications*
  • Vasculitis / diagnosis
  • Vasculitis / immunology
  • Vasodilation

Substances

  • Anti-Inflammatory Agents
  • Antibodies, Antineutrophil Cytoplasmic
  • Immunosuppressive Agents
  • Rheumatoid Factor
  • Methylprednisolone