J Neurol Neurosurg Psychiatry 76:1304-1305 doi:10.1136/jnnp.2004.057588
  • Letter

Remission of progressive multifocal leucoencephalopathy in SLE after treatment with cidofovir: a 4 year follow up

  1. R Reilmann1,
  2. T Imai1,
  3. E B Ringelstein1,
  4. M Gaubitz2,
  5. T U Niederstadt3,
  6. W Paulus4,
  7. I W Husstedt5
  1. 1Department of Neurology, University of Münster, Münster, Germany
  2. 2Department of Internal Medicine B, University of Münster
  3. 3Department of Radiology, University of Münster
  4. 4Department of Neuropathology, University of Münster
  5. 5Department of Neurology, HIV Clinic, University of Münster
  1. Correspondence to:
 Dr R Reilmann
 Department of Neurology, University Clinic Münster (UKM), University of Münster, Albert-Schweitzer Strasse 33, 48129 Münster, Germany;

    Progressive multifocal leucoencephalopathy (PML) is an opportunistic infection caused by human polyomaviruses such as the JC virus. It usually occurs as a severe complication of immunosuppression in patients with primary disorders of the immune system or secondary impairment of immune function, for example, after iatrogenic states of immunosuppression. PML usually takes a rapidly progressive course and advances to death within 1 to 18 months. Today, PML is mainly seen in AIDS, while previously it was typically found in patients with granulomatous, neoplastic, or infectious diseases. In granulomatous diseases particularly, PML is thought to occur as a result of iatrogenic states of immunosuppression, but it is also seen in patients aggressively treated with immunosuppressive agents for systemic lupus erythematosus (SLE).1,2 PML progresses to death in most of these patients even after withdrawing immunosuppressive therapy.2 Therefore additional therapy, aimed at supporting a more rapid restoration of immune function his warranted.

    Case report

    We report a 40 year old woman diagnosed with SLE at the age of 20 years, based on four American College of Rheumatology criteria (erythema, arthritis, elevated antinuclear antibodies, and anti-dsDNA antibodies). Owing to neuropsychiatric lupus (the patient had experienced several psychotic episodes) with suspected vasculitic changes on cerebral magnetic resonance imaging (MRI), the patient had undergone 12 cycles of cyclophosphamide pulse …