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Reversible posterior leucoencephalopathy in scleroderma
  1. W L Poon1,
  2. C C Mok2
  1. 1Department of Diagnostic Radiology, Tuen Mun Hospital, Hong Kong
  2. 2Department of Medicine, Tuen Mun Hospital, Hong Kong
  1. Correspondence to:
    Dr C C Mok
    Department of Medicine, Tuen Mun Hospital, Tsing Chung Koon Road, New Territories, Hong Kong;

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A 34 year old Chinese woman with limited scleroderma presented with rapid onset of mental confusion and generalised tonic-clonic seizures. Her blood pressure control had been unsatisfactory in the preceding 4 weeks despite the use of three anti-hypertensive agents, which included an angiotensin converting enzyme inhibitor. Malignant hypertension (blood pressure 240/140 mm Hg on admission) was evident, with typical fundoscopic abnormalities, microangiopathic haemolytic anaemia, and rapidly deteriorating renal function with acute oligouric renal failure (increase in serum creatinine from baseline of 86 to 495 μmol/l in 3 days). There was, however, no evidence of left ventricular failure.

Treatment was given in the intensive care unit with infusions of labetalol (up to 150 mg/h) and iloprost (up to 10 μg/h), large doses of captopril (150 mg/day), and haemodialysis. An urgent magnetic resonance imaging (MRI) scan of the brain showed marked vasogenic oedema distributed symmetrically at the cortex, and subcortical white matter of the occipital lobes, the cerebellum, and the brain stem (fig 1). With control of hypertension and dialysis support, she gradually regained full consciousness without neurological deficits. A repeat MRI scan 2 weeks later demonstrated complete resolution of the lesions. The clinical picture was compatible with a reversible posterior leucoencephalopathy syndrome (RPLS).

Figure 1

 (A) Axial T2 weighted and (B) coronal fluid attenuated inversion recovery (FLAIR) images showing bilateral abnormal hyperintensities in the white matter of the cerebellum, cortex, and subcortical white matter of the occipital lobes (long arrows), and in the brain stem (short arrows).

RPLS is a clinical syndrome characterised by headache, seizures, visual disturbances, and confusion. The MRI finding is often characteristic, with abnormal T2 weighted hyperintensity affecting primarily the white matter of the territories of the posterior circulation.1 The cerebral cortex and the anterior circulation territories may also be affected, but usually to a lesser extent. RPLS has been described in an increasing number of medical conditions, including hypertensive encephalopathy, eclampsia, neurotoxicity related to calcineurin inhibitors, and uraemic encephalopathy. Reversible vasogenic oedema is the underlying pathology of the abnormal MRI signal intensities. The exact pathogenesis of RPLS remains elusive but a break down of the autoregulation of cerebral blood flow and endothelial dysfunction resulting in leakage of fluid into the interstitium has been postulated.

RPLS has been described in many rheumatic diseases, including systemic lupus erythematosus, systemic vasculitides, and the overlap syndromes.2,3 However, we believe that this is the first report of RPLS in adult patients with limited scleroderma. Prompt recognition and treatment of this condition is essential as it is potentially reversible.