Stiff and partly blind--after a first dose of steroid.

In June, 2006, a 29-year-old woman, fl ying back to England after a 2-week holiday in Cyprus, started to feel unwell and feverish. The day after landing, she saw her general practitioner—who sent her to us, since she had a 13-year history of systemic lupus erythematosus (SLE), manifesting as rash, joint pains, lymphopenia, and feeling tired and unwell. She had no other medical history of note, and took no drugs, other than methotrexate 12·5 mg weekly. She had a red, macular rash all over her body, and blistering sunburn on her chest. When admitted at 1600 h, she was alert; however, she started to lose consciousness, developed petechiae, and went into shock, despite being given intravenous fl uids. The patient was transferred to the intensive-care unit at 2400 h, by which time she was unresponsive and incoherent. Before transfer, we administered ceftriaxone and aciclovir, since we suspected septicaemia or herpes-simplex-virus encephalitis. Blood tests showed anaemia (haemoglobin concentration 52 g/L), severe thrombocytopenia (platelet count 17×109 per L), and renal failure (creatinine concentration 243 μmol/L). However, the white-cell count was only 5·0×109 per L (neutrophils 4·8×109 per L, lymphocytes 0·2×109 per L), the concentration of C-reactive protein only 17 mg/L, and the ESR 14 mm/h—indicating that perhaps the patient did not, after all, have an infection. We did not do a lumbar puncture, because of the severe thrombocytopenia. We were unable to fi nd evidence of a focal infection, and microscopy of cultured fl uid from the petechiae showed no bacteria. To our surprise, however, a blood culture was positive for Neisseria meningitidis. A possible diagnosis, other than sepsis, was thrombotic thrombycytopenic purpura (TTP). However, blood tests, including a blood fi lm and direct antiglobulin test, showed no evidence of haemolytic anaemia—so we concluded that TTP was unlikely. We continued to give intravenous fl uids and ceftriaxone: 7 days after admission, the anaemia and thrombocytopenia had resolved, the rash and bruises had disappeared, and the patient was alert. However, the patient became visibly oedematous: on day 8 of her hospital stay, we diagnosed nephrotic syndrome (24 h urinary protein excretion 4·92 g)—which, in the context of low concentrations of complement components (concentrations of C3 and C4 0·10 g/L and 0·03 g/L, respectively), led us to diagnose lupus nephritis. We therefore prescribed methylprednisolone; within 3 h of being given the fi rst dose, the patient developed an intense frontal headache and photophobia, and had two tonic-clonic seizures in 1 h. Examination showed increased tone in her arms, and cortical blindness (homonymous hemianopia, with normal pupillary refl exes). Urgent MRI revealed reversible posterior leucoencephalopathy syndrome (RPLS; fi gure). The seizures were controlled with phenytoin; within 2 weeks, the patient recovered fully. 3 months later, repeat MRI, blood tests, and urine tests showed nothing abnormal. When last seen, in February, 2008, the patient was well. RPLS was fi rst recognised in 1996; it is characterised by oedema of the parietal and occipital lobes, in which T2-weighted MRI shows hyperintense white-matter abnormalities. Clinical features include headache, vomiting, altered mental state, seizures, cortical blindness, and transient motor abnormalities. RPLS is thought to occur when a rapid increase in mean arterial pressure causes arteriolar dilation—which, in the presence of endothelial injury, causes the blood-brain barrier to leak. RPLS is associated with hypertension, and also with nephrotic syndrome, and treatment with immuno suppressive drugs or steroids. RPLS is treated by addressing its cause. Although its name implies that RPLS is reversible, some patients have persistent brain damage; RPLS can cause infarction, haemorrhage, or intracranial hypertension, leading to death.