A fulminant neurologic presentation of systemic lupus erythematosus

Dear Editor, A 23-year-old woman presented with a few months history of an intermittent rash involving her lower extremities. She then acutely developed left hand weakness and numbness and drowsiness. Physical examination revealed a blood pressure (BP) of 200/130 mmHg, drowsiness, visual acuity of 20/40 each eye, and weakness in the median and ulnar intrinsic muscles of the left hand. Right Babinski’s sign was present and sensation was decreased in essentially the entire left hand. The leg rash described was not initially present. Laboratory investigations revealed a normal complete blood count and creatinine. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were 86 mm/h and 58 mg/L, respectively. Urinalysis showed mildly elevated protein (1.0 g/L) and bland sediment. Pregnancy test was negative. Computed tomography scan of the head showed bilateral hypodensities in the parieto-occipital regions, suggestive of posterior reversible encephalopathy syndrome (PRES), but also bilateral frontal and basal ganglia hypodensities, which is atypical. A vasculitic process was suspected and a work-up was ordered; in the interim, the patient’s BP was lowered, and she was started on methylprednisolone pulse therapy. Magnetic resonance imaging (MRI) of the brain showed hyperintensities bilaterally in the fronto-parieto-occipital regions and basal ganglia on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images (Fig. 1), consistent with PRES. Diffusion-weighted images (DWI) and apparent diffusion coefficient (ADC) maps showed evidence of restricted diffusion in the cerebral and cerebellar hemispheres and in the basal ganglia, consistent with infarcts. Magnetic resonance venography revealed a right sigmoid sinus nonocclusive thrombus (Fig. 2) and aspirin was started. Anticoagulation therapy was not started as the venous sinus thrombus was not hemodynamically significant and there was risk of hemorrhagic transformation of the infarcts. The rash the patient had described was now apparent and consistent with livedo reticularis. Magnetic resonance imaging of the brain. Axial fluid-attenuated inversion recovery images show bilateral fronto-parietal hyperintensities (left panel), bilateral basal ganglia hyperintensities (middle panel) and bilateral occipital hyperintensities (right panel). Magnetic resonance imaging of the brain. Diffusion-weighted image demonstrates restricted diffusion in bilateral basal ganglia (left panel). Apparent diffusion coefficient (ADC) maps show corresponding decreased ADC value (not shown), and T1 image post-gadolinium shows enhancement in the basal ganglia bilaterally (middle panel), consistent with infarcts. Magnetic resonance venography demonstrates a non-occlusive thrombus in the right sigmoid sinus (right panel). Anti-nuclear antibody (ANA) titre was positive at > 1 : 5120 and C4 was decreased at 0.09 g/L. A catheter cerebral angiogram did not show vasculitis. A renal biopsy was done to investigate the proteinuria and revealed necrotizing vasculitis and focal proliferative glomerulonephritis. Five days after admission, the patient developed new weakness and numbness in the right leg, and the right ankle jerk was now absent. Nerve conduction studies (NCS) confirmed severe axonal loss within the distribution of the left median and ulnar nerves. The right leg NCS was normal, consistent with the very early time course. A repeat MRI of the brain showed new cerebellar infarcts, despite good BP management, aspirin and a course of pulse steroids. The patient was treated with heparin due to concern for antiphospholipid antibody syndrome, as well as cyclophosphamide IV and restarted on pulse methylprednisolone for suspected systemic lupus erythematosus (SLE). Later, the anti-double stranded DNA antibodies, anticardiolipin antibodies, lupus anticoagulant and anti-β2-glycoprotein type 1 antibodies came back positive. The clinical presentation in the context of these autoantibodies confirmed the diagnoses of SLE and antiphospholipid syndrome (APS). The patient’s neurological status remained stable, and she was discharged home with the plan to continue cyclophosphamide, ASA and anticoagulation for APS, along with antihypertensive therapy, tapered prednisone, and commencement of maintenance therapy with azathioprine. A follow-up MRI showed resolution of the PRES changes and venous sinus thrombosis and expected radiologic evolution of the infarcts. Repeat NCS demonstrated electrophysiological evolution with a right tibial neuropathy and ongoing severe changes in the left median and ulnar distributions consistent with the clinical diagnosis of mononeuritis multiplex. Her renal function remained normal and the proteinuria resolved. Neuropsychiatric systemic lupus erythematosus (NPSLE) refers to neurologic syndromes that can involve the central, peripheral and autonomic nervous systems in patients with SLE. The American College of Rheumatology (ACR)1 has described and provided case definitions for 19 NPSLE syndromes. It is rare for SLE to present initially with neurologic manifestations, especially with multiple neurologic diagnoses. Although not defined as an NPSLE syndrome, there have been reported cases in recent years of PRES in SLE patients. PRES can present with headache, visual disturbances, seizures, and altered levels of consciousness. It is most commonly associated with hypertension, but other associations include preeclampsia/eclampsia, renal disease, and immunosuppressive medications.2 The mechanism of PRES remains unclear. An older hypothesis suggests that hypertension leads to autoregulatory vasoconstriction and hypoperfusion leading to ischemia and cerebral edema. A newer hypothesis is that severe hypertension exceeds the autoregulatory limits and leads to hyperperfusion and vasogenic edema. However, PRES can occur in patients without hypertension. Some form of endothelial injury also appears to play a key role in the pathogenesis of PRES.3,4 Magnetic resonance imaging typically shows increased signal of PRES lesions on T2 and FLAIR imaging, and the lesions most commonly involve the parietal and occipital lobes, symmetrically. There is usually no restricted diffusion on DWI and ADC sequences, indicating the presence of vasogenic rather than cytotoxic edema. Management of PRES involves treatment of BP and seizures, removing any offending drugs, and possible delivery in pregnant patients. PRES usually completely reverses when treatment is started early, but there can be long-term clinical and radiological sequelae.2,4 There have been fewer than 100 reported cases of PRES in patients with SLE.5,6 A review article described several contributing factors to the development of PRES in these patients: hypertension (95%), renal involvement (91%), recent onset of immunosuppressive drugs (54%) and/or recent treatment with high intravenous dose of steroids (43%).5 SLE itself, may possibly be a risk factor for PRES in the absence of any of these confounding factors. Antiphospholipid syndrome is a disorder of coagulation predisposing individuals to arterial and venous thrombosis and pregnancy-related complications, such as miscarriages. Thrombotic events can occur anywhere in the vascular bed and cerebral involvement is common. The basis for diagnosing APS involves a clinical event and the presence of specific antibodies. APS was first described in patients with SLE, and 20–35% of patients will develop antiphospholipid syndrome. The mainstay of treatment for APS is anticoagulation/antiplatelet therapy.7,8 Patients with mononeuritis multiplex classically present with acute or sub-acute onset of painful sensory loss and weakness in the distribution of multiple peripheral nerves. This condition can be due to a ‘primary’ vasculitis (e.g. polyarteritis nodosa) or ‘secondary’ to connective tissue diseases, including SLE, although rarely. Electrodiagnostic studies show axonal loss of sensory and motor fibers, usually in a multifocal distribution. Treatment involves immunosuppresants, including steroids.9,10 All authors were involved in the preparation of this manuscript and in the care of the patient presented in this report. The authors did not receive any grants or other financial support for this study. The authors do not have any conflicts of interest to disclose.

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