Systemic lupus erythematosus (SLE) is a multisystemic auto immune disorder, with neurological manifestations being common. Attribution of neurological features to SLE is often a challenging process that requires a thorough clinical evaluation, relevant laboratory investigations and imaging studies. Acute cerebellar ataxia has been described in SLE, although little has been published in this regard. Wernicke's encephalopathy (WE) is a neurological complication of thiamine deficiency. Poor eating habits and vomiting are well-described causes of non-alcoholic WE. Gastrointestinal manifestations commonly occur, with nausea and vomiting reported in >50% of cases in some series. It is important that WE is considered in patients with SLE and ataxia, as it is treatable and carries a significant morbidity and mortality without appropriate treatment.
Case reportA 24-year-old black female patient was referred to the rheumatology clinic at Dr George Mukhari Academic Hospital, a tertiary hospital in Pretoria, South Africa, with suspected SLE. Dermatologists had been treating her for a biopsy-proven bullous lupus for a few months. The initial consultation at the rheumatology clinic confirmed the diagnosis of SLE, based on the presence of acute cutaneous lupus, non-scarring generalised alopecia, haemolytic anaemia, positive antinuclear antibody, positive anti-Smith antibody, a weakly positive lupus anticoagulant test, and a low level of serum complement 3 (C3), unequivocally fulfilling the Systemic Lupus International Collaborating Clinics classification criteria for SLE.[1] Although her bullous lesions had healed, she had an active interarticular rash on her fingers and haemolytic anaemia, with a haemoglobin of 6.4 g/dL, confirming the presence of active disease. She received 2 U of blood and was treated with oral prednisone and oral azathioprine.Five weeks later, she presented again, complaining of intermittent vomiting and nausea of ~3 weeks' duration. She also reported weight loss and vertigo. A physical examination revealed sinus tachycardia, drowsiness, mild confusion, nystagmus, dysarthria, truncal and limb ataxia and bilateral partial 6th cranial nerve palsies. Her haemoglobin was 11.4 g/dL. All other laboratory tests for detecting active SLE in various organ systems were normal, except for hypoalbuminaemia, with a serum albumin of 26 g/L. Her renal function test was normal and there was no proteinuria. Hypoalbuminaemia seemed to reflect the severity of vomiting and possibly the negative acute-phase response. Typical features of SLE-related protein-losing enteropathy, such as diarrhoea, profound pitting oedema and effusions in serous cavities, were absent. Barium studies and gastroscopy did not show any abnormalities to explain the reasons for her vomiting, suggesting that SLE itself was the likely cause. SLE activity was thought to be the cause of sinus tachycardia, as other causes such as thyrotoxicosis, lung diseases, pulmonary embolism and myocarditis were excluded. With regard to her neurological symptoms and sign...
