Longitudinal brainstem involvement in systemic lupus erythematosus

Abstract

Longitudinal brainstem involvement in systemic lupus erythematosusSir, Central nervous system (CNS) involvement in systemic lupus erythematosus (SLE) is highly diverse. 1 In their extensive literature review, Jennekens and Kater delineated 16 different CNS syndromes that may be induced by SLE. 1 However, brainstem involvement is extremely rare and has not been included in their modified nomenclature system. So far, small distinct brainstem lesions 2-6 as well as vasculitic brainstem involvement 7,8 have been reported. Here we present a patient with an extensive longitudinal brainstem lesion as a first CNS manifestation of SLE.A 48 year-old female patient presented with a 4-day history of gradual onset diplopia and gait disturbance. She had a medical history of SLE without renal or hepatic involvement and was on 20 mg prednisolone. The diagnosis was established 20 years prior to the current admission on the basis of malar rash, arthritis, photosensitivity, haematological disorder and positive antinuclear antibodies. Examination revealed esotropia and a highfrequency gaze-evoked nystagmus on lateral and upward gaze. The gait was severely ataxic. The remainder of the neurologic examination was unremarkable, in particular, the examination of the lower cranial nerves and the sensory system was normal. The patient was afebrile and normotensive (blood pressure 110/75 mmHg). Antiphospholipid antibodies were negative. Lumbar puncture showed 12 WBC/mm 3 (90% lymphocytes) with normal lactate and glucose levels. A complete