Seizure as a Presenting Symptom in Neuropsychiatric Systemic Lupus Erythematosus

Abstract

This magnetic resonance imaging was obtained during the evaluation of a 30-yr-old woman who had experienced gait dysfunction and seizures for the past several years and more recently had reported headaches and dizziness. The patient had been evaluated by several neurologists, and they had come to no definitive diagnosis. The original magnetic resonance imaging of the brain and the electromyography of the lower limb disclosed nothing abnormal. Lumbar puncture was performed under fluoroscopy, and subsequently the patient developed weakness and headache. She was admitted to the hospital for change in mental status and was started on intravenous antibiotics. A muscle biopsy was negative, and then the patient developed generalized tonic-clonic seizures and was admitted to the ICU. The computed tomographic scan of the head showed vasogenic edema. Rheumatology evaluation revealed a positive anti-nuclear antibody 1:160 with speckled pattern, and therefore she was started on pulsed intravenous steroids. The image represents her first inpatient magnetic resonance imaging, which revealed a 55 mm focus of vasogenic edema in the left parietal lobe with mild focal mass effect (Fig. 1). This, along with the positive anti-nuclear antibody, contributed to a diagnosis of neuropsychiatric lupus. She was transferred to the Brain Trauma Unit of inpatient rehabilitation, while still on intravenous steroids, oral hydroxychloroquine, and intravenous levetiracetam. An electroencephalogram showed an epileptogenic focus over the left occipital region. No further seizures were witnessed when she was in the Brain Trauma Unit. Further evaluation revealed a positive anticardiolipin IgG, and therefore aspirin therapy was begun.FIGURE 1: Magnetic resonance imaging axial T2-weighted fluid attenuation inversion recovery (FLAIR) image showing vasogenic edema in the left parietal lobe.When in the Brain Trauma Unit, the patient was noted to have generalized weakness, bilateral hand tremor, mildly decreased impulsivity control, decreased organizational skills, slow speed of processing, and poor short-term memory. Bilateral foot orthoses and a right ankle brace for ambulation were needed. She was found to have a right peripheral vision cut but was able to compensate by scanning. The patient was discharged home with supervision and outpatient cognitive, physical, occupational, and speech therapy. Cognitive dysfunction is one the most common neuropsychiatric complaints in patients with systemic lupus erythematosus (SLE). It is estimated to affect as many as 87% of patients with known SLE.1 The American College of Rheumatology has 19 specific syndromes identified that are associated with neuropsychiatric lupus. One such syndrome includes seizures, which have a prevalence of 8%–18% in the context of neuropsychiatric lupus. It is important to remember that neuropsychiatric lupus can occur in the absence of other systemic manifestations of SLE and that neuropsychiatric lupus is associated with a worse prognosis than if the central nervous system is not involved in SLE.2