Aseptic Meningitis and White Matter Disease in Childhood-Onset Neuropsychiatric Lupus

Abstract

We reported a 10-year-old girl who had an atypical demyelinating disease as the presentation of her neuropsychiatric lupus. The patient had a 4-year history of systemic lupus erythematosus which had been on remission until she presented with fever and headache at the age of 10 years. Physical examination showed meningism. Extensive microbiological workup for infective meningitis was unrevealing. There was a radiographic finding of an extensive white matter hyperintensity on the magnetic resonance imaging (MRI) of the brain. At the initial stage of our case, as it was difficult to differentiate between infection of the central nervous system and neuropsychiatric manifestation of lupus, a course of intravenous immunoglobulin was given empirically instead of high-dose corticosteroid while awaiting the microbiological workup results. The fever and headache subsided shortly after commencement of intravenous immunoglobulin without use of pulse corticosteroid. After the active neurological symptoms remitted, she was given a total of six monthly doses of intravenous immunoglobulin at 2 g/kg/cycle and six biweekly doses of intravenous cyclophosphamide at 500 mg/m²/month. Interval MRI showed resolution of the white matter hyperintensity. Despite the extensive demyelinating disease on initial presentation, she remitted successfully without residual neurological sequelae.

Figure 1

CT brain without contrast enhancement taken on Day 1 (a), Day 4 (b), and Day 10 (c) of admission, respectively. There was an extensive white matter hypodensity in the left temporal lobe on plain CT brain on admission (a) with mild enlargement on Day 4 of admission (b). There was no intracranial hemorrhage on initial presentation. The interval scan on Day 10 of admission (c) showed new hyperdensities with the hypodensity in left temporal lobe, which may be compatible with hemorrhagic component.

Figure 2

T2-weighted MRI brain taken on Day 3 and in week 4 of admission, respectively. On initial presentation (a), an area of hyperintensity was seen in the subcortical and deep white matter of left temporal lobe in T2W images. The overlying cortex in the left temporal lobe is mildly swollen with sulcal spaces effacement. The differential causes of the above radiological findings included encephalitis and demyelinating diseases. The MRI 4 weeks later (b) showed resolving T2W hyperintensity and cerebral edema after start of high dose systemic corticosteroids and intravenous immunoglobulin. However, blooming artefact was present at the posteroinferior aspect, suggestive of subacute hemorrhage product. Microhemorrhage could be a result of cerebral vasculitis. (a) On Day 3 of admission. (b) In week 4 of admission.