A neuroimaging follow up study of a patient with juvenile central nervous system systemic lupus erythematosus.
BACKGROUND
The course of central nervous system systemic lupus erythematosus (CNS-SLE) is largely unknown. New imaging techniques are available to assist in monitoring the disease course.


OBJECTIVE
To report a case of juvenile CNS-SLE, in which magnetic resonance imaging (MRI) was used to assess disease activity.


METHODS
A 10-year-old female patient with SLE presented with convulsions; MRI and computed tomography (CT) of the cerebrum disclosed abnormalities. Despite adequate treatment, two years later she had a generalised convulsion, and MRI showed new lesions. MR spectroscopy (MRS) indicated neuronal loss, inflammation, and metabolically compromised tissue; magnetisation transfer imaging (MTI) showed an increase in whole brain lesion load. After exclusion of a malignancy, CNS-SLE was the most likely diagnosis, and cyclophosphamide pulses were administered. Initially, multiple sclerosis (MS)-like lesions regressed, but despite maximal immunosuppressive drugs, new lesions formed and disappeared. When immunosuppressive drugs had been stopped for six months MRI showed improved lesions and MTI histograms.


CONCLUSIONS
In this case report, the anatomical substrate, metabolic aspect, neuroimaging, and clinical course of MS-like lesions in a child with CNS-SLE are described. The way in which radiological techniques can support clinical decision making in this young patient with progressive CNS-SLE is illustrated.
Steens
S C A
SC
Department of Radiology, Leiden University Medical Centre, Leiden, The Netherlands. s.c.a.steens@lumc.nl
Bosma
G P Th
GP
ten Cate
R
R
Doornbos
J
J
Kros
J M
JM
Laan
L A E M
LA
Steup-Beekman
G M
GM
van Buchem
M A
MA
Huizinga
T W J
TW
D002648:Child; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D020945:Lupus Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy