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Competing interests
The authors, the Associate Publisher R Ashton and the
CME questions author D Lie declared no competing
interests.
THE CASE
A 38-year-old AfricanAmerican woman, with a
2year history of systemic lupus erythematosus
(SLE), was referred for evaluation of recurrent
episodes of myelitis and optic neuritis (ON).
The patients course of SLE was previously
characterized by antinuclear antibody posi-
J Birnbaum is a Post-doctoral Fellow in the Division of Rheumatology and tivity, anti-dsDNA antibody positivity, poly
DKerr is an Associate Professor in the Department of Neurology at The Johns arthritis, and malar rash with photosensitivity;
Hopkins University of Medicine, Baltimore, MD, USA. her symptoms were controlled with hydroxy
chloroquine treatment. The patient experi
Correspondence
*Division of Rheumatology, The Johns Hopkins University of Medicine, Mason F Lord Building,
enced subacute onset of right ear otalgia and a
4100 Center, 5200 Eastern Avenue, Baltimore, MD 21224, USA throbbing, temporal headache. She concom-
jbirnba2@jhmi.edu itantly developed bilateral sensorineural
hearing loss. At the time of these symptoms
Received 7 December 2007 Accepted 25 March 2008 Published online 20 May 2008
www.nature.com/clinicalpractice
(designated as month0), MRI of the brain
doi:10.1038/ncprheum0818 revealed a cystic lesion in the left frontal lobe,
Table 1 Laboratory results from tests performed 20months after initial hearing loss and headache.
Variable (units) Value (normal range)
Hemoglobin (g/dl) 12.8 (12.015.0)
Hematocrit (%) 37.2 (36.046.0)
White-cell count (cells/l) 8.6 (4,50011,000)
Neutrophils (%) 77.7 (40.070.0)
Lymphocytes (%) 17.5 (24.044.0)
Platelet count (cells/l) 286,000 (150,000350,000)
Mean corpuscular volume (fl) 86.9 (80.0100.0)
Prothrombin time (s) 1.0 (0.91.1)
Partial thromboplastin time (s) 24.9 (23.534.0)
Glucose (mg/dl) 80 (6099)
Sodium (mEq/l) 138 (135148)
Potassium (mEq/l) 3.8 (3.55.1)
Chloride (mEq/l) 106 (96109)
Bicarbonate (mEq/l) 26 (2131)
Urea nitrogen (mg/dl) 7 (722)
Creatinine (mg/dl) 0.5 (0.51.2)
Albumin (g/dl) 3.8 (3.55.3)
Calcium (mg/dl) 9.2 (8.410.5)
Alkaline phosphatase (U/l) 69 (30120)
Aspartate aminotransferase (U/l) 23 (031)
Alanine aminotransferase (U/l) 30 (031)
Urine
Specific gravity (g/ml) 1.005 (1.0031.030)
Protein Negative (negative)
Red blood cells (per high power field) 1 (05)
White blood cells (per high power field) 0 (05)
Serology and autoantibodies associated with SLE
ANA titer, pattern 1:1,280, speckled (<1:40)
Anti-dsDNA (U/ml) 168 (079)
Anticardiolipin IgM (MPL units) 9 (<10)
Anticardiolipin IgG (GPL units) 8 (<10)
Russells viper venom time (s) 34.7 (27.045.0)
C3 (mg/dl) 101 (79152)
C4 (mg/dl) 21 (1242)
Perinuclear ANCA Negative (negative)
Cytoplasmic ANCA Negative (negative)
Abbreviations: ANA, antinuclear antibody; ANCA, antineutrophil cytoplasmic antibody; dsDNA, double-stranded DNA;
SLE, systemic lupus erythematosus.
these featuresthe severity and intractability the absence of oligoclonal bands in cerebro-
of ON to treatment, the nonspecificity of spinal fluidsuggests an alternative diagnosis
white-matter lesions in patients with SLE, and to that of MS.
384 nature clinical practice RHEUMATOLOGY BIRNBAUM AND KERR july 2008 vol 4 no 7
CA S E S TU DY
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july 2008 vol 4 no 7 BIRNBAUM AND KERR nature clinical practice RHEUMATOLOGY 385
CA S E S TU DY
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Acknowledgments serological survey that included patients with prognostic features of functional blindness
Dsire Lie, University SLE, however, Pittock et al. did not detect and wheelchair-dependence that occur in
of California, Irvine, CA,
is the author of and is NMOIgG antibodies in any patient with SLE untreated NMO patients. In patients with SLE
solely responsible for the who did not have an opticospinal syndrome and ON or myelitis, identification of clinical
content of the learning
objectives, questions and
indicative of NMO.15 This suggests that NMO- or prognostic features consistent with NMO
answers of the Medscape- IgG positivity in patients with SLE is not should lead to testing for the presence of the
accredited continuing
medical education activity
due to nonspecific B-cell stimulation and is NMO-IgG autoantibody.
associated with this article. highly specific for NMO, even in patients with
coexisting SLE. In contrast to NMO-IgG anti- References
Competing interests bodies, antinuclear antibodies are nonspecific: 1 Dyck PJ et al. (2005) History of standard scoring,
The authors declared no notation, and summation of neuromuscular signs.
competing interests. these occur in up to 27% of patients with MS16 A current survey and recommendation. J Peripher Nerv
and in nearly 50% of patients with NMO.15 Syst 10: 158173
2 Frohman EM (2003) Multiple sclerosis. Med Clin North
The aquaporin-4 antigen is normally seques- Am 87: 867897
tered behind the bloodbrain barrier, including 3 Balcer LJ (2006) Clinical practice. Optic neuritis. N Engl
on abluminal surfaces of pial microvessels.17 In J Med 354: 12731280
4 Beck RW et al. (2003) High- and low-risk profiles for
this patient, previous episodes of inflammation the development of multiple sclerosis within 10years
resulting from active NPSLE (as evidenced by after optic neuritis: experience of the optic neuritis
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5 Charil A et al. (2006) MRI and the diagnosis of multiple
tiated NMO disease by permitting entry of the sclerosis: expanding the concept of no better
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first presentation to predict conversion to clinically
definite multiple sclerosis. Brain 120: 20592069
TREATMENT AND MANAGEMENT 7 Link H and Huang YM (2006) Oligoclonal bands in
NMO is relentlessly polyphasic in more than multiple sclerosis cerebrospinal fluid: an update on
methodology and clinical usefulness. J Neuroimmunol
85% of cases.13 Identification of the NMO- 180: 1728
IgG antibody represents a narrow therapeutic 8 Salvarani C et al. (2007) Primary central nervous
window to employ appropriate immuno system vasculitis: analysis of 101 patients. Ann Neurol
62: 442451
suppressant treatment. Without such treatment, 9 Johnson RT and Richardson EP (1968) The
more than 50% of patients with NMO will be neurological manifestations of systemic lupus
erythematosus. Medicine (Baltimore) 47: 337369
functionally blind, or will progress to wheelchair- 10 Birnbaum J and Kerr D (2007) Devics syndrome in a
dependence, within 5years.17 Plasmapheresis is woman with systemic lupus erythematosus:
recommended for patients with severe optico- diagnostic and therapeutic implications of testing for
the neuromyelitis optica IgG autoantibody. Arthritis
spinal complications arising from NMO,18 and Rheum 57: 347351
might have helped avert functional blindness 11 Mathews MK (2005) Nonarteritic anterior ischemic
in this patient. Spinal cords and brains from optic neuropathy. Curr Opin Ophthalmol 16: 341345
12 Wingerchuk DM et al. (2006) Revised diagnostic
patients with NMO demonstrate vasculocentric criteria for neuromyelitis optica. Neurology 66:
IgG and C9 neoantigen deposition, suggestive 14851489
of a humorally mediated microangiopathy.19 13 Wingerchuk DM et al. (1999) The clinical course of
neuromyelitis optica (Devics syndrome). Neurology 53:
The B-cell arm of the immune system, there- 11071114
fore, constitutes an important therapeutic 14 Lennon VA et al. (2005) IgG marker of optic-spinal
target. An open-label study of eight patients multiple sclerosis binds to the aquaporin-4 water
channel. J Exp Med 202: 473477
with severe and relapsing NMO treated with 15 Pittock SJ et al. (2008) Neuromyelitis optica and non
rituximab demonstrated improvements in the organ-specific autoimmunity. Arch Neurol 65: 7883
16 Ferreira S et al. (2005) Multiple sclerosis,
frequency and severity of relapses.20 Recognition neuropsychiatric lupus and antiphospholipid
of NMO as a unique diagnostic entity might syndrome: where do we stand? Rheumatology
prompt earlier use of B-cell-depleting agents, (Oxford) 44: 434442
17 Wingerchuk DM et al. (2007) The spectrum of
especially during the therapeutic window of neuromyelitis optica. Lancet Neurol 6: 805815
earlier attacks. 18 Wingerchuk DM and Weinshenker BG (2005)
Neuromyelitis optica. Curr Treat Options Neurol 7:
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CONCLUSION 19 Lucchinetti CF et al. (2002) A role for humoral
In summary, we present a patient with SLE and mechanisms in the pathogenesis of Devics
a coincidental but distinct syndrome of NMO. neuromyelitis optica. Brain 125: 14501461
20 Cree BA et al. (2005) An open label study of the effects
Early use of plasmapheresis and B-cell-depleting of rituximab in neuromyelitis optica. Neurology 64:
agents can potentially mitigate the ominous 12701272
386 nature clinical practice RHEUMATOLOGY BIRNBAUM AND KERR july 2008 vol 4 no 7