Medicine

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Clinical Case Report

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Cortical blindness and not optic neuritis as
a cause of vision loss in a Sjögren’s syndrome
(SS) patient with the neuromyelitis optica
spectrum disorder (NMOSD)
Challenges of ascribing demyelinating syndromes to SS:
a case report
Aliya Lalji, MDa, Izlem Izbudak, MDb, Julius Birnbaum, MD, MHSc,

∗

Abstract
Rationale: The conception that multiple sclerosis may be challenging to distinguish from demyelinating manifestations of Sjögren’s

Syndrome (SS) was introduced more than 30 years ago. However, it is now recognized that the neuromyelitis optica spectrum
disorder (NMOSD) may occur more frequently in SS as opposed to multiple sclerosis. Characteristic NMOSD features can include
severe attacks of optic neuritis, myelitis which is frequently longitudinally-extensive (spanning at least three vertebral segments on
magnetic resonance imaging [MRI]), and an association with anti-aquaporin-4 antibodies. In addition, whereas NMOSD was initially
thought to spare the brain, it is now recognized that brain lesions occur in a majority of NMOSD patients. Therefore, it is important for
the multi-disciplinary team of physicians who care for SS patients to understand this widening spectrum of NMOSD as
encompassing brain lesions. In this case-report we describe clinical features, radiographic findings, and treatment of a SS NMOSD
patient presenting with severely decreased visual acuity, visual hallucinations, and encephalopathy.
Patient concerns: The SS NMOSD patient presented with rapid, bilateral onset of severely decreased visual acuity and was

therefore suspected as having bilateral optic neuritis.
Diagnosis: However, the patient lacked stigmata of optic neuritis, instead had visual hallucinations and encephalopathy suggestive
of cortical blindness, and was noted to have occipital lobe lesions on brain MRI. Other radiographic findings included simultaneous
enhancement of brainstem and periventricular lesions.
Interventions: The patient was initially treated with methylprednisolone with no change in her neurological deficits. She was then

treated with plasma exchange therapy.
Outcomes: The patient had resolution of decreased visual acuity, visual hallucinations, encephalopathy, and contrast-enhancing
brain lesions in response to plasma exchange therapy.
Lesson: We provide the first example of severely decreased visual acuity in a NMOSD patient due to cortical blindness and not
bilateral optic neuritis. This finding expands the spectrum of central nervous system syndromes and brain lesions which may occur in
NMOSD. The synchronous enhancement of a brainstem lesion (known to occur in NMOSD) with occipital lobe lesions also suggests
that our patient’s occipital lobe findings were due to NMOSD. All of our patient’s findings had an excellent clinical and radiographic
response to plasma exchange therapy.
Abbreviations: ACR/EULAR = American College of Rheumatology/European League Against Rheumatism, AQP4 = Aquaporin4, CNS = Central nervous system, ELISA = enzyme-linked immunosorbent assay, LETM = Longitudinally-extensive transverse
myelitis, mg = milligrams MRI = Magnetic resonance imaging, MS = Multiple sclerosis, NMOSD = Neuromyelitis optica spectrum
disorder, PT = prothrombin, PTT = partial thromboplastin time, RVVT = Russell’s viper venom time, SS = Sjögren’s syndrome.
Keywords: central nervous system sjögren’s syndrome, cortical blindness, multiple sclerosis, neuromyelitis optica, neuromyelitis

optica spectrum disorder, optic neuritis, plasma exchange, sjögren’s syndrome

Editor: Francesco Carubbi.
The authors report no conflicts of interest.
a
The Johns Hopkins University School of Medicine, b Division of Neuroradiology, Department of Radiology and Radiological Sciences, c Division of Rheumatology and
Department of Neurology, The Johns Hopkins University School of Medicine, Baltimore, MD.
∗

Correspondence: Julius Birnbaum, Division of Rheumatology/Department of Neurology, The Johns Hopkins University School of Medicine, 5200 Eastern Avenue,
Mason F. Lord Building, Center Tower, Suite 4000, Baltimore, MD 21224 (e-mail: jbirnba2@jhmi.edu).
Copyright © 2017 the Author(s). Published by Wolters Kluwer Health, Inc.
This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is
permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the
journal.
Medicine (2017) 96:34(e7454)
Received: 3 March 2017 / Received in final form: 25 May 2017 / Accepted: 31 May 2017
http://dx.doi.org/10.1097/MD.0000000000007454

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In this report, we describe the first example of a NMOSD
patient surprisingly found to have severely decreased visual
acuity due to cortical blindness and not optic neuritis. In this
patient, other characteristic features of cortical blindness
included visual hallucinations, encephalopathy, and contrastenhancing occipital lobe lesions. All of these clinical and
radiographic findings responded to plasma exchange (PLEX)
therapy. Further discussions considering the management of such
NMOSD presentations in SS patients are considered. The patient
provided informed written consent to be included in this case
report.

1. Introduction
The demyelinating syndromes associated with Sjögren’s
syndrome (SS) have been proposed to recapitulate findings
seen in multiple sclerosis (MS).[1] Cohort studies starting in the
1980s asserted that clinical and neuroimaging features of
demyelinating disease in SS could therefore be difficult to
discriminate from MS.[2] This legacy that MS may exist on a
clinical and mechanistic spectrum with SS central nervous
system disease is embedded in current SS activity indices, which
describe demyelinating syndromes as “MS-type” disease.[3]
However, with increasing recognition over the past decade that
demyelinating syndromes are heterogeneous and encompass
disorders other than MS, the demyelinating syndromes in
SS have equivalently been recognized to include diverse
presentations.[4]
In particular, the neuromyelitis optica spectrum disorder
(NMOSD) is a distinct, immune-mediated, central nervous
system (CNS) demyelinating syndrome with strikingly different
clinical, autoantibody, and neuroimaging features from MS.[5]
Recent studies have suggested that NMOSD may occur more
commonly in SS than in MS.[6] NMOSD is associated with an
antibody biomarker targeting aquaporin-4, expressed on CNS
astrocytic foot processes, which is >95% specific for NMOSD
versus MS.[4,5] Other characteristic features of NMOSD can
include myelitis that spans at least 3 vertebral segments on
magnetic resonance imaging (MRI, longitudinally extensive
transverse myelitis [LETM]), and severe attacks of optic neuritis
that may occupy >1/2 distance of optic nerves on MRI.
With this increasing recognition that SS patients may have a
proclivity to be affected by NMOSD as opposed to MS, it is
important to further consider recent studies suggesting a
widening spectrum of NMOSD. For example, earlier iterations
of NMOSD criteria emphasized its restriction primarily to cause
optic neuritis and myelitis.[4] However, with increasing recognition that NMOSD can also more ubiquitously affect brain
regions enriched in aquaporin-4 (AQP4), a more expansive
panoply of clinical features can occur and reflects this diverse
targeting of different CNS anatomic sites. Such clinical findings
include nausea and vomiting (due to area postrema lesions), as
well as narcolepsy and endocrinopathies (due to diencephalic
lesions).[5]
When encephalopathies occur in a SS patient with NMOSD,
such encephalopathies may be attributed to underlying SS disease
activity, or to infections potentiated by immunosuppressive
therapy. However, the possibility that such encephalopathies
may reflect more aggressive NMOSD activity, and warrant
intensification rather than withdrawal of immunomodulatory
therapy, may not be considered.

2. Case presentation
The patient is a 73-year old, right-handed Caucasian female, who
presented with bilateral decreased visual acuity, encephalopathy,
and episodes of visual hallucinations. The patient was initially
referred to us 2 years ago, for evaluation of whether her
demyelinating syndrome was occurring in the context of a
rheumatic disease (see Fig. 1, timeline). In the 6 years before this
initial evaluation, she experienced 3 attacks of recurrent LETM.
Neuroimaging studies collectively demonstrated LETM from C7
to T5, and from T9 to the conus medullaris. With successive
attacks, the patient cumulatively had loss of antigravity strength
in the lower extremities, and was relegated to a wheelchair after
the third attack. However, her outside physicians only treated
each LETM attack with pulse dosages of intravenous methylprednisolone 1000 mg (for 3 to 5 successive days), but without
starting any immunosuppressive therapies. The patient complained of dry eyes and dry mouth, and was therefore referred to
our center for further evaluation. Upon our initial assessment, the
patient stated that in retrospect, she had experienced symptoms
of dry eyes and dry mouth for 5 years, but recently these
symptoms had become more pronounced. Our evaluation
confirmed the diagnosis of SS according to the 2016 ACR/
EULAR classification criteria.[7] This was evidenced by objective
testing revealing decreased tear production by Schirmer’s test
(<5 mm of tear production) (1 point), associated with the
presence of both anti-Ro (SS-A) (3 points), and anti-La (SS-B)
antibodies, which in our patient were qualitatively assessed by
serum enzyme-linked immunosorbent assay (ELISA). According
to these criteria, a score of ≥4 can establish the diagnosis of SS.
With regard to other serologies, antinuclear antibodies were
present at a titer of 1:320 in a speckled pattern, with no
rheumatoid factor, normal C3 (115 mg/dL) and C4 (14 mg/dL)
levels. Assessment for antiphospholipid antibodies revealed no
evidence of anticardiolipin IgM/IgG nor beta-2-glycoprotein
IgM/IgG antibodies, and lupus anticoagulant was excluded on
the basis of normal prothrombin time/partial thromboplastin

Figure 1. Timeline of disease, diagnostic features, and treatment with plasma exchange resulting in resolution of cortical blindness in a Sjögren syndrome patient
with the neuromyelitis optica spectrum disorder.

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to cortical blindness. As described, cortical blindness is defined as
the total or partial loss of vision in a normal-appearing eye (i.e.,
no optic neuritis), caused by damage to the brain’s occipital
cortex.[13,14]
Although most commonly associated with bilateral lesions in
the occipital lobe, unilateral lesions have also been described.[13,14] The patient was treated with 5 consecutive days
of 1000 mg methylprednisolone with no change in her neurological deficits. Therefore, as has been recommended for severe and
steroid-refractory demyelinating diseases,[15,16] the patient was
treated with PLEX. She received 5 courses of PLEX, given on
alternate days. After the 5 courses of PLEX were completed, there
was improvement in her mental status. She was alert and now
oriented to place and time, and did not describe any further visual
hallucinations. There was no immediate change in her visual
acuity. She was discharged on a tapering course of prednisone,
starting at 60 mg per day. The patient stated she wanted to
continue therapy with mycophenolate mofetil.
She was discharged to a subacute rehabilitation center, and
then re-evaluated 1 month later. There were again no further
visual hallucinations or findings of an encephalopathy. In
addition, the patient was now noted to have completely recovered
her vision, back to her premorbid baseline of 20/60 in each eye,
respectively. Repeat MRI studies are noted in Fig. 3. On axial
FLAIR sequence, there was decreased hyperintensity around the
right ventricular atrium, which no longer extended as far
posteriorly to the juxta-cortical white matter of the right occipital
lobe (Fig. 3A). On T1 post-contrast sequences, there was
resolution of contrast enhancement in the right peri-atrial lesions,
right occipital lobe, and pons (Fig. 3B–D).
When followed over the subsequent year, the prednisone was
tapered to off after 3 months, and there were no further episodes
of demyelinating disease.

time (PT/PTT) as well as no prolongation of Russell viper venom
time (RVTT). The serum protein electrophoresis was suggestive
of hypogammaglobulinemia with decreased gamma-globulin of
0.6 g/dL (normal 0.7–1.7 g/dL). IgG was mildly decreased at 649
mg/dL (normal 751–1560 mg/dL), IgM was mildly decreased at
33 mg/dL (normal 46–304 mg/dL), and IgA was 172 mg/dL
(normal 82–453 mg/dL). The patient’s mild decrease in IgG and
IgM levels was not associated with pulmonary, sinonasal, or
gastroenterological infections. In addition, we evaluated for and
detected anti-AQP4 antibodies by a commercially available
immunohistochemical assay.[4,5] Therefore, given recurrent
episodes of LETM associated with anti-AQP4 antibodies, the
patient satisfied criteria for NMOSD.[8]
On our initial neurological evaluation, we noted visual acuity
of 20/60 in each eye, pupils were symmetric at 3 mm and reactive,
there was no relative afferent pupillary defect, and funduscopic
examination was unremarkable. She was noted to have a spastic
tetraparesis, with 5/5 strength assessed on manual research
council scale[9] in the proximal upper extremities, 4/5 strength in
the intrinsic hand muscles, 3/5 strength in hip flexors, and 1/5
strength in foot dorsiflexion. Reflexes were 3+ symmetrically in
upper and lower extremities, with bilateral Babinski responses.
There was decreased pinprick and temperature sensation in the
lower extremity. She could only walk 5 yards with assistance of a
walker and otherwise was relegated to a wheelchair. We elected
to start treatment with mycophenolate mofetil titrated to 2000
mg (higher dosages not tolerated due to dyspepsia), which is
among other immunosuppressive agents (i.e., rituximab and
azathioprine) recommended to prevent relapses.[10–12]
This represented the patient’s state of health until 2 years later,
when her family brought her to our clinic with new symptoms of
decreased visual acuity. Two weeks before this most recent
evaluation, she suffered a urinary tract infection. She was
admitted to an outside hospital, treated with antibiotics, and
discharged. However, after discharge, her family reported
episodes of visual hallucinations. The patient described seeing
family members in the home who were not actually present,
including some of whom were deceased. In addition, the patient
complained of difficulty reading, and then seeing the television
set. On our evaluation, she was only oriented to person, but not
to place or time. She was only able to detect gross hand
movements inches from her eyes but could not count fingers.
However, she surprisingly did not have any findings supportive of
optic neuritis. Her pupils were 3 mm, symmetrically constricting
to light in both eyes, without findings of an afferent pupillary
defect, and funduscopic examination was normal. The remainder
of her neurological examination revealed only unchanged
findings of a spastic tetraparesis. She continued to have visual
hallucinations of doctors and other family members who were
not actually present in her room. She was therefore hospitalized
for further evaluation.
MRI of the brain revealed disseminated pontine, periventricular, and occipital lobe lesions (Fig. 2). On axial FLAIR
sequence (Fig. 2A), there was extensive T2 hyperintensity around
the right ventricular atrium, which extended posteriorly to
involve the juxta-cortical white matter of the right occipital lobe.
There was also T2 hyperintensity affecting the pons (Fig. 2B). On
T1 post-contrast sequences, there was enhancement in the
periventricular regions (Fig. 2C,D), the occipital lobe (Fig. 2E),
and along the anterior aspects of the pons (Fig. 2F). Given
findings of severely decreased visual acuity with encephalopathy
and hallucinations, an occipital lobe lesion, and no evidence of
optic neuritis, the patient’s decreased visual acuity was attributed

3. Discussion
We here describe the first case of severely decreased visual acuity
in a NMOSD patient due to cortical blindness and not due to
bilateral optic neuritis. Our patient lacked clinical findings of
optic neuritis (i.e., with preserved pupillary constriction and no
afferent pupillary defect), and instead presented with visual
hallucinations, encephalopathy, and an occipital lobe lesion.
Furthermore, an important feature is that our patient also
presented with additional brain lesions, which have recently been
recognized to be part of NMOSD criteria and reflecting antiAQP4 antibody autoimmunity.[8] Further diagnostic, mechanistic, and therapeutic implications are considered below.
A challenging task when a SS patient presents with a
demyelinating syndrome is to assess whether clinical syndromes
and brain lesions are potentially consistent with NMOSD, or due
to competing morbidities such as MS or CNS manifestations of SS
disease. Over the 6 years before her initial evaluation at our center,
she had 3 recurrent attacks of LETM. Upon our initial assessment,
we evaluated for and detected the presence of anti-AQP4
antibodies. These collective manifestations would have been
consistent with early conceptions of NMOSD (previously called
NMO), as a syndrome causing optic neuritis and myelitis, but
otherwise largely sparing the brain.[4] Under such earlier criteria,
our patient’s development of brain lesions would have been
problematic and not consistent with NMOSD. Instead, to account
for such brain lesions, a second and separate disorder such as CNS
SS would need to be invoked. This unwieldy combination of
2 disorders would have clouded judgment about therapy.
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Figure 2. Magnetic resonance imaging (MRI) imaging of the brain before treatment with plasma exchange. (A) An axial FLAIR image demonstrates extensive T2
hyperintensity in the periventricular white-matter around the right ventricular atrium (arrows), and less prominently in other periventricular regions (arrowheads). The
hyperintense signal changes extend posteriorly to involve the deep and juxta-cortical white matter of the right occipital lobe (open arrow). (B) Axial FLAIR image
shows T2 hyperintensity affecting the pons (arrow). Axial (C) and coronal (D) T1 post-contrast sequences demonstrate periventricular contrast enhancement most
pronounced surrounding not only the right atrial trigone of the lateral ventricle (arrow) but also in other periventricular regions (arrowheads). (E) Coronal T1 postcontrast enhanced image shows the enhancement in the right occipital lobe (arrow). (F) Axial T1 post-contrast sequences also show enhancement along the
anterior aspect of the pons (open arrow).

patient, given that infection is known to breach the blood–brain
barrier, the antecedent episode of a urinary tract infection likely
potentiated anti-AQP4 antibody autoimmunity. In addition, a
notable finding was that there was joint enhancement of the
occipital lobe lesion with brainstem and periventricular lesions.
Such synchronous enhancement further attests to a unifying
diagnosis of NMOSD, and again illustrates how invoking a
secondary disorder (i.e. MS or SS) is not necessary.
Recognition that NMOSD was coincidental to her SS was
instrumental in the decision to treat our patient with PLEX. For
example, PLEX is used in severe, steroid-unresponsive attacks of
demyelinating disease,[15,16] but is otherwise not used for
extraglandular manifestations of SS. Our patient initially failed
therapy with corticosteroids, but had a substantial response to
PLEX therapy. She was no longer blind, did not have further
visual hallucinations, had resolution of encephalopathy, and
strikingly also had resolution of contrast-enhancing lesions in the
brainstem, periventricular regions, and occipital lobe. Therefore,
knowledge that NMSOD and SS are coincidental can reinforce
the need of using PLEX when SS patients experience severe flares
of demyelinating attacks. These MRI findings also complement
other immunological studies suggesting NMOSD as being
entirely coincidental to SS.[6,19–21] Such studies identified anti-

However, studies have emphasized that brain lesions occur in
the majority of NMOSD patients,[17] and suggest how NMOSD
can accommodate brain lesions seen in our patient’s most recent
flare.[8] As seen in our patient, brainstem lesions are now included
in NMOSD criteria,[8] and periventricular lesions infrequently
occur and do not necessitate the diagnosis of MS. Whereas our
patient’s findings of cortical blindness have not been described as
stemming from an occipital lobe lesion in NMOSD, cortical
lesions associated with seizures and confusion have been
reported.[18] These findings suggest that cortical blindness and
occipital lobe lesions are consistent with this widening spectrum
of NMOSD. In fact, given that anti-AQP4 antibodies are ∼100%
specific for NMOSD versus MS,[5] the diagnosis of MS in our
patient can be excluded. Therefore, the patient’s initial
presentation (recurrent LETM) and current presentation are all
consistent with a singular diagnosis of NMOSD, and does not
necessitate invoking a second autoimmune disease.
Our patient’s brain MRI had other findings suggesting
NMOSD and associated anti-AQP4 antibody autoimmunity as
a unifying syndrome for her CNS disease. Brain regions that
upregulate AQP4 autoantigen may be vulnerable to damage by
anti-AQP4 antibodies, especially in situations when there is
increased permeability of the blood–brain barrier.[5] In our
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Figure 3. Magnetic resonance imaging (MRI) of the brain performed after treatment with plasma exchange. (A) Axial FLAIR sequences demonstrate decreased T2
hyperintensity of the peri-atrial white matter surrounding the right ventricle (arrows), as well as other periventricular regions (arrowheads). There is also decreased T2
hyperintensity of the hemispheric lesion extending from the right peri-atrial region into the occipital lobe (open arrow). (B) Axial T1 post-contrast sequence shows
resolution of contrast enhancement affecting the right peri-atrial lesions (open arrows). (C) Coronal T1 post-contrast sequence shows resolution of contrast
enhancement affecting the right occipital lobe (open arrows). (D) In addition, on axial T1 post-contrast sequence, no contrast enhancement is noted in the pons.

consensus systemic disease activity index for primary Sjogren’s
syndrome. Ann Rheum Dis 2010;6:1103–9.
[4] Wingerchuk DM, Lennon VA, Lucchinetti CF, et al. The spectrum of
neuromyelitis optica. Lancet Neurol 2007;9:805–15.
[5] Zekeridou A, Lennon VA. Aquaporin-4 autoimmunity. Neurol Neuroimmunol Neuroinflamm 2015;4:e110.
[6] Birnbaum J, Atri NM, Baer AN, et al. The relationship between the
neuromyelitis optica spectrum disorder and Sjogren’s syndrome:
central nervous system extraglandular disease or unrelated, cooccurring autoimmunity? Arthritis Care Res (Hoboken) 2017;69:
1069–75.
[7] Shiboski CH, Shiboski SC, Seror R, et al. International Sjogren’s
Syndrome Criteria Working Group. 2016 American College of
Rheumatology/European League against rheumatism classification
criteria for primary Sjogren’s syndrome: a consensus and data-driven
methodology involving three international patient cohorts. Arthritis
Rheumatol 2017;1:35–45.
[8] Wingerchuk DM, Banwell B, Bennett JL, et al. International Panel
for NMO Diagnosis. International consensus diagnostic criteria for
neuromyelitis optica spectrum disorders. Neurology 2015;2:
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[9] Dyck PJ, Boes CJ, Mulder D, et al. History of standard scoring, notation,
and summation of neuromuscular signs. A current survey and
recommendation. J Peripher Nerv Syst 2005;2:158–73.

AQP4 antibodies exclusively in SS patients with NMOSD, not in
any SS non-NMOSD patients, and with such 100% syndrome
specificity indicating that our patient’s NMOSD was not a CNS
manifestation of SS.[6,19–21]
In summary, we have provided the first example of a patient
with NMOSD who had severely decreased visual acuity due to
cortical blindness and not bilateral optic neuritis. Our patient’s
brain MRI findings emphasize how NMOSD is an expansive
syndrome not limited to optic neuritis and myelitis. Therefore, a
SS patient with steroid-refractory NMOSD should be treated
with PLEX, similar to a NMOSD patient without an accompanying autoimmune disease.

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