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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.

Abstract 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.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 28834867
OWN - NLM
STAT- In-Process
DA  - 20170823
LR  - 20170823
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 34
DP  - 2017 Aug
TI  - Cortical blindness and not optic neuritis as a cause of vision loss in a
      Sjogren's syndrome (SS) patient with the neuromyelitis optica spectrum disorder
      (NMOSD): Challenges of ascribing demyelinating syndromes to SS: a case report.
PG  - e7454
LID - 10.1097/MD.0000000000007454 [doi]
AB  - RATIONALE: The conception that multiple sclerosis may be challenging to
      distinguish from demyelinating manifestations of Sjogren'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.
FAU - Lalji, Aliya
AU  - Lalji A
AD  - aThe Johns Hopkins University School of Medicine bDivision of Neuroradiology,
      Department of Radiology and Radiological Sciences cDivision of Rheumatology and
      Department of Neurology, The Johns Hopkins University School of Medicine,
      Baltimore, MD.
FAU - Izbudak, Izlem
AU  - Izbudak I
FAU - Birnbaum, Julius
AU  - Birnbaum J
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
EDAT- 2017/08/24 06:00
MHDA- 2017/08/24 06:00
CRDT- 2017/08/24 06:00
AID - 10.1097/MD.0000000000007454 [doi]
AID - 00005792-201708250-00004 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Aug;96(34):e7454. doi: 10.1097/MD.0000000000007454.