Fall 2017 Newsletter

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Fall 2017 Newsletter
7th Residency Announcement

Exciting Residency Announcement!

ACGME approves seventh resident complement; first increase in over 30 years!

Grand Rounds

Grand Rounds and Case Studies

Check out our weekly presentations

OTEP

OTEP

Ophthalmic Technician Education Program

LARGEST STUDY ON CHINESE AMERICANS PUBLISHED

LARGEST STUDY ON CHINESE
AMERICANS PUBLISHED

USC Ophthalmology Researchers Find More
Effective Treatments For Blinding Eye Diseases

EDUCATION

Case Study: When it Rains, it PORs

Rayess Patel
Presenter: Nadim Rayess, MD Discussant: Vivek Patel, MD
 

History

  • Previously healthy 56-year-old female
  • Presents with acute simultaneous bilateral vision loss of one day duration
  • Associated with bilateral eye pain and pain with eye movements

Exam Findings

  • VA: NLP; 20/400
  • Pupils: Sluggishly reactive OU, +2 RAPD OD
  • IOP: 15; 15
  • EOM: Full OU
Figure 1
Figure 1: Disc photo of the right eye reveals a 0.2 CDR with 360° blurring of the disc margin and peripapillary swelling with obscuration of finer vessels superiorly and inferiorly.

 

Figure 2
Figure 2: Disc photo of the left eye reveals a 0.2 CDR with subtle 360° peripapillary swelling with a temporal disc hemorrhage.

 

Figure 3
Figure 3: MRI brain post-gadolinium contrast with fat suppression demonstrating enhancement of the entire intra-orbital segment of the right and left optic nerves with associated optic nerve sheath thickening. Focal enhancement at the optic nerve head can be seen extending into the vitreous cavity.

 

Differential Diagnosis – Acute Optic Neuropathy

  • Infectious
    • Syphilis
    • Tuberculosis
    • Lyme
  • Inflammatory
    • Demyelinating optic neuritis (multiple sclerosis)
    • Neuromyelitis optica (NMO)
    • Sarcoidosis
    • Systemic Lupus Erythematosis (SLE)
    • Granulomatosis with Polyangiitis
    • Chronic relapsing inflammatory optic neuropathy (CRION)
    • Autoimmune optic neuritis
  • Compressive/Infiltrative
    • Optic nerve sheath meningioma
    • Glioma
    • Metastasis
    • Leukemia/lymphoma
  • Toxic
    • Methanol
  • Ischemic
    • Giant cell arteritis (GCA)
    • Non-Arteritic anterior ischemic optic neuropathy

Additional Investigations

  • Lab work and a lumbar puncture were performed for various infectious and inflammatory causes of atypical optic neuritis
  • MRI of the cervical and thoracic spine to detect any myelitis given the high suspicion for NMO

Lab Testing Results

  • Infectious
    • RPR and FTA-ABS-ve
    • QuantGold-ve
    • B. henselae-ve
  • Inflammatory
    • Antinuclear antibody (ANA)-ve
    • c-ANCA, pANCA-ve
    • SS-A, SS-B Ab-ve
    • ACE-ve
    • Anti-aquaporin 4-ve
  • Lumbar puncture
    • Glucose, 106mg/ml (mildly elevated)
    • Protein, 22mg/ml (normal)
    • Two nucleated cells (equivocal)
    • No growth on culture
  • MRI C-spine w/wo contrast
    • No intrinsic cord signal abnormality or abnormal post contrast enhancement
  • MRI T-spine w/wo contrast
    • No evidence of abnormal signal

Diagnosis

  • Neuromyelitis Optica (NMO) spectrum disorder

Pathophysiology

  1. NMO (Devic’s disease) is an autoimmune disease in which auto-antibodies attack myelin. Specifically IgG antibodies against aquaporin 4 protein channels present in the cell membrane of astrocytes has been implicated in the pathophysiology of NMO.
  2. Aquaporin 4 channels are found on astrocytes in the blood brain barrier. Therefore, auto-antibodies in NMO are believed to lead to a breakdown in the blood brain barrier.
  3. A second subset of patients is NMO IgG negative for aquaporin 4. Some of these patients may test positive for another antibody, anti-MOG, which is thought to primarily attack myelin followed by degeneration of axons and astrocytes.

Treatment

  • The patient was treated with five days of IV 1G Solu-Medrol
  • Given the suspicion of an aggressive inflammatory optic neuropathy as the patient presented with acute NLP vision, plasmapheresis was started at the same time as the IV Solu-Medrol. The patient received five doses of plasmapheresis (2on-1off-2on-1off-1on schedule).
  • She was then transitioned to 60mg PO prednisone for two weeks
  • Prednisone was tapered to 40mg PO prednisone for the subsequent two weeks
Figure 4
Figure 4: Optic disc photo of the right eye at one month follow-up visit demonstrating an increase cup to disc ratio from 0.2 at initial presentation to 0.4 CDR with increasing pallor of the nerve. The margins of the disc are sharp with associated parapapillary atrophy.

 

Figure 5
Figure 5: Optic disc photo of the left eye at one month follow-up visit demonstrating an increased cup to disc ratio from 0.2 to 0.4 CDR with temporal pallor of the nerve. The margins of the disc are sharp with associated peripapillary atrophy and a persistent small temporal optic disc hemorrhage.

 

Prognosis and Future Directions

  • The prognosis for typical demyelinating optic neuritis is very good, with more than 90 percent of patients having improved visual acuity in the first two weeks. Patients presenting typically have an excellent prognosis (~95 percent have vision at six months better than 20/30).
  • At 15 years, approximately 50 percent of these patients will have developed MS, and 75 percent will have developed MS if they demonstrated at least one typical demyelinating lesion on MRI at presentation.
  • IV steroids may be associated with more rapid improvement in vision, but no change in final visual prognosis or long-term protection in the development of MS.
  • If a patient presents with the following typical features, further workup beyond MRI brain and orbits is not recommended (i.e. Lumbar puncture, extensive serological testing)
    • Ages 18 to 50 years old
    • Pain with eye movements
    • No to minimal intraocular inflammation
    • Unilateral
    • No hemorrhages or exudates in posterior pole
    • Demonstrate improvement in vision several weeks after onset
  • Based on Optic Neuritis Treatment Trial findings, an optic neuritis is considered “atypical” for the classical MS-associated form when any of the above features are absent, precipitating further workup.
  • Neuromyelitis Optica is now defined as:
    • Major criteria (need both):
      • Unilateral or bilateral optic neuritis
      • Transverse Myelitis (TM)
  • Supportive criteria (need two of three):
    • Longitudinally extensive (>= 3 vertebral segments) TM
    • MRI brain not consistent with MS pattern
    • Aquaporin-4 antibody positive
  • For NMO-associated optic neuritis, the prognosis is less favorable than typical demyelinating optic neuritis. Approximately 85 percent of patients have relapses of either optic neuritis or transverse myelitis.
  • In patients classified as NMO spectrum disease (i.e. either optic neuritis or transverse myelitis but do not meet the full diagnostic criteria for NMO), prognosis has been shown to vary depending on whether patients are anti-AQP4-Ab positive or anti-MOG-Ab (myelin-oligodendrocyte glycoprotein antibody) positive. Patients who are anti-AQP4 are more likely to have optic neuritis that progresses to significant optic atrophy with worse visual prognosis compared to anti-MOG-Ab. Patients who are anti-MOG-Ab are more likely to present with bilateral optic neuritis, respond better to treatment with steroids and are less likely to have extensive optic atrophy.
  • While azathioprine and rituximab are commonly used immunosuppressive therapies in patients with NMO, there are no randomized controlled trials comparing the effectiveness of various immunosuppressive therapies. Future randomized controlled trials may help guide immunosuppressive treatment in patients with NMO or NMO spectrum disease.

References

  • Palace J, Leite MI, Nairne A and Vincent A. (2010) Interferon Beta treatment in neuromyelitis optica: increase in relapses and aquaporin 4 antibody titers. Arch Neurol. 2010 Aug;67(8):1016-1017.
  • Paty DW, Oger JJ, Kastrukoff LF, et al. MRI in the diagnosis of MS: a prospective study with comparison of clinical evaluation, evoked potentials, oligoclonal banding, and CT. Neurology. 1988 Feb;38(2):180-5.
  • Akaishi T, Nakashima I, Sato DK, Takahashi T, Fujihara K. Neuromyelitis Optica Spectrum Disorders. Neuroimaging Clin N Am. 2017 May;27(2):251-265.
  • Voss E, Raab P, Trebst C, Stangel M. Clinical approach to optic neuritis: pitfalls, red flags and differential diagnosis. Ther Adv Neurol Disord. 2011;4(2):123-34.
  • Ikeda K, Kiyota N, Kuroda H, et al. Severe demyelination but no astrocytopathy in clinically definite neuromyelitis optica with anti-myelin-oligodendrocyte glycoprotein antibody. Mult Scler. 2015;21(5):656-9.
  • Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti, CF, Weinshenker BG. (2006) Revised diagnostic criteria for neuromyelitis optica. Neurology 2006 May23;66(10):1485-1489.
  • Sellner J, Boggild M, Clanet M, Hintzen RQ, Illes Z, Montalban X, et al. EFNS guidelines on diagnosis and management of neuromyelitis optica. Eur J Neurol. 2010 Aug;17(8):1019-32.

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Produced by: Monica Chavez, John Daniel, Joseph Yim and Dr. Vivek Patel
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