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Poll

Which assay do you use for AQP4-IgG and MOG-IgG testing in your practice/centre?

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CBA
   
ELISA
   
IHC
   
Other
   

Tutorial

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Poll

How confident are you in identifying the presenting symptoms of NMOSD?

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A little confident
   
Moderately confident
   
Very confident
   

Tutorial

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Poll

What is the biggest challenge you face in managing NMOSD?

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Identifying an attack
   
Treating the first attack
   
Selecting a biologic for long-term treatment
   
Monitoring long-term treatment efficacy
   
 
Understanding the clinical features and presenting symptoms of NMOSD
Initial assessment and differential diagnosis of NMOSD
Early management of NMOSD to mitigate symptoms and reduce the risk of further attacks
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Neuro-ophthalmology CE/CME accredited

touchIN CONVERSATION
A relaxed discussion between two faculty focussed on real world clinical issues. Useful tips below will show how to navigate the activity. Join the conversation. Close

Early diagnosis and treatment of NMOSD: Practical insights

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Learning Objectives

After watching this activity, participants should be better able to:

  • Identify the initial symptoms of NMOSD and its clinical characteristics at presentation
  • Apply appropriate processes to ensure accurate and rapid diagnosis of NMOSD
  • Choose appropriate management strategies based on patient symptoms and biomarker tests
Overview

In this activity, a neurologist and a neuro-ophthalmologist, both renowned experts in neuromyelitis optica spectrum disorder (NMOSD), answer questions from the ophthalmology and neurology communities on the management of NMOSD. They discuss presenting symptoms, differential diagnoses, management of acute attacks and long-term treatment.

This activity is jointly provided by USF Health and touchIME. read more

Target Audience

Neurologists and ophthalmologists, including neuro-immunologists and neuro-ophthalmologists, involved in the management of NMOSD.

Disclosures

USF Health adheres to the Standards for Integrity and Independence in Accredited Continuing Education. All individuals in a position to influence content have disclosed to USF Health any financial relationship with an ineligible organization. USF Health has reviewed and mitigated all relevant financial relationships related to the content of the activity. The relevant relationships are listed below. All individuals not listed have no relevant financial relationships.

Faculty

Prof. John Chen Discloses: Consultancy fees from Horizon and UCB.

Prof. Friedemann Paul Discloses: Advisory board or panel fees from Alexion Pharma, Amgen, Biogen, Chugai Pharma, Förderpreis, Hexal, Horizon, Merck, Novartis, Novartis Pharma, Ridgeline Discovery, Roche, Sanofi, Teva GmbH and UCB. Consultancy fees from BioNTech, Bristol Myers Squibb, Hexal AG, Hoffmann-La Roche, MIAC AG, Roche Pharma and UCB Biopharma. Grants/research Support from Alexion, Amgen, Hexal (relationship terminated), Horizon, Novartis, Roche, Sanofi (relationship terminated) and UCB.

Content Reviewer

Danielle Walker, DNP, APRN, AGNP-C has no financial interests/relationships or affiliations in relation to this activity.

Touch Medical Contributor

Adriano Boasso has no financial interests/relationships or affiliations in relation to this activity.

USF Health Office of Continuing Professional Development and touchIME staff have no financial interests/relationships or affiliations in relation to this activity.

Requirements for Successful Completion

In order to receive credit for this activity, participants must review the content and complete the post-test and evaluation form. Statements of credit are awarded upon successful completion of the post-test and evaluation form.

If you have questions regarding credit please contact cpdsupport@usf.edu.

Accreditations

Physicians

This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through a joint providership of USF Health and touchIME. USF Health is accredited by the ACCME to provide continuing medical education for physicians.

USF Health designates this enduring material for a maximum of 0.75 AMA PRA Category 1 CreditTM.  Physicians should claim only the credit commensurate with the extent of their participation in the activity.

The European Union of Medical Specialists (UEMS) – European Accreditation Council for Continuing Medical Education (EACCME) has an agreement of mutual recognition of continuing medical education (CME) credit with the American Medical Association (AMA). European physicians interested in converting AMA PRA Category 1 CreditTM into European CME credit (ECMEC) should contact the UEMS (www.uems.eu).

Advanced Practice Providers

Physician Assistants may claim a maximum of 1.0 Category 1 credits for completing this activity. NCCPA accepts AMA PRA Category 1 CreditTM from organizations accredited by ACCME or a recognized state medical society.

The AANPCP accepts certificates of participation for educational activities approved for AMA PRA Category 1 CreditTM by ACCME-accredited providers. APRNs who participate will receive a certificate of completion commensurate with the extent of their participation.

Date of original release: 25 July 2024. Date credits expire: 25 July 2025.

If you have any questions regarding credit please contact cpdsupport@usf.edu.

This activity is CE/CME accredited

To obtain the CE/CME credit(s) from this activity, please complete this post-activity test.

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  • Downloads including slides are available for this activity in the Toolkit

Topics covered in this activity

Neuro-ophthalmology
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touchIN CONVERSATION
Early diagnosis and treatment of NMOSD: Practical insights
0.75 CE/CME credit

Question 1/5
What are the three most common core clinical features of NMOSD?

NMOSD, neuromyelitis optica spectrum disorder.

NMOSD is an autoimmune-mediated idiopathic inflammatory demyelinating disease with typical clinical presentation of optic neuritis, acute myelitis, and area postrema syndrome.

Abbreviation
NMOSD, neuromyelitis optica spectrum disorder.

Reference
Zhang Y-X, et al. Front Immunol. 2022;13:864664.

Question 2/5
Which of the following statements about NMOSD relapses is correct?

NMOSD, neuromyelitis optica spectrum disorder.

The clinical course of NMOSD historically took one of two forms: monophasic or relapsing, with relapsing forms comprising approximately 80–90% of cases. However, after an index event, the distinction between monophasic and relapsing NMOSD is often difficult to make since relapses can occur many years after an event. In the vast majority of cases, a relapse occurs within 3 years after the index event.

Abbreviation
NMOSD, neuromyelitis optica spectrum disorder.

Reference
Oh J and Levy M. Neurol Res Int. 2012;2012:460825.

Question 3/5
You have gathered details of your patient’s history/clinical presentation and carried out a physical examination which leads you to suspect NMOSD. What would you do to confirm a diagnosis of NMOSD?

AQP4-IgG, aquaporin-4 immunoglobulin G; CSF, cerebrospinal fluid; MOG-IgG, myelin oligodendrocyte glycoprotein-immunoglobulin G; MRI, magnetic resonance imaging; NMOSD, neuromyelitis optica spectrum disorder.

The initial workup in people with suspected NMOSD must include a detailed medical history and an extended physical and neurological examination. Additional mandatory diagnostic tests include cranial and spinal MRI and serum AQP4-IgG antibody testing. The most important antibody-related differential diagnosis of NMOSD is MOGAD, so MOG-IgG should also be tested to rule out this condition. As AQP4-IgG and MOG-IgG are produced mainly extrathecally, serum should be used for AQP4-IgG determination as the biomaterial of choice.

Abbreviations
AQP4-IgG, aquaporin-4 immunoglobulin G; MOGAD, myelin oligodendrocyte glycoprotein antibody-associated disease; MOG-IgG, myelin oligodendrocyte glycoprotein-immunoglobulin G; MRI, magnetic resonance imaging; NMOSD, neuromyelitis optica spectrum disorder.

Reference
Jarius S, et al. J Neurol. 2023;270:3341–68.

Question 4/5
You are seeing a 45-year old female patient who presented at the emergency room with acute severe optic neuritis and transverse myelitis. You send a blood sample for AQP4-IgG and MOG-IgG testing, but the results may take up to 1 week. How do you treat her considering that her symptoms are consistent with a severe NMOSD attack?

AQP4-IgG, aquaporin-4 immunoglobulin G; IV, intravenous; MOG-IgG, myelin oligodendrocyte glycoprotein-immunoglobulin G; NMOSD, neuromyelitis optica spectrum disorder.

The standard of care for acute attacks in both AQP4 IgG-positive and double-negative NMOSD are high-dose glucocorticoids and plasma exchange. For patients with severe NMOSD attacks, simultaneous treatment with glucocorticoids and plasma exchange should be considered. IV methylprednisolone should be usually administered at a dose of 1,000 mg per day for 3–5 days, followed by an oral methylprednisolone taper (starting with either 1 mg/kg/day or 20–30 mg/day and then tapered to 10–15 mg/day within 2–3 weeks) in combination with proton pump inhibition and possible thrombosis prophylaxis.

Abbreviations
AQP4-IgG, aquaporin-4 immunoglobulin G; IV, intravenous; NMOSD, neuromyelitis optica spectrum disorder.

Reference
Kümpfel T, et al. J Neurol. 2024;271:141–76.

Question 5/5
You are now considering the long-term management of your patient with NMOSD after the first attack. What do you do to reduce the risk of future attacks and accumulation of disability?

NMOSD, neuromyelitis optica spectrum disorder.

Currently, there is no known curative treatment for NMOSD; therefore, the main goals of therapy are to counteract acute attacks promptly and effectively and to prevent future attacks by initiating immunotherapy as soon as a definite diagnosis of NMOSD is established.1 Several prospective randomized controlled trials have led to approval of immunotherapies for patients with AQP4-IgG-positive NMOSD. Eculizumab,2,3 inebilizumab,4,5 ravulizumab,6,7 and satralizumab8,9 are all approved for treatment by the FDA and the EMA, and rituximab is approved for treatment in Japan and used off-label in many other countries.1

Abbreviations
AQP4-IgG, aquaporin-4 immunoglobulin G; NMOSD, neuromyelitis optica spectrum disorder.

References

  1. Kümpfel T, et al. J Neurol. 2024;271:141–76.
  2. FDA. Eculizumab PI. 2020. Available at: https://bit.ly/3LaMIO6 (accessed 25 June 2024).
  3. EMA. Eculizumab SmPC. 2023. Available at: https://bit.ly/3ROBJOk (accessed 25 June 2024).
  4. FDA. Inebilizumab PI. 2020. Available at: https://bit.ly/3RRWvwg (accessed 25 June 2024).
  5. EMA. Inebilizumab SmPC. 2024. Available at: https://bit.ly/3RUJemJ (accessed 25 June 2024).
  6. FDA. Ravulizumab PI. 2024. Available at: https://bit.ly/3xECS47 (accessed 25 June 2024).
  7. EMA. Ravulizumab SmPC. 2023. Available at: https://bit.ly/4cH9eKe (accessed 25 June 2024).
  8. FDA. Satralizumab PI. 2022. Available at: https://bit.ly/4cPpY2d (accessed 25 June 2024).
  9. EMA. Satralizumab SmPC. 2023. Available at: https://bit.ly/4cKXF4L (accessed 25 June 2024).
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