Neuromyelitis Optica

Published: 01/11/2021

This is a rare condition which I was lucky enough to meet an individual on my neurological placement who had this condition. It is a condition that is not very well known by both physiotherapists and the medical community. 

Neuromyelitis Optica

•        Also known as NMO spectrum disorder (NMOD) or Devic’s disease

•        Rare inflammatory demyelinating disorder of the central nervous system

•        80% more common in women than men

•        Most often occurs between 40 to 50 years old

(NHS England, 2013; National MS Society, 2019)

Diagnostic criteria

•        Optic neuritis and acute myelitis

And at least two of three supportive criteria:

•        contiguous spinal cord MRI lesion extending over three vertebral segments

•        brain magnetic resonance imaging (MRI) not meeting diagnostic criteria for multiple sclerosis

•        Aquaporin-4 antibodies seropositive status

(NHS England, 2013)

Pathophysiology

•        Autoimmune disorder where white blood cells and antibodies primarily attack the optic nerves and spinal cord

•        Damage to optic nerves cause pain and loss of vision

•        Damage to the spinal cord causes weakness or paralysis in legs/arms, loss of sensation, bladder and bowel problems

•        Acute episodes are usually severe, leading to accumulating disability

•        50% of patients require a wheelchair or are blind at five years from the onset if not managed appropriately

•        May be misdiagnosed as MS (1% of patients in an MS study were found to have NMO)

•        Reports of MS disease-modifying therapies causing NMO deterioration

•        Antibody-mediated disorder, early immunosuppression reduces relapses and hospitalisation

•        Aggressive treatment can reduce relapse rate to under 20% and enable long-term stabilisation

(NHS England, 2013; National MS Society, 2019)

Medical management

•        Incurable disease

•        Treatment aims

•        Immunosuppression to prevent relapses

•        If relapses, aim to reduce symptoms and restore neurological function

•        Relapses treated with high-dose intravenous methylprednisolone or plasma exchange

•        Long term immunosuppression medication (IV rituximab or oral azathioprine and/or prednisolone)

(Jarius et al, 2014)

Evidence

•        Only two case study reports and one pilot randomised control trial for rehabilitation of neuromyelitis optica

•        Won Bin et al (2019) and Schreiber et al (2008) case studies found that rehabilitation improved NMO patients’ functional outcomes.

Suo et al’s (2019) pilot RCT

•        Assessed 39 NMO patients’ disability and functional ability at baseline, 4 weeks and 3 months.

•        Intervention group received 4 weeks of MDT inpatient rehabilitation (2 to 4 hours a day, 5 days a week) whereas the control group received usual care

•       The intervention group had a significant reduction in disability scores, improvements in walking ability and voluntary muscular control compared to the control group at 4 weeks and 3 months.

References

•        Jarius, S., Wildermann, B. and Paul, F. (2014) ‘Neuromyelitis optica: clinical features, immunopathogenesis and treatment’, Clinical & Experimental Immunology, 176(2), pp.149-164. [Online] Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3992027/ (Accessed: 24 July 2021).

•        National Multiple Sclerosis Society (2019) Neuromyelitis Optica (NMO). Available at: https://www.nationalmssociety.org/What-is-MS/Related-Conditions/Neuromyelitis-Optica-(NMO) (Accessed: 19 July 2021).

•        NHS England (2013) 2013/2014 NHS Standard Contract for Neuromyelitis Optica Service (Adults and Adolescents). [pdf] Available at: https://www.england.nhs.uk/wp-content/uploads/2013/06/d04-neuromyelitis-optica-serv.pdf (Accessed: 21 July 2021).

•        Schreiber, A.L., Fried, G.W., Formal, C.S. and DeSouza, B.X. (2008) ‘Rehabilitation of Neuromyelitis Optica (Devic’s Syndrome): 3 Case Reports’, Department of Rehabilitation Medicine Faculty Papers. [Online] Available at: https://jdc.jefferson.edu/cgi/viewcontent.cgi?article=1002&context=rmfp (Accessed: 31 July 2021).

•        Suo, D-M., Liu, L-L. Jia, K., Zhang, L-J., Li, L-M., Wang, J., Qi, Y., Liu, H-J., Wan, C-X. and Yang, L. (2019) ‘Multidisciplinary Rehabilitation for Adults with Neuromyelitis Optica Spectrum Disorders: A Pilot Study’, Journal of Rehabilitation Medicine, 51, pp.692-697.

•        Won Bin, K., So Young, L., Bo Ryun, K. and Youn Ji, K. (2019) ‘Rehabilitation of neuromyelitis optica Two CARE-compliant case reports’, Medicine, 98(41). [Online] Available at: https://journals.lww.com/md-journal/fulltext/2019/10110/rehabilitation_of_neuromyelitis_optica__two.30.aspx (Accessed: 31 July 2021).