A 58‐year‐old woman with a history of Sjogren's syndrome, neuromyelitis optica (NMO; Devic's disease) diagnosed 12 years ago, transverse myelitis, and sleep apnea presented with worsening left leg weakness for 4 months. The patient was diagnosed with NMO by NMO IgG antibodies and typical signal changes on MRI and had been on immunosuppressive treatment for exacerbation. Her present exacerbation, however, was unresponsive to mycophenolate mofetil (MMF) and high‐dose intravenous steroids. On presentation, her exam was significant for 3/5 left hip flexion, 2/5 knee flexion, and 2/5 ankle dorsiflexion with foot drop. Before this exacerbation, she was able to walk without any assistive devices. The patient was started on plasma exchange, and improvement was seen after 3 plasma exchanges. She received a total of 5 plasma exchange therapies over 7 days. She was discharged home with outpatient physical therapy and with ankle–foot orthosis (AFO) support. On the day of her discharge, she could ambulate unassisted for 400 feet and had 5/5 knee flexion and 4/5 ankle dorsiflexion. Now after 1 month, she reports that her foot drop has resolved, and she can ambulate without a walker.
NMO is an uncommon demyelinating disorder of the central nervous system (CNS) primarily affecting the optic nerve and spinal cord. In earlier years, it was thought to be a variant of multiple sclerosis but now is considered to be a separate entity. Recently, it has been discovered that it is associated with the NMO IgG antibody that targets water channel aquaporin‐4, which is the most abundant water channel in the CNS. This discovery led to the revision of diagnostic criteria for NMO. The treatment of NMO is based on case series, case reports and personal clinical experiences, as there are no randomized control trials comparing treatments. First‐line treatment of an exacerbation is high‐dose intravenous corti‐costeroids. In unresponsive and resistant cases, plasma exchange has been tried. On average, there is moderate to marked improvement in 50%–60% of cases. Removal of humoral immune factors by plasma exchange is the proposed mechanism by which it helps, especially in NMO‐positive patients (refractory to standard immunosuppressant therapy).
Our patient showed marked improvement in clinical status after plasma exchange. This supports the use of plasma exchange in treating high‐dose intravenous steroid‐resistant and immunosuppressive agent–resistant NMO exacerbations. At the same time, we realize the need for a randomized controlled trial or case–control study to further evaluate the role of plasma exchange for this disease process.
K. Malhotra ‐ none; S. Sader ‐ none
To cite this abstract:Malhotra K, Sader S. A Case of Steroid and Mycophenolate Mofetil‐Resistant Neuromyelitis Optica Exacerbation Treated with Plasma Exchange. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 331. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/a-case-of-steroid-and-mycophenolate-mofetilresistant-neuromyelitis-optica-exacerbation-treated-with-plasma-exchange/. Accessed April 10, 2020.