Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus

Abstract

Neuropsychiatric forms of systemic lupus erythematosus (SLE) vary, most commonly consisting of seizures, psychiatric disturbances, or focal central nervous deficits. This is a new case of neuromyelitis optica or Devic's syndrome during the course of SLE. Few reports of this association exist in the literature. Our objective is to report this unique case of Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus. A 28-year-old woman had been diagnosed as having SLE with cutaneous and articular involvement in 1987 when she was 17 years old. She was treated with a synthetic antimalarial agent associated with corticosteroids. In 1994, during the fourth month of pregnancy, she had signs of transverse myelitis with a sensory level at T6 associated with an optic neuropathy suggesting a Devic's syndrome. The patient was managed by plasmapheresis sessions and intravenous corticosteroids. Transverse myelitis recurred postpartum and three years later at the same thoracic level. Management by bolus administration of a steroid and cyclophosphamide resulted in remission again. There have only been around a dozen reports in the literature of patients who had both Devic's neuromyelitis optica and SLE. Magnetic resonance imaging is contributive to diagnosis and therapeutic follow-up, showing spinal cord lesions with increased intensity on T2-weighted sequences. Although the clinical course of the present patient has been favourable so far, the prognosis of this neurologic disease is generally considered to be poor with elevated mortality.

Devic’s neuromyelitis optica: a critical review

“…Firstly, there are striking similarities between NMO and a variant of MOG-induced experimental allergic encephalomyelitis (EAE) in Brown Norway rats which develop a marked antibody response associated with pronounced demyelination mainly affecting the optic nerves and the spinal cord 93 . Secondly, NMO patients have a number of circulating autoantibodies and many of them have autoimmune co-morbidities such as Sjögren syndrome, systemic lupus erythematosus and mixed connective tissue disease [94][95][96][97][98][99][100][101][102][103] . Finally, some treatment peculiarities such as improvement of corticosteroid-refractory acute attacks following plasma exchange 104,105 and a better response with general immunossupression than with standard MS immunomodulatory drugs 106,107 strongly support the role of autoantibodies in the disease process.…”

Pregnancy outcomes in aquaporin-4–positive neuromyelitis optica spectrum disorder

“…1 NMOSD is up to 8 times more prevalent in women, 4 many of whom have active disease during childbearing years. [5][6][7] Recently, experimental and clinical reports have demonstrated the presence of AQP4 in human and animal placenta, and have linked AQP4-mediated placental inflammation to fetal death. [8][9][10] It is clear that the annualized relapse rate (ARR) of NMOSD is significantly increased in the 0-to 3-month postpartum period, [11][12][13] but there is a lack of information on the influence of NMOSD on the course of pregnancy.…”

Plasma exchange therapy for a severe relapse of Devic’s disease in a pregnant woman: A case report and concise review

“…Recent case reports and reviews on the treatment of pregnancy-related relapses(13) mostly concern preemptive immunosuppression, steroids for acute attacks during pregnancy, and only scarcely use of other agents. To the best of our knowledge there are three cases reported in the literature where plasma exchange has been used during pregnancy (Bonnet et al, 1999;Cornelio et al, 2009; 1 patient in the retrospective cohort by Nour et al, 2016) (10,14,15); only one of them provided posterior follow-up details on the patient's status. This is the first report of the successful use of plasma exchange therapy in a Hispanic patient from a developing country such as our own, where there are very few comprehensive and specialized centers in the management of these patients and their neurological diseases.…”