Devic syndrome and pregnancy: A case series

Background Devic syndrome or neuromyelitis optica is an autoimmune neurological condition characterized by relapsing symptoms of optic neuritis and transverse myelitis. Women with neuromyelitis optica suffer from adverse pregnancy outcomes and high relapse rates during pregnancy and the postpartum period. Methods This case series describes 13 pregnancies in four women with neuromyelitis optica managed at a tertiary hospital in Toronto, Canada. Results In most cases, neurologic symptoms either worsened or developed for the first time during pregnancy or the postpartum period, and often responded to a combination of steroids, immunosuppressant medications, plasma exchange and intravenous immunoglobulin. The 13 pregnancies resulted in two miscarriages, three preterm and eight term births. One fetus whose mother was on gabapentin, prednisone and spironolactone, had congenital malformations (aplastic lung and fused fingers). Conclusions Despite high frequency of relapses in pregnancy and the postpartum period, with multidisciplinary team management, outcomes for women with neuromyelitis optica are encouraging.

Neuromyelitis optica spectrum disorders in patients with myasthenia gravis: ten new aquaporin-4 antibody positive cases and a review of the literature

Background: Neuromyelitis optica (NMO, Devic syndrome) and myasthenia gravis (MG) are rare antibody-mediated autoimmune disorders. Concurrent incidence has been reported in only few patients, mostly non-Caucasians. Objective: To report on ten Caucasian patients with NMO spectrum disorders (NMOSD) and MG and to provide a comprehensive review of the literature. Method: Retrospective study. Results: In total, 26 patients (m:f = 1:12; Caucasian in 12) with MG (generalized in 17) and NMOSD (NMO in 21, longitudinally extensive transverse myelitis in five) were identified from the authors’ own files ( n = 10) and the previous literature ( n = 16). MG preceded NMOSD in 24/25 cases (96%). AQP4-Ab were tested in 20 patients and were positive in 17 (85%). Twenty out of 25 patients (80%) had been treated with thymectomy or thymic irradiation, which preceded NMOSD in all cases (median latency, 12 years; range, 0.3–32). At last follow-up, complete remission of MG was reported in 15/22 (68%), and MG was well controlled with pyridostigmine in three. Co-existing autoimmune disorders or autoimmune antibodies were reported in 17 patients. Conclusion: Our study demonstrates that i) AQP4-Ab-positive NMOSD are more commonly associated with MG in Caucasians than previously thought; ii) MG precedes NMOSD in most cases, often by more than a decade; iii) NMOSD almost exclusively occur in females with juvenile or early-onset MG; and iv) MG frequently takes an unusually mild course in patients with NMOSD. A history of thymectomy could be a possible risk factor for the later development of NMOSD. We recommend testing for AQP4-Ab in MG patients presenting with atypical motor or optic symptoms.