Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset.

BACKGROUND: Few data are available for patients with a late onset (≥ 50 years) of neuromyelitis optica (LONMO) or neuromyelitis optica spectrum disease (LONMOSD), defined by an optic neuritis/longitudinally extensive transverse myelitis with aquaporin-4 antibodies (AQP4-Ab). OBJECTIVE: To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death. METHODS: We identified 430 patients from four cohorts of NMO/NMOSD in France, Germany, Turkey and UK. We extracted the late onset patients and analyzed them for predictive factors of disability and death, using the Cox proportional model. RESULTS: We followed up on 63 patients with LONMO and 45 with LONMOSD during a mean of 4.6 years. This LONMO/LONMOSD cohort was mainly of Caucasian origin (93%), women (80%), seropositive for AQP4-Ab (85%) and from 50 to 82.5 years of age at onset. No progressive course was noted. At last follow-up, the median Expanded Disability Status Scale (EDSS) scores were 5.5 and 6 in the LONMO and LONMOSD groups, respectively. Outcome was mainly characterized by motor disability and relatively good visual function. At last follow-up, 14 patients had died, including seven (50%) due to acute myelitis and six (43%) because of opportunistic infections. The EDSS 4 score was independently predicted by an older age at onset, as a continuous variable after 50 years of age. Death was predicted by two independent factors: an older age at onset and a high annualized relapse rate. CONCLUSION: LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death.

Influence of pregnancy on neuromyelitis optica spectrum disorder.

OBJECTIVE: To investigate the influence of pregnancy on patients with neuromyelitis optica spectrum disorder (NMOSD). METHODS: A total of 190 women with NMOSD were enrolled from 7 referral hospitals in 4 countries. We reviewed medical records and used a structured questionnaire to investigate gravidity, parity, and the number of relapses during the 2 years before pregnancy, during each trimester of pregnancy, during the first and second trimesters after delivery, and for 6 months thereafter. The annualized relapse rate (ARR) was calculated for each period. RESULTS: Of the 190 women with NMOSD, 40 patients experienced 54 informative pregnancies, and all of them were seropositive for aquaporin-4 antibody. Fourteen patients developed the first symptoms of NMOSD either during the pregnancy (3 patients) or within a year after delivery or abortion (8 and 3 patients, respectively). Twenty-six patients experienced 40 pregnancies after the onset of NMOSD (26 deliveries and 14 abortions [1 spontaneous and 13 elective]). There was one preterm delivery with birth defects and no stillbirths. The ARR during pregnancy did not differ from that before pregnancy, but it increased significantly during the first and second trimesters after delivery (5.3 and 3.7 times, respectively). Moreover, 77% of the deliveries were associated with postpartum relapses. CONCLUSION: The significantly increased relapse rate and numerous cases of NMOSD onset after pregnancy suggest that delivery adversely affects the course of NMOSD. Prospective studies are needed to confirm our findings.

The differential diagnosis of longitudinally extensive transverse myelitis.

Longitudinally extensive transverse myelitis refers to florid and widespread inflammation of the spinal cord causing T2 hyperintensity on spinal magnetic resonance imaging that is seen to extend over three or more vertebral segments. Whilst rare, longitudinally extensive transverse myelitis is clinically important as it can lead to catastrophic morbidity, and a group of these patients are at risk of further attacks. Early identification and establishment of the underlying aetiology is vital in order to initiate appropriate therapy and optimize outcomes. Whilst longitudinally extensive transverse myelitis is classically associated with neuromyelitis optica, there are many other causes. These include other inflammatory aetiologies, infection, malignancy and metabolic disturbance. Some of these are readily treatable. Laboratory and radiological investigations can help to differentiate these causes. Treatment of longitudinally extensive transverse myelitis hinges on distinguishing inflammatory and non-inflammatory aetiologies and identifying patients who are at high risk of a recurrent course.