Treatment response, risk of relapse and clinical characteristics of Taiwanese patients with neuromyelitis optica spectrum disorder.

BACKGROUND/PURPOSE: The long-term disease course and efficacy of maintenance therapies have rarely been investigated in Asian patients with neuromyelitis optica spectrum disorder (NMOSD). METHODS: Medical records of patients fulfilling the 2015 International Consensus Diagnostic Criteria for NMOSD at three medical centers in Taiwan were systematically analyzed. Linear regression analysis was performed to investigate factors related to annualized relapse rate (ARR); survival analysis was used to estimate the relapse-free intervals among therapies. RESULTS: A total of 557 relapses affecting 648 regions (202 optic neuritis, 352 acute myelitis, and 94 brain syndromes) in 204 patients were analyzed during a follow-up period of 69.5 months (range, 1-420). Up to 36.1% of myelitis-onset patients and 24.0% of optic neuritis-onset patients exhibited a limited form disease, defined as having one or more relapses confined to the same region. The median ARR was significantly lower in patients with limited form disease than those with relapses involving multiple regions (0.30 vs. 0.47, respectively). An older age at disease onset was associated with a lower ARR (p = 0.023). Kaplan-Meier analysis showed that the estimated time (months) to next relapse was longest in rituximab-treatment group (58.0 ± 13.2), followed by immunosuppressant (48.5 ± 4.8) or prednisone (29.6 ± 4.6) groups, and shortest in those without maintenance therapy (27.6 ± 4.2) (p = 8.1 × 10-7). CONCLUSION: Limited form disease and older age at disease onset are associated with a lower relapse rate in NMOSD. Compared to no maintenance therapy, rituximab and immunosuppressant significantly reduce the relapse risks.

Determinants of arterial stiffness progression in a Han-Chinese population in Taiwan: a 4-year longitudinal follow-up.

BACKGROUND: Arterial stiffness predicts the future risk of macro- and micro-vascular diseases. Only a few studies have reported longitudinal changes. The present study aimed to investigate the progression rate of arterial stiffness and the factors influencing stiffness progression in a Han Chinese population residing in Taiwan. METHODS: The pulse wave velocity (PWV), elasticity modulus (Ep) and arterial stiffness index (ß) of the common carotid artery were measured in 577 stroke- and myocardial infarction-free subjects at baseline and after an average interval of 4.2 ± 0.8 years. Stepwise multivariate linear regression was conducted to elucidate the predictors of stiffness progression. RESULTS: For both baseline and follow-up data, men had significantly higher values of PWV, Ep and ß in comparison to women. The progression rates of PWV, Ep and ß were faster in men, but the difference was not statistically significant (ΔPWV = 0.20 ± 0.20 and 0.18 ± 0.20 m/s/yr; ΔEp = 8.17 ± 8.65 and 6.98 ± 8.26 kPa/yr; Δß = 0.70 ± 0.64 and 0.67 ± 0.56 for men and women, respectively). In the multivariate regression analyses, age, baseline stiffness parameters, baseline mean arterial pressure (MAP), baseline body mass index (BMI) and changes in MAP (ΔMAP) were independent predictors of PWV and Ep progression. There was an inverse correlation between the stiffness parameters at baseline and their progression rate (correlation coefficient (r) = -0.12 to -0.33, p = 0.032-1.6 × 10(-16)). Changes in MAP (ΔMAP) rather than baseline MAP were more strongly associated with PWV progression (p = 8.5 × 10(-24) and 1.9 × 10(-5) for ΔMAP and baseline MAP, respectively). Sex-specific analyses disclosed that baseline BMI and changes in BMI (ΔBMI) were significantly associated with stiffness progression in men (p = 0.010-0.026), but not in women. CONCLUSIONS: Aging and elevated blood pressure at baseline and during follow-up were the major determinants of stiffness progression in the Han Chinese population. For men, increased baseline BMI and changes in BMI were additional risk factors.

Paraneoplastic Limbic Encephalitis Associated with Adenocarcinoma of Lung.

PURPOSE: Paraneoplastic limbic encephalitis (PLE) is a rare, immune-mediated entity. We present an unusual case of a patient who has double cancers and two different paraneoplastic neurological syndromes. CASE REPORT: A 58-year-old gentleman has histories of adenocarcinoma of lung and malignant thymoma associated with myasthenia gravis, which underwent surgery and chemotherapy 3 years ago. This time, he presented to our ward with rapidly progressive memory decline and myoclonic jerks in his limbs for two weeks. Magnetic resonance imaging (MRI) of brain showed increased signal intensity over bilateral mesial temporal regions on T2 Fluid Attenuated Inversion Recover (FLAIR) series. Chest computed tomography showed cancer recurrence. He received steroid pulse therapy firstly and right lung lower lobe lobectomy later. Pathology report of the tumor was recurrent adenocarcinoma. After the immunotherapy and tumor resection, his mentality improved gradually. Six months later, brain MRI showed resolution of bilateral temporal hyperintensity with residual mesial temporal atrophy. CONCLUSION: From our case, we would like to emphasize that paraneoplastic limbic encephalitis should be considered among the differential diagnosis of rapidly progressive dementia associated with myoclonus, along with other neurodegenerative diseases. Depending on its underlying malignancy, the cognitive impairment may be substantially reversible, despite atrophy of mesial temporal lobes.