ObjectiveTo investigate whether the use of mycophenolate mofetil (MMF) could reduce the relapse risk in patients with myelin oligodendrocyte glycoprotein (MOG)-immunoglobulin G (IgG)-associated disorders (MOGADs).MethodsThis prospective observational cohort study included patients with MOGAD at Peking Union Medical College Hospital between January 1, 2017, and April 30, 2019. The patients were divided into 2 groups: those with (MMF+) or without (MMF−) MMF therapy. The primary outcome was relapse at follow-up. We used Cox proportional hazards models to calculate hazard ratios (HRs) for relapse.ResultsSeventy-nine patients were included in our MOG cohort. Fifty (63.3%) were adults at index date, and 47 (59.5%) were women. Fifty-four (68.4%) were in the MMF+ group, and 25 (31.6%) were in the MMF− group. Clinical and demographic factors, MOG-IgG titer, and follow-up time (median, 472.5 days for MMF+, 261.0 days for MMF−) were comparable between the groups. Relapse rates were 7.4% (4/54) in the MMF+ group and 44.0% (11/25) in the MMF− group. Of all potential confounders, only the use of MMF was associated with reduced risk of relapse. The HR for relapse among patients in the MMF+ group was 0.14 (95% CI, 0.05–0.45) and was 0.08 (95% CI, 0.02–0.28) in a model adjusted for age, sex, disease course, and MOG-IgG titer. MMF therapy also remained associated with a reduced relapse risk in sensitivity analyses. Only one patient (1.9%) discontinued MMF therapy because of adverse effect.ConclusionsThese findings provide a clinical evidence that MMF immunosuppression therapy may prevent relapse in patients with MOGAD.Classification of evidenceThis study provides class IV evidence that for patients with MOGAD, MMF reduces relapse risk.

There is an increasing need for better understanding of the impact of coronavirus disease 2019 (COVID-19) on patients with neuromyelitis optica spectrum disorder (NMOSD). A few pilot studies have investigated COVID-19 infections in NMOSD, but few studies have addressed disease activity and immune status of these patients during the pandemic. We carried out a cross-sectional study to examine immune status, relapses, and COVID-19 infections in a cohort of NMOSD patients using an electronic patient registry (MSNMOBase) for multiple sclerosis and related disorders. An online questionnaire was administered to all NMOSD patients in the registry from January 1, 2011, to June 1, 2020. Clinical demographic characteristics, immune status, relapses, treatments, COVID-19 infections, and preventive measures were evaluated. Of the 752 registered patients, 535 (71.1%) with qualified data were included. A total of 486 used preventive therapies during the pandemic, including mycophenolate mofetil (71.2%), azathioprine (13.3%), and other immunosuppressants (6.4%). Neither median immune cell counts nor immunoglobulin levels (p > 0.05) were significantly different between patients with or without immunosuppression. During the pandemic, no patients were diagnosed with COVID-19, and the majority (>95%) took one or more effective protective measures (e.g., wearing a mask and social distancing). However, a significantly higher annualized relapse rate (ARR) was observed in the 33 patients with treatment interruptions due to the pandemic compared to before it (p < 0.05), whereas ARR changes were not found in patients with continuous treatments or those without treatments (p > 0.05). Interruption frequency was significantly higher in patients with relapses compared to those without (34.9 vs. 15.7%, p < 0.01). For stable NMOSD patients during the pandemic, the risk of relapse due to treatment interruption may be higher than the risk of COVID-19 infection when protective measures are used, and continuous relapse-prevention treatments may be necessary.

VPS along with corticosteroids and immunosuppressants represents an effective treatment approach for patients who suffer from hydrocephalus associated with autoimmune diseases.

The current perception threshold (CPT) is a device which can evaluate different sensory fibers quantitatively through different frequencies of the electrical stimulus and has been applied in clinical practice. Previous studies have implied that CPT values may be affected by age, gender, and other factors, yet not conclusively. The objective of our study is to clarify the influencing factors of CPT values and establish a reference value range. Twenty healthy volunteers recruited publicly and 146 subjects who took CPT tests in the census of the national project cardiovascular and cerebrovascular diseases in rural areas of China from 2013 to 2015 were analyzed. Past medical history and demographic characteristics such as age, gender, and occupation were collected. Each subject was tested on the left index finger (or back of the left hand) and the right hallux. CPT values of 2000, 250, and 5 Hz on both sites were recorded for statistical analysis. Gender differences were shown at 2000 Hz CPT on the back of the hand and hallux (p < 0.01), and male subjects had a higher CPT. Age had a positive correlation with 250 Hz CPT on the index finger (p < 0.05, r = 1.5), 2000 Hz CPT on the back of the hand (p < 0.001, r = 1.2) and index finger (p < 0.05, r = 2.5). Manual workers had a higher 250 Hz CPT on the hallux than mental workers (p < 0.01). After investigating the impact of different factors on CPT testing, we established the reference value for subjects with different characteristics.

Background:Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving both upper and lower motor neurons with no effective cure. Electrophysiological studies have found decremental responses during low-frequency repetitive nerve stimulation (RNS) except for diffused neurogenic activities. However, the difference between ALS and generalized myasthenia gravis (GMG) in terms of waveform features is unclear. In the current study, we explored the variation trend of the amplitudes curve between ALS and GMG with low-frequency, positive RNS, and the possible mechanism is discussed preliminarily.Methods:A total of 85 ALS patients and 41 GMG patients were recruited. All patients were from Peking Union Medical College Hospital (PUMCH) between July 1, 2012 and February 28, 2015. RNS study included ulnar nerve, accessory nerve and facial nerve at 3 Hz and 5 Hz stimulation. The percentage reduction in the amplitude of the fourth or fifth wave from the first wave was calculated and compared with the normal values of our hospital. A 15% decrease in amplitude is defined as a decrease in amplitude.Results:The decremental response at low-frequency RNS showed the abnormal rate of RNS decline was 54.1% (46/85) in the ALS group, and the results of different nerves were 54.1% (46/85) of the accessory nerve, 8.2% (7/85) of the ulnar nerve and 0% (0/85) of the facial nerve stimulation, respectively. In the GMG group, the abnormal rate of RNS decline was 100% (41/41) at low-frequency RNS of accessory nerves. However, there was a significant difference between the 2 groups in the amplitude after the sixth wave.Conclusions:Both groups of patients are able to show a decreasing amplitude of low-frequency stimulation RNS, but the recovery trend after the sixth wave has significant variation. It implies the different pathogenesis of NMJ dysfunction of these 2 diseases.

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Cases of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy who were initially diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) were rarely reported. Herein, we reported a 31-year-old woman who presented with 7 years of recurrent headache. Her clinical history, symptoms, brain MRI enhancement features, and response to treatment during each attack were reviewed. Her brain MRI 7 years ago demonstrated characteristic pepper-like enhancement of pontine and cerebellum and her symptoms resolved completely after taking a high-dose of steroids. She was suspected with the diagnosis of CLIPPERS, and she experienced five relapses once the oral steroid was tapered below 20 mg/day. During her last relapse, she experienced fever and psychosis, and GFAPα-antibodies were detected in her serum and cerebrospinal fluid by antigen-transfected HEK293 cell-based assay (indirect immunofluorescence assay). She obtained relief again after steroid therapy, and her diagnosis converted to autoimmune GFAP astrocytopathy. Autoimmune GFAP astrocytopathy may mimic CLIPPERS, both clinically and radiologically. Long-term follow-up is essential for necessary diagnosis revision at each new attack in patients with a diagnosis of CLIPPERS.

Systemic autoimmune diseases (SAIDs) represent a group of syndromes involving at least two organ systems. Classical SAIDs include connective tissue diseases, vasculitis, and granulomatous diseases, many of which involve the nervous system and result in different neurological manifestations. Hydrocephalus can be a rare but lethal complication of various SAIDs, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), sarcoidosis, and primary vasculitis. However, the pathogenesis of SAIDs complicated with different types of hydrocephalus is varied and difficult to determine using the existing published data, and various manifestations and expressive forms of the conditions bring a substantial challenge to a timely clinical diagnosis and treatment. The commonly used medical management programs based on the etiology of hydrocephalus are anti-inflammatory or anti-infectious therapies, while surgical management such as ventriculoperitoneal shunts is effective most of the time. Further research should be directed toward improving our understanding of the pathogenesis of these conditions and determining the most effective method for treating this life-threatening condition.