Central nervous system (CNS) idiopathic inflammatory demyelinating diseases (IDD) are mainly diseases caused by autoimmune factors that result in CNS demyelination damage and loss. It tends to accumulate in the brain, spinal cord and optic nerves. Multiple sclerosis (MS), clinically isolated syndrome (CIS), neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and acute disseminated encephalomyelitis (ADEM) are all common IDDs of the CNS. Besides, primary angiitis of the central nervous system (PACNS), autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A), etc. may also be included because they are important differential diagnoses. This study will establish a large prospective cohort study database of Chinese IDD, which will record detailed electronic information on IDD patients, including demographic and socioeconomic data, medical history, clinical information, medication, and relevant examination results. The long-term observational study will be used to understand the natural history of disease, disability progression rates, imaging and biological indicators, long-term treatment approaches and prognosis of Chinese patients with IDD, to find predictive markers for IDD progression and prognosis, and to identify factors that influence the treatment and prognosis of patients with IDD.
1. No requirement for age and sex
2. Need to meet the diagnosis of at least one IDD (clinically isolated syndrome (CIS)/multiple sclerosis (MS)/neuromyelitis optica spectrum disorder (NMOSD)/MOG antibody-associated disease (MOGAD)/acute disseminated encephalomyelitis (ADEM).
3. Signed informed consent form.
Those with severe mental disease unable to cooperate with the examination and/or follow-up.
Any patient (or the patient's legal representative) who is unable or refuses to sign informed consent.
10000 IDD patients are planned to be recruited from many sites throughout the country.
Cohort
Prospective
Intravenous steroid
DrugThis study does not limit treatment methods. Patients commonly use high-dose intravenous steroid therapy (HD-S) during acute stage. Immunomodulatory therapies are necessary for the remission stage. All drugs are used in accordance with relevant guidelines.
Annual relapse rate (ARR) between baseline and follow-up in patients with IDD
a new neurological worsening lasting for at least 24 hours and occurring more than 30 days after the previous attack.
Change in EDSS scores of patients with IDD between baseline and follow-up over time
The Expanded Disability Status Scale (EDSS) is a rating system that is frequently used for classifying and standardizing the severity and progression. EDSS ranges from 0 to 10. The higher the score, the worse the clinical symptoms.
The brain structural change over time between the baseline MRI and the follow-up MRIs
To describe changes of lesions, grey matter and white matter in patients with neuroinflammatory and demyelination disease measured by Brain Magnetic Resonance Imaging (MRI). The MRI sequence includes T2W\_TRA、DWI、3DT1W\_TFE\_SAG、fMRI\_EPI\_TRA、mb2DKI\_iso48\_b1k2k\_TRA、3DFLAIR\_TSE\_SAG、3DpCASL\_GRASE\_TRA、3DDIR\_SAG、3DT2W\_TSE、SWI\_QSM\_TRA. The secondary endpoint is the change over time between the baseline MRI and the follow-up MRIs, of the lesions and brain volumes.
The spinal cord change over time between the baseline MRI and the follow-up MRIs.
To describe changes of lesions and integrity of fiber bundle in spinal cord in neuroinflammatory and demyelination disease patients measured by MRI. The primary endpoint is the change over time between the baseline MRI and the follow-up MRIs, of structural change in spinal cord.
Change of the central vein sign at 7T MRI.
The central vein sign has been proposed as a specific imaging biomarker for distinguishing between multiple sclerosis (MS) and not MS.
Change of rim of iron at 7T MRI.
multiple sclerosis white matter lesions surrounded by a rim of iron containing microglia, termed iron rim lesions, signify patients with more severe disease course and a propensity to develop progressive multiple sclerosis.
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