Severe Neuroimmune Disease
Conditions
Interventions
Myasthenia gravis:plasma exchange
Myasthenia gravis:Lymphatic plasma exchange
Myasthenia gravis:Intravenous immunoglobulin
Acute inflammatory demyelinating polyneuropathies:plasma exchange
Acute inflammatory demyelinating polyneuropathies:Lymphatic plasma exchange
Acute inflammatory demyelinating polyneuropathies:Intravenous immunoglobulin
Neuromyelitis optica spectrum disorders:plasma exchange
Neuromyelitis optica spectrum disorders:Lymphatic plasma exchange
Neuromyelitis optica spectrum disorders:Methylprednisolone Intravenous Shock
Anti-N-Methyl-D-aspartate Receptor Encephalitis:plasma exchange
Anti-N-Methyl-D-aspartate Receptor Encephalitis:Lymphatic plasma exchange
Anti-N-Methyl-D-aspartate Receptor Encephalitis:Methylprednisolone Intravenous Shock
Anti-N-Methyl-D-aspartate Receptor Encephalitis:Intravenous immunoglobulin
Sponsors
Department of Neurology, Xiangya Hospital, Central South University
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. Severe MG: refers to MG patients with crisis or threatened crisis. Fluctuating weakness and morbid fatigue exist in the patients. neostigmine test (+), low frequency and high frequency of repetitive nerve stimulation amplitude decrease (> 10%), and any one of AchR-ab, Musk-Ab, Titin-Ab or RyR-Ab is positive or negative. MG crisis or threatened crisis is defined as: MG patients' QMG score >=20 points or severe dysphagia (corresponding single score 3 points) in spite of QMG 4 points. Severe AIDP: meets NINDS1990 diagnostic criteria and is difficult to walk independently (Huges grade 3-5). Severe NMOSDs: meets IPND 2015 standard and EDSS score >=6 points when symptoms are most severe, or EDSS increases by 0.5 points when EDSS baseline score >=6 points; When optic neuritis is the main symptom, severe attack is defined as new onset or worsening visual impairment leading to VA 0.1). If baseline vision is only light perception and finger movement or counting index can be felt, any evidence of visual deterioration accompanied by optic neuritis on MRI is severe attack. Severe AIE: meets the diagnostic criteria of AIE in 2016, and has consciousness change or status epilepticus; 2. willing to receive PE/LPE or IVIG or IVMP treatment; 3. Willing to follow research procedures; 4. Signed informed consent form.
Exclusion criteria
Exclusion criteria: 1. Combined with tumor (excluding MG patients with thymoma; Excluding NMDAR encephalitis patients complicated with teratoma or other tumors); 2. Patients with other neurological or musculoskeletal diseases affecting neurological function assessment; 3. Pregnant or lactating women and women planning pregnancy in the near future; 4. Serious cardiac diseases include cardiac insufficiency (NYHA III-IV grade), acute coronary syndrome, severe arrhythmia or severe cardiomyopathy, and patients with allergic reaction to blood products are not suitable for lymphatic plasma exchange, plasma exchange or IVIG treatment. The inappropriate central venous catheterization includes severe coagulation dysfunction and thrombocytopenia, venous thrombosis of the catheterization vein, and history of surgery or trauma. Patients with uncontrolled hypertension, diabetes, active peptic ulcer, severe osteoporosis and other inappropriate corticosteroids (confirmed NMOSDs/AIE patients); 5. GBS patients with onset of illness over 4 weeks and not receiving regular treatment; 6. Severe systemic infection, sepsis or allergy to heparin fish and protamine (most patients are predicted to be treated with IVIG, which may cause selection bias); 7. Patients with renal insufficiency (it can be predicted that most patients are treated with plasma exchange or lymphatic plasma exchange, which may lead to bias).
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| quantitative myasthenia gravis score (QMG);Medical Research Council score;Hughes classification;Overall Neuropathy Limitation Scale (ONLS);modified Rankin Scale (mRS);Mini-mental State Examination (MMSE);Functional system score (FSS);Expanded Disability Status Scale (EDSS);Visual acuity; | — |
Secondary
| Measure | Time frame |
|---|---|
| 15-item Myasthenia Gravis Quality of Life scale revised (MG-QOL15r);myasthenia gravis-activities of daily living (MG-ADL);Visual Analogue Score;Myasthenia Gravis Foundation of America Clinical Classification; | — |
Countries
China
Contacts
Public ContactHuan Yang
Xiangya Hospital Central South University
Outcome results
None listed