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Autoimmune encephalitis (AE) and paraneoplastic neurological syndromes (PNS) are rare neuroimmune syndromes with a wide range of clinical presentation but without pathognomonic clinical sign facilitating the diagnosis. A lot of differential diagnoses are possible such as neurodegenerative diseases or viral infections. Although rare the diagnosis of AE or PNS is essential because despite severe neurological symptoms, patients can be cured by appropriate immunotherapy. Autoantibodies highly specific of AE and PNS has been described in the serum and cerebrospinal fluid of the patients and can be used as biomarkers of the disease. Their presence can predict an autoimmune origin and in many cases a good prognosis after immunotherapy. However, if some autoantibodies are now well-characterized and industrial kits have been developed to detect them, in numerous cases of highly suspect AE or PNS no specific autoantibodies are identified leading frequently to an inappropriate treatment. Furthermore, as the mechanisms of AE and PNS is still unknown, treatments are not optimal and in some cases inefficient. There is no prognosis biomarker able to predict the patient's sensitivity to immunotherapy and there are only few clues to know how the immune system can provoke the neuropsychiatric symptoms observed in the patients.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Antibody characterized | Patients with well-characterized antibody (HU, YO, RI, CASPR2, NMDAr, GAD, …) |
| |
| Atypical | Patients with atypical antibody |
| |
| Without antibody | Patients without antibody |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Genetic and immunology tests | Biological | This is a non-interventional study involving clinical data and biological samples (blood, DNA, CSF, cells). Clinical data are collected for the center and samples are already stored in biobank repositories and collected as part of "good clinical practice" in the diagnostic process of patients with suspected autoimmune encephalitis, meaning that the standard diagnostic and therapeutic approaches will not be altered in the selected study population. Patients have already gave explicit written consent for biological specimens sampling and storage at the "Centre de Ressources Biologiques des Hospices Civils de Lyon" (CRB-HCL) (including tissue, cells or biological fluids) and genetic analysis for research purposes. |
| Measure | Description | Time Frame |
|---|---|---|
| Correlation between biological results and clinical data | This measure will compare the result of immunological and genetic makers with clinical data of each patient. | Through study completion, an average of 1year |
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Inclusion Criteria:
Exclusion Criteria:
- No available clinical data
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Patient with auto-immune encephalitis suspected and antibody characterized or not.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Jerome HONNORAT, MD | Contact | +33 4 72 35 78 06 | jerome.honnorat@chu-lyon.fr | |
| Marine VILLARD, RCA | Contact | +33 4 27 85 54 60 | marine.villard@chu-lyon.fr |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Centre de référence des syndromes neurologiques paranéoplasiques et encéphalites autoimmunes | Recruiting | Lyon | 69677 | France |
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| ID | Term |
|---|---|
| D020274 | Autoimmune Diseases of the Nervous System |
| ID | Term |
|---|---|
| D009422 | Nervous System Diseases |
| D001327 | Autoimmune Diseases |
| D007154 | Immune System Diseases |
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| ID | Term |
|---|---|
| D007159 | Immunologic Tests |
| ID | Term |
|---|---|
| D019411 | Clinical Laboratory Techniques |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
| D008919 | Investigative Techniques |
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Blood, DNA, cells, CSF collected at diagnosis time
|
| D007158 | Immunologic Techniques |