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| ID | Type | Description | Link |
|---|---|---|---|
| 2019-A00888-49 | Other Identifier | ANSM |
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Since the initial study of cases reported between 2009 and 2015, adalimumab has become in France the immunomodulatory reference treatment used after failure of corticosteroids and immunoglobulins before a possible recourse to the hemispherotomy. This observational study is intended to document the long-term efficacy and safety of Adalimumab therapy in patients with Rasmussen encephalitis.
Rasmussen Encephalitis (ER) is a particularly severe chronic inflammatory brain disorder resulting in the progressive destruction of a hemisphere. It is a rare disease although at present no precise prevalence is available. It begins preferentially in children.
This inflammatory process is accompanied by a progressive loss of function of the affected hemisphere, associated with a pharmaco-resistant partial epilepsy. The diagnosis is based on a bundle of clinical, radiological and electroencephalographic arguments. CSF analysis directs diagnosis in 50% of cases.
No anti-epileptic treatment can stop seizures. Only hemispherotomy (surgical disconnection of a cerebral hemisphere) allows it but it is associated with definitive motor and cognitive deficits.
Over the last 20 years, new therapeutic trials have focused on immuno-modulatory treatments targeted at the T-lymphocyte pathway, including tacrolimus. Although they seem to be more effective than immunoglobulins or corticosteroids, it remains transient. In addition, the number of published cases is low. In this context, starting in 2009, it has been proposed to use adalimumab (Ab anti TNF) based on:
Thus, in the continuation of the work carried out previously (French study on the cases between 2009 and 2015), the aim of this research projet is to complete the follow-up of the patients who previously took part in in the first study and to establish the follow-up of the patients treated by adalimumab since then.
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Adalimumab treatment | Other | Retrospective observational study initially and then prospective with inclusion of all the patients carrying an ER treated by Adalimumab in France. Follow-up will continue for one year if adalimumab is discontinued. |
| Measure | Description | Time Frame |
|---|---|---|
| Efficiency of the treatment measured by the reduction of the number of epileptic seizures | The change of epileptic seizures (Will be considered as a responder patient any patient with a 50% decrease in the number of seizures between the time of inclusion and 6 months after the start of treatment). This response to treatment is re-evaluated at 12 months then every 6 months for 5 years. | 5 years |
| Efficiency of the treatment measured by the stability if the cognitive assessment | Stability of the cognitive assessment: variation of less than 10 points on each of the different composite indices (QIT, ICV, IRF, IMT, IVT) of age-appropriate Weschler scales, between two cognitive assessments of 6 months. | 6 months |
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Inclusion Criteria:
Exclusion Criteria:
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The implementation of a treatment with adalimumab in a patient with an ER requires the performance of a CPR within a reference center Epilepsies Rares if one complies with the rules of good clinical practice. Thus, all ER patients treated with adalimumab are referenced in a referral center. There are 7 reference centers in France, they are all partners of the study.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Anne Lépine | Contact | 0491387267 | 33 | anne.lepine@ap-hm.fr |
| DRS AP-HM | Contact | 0491381499 | 33 | drci@ap-hm.fr |
| Name | Affiliation | Role |
|---|---|---|
| Jean-Olivier ARNAUD | Assistance Publique- Hôpitaux de Marseille | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Chu de Bordeaux | Not yet recruiting | Bordeaux | France |
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| Chu Brest | Not yet recruiting | Brest | France |
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| Chru de Lille | Not yet recruiting | Lille | France |
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| Chu Limoges | Not yet recruiting | Limoges | France |
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| Hospices Civils de Lyon | Not yet recruiting | Lyon | France |
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| Service Neuropédiatrie | Recruiting | Marseille | 13005 | France |
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| Chu Montpellier | Not yet recruiting | Montpellier | France |
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| Chu de Nancy | Not yet recruiting | Nancy | France |
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| Chu Necker Ap-Hp | Not yet recruiting | Paris | France |
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| Chu Pitie Salpietriere Ap-Hp | Not yet recruiting | Paris | France |
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| Chu Robert Debre | Not yet recruiting | Paris | France |
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| Fondation Adolphe de Rothshild | Not yet recruiting | Paris | France |
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| Chu de Rennes | Not yet recruiting | Rennes | France |
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| Chu Strasbourg | Not yet recruiting | Strasbourg | France |
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| Chu Toulouse | Not yet recruiting | Toulouse | France |
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| Chru de Tours | Not yet recruiting | Tours | France |
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| ID | Term |
|---|---|
| D004660 | Encephalitis |
| ID | Term |
|---|---|
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D000090862 | Neuroinflammatory Diseases |
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