Efficacy of Tacrolimus and I.V.-Immunoglobulins in Rasmussen Encephalitis
Rasmussen Encephalitis
About this trial
This is an interventional treatment trial for Rasmussen Encephalitis focused on measuring Rasmussen encephalitis, Chronic encephalitis, Cerebral hemiatrophy, Hemiparesis, Epilepsy
Eligibility Criteria
Inclusion Criteria:
Patients meeting at least two of the following three criteria:
- Clinical: Epilepsia partialis continua or progressive* hemiparesis
- MRI: Progressive* cerebral hemiatrophy
Histopathology: T cell dominated encephalitis with activated microglial cells (typically, but not necessarily forming nodules) and reactive astrogliosis. Numerous macrophages, B cells or plasma cells or positive signs of viral infections (viral inclusion bodies or immunohistochemical demonstration of viral protein) exclude the diagnosis of RE.
- "Progressive" means that at least two sequential clinical examinations or MRI studies documenting increasing deficits or tissue loss are required to meet the respective criteria.
Exclusion Criteria:
- Neuroradiological signs of a bihemispheric encephalitis.
- Wave-like course with history of repeated remissions.
- Infectious disease as a contraindication to an immunosuppressive therapy.
- Paraneoplastic encephalitis.
- Previous treatment with > 3 weeks of corticosteroids or tacrolimus or > 1,2 g/kg IVIG or > 5 PEX/PAI within the last three months.
- Onset of acute disease stage more than 12 months ago.
- Patient already in residual stage, i.e., stable neurological deficit since >6 months.
- Hemispheric Ratio < 80% (< 90% in patients > 11 years)
- Histopathological evidence of cerebral inclusion bodies indicating a viral infection
Sites / Locations
- University of Bonn, Dept. of Epileptology
Arms of the Study
Arm 1
Arm 2
Experimental
Experimental
Group 1
Group 2
Tacrolimus capsules ("Prograf"; dosing according to blood trough levels: 12-15 ng/ml during months 1-6, 5-10 ng/ml during months 7-12 and 5-8 ng/ml thereafter)
Intravenous immunoglobulins (IVIG) infusions ("Octagam"; dosing: initially on three consecutive days 0,4 g/kg KG, thereafter 0,4 g/kg KG every month, after 12 months of treatment every two months).