A Pilot Study of the Use of Rituximab in the Treatment of Chronic Focal Encephalitis

NCT ID: NCT00259805

Last Updated: 2013-03-22

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE1

Total Enrollment

10 participants

Study Classification

INTERVENTIONAL

Study Start Date

2005-01-31

Study Completion Date

2008-12-31

Brief Summary

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The purpose of this study is to assess the safety, tolerability and effectiveness of rituximab in the treatment of chronic focal encephalitis.

Detailed Description

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Chronic Focal Encephalitis (Rasmussen's Encephalitis) is a condition characterized by a progressive hemiparesis, cognitive decline (including loss of language skills if the language dominant hemisphere is involved) and epileptic seizures that are typically refractory to medical treatment (Rasmussen). Attempts to control the seizures with anticonvulsants are ineffective and the only effective treatment to date is a hemispherectomy (surgical removal of half of the brain). Children with CFE who undergo cortical resections or hemispherectomies demonstrate an inflammatory histopathology consisting of perivascular lymphocytic cuffing, gliosis, neuronal loss, microglial nodules and later laminar necrosis and spongy degeneration

Rituximab is a genetically engineered, chimeric; murine/human monoclonal antibody directed against the CD20 antigen found on the surface of normal and malignant pre-B and mature B cells. It was approved by the FDA in 1997 for the treatment of relapsed or refractory low grade or follicular, CD20+, B-cell non-Hodgkin's lymphoma (NHL). Rituximab binds specifically to the CD20 antigen expressed on the surface of both normal and malignant pre-B and mature B cells. In vitro mechanism of action studies have demonstrated that the Fc portion of Rituximab binds human complement and can lead to cell lysis of the targeted cell through complement-dependent cytotoxicity. Additionally, it has been demonstrated that Rituximab has significant activity in assays of antibody dependent cellular cytotoxicity (Reff et al. 1994). More recently, Rituximab has been shown to induce apoptosis in vitro in DHL-4, a human B cell lymphoma line (Maloney et al. 1997). The relative extent to which these individual mechanisms account for the observed depletion of normal and malignant B cells in vivo is unknown.

While CFE represents only a very small percentage of patients with epilepsy, the devastating progressive nature of the disease with out any adequate treatments, relegates these children to the relentless loss of cognitive and motor skills, and continuing seizures. Recent evidence suggests this condition is immune mediated and includes the development of antibodies directed against various brain components including glutamate receptors (GluR3) (Rogers). Brain samples from patients with CFE have demonstrated immunoreactivity for IgG, C4 C8, and MAC (Andrews and Whitney) and involvement of both B and T-lymphocytes. Evidence supporting a role for clonally expanded B lymphocytes was found by Baranzini . By analyzing the T-cell receptor expression in brain lesions using PCR these investigators also demonstrated the local immune response in CFE included restricted T-cell populations probably expanding from a few precursor T-cells responding to discrete antigenic epitopes (Li). Following demonstration of antibodies directed against brain elements in CFE, a patient was treated with plasma exchange which produced a significant improvement in seizure frequency, cognition and hemiparesis, lending support to the hypothesis that circulating antibodies contribute to the disease pathogenesis. Subsequently attempts to modify this disease by immune modification (plasmaphoresis, steroids, gamma globulin) have demonstrated modest improvements but the improvements have been short-lived and have not affected the natural progression of this disease. This pilot study proposes to directly attack the cells (B-cells) thought to be instrumental in the development of this condition. Should this approach to treating CFE be successful it will have a major impact on these children's lives.

Conditions

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Chronic Focal Encephalitis Rasmussen's Encephalitis

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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IV Infusion

Group Type EXPERIMENTAL

Rituximab

Intervention Type DRUG

375 mg/m2 given as an IV infusion once weekly for four doses

Interventions

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Rituximab

375 mg/m2 given as an IV infusion once weekly for four doses

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Diagnosis of chronic focal encephalitis
* IgG \& IgM levels within normal limits
* Adequate renal function
* Stable anticonvulsant drug regimen

Exclusion Criteria

* Evidence of significant ongoing medical condition or progressive neurologic condition (other than CFE)
* Previous treatment with rituximab
* History of significant recurrent infections, or ongoing active infection
* Receipt of a live vaccine within 4 weeks prior to treatment
* History of severe allergic reactions to humanized or murine monoclonal antibodies
* History of drug, alcohol or chemical abuse within 6 months
* Concomitant malignancies or previous malignancy
* Use of steroids or immunoglobulins during the 4 weeks prior to treatment
* Hemoglobin \<8.5 gm/dL, Platelets \< 100,00/mm, AST or ALT \>2.5 ULN
* Positive Hepatitis B or C serology
* History of positive HIV
Minimum Eligible Age

5 Years

Maximum Eligible Age

25 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Genentech, Inc.

INDUSTRY

Sponsor Role collaborator

California Pacific Medical Center Research Institute

OTHER

Sponsor Role lead

Responsible Party

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Kenneth D. Laxer, M.D.

Medical Doctor

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Kenneth D Laxer, M.D.

Role: PRINCIPAL_INVESTIGATOR

California Pacific Medical Center

Locations

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California Pacific Medical Center

San Francisco, California, United States

Site Status

Countries

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United States

References

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Baranzini SE, Laxer K, Bollen A, Oksenberg JR. Gene expression analysis reveals altered brain transcription of glutamate receptors and inflammatory genes in a patient with chronic focal (Rasmussen's) encephalitis. J Neuroimmunol. 2002 Jul;128(1-2):9-15. doi: 10.1016/s0165-5728(02)00109-1.

Reference Type BACKGROUND
PMID: 12098505 (View on PubMed)

Prayson RA, Frater JL. Rasmussen encephalitis: a clinicopathologic and immunohistochemical study of seven patients. Am J Clin Pathol. 2002 May;117(5):776-82. doi: 10.1309/AD8R-560C-4V11-C5E2.

Reference Type BACKGROUND
PMID: 12090428 (View on PubMed)

Baranzini SE, Laxer K, Saketkhoo R, Elkins MK, Parent JM, Mantegazza R, Oksenberg JR. Analysis of antibody gene rearrangement, usage, and specificity in chronic focal encephalitis. Neurology. 2002 Mar 12;58(5):709-16. doi: 10.1212/wnl.58.5.709.

Reference Type BACKGROUND
PMID: 11889232 (View on PubMed)

Whitney KD, Andrews PI, McNamara JO. Immunoglobulin G and complement immunoreactivity in the cerebral cortex of patients with Rasmussen's encephalitis. Neurology. 1999 Sep 11;53(4):699-708. doi: 10.1212/wnl.53.4.699.

Reference Type BACKGROUND
PMID: 10489029 (View on PubMed)

Prayson RA, Bingaman W, Frater JL, Wyllie E. Histopathologic findings in 37 cases of functional hemispherectomy. Ann Diagn Pathol. 1999 Aug;3(4):205-12. doi: 10.1016/s1092-9134(99)80052-5.

Reference Type BACKGROUND
PMID: 10459046 (View on PubMed)

Li Y, Uccelli A, Laxer KD, Jeong MC, Vinters HV, Tourtellotte WW, Hauser SL, Oksenberg JR. Local-clonal expansion of infiltrating T lymphocytes in chronic encephalitis of Rasmussen. J Immunol. 1997 Feb 1;158(3):1428-37.

Reference Type BACKGROUND
PMID: 9013988 (View on PubMed)

Hart YM, Cortez M, Andermann F, Hwang P, Fish DR, Dulac O, Silver K, Fejerman N, Cross H, Sherwin A, et al. Medical treatment of Rasmussen's syndrome (chronic encephalitis and epilepsy): effect of high-dose steroids or immunoglobulins in 19 patients. Neurology. 1994 Jun;44(6):1030-6. doi: 10.1212/wnl.44.6.1030.

Reference Type BACKGROUND
PMID: 8208394 (View on PubMed)

Rasmussen T. Further observations on the syndrome of chronic encephalitis and epilepsy. Appl Neurophysiol. 1978;41(1-4):1-12. doi: 10.1159/000102395.

Reference Type BACKGROUND
PMID: 103497 (View on PubMed)

Other Identifiers

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Genentech CFE-001

Identifier Type: -

Identifier Source: secondary_id

CPMC-IRB25.102

Identifier Type: -

Identifier Source: org_study_id

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