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Phase 1 N=10 Treatment

C1-esterase Inhibitor (Cinryze) for Acute Treatment of Neuromyelitis Optica Exacerbation

Neuromyelitis Optica

Enrolled (actual)
10
Serious AEs
0.0%
Results posted
Jul 2014
Primary outcome: Primary: Number of Adverse Safety Events During Hospitalization — 0 adverse safety events

Study Design & Population

Study type
Interventional
Phase
Phase 1
Interventions
C1-esterase inhibitor (Cinryze) (Drug)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Michael Levy
Primary completion
Nov 2013

Outcome Measures

OutcomeResultp-value
PRIMARY
Number of Adverse Safety Events During Hospitalization
SECONDARY
Frequency of Serious Adverse Events.
SECONDARY
Percentage of Subjects Withdrawing Due to Adverse Events.
SECONDARY
Change From Baseline in Hematology, Chemistry, and Urinalysis Parameters.
1; 0; 0
SECONDARY
Expanded Disability Status Score (EDSS)
2.5

Summary

The overall objective is to evaluate the tolerability/safety and preliminary efficacy of CINRYZE® (C1 esterase inhibitor [human]) as add-on therapy for treatment of acute optic neuritis and/or transverse myelitis in NMO and NMOSD. Primary Objective: To evaluate the safety and tolerability of 3-5 doses of 1000 - 2000 Units intravenous CINRYZE in NMO/NMOSD patients during an acute exacerbation. Secondary Objectives: * To determine the frequency of adverse events with CINRYZE in this patient population. * To determine the effect of CINRYZE on NMO clinical scores (Expanded Disability Status Scale and Low Contrast Visual Acuity). * To compare the change in MRI lesion size and extent following a course of CINRYZE.

Eligibility Criteria

Inclusion Criteria

  • Able and willing to provide written informed consent.
  • 18-65 years of age.
  • New acute optic neuritis and/or transverse myelitis. A clinical event is defined as an episode of inflammation in the spinal cord and/or optic nerve leading to neurologic symptoms not ascribed to another disease process.
  • Diagnosis of NMO according to the 2006 revisions of the Wingerchuk diagnostic criteria for NMO (Wingerchuk, 2006), or AQP4 positive NMOSD per the EFNS Guidelines. For NMO, subjects must have two absolute criteria:
  • optic neuritis
  • myelitis and at least two of three supportive criteria:
  • presence of a contiguous spinal cord MRI lesion extending over three or more vertebral segments,
  • MRI criteria NOT satisfying the revised McDonald diagnostic criteria for MS [Polman, 2011]
  • NMO-IgG (AQP4) in serum. For NMOSD, subjects must have longitudinally extensive transverse myelitis (LETM) recurrent isolated optic neuritis (RION)/bilateral optic neuritis (BON), or opticospinal multiple sclerosis (OSMS) that is AQP4 antibody positive.
  • A female subject is eligible to enter the study if she is:

A. Not pregnant or nursing; B. Of non-childbearing potential (i.e. women who have had a hysterectomy, are post-menopausal, which is defined as >2 years without menses or, in female subjects who have been post-menopausal for 5 years

  • A history of hematologic malignancy excludes a subject from participation, regardless of response.
  • Significant concurrent, uncontrolled medical condition including, but not limited to, cardiac, renal, hepatic, hematological, gastrointestinal, endocrine, immunodeficiency syndrome, pulmonary, cerebral, psychiatric, or neurological disease which could affect the subject's safety, impair the subject's reliable participation in the trial, impair the evaluation of endpoints, or necessitate the use of medication not allowed by the protocol, as determined by the PI of the study.
  • Use of an investigational drug or other experimental therapy for a condition other than NMO within 4 weeks, 5 pharmacokinetic half lives or duration of biological effect (whichever is longer) prior to screening.
  • Current participation in any other interventional clinical trial. Participation in non-interventional trial requires approval of the protocol by investigator.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT01759602). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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