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MD1003-AMN MD1003 in Adrenomyeloneuropathy

Phase 2
Completed
Conditions
Adrenoleukodystrophy
AMN
Adrenomyeloneuropathy
Interventions
Drug: MD1003 100 mg capsule
Drug: Placebo
Registration Number
NCT02961803
Lead Sponsor
MedDay Pharmaceuticals SA
Brief Summary

The primary objective of the trial is to demonstrate the superiority of biotin at 300 mg/day over placebo in the clinical improvement (walking tests) of patients with adrenomyeloneuropathy

Detailed Description

AMN and progressive multiple sclerosis share some similarities including progressive spastic paraparesis and secondary energy failure leading to progressive axonal degeneration. Therefore, it was hypothesized that high doses of biotin might be efficient in patients with AMN.

Recruitment & Eligibility

Status
COMPLETED
Sex
Male
Target Recruitment
67
Inclusion Criteria
  • ABCD1 gene mutation identified
  • Elevated plasma VLCFA
  • Clinical signs of AMN with at least pyramidal signs in the lower limbs and difficulties to walk
  • EDSS score ≥ 3.5 and ≤ 6.5
  • Normal brain MRI or brain MRI showing :
  • abnormalities that can be observed in AMN patients without cerebral demyelination with a maximum Loes score of 4
  • and/or stable (≥6 months) cerebral demyelination without gadolinium enhancement with a Loes score ≤12.
  • Appropriate steroid replacement if adrenal insufficiency is present
  • Likely to be able to participate in all scheduled evaluation visits and complete all required study procedures
  • Signed and dated written informed consent to participate in the study in accordance with local regulations
  • Affiliated to a Health Insurance
Exclusion Criteria
  • Brain MRI abnormalities with a Loes score > 12 or with gadolinium enhancement
  • Any progressive neurological disease other than AMN
  • Impossibility to perform the walk tests and the TUG test
  • Patients with uncontrolled hepatic disorder, renal or cardiovascular disease, or any progressive malignancy
  • Any new medication for AMN including Fampridine initiated less than 1 month prior to inclusion
  • Contra-indications for MRI procedure such as subjects with paramagnetic materials in the body, such as aneurysm clips, pacemakers, intraocular metal or cochlear implants.
  • Inclusion in another therapeutic clinical trial for ALD
  • Not easily contactable by the investigator in case of emergency or not capable to call the investigator

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
MD1003MD1003 100 mg capsuleMD1003 100mg capsules, 1 capsule tid for 24 months
PlaceboPlaceboPlacebo capsule, 1 capsule tid for 12 months, then switch to MD1003 100mg capsule, 1 capsule tid for 12 months
PlaceboMD1003 100 mg capsulePlacebo capsule, 1 capsule tid for 12 months, then switch to MD1003 100mg capsule, 1 capsule tid for 12 months
Primary Outcome Measures
NameTimeMethod
Mean change of 2 minutes walking test (2MWT) between Months 12 and baselineBaseline and 12 Months
Secondary Outcome Measures
NameTimeMethod
Proportion of patients with improved TW25 (time to walk 25 feet) of at least 20%Baseline, 9 months, 12 months

at Months 9 and Months 12 compared to the best value among screening and baseline

Timed up and Go test (TUG)12 Months
Euroqol EQ-5D questionnaire12 months

Quality of Life questionnaire

Mean Change in TW25 (time to walk 25 feet)Baseline and 12 months
Qualiveen Questionnaire12 Months

Qualiveen to evaluate urinary function

Proportion of patients with improved 2-Minutes-Walk-Tests (2MWT) of at least 20%Baseline, 9 months, 12 months

at Months 9 and Months 12 compared to the best value among screening and baseline.

Trial Locations

Locations (4)

Hospital Duran i Reynals / Bellvitge

🇪🇸

Barcelona, Spain

Groupe Hospitalier Pitié-Salpêtrière

🇫🇷

Paris, France

Hôpital Bicêtre

🇫🇷

Le Kremlin Bicêtre, France

MS-Ambulanz Fachkrankenhaus Hubertusburg

🇩🇪

Wermsdorf, Germany

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