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CompletedNCT02164253SAFEFAIRALSUpdated Feb 20, 2026

Focal Accumulation of Iron in Cerebral Regions in Early ALS (Amyotrophic Lateral Sclerosis) Patients

A Phase 2 interventional study of Deferiprone in ALS (Amyotrophic Lateral Sclerosis) and Iron Overload, sponsored by University Hospital, Lille. Completed at 1 site in France. Open to participants aged 18 Years to 85 Years. Per ClinicalTrials.gov, last updated 2026-02-20.

Sponsored by University Hospital, Lille · Phase 2, Interventional, and Supportive care

Phase
Phase 2
Study type
Interventional
Enrollment
23
Allocation
Not applicable
Ages
18 Years to 85 Years
Sex
All
01

Study summary

The FAIR-ALS study is to investigate the safety and efficacy of a scavenger treatment of iron deferiprone, which would reduce the brain iron to limit the development of amyotrophic lateral sclerosis.

It has been shown an excess of iron in the central nervous system carrying a sporadic ALS patients. Iron overload associated with a loss of motor neurons may explain the signs of the disease (atrophy).

The investigators discuss the hypothesis that reducing excess iron, the investigators can reduce the loss of neurons and thus the progression of signs of the disease.

Read the detailed description

At the end of the study, it will propose to continue the usual quarterly patient follow up, as recommended by the French ALS centers.

Deferiprone can be administered as part of a compassionate use, for patients who want it and who do not have hypoxemia.

We therefore plan a treatment period compassionate relatively short and less than 3 years.

02

Conditions studied

  • ALS (Amyotrophic Lateral Sclerosis)
  • Iron Overload
03

Who can participate

Ages eligible
18 Years to 85 Years
Sexes eligible
All
Accepts healthy volunteers
No

Inclusion criteria

  • Amyotrophic lateral sclerosis defined in accordance to the El Escorial criteria (possible, probable or defined)
  • 18 to 85 years old patient, male or female
  • Patient with social security cover

Exclusion criteria

Exclusion Criteria:

  • Achieved respiratory defined by a FVC \<70%
  • Evolution of more than 24 months
  • Demented subject
  • Severe malnutrition
  • Patients with treatment potentially at risk of agranulocytosis and neutropenia
  • Patients with a history of agranulocytosis or iatrogenic under haematological disease
  • Incapable of giving consent
  • Indication against MRI
  • Indication against lumbar puncture
  • Patient refused lumbar puncture
  • Hypersensitivity to iron chelators
  • Concomitant treatment with antacids containing aluminum
  • Presence of another serious illness to life-threatening or disabling cons to the use of the treatment mixture of oxygen and nitrous oxide equally
04

Study design

Phase
Phase 2
Primary purpose
Supportive care
Allocation
Not applicable
Intervention model
Single group
Masking
None (open label)
Enrollment
23 participants (actual)

Study arms

  • Experimental
    Deferiprone

    Deferiprone, 25 to 30 mg/kg per day, oral use

    Drug: Deferiprone

Interventions

  • DrugDeferiprone

    30 mg/kg per day, oral use

    Also known as: Ferriprox

05

What researchers measure

Primary outcomes

  1. Evolution of Amyotrophic Lateral Sclerosis Functional Rating Scale

    Time frame: V3, V6, V9, V12, V15

Secondary outcomes

  1. Comparison of the progression of ALSFRS-R (Amyotrophic Lateral Sclerosis Functional Rating Scale ) score for 3 months without treatment (V0 to V3) in the period of the first three months under treatment (V3 to V6).

    Time frame: SCREENING, V0, V3, V6

  2. The proportion of patients who become non-self-sufficient after 12 months with the appearance of a sub scores ALSFRS-R less than or equal to 2 on swallowing, cut food using utensils or walk.

    Time frame: SCREENING, V0, V3, V6, V9, V12, V15

  3. Number of patients with anemia at 12 months defined by a hemoglobin / dL Hb <12 g

    Time frame: V3, V6, V9, V12, V15

  4. Number of serious and non-serious adverse events

    Time frame: SCREENING, V0, V3, V6, V9, V12, V15

  5. Progression of respiratory vital capacity tests

    Time frame: SCREENING, V0, V3, V6, V9, V12, V15

  6. Values of R2 * in MRI

    Time frame: V3, V6, V19

  7. Oxidative stress markers analyzed blindly in blood and cerebrospinal fluid

    Time frame: V3, V9

  8. No alteration of energy metabolism in aerobic and anaerobic blood and cerebrospinal fluid

    Time frame: V3, V9

06

Study locations

1 site
  • Hôpital Roger Salengro, CHRU de Lille
    Lille, 59000, France
07

References and documents

Publications

  • Bensimon G, Lacomblez L, Meininger V. A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N Engl J Med. 1994 Mar 3;330(9):585-91. doi: 10.1056/NEJM199403033300901. PubMed 8302340 ↗
  • Boddaert N, Le Quan Sang KH, Rotig A, Leroy-Willig A, Gallet S, Brunelle F, Sidi D, Thalabard JC, Munnich A, Cabantchik ZI. Selective iron chelation in Friedreich ataxia: biologic and clinical implications. Blood. 2007 Jul 1;110(1):401-8. doi: 10.1182/blood-2006-12-065433. Epub 2007 Mar 22. PubMed 17379741 ↗
  • Danzeisen R, Achsel T, Bederke U, Cozzolino M, Crosio C, Ferri A, Frenzel M, Gralla EB, Huber L, Ludolph A, Nencini M, Rotilio G, Valentine JS, Carri MT. Superoxide dismutase 1 modulates expression of transferrin receptor. J Biol Inorg Chem. 2006 Jun;11(4):489-98. doi: 10.1007/s00775-006-0099-4. Epub 2006 Apr 26. PubMed 16680451 ↗
  • Felice KJ. A longitudinal study comparing thenar motor unit number estimates to other quantitative tests in patients with amyotrophic lateral sclerosis. Muscle Nerve. 1997 Feb;20(2):179-85. doi: 10.1002/(sici)1097-4598(199702)20:23.0.co;2-9. PubMed 9040656 ↗
  • Goodall EF, Greenway MJ, van Marion I, Carroll CB, Hardiman O, Morrison KE. Association of the H63D polymorphism in the hemochromatosis gene with sporadic ALS. Neurology. 2005 Sep 27;65(6):934-7. doi: 10.1212/01.wnl.0000176032.94434.d4. PubMed 16186539 ↗
  • Kwiatkowski A, Ryckewaert G, Jissendi Tchofo P, Moreau C, Vuillaume I, Chinnery PF, Destee A, Defebvre L, Devos D. Long-term improvement under deferiprone in a case of neurodegeneration with brain iron accumulation. Parkinsonism Relat Disord. 2012 Jan;18(1):110-2. doi: 10.1016/j.parkreldis.2011.06.024. Epub 2011 Aug 6. No abstract available. PubMed 21821461 ↗
  • Kong J, Xu Z. Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1. J Neurosci. 1998 May 1;18(9):3241-50. doi: 10.1523/JNEUROSCI.18-09-03241.1998. PubMed 9547233 ↗
  • Langkammer C, Enzinger C, Quasthoff S, Grafenauer P, Soellinger M, Fazekas F, Ropele S. Mapping of iron deposition in conjunction with assessment of nerve fiber tract integrity in amyotrophic lateral sclerosis. J Magn Reson Imaging. 2010 Jun;31(6):1339-45. doi: 10.1002/jmri.22185. PubMed 20512885 ↗
  • Lin J, Diamanduros A, Chowdhury SA, Scelsa S, Latov N, Sadiq SA. Specific electron transport chain abnormalities in amyotrophic lateral sclerosis. J Neurol. 2009 May;256(5):774-82. doi: 10.1007/s00415-009-5015-8. Epub 2009 Feb 25. PubMed 19240958 ↗
  • Miller R, Bradley W, Cudkowicz M, Hubble J, Meininger V, Mitsumoto H, Moore D, Pohlmann H, Sauer D, Silani V, Strong M, Swash M, Vernotica E; TCH346 Study Group. Phase II/III randomized trial of TCH346 in patients with ALS. Neurology. 2007 Aug 21;69(8):776-84. doi: 10.1212/01.wnl.0000269676.07319.09. PubMed 17709710 ↗
  • Moreau C, Gosset P, Kluza J, Brunaud-Danel V, Lassalle P, Marchetti P, Defebvre L, Destee A, Devos D. Deregulation of the hypoxia inducible factor-1alpha pathway in monocytes from sporadic amyotrophic lateral sclerosis patients. Neuroscience. 2011 Jan 13;172:110-7. doi: 10.1016/j.neuroscience.2010.10.040. Epub 2010 Oct 25. PubMed 20977930 ↗
  • Moreau C, Devos D, Gosset P, Brunaud-Danel V, Tonnel AB, Lassalle P, Defebvre L, Destee A. [Mechanisms of deregulated response to hypoxia in sporadic amyotrophic lateral sclerosis: a clinical study]. Rev Neurol (Paris). 2010 Mar;166(3):279-83. doi: 10.1016/j.neurol.2009.05.018. Epub 2009 Aug 5. French. PubMed 19660777 ↗
  • Moreau C, Gosset P, Brunaud-Danel V, Lassalle P, Degonne B, Destee A, Defebvre L, Devos D. CSF profiles of angiogenic and inflammatory factors depend on the respiratory status of ALS patients. Amyotroph Lateral Scler. 2009 Jun;10(3):175-81. doi: 10.1080/17482960802651725. PubMed 19177252 ↗
  • Moreau C, Devos D, Brunaud-Danel V, Defebvre L, Perez T, Destee A, Tonnel AB, Lassalle P, Just N. Paradoxical response of VEGF expression to hypoxia in CSF of patients with ALS. J Neurol Neurosurg Psychiatry. 2006 Feb;77(2):255-7. doi: 10.1136/jnnp.2005.070904. PubMed 16421133 ↗
  • Moreau C, Devos D, Brunaud-Danel V, Defebvre L, Perez T, Destee A, Tonnel AB, Lassalle P, Just N. Elevated IL-6 and TNF-alpha levels in patients with ALS: inflammation or hypoxia? Neurology. 2005 Dec 27;65(12):1958-60. doi: 10.1212/01.wnl.0000188907.97339.76. PubMed 16380619 ↗
  • Noto Y, Misawa S, Kanai K, Shibuya K, Isose S, Nasu S, Sekiguchi Y, Fujimaki Y, Nakagawa M, Kuwabara S. Awaji ALS criteria increase the diagnostic sensitivity in patients with bulbar onset. Clin Neurophysiol. 2012 Feb;123(2):382-5. doi: 10.1016/j.clinph.2011.05.030. Epub 2011 Jul 20. PubMed 21764635 ↗
  • Schrooten M, Smetcoren C, Robberecht W, Van Damme P. Benefit of the Awaji diagnostic algorithm for amyotrophic lateral sclerosis: a prospective study. Ann Neurol. 2011 Jul;70(1):79-83. doi: 10.1002/ana.22380. Epub 2011 Mar 17. PubMed 21437935 ↗
  • Shefner JM, Cudkowicz ME, Zhang H, Schoenfeld D, Jillapalli D; Northeast ALS Consortium. Revised statistical motor unit number estimation in the Celecoxib/ALS trial. Muscle Nerve. 2007 Feb;35(2):228-34. doi: 10.1002/mus.20671. PubMed 17058270 ↗
  • Shefner JM, Watson ML, Simionescu L, Caress JB, Burns TM, Maragakis NJ, Benatar M, David WS, Sharma KR, Rutkove SB. Multipoint incremental motor unit number estimation as an outcome measure in ALS. Neurology. 2011 Jul 19;77(3):235-41. doi: 10.1212/WNL.0b013e318225aabf. Epub 2011 Jun 15. PubMed 21676915 ↗
  • Yuen EC, Olney RK. Longitudinal study of fiber density and motor unit number estimate in patients with amyotrophic lateral sclerosis. Neurology. 1997 Aug;49(2):573-8. doi: 10.1212/wnl.49.2.573. PubMed 9270599 ↗
  • Zigmond AS, Snaith RP. The hospital anxiety and depression scale. Acta Psychiatr Scand. 1983 Jun;67(6):361-70. doi: 10.1111/j.1600-0447.1983.tb09716.x. PubMed 6880820 ↗
  • Moreau C, Danel V, Devedjian JC, Grolez G, Timmerman K, Laloux C, Petrault M, Gouel F, Jonneaux A, Dutheil M, Lachaud C, Lopes R, Kuchcinski G, Auger F, Kyheng M, Duhamel A, Perez T, Pradat PF, Blasco H, Veyrat-Durebex C, Corcia P, Oeckl P, Otto M, Dupuis L, Garcon G, Defebvre L, Cabantchik ZI, Duce J, Bordet R, Devos D. Could Conservative Iron Chelation Lead to Neuroprotection in Amyotrophic Lateral Sclerosis? Antioxid Redox Signal. 2018 Sep 10;29(8):742-748. doi: 10.1089/ars.2017.7493. Epub 2018 Feb 8. PubMed 29287521 ↗
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Registry details

Key details

Study ID
NCT02164253
Lead sponsor
University Hospital, Lille
Collaborators
ApoPharma
Responsible party
Sponsor
First posted
Jun 16, 2014
Start date
Sep 2013
Primary completion
Dec 2016
Completion
Dec 2016
Last update
Feb 20, 2026

Study contacts

Caroline Moreau, MD
principal investigator · UH Lille

Oversight

Data monitoring committee
No
View the source record on ClinicalTrials.gov ↗

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