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No clinical improvement in patients with low biotinidase activity levels in a cohort of progressive multiple sclerosis patients treated with high dose biotin
Author(s): ,
C. Dulau
Affiliations:
Hopital Pellegrin, Bordeaux, France
,
B. Brochet
Affiliations:
Hopital Pellegrin, Bordeaux, France
,
S. Mesli
Affiliations:
Hopital Pellegrin, Bordeaux, France
,
J.-S. Liegey
Affiliations:
Hopital Pellegrin, Bordeaux, France
,
M. Deloire
Affiliations:
Hopital Pellegrin, Bordeaux, France
,
A. Moroso
Affiliations:
Hopital Pellegrin, Bordeaux, France
,
J.-C. Ouallet
Affiliations:
Hopital Pellegrin, Bordeaux, France
,
A. Ruet
Affiliations:
Hopital Pellegrin, Bordeaux, France
I. Redonnet-Vernhet
Affiliations:
Hopital Pellegrin, Bordeaux, France
ECTRIMS Online Library. Brochet B. Oct 12, 2018; 229064
Bruno Brochet
Bruno Brochet
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Abstract
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Abstract: P1224

Type: Poster Sessions

Abstract Category: Therapy - Neuroprotection and Repair

Background: Recently a letter to the editor (Wolf, 2018) suggested that patients with multiple sclerosis (MS) improved by high dose biotin might have biotinidase deficiency (BD). BD occurs after birth and is a severe autosomal recessively inherited disorder that can lead to death without pharmacological biotin substitution. BD can reveal at late-onset or be asymptomatic when activity of biotin-related enzymes remains at residual levels. Partial BD represents a biochemical feature related with heterozygote mutation.
Objectives: To estimate for the first time biotinidase activity levels in a cohort of progressive multiple sclerosis patients treated by high dose biotin and report any relation with clinical evolution.
Methods: We conducted a prospective monocentric observational study of multiple sclerosis patients treated with high dose biotin (300 mg/day) from September 2015 to February 2018. Serum biotinidase activity levels was quantified before initiation of the treatment. Prospective follow-up concerned clinical data.
Results: Seventy progressive MS patients treated with high dose biotin were included in our study. Normal value in our biochemical laboratory for biotinidase activity level was greater than 90 nkat/l.Three patients had low levels of biotinidase activity. They met MS diagnostic criteria. Patient 1 was a man, aged 70, with primary progressive MS for 7 years, EDSS 6. Patient 2 was a woman, aged 48, with a secondary progressive MS for 22 years, EDSS 4. Patient 3 was a woman, aged 55, with a secondary progressive MS for 26 years, EDSS 5. Patients 1, 2 and 3 had low levels of biotinidase activity, respectively 50; 75,8 and 85 nkat/l.Following well-conducted treatment 12 months after initiation, patient 1 and 3 felt no clinical improvement. Patient 2 had a worsening of 1,5 of her EDSS at month 12. High dose biotin was stopped at month 12 for all 3 patients.
Conclusion: In our study of 70 MS patients, 4,3% presented low-levels of biotinidase activity. This ratio is in line with heterozygote mutation of BD estimated at about 1/120. Correlations with clinical parameters were impossible to analyse because of the small size of our partial BD group. Nevertheless, these 3 patients did not have any clinical amelioration under treatment. Multicentric study with quantification of biotinidase activity levels and prospective clinical evaluation could investigate more precisely this biological marker as a factor of better response of high dose biotin treatment.
Disclosure: Dr. Dulau received honoraria for speaking at scientific symposia by Biogen, Genzyme, Novartis and Teva.
Dr. Ruet or her institution received honoraria for consulting, speaking at scientific symposia, serving in advisory board or research support from Biogen Idec, Medday, Merck Serono, Novartis, Roche, Sanofi-Genzyme, Teva (all with approval by general director of the Hospital of Bordeaux and the University of Bordeaux).Pr. Brochet or its institution received honoraria for consulting, speaking at scientific symposia, serving in advisory board or research support from Actelion, Biogen Idec, Merck Serono, Sanofi-Genzyme, Bayer, Medday, Roche, Teva, Novartis (all with approval by general director of the Hospital of Bordeaux).
Dr. Ouallet reportedpersonal fees from Biogen, Roche, Genzyme; grants, personal fees and non-financial support from Novartis and Merck, outside the submitted work.
Drs. Redonnet, Mesli, Liegey, Deloire, reported no disclosures.
I. Redonnet-Vernhet and A. Ruet share co-seniorship of this study.

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