Abstract Details

Title Evaluation of Adult Biotinidase Activity in Patients with Idiopathic Inflammatory Demyelinating Diseases

Topic Multiple Sclerosis

Presentation(s) P1 - Poster Session 1 (9:00 AM-5:00 PM)

Poster/Presentation
Number 318

Objective Delayed-onset biotinidase deficiency can be presented with optic neuropathy and myelitis.

Background We aimed to evaluate the adult biotinidase activity values in idiopathic central nervous system inflammatory demyelinating diseases.

Design/Methods 187 patients (134 female, 53 male) with the diagnosis of multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), radiologically isolated syndrome (RIS) or clinically isolated syndrome (CIS) were recruited for the study. Patients' demographic, clinical and laboratory data were noted. Serum biotinidase activity were determined by enzymatic analysis cross-sectionally.

Results The mean biotinidase activity of whole group was 8.61 nmol/mL/min and 128 of patients (%68.4) had lower biotinidase activity (mean 7.63 nmol/mL/min) than average value of laboratory (9.61 nmol/mL/min). The patients with lower biotinidase activity were not more prone to develop optic neuritis, myelitis or both (p=0.508, p=0.203 and p=0.269, respectively). Females had significantly lower biotinidase activity than males (8.34 and 9.18, respectively, p=0.005). The mean biotinidase activity of patients with MS (n=137) was 8.64, NMOSD (n=19) was 9.19, RIS (n=13) was 8.05 and CIS (n=18) was 8.24 nmol/mL/min. 65.7% of MS patients; 57.9% of NMOSD patients; 92.3% of RIS patients and 83.3% of CIS patients had lower biotidinase activity than average value. There was no statistically significant difference between groups (p=0.081). When we compared patients with and without a history of optic neuritis or myelitis, there was no significant difference (p=0.480 and p=0.614, respectively).We found no correlation between biotinidase activity and expanded disability status scale (EDSS) scores of MS patients (r=0.029).

Conclusions

This study suggested that patients with inflammatory demyelinating diseases could have lower biotinidase activity than healthy people. Determination of biotinidase activity and appropriate supplementation of biotine could improve clinical outcomes of these patients. Further clinical studies are needed for this field.

Authors/Disclosures
Aysegul Akkan Suzan (MINISTRY OF HEALTH, ISTANBUL KARTAL DR. LUTFI KIRDAR CITY HOSPITAL) PRESENTER	Aysegul Akkan Suzan has nothing to disclose.
Ahmet Kasim Kilic	Ahmet Kasim Kilic has nothing to disclose.

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