RecruitingPhase 2NCT07432490

A Phase II Study With Exploratory Outcomes of Fucose Supplementation in GLUT1 Deficiency Syndrome

A Phase II Randomized, Double-blind, Placebo-controlled, Cross-over Study With Exploratory Outcomes of Fucose Supplementation in GLUT1 Deficiency Syndrome


Sponsor

Oregon Health and Science University

Enrollment

16 participants

Start Date

May 5, 2026

Study Type

INTERVENTIONAL

Conditions

Summary

This is a single-center, randomized, double-blind, placebo-controlled, cross-over study to evaluate the efficacy and safety of L-fucose supplementation in subjects with GLUT1 deficiency syndrome (GLUT1DS).


Eligibility

Min Age: 18 Years

Inclusion Criteria3

  • Age ≥ 18 years
  • Confirmed diagnosis of GLUT1DS, including at least 2 out of the following 3: molecular genetic testing showing a pathogenic or likely pathogenic variant in SLC2A1; documented hypoglycorrhachia with a CSF:blood glucose ratio ≤ 0.6; clinical features consistent with GLUT1DS (epilepsy, movement disorders, ataxia, intellectual disability, dysarthria)
  • Presence of ataxia

Exclusion Criteria11

  • Inability to swallow liquids
  • Change in neurological medications (either medication itself or medication dosages) in the past 90 days
  • Use of fucose- or mannose-containing supplements within one year of enrollment
  • Presence of hepatic, renal, hematological, or concomitant metabolic disorders, as assessed by the presence of a previous diagnosis of such disorders (for instance, chronic kidney disease, liver cirrhosis, diabetes mellitus) or by the following laboratory values, which will be considered if obtained clinically up to 90 days before enrollment (if this is not available, laboratory tests will be obtained prior to first study visit):
  • Any degree of hepatic impairment based on the Child-Pugh classification
  • eGFR (as measured by serum creatinine or cystatin C) < 60 mg/min/1.73m2
  • Hemoglobin A1c > 6.5%
  • Hemoglobin level below the lower limit of normal (LLN) for sex and age
  • Platelet counts below the LLN for sex and age
  • Subjects who are pregnant, breastfeeding, or planning to become pregnant within one year of enrollment
  • Enrollment in an investigational new drug trial for G1DS within one year of enrollment

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Interventions

DRUGL-fucose

L-fucose will be administered as 500 mg/kg to a maximum of 10 g three times per day by mouth.

OTHERPlacebo

Placebo will be composed of micro-cellulose powder with a small amount of Stevia for taste mimicking, to be taken at 500 mg/kg for a maximum of 10 g three times per day by mouth.


Locations(1)

Oregon Health and Science University

Portland, Oregon, United States

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NCT07432490