CI

At a glance

ClinicalIndex Comparison Record
Phase 2Completed· 6 enrolled
Drug / intervention
Miglustat 100 milligrams (mg) Oral Capsuledrug
Likely dose
Miglustat 100 milligrams (mg) Oral Capsulefrom record
Structured eligibility isn't available for this trial yet — see the full criteria in the Eligibility tab below.

Standardized by ClinicalIndex from the ClinicalTrials.gov record · verify against the source.

Search/NCT05174039
NCT05174039Phase 2Completed

An Open-label Safety, Pharmacokinetic, and Efficacy Study of the Combination of Miglustat for the Treatment of CLN3 Disease in Patients 17 Years of Age and Older

Beyond Batten Disease Foundation·interventional·Posted Dec 30, 2021·Updated Sep 9, 2025

In Brief

A Phase 2 clinical trial evaluating Miglustat 100 milligrams (mg) Oral Capsule for Batten Disease. Completed, enrolled 6 participants across 1 site.

Detailed Summary

This is an open label study in approximately 6 subjects in 2 centers to assess the safety, PK, and efficacy of the maximum tolerable dose (MTD) of oral miglustat (100 mg once daily \[QD\] to 200 mg 3 times daily \[TID\]) in subjects ≥ 17 years of age with CLN3 disease over a period of 104 weeks.

Study Details

Study Typeinterventional
Allocation--
Masking--
Primary Purpose--
ConditionsBatten Disease
CountriesUnited States
CollaboratorsTheranexus

Timeline

Phase 2CompletedFinished
20222023202420252026
First PostedDec 30, 2021
Enrollment StartMar 10, 2022
Primary CompletionMay 30, 2024
TodayJul 2, 2026
Enrollment to primary: 2.2 yearsPosted 4.5 years ago

Interventions

Miglustat 100 milligrams (mg) Oral Capsuledrug

Subjects will initiate miglustat at Week 1 and dosing will be escalated until 600mg/d. If a subject has not reached the maximum dose (600 mg/d) by Week 8, the Week 8 dose will be subject's MTD.