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NCT05750979: LBSL

Quantifying Disease Progression in LBSL

Status unknown Last updated 2 March 2023
What this trial tests

trial in LBSL in 20 participants. Status unknown.

Timeline
11 March 2021
Primary endpoint
31 December 2025
31 December 2025

Quick facts

Lead sponsorM. Engelen
StatusStatus unknown
Study typeOBSERVATIONAL
Enrollment20
Start date11 March 2021
Primary completion31 December 2025
Estimated completion31 December 2025
Sites1 location across Netherlands

Conditions studied

Sponsor

M. Engelen

Who can join

16 and older, any sex, with LBSL or Leukoencephalopathies. Patients with the condition only — healthy volunteers not accepted.

Sponsor's own description

Leukoencephalopathy with brain stem involvement and lactate elevation (LBSL) is a genetic disorder caused by biallelic mutations in the DARS2 gene that encodes mitochondrial aspartyl tRNA synthase.(1, 2) It is characterized by typical abnormalities on MRI of the brain and spinal cord.(3) Clinically, the disorder is heterogeneous and can present in the neonatal period, later in childhood or even in adults.(3) In general it can be stated that the earlier presentations are characterized by rapid progression leading to severe disability and death. Presentation at a later age is typically characterized by a more benign disease course, although considerable disability is common. Clinically, the disease presents as a slowly progressive myelopathy with mainly involvement of the corticospinal tracts and the dorsal columns. Although the natural history has been studied in large cohorts, the rate of progression has not been systematically studied with clinimetric outcome scales or potential surrogate outcomes for spinal cord disease.

Publications & conference data

No peer-reviewed publications indexed yet for this trial.

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Data sources for this page

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