MRI and Muscle Involvement in Patients With Mutations in GMPPB

NCT ID: NCT02635321

Last Updated: 2016-04-06

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

4 participants

Study Classification

OBSERVATIONAL

Study Start Date

2015-11-30

Study Completion Date

2016-04-30

Brief Summary

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Limb girdle muscular dystrophies (LGMD) are a very heterogeneous group of muscle disorders characterized by muscle weakness and atrophy of the proximal muscles of the shoulder and pelvic girdles. LGMD is classified based on its inheritance pattern and genetic cause into more than 31 different types.

A new type - type 2T has been found. The genetic cause of type 2T is mutations in Guanosine Diphosphate (GDP)-mannose pyrophosphorylase B (GMPPB). Mutations in GMPPB can also cause Congenital muscular dystrophies (CMD). Only 41 patients with mutations in GMPPB has been reported.

In this study, the investigators examine five new cases with the LGMD phenotype. The primary aim is to examine the muscle involvement using MRI.

Detailed Description

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Conditions

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Limb-girdle Muscular Dystrophy

Study Design

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Observational Model Type

CASE_ONLY

Study Time Perspective

CROSS_SECTIONAL

Study Groups

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Patients with LGMD 2T

Four patients over 18 years old with genetically verified LGMD 2T.

No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

* Persons with genetically verified mutations in GMPPB

Exclusion Criteria

* All contraindications for undergoing an MRI scan
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Rigshospitalet, Denmark

OTHER

Sponsor Role lead

Responsible Party

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Sofie Thurø Østergaard

Bachelor of Science

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Sofie T Østergaard, Bsc.

Role: PRINCIPAL_INVESTIGATOR

Copenhagen Neuromuscular Center, Department of Neurology, Rigshospitalet, Copenhagen University

Locations

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Copenhagen Neuromuscular Center

Copenhagen, Copenhagen, Denmark

Site Status

Countries

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Denmark

Other Identifiers

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STO-GMPPB

Identifier Type: -

Identifier Source: org_study_id

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