Characterization of Inclusion Body Myopathy Associated With Paget's Disease of Bone and Frontotemporal Dementia (IBMPFD)

Description

The investigators are researching families with inherited inclusion body myopathy (IBM) and/or Paget disease of bone (PDB) and/or dementia (FTD) which is also called IBMPFD. IBMPFD is caused by mutations in the VCP gene. Our main goal is to understand how changes in the VCP gene cause the muscle, bone and cognitive problems associated with the disease. The investigators are collecting biological specimen such as blood and urine samples, family and medical histories, questionnaire data of patients with a personal or family history of VCP associated disease. Participants do not need to have all symptoms listed above in order to qualify. A select group of participants may be invited to travel to University of California, Irvine for a two day program of local procedures such as an MRI and bone scan. Samples are coded to maintain confidentiality. Travel is not necessary except for families invited for additional testing.

Conditions

Inclusion Body Myopathy With Early-onset Paget Disease and Frontotemporal Dementia, Paget Disease of Bone, Frontotemporal Dementia, Myopathy

Study Overview

Study Details

Study overview

The investigators are researching families with inherited inclusion body myopathy (IBM) and/or Paget disease of bone (PDB) and/or dementia (FTD) which is also called IBMPFD. IBMPFD is caused by mutations in the VCP gene. Our main goal is to understand how changes in the VCP gene cause the muscle, bone and cognitive problems associated with the disease. The investigators are collecting biological specimen such as blood and urine samples, family and medical histories, questionnaire data of patients with a personal or family history of VCP associated disease. Participants do not need to have all symptoms listed above in order to qualify. A select group of participants may be invited to travel to University of California, Irvine for a two day program of local procedures such as an MRI and bone scan. Samples are coded to maintain confidentiality. Travel is not necessary except for families invited for additional testing.

Characterization of Familial Myopathy and Paget Disease of Bone

Characterization of Inclusion Body Myopathy Associated With Paget's Disease of Bone and Frontotemporal Dementia (IBMPFD)

Condition
Inclusion Body Myopathy With Early-onset Paget Disease and Frontotemporal Dementia
Intervention / Treatment

-

Contacts and Locations

Irvine

University of California, Irvine, Irvine, California, United States, 92697-1385

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

For general information about clinical research, read Learn About Studies.

Eligibility Criteria

  • * Inclusion criteria include all individuals with a combination of medical problems including muscle and bone disease and their family members. Because historically VCP related muscle disease has been erroneously diagnosed with the following diagnoses, therefore if these patients also have a personal or family history of bone disease they will be considered eligible for the study:
  • * Limb Girdle Muscular Dystrophy
  • * Myopathy
  • * Inclusion body myopathy
  • * FSH (Facioscapular muscular dystrophy) without the mutation
  • * Scapuloperoneal muscular dystrophy
  • * Amyotrophic Lateral Sclerosis
  • * Non specific muscular dystrophy
  • * Bone disorders including:
  • * Paget disease of bone
  • * Fibrous dysplasia
  • * Diaphyseal medullary stenosis with malignant fibrous histiocytoma (DMS-MFH)
  • * Non-specific bone disease
  • * Subjects must to 18 years or older
  • * Subjects must to able to give consent
  • * Adult family members or spouses over the age of 18 of the affected individuals
  • * Under the age of 18.

Ages Eligible for Study

18 Years to

Sexes Eligible for Study

ALL

Accepts Healthy Volunteers

Yes

Collaborators and Investigators

University of California, Irvine,

Virginia Kimonis, MD, PRINCIPAL_INVESTIGATOR, University of California, Irvine

Study Record Dates

2026-12