Structural and Functional Changes in Neuronal Intranuclear Inclusion Disease(NIID)

Structural and Functional Changes in Neuronal Intranuclear Inclusion Disease: A Pioneer Multi-modal 7T MRI Observational Study.

To discover the microstructure, macrostructure and functional changes with 7T multi-modal magnetic resonance imaging of Neuronal Intranuclear Inclusion Disease, and to explore new biomarkers for evaluating the severity and the progression of Neuronal Intranuclear Inclusion Disease.

Study Overview

• Status: Unknown
• Conditions: Neuronal Intranuclear Inclusion Disease
• Intervention / Treatment: Other

• Study Type: Observational
• Enrollment (Anticipated): 30

Contacts and Locations

Study Locations

• China
  • Zhejiang
    • Hangzhou, Zhejiang, China, 310003
      • Recruiting
      • Siemens 7T MR
      • Contact: Ming Xiong

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 14 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: N/A
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Subjects enrolled in the study shall be selected from NIID patients who are diagnosed with skin biopsy and gene detection. The Investigator is responsible for screening potential subjects and selecting those who meet the eligibility criteria for the study as described.

Description

Inclusion Criteria:

1. Patients diagnosed with NIID
2. Older than 18 years;
3. Sign the informed consent form.

Exclusion Criteria:

1. The informed consent is withdrawn by the subject;
2. Contraindications or intolerance to MRI: metallic foreign bodies, instability of vital signs, epilepsy, claustrophobia, etc.

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort
NIID; NIID patients
HC; healthy control

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
fractional anisotropy (FA)	Parameters to describe integrity of white matter in diffusion MR Imaging	baseline
mean diffusivity (MD)	Parameters to describe integrity of white matter in diffusion MR Imaging	baseline
Intracellular volume fraction(icvf)	Parameters to describe integrity of white matter in diffusion MR Imaging	baseline
orientation dispersion index (ODI)	Parameters to describe integrity of white matter in diffusion MR Imaging	baseline
fiber number(fiber pass through the lesions)	Parameters to describe integrity of white matter in diffusion MR Imaging	baseline
fractional anisotropy (FA)	Parameters to describe integrity of white matter in diffusion MR Imaging	6 months
mean diffusivity (MD)	Parameters to describe integrity of white matter in diffusion MR Imaging	6 months
Intracellular volume fraction(icvf)	Parameters to describe integrity of white matter in diffusion MR Imaging	6 months
orientation dispersion index (ODI)	Parameters to describe integrity of white matter in diffusion MR Imaging	6 months
fiber number(fiber pass through the lesions)	Parameters to describe integrity of white matter in diffusion MR Imaging	6 months
short-range and long-range functional connectivity around the lesions at rest	Functional network connectivity impairments in patients	baseline
short-range and long-range functional connectivity around the lesions at rest	Functional network connectivity impairments in patients	6 months

Collaborators and Investigators

• Sponsor: First Affiliated Hospital of Zhejiang University
• Collaborators: Zhejiang University Interdisciplinary Institute of Neuroscience and Technology
• Investigators: Principal Investigator: Ruiliang Bai, Doctor, Zhejiang University Interdisciplinary Institute of Neuroscience and Technology (ZIINT); Principal Investigator: Hui Liang, Doctor, Zhejiang University

Study record dates

Study Major Dates

• Study Start (Actual): July 31, 2018
• Primary Completion (Anticipated): December 31, 2020
• Study Completion (Anticipated): December 31, 2020

Study Registration Dates

• First Submitted: July 31, 2019
• First Submitted That Met QC Criteria: August 11, 2019
• First Posted (Actual): August 14, 2019

Study Record Updates

• Last Update Posted (Actual): August 14, 2019
• Last Update Submitted That Met QC Criteria: August 11, 2019
• Last Verified: June 1, 2019

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Nervous System Diseases; Neurodegenerative Diseases
• Other Study ID Numbers: 7TMRINIID

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No