A Study of Memory, Thinking, and Brain Imaging in Adults With Histiocytosis - Full Text View

Purpose

The purpose of this study is to try to understand how histopcytosis can cause symptoms or problems in the brain. The tests being done in the study will look at memory and thinking as well as brain function via MRI scan.

Condition	Intervention
Histiocytosis	Behavioral: Trail Making Test, Parts A & B Behavioral: Brief Test of Attention Behavioral: Symbol Span Behavioral: Controlled Oral Word Association Test Behavioral: The Hopkins Verbal Learning Test-Revised Behavioral: Brief Visuospatial Memory Test-Revised Behavioral: Hospital Anxiety and Depression Scale Behavioral: McGill Quality of Life Scale Diagnostic Test: MR Brain Imaging Diagnostic Test: Resting state functional MRI

Condition

Intervention

Histiocytosis

Behavioral: Trail Making Test, Parts A & B Behavioral: Brief Test of Attention Behavioral: Symbol Span Behavioral: Controlled Oral Word Association Test Behavioral: The Hopkins Verbal Learning Test-Revised Behavioral: Brief Visuospatial Memory Test-Revised Behavioral: Hospital Anxiety and Depression Scale Behavioral: McGill Quality of Life Scale Diagnostic Test: MR Brain Imaging Diagnostic Test: Resting state functional MRI


Study Type:	Interventional
Study Design:	Intervention Model: Single Group Assignment Masking: No masking Primary Purpose: Other
Official Title:	A Clinical, Structural, and Functional Neuroimaging Study of Cognition in Adults With Erdheim-Chester Disease, Langerhans Cell Histiocytosis, Rosai-Dorfman Disease, and Other Histiocytoses

Resource links provided by NLM:

Genetics Home Reference related topics: Erdheim-Chester disease Langerhans cell histiocytosis

MedlinePlus related topics: Memory

Genetic and Rare Diseases Information Center resources: Langerhans Cell Histiocytosis Lipogranulomatosis Erdheim-Chester Disease Rosai-Dorfman Disease

U.S. FDA Resources

Further study details as provided by Memorial Sloan Kettering Cancer Center:

Primary Outcome Measures:

neurocognitive function [ Time Frame: 1 year ]
Neurocognitive tests will yield raw scores that are converted to z-scores, normalized for participant's age, sex and educational level.The proportion of participants demonstrating cognitive impairment (as defined by 2 or more z-scores less than -1.5) will be summarized. Also, the proportion of participants with impairment (z < -1.5) for each cognitive test will be summarized. In this way the cognitive assessment yields a dichotomous outcome of impaired versus not impaired for each participant but also allow for more granular assessment of impairment in specific cognitive domains.
quality of life [ Time Frame: 1 year ]
questionnaires will yield raw scores (total score for HADS and McGill QOL, as well as anxiety and depression subscores for the HADS). QOL scores will be analyzed as raw scores. Cognitive test scores will be transformed into z-sores (based on score distributions from established normative samples with a mean of 0 and standard deviation of 1) to define the presence/severity of cognitive dysfunction. Scores will be normalized for age, sex and education.
comparing of grey matter volume [ Time Frame: 1 year ]
In a whole-brain analysis, first, cortical thickness will be compared between participants and controls and regions of statistically significant grey matter loss will be identified. This is done computionally with a whole-brain, vertex-by-vertex approach, as stated above. Also, as stated above (Section 7), statistical significance thresholding is at p<0.001, correcting for multiple comparisons using the False Discovery Rate (FDR) method and clustering thresholds, methods used in various high-throughput contexts, including MRI analysis.


Estimated Enrollment:	30
Actual Study Start Date:	April 7, 2017
Estimated Study Completion Date:	April 2020
Estimated Primary Completion Date:	April 2020 (Final data collection date for primary outcome measure)

Arms	Assigned Interventions
Experimental: Participants with a histiocytic disorder diagnosis Participants will have a histiocytic disorder as determined by a corroborating constellation of histopathology, clinical, and/or radiologic findings.	Behavioral: Trail Making Test, Parts A & B Part A is a timed measure of visual scanning and graphomotor speed. Part B is a timed measure of cognitive flexibility. Behavioral: Brief Test of Attention Assesses auditory working memory Other Name: BTA Behavioral: Symbol Span Assesses visual working memory Other Name: WMS-IV Behavioral: Controlled Oral Word Association Test The COWA timed test of phonemic verbal fluency. It requires the subject to generate as many words as possible, beginning with a given letter of the alphabet (i.e., F, A, and S). Other Name: COWA Behavioral: The Hopkins Verbal Learning Test-Revised HVLT-R is a test of verbal learning and memory. Scores obtained are the total number of words. Other Name: HVLT-R Behavioral: Brief Visuospatial Memory Test-Revised The BVMT-R is a test of visuospatial learning and memory. Other Name: BVMT-R Behavioral: Hospital Anxiety and Depression Scale The HADS is a brief assessment validated in many cancer populations. Other Name: HADS Behavioral: McGill Quality of Life Scale This is a questionnaire that is a validated 17-item QOL instrument validated for ill patients and in the particular context of the ancer. Other Name: McGill QOL Diagnostic Test: MR Brain Imaging Standard of care brain MR imaging includes high-resolution 3D T1-weighted images in the axial plane. The axial T1-weighted images will be isotropic with a typical matrix size of 256 x 256, 256 mm field-of-view (FOV) and 1 mm slice thickness covering the whole brain. Diagnostic Test: Resting state functional MRI Scanning will be performed on a 3 Tesla General Electric scanner (Optima 750W) with a GEM HNU 24-channel head coil. rsFMRI matching the FLAIR and T1 post-contrast images will be obtained. For resting state fMRI, T2*-weighted images will be acquired with a single-shot gradient echo echo-planar imaging (EPI) sequence in the axial orientation (TR = 2500 ms, TE = 30 ms, FA = 80°, slice thickness = 4 mm, FOV= 240mm2, matrix=64×64) covering whole brain. For the resting state fMRI portion of the scan, patients will be instructed by the technologist or research staff to leave eyes open, focus on looking at a crosshair, and not think about anything during the scan. Other Name: rsFMRI

Arms

Assigned Interventions

Experimental: Participants with a histiocytic disorder diagnosis

Participants will have a histiocytic disorder as determined by a corroborating constellation of histopathology, clinical, and/or radiologic findings.

Behavioral: Trail Making Test, Parts A & B

Part A is a timed measure of visual scanning and graphomotor speed. Part B is a timed measure of cognitive flexibility.

Behavioral: Brief Test of Attention

Assesses auditory working memory

Other Name: BTA

Behavioral: Symbol Span

Assesses visual working memory

Other Name: WMS-IV

Behavioral: Controlled Oral Word Association Test

The COWA timed test of phonemic verbal fluency. It requires the subject to generate as many words as possible, beginning with a given letter of the alphabet (i.e., F, A, and S).

Other Name: COWA

Behavioral: The Hopkins Verbal Learning Test-Revised

HVLT-R is a test of verbal learning and memory. Scores obtained are the total number of words.

Other Name: HVLT-R

Behavioral: Brief Visuospatial Memory Test-Revised

The BVMT-R is a test of visuospatial learning and memory.

Other Name: BVMT-R

Behavioral: Hospital Anxiety and Depression Scale

The HADS is a brief assessment validated in many cancer populations.

Other Name: HADS

Behavioral: McGill Quality of Life Scale

This is a questionnaire that is a validated 17-item QOL instrument validated for ill patients and in the particular context of the ancer.

Other Name: McGill QOL

Diagnostic Test: MR Brain Imaging

Standard of care brain MR imaging includes high-resolution 3D T1-weighted images in the axial plane. The axial T1-weighted images will be isotropic with a typical matrix size of 256 x 256, 256 mm field-of-view (FOV) and 1 mm slice thickness covering the whole brain.

Diagnostic Test: Resting state functional MRI

Scanning will be performed on a 3 Tesla General Electric scanner (Optima 750W) with a GEM HNU 24-channel head coil. rsFMRI matching the FLAIR and T1 post-contrast images will be obtained. For resting state fMRI, T2*-weighted images will be acquired with a single-shot gradient echo echo-planar imaging (EPI) sequence in the axial orientation (TR = 2500 ms, TE = 30 ms, FA = 80°, slice thickness = 4 mm, FOV= 240mm2, matrix=64×64) covering whole brain. For the resting state fMRI portion of the scan, patients will be instructed by the technologist or research staff to leave eyes open, focus on looking at a crosshair, and not think about anything during the scan.

Other Name: rsFMRI

Eligibility


Ages Eligible for Study:	18 Years and older (Adult, Senior)
Sexes Eligible for Study:	All
Accepts Healthy Volunteers:	No

Criteria

Inclusion Criteria:

Age greater than or equal to 18 years.
Fluency in English
Diagnosis of a histiocytic disorder as determined, in the opinion of the study PI, by a corroborating constellation of histopathology, clinical, and/or radiologic findings.
Will undergo Standard of Care MRI.

Exclusion Criteria:

Known intracranial involvement of histiocytosis (including dura, leptomeninges and brain parenchyma)
Prior stroke or intracranial hemorrhage
Other (non-histiocytic) intracranial neoplasm or neurological disorder deemed by the PI or Co-PI to confound neuroimaging studies (e.g., demyelinating disease)
Existing diagnosis of a psychiatric disorder or untreated mood disturbance
Existing diagnosis of a neurodegenerative disease, such as Alzheimer's disease
Chronic or daily excessive alcohol consumption as determined by the PI.
History of chronic use of corticosteroids, defined as continuous treatment for six months or longer at any time in the past
History of severe claustrophobia or other contraindications to patient SOC brain MRI
Prior intravenous cytarabine or cladribine
Other current or prior treatments (e.g., high-dose chemotherapy for a different cancer) deemed by the PI or Co-PI to confound imaging studies or cognitive performance

Contacts and Locations

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT03127709

Contacts


Contact: Eli Diamond, MD	212-610-0243	diamone1@mskcc.org
Contact: Denise Correa, PhD	212-610-0491

Locations


United States, New Jersey
Memoral Sloan Kettering Basking Ridge	Recruiting
Basking Ridge, New Jersey, United States, 07920
Contact: Eli Diamond, MD 212-610-0243
Memorial Sloan Kettering Monmouth	Recruiting
Middletown, New Jersey, United States, 07748
Contact: Eli Diamond, MD 212-610-0243
United States, New York
Memorial Sloan Kettering Cancer Center @ Suffolk	Recruiting
Commack, New York, United States, 11725
Contact: Eli Diamond, MD 212-610-0243
Memoral Sloan Kettering Westchester	Recruiting
Harrison, New York, United States, 10604
Contact: Eli Diamond, MD 212-610-0243

Sponsors and Collaborators

Memorial Sloan Kettering Cancer Center

More Information

Additional Information:

Memorial Sloan Kettering This link exits the ClinicalTrials.gov site


Responsible Party:	Memorial Sloan Kettering Cancer Center
ClinicalTrials.gov Identifier:	NCT03127709 History of Changes
Other Study ID Numbers:	17-199
Study First Received:	April 11, 2017
Last Updated:	April 20, 2017
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD:	Undecided


Studies a U.S. FDA-regulated Drug Product:		No
Studies a U.S. FDA-regulated Device Product:		Yes
Device Product Not Approved or Cleared by U.S. FDA:		No
Pediatric Postmarket Surveillance of a Device Product:		No

Keywords provided by Memorial Sloan Kettering Cancer Center:


17-199 histiocytosis

Additional relevant MeSH terms:


Histiocytosis Lymphatic Diseases

ClinicalTrials.gov processed this record on July 14, 2017