A Study of Memory, Thinking, and Brain Imaging in Adults With Histiocytosis
A Clinical, Structural, and Functional Neuroimaging Study of Cognition in Adults With Erdheim-Chester Disease, Langerhans Cell Histiocytosis, Rosai-Dorfman Disease, and Other Histiocytoses
1 other identifier
observational
13
1 country
4
Brief Summary
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.
Trial Health
Trial Health Score
Automated assessment based on enrollment pace, timeline, and geographic reach
participants targeted
Target at below P25 for all trials
Started Apr 2017
Longer than P75 for all trials
4 active sites
Health score is calculated from publicly available data and should be used for screening purposes only.
Trial Relationships
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Study Timeline
Key milestones and dates
Study Start
First participant enrolled
April 7, 2017
CompletedFirst Submitted
Initial submission to the registry
April 11, 2017
CompletedFirst Posted
Study publicly available on registry
April 25, 2017
CompletedPrimary Completion
Last participant's last visit for primary outcome
April 1, 2026
CompletedStudy Completion
Last participant's last visit for all outcomes
April 1, 2026
CompletedMay 2, 2025
May 1, 2025
9 years
April 11, 2017
May 1, 2025
Conditions
Keywords
Outcome Measures
Primary Outcomes (3)
neurocognitive function
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.
1 year
quality of life
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.
1 year
comparing of grey matter volume
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.
1 year
Study Arms (1)
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.
Interventions
Part A is a timed measure of visual scanning and graphomotor speed. Part B is a timed measure of cognitive flexibility.
Assesses auditory working memory
Assesses visual working memory
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).
HVLT-R is a test of verbal learning and memory. Scores obtained are the total number of words.
The BVMT-R is a test of visuospatial learning and memory.
The HADS is a brief assessment validated in many cancer populations.
This is a questionnaire that is a validated 17-item QOL instrument validated for ill patients and in the particular context of the ancer.
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.
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.
This is a validated 37-item QOL instrument for cancer patients with cognitive complaints. The measures consist of FACT-Cog total, perceived cognitive impairments, perceived cognitive abilities, and impact on quality of life.
Eligibility Criteria
Participants will have a 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.
You may qualify if:
- 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.
You may not qualify if:
- 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
Contact the study team to confirm eligibility.
Sponsors & Collaborators
Study Sites (4)
Memorial Sloan Kettering Basking Ridge
Basking Ridge, New Jersey, 07920, United States
Memorial Sloan Kettering Monmouth
Middletown, New Jersey, 07748, United States
Memorial Sloan Kettering Cancer Center @ Suffolk
Commack, New York, 11725, United States
Memorial Sloan Kettering Westchester
Harrison, New York, 10604, United States
Related Links
MeSH Terms
Conditions
Interventions
Condition Hierarchy (Ancestors)
Intervention Hierarchy (Ancestors)
Study Officials
- PRINCIPAL INVESTIGATOR
Eli Diamond, MD
Memorial Sloan Kettering Cancer Center
Study Design
- Study Type
- observational
- Observational Model
- COHORT
- Time Perspective
- PROSPECTIVE
- Sponsor Type
- OTHER
- Responsible Party
- SPONSOR
Study Record Dates
First Submitted
April 11, 2017
First Posted
April 25, 2017
Study Start
April 7, 2017
Primary Completion
April 1, 2026
Study Completion
April 1, 2026
Last Updated
May 2, 2025
Record last verified: 2025-05