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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| 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. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Trail Making Test, Parts A & B | Behavioral | Part A is a timed measure of visual scanning and graphomotor speed. Part B is a timed measure of cognitive flexibility. |
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| Measure | Description | Time Frame |
|---|---|---|
| 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 |
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Inclusion Criteria:
Exclusion Criteria:
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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.
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| Name | Affiliation | Role |
|---|---|---|
| Eli Diamond, MD | Memorial Sloan Kettering Cancer Center | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Memorial Sloan Kettering Basking Ridge | Basking Ridge | New Jersey | 07920 | United States | ||
| Memorial Sloan Kettering Monmouth |
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| Label | URL |
|---|---|
| Memorial Sloan Kettering | View source |
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| Brief Test of Attention | Behavioral | Assesses auditory working memory |
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| Symbol Span | Behavioral | Assesses visual working memory |
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| Controlled Oral Word Association Test | Behavioral | 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). |
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| The Hopkins Verbal Learning Test-Revised | Behavioral | HVLT-R is a test of verbal learning and memory. Scores obtained are the total number of words. |
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| Brief Visuospatial Memory Test-Revised | Behavioral | The BVMT-R is a test of visuospatial learning and memory. |
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| Hospital Anxiety and Depression Scale | Behavioral | The HADS is a brief assessment validated in many cancer populations. |
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| McGill Quality of Life Scale | Behavioral | This is a questionnaire that is a validated 17-item QOL instrument validated for ill patients and in the particular context of the ancer. |
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| MR Brain Imaging | Diagnostic Test | 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. |
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| Resting state functional MRI | Diagnostic Test | 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. |
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| Functional Assessment of Cancer Therapy Cognitive Scale (FACT-Cog) | Behavioral | 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. |
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| 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 |
| ID | Term |
|---|---|
| D015614 | Histiocytosis |
| ID | Term |
|---|---|
| D008206 | Lymphatic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
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| ID | Term |
|---|---|
| D014145 | Trail Making Test |
| D014888 | Wechsler Scales |
| D009483 | Neuropsychological Tests |
| C018209 | 4-amino-4'-hydroxylaminodiphenylsulfone |
| ID | Term |
|---|---|
| D011581 | Psychological Tests |
| D004191 | Behavioral Disciplines and Activities |
| D007361 | Intelligence Tests |
| D001077 | Aptitude Tests |
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