Serial Changes of Cerebral Glucose Metabolism and Caudate Size in Persons at Risk for Huntington's Disease
Abstract
OBJECTIVE: To determine the rate of change of glucose metabolism and caudate size in persons at risk for Huntington's disease. DESIGN: Eighteen persons at risk for Huntington's disease had two positron emission tomographic glucose metabolic studies and two magnetic resonance imaging scans separated by 42 (+/- 9) months. SETTING: Ambulatory research subjects at a teaching hospital with magnetic resonance imaging and positron emission tomographic technology. SUBJECTS: Seven of the individuals were Huntington' disease gene negative by testing at the polymorphic DNA loci D4S10, D4S43, and D4S125; the remainder were gene positive by genetic testing or onset of chorea after study entry. INTERVENTIONS: None. OUTCOME MEASURES: Onset of chorea and imaging results. RESULTS: The gene-positive group demonstrated a significant 3.1% loss of glucose metabolic rate per year in the caudate nucleus (95% confidence interval [CI], -4.64, -1.48) compared with the gene-negative group. There was a 3.6% per year increase in the magnetic resonance imaging bicaudate ratio (95% CI, 1.81, 5.37), a linear measure of caudate atrophy. The rate of change in caudate size did not correlate with the rate of change in caudate metabolism, suggesting that metabolic loss and atrophy may develop independently. CONCLUSIONS: The results suggest that a reduction in caudate glucose metabolism and atrophy develop rapidly in Huntington's disease. The findings establish a strategy for using serial positron emission tomographic imaging to monitor experimental pharmacologic interventions in presymptomatic individuals who have developed caudate hypometabolism.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Glucose metabolism is reduced in the basal ganglia and cerebral cortex of symptomatic HD patients and even presymptomatic gene carriers, as assessed by PET imaging.
“Glucose metabolism is reduced in the basal ganglia and cerebral cortex of symptomatic HD patients and even of presymptomatic gene carriers, as assessed by PET imaging35-37.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [35]
Cites
Neuropathological Classification of Huntingtonʼs Disease
Vonsattel, J., Myers, R. H., STEVENS, T. J., Ferrante, R. J., Bird, E. D., & Richardson, E. P. (1985) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-198511000-00003 · Cited by 2,598 · see citing works
Strategies for multilocus linkage analysis in humans.
Lathrop, M. G., Lalouel, J.-M., Julier, C., & Ott, J. (1984) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.81.11.3443 · Cited by 2,560 · see citing works
Phelps, M. E., Huang, S. C., Hoffman, E. J., Selin, C. E., Sokoloff, L., & Kuhl, D. E. (1979) · Annals of Neurology · doi:10.1002/ana.410060502 · Cited by 2,417 · see citing works
Self-citationA polymorphic DNA marker genetically linked to Huntington's disease
Gusella, J. F., Wexler, N. S., Conneally, P. M., Naylor, S. L., Anderson, M. A., Tanzi, R. E., Watkins, P. C., Ottina, K. A., Wallace, M. R., Sakaguchi, A. Y., et al. (1983) · Nature · doi:10.1038/306234a0 · Cited by 2,340 · see citing works
Self-citationFundamentals of Biostatistics.
Cornell, R. G., & ROSNER, B. A. (1987) · Journal of the American Statistical Association · doi:10.2307/2289416 · Cited by 1,630 · see citing works
Kuhl, D. E., Phelps, M. E., Markham, C. H., Metter, E. J., Riege, W. H., & Winter, J. (1982) · Annals of Neurology · doi:10.1002/ana.410120504 · Cited by 458 · see citing works
Self-citationQuantitation in Positron Emission Computed Tomography: 5. Physical–Anatomical Effects
Mazziotta, J. C., Phelps, M. E., Plummer, D. L., & Kuhl, D. E. (1981) · Journal of Computer Assisted Tomography · doi:10.1097/00004728-198110000-00029 · Cited by 337 · see citing works
Self-citationReduced Cerebral Glucose Metabolism in Asymptomatic Subjects at Risk for Huntington's Disease
Mazziotta, J. C., Phelps, M. E., Pahl, J. J., Huang, S., Baxter, L. R., Riege, W. H., Hoffman, J. M., Kuhl, D. E., Lanto, A. B., Wapenski, J. A., et al. (1987) · New England Journal of Medicine · doi:10.1056/nejm198702123160701 · Cited by 314 · see citing works
Self-citationCORTICAL AND SUBCORTICAL GLUCOSE CONSUMPTION MEASURED BY PET IN PATIENTS WITH HUNTINGTON'S DISEASE
Kuwert, T., Lange, H. W., Langen, K., Herzog, H. R., Aulich, A., & Feinendegen, L. E. (1990) · Brain · doi:10.1093/brain/113.5.1405 · Cited by 274 · see citing works
Human brain glucose utilization and cognitive function in relation to age
Duara, R., PhD, C. G., PhD, J. H., Ingvar, D. H., Sokoloff, L., Margolin, R. A., PhD, R. G. M., Cutler, N. R., & Рапопорт, С. И. (1984) · Annals of Neurology · doi:10.1002/ana.410160613 · Cited by 262 · see citing works
Clinical and neuropathologic assessment of severity in Huntington's disease
Myers, R. H., Vonsattel, J. P., Stevens, T. J., Cupples, L. A., Richardson, E. P., Martin, J. B., & Bird, E. D. (1988) · Neurology · doi:10.1212/wnl.38.3.341 · Cited by 217 · see citing works
Calkins, R. A., & Allen, M. W. V. (1967) · PubMed · Cited by 204 · see citing works
Huntington disease: genetics and epidemiology.
Conneally, P. M. (1984) · PubMed · Cited by 202 · see citing works
de Leon, M. J., Ferris, S. H., GEORGE, A. E., Reisberg, ., Christman, D. R., Kricheff, I. I., & Wolf, A. P. (1983) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/jcbfm.1983.57 · Cited by 197 · see citing works
Positron emission tomography in the early diagnosis of Huntington's disease
Hayden, M. R., Martin, W. R. W., Stoessl, A. J., et al. (1986) · Neurology · doi:10.1212/wnl.36.7.888 · Cited by 191 · see citing works
Huntington's disease in Venezuela
Young, A. B., Shoulson, I., Penney, J. B., Starosta‐Rubinstein, S., Gomez, F., Travers, H., Ramos-Arroyo, M. A., Snodgrass, S. R., Bonilla, E., Moreno, H., et al. (1986) · Neurology · doi:10.1212/wnl.36.2.244 · Cited by 179 · see citing works
Harris, G. J., Pearlson, G. D., Peyser, C. E., et al. (1992) · Annals of Neurology · doi:10.1002/ana.410310113 · Cited by 171 · see citing works
Yoshii, F., Barker, W. W., Chang, J. Y., Loewenstein, D., Apicella, A., Smith, D. D., Boothe, T. E., Ginsberg, M. D., Pascal, S., & Duara, R. (1988) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/jcbfm.1988.112 · Cited by 168 · see citing works
The diagnosis of Huntington's disease
Folstein, S. E., Leigh, R. J., Parhad, I. M., & Folstein, M. F. (1986) · Neurology · doi:10.1212/wnl.36.10.1279 · Cited by 159 · see citing works
Reivich, M., Alavi, A. A., Wolf, A. P., Greenberg, J. H., Fowler, J. S., Christman, D. R., MacGregor, R. R., Jones, S. C., London, J., Shiue, C., et al. (1982) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/jcbfm.1982.32 · Cited by 139 · see citing works
Reproducibility of Cerebral Glucose Metabolic Measurements in Resting Human Subjects
Bartlett, E. J., Brodie, J. D., Wolf, A. P., Christman, D. R., LASKA, E. M., & Meissner, M. (1988) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/jcbfm.1988.91 · Cited by 136 · see citing works
Late onset of Huntington's disease.
Myers, R. H., Sax, D. S., Schoenfeld, M., Bird, E. D., Wolf, P. A., Vonsattel, J. P., White, R. F., & Martin, J. B. (1985) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.48.6.530 · Cited by 127 · see citing works
Grafton, S. T., Mazziotta, J. C., Pahl, J. J., George‐Hyslop, P. S., Haines, J. L., Gusella, J. F., Hoffman, J. M., Baxter, L. R., & Phelps, M. E. (1990) · Annals of Neurology · doi:10.1002/ana.410280503 · Cited by 110 · see citing works
Self-citationHayden, M. R., Hewitt, J., Stoessl, A. J., et al. (1987) · Neurology · doi:10.1212/wnl.37.9.1441 · Cited by 109 · see citing works
A controlled clinical trial of baclofen as protective therapy in early huntington's disease
Shoulson, I., Odoroff, C. L., Oakes, D., Behr, J., Goldblatt, D., Caine, E. D., Kennedy, J., Miller, C., Bamford, K. A., Rubin, A., et al. (1989) · Annals of Neurology · doi:10.1002/ana.410250308 · Cited by 106 · see citing works
A BOUNDARY METHOD FOR ATTENUATION CORRECTION IN POSITRON COMPUTED TOMOGRAPHY
Huang, S. H., Carson, R. E., Phelps, M. E., et al. (1981) · Journal of Computer Assisted Tomography · doi:10.1097/00004728-198112000-00087 · Cited by 104 · see citing works
Self-citationCortical glucose metabolism in Huntington's disease
Martin, W. R. W., Clark, C. C., Ammann, W., Stoessl, A. J., Shtybel, W., & Hayden, M. R. (1992) · Neurology · doi:10.1212/wnl.42.1.223 · Cited by 100 · see citing works
Performance evaluation of a positron tomograph designed for brain imaging.
Hoffman, E. J., Phelps, M. E., & Huang, S. (1983) · PubMed · Cited by 93 · see citing works
Self-citationRecombination events suggest potential sites for the Huntington's disease gene
MacDonald, M. E., Haines, J. L., Zimmer, M., Cheng, S. V., Youngman, S., Whaley, W. L., Wexler, N. S., Bućan, M., Allitto, B. A., Smith, B. A., et al. (1989) · Neuron · doi:10.1016/0896-6273(89)90031-7 · Cited by 91 · see citing works
Self-citationNormal Caudate Glucose Metabolism in Persons at Risk for Huntington's Disease
Young, A. B., Penney, J. B., Starosta‐Rubinstein, S., Markel, D. S., Berent, S., Rothley, J., Betley, A. T., & Hichwa, R. D. (1987) · Archives of Neurology · doi:10.1001/archneur.1987.00520150010010 · Cited by 88 · see citing works
Brooks, R. A., Chiro, G. D., ZUKERBERG, B., et al. (1987) · PubMed · Cited by 80 · see citing works
Tyler, J. L., Strother, S. C., Zatorre, R. J., Alivisatos, B., Worsley, K. J., Dikšić, M., & Yamamoto, Y. (1988) · PubMed · Cited by 80 · see citing works
What excitotoxin kills striatal neurons in Huntington's disease? Clues from neurochemical studies
Perry, T. L., & Hansen, S. (1990) · Neurology · doi:10.1212/wnl.40.1.20 · Cited by 76 · see citing works
Albin, R. L., Qin, Y., Young, A. B., Penney, J. B., & Chesselet, M. (1991) · Annals of Neurology · doi:10.1002/ana.410300406 · Cited by 66 · see citing works
Stober, T., Wussow, W., & Schimrigk, К. (1984) · Neuroradiology · doi:10.1007/bf00328198 · Cited by 58 · see citing works
Brain Imaging: Aging and Dementia
Cutler, N. R., Duara, R., Creasey, H. M., Grady, C. L., Haxby, J. V., Schapiro, M. B., & Rapoport, S. I. (1984) · Annals of Internal Medicine · doi:10.7326/0003-4819-101-3-355 · Cited by 43 · see citing works
Huntington disease in Georgia: age at onset.
Adams, P., Falek, A., & Arnold, J. (1988) · PubMed · Cited by 33 · see citing works
Predictability of Phenotype in Huntington's Disease
Farrer, L. A., & Conneally, P. M. (1987) · Archives of Neurology · doi:10.1001/archneur.1987.00520130083023 · Cited by 27 · see citing works
Studies in Persons at Risk for Huntington's Disease
(1987) · New England Journal of Medicine · doi:10.1056/nejm198708063170612 · Cited by 27 · see citing works
Clark, C. M., Hayden, M. R., Stoessl, A. J., et al. (1986) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/jcbfm.1986.132 · Cited by 21 · see citing works
Grady, C. L. (1991) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/jcbfm.1991.38 · Cited by 19 · see citing works
Phenotypic variation in 2 Huntington's disease families with linkage to chromosome 4
Sax, D. S., Bird, E. D., Gusella, J. F., et al. (1989) · Neurology · doi:10.1212/wnl.39.10.1332 · Cited by 13 · see citing works
Self-citation
Flagged first, then by citations
Cited by
Sorted by citations
Reiman, E. M., Caselli, R. J., Yun, L. S., Chen, K., Bandy, D., Minoshima, S., Thibodeau, S. N., & Osborne, D. (1996) · New England Journal of Medicine · doi:10.1056/nejm199603213341202 · Cited by 1,366 · see citing works
Cui, L., Jeong, H., Borovecki, F., Parkhurst, C. N., Tanese, N., & Krainc, D. (2006) · Cell · doi:10.1016/j.cell.2006.09.015 · Cited by 1,012 · see citing works
Brain metabolism in health, aging, and neurodegeneration
Camandola, S., & Mattson, M. P. (2017) · The EMBO Journal · doi:10.15252/embj.201695810 · Cited by 752 · see citing works
Mitochondrial Approaches for Neuroprotection
Chaturvedi, R. K., & Beal, M. F. (2008) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1427.027 · Cited by 294 · see citing works
Cognitive effects of insulin in the central nervous system
Park, C. R. (2001) · Neuroscience & Biobehavioral Reviews · doi:10.1016/s0149-7634(01)00016-1 · Cited by 270 · see citing works
The Energetics of Huntington's Disease
Browne, S. E., & Beal, M. F. (2004) · Neurochemical Research · doi:10.1023/b:nere.0000014824.04728.dd · Cited by 240 · see citing works
Huntingtin-deficient zebrafish exhibit defects in iron utilization and development
Lumsden, A. L., Henshall, T. L., Dayan, S., Lardelli, M. T., & Richards, R. I. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddm138 · Cited by 156 · see citing works
Parallel explicit and implicit control of reaching
Mazzoni, P., & Wexler, N. S. (2009) · PLoS ONE · doi:10.1371/journal.pone.0007557 · Cited by 24 · see citing works
- Authors
- Grafton, S. T., Mazziotta, J. C., Pahl, J. J., George-Hyslop, P. S., Haines, J. L., Gusella, J., Hoffman, J. M., Baxter, L. R., & Phelps, M. E.
- Affiliations
- University of Southern California (US)
- University of California, Los Angeles (US)
- University of Oklahoma (US)
- Harvard University (US)
- Duke University (US)
- Venue
- Archives of Neurology
- Publisher
- American Medical Association (AMA)
- Published
- 1992-11-01
- Volume
- 49
- Issue
- 11
- Pages
- 1161-1167
- ISSN
- 0003-9942 (ISSN-L)
- OpenAlex
- W1984548880
- Cited by
- 157
- References
- 42
- Citations by year
- 2025: 3 · 2024: 1 · 2023: 4 · 2022: 3 · 2021: 2 · 2020: 4 · 2019: 3 · 2018: 4 · 2017: 3 · 2016: 5 · 2015: 4 · 2014: 4 · 2013: 9 · 2012: 7
- FWCI
- 1.36
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Archives of Neurology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (5k01mh000752-04, 5p01ns015654-15)
- National Institute of Mental Health (MH00752)
- National Institute of Neurological Disorders and Stroke (NS 16367, P01 NS15654)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Neurological and metabolic disorders
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Keywords
- Huntington's disease, caudate nucleus, brain glucose metabolism, caudate atrophy, presymptomatic carriers, disease progression monitoring
- MeSH terms
- Adult, Caudate Nucleus, Cerebral Cortex, Disease Susceptibility, Female, Genetic Markers, Glucose, Humans, Huntington Disease, Male, Middle Aged, Tomography, Emission-Computed
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
