Skip to main content
AAN.com
ARTICLES
October 1, 1995

A prospective evaluation of cognitive decline in early Huntington's disease
Functional and radiographic correlates

October 1995 issue
45 (10) 1867-1873

Abstract

We examined prospectively the relationship between progressive disability in Huntington's disease (HD) and concomitant alterations in neuropsychological functioning and brain imaging indices in a cohort of 60 patients who were enrolled and followed for 30 to 42 months in a controlled clinical trial.Standardized measures of functional capacity and neuropsychological performance were collected, and CT was performed, at regular intervals every 6 to 12 months. Psychomotor skills showed the most significant and consistent decline among the cognitive functions assessed. Memory disturbances were already present at the time of enrollment, but memory did not deteriorate until patients reached advanced stages. Other cognitive operations, such as visual construction and semantic knowledge, manifested small and variable changes over time. CT indices of striatal atrophy correlated only with changes in psychomotor function, while the CT index of frontal atrophy weakly predicted memory and semantic knowledge scores at 42 months. These results confirmed earlier cross-sectional findings and extended our knowledge of the evolution of cognitive dysfunction in HD.
NEUROLOGY 1995;45: 1867-1873

Get full access to this article

View all available purchase options and get full access to this article.

REFERENCES

1.
Brandt J, Strauss ME, Larus J, Jensen B, Folstein SE, Folstein MF. Clinical correlates of dementia and disability in Huntington's disease. J Clin Neuropsychol 1984;6:401-412.
2.
Taylor HG, Hansotia P. Neuropsychological testing of Huntington's patients. J Nerv Ment Dis 1983;171:492-496.
3.
Josiassen RC, Curry C, Mancall EL. Development of neuropsychological deficits in Huntington's disease. Arch Neurol 1983;32:776-778.
4.
Moses JA, Golden CJ, Berger PA, Wisniewski AM. Neuropsychological deficits in early, middle and late stage Huntington's disease as measured by the Luria-Nebraska neuropsychological battery. Int J Neurosci 1981;14:95-100.
5.
Butters N, Sax D, Montgomery K, Tarlow S. Comparison of the neuropsychological deficits associated with early and advanced Huntington's disease. Arch Neurol 1978;35:585-589.
6.
Lyle OE, Gottesman I. Subtle cognitive deficits as 15-20 year precursors of Huntington's disease. In: Chase TN, Wexler NS, Barbeau A, eds. Advances in neurology, vol 23. New York: Raven Press, 1979:227-238.
7.
Lyle OE, Quast W. The Bender gestalt: use of clinical judgment versus recall scores in prediction of Huntington's disease. J Consult Clin Psychol 1976;44:229-232.
8.
Shoulson I, Odoroff C, Oakes D, et al. A controlled clinical trial of baclofen as protective therapy in early Huntington's disease. Ann Neurol 1989;25:252-259.
9.
Shoulson I, Kurlan R, Rubin A, et al. Assessment of functional capacity in neurodegenerative movement disorders: Huntington's disease as a prototype. In: Munsat TL, ed. Quantification of neurologic deficit. Boston: Butterworths, 1989:271-283.
10.
Shoulson I, Bamford K, Caine E, et al. Inter-observer reliability of functional capacity ratings for Huntington's disease [abstract]. Neurology 1985;35(suppl 1):176.
11.
Shoulson I, Plassche W, Odoroff C. Huntington's disease: caudate atrophy parallels functional impairment [abstract]. Neurology 1982;32(suppl 2):A143.
12.
Bamford KA, Caine ED, Kido DK, Plassche WM, Shoulson I. Clinical-pathologic correlation in Huntington's disease: a neuropsychological and computed tomography study. Neurology 1989;39:796-801.
13.
Wechsler D. Wechsler Adult Intelligence Scale-Revised. Manual. New York: Psychological Corp, 1981.
14.
Dunn LM. Expanded manual for the Peabody Picture Vocabulary Test. Minneapolis: American Guidance Service, 1970.
15.
Stroop JR. Studies of interference in serial verbal reactions. J Exp Psychol 1935;18:643-662.
16.
Reitan RM. Instructions and procedures for administering the Neuropsychological Test Battery used at the Neuropsychology Laboratory, Indiana University Medical Center. Trail Making Test. Manual (undated).
17.
Buschke H. Selective reminding for analysis of memory and learning. J Verbal Learn Verbal Behav 1973;12:543-550.
18.
Caine E, Bamford K, Schiffer R, Shoulson I, Levy S. A controlled neuropsychological comparison of Huntington's disease and multiple sclerosis. Arch Neurol 1986;43:249-254.
19.
Spreen O, Benton A. Neurosensory Center Comprehensive Examination for Aphasia (NCCEA)--Revised edition. Victoria: University of Victoria, Neuropsychology Laboratory, 1977.
20.
Greenhouse SW, Geisser S. Methods in the analysis of profile data. Psychometrika 1959;24:95-112.
21.
Caine ED, Hunt RD, Weingartner H, et al. Huntington's dementia: clinical and neuropsychological features. Arch Gen Psychiatry 1978;35:377-384.
22.
Caine ED, Ebert MH, Weingartner H. An outline for the analysis of dementia. The memory disorder of Huntington's disease. Neurology 1977;27:1087-1092.
23.
Bamford KA, Caine ED. The neuropsychology of Huntington's disease: problems of clinical-pathological correlation in a progressive brain illness. In: Goldstein G, Tarter RE, eds. Advances in clinical neuropsychology, vol 3. New York: Plenum, 1986:181-212.
24.
Goldman-Rakic P, Selemon L. New frontiers in basal ganglia research. Trends Neurosci 1990;13:241-244.
25.
Parent A. Extrinsic connections of the basal ganglia. Trends Neurosci 1990;13:254-258.
26.
Gerfen C. The neostriatal mosaic: striatal-patch matrix organization is related to cortical lamination. Science 1989;246:385-388.
27.
Graveland G, Williams RS, DiFiglia M. Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease. Science 1985;227:770-773.
28.
Reiner A, Albin R, Anderson KD, D'Amato C, Penney J, Young A. Differential loss of striatal projection neurons in Huntington's disease. Proc Natl Acad Sci USA 1988;85:5733-5737.
29.
Goto S, Hirona A, Rojas-Corona R. Immunohistochemical visualization of afferent nerve terminals in human globus pallidus and its alteration in neostriatal neurodegenerative disorders. Acta Neuropathol (Berl) 1989;78:543-550.
30.
Vonsattel J, Myers R, Stevens T, Ferrante R, Bird E, Richardson E. Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurol 1985;44:559-577.
31.
Alheid GF, Switzer RC III, Heimer L. Basal ganglia. In: Paxinos G, ed. The human nervous system. New York: Academic Press, 1990:483-582.
32.
Stuss DT, Benson DF. The frontal lobes. New York: Raven Press, 1986.
33.
Posner M, Walker J, Friedrich F, Rafal R. Effects of parietal lobe injury on covert orienting of visual attention. J Neurosci 1984;4:1863-1874.
34.
Brouwers P, Cox C, Martin A, Chase T, Fedio P. Differential perceptual-spatial impairment in Huntington's and Alzheimer's disease. Arch Neurol 1984;41:1073-1076.
35.
Squire L, Zola-Morgan S. The medial temporal lobe memory system. Science 1991;253:1380-1386.

Information & Authors

Information

Published In

Neurology®
Volume 45Number 10October 1995
Pages: 1867-1873
PubMed: 7477984

Publication History

Published online: October 1, 1995
Published in print: October 1995

Permissions

Request permissions for this article.

Authors

Affiliations & Disclosures

From the UR-NIMH Clinical Research Center for the Study of Psychopathology of the Elderly (Drs. Bamford, Caine, and Cox) and the Departments of Psychiatry (Drs. Bamford and Caine), Biostatistics (Dr. Cox), and Neurology (Dr. Shoulson), University of Rochester School of Medicine, Rochester, NY; and the Department of Radiology (Dr. Kido), Washington University School of Medicine, St. Louis, MO.
Supported in part by grants NS17978 (K.A.B., I.S., D.K.K.), MH40381 (K.A.B., E.D.C., D.K.K., C.C.), and RR 00044 to the University of Rochester Clinical Research Center.
Received March 24, 1994. Accepted in final form March 1, 1995.
Address correspondence and reprint requests to Dr Caine, University of Rochester Medical Center, 300 Crittenden Boulevard, Rochester, NY 14642-8409.

Metrics & Citations

Metrics

Citations

Download Citations

If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Select your manager software from the list below and click Download.

Cited By
  1. A Practical Guide for Clinical Approach to Patients With Huntington’s Disease in Korea, Journal of Movement Disorders, 17, 2, (138-149), (2024).https://doi.org/10.14802/jmd.24040
    Crossref
  2. Magnetic resonance imaging in Huntington's disease, Neuroimaging in Parkinson�s Disease and Related Disorders, (449-489), (2023).https://doi.org/10.1016/B978-0-12-821651-4.00001-4
    Crossref
  3. Evaluation of Oxidative Stress potential of some Nsaıds against Hydrogen Peroxide in experimental animal, Research Journal of Pharmacy and Technology, (6194-6200), (2021).https://doi.org/10.52711/0974-360X.2021.01072
    Crossref
  4. RTP801/REDD1 Is Involved in Neuroinflammation and Modulates Cognitive Dysfunction in Huntington’s Disease, Biomolecules, 12, 1, (34), (2021).https://doi.org/10.3390/biom12010034
    Crossref
  5. Prionopathies and Prionlike Protein Aberrations in Neurodegenerative Diseases, Neurographics, 11, 2, (127-148), (2021).https://doi.org/10.3174/ng.2000035
    Crossref
  6. Circadian rhythms in neurodegenerative disorders, Nature Reviews Neurology, 18, 1, (7-24), (2021).https://doi.org/10.1038/s41582-021-00577-7
    Crossref
  7. Huntington’s Disease, Mind and Brain, (81-108), (2020).https://doi.org/10.1007/978-3-030-38606-1_8
    Crossref
  8. Impaired intracellular trafficking of sodium‐dependent vitamin C transporter 2 contributes to the redox imbalance in Huntington’s disease, Journal of Neuroscience Research, 99, 1, (223-235), (2020).https://doi.org/10.1002/jnr.24693
    Crossref
  9. Normalizing glucocorticoid levels attenuates metabolic and neuropathological symptoms in the R6/2 mouse model of huntington's disease, Neurobiology of Disease, 121, (214-229), (2019).https://doi.org/10.1016/j.nbd.2018.09.025
    Crossref
  10. The Urologic Management of Huntington Chorea, Urological Care for Patients with Progressive Neurological Conditions, (105-115), (2019).https://doi.org/10.1007/978-3-030-23277-1_12
    Crossref
  11. See more
Loading...

View Options

Get Access

Login options

Check if you have access through your login credentials or your institution to get full access on this article.

Personal login Institutional Login
Purchase Options

The neurology.org payment platform is currently offline. Our technical team is working as quickly as possible to restore service.

If you need immediate support or to place an order, please call or email customer service:

  • 1-800-638-3030 for U.S. customers - 8:30 - 7 pm ET (M-F)
  • 1-301-223-2300 for customers outside the U.S. - 8:30 - 7 pm ET (M-F)
  • [email protected]

We appreciate your patience during this time and apologize for any inconvenience.

View options

Full Text

View Full Text

Full Text HTML

View Full Text HTML

Media

Figures

Other

Tables

Share

Share

Share article link

Share