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Cerebellar Volume in Adults With Down Syndrome

Cerebellar Volume in Adults With Down Syndrome Abstract Objectives: To determine the effects of aging on cerebellar volume in individuals with Down syndrome (DS). To determine whether volume of cerebellum is associated with dementia or with age-related decline in fine-motor control. Design: Case-control study involving comparison of cerebellar volumes in adults with DS and matched control subjects; survey study involving correlations between cerebellar volume and subjects' age and performance on a test of fine-motor control; and longitudinal study assessing change in cerebellar volume in adults with DS. Setting: The Johns Hopkins University School of Medicine, Baltimore, Md. Patients and Other Participants: Subjects included 40 adults with DS. Thirty of them were matched on age, sex, and race with cognitively normal subjects. A diagnosis of probable dementia was made for 5 of the subjects with DS. Longitudinal data were available for 23 of the 40 subjects with DS, with a mean interscan interval of 2 years. Main Outcome Measures: Volumes of cerebellum, total brain, and intracranial region were measured on magnetic resonance imaging scans. The Purdue Pegboard, a test of fine-motor control, was administered to 38 of the subjects with DS. Results: Subjects with DS had significantly smaller cerebellar volumes than matched controls, even after adjusting for total brain volume or total intracranial volume. Volume of cerebellum did not correlate significantly with age for either the subjects with DS or controls. Longitudinal change in cerebellar volume in subjects with DS was not significant. Volume of total brain, but not cerebellum, correlated significantly with performance on the Purdue Pegboard. Conclusions: Although cerebellar volumes are disproportionately small in individuals with DS, they do not diminish significantly with age and do not undergo agerelated atrophy that is different from that of normal controls. Volume reduction in the cerebellum does not appear to be specifically responsible for the age-related decline in finemotor control that is observed in adults with DS. References 1. Wisniewski KE, Wisniewski HM, Wen GY. Occurrence of neuropathological changes and dementia of Alzheimer's disease in Down's syndrome . Ann Neurol . 1985; 17:278-282.Crossref 2. Mann DMA, Jones D, Prinja D, Purkiss MS. The prevalence of amyloid (A4) protein deposits within the cerebral and cerebellar cortex in Down's syndrome and Alzheimer's disease . Acta Neuropathol . 1990;80:318-327.Crossref 3. Cole G, Neal JW, Singhrao SK, Jasani B, Newman GR. The distribution of amyloid plaques in the cerebellum and brain stem in Down's syndrome and Alzheimer's disease . Acta Neuropathol . 1993;85:542-552.Crossref 4. Braak H, Braak E, Bohl J, Lang W. Alzheimer's disease: amyloid plaques in the cerebellum . J Neurol Sci . 1898;93:277-287.Crossref 5. Tiffin J, Asher EJ. The Purdue Pegboard . J Appl Psychol . 1948;32:234-247.Crossref 6. Brown RG, Jahanshahi M, Marsden CD. The execution of bimanual movements in patients with Parkinson's, Huntington's, and cerebellar disease . J Neurol Neurosurg Psychiatry . 1993;56:295-297.Crossref 7. Aylward EH, Reiss A. Area and volume measurement of posterior fossa structures in MRI . J Psychiatr Res . 1991;25:159-168.Crossref 8. Barta PE, Dhingra L, Schwartz E. Efficient volume estimation using voxel data: picking a good stereologic grid. J Neurosci Methods. In press. 9. Zellweger H. Handbook of Clinical Neurology . New York, NY: North Holland; 1977. 10. Davidoff LM. The brain in Mongolian idiocy . Arch Neurol Psychiatry . 1928;20:1229-1257.Crossref 11. Jernigan TL, Bellugi U, Sowell E, Doherty S, Hesselink JR. Cerebral morphologic distinctions between Williams and Down syndromes . Arch Neurol . 1993;50:186-191.Crossref 12. Jernigan TL, Bellugi U. Anomalous brain morphology on magnetic resonance images in Williams syndrome and Down syndrome . Arch Neurol . 1990;47:529-533.Crossref 13. Weis S, Weber G, Neuhold A, Rett A. Down syndrome . Am J Neuroradiol . 1991; 12:1207-1211. 14. Raz N, Torres IJ, Briggs SD, et al. Selective neuroanatomic abnormalities in Down's syndrome and their cognitive correlates . Neurology . 1995;45:356-366.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1997 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1997.00550140077016
Publisher site
See Article on Publisher Site

Abstract

Abstract Objectives: To determine the effects of aging on cerebellar volume in individuals with Down syndrome (DS). To determine whether volume of cerebellum is associated with dementia or with age-related decline in fine-motor control. Design: Case-control study involving comparison of cerebellar volumes in adults with DS and matched control subjects; survey study involving correlations between cerebellar volume and subjects' age and performance on a test of fine-motor control; and longitudinal study assessing change in cerebellar volume in adults with DS. Setting: The Johns Hopkins University School of Medicine, Baltimore, Md. Patients and Other Participants: Subjects included 40 adults with DS. Thirty of them were matched on age, sex, and race with cognitively normal subjects. A diagnosis of probable dementia was made for 5 of the subjects with DS. Longitudinal data were available for 23 of the 40 subjects with DS, with a mean interscan interval of 2 years. Main Outcome Measures: Volumes of cerebellum, total brain, and intracranial region were measured on magnetic resonance imaging scans. The Purdue Pegboard, a test of fine-motor control, was administered to 38 of the subjects with DS. Results: Subjects with DS had significantly smaller cerebellar volumes than matched controls, even after adjusting for total brain volume or total intracranial volume. Volume of cerebellum did not correlate significantly with age for either the subjects with DS or controls. Longitudinal change in cerebellar volume in subjects with DS was not significant. Volume of total brain, but not cerebellum, correlated significantly with performance on the Purdue Pegboard. Conclusions: Although cerebellar volumes are disproportionately small in individuals with DS, they do not diminish significantly with age and do not undergo agerelated atrophy that is different from that of normal controls. Volume reduction in the cerebellum does not appear to be specifically responsible for the age-related decline in finemotor control that is observed in adults with DS. References 1. Wisniewski KE, Wisniewski HM, Wen GY. Occurrence of neuropathological changes and dementia of Alzheimer's disease in Down's syndrome . Ann Neurol . 1985; 17:278-282.Crossref 2. Mann DMA, Jones D, Prinja D, Purkiss MS. The prevalence of amyloid (A4) protein deposits within the cerebral and cerebellar cortex in Down's syndrome and Alzheimer's disease . Acta Neuropathol . 1990;80:318-327.Crossref 3. Cole G, Neal JW, Singhrao SK, Jasani B, Newman GR. The distribution of amyloid plaques in the cerebellum and brain stem in Down's syndrome and Alzheimer's disease . Acta Neuropathol . 1993;85:542-552.Crossref 4. Braak H, Braak E, Bohl J, Lang W. Alzheimer's disease: amyloid plaques in the cerebellum . J Neurol Sci . 1898;93:277-287.Crossref 5. Tiffin J, Asher EJ. The Purdue Pegboard . J Appl Psychol . 1948;32:234-247.Crossref 6. Brown RG, Jahanshahi M, Marsden CD. The execution of bimanual movements in patients with Parkinson's, Huntington's, and cerebellar disease . J Neurol Neurosurg Psychiatry . 1993;56:295-297.Crossref 7. Aylward EH, Reiss A. Area and volume measurement of posterior fossa structures in MRI . J Psychiatr Res . 1991;25:159-168.Crossref 8. Barta PE, Dhingra L, Schwartz E. Efficient volume estimation using voxel data: picking a good stereologic grid. J Neurosci Methods. In press. 9. Zellweger H. Handbook of Clinical Neurology . New York, NY: North Holland; 1977. 10. Davidoff LM. The brain in Mongolian idiocy . Arch Neurol Psychiatry . 1928;20:1229-1257.Crossref 11. Jernigan TL, Bellugi U, Sowell E, Doherty S, Hesselink JR. Cerebral morphologic distinctions between Williams and Down syndromes . Arch Neurol . 1993;50:186-191.Crossref 12. Jernigan TL, Bellugi U. Anomalous brain morphology on magnetic resonance images in Williams syndrome and Down syndrome . Arch Neurol . 1990;47:529-533.Crossref 13. Weis S, Weber G, Neuhold A, Rett A. Down syndrome . Am J Neuroradiol . 1991; 12:1207-1211. 14. Raz N, Torres IJ, Briggs SD, et al. Selective neuroanatomic abnormalities in Down's syndrome and their cognitive correlates . Neurology . 1995;45:356-366.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Feb 1, 1997

References