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Abstract

Neuropathologic and neuroimaging studies have suggested that frontal lobes are affected in Huntington's disease (HD), and that atrophy in this region may be associated with some of the cognitive impairment and clinical decline observed in patients with HD. We measured gray and white matter volumes within the frontal lobes on MRI for 20 patients with HD (10 mildly affected and 10 moderately affected) and 20 age- and sex-matched control subjects. We also correlated frontal lobe measurements with measures of symptom severity and cognitive function. Patients who were mildly affected had frontal lobe volumes (both gray and white matter) essentially identical to those of control subjects, despite clearly abnormal basal ganglia. Patients who were moderately affected demonstrated significant reductions in total frontal lobe volume (17%) and frontal white matter volume (28%). Frontal lobe white matter volume reductions, but not total frontal lobe volume reductions, were disproportionately greater than overall brain volume reductions (17%). Frontal lobe volume correlated with symptom severity and general cognitive function, but these correlations did not remain significant after taking into account total brain volume. We conclude that cognitive impairment and symptom severity are associated with frontal lobe atrophy, but this association is not specific to the frontal lobes. Frontal lobe atrophy (like total brain atrophy) occurs in later stages of increasing HD symptom severity and this atrophy primarily involves white matter.

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Published In

Neurology®
Volume 50Number 1January 1998
Pages: 252-258
PubMed: 9443488

Publication History

Published online: January 1, 1998
Published in print: January 1998

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Authors

Affiliations & Disclosures

E. H. Aylward, PhD
From the Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine, the Division of Psychiatric Neuroimaging (Drs. Aylward, Barta, and Pearlson, and N.B. Anderson, J. Feeney, and A. Davis); Baltimore Huntington's Disease Center (Drs. Aylward, Bylsma, Wagster, Rosenblatt, and Ross, and M. Sherr); the Department of Pathology, Neuropathology Laboratory (Dr. Wagster); the Department of Neurology (N. B. Anderson); the School of Hygiene and Public Health (Dr. Pearlson); and the Department of Neuroscience (Dr. Ross), Baltimore, MD.
N. B. Anderson, BA
From the Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine, the Division of Psychiatric Neuroimaging (Drs. Aylward, Barta, and Pearlson, and N.B. Anderson, J. Feeney, and A. Davis); Baltimore Huntington's Disease Center (Drs. Aylward, Bylsma, Wagster, Rosenblatt, and Ross, and M. Sherr); the Department of Pathology, Neuropathology Laboratory (Dr. Wagster); the Department of Neurology (N. B. Anderson); the School of Hygiene and Public Health (Dr. Pearlson); and the Department of Neuroscience (Dr. Ross), Baltimore, MD.
F. W. Bylsma, PhD
From the Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine, the Division of Psychiatric Neuroimaging (Drs. Aylward, Barta, and Pearlson, and N.B. Anderson, J. Feeney, and A. Davis); Baltimore Huntington's Disease Center (Drs. Aylward, Bylsma, Wagster, Rosenblatt, and Ross, and M. Sherr); the Department of Pathology, Neuropathology Laboratory (Dr. Wagster); the Department of Neurology (N. B. Anderson); the School of Hygiene and Public Health (Dr. Pearlson); and the Department of Neuroscience (Dr. Ross), Baltimore, MD.
M. V. Wagster, PhD
From the Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine, the Division of Psychiatric Neuroimaging (Drs. Aylward, Barta, and Pearlson, and N.B. Anderson, J. Feeney, and A. Davis); Baltimore Huntington's Disease Center (Drs. Aylward, Bylsma, Wagster, Rosenblatt, and Ross, and M. Sherr); the Department of Pathology, Neuropathology Laboratory (Dr. Wagster); the Department of Neurology (N. B. Anderson); the School of Hygiene and Public Health (Dr. Pearlson); and the Department of Neuroscience (Dr. Ross), Baltimore, MD.
P. E. Barta, MD, PhD
From the Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine, the Division of Psychiatric Neuroimaging (Drs. Aylward, Barta, and Pearlson, and N.B. Anderson, J. Feeney, and A. Davis); Baltimore Huntington's Disease Center (Drs. Aylward, Bylsma, Wagster, Rosenblatt, and Ross, and M. Sherr); the Department of Pathology, Neuropathology Laboratory (Dr. Wagster); the Department of Neurology (N. B. Anderson); the School of Hygiene and Public Health (Dr. Pearlson); and the Department of Neuroscience (Dr. Ross), Baltimore, MD.
M. Sherr, RN, BSN
From the Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine, the Division of Psychiatric Neuroimaging (Drs. Aylward, Barta, and Pearlson, and N.B. Anderson, J. Feeney, and A. Davis); Baltimore Huntington's Disease Center (Drs. Aylward, Bylsma, Wagster, Rosenblatt, and Ross, and M. Sherr); the Department of Pathology, Neuropathology Laboratory (Dr. Wagster); the Department of Neurology (N. B. Anderson); the School of Hygiene and Public Health (Dr. Pearlson); and the Department of Neuroscience (Dr. Ross), Baltimore, MD.
J. Feeney, BA
From the Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine, the Division of Psychiatric Neuroimaging (Drs. Aylward, Barta, and Pearlson, and N.B. Anderson, J. Feeney, and A. Davis); Baltimore Huntington's Disease Center (Drs. Aylward, Bylsma, Wagster, Rosenblatt, and Ross, and M. Sherr); the Department of Pathology, Neuropathology Laboratory (Dr. Wagster); the Department of Neurology (N. B. Anderson); the School of Hygiene and Public Health (Dr. Pearlson); and the Department of Neuroscience (Dr. Ross), Baltimore, MD.
A. Davis
From the Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine, the Division of Psychiatric Neuroimaging (Drs. Aylward, Barta, and Pearlson, and N.B. Anderson, J. Feeney, and A. Davis); Baltimore Huntington's Disease Center (Drs. Aylward, Bylsma, Wagster, Rosenblatt, and Ross, and M. Sherr); the Department of Pathology, Neuropathology Laboratory (Dr. Wagster); the Department of Neurology (N. B. Anderson); the School of Hygiene and Public Health (Dr. Pearlson); and the Department of Neuroscience (Dr. Ross), Baltimore, MD.
A. Rosenblatt, MD
From the Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine, the Division of Psychiatric Neuroimaging (Drs. Aylward, Barta, and Pearlson, and N.B. Anderson, J. Feeney, and A. Davis); Baltimore Huntington's Disease Center (Drs. Aylward, Bylsma, Wagster, Rosenblatt, and Ross, and M. Sherr); the Department of Pathology, Neuropathology Laboratory (Dr. Wagster); the Department of Neurology (N. B. Anderson); the School of Hygiene and Public Health (Dr. Pearlson); and the Department of Neuroscience (Dr. Ross), Baltimore, MD.
G. D. Pearlson, MD
From the Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine, the Division of Psychiatric Neuroimaging (Drs. Aylward, Barta, and Pearlson, and N.B. Anderson, J. Feeney, and A. Davis); Baltimore Huntington's Disease Center (Drs. Aylward, Bylsma, Wagster, Rosenblatt, and Ross, and M. Sherr); the Department of Pathology, Neuropathology Laboratory (Dr. Wagster); the Department of Neurology (N. B. Anderson); the School of Hygiene and Public Health (Dr. Pearlson); and the Department of Neuroscience (Dr. Ross), Baltimore, MD.
C. A. Ross, MD, PhD
From the Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine, the Division of Psychiatric Neuroimaging (Drs. Aylward, Barta, and Pearlson, and N.B. Anderson, J. Feeney, and A. Davis); Baltimore Huntington's Disease Center (Drs. Aylward, Bylsma, Wagster, Rosenblatt, and Ross, and M. Sherr); the Department of Pathology, Neuropathology Laboratory (Dr. Wagster); the Department of Neurology (N. B. Anderson); the School of Hygiene and Public Health (Dr. Pearlson); and the Department of Neuroscience (Dr. Ross), Baltimore, MD.

Notes

Address correspondence and reprint requests to Dr. Elizabeth H. Aylward, 6004 E. Mercer Way, Mercer Island, WA 98040.

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