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Clinical and Neuropathological Characteristics of Hippocampal Sclerosis
A Community-Based Study
James B. Leverenz, MD;
Christina M. Agustin, BS;
Debby Tsuang, MD;
Elaine R. Peskind, MD;
Steven D. Edland, PhD;
David Nochlin, MD;
Lillian DiGiacomo, BS;
James D. Bowen, MD;
Wayne C. McCormick, MD;
Linda Teri, PhD;
Murray A. Raskind, MD;
Walter A. Kukull, PhD;
Eric B. Larson, MD, MPH
Arch Neurol. 2002;59:1099-1106.
Background Hippocampal sclerosis (HS) is a neuropathologic finding characterized
by neuronal loss and gliosis in the CA-1 and subiculum of the hippocampus.
Previous studies of HS have shown that this is a common postmortem finding
in elderly subjects with dementia. However, these studies were from selected
samples and therefore are not necessarily representative of patients seen
in the general medical community.
Objectives To examine the clinical and pathologic characteristics of HS in a community-based
case series of dementia and to compare these characteristics with those observed
in subjects with Alzheimer disease (AD) from the same study sample.
Methods One hundred thirty-four autopsy cases were available from a community-based
registry of dementia. Sixteen cases (12%) had a postmortem diagnosis of HS.
Thirty-two comparison control cases with a neuropathologic diagnosis of AD
were selected from the same files. Each case of HS was reviewed for HS neuropathologic
features, including severity, distribution, and additional pathologic processes.
Blinded review of clinical characteristics for the HS and control groups was
performed to assess risk factors.
Results There was a wide range of severity and distribution of HS lesions between
cases and substantial variability in lesion severity and age within individual
cases. Serial neuropsychologic and behavioral assessments revealed similar
clinical features and rates of dementia progression between HS and AD groups.
Of all neuropsychologic tests performed at enrollment, only enhanced performance
on Trails A differentiated the HS from the AD group (64 seconds, 0 errors
vs 114 seconds, 0.6 errors; P .05). The number
of AD cases with at least 1 apolipoprotein  4 allele was significantly
greater than the HS cases (61% vs 31%;  2 = 3.81, P.05). Although medical record review indicated higher frequencies
of clinical stroke and neuroradiologic white matter abnormalities in the HS
group, risk factors for vascular disease and neuropathologic evidence of cerebrovascular
disease did not differ between the groups.
Conclusions Our results suggest that HS is a frequent pathologic finding in community-based
dementia. Individuals with HS have similar initial symptoms and rates of dementia
progression to those with AD and therefore are frequently misclassified as
having AD. Our clinical and pathologic findings suggest that HS has characteristics
of a progressive disorder although the underlying cause remains elusive.
From the Department of Veterans Affairs, Northwest Network Mental Illness
Research, Education, and Clinical Center, Seattle, Wash (Drs Leverenz, Tsuang,
Peskind, and Raskind); Departments of Epidemiology (Dr Kukull), Medicine (Drs
McCormick and Larson), Neurology (Drs Leverenz and Bowen), Nursing (Dr Teri),
Pathology, Division of Neuropathology (Dr Nochlin), and Psychiatry and Behavioral
Sciences (Drs Leverenz, Tsuang, Peskind, and Raskind, and Mss Agustin and
DiGiacomo), University of Washington, Seattle; and Mayo Clinic, Rochester,
Minn (Dr Edland).
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