NEUROPSYCHOLOGY OF SUBCORTICAL DEMENTIAS

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The term s ubcortical dementia was first used to describe a distinct clinical syndrome in a 1974 literature review and case report of five patients with progressive supranuclear palsy (PSP). Albert et al1 described subcortical dementias as sharing a characteristic configuration of deficits, including “forgetfulness,” slowed thought processes (bradyphrenia), psychiatric changes (especially apathy, depression, and irritability), and impaired ability to manipulate acquired knowledge. They distinguished this pattern of deficits from the one seen in “cortical dementias,” such as Alzheimer's disease (AD), which includes anterograde amnesia, aphasia, agnosia, and apraxia. This term has since been broadened to encompass a wide range of disorders sharing primary pathology in subcortical structures, such as the basal ganglia, brain stem, thalamus, and central white matter—including such heterogeneous disorders as Parkinson's disease (PD); Huntington's disease (HD); multiple system atrophy; idiopathic basal ganglia calcification; multi-infarct dementia; and, most recently, AIDS dementia complex.24, 27, 105

This article describes the neurobiologic and cognitive features of three prototypical subcortical dementias: Parkinson's disease (PD), Huntington's disease (HD), and progressive supranuclear palsy (PSP). Although many others could be covered here, these three have been tied most closely to the concept of subcortical dementia. The article also summarizes common neuropsychologic features shared by subcortical dementias, overviews the anatomic and functional organization of frontal-striatal systems, considers the validity of using “subcortical dementia” as a classification label, and discusses clinical implications for assessing these patients.

Section snippets

PARKINSON'S DISEASE

Idiopathic PD is a common neurologic disorder, characterized by resting tremor, rigidity (especially “cogwheel”), akinesia, and postural as well as gait abnormalities. In addition, psychiatric disturbances are common, especially depression, which may predate neuropsychological symptoms. Some PD groups show more significant decline in cognitive abilities than others, which has led investigators to distinguish between “demented” and “nondemented” variants. In addition, there is evidence that some

HUNTINGTON'S DISEASE

Huntington's disease (HD) is an inherited progressive neurologic disease characterized by choreiform (involuntary spasmodic) and athetotic (writhing) movements. Psychiatric symptoms, including depression and psychosis, are also common and may precede motor manifestations of the disorder.24 In contrast to PD, HD invariably leads to the development of generalized dementia; thus, HD strictly conforms to characteristics of subcortical dementia only during the early to middle stages, prior to the

PROGRESSIVE SUPRANUCLEAR PALSY

PSP is also called Steele-Richardson-Olszewski syndrome, after the investigators who first defined it as a distinct clinical syndrome in 1964.110 It is also the primary disorder on which Albert and colleagues1 originally based the concept of “subcortical dementia.” Albert et al1 described PSP patients as having characteristic “forgetfulness,” slowed cognitive processing, psychiatric changes, and problems manipulating acquired knowledge. In addition to cognitive problems, clinical motor features

SHARED NEUROPSYCHOLOGICAL FEATURES OF SUBCORTICAL DEMENTIAS

Although PD, HD, and PSP groups share a number of deficits, not all are useful in differentiating them from the so-called cortical dementias, such as AD. For instance, both subcortical dementias and AD commonly show abnormalities in visuospatial processes,90 and performance on immediate and delayed free recall memory tests is often similar.89 The specific pattern of memory and executive dysfunction, however, does differ and this provides a means to functionally segregate cortical and

ANATOMIC AND FUNCTIONAL ORGANIZATION OF THE FRONTAL-STRIATAL SYSTEM

The past years have witnessed a tremendous growth in knowledge of the structural and functional interconnectivity between the basal ganglia and cortex.3, 126 A basic grasp of the organization of these networks is critical to understanding the impact of brain dysfunction on cognition in subcortical dementias (see elsewhere in this issue). The cortical—basal ganglia connections are grouped into a number of segregated parallel circuits that originate with projections from widespread regions of the

VALIDITY OF “SUBCORTICAL DEMENTIA” AS A CLASSIFICATION LABEL

Some discussion is necessary regarding the weaknesses and strengths of “subcortical dementia” as a clinical classification label, as its use has been challenged on a number of grounds. For instance, close analysis of anatomic changes in dementia indicate that the term “subcortical” is probably too general.124 Subcortical dementia groups have cortical involvement in the frontal cortex and possibly even in the medial temporal cortex.59 They also have significant loss of ascending cholinergic

CLINICAL CONSIDERATIONS

The value of the label “subcortical dementia” probably comes primarily from its ability to accurately differentiate groups of patients in clinically useful ways. Although it certainly cannot account for the inherent complexity of these diverse disorders, consideration of certain key features can provide an effective means of classifying cognitive dysfunction. It can be beneficial to determine if dementia is more characteristic of a subcortical profile or more similar to an AD-type dementia.

CONCLUSION

“Subcortical dementias” are a heterogeneous group of disorders that share the relatively specific disruption of frontal-striatal system functions. This article discussed three prototypical subcortical dementias—Parkinson's disease, Huntington's disease, and progressive supranuclear palsy. Although there are important cognitive differences among these disorders, they share certain characteristic features that are useful in discriminating them from the so-called cortical dementias, such as

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    Address reprint requests to Cary Savage, PhD, Department of Psychiatry, Cognitive Neuroscience Group, 149–9102, Massachusetts General Hospital, Building 149, 13th Street, Charlestown, MA 02129

    This work was supported in part by a grant from the National Institute of Mental Health (01230).

    *

    From the Cognitive Neuroscience Group and Biostatistics, Department of Psychiatry, Massachusetts General Hospital, Charlestown; and the Department of Psychiatry, Harvard Medical School, Boston, Massachusetts

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    Copyright © 1997 W. B. Saunders Company. Published by Elsevier Inc. All rights reserved.