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This chapter addresses the following Geriatric Fellowship Curriculum Milestone: #60


Learning Objectives

  • Learn about the epidemiology, common clinical presentations, diagnosis, and treatment of non-Alzheimer type of neurodegenerative diseases.

  • Gain new knowledge about recent discoveries related to the genetics, pathology, and pathobiology of common tau- and α-synucleopathies in older adults.

  • Learn about the specific behavioral and nonbehavioral symptoms, clinical signs, diagnostic criteria and common neuroimaging, genetic and laboratory tests used to diagnose non-Alzheimer type diseases of dementia.

  • Understand the scientific rationale, indications and limitations of currently available and emerging therapies for common non-Alzheimer–type neurodegenerative diseases.

Key Clinical Points

  1. The evaluation of neurodegenerative disorders includes careful attention to the cognitive, behavioral, and motor symptoms along with a thorough neurologic examination.

  2. In older age, it becomes increasingly common for there to be more than one type of pathology causing a dementia syndrome.

  3. The characteristic features of Lewy body dementia include cognitive impairments with profound fluctuation, spontaneous parkinsonism, random eye movement (REM) behavior sleep disorder, and visual hallucinations.

  4. About 30% of patients with Parkinson disease develop dementia. In these patients, unlike Alzheimer disease, the motor and other symptoms of Parkinson disease generally predate dementia by many years.

  5. The dominance of behavioral and personality changes in the absence of memory and perceptual symptoms is highly suggestive of the behavioral variant of frontotemporal dementia (FTD).

  6. History of falls and dysphagia with abnormalities in vertical gaze and preserved oculocephalic reflex is suggestive of a diagnosis of progressive supranuclear palsy (PSP).

Alzheimer disease (AD) is the most common neurodegenerative disorder encountered by the practicing geriatrician, however, a sizable number of other neurodegenerative diseases will be seen in a typical practice, rendering a working knowledge of these disorders critical for clinicians. Furthermore, there is increasing evidence that with greater age, many individuals die with mixed pathology—with AD, vascular changes, frontotemporal lobar degeneration (FTLD), and Lewy body changes often seen in a single brain. This chapter provides an overview of the more common neurodegenerative disorders with emphasis on those that influence behavior and cognition early in the course. We begin by reviewing the clinical approach to neurodegenerative cognitive disorders and then review the clinical presentation, epidemiology, and examination findings of the more common neurodegenerative syndromes. We attempt to link clinical presentation to anatomy and neuropathology whenever possible.


The evaluation of neurodegenerative disorders is multifaceted, requiring careful attention to the cognitive, behavioral, and motor history combined with a comprehensive neurologic examination aiming to identify the brain regions involved. Isolating anatomy in patients who present with slowly progressive neurodegenerative disorders greatly facilitates the determination of the correct diagnosis.

Emphasis should be placed on the earliest presenting symptoms, whether cognitive, behavioral, or motor in origin. These early features may be critical to the identification of the pathologic substrate. As diseases progress, signs ...

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