Dementia

 

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Although there has been remarkable progress in our understanding of their pathophysiologies, differentiation of the various dementias continues to challenge clinicians. This issue of Seminars celebrates the intriguing spectrum of dementing and other cognitive disorders. The first article by Drs. Salmon and Filoteo provides an extensive overview of the prominent differences in patterns of cognitive deficits seen in the so-called “cortical” and “subcortical” syndromes. Next, Ron Petersen examines the notion of mild cognitive impairment, a transitional state between the cognitive changes of normal aging and the fully developed clinical features of dementia. He presents criteria for mild cognitive impairment and discusses the clinical outcome of individuals with mild cognitive impairment, as well as genetic, neuroimaging, biomarker, and clinical predictors of progression. The recently completed mild cognitive impairment clinical trials are discussed and the lessons learned from them translated into recommendations for future research.

Roy Yaari and I review Alzheimer's disease (AD), the most common cause of dementia in the elderly. Currently, 4 million individuals in the United States are estimated to have AD, and that number is projected to increase to at least 14 million by the year 2050. Although AD typically presents with an insidious decline in memory that progresses to affect all spheres of cognitive functioning, behavioral and psychiatric symptoms are also frequent. Drs. Weisman and McKeith examine dementia with Lewy bodies, the second most common dementing illness in the elderly. Core clinical features of dementia with Lewy bodies include fluctuating attention, recurrent visual hallucinations, and parkinsonism. Revised criteria for the clinical and pathologic diagnosis of dementia with Lewy bodies, incorporating new information from a recent international consortium on dementia with Lewy bodies, are reviewed. Drs. Graff-Radford and Woodruff discuss frontotemporal dementia, an important form of degenerative disease that may comprise up to 50% of dementia cases presenting before the age of 60. While most cases are sporadic, some are genetic, and the authors examine the known genes and chromosomal loci related to frontotemporal dementia.

Drs. Relkin and Tsakanikas review idiopathic normal pressure hydrocephalus, a treatable neurologic disorder in older adults involving disturbances of gait and balance, control of micturition, and cognition. Diagnosis can be challenging owing its varied presentation and overlap with other disorders common in the elderly. Drs. Aggarwal and DeCarli examine emerging trends in vascular dementia. Vascular dementia is a term used to describe a constellation of cognitive and functional impairment that can be viewed as a subset of the larger syndrome of vascular cognitive impairment associated with cerebrovascular brain injury. Despite a well-recognized etiology, lack of established diagnostic criteria for vascular dementia have hampered effective research into its causes, prognosis, and treatment.

Drs. Benedict and Bobholz discuss the cognitive impairment that commonly accompanies multiple sclerosis (MS). With the advent of standardized neuropsychological testing and quantitative brain imaging, the frequency, quality and correlates of cognitive impairment in MS are becoming increasingly better understood. Finally, Drs. Ances and Ellis review HIV-associated neurocognitive impairment, which is typically subcortical and consists of the triad of cognitive, behavior, and motor dysfunction.

I am greatly indebted to the Editor of Seminars in Neurology for allowing me the opportunity of asking my colleagues, all leaders in their field, to compile this issue that I believe represents a veritable tour de force of the clinical features, diagnosis, and management of dementing and other cognitive syndromes frequently encountered in clinical practice. It is my hope that you will agree and that this issue quickly becomes indispensable in your bedside and office decision making.

Jody Corey-BloomM.D. Ph.D. 

UCSD Department of Neurosciences

3350 La Jolla Village Drive, San Diego, CA 92161