Dementia Untangled

STEVEN L. LEWIS, MD, FAAN p. 12-13 February 2019, Vol.25, No.1 doi: 10.1212/01.CON.0000553293.87198.a4
EDITOR’S PREFACE
BROWSE ARTICLES

EDITOR-IN-CHIEF

This issue of Continuum is devoted to the diagnosis and management of our patients with dementia. I am delighted that Dr Jonathan Graff-Radford accepted my invitation to be the guest editor of this issue. I am also so appreciative that Dr Graff-Radford brought together such an esteemed group of experts to share their extensive experience and insights regarding the many causes of dementia we encounter in our practices.

The issue begins with the article by Dr Gil D. Rabinovici on the risk factors, diagnosis (including use of biomarkers), and management of late-onset Alzheimer disease (AD), defined as having an age of onset of 65 years and older and likely the most common cause of dementia we encounter. Dr Mario F. Mendez next reviews early-onset AD and its variants, highlighting the clinical, genetic, and pathologic differences from late-onset AD. Drs Jonathan M. Schott and Sebastian J. Crutch then review the syndrome of posterior cortical atrophy, including its striking clinical presentation, diagnosis, pathologic considerations (most commonly a form of early-onset AD), and the unique management considerations for improving the quality of life of our patients with this disorder.

Dr William W. Seeley next reviews the current clinical, genetic, and pathologic considerations in behavioral variant frontotemporal dementia and provides us with the diagnostic strategies and pitfalls we need to be aware of when we encounter patients with this clinical syndrome. Drs Hugo Botha and Keith A. Josephs then discuss primary progressive aphasias (PPAs)—specifically nonfluent/agrammatic variant PPA and semantic variant PPA—and primary progressive apraxia of speech, focusing on the current classification, evaluation, diagnosis, genetics, and neuropathology of these disorders of language and speech.

Dr Melissa J. Armstrong next reviews the Lewy body dementias, clarifying the terminology and diagnostic considerations of both dementia with Lewy bodies and Parkinson disease dementia and highlighting the management issues we need to be aware of as we treat patients with these overlapping entities. Dr Jonathan Graff-Radford then reviews vascular cognitive impairment, clarifying the most up-to-date terminology for the various vascular syndromes that may be primary causes of dementia as well as pathologies that may coexist with the neurodegenerative causes of dementia discussed in this issue.

Drs Neill R. Graff-Radford and David T. Jones next discuss the pathophysiology and current recommendations on diagnosis and management of normal pressure hydrocephalus, an important, albeit rare, reversible cause of dementia. Drs Katherine W. Turk and Andrew E. Budson then provide a review of the current state of knowledge regarding the clinical features, epidemiology, risk factors, and pathophysiology of chronic traumatic encephalopathy. Drs Gregory A. Jicha and Peter T. Nelson next review hippocampal sclerosis, argyrophilic grain disease, and primary age-related tauopathy, three pathologic entities associated with dementia that many of our readers may not have heard of yet but, it turns out, are surprisingly common and important for us to now have on our diagnostic radar. In the final review article of the issue, Dr Gregory S. Day uses a number of very instructive brief illustrative cases to discuss the diagnosis and management of the very important reversible causes of dementia.

In the Medicolegal Issues article, Ms Rachel V. Rose and Dr Joseph S. Kass provide a familiar scenario as a prologue to their practical and clinically relevant discussion regarding the issues neurologists need to consider when prescribing antipsychotic medications to patients with dementia, especially given the US Food and Drug Administration (FDA) boxed warnings for their use in this scenario.

After reading the issue and taking the Postreading Self-Assessment and CME Test written by Drs D. Joanne Lynn and Allyson R. Zazulia, you may earn up to 20 AMA PRA Category 1 CreditsTM toward self-assessment and CME or, for Canadian participants, a maximum of 20 hours toward the Self-Assessment Program (Section 3) of the Maintenance of Certification Program of the Royal College of Physicians and Surgeons of Canada. Additional credit can be obtained by listening to Continuum Audio interviews associated with this and other Continuum issues, available to all subscribers, and completing tests on the Continuum Audio web platform or app. Continuum Audio is also accredited by the Royal College of Physicians and Surgeons of Canada.

My sincere thanks to Dr Graff-Radford and the renowned dementia experts who contributed to this issue for their state-of-the art and skillful unraveling of the current and rapidly growing knowledge of the clinicopathologic underpinnings and the diagnostic and management considerations of these disorders.

My sincere thanks to Dr Graff-Radford and the renowned dementia experts who contributed to this issue for their state-of-the art and skillful unraveling of the current and rapidly growing knowledge of the clinicopathologic underpinnings and the diagnostic and management considerations of these disorders.

—STEVEN L. LEWIS, MD, FAAN EDITOR-IN-CHIEF

© 2019 American Academy of Neurology.