A Case of Progressive Apraxia of Speech in Pathologically Verified Alzheimer Disease : Cognitive and Behavioral Neurology

Journal Logo

Case Reports

A Case of Progressive Apraxia of Speech in Pathologically Verified Alzheimer Disease

Gerstner, Elizabeth MD; Lazar, Ronald M. PhD; Keller, Christian MD; Honig, Lawrence S. MD, PhD; Lazar, Gloria S. MS; Marshall, Randolph S. MD

Author Information
Cognitive and Behavioral Neurology 20(1):p 15-20, March 2007. | DOI: 10.1097/WNN.0b013e31802b6c45

Abstract

Objective 

To present the case of a man with progressive speech loss and other clinical features and diagnostic tests consistent with fronto-temporal dementia but whose postmortem neuropathologic findings revealed Alzheimer disease (AD).

Background 

Progressive apraxia of speech presents without true language abnormalities, usually seen with frontal lesions and not associated with AD pathology.

Method 

We describe the clinico-pathologic case of an 87-year-old man with progressive loss of speech function and present the prospective presentation of his syndrome using structural (magnetic resonance imaging) and metabolic (positron emission tomography) neuro-imaging studies, neuropsychologic testing, and pathology.

Results 

His syndrome was characterized over the first 6 to 9 years by progressive deterioration of speech production, alteration of mood, and dysphagia but near normal language, memory, and visual-spatial function. At 8 years, fluorodeoxyglucose-positron emission tomography showed largely frontal metabolic abnormality. Over his final 1½ years, he was mute and withdrawn. Neuropathologic findings showed neuritic plaques and neurofibrillary tangles, but no signs of frontotemporal dementias such as Pick bodies or ubiquitinated tau-negative inclusions.

Conclusions 

There can be overlap in the presentation of fronto-temporal dementia and AD despite the disparate pathologic bases of the underlying diseases. It has yet to be determined how to differentiate these diseases in such variant presentations and whether such atypical AD syndromes are equally amenable to standard therapies for AD.

© 2007 Lippincott Williams & Wilkins, Inc.

You can read the full text of this article if you:

Access through Ovid