Elsevier

Cortex

Volume 48, Issue 9, October 2012, Pages 1234-1239
Cortex

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Progressive aphasia presenting with deep dyslexia and dysgraphia

https://doi.org/10.1016/j.cortex.2012.02.010Get rights and content

Abstract

Primary progressive aphasia is clinically heterogeneous. We report a patient, alias Don, with a novel form of progressive aphasia, characterised by deep dyslexia and dysgraphia and dissociated access to phonological and orthographic word forms. The hallmarks of deep dyslexia and dysgraphia were present early in the course and persisted over time. Writing was initially poorer than reading, but this reversed over time. There was a lack of concordance between reading and writing errors. Don benefited from a semantic mediation strategy to learn letter sounds, involving associating letters with a country name (e.g., A = Afghanistan). Remarkably, he continued to be able to generate those phonologically complex country names when no longer able to name or sound letters. Don’s performance is compatible with a traditional dual-route account of deep dyslexia and dysgraphia. The findings have potential practical implications for speech and language therapy in progressive aphasia. Moreover, they illustrate both the remarkable specificity yet clinical diversity in presentation of progressive aphasia.

Introduction

Primary progressive aphasia (PPA) is clinically heterogeneous. Contemporary classifications distinguish three major variants: semantic, non-fluent and logopenic (Amici et al., 2006, Gorno-Tempini et al., 2004, Gorno-Tempini et al., 2011, Grossman, 2010, Grossman and Ash, 2004, Mesulam et al., 2008). Nevertheless, not all patients conform to these prototypical syndromes. We describe a patient who presented with a striking disorder of reading and writing, characterised by deep dyslexia and dysgraphia. We have had the rare opportunity to examine the evolution of his language disorder over 9 years. His case is important because it broadens the range of possible presentations of PPA, highlighting the remarkable specificity with which language systems can be affected. It also has potential implications for therapy.

Section snippets

Initial presentation

At the age of 58 years this male University academic, pseudonym Don, noticed difficulty transcribing telephone messages recorded on his answering machine, and spelling ‘little’ words such as prepositions, conjunctions and auxiliary verbs. He also noticed problems reading hyphenated words that spanned columns. In conversational speech he experienced mild problems in word retrieval. Neither he nor his wife noticed difficulty in comprehension or in other cognitive domains. There was no change in

Methods

Don’s reading, writing and naming skills were monitored longitudinally.

Speech and language therapy: semantic mediation in letter naming and sounding

Speech therapy was implemented approximately 6 months after initial assessment (before test 2). Don reported frustration at being unable to read aloud (i.e., sound out) written words. He was noted spontaneously to attempt to facilitate spoken word reading by placing the word in a meaningful, semantic context (e.g., dinner: “I eat my dinner”). As part of his language therapy, a semantic mediation strategy was implemented with the aim of facilitating access to letter names and sounds, enabling him

Discussion

Don exhibits a progressive disorder of expressive language, associated with left perisylvian atrophy, in keeping with PPA. Unusually, his presenting complaints were predominantly in literacy: difficulty transcribing telephone messages, spelling prepositions and auxiliaries and reading words hyphenated across newspaper columns. Investigations elicited the hallmarks of deep dyslexia and dysgraphia: marked imageability effects, semantic errors in reading and writing and an inability to read and

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