NoteProgressive aphasia presenting with deep dyslexia and dysgraphia
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
References (29)
- et al.
The arcuate fasciculus and the disconnection theme in language and aphasia: History and current state
Cortex
(2008) The anatomo-functional connectivity of language revisited: New insights provided by electrostimulation and tractography
Neuropsychologia
(2008)- et al.
The dissolution of language in Pick’s disease with neurofibrillary tangles: A case study
Brain and Language
(1985) - et al.
Do deep dyslexia, dysphasia and dysgraphia share a common phonological impairment?
Neuropsychologia
(2007) - et al.
Syntactic and semantic errors in paralexia
Neuropsychologia
(1966) A modified card sorting test sensitive to frontal lobe defects
Cortex
(1976)- et al.
Diagnosis and rehabilitation attempt of a patient with acquired deep dyslexia
Brain and Cognition
(2003) - et al.
An overview on primary progressive aphasia and its variants
Behavioural Neurology
(2006) - et al.
Measuring dysgraphia. A graded-difficulty spelling test
Behavioural Neurology
(1994) TROG. Test of Reception of Grammar
(1989)