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Language and Language Disorders: Neuroscience to Clinical Practice REVIEW Pract Neurol: first published as 10.1136/practneurol-2018-001961 on 26 July 2019. Downloaded from Language and language disorders: neuroscience to clinical practice Michael O'Sullivan, 1,2 Sonia Brownsett,1,3 David Copland1 1UQ Centre for Clinical ABSTRACT terminology. Finally, the approach to Research, University of Language disorders are common in neurological the language system in medical text- Queensland, Brisbane, Queensland, Australia practice but their accurate recognition and books remains dominated by Wernicke– 2Department of Neurology, description can be challenging. In this review, Lichtheim’s 1874 model of the language Royal Brisbane and Women's we summarise the major landmarks in the system and the notion of canonical Hospital, Herston, Queensland, understanding of language disorders and the aphasia syndromes. This view is outdated Australia 3Institute of Health and organisation of language in the brain. We and often creates misunderstanding. Biomedical Innovation, describe approaches to assessing language Queensland University disorders at the bedside or in the clinic as of Technology, Brisbane, HISTORICAL PERSPECTIVE Queensland, Australia well as the treatment and rehabilitation of Pierre Paul Broca’s first report of the aphasia. Finally, we describe how the field of famous patient Tan was published in Correspondence to neuroscience is providing new computational 1861.5 6 Over the following 4 years, Prof Michael O'Sullivan, UQ and neuroscientific approaches to study the Centre for Clinical Research, Broca expanded and refined his analysis University of Queensland, mechanisms of recovery and rehabilitation of of disorders of articulatory speech. In Building 71/918, Royal Brisbane aphasia. 1863, he reported 10 further cases and Hospital Campus, Herston, in 1865 summarised his conclusions in a 4029, QLD, Australia; m. osullivan1@ uq. edu. au paper titled, ‘On the site of the faculty of 7 INTRODUCTION articulated speech’. Writing just over a Accepted 1 April 2019 decade later,8 David Ferrier made it clear Published Online First Language is pivotal to everyday life and 26 July 2019 to human culture. The flexibility and vast that Broca’s conclusions were, by then, widely accepted: range of possible combinations in human language exceeds the scope of any other The cause of this affection was shown system for vocal communication between by Broca—and his observations have primates.1 Disorders of this system are been confirmed by thousands of other http://pn.bmj.com/ common in everyday neurological prac- cases—to be associated with disease in tice, typically arising from focal injury to the region of the posterior extremity of the third left frontal convolution, where the left hemisphere and also from forms of it abuts on the fissure of Sylvius. selective neuronal degeneration.2 3 Disor- ders of language are disabling and cause Carl Wernicke’s famous contribution to 4 distress to patients, carers and relatives. the understanding of aphasia came later, on September 29, 2021 by guest. Protected copyright. The presence of aphasia also creates diffi- in 1874.9 Wernicke published his paper, culties in case history taking, assessment ‘Der aphasische Symptomencomplex’ at and discussion about treatment options the age of only 26, 4 years after gradu- and decisions. ation and after only 3 years of neurology Despite being common, language disor- training. The crux of Wernicke’s anal- ders are not always straightforward to ysis was that disorders of language could evaluate in the clinic or at the bedside. occur with lesions in other parts of the The presentations are varied and there are brain, not involving the area described known pitfalls, such as the mislabelling of by Broca. He described 10 patients, but fluent aphasia as ‘confusion’. There are the notion of Wernicke’s area arose from various schemes to classify language disor- just one (although another was added as © Author(s) (or their ders, which create overlapping termi- an addendum later): a 75-year-old woman employer(s)) 2019. Re-use nology (eg, the expressive/receptive and whose severe comprehension deficit made permitted under CC BY. Published by BMJ. fluent/dysfluent divisions of aphasia, see some people believe she was deaf. At post- glossary in box 1). Different disciplines mortem, the only focal lesion was in the To cite: O'Sullivan M, Brownsett S, Copland D. have different traditions and approaches first (superior) temporal gyrus on the left. Pract Neurol 2019;19:380– to the analysis of language disorders, Broca’s contributions were made in the 388. which further reduces consistency of French language literature and Wernicke’s 380 O'Sullivan M, et al. Pract Neurol 2019;19:380–388. doi:10.1136/practneurol-2018-001961 REVIEW Pract Neurol: first published as 10.1136/practneurol-2018-001961 on 26 July 2019. Downloaded from context, they were not the only investigators in the Box 1 Glossary field. Between 1861 and 1874, aphasia was an active Alexia without agraphia and rapidly growing area, attracting the attention of Charlton Bastian and Hughlings Jackson in the UK, This is a syndrome characterised by the inability to read 10 with preserved writing. It was conceptualised as a form among others. of disconnection syndrome with a lesion of left primary In 1885, Lichtheim modified Wernicke’s model visual cortex accompanied by a lesion to the splenium of of language by adding a ‘concept centre’ (figure 2). the corpus callosum, cutting off visual input to the angular This extension accommodates the fact that there are gyrus and thereby abolishing reading. Writing is intact several aspects of normal language function in which because left hemisphere language centres remain intact. repetition plays no role, but which do depend on Aphasia other mental processes, for example, in producing a The term aphasia, interpreted literally, should mean monologue based on internal reflections or goals, or silent listening and comprehension. The Wernicke– complete absence of language function. However, this situ- 11 ation is so uncommon in practice that the terms aphasia Geschwind model of the 1960s additionally included and dysphasia have come to be used interchangeably. This a role for the angular gyrus in silent reading (with convention is followed in this article and aphasia has been input to Wernicke’s area) and Heschl’s gyrus (primary adopted for consistency. Complete loss of speech output is auditory cortex) in silent listening (figure 1). more likely to be due to anarthria, that is, a motor disorder of articulation not limited to language. In this respect, and FEATURES OF APHASIA SYNDROMES in contrast to language, anarthria and dysarthria describe Language includes the processes by which thoughts qualitatively different deficits. are translated into an ordered pattern of motor output Dyspraxia of speech producing speech. At the sensory pole, language This term describes a difficulty converting a motor speech processes decode symbols that we see, and sequences plan into a motor speech action. The patient is typically of sound that we hear, and link them to representa- aware, online, of their inaccurate motor actions. This leads tions of words. The observable features of language to the attempts to unsuccessfully self-repair those errors, therefore include syntax, the grammatical structure as they occur, which translates into the frequently observed of sentences, the morphology of words—that is, how oral ‘groping’ of the speech muscles. The acute awareness speech sounds (phonemes) are combined together— and groping features of this disorder are characteristic and and comprehension, based on both the structure of can aid in its differential diagnosis. language and a mental lexicon for words. Aphasia is Pragmatic language “…a breakdown in the two-way translation process that establishes a correspondence between thoughts This refers to the social use of language, rather than the 12 language itself. It pertains to the rules that govern our use and language.” (Mesulam, 2000). It follows that of language in any given context and social interaction. This aphasia has multiple manifestations, affects the http://pn.bmj.com/ includes what, how and why something is said, non-verbal features of linguistic processing that we are able to communication skills (such as eye contact, facial expres- observe during communication and essentially is part sions, body language and so on) and the appropriateness of a two-way breakdown in function. of interactions in a given situation. Importantly, it includes The features of aphasia depend on the underlying the skills with which we ‘repair’ breakdowns in communi- anatomical pattern of pathology. In ischaemic stroke, cation (such as requesting repetition or reforming a ques- the most common cause, the clustering of features into on September 29, 2021 by guest. Protected copyright. tion to clarify interpretation). aphasia syndromes is mostly a function of underlying Surface dyslexia vascular anatomy. The superior division of the left This is a disorder of reading in which people cannot middle cerebral artery supplies the inferior and lateral perceive words as single whole entities. As a result, they part of the frontal lobe (including Broca’s area), with cannot retrieve their pronunciation from memory. Patients infarction typically causing dysfluency, agrammatism with surface dyslexia can pronounce words using pronun- and disruption of motor aspects of language. Impaired ciation rules and therefore pronounce non-words fluently grammatical structure
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