CHAPTER 3 DISORDERS OF LANGUAGE
There are two main schools of thought in the history of neurogenic language disorders, both of which have relevance to modern aphasiology. The first, the Wernicke-Lichtheim-Geschwind tradition, emphasized that primary language functions are represented in discrete regions of cortex (“centers”) and that the activities of these loci are integrated through connecting fiber tracts. The Wernicke-Lichtheim scheme consisted of a center for motor images of words, located in the posterior third of the inferior frontal convolution (Broca’s area), as well as a center for acoustic images of words (Wernicke’s area). A fiber tract (arcuate fasciculus) joined the two centers, with the flow of information running from posterior to anterior. A third center for concepts was located in the extrasylvian cortex, with an outflow to Broca’s area and input from Wernicke’s area. There was an output from Broca’s area to the motor cortex and an input to Wernicke’s area from the auditory cortex. This simple scheme systematized the main perisylvian and transcortical aphasia syndromes observed before 1885 and predicted the existence of a syndrome as yet unobserved at that time: conduction aphasia. The Wernicke-Lichtheim model was later refined by members of the Boston School, principally Frank Benson and Norman Geschwind, with the addition of three new syndromes, and the inclusion of the inferior parietal lobule as language cortex (see Benson and Ardila, 1996).
The second main stream is represented by neurologists such as Hughlings Jackson, Sigmund Freud, and Aleksandr Luria who conceived of language as represented in broader hierarchical cortical zones or gradients, organized around the centers of the Wernicke-Lichtheim model. The role of connecting tracts was deemphasized. Luria’s aphasiology preserves the anteroposterior schema of the Wernicke-Lichtheim model but redefines localization of language as hierarchical and distributed. Modern clinical aphasiology is based on the classical syndromes described in the Wernicke-Lichtheim tradition and their modifications. Concepts of their localization, however, have come to be shaped further by ongoing clinicopathological observation and functional neuroimaging, and current views on the localization of language are not too distant from those of the Jackson-Freud-Luria tradition. Linguistic ideas have also become part and parcel of modern aphasiology. A glossary of important linguistic terms and concepts is given in Table 3-1.
Phonological | Phonology is the study of the structure and patterning of the sounds of language. Many of the clinical features of language disorders are phonological. The units of analysis are phonemes, which can be defined as the basic meaning-distinguishing sounds (essentially consonant and vowel sounds) of the language. |
Lexical | Whereas phonology is concerned with the patterning of sounds within a word, and is therefore a sublexical discipline, lexicology is concerned with the whole word as a single entity. At a lexical level, there is a dichotomy that is fundamental to the understanding of aphasic disorders, because aphasic variants affect the two classes of words differentially. The open class, so-called because it is conceptually unlimited and new items are steadily added as vocabularies increase, consists principally of nouns, verbs, adverbs, and adjectives—that is, words that refer to specific objects, actions, and attributes and that convey substantive content. The closed class, which is conceptually limited and does not increase in size as vocabularies increase, consists of articles (e.g., “a,” “the,” “that”), conjunctions (e.g., “and,” “but”), pronouns (e.g., “you,” “they”), and prepositions (e.g., “up,” “along,” “below”). The distinction between open- and closed-class words parallels the distinction between the meaning of a sentence (semantics) and the form or sequential structure of a sentence (syntax). |
Morphological | Morphology concerns the internal structure of words. The aspect of morphology that is most important for understanding language disturbances is word formation, or the construction of a new word from an existing word by adding an affix. This can be derivational, whereby an adjective such as “good” is converted to a noun such as “goodness,” or inflectional, whereby a word is changed to suit the grammar of a sentence; for example, “run” might become “running,” or “pencil” might become “pencils.” Because the suffixes “-ing” and “-s” cannot stand alone, they are referred to as bound morphemes. |
LANGUAGE PRODUCTION
At a clinical level, language disorders are more easily recognized and identified in language production, either spoken or written, than in disturbances of comprehension. Production consists of three broad stages: conceptualization, formulation, and overt execution. The first two of these stages are described in detail in the following sections. From a neuroanatomical perspective, conceptualization (the development of an intention to speak, and a decision about what will be said) depends on the dorsolateral prefrontal cortex. Formulation (the conversion of ideas into the structure of spoken language) depends on Broca’s region. Execution is the production of physical speech and depends on all of the motor mechanisms associated with speech (see Duffy, 2005).
Conceptualization
This is a largely prelinguistic phase that involves the development of an intention to speak and a decision as to what will be said. Development of an intention and a decision about the message to be conveyed is often referred to as macroplanning. From that point on, the message must be reshaped into a particular set of logical relationships (propositions) that can be expressed in terms of the syntactic and semantic structure of language. This is often referred to as microplanning. Propositions form a link between thought and its expression in language. It is noteworthy that this concept was anticipated by the British neurologist Hughlings Jackson, who regarded it as essential for understanding the relationship between thought and speech in aphasia. Conceptualization depends on connectivity between extrasylvian association cortex and the classical perisylvian language axis, a concept borne out by functional neuroimaging (see Blank, Scott, Murphy, et al, 2002).
Formulation
This phase deals with the conversion of propositions into actual sentences (sentence encoding). It is governed directly by the rules of syntax and semantics and consists of two important components. The first involves the selection of appropriate open class of lexical items (see Table 3-1) to convey the intended meaning. The linguistic concept of selection is of central importance in aphasic disorders. Selection implies the possibility of choice among alternatives, and errors in selection manifest clinically as paraphasias. A paraphasia has two essential features: (1) It is an error of selection resulting in the substitution of a word or part of a word with a frequently incorrect or inappropriate alternative, and (2) it is unintended. Selection processes occur at the phonemic and the semantic levels (see Table 3-1). In neuroanatomical terms, selection processes are heavily, but not exclusively, dependent on posterior perisylvian association cortices. The second component of formulation involves the genesis of correctly ordered positional slots into which the words of the sentence are inserted. These sequentially ordered schemas are often called sentence frames, and their construction is contributed to and defined by closed class (function) words and bound morphemes (see Table 3-1) Sentence frames are constructed according to the rules of syntax (see Table 3-1). The functional neuroanatomy of syntactic processing is complex, involving a network of left perisylvian structures, and it appears that Broca’s area is a key nodal structure within this network. Disorders of syntax, including Broca’s aphasia, occur most prominently with lesions involving the anterior aspects of the perisylvian language zone.
Fluency
The division of language disturbances into fluent and nonfluent is the most fundamental and clinically appreciable dichotomy in diagnostic aphasiology. The major aphasia syndromes are encompassed within the distinction of fluent versus nonfluent (see Table 3-2).
Nonfluent Production | Fluent Production | |
---|---|---|
Anatomical | Anterior (prerolandic) language areas | Posterior (postrolandic) language areas |
Fundamental disorder | Sequential organization (conceptualization, formulation) | Selection |
Syndrome | Perisylvian | Perisylvian |
Aphemia | Pure word deafness | |
Broca’s aphasia | Wernicke’s aphasia | |
Extrasylvian | Conduction aphasia | |
Transcortical motor aphasia | Extrasylvian | |
Transcortical sensory aphasia | ||
Anomic aphasia |
Note: Mixed transcortical aphasia and global aphasia are associated with clinically nonfluent production but also involve an underlying selection disorder.
Fluent language output in an aphasic patient is defined by the use of sentences that are syntactically intact but are semantically compromised because of a selection disorder. The following example, taken from a description of a severe form of fluent dysphasia, namely jargonaphasia (a variant of Wernicke’s aphasia), illustrates this point:1
Paraphasia and Other Deviations
Paraphasias
Paraphasias are defined as unintended utterances. In essence, there is a failure of selection at the phonemic level, producing a phonemic (literal) paraphasia (e.g., “I drove home in my lar”) or at a word (lexical) level (e.g., “I drove home in my wagon”), producing a verbal paraphasia (Table 3-3). Paraphasias are said to be neologistic when the unintended word is heavily contaminated with extraneous phonemes and, as a result, contains juxtapositions of sublexical fragments that are not characteristic of the language (phonemic neologisms) and are nonsensical in context. For example:5
Type | Examples |
---|---|
Phonemic (literal) | “glear” instead of “clear” |
“spink” instead of “sphinx” | |
“gedrees” instead of “degrees” | |
“tums” instead of “tongs” | |
Conduit d’approche (successive phonemic approximations to a target word) | “trep”→“tretz”→“fretful”→“pretzel” |
Verbal | |
Formal (similar form, different meaning) | “dare” instead of “pear” |
Morphemic (assembled from legal morphemes) | “man-a-time,”* “summer-ly” |
Semantic (substituted word belongs to the same general category) | “train” instead of “car” |
“Taj Mahal” instead of “pyramid” | |
“cloth” instead of “blanket” | |
“seahorse” instead of “unicorn” | |
Circumlocutions (word substituted with a phrase of the same meaning) | “drinking container” instead of “cup” |
* Morphemic assemblies that do not produce acceptable words are called neologisms.
EXAMINER: Are you feeling better than this morning?
PATIENT: Not too melsise, I don’t think.
Unintended substitutions also occur in writing (paragraphias). Like paraphasias, they can be literal or they can involve semantic substitutions (see Table 3-3).
APHASIA SYNDROMES
Contrary to earlier views, more recent tractography findings indicate that the arcuate fasciculus consists of two components. The first is a direct tract connecting the posterior segments of the inferior and middle temporal gyri with Broca’s area (Brodmann’s areas 44 and 45), as well as with parts of the middle frontal gyrus and inferior precentral gyrus. The second component is an indirect tract with anterior and posterior segments. The posterior segment connects Wernicke’s area with the inferior parietal lobule (Brodmann’s areas 39 and 40), whereas the anterior segment connects the inferior parietal lobule with frontal language cortex (see Catani et al, 2005). Structures connected by the arcuate fasciculus are somewhat more extensive than classical views suggested, and this broader concept of the perisylvian region accommodates the clinicopathological studies of fundamental language disorders since the 1950s more successfully. Furthermore, it suggests that the arcuate fasciculus might be important in uniting perisylvian and extrasylvian language regions.
Aphasias caused by anterior (prerolandic) lesions are associated with nonfluent language production, whereas those caused by posterior lesions (postrolandic) are associated with fluent disorders. There are two major nonfluent aphasias: Broca’s aphasia, in which repetition is disturbed, and transcortical motor aphasia (TMA), in which repetition is normal. The fluent aphasias are Wernicke’s and conduction aphasias, in which repetition is disturbed, and anomic and transcortical sensory aphasias (TSA), in which repetition is preserved (Table 3-4) (see LaPointe, 2005). In addition, there are two aphasias in which the dysfluency typical of anterior dysphasias is combined with the impaired comprehension typical of posterior dysphasias: global aphasia and mixed transcortical aphasia.
Impaired Repetition | Normal Repetition |
---|---|
Nonfluent | |
Broca’s* | Transcortical motor |
Global† | Mixed transcortical |
Fluent | |
Conduction* | Anomic |
Wernicke’s† | Transcortical sensory |
Nonfluent Production with Impaired Repetition: Speech Dyspraxia and Broca’s Aphasia
Speech Dyspraxia (Aphemia)
The syndrome of speech dyspraxia occurs quite separately from the other anterior aphasias. There is, however, a widely recognized dictum in aphasiology that lesions restricted to Broca’s area do not necessarily cause Broca’s aphasia. Embolic infarctions of Brodmann’s areas 44 and 45, often involving subjacent white matter and extension into the anterior insula, cause a wide spectrum of acute effects ranging from subtle hesitancy to mutism. Recovery is rapid—within days, weeks, or months—and in some cases, minimal residual dysfluency may be the only detectable language feature. Dyspraxia of facial, oropharyngeal, lingual, and respiratory functions is an associated feature that might persist beyond the resolution of language deficits, manifesting, in many cases, with some features reminiscent of the syndrome of speech dyspraxia. The chronic picture is “deficits in the smoothness with which vocalization of one phoneme in a series can be ceased and changed to the next, in precise control of the respiratory component of vocalization, and/or in precise positioning of the oral cavity to produce desired phonemes … better explained by inadequacy in skilled execution of movements, an apraxia in speaking … but not an associated disorder in language usage.”6 This resembles Luria’s idea of efferent motor aphasia, in which the primary disorder relates to skilled sequential movements or kinetic melodies in which the patient is able to position the articulators correctly but is not capable of moving smoothly from one articulatory position to the next.7 Originally, Pierre Paul Broca used the term aphemia to refer to this condition. There appears to be a revival in the use of this term in relation to progressive speech disturbances. Michael Alexander’s group at Boston University has taken the view that aphemia is a distinctive syndrome arising from small lesions in the left inferior frontal gyrus (pars opercularis), inferior precentral gyrus, and underlying white matter.8
The main features of speech dyspraxia are shown in Table 3-5.
Functional neuroanatomy of speech dyspraxia
It is now recognized that articulatory function depends on a hierarchically organized network of structures involving cerebellum, thalamus, striatum, anterior insula, and sensorimotor cortex. Current concepts of Broca’s area, particularly the posterior portion (Brodmann’s area 44) abutting on the precentral sulcus (Brodmann’s area 6), include the view that it mediates the encoding of phonological word forms into articulatory plans. This places it at the apex of the articulatory hierarchy. Broca’s area also shows increased activity during syntactic processing, although lesions restricted to this area cause impairments in speaking rather than language,6 a view dramatically foreshadowed by the neurologist Pierre Marie in 1906 with a paper titled “The Third Frontal Convolution Does Not Play Any Special Role in the Function of Language” (see Harrington). Functional neuroimaging findings have raised the possibility that Broca’s area represents a bridge between articulation and language production.