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Other Cerebral Disorders of Language
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It would be incorrect to conclude that the syndromes described above, all of which are related to perisylvian lesions of the dominant cerebral hemisphere, represent all the ways in which cerebral disease disturbs language The effects on speech and language of diffuse cerebral disorders, such as delirium tremens and Alzheimer disease, have already been mentioned (see pages 364 and 417) Pathologic changes in parts of the cerebrum other than the perisylvian regions may secondarily affect language function The lesions that occur in the border zones between major cerebral arteries and effectively isolate perisylvian areas from other parts of the cerebrum fall into this category (transcortical aphasias, see above) Other examples are the lesions in the medio-orbital or superior and lateral parts of the frontal lobes, which impair all motor activity, to the point of abulia or akinetic mutism (page 359) The mute patient, in contrast to the aphasic one, emits no sounds If the patient is less severely hypokinetic, his speech tends to be laconic, with long pauses and an inability to sustain a monologue Extensive occipital lesions will, of course, impair reading, but they also reduce the utilization of all visual and lexical stimuli Deep cerebral lesions, by causing uctuating states of inattention and disorientation, induce fragmentation of words and phrases and sometimes protracted, uncontrollable talking (logorrhea) Also common in global or multifocal cerebral diseases are defects in prosody, both expressive and receptive These appear in numerous states that affect global cerebral function, such as Alzheimer disease as well as with lesions of the nondominant (right) perisylvian region as noted in Chap 22 Severe mental retardation often results in failure to acquire even spoken language, as pointed out in Chaps 28 and 38 If there is any language skill, it consists only of the understanding of a few simple spoken commands The subject of developmental dyslexia is discussed on page 510
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Subcortical Aphasia (Thalamic and Striatocapsular Aphasias)
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A lesion of the dominant thalamus, usually vascular and involving the posterior nuclei, may cause an aphasia, the clinical features of which are not entirely uniform Usually there is mutism initially and comprehension is impaired During the early phases of recovery, spontaneous speech is reduced in amount; less often, speech is uent and paraphasic to the point of jargon Reading and writing may or may not be affected Anomia has been described with a ventrolateral thalamic lesion (Ojemann) Characteristically, the patient s ability to repeat dictated words and phrases is unimpaired, (transcortical sensory aphasia) Complete recovery in a matter of weeks is the rule
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Approach to the Patient with Language Disorders
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In the investigation of aphasia, it is rst necessary to inquire into the patient s native language, handedness, and previous level of literacy and education For many years it has been taught that following the onset of aphasia, individuals who had been uent in more than one language (polyglots) improved more quickly in their native language than in a subsequently acquired one (a derivative of Ribot s law of retained distant memory) This rule seems to hold
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if the patient is not truly uent in the more recently acquired language or has not used it for a long time More often, the language most used before the onset of the aphasia will recover rst (Pitres law) Usually, if adequate testing is possible, more or less the same aphasic abnormalities are found in both the rst and the more recently acquired language Many naturally left-handed children are trained to use the right hand for writing; therefore, in determining handedness, one must ask which hand is preferred for throwing a ball, threading a needle, sewing, or using a tennis racket or hammer, and which eye is used for sighting a target with a ri e or other instrument It is important, before beginning the examination, to determine whether the patient is alert and can participate reliably in testing, since accurate assessment of language depends on these factors One should quickly ascertain whether the patient has other gross signs of a cerebral lesion such as hemiplegia, facial weakness, homonymous hemianopia, or corticosensory loss When a constellation of these major neurologic signs is present, the aphasic disorder is usually of the total (global) type A right brachiofacial paralysis aligns with Broca s aphasia; in contrast, a restricted right hemianopia or quadrantanopia is a common accompaniment of Wernicke s aphasia, and hemiparesis is absent Dyspraxia of limbs and speech musculature in response to spoken commands or to visual mimicry is generally associated with Broca s aphasia and sometimes with Wernicke s aphasia Bilateral or unilateral homonymous hemianopia without motor weakness tends often to be linked to pure word-blindness, to alexia with or without agraphia, and to anomic aphasia The bedside analysis of aphasic disorders that we nd most useful entails the systematic testing of six aspects of language function: conversational speech, comprehension, repetition, reading, writing, and naming Simply engaging the patient in conversation permits assessment of the motor aspects of speech (praxis and prosody), uency, and language formulation If the disability consists mainly of sparse, laborious, non uent speech, it suggests, of course, Broca s aphasia, and this possibility can be pursued further by tests of repeating from dictation and by special tests of praxis of the oropharyngeal muscles Fluent but empty paraphasic speech with impaired comprehension is indicative of Wernicke s aphasia Impaired comprehension but perfectly normal formulated speech and intact ability to read suggest the rare syndrome of pure worddeafness When conversation discloses virtually no abnormalities, other tests may still be revealing The most important of these are reading, writing, repetition, and naming Reading aloud single letters, words, and text may disclose the dissociative syndrome of pure word-blindness Except for this syndrome and pure word mutism (see above), writing is disturbed in all forms of aphasia Literal and verbal paraphasic errors may appear in milder cases of Wernicke s aphasia as the patient reads aloud from a text or from words in the examiner s handwriting Similar errors appear even more frequently when the patient is asked to explain the text, read aloud, or give an explanation in writing Testing the patient s ability to repeat spoken language is a simple and important maneuver in the evaluation of aphasic disorders As with other tests of aphasia, it may be necessary to increase the complexity of the test from digits and simple words to complex words, phrases, and sentences in order to disclose the full disability Defective repetition occurs in all the major forms of aphasia (Broca s, Wernicke s, and global) due to lesions in the perisylvian language areas The patient may be unable to repeat
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what is said to him, despite relatively adequate comprehension the hallmark of conduction aphasia Contrariwise, normal repetition in an aphasic patient (transcortical aphasia) indicates that the perisylvian area is largely intact In fact, the tendency to repeat may be excessive (echolalia) Preserved repetition is also characteristic of anomic aphasia and occurs occasionally with subcortical lesions Disorders con ned to naming, other language functions (reading, writing, spelling, etc) being adequate, are diagnostic of amnesic, or anomic, aphasia and referable usually to lower temporal lobe lesions These de cits can be quanti ed by the use of any one of several examination procedures Those of Goodglass and Kaplan (Boston Diagnostic Aphasia Examination, or BDAE) and of Kertesz (Western Aphasia Battery, or WAB) are the most widely used in the United States The use of these procedures will enable one to predict the type and localization of the lesion in approximately twothirds of the patients, which is not much better than detailed bedside examination Using these tests, aphasia of the Broca, Wernicke, conduction, global, and anomic types accounted for 392 of 444 unselected cases studied by Benson Treatment The sudden onset of aphasia would be expected to cause great apprehension, but except for cases of pure or almost pure motor disorders of speech, most patients show remarkably little concern It appears that the very lesion that deprives them of speech also causes at least a partial loss of insight into their own disability This reaches almost a ludicrous extreme in some cases of Wernicke s aphasia, in which the patient becomes indignant when others cannot understand his jargon Nonetheless, as improvement occurs, many patients do become discouraged Reassurance and a program of speech rehabilitation are the best ways of helping the patient at this stage Whether contemporary methods of speech therapy accomplish more than can be accounted for by spontaneous recovery is still uncertain Most aphasic disorders are due to vascular disease and trauma, and they are nearly always accompanied by some degree of spontaneous improvement in the days, weeks, and months that follow the stroke or accident A Veterans Administration Cooperative Study (Wertz et al) has suggested that intensive therapy by a speech pathologist does hasten improvement Also, Howard and colleagues have shown increased ef cacy of word retrieval in a group of chronic stable aphasics treated by two different techniques More studies of this type, which control for the effects of the patient s motivation and the interest of family and therapist, are needed In an interesting personal experiment by Wender, a classicist who had become aphasic, practice of Greek vocabulary and grammar led to recovery in that language, but there was little recovery of her facility with Latin, which was not similarly exercised One must decide for each patient when speech training should be started As a rule, therapy is not advisable in the rst few days of an aphasic illness, because one does not know how severe or lasting it will be Also, if the patient suffers a severe global aphasia and can neither speak nor understand spoken and written words, the speech therapist is virtually helpless Under such circumstances, one does well to wait a few weeks until one of the language functions has begun to return Then the physician and therapist can begin to help the patient to use that function to a maximum degree In milder aphasic disorders, the patient may be sent to the speech therapist as soon as the illness has stabilized The methods of language rehabilitation are specialized, and it is advisable to call in a person who has been trained in this eld
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