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CARDINAL MANIFESTATIONS OF NEUROLOGIC DISEASE
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Pure Word-Blindness (Alexia without Agraphia, Visual Verbal Agnosia) This is a not uncommon syndrome, in which a literate person loses the ability to read aloud, to understand written script, and, often, to name colors ie, to match a seen color to its spoken name visual verbal color anomia Such a person can no longer name or point on command to words, although he is sometimes able to read letters or numbers Understanding spoken language, repetition of what is heard, writing spontaneously and to dictation, and conversation are all intact The ability to copy words is impaired but is better preserved than reading, and the patient may even be able to spell a word or to identify a word by having it spelled to him or by reading one letter at a time (letter-by-letter reading) In some cases, the patient manages to read single letters but not to join them together (asyllabia) The most striking feature of this syndrome is the retained capacity to write uently, after which the patient cannot read what has been written (alexia without agraphia) When the patient with alexia also has dif culty in auditory comprehension and in repeating spoken words, the syndrome corresponds more closely to Wernicke s aphasia Autopsies of such cases have usually demonstrated a lesion that destroys the left visual cortex and underlying white matter, particularly the geniculocalcarine tract, as well as the callosal connections of the right visual cortex with the intact language areas of the dominant hemisphere (page 409) In the case originally described by Dejerine (1892), the disconnection occurred in the pos terior part (splenium) of the corpus callosum, wherein lie the connections between the visual association areas of the two hemispheres (see Fig 22-6) More often the callosal pathways are interrupted in the forceps major or in the paraventricular region (Damasio and Damasio) In either event, the patient is blind in the right half of each visual eld by virtue of the left occipital lesion, and visual information reaches only the right occipital lobe; however, this information cannot be transferred, via the callosal pathways, to the language area of the left hemisphere A rare variant of this syndrome takes the form of alexia without agraphia and without hemianopia A lesion deep in the white matter of the left occipital lobe, at its junction with the parietal lobe, interrupts the projections from the intact (right) visual cortex to the language areas but spares the geniculocalcarine pathway (Greenblatt) This lesion, coupled with one in the splenium, prevents all visual information from reaching the language areas, including the angular gyrus and Wernicke s area In yet other cases, the lesion is con ned to the angular gyrus or the subjacent white matter In such cases also, a right homonymous hemianopia will be absent, but the alexia may be combined with agraphia and other elements of the Gerstmann syndrome ie, right-left confusion, acalculia, and nger agnosia (page 402) This constellation of symptoms is sometimes referred to as the syndrome of the angular gyrus Anomic aphasia may be added (see below) Pure Word Mutism (Aphemia, Pure Motor Aphasia of Dejerine) Occasionally, as a result of a vascular lesion or other type of localized injury of the dominant frontal lobe, the patient loses all capacity to speak while retaining perfectly the ability to write, to understand spoken words, to read silently with comprehension, and to repeat spoken words Right facial and brachial paresis may be associated From the time speech becomes audible, language may be syntactically complete, showing neither loss of vocabulary nor agrammatism; or there may be varying degrees of dysarthria (hence cortical dysarthria ), anomia, and paraphasic
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substitutions, especially for consonants The most notable feature of this type of speech disorder is its transience; within a few weeks or months, language is restored to normal Bastian and more recently other authors have called this syndrome aphemia, a term that was used originally by Broca in another context to describe the severe motor aphasia that now carries his name Probably the syndrome is closely allied to the mini-Broca s aphasia described on page 417 The anatomic basis of pure word mutism has not been determined precisely In a few postmortem cases, reference is made to a lesion in Broca s area Damasio and Geschwind have stated that the lesion is anterior and superior to this area A well-studied case has been reported by Roch-LeCours and Lhermitte Their patient uttered only a few sounds for 4 weeks, after which he recovered rapidly and completely From the onset of the stroke, the patient showed no disturbance of comprehension of language or of writing Autopsy disclosed an infarct that was con ned to the cortex and subjacent white matter of the lowermost part of the precentral gyrus; Broca s area, one gyrus forward, was completely spared Other cases have involved mainly Broca s area Anomic (Amnesic, Nominal) Aphasia Some degree of word nding dif culty is probably part of every type of language disorder, including that which occurs with the confusional states and dementia In fact, without an element of anomia, a diagnosis of aphasia is usually incorrect Only when this feature is the most notable aspect of language dif culty is the term anomic aphasia employed In this latter condition, a relatively uncommon form of aphasia, the patient loses only the ability to name people and objects There are typical pauses in speech, groping for words, circumlocution, and substitution of another word or phrase that is intended to convey the meaning Perseveration may be prominent Or the patient may simply fail to name a shown object, in contrast to the usual aphasic patient, who produces a paraphasic error Less frequently used words give more trouble When shown a series of common objects, the patient may tell of their use, or demonstrate the same, instead of giving their names The dif culty applies not only to objects seen but to the names of things heard or felt (Geschwind), but this is more dif cult to demonstrate In addition to displaying normal uency of spontaneous speech and preserved comprehension and repetition, the patients we have seen with anomic aphasia have been surprisingly adept in spelling Beauvois and coworkers have described a form of bilateral tactile aphasia due to a left parieto-occipital lesion in which objects seen and verbally mentioned could be named, but not those felt with either hand Recall of the names of letters, digits, and other printed verbal material is almost invariably preserved, and immediate repetition of a spoken name is intact That the de cit is principally one of naming is shown by the patient s correct use of the object and, usually, by an ability to point to the correct object on hearing or seeing the name and to choose the correct name from a list The patient s understanding of what is heard or read is normal There is a tendency for patients to attribute their failure to forgetfulness or to give some other lame excuse for the disability, suggesting that they are not completely aware of the nature of their dif culty Of course, there are patients who fail not only to name objects but also to recognize the correct word when it is given to them In such patients, the understanding of what is heard or read is not normal, ie, the naming dif culty is but one symptom of another type of aphasic disorder
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