Chapter 027. Aphasia, Memory Loss, and Other Focal Cerebral Disorders (Part 7) Gerstmann's Syndrome The combination of acalculia (impairment of simple arithmetic), dysgraphia (impaired writing), finger anomia (an inability to name individual fingers such as the index or thumb), and right-left confusion (an inability to tell whether a hand, foot, or arm of the patient or examiner is on the right or left side of the body) is known as Gerstmann's syndrome. In making this diagnosis it is important to establish that the finger and left-right naming deficits are not part of a more generalized anomia and that the patient is not otherwise aphasic. When Gerstmann's syndrome is seen in isolation, it is commonly associated with damage to the inferior parietal lobule (especially the angular gyrus) in the left hemisphere. Aprosodia Variations of melodic stress and intonation influence the meaning and impact of spoken language. For example, the two statements "He is clever." and "He is clever?" contain an identical word choice and syntax but convey vastly different messages because of differences in the intonation and stress with which the statements are uttered. This aspect of language is known as prosody. Damage to perisylvian areas in the right hemisphere can interfere with speech prosody and can lead to syndromes of aprosodia. Damage to right hemisphere regions corresponding to Wernicke's area can selectively impair decoding of speech prosody, whereas damage to right hemisphere regions corresponding to Broca's area yields a greater impairment in the ability to introduce meaning-appropriate prosody into spoken language. The latter deficit is the most common type of aprosodia identified in clinical practice—the patient produces grammatically correct language with accurate word choice but the statements are uttered in a monotone that interferes with the ability to convey the intended stress and affect. Patients with this type of aprosodia give the mistaken impression of being depressed or indifferent. Subcortical Aphasia Damage to subcortical components of the language network (e.g., the striatum and thalamus of the left hemisphere) can also lead to aphasia. The resulting syndromes contain combinations of deficits in the various aspects of language but rarely fit the specific patterns described in Table 27-1. In a patient with a CVA, an anomic aphasia accompanied by dysarthria or a fluent aphasia with hemiparesis should raise the suspicion of a subcortical lesion site. Progressive Aphasias In clinical practice, acquired aphasias are most commonly encountered in one of two contexts: CVAs and degenerative diseases. Aphasias caused by CVAs start suddenly and display maximal deficits at the onset. The underlying lesion is relatively circumscribed and associated with a total loss of neural function at the lesion site. These are the "classic" aphasias described above where relatively reproducible relationships between lesion site and aphasia pattern can be discerned. Aphasias caused by neurodegenerative diseases have an insidious onset and relentless progression so that the symptomatology changes over time. Since the neuronal loss within the areas encompassed by the neurodegeneration is partial and since it tends to include multiple components of the language network, distinctive clinical patterns and clinico-anatomic correlations are less obvious. Dementia is a generic term used to designate a neurodegenerative disease that impairs intellect and behavior to the point where customary daily living activities become compromised (Chap. 365). Alzheimer's disease is the single most common cause of dementia. The neuropathology of Alzheimer's disease causes the earliest and most profound neuronal loss in memory-related parts of the brain such as the entorhinal cortex and the hippocampus. This is why progressive forgetfulness for recent events and experiences is the cardinal feature of Alzheimer's disease. In time, the neuronal pathology in Alzheimer's disease spreads to the language network and a progressive aphasia, usually of the anomic type, becomes added to the progressive amnesia. There are other patterns of dementia, however, where neurodegeneration initially targets the language rather than memory network of the brain, leading to the emergence of a progressive aphasia that becomes the most prominent aspect of the clinical picture during the initial phases of the disease. Primary progressive aphasia (PPA) is the most widely recognized syndrome with this pattern of selective language impairment. Clinical Presentation and Diagnosis of PPA The patient with PPA comes to medical attention because of word-finding difficulties, abnormal speech patterns, and spelling errors of recent onset. PPA is diagnosed when other mental faculties such as memory for daily events, visuospatial skills (assessed by tests of drawing and face recognition), and comportment (assessed by history obtained from a third party) remain relatively intact; when language is the major area of dysfunction for the first few years of the disease; and when structural brain imaging does not reveal a specific lesion, other than atrophy, to account for the language deficit. Impairments in other cognitive functions may also emerge, but the language dysfunction remains the most salient feature and deteriorates most rapidly throughout the illness. . Chapter 027. Aphasia, Memory Loss, and Other Focal Cerebral Disorders (Part 7) Gerstmann's Syndrome The combination of acalculia. earliest and most profound neuronal loss in memory- related parts of the brain such as the entorhinal cortex and the hippocampus. This is why progressive forgetfulness for recent events and experiences. onset. PPA is diagnosed when other mental faculties such as memory for daily events, visuospatial skills (assessed by tests of drawing and face recognition), and comportment (assessed by history