A suspected case of Pick disease with peculiar echolalia accompanied by aphasia was reported. The patient was a right-handed 67-year-old woman. On admission, she was initially categorized into the second stage of classification of Pick disease as defined by Schneider and Braunmuhl. After roughly one year, the case seemed to proceed to the third stage. This report describes the case in detail and discusses the following characteristics of her echolalia : (1) The patient showed moderate or severe mixed transcortical aphasia on admission and advanced to the mutism stage. (2) The types of echolalia observed were mitigated echolalia, complete echolalia and partial echolalia. These also seemed to involve characteristics of “effortful echolalia” (Hadano, et al.). Furthermore the case suggested the presence of peculiar partial echolalia, with symptoms of speaking the first sound of words. (3) Generally the echolalia of cerebrovascular disease takes the following course : mitigated echolalia→complete echolalia→partial echolalia. But the echolalia of the present case took the following course : mix of complete, mitigated and partial echolalia→predominance of partial echolalia→mutism. These findings suggest that in degenerative diseases, three types of echolalia might develop during the same period.

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    A 83-year-old right handed woman developed selective kana agraphia. She showed no neurological sign other than dysarthria and dysphagia. Although dysarthria was striking, auditory comprehension, reading comprehension, naming objects, intellectual and behavioral abilities were well preserved. Brain MRI and SPECT studies could not detect the specific lesions. The clinical course and negative EMG study led us to her diagnosis of progressive aphemia syndrome. The most conspicuous sign of her writing disturbances was characterized by omitting kana letters. We performed some deep test about writing process of kana words to clarify what stage her writing disturbances occurred. It may be reasonably assumed that there are three levels of processing in writing kana : moraic segmentation level, the moraic identification level and the sound-kana correspondence level. She had no problems with the first level and third level.
    When asked to point out where a specific mora was among a word, she always pointed out the position forward to the correct one. We thought this disturbance is closely related to her actual writing disturbances with omission of kana letters. We concluded that she omitted the kana letters because the frame of the kana word decreased between the moraic segmentation level and the moraic identification level.

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    We investigated the daily life of chronic aphasic patients. The subjects were 71 aphasic patients who had undergone speech therapy at our hospital. We created a questionnaire which included items regarding frequency of daily activities, kinds of activities engaged in, problems encountered, and so forth. According to their activities, the subjects were divided into two groups. One was a high-activity group, and the other was a low-activity group. The responses to the questionnaire were analyzed.
    The results were as follows :
    1. The mean age was significantly younger, and the severity of language disorders was relatively mild in the high-activity group.
    2. With respect to social activities with friends or other people and recreation, the patients in the low-activity group tended to feel they had extremely changed from their premorbid situations. Responses showed they are not satisfied with their present condition.
    3. As to hours spent with their families, compared to the low-activity group, the subjects in the high-activity group spend fewer hours with their families. Responses showed they spend their lives more independently. Also, there was a trend that not only patients but also spouses of patients in the high-activity group engage in more social activities. This suggests interactions between patients and their families.
    4. Concerning character, the ratio of extroverted character was higher in the high-activity group than in the low-activity group.

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    The purpose of this study was to investigate working memory : phonological loop function in two patients with selective impairment of auditory-verbal span, using a dual-task paradigm.
    Patient MO showed verbal short-term memory defects without other cognitive dysfunctions. Patient TU closely resembled patients with pure verbal short-term memory defects, except that he had minimal aphasia.
    According to Baddeley's working memory model, verbal short-term memory relies basically upon a specialized “slave” system : the phonological loop. Vallar et al. indicated that selective impairment of verbal short-term memory is conceivably a capacity limitation of the phonological short-term store.
    Our dual-task paradigm was measured by performance on visual checking or reading tasks and during concurrent digit span tasks. We compared performance to that of a group of age-matched controls. Performance measurements of the two patients were normal on tasks requiring central executive function. TU exhibited greater deficits than the control subjects when he was required to rehearse uttering of digits. In reading performance on dual-task conditions, MO needed longer reading time than the control subjects ; TU, however, was unable to continue this task.
    These contrasting patterns of impairment suggest that a “specific” verbal short-term memory defect in TU may be associated with other defects in phonological processing.

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    A 50-year-old right-handed woman with systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS) presented with progressive impairments of the dorsal visual system and other focal cognitive functions that resulted in ultimate dementia.
    A definitive diagnosis of SLE and APS was made in 1993 based on a medical history of difficult pregnancies and recurrent seizures, presence of hematological abnormalities and various autoantibodies, and results of renal and skin biopsies. By 1996, the patient developed Balint's and Gerstmann's syndromes, dressing and constructional difficulties, ideomotor and ideational apraxia, mild amnesia, word finding difficulty, left-side sensorimotor neglect and executive dysfunction, and added afterwardsle signe de la main etrangere” and dementia. Longitudinal magnetic resonance (MR) imaging of the brain revealed diffuse progressive atrophy and parietal white matter changes that extended to the frontal lobes. Single photon emission computed tomography showed diffuse progressive uptake defects that were most prominent in the borderzones. MR angiography was negative. We presume that the underlining immunological abnormalities resulted in progressive microvasculopathy and microinfarcts especially in the bilateral parieto-occipital regions including the dorsal visual system. We also suggest that the dorsal visual system may be associated with the neural fibers passing through the posterior boderzone among three major cerebral arteries and, more specifically, the hemodynamic terminal zone between cortical and deep penetrating arteries

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    In order to examine long-term recovery processes of aphasia, we studied the continuous recovery process of aphasia in various lesion and age-of-onset subgroups. The examination was conducted on 132 right-handed aphasics with localized lesions in the left hemisphere, validated by CT scan or MRI study. Recovery processes of language impairment in each subgroup were examined and compared using the SLTA (Standard Language Test of Aphasia) 10-scale severity ratings.
    Major findings were as follows :
    1) Aphasics with localized lesions in the frontal lobe, putamen, or thalamus showed rapid recovery early after onset. 2) Many aphasics with posterior lesions, extensive lesions in the middle cerebral artery territory, or expanded lesions in the putaminal area showed continuous recovery for more than three years, although the final level of achievement varied in each case. 3) Since language impairment may recover over a long period of time, it is necessary to conduct sustained rehabilitation programs based on appropriate evaluations of prognosis. 4) Because numerous factors affect prognosis, it is imperative to avoid making indiscriminate decisions on the effects of rehabilitation merely on the basis of the period of time from onset.

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    A 60-year-old right-handed female developed non-fluent aphasia and right mild hemiparesis following a traumatic head injury. Magnetic resonance imaging showed multiple cerebral damages in left lateral cerebral cortex. The most striking aphasic feature was disorders of verb retrieval and particle choice in her spontaneous speech and verbal description tasks. On the serial tasks of confrontation naming, pointing by auditory stimulus, and oral use description for 10 usual manipulable objects, her verbal description for object use was selectively and severely impaired, whereas her abilities of oral naming and pointing were perfectly preserved. On the verbal description task for 100 line-drawing action pictures, she correctly produced only 29% of verbs and 44% of particles, in which her errors for verb retrieval were unassociated with those for particle choice. For six months, her verb retrieval and particle production improved. And whether or not she could retrieve the verbs, the correct particles in her description were similarly increased. These data suggest that abilities of verb retrieval and particle choice constitute separate neural structures, although multiple lesions in this patient preclude discussion of the clinico-anatomical correlation.

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    A 49-year-old right-handed Japanese woman exhibited transcortical motor aphasia associated with cerebral hemorrhage. Magnetic resonance imaging revealed a lesion involving the medial aspect of the left frontal lobe, part of the anterior cingulate gyrus, and the body of the corpus callosum.
    This study was undertaken to investigate the mechanism of speech dysfluency in this case. The subject's speech dysfluency was thought to be stuttering. The stuttering features included aspects of both neurogenic and psychogenic stuttering.
    In the present case, the neurogenic stuttering may be caused by (1) neural disconnections between the right and left hemispheres resulting from damage to the body of the corpus callosum, as well as by (2) collapse of the basal ganglia circuits resulting from damage to the prefrontal and pre-supplementary motor areas. We concluded that the neurogenic stuttering may be associated with motor feedback loops from the cortex to the basal ganglia, to the thalamus, and back to the cortex.

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    We reported a patient who manifested optic anomia, tactile aphasia of the left hand, and gustatory (taste) aphasia after cerebral infarction of the left hemisphere. Naming of objects presented to the auditory modality was preserved. Performance in a semantic association test executed under the conditions of visual, tactile and gustatory modalities was preserved. MRI showed a high-intensity area in the callosal radiations running through the splenium of the corpus callosum. The other lesion involved the subcortical regions of the left frontal lobe. The mechanism of both optic anomia and tactile aphasia can be explained as a disconnection of their respective recognition systems from the speech system. We assumed that gustatory aphasia is also caused by disconnection of the taste recognition system (1. gustatory area of the operculum of the parietal lobe and upper border of the insula, 2. semantic memory system of the temporal lobe, and 3. their connecting pathways) from the speech system. Visual naming of objects included in two categories body parts and clothing was significantly better than that of the other eight categories (vegetables, fruits, vehicles, birds, animals, instruments, electrical appliances, flowers). The regions responsible for the naming of body parts seem to involve both the somatotopy (body image) region of the parietal lobe and the pathway from the somatotopy region to the speech system, both of which were preserved in our case.

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