Familial benign chorea with intention tremor: a clinical entity.
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Following a unique infarction, restricted to the left anterior insula and the adjacent part of the intrasylvian frontal opercular cortex, an 83-year-old right-handed patient acutely developed a severe speech disorder that evolved into mere mutism within a few hours. After rapid recovery from mutism, oral language was characterized by severe apraxia of speech. In-depth language investigations further disclosed an isolated, highly selective disturbance of the spelling system (phonological agraphia) which resolved rapidly. One year after onset of neurological symptoms, the apraxia of speech had almost completely receded. The anatomoclinical findings in this first representative of pure and nearly isolated phonological agraphia complement previous neuroanatomical and neurolinguistic accounts of phonological agraphia. The data not only seem to enrich current insights in the anatomical locus for phonological agraphia, they also seem to contribute to a further delineation of the insular role in phonologically mediated aphasic manifestations.
Among 1848 young men appearing before the Danish draft board, 232 (13%) were left-handed. Of these, 118 (51%) used an inverted, or hook-like, writing posture, 49 (21%) used a non-inverted posture and the remaining 65 (28%) could not be categorized. There were no differences between left- and right-handers on a battery of four cognitive tests. However, inverted left-handers performed significantly or near-significantly better than the non-inverted left-handers on two of the four tests and significantly better on the total score for the test battery. These results support the contention that the inverted posture is adaptive for left-handers and suggest that it may be more likely to be adopted by those with better cognitive abilities. Our findings conflict with earlier reports from two decades ago, however, and the association may therefore be socially and culturally dependent.
This study reports the unusual writing performance of a 72-year-old woman, Pp, who was unable to maintain her writing within a particular case, and made numerous case mixing errors (e.g. insTEAd). Her oral spelling was flawless, but when tested using a written spelling task (including words and non-words), a picture description task, a sentence writing task and a transcription task (from upper case to lower case, and vice versa), she showed a high proportion of case mixing errors, though spelling per se was correct. The problem, although less severe, was also present when spelling with letter cards. Despite a spared ability to select the correct letters in the stimulus, specify the correct number of letters and place them in the correct order, she had a specific problem in selecting the appropriate case. This impairment is indicative of peripheral dysgraphia. She appeared to have intact orthographic representations, but selection of the appropriate allographic codes for upper and lower case was impaired. Her longitudinal neuropsychological profile and MRI evidence suggest that Pp suffers from mild vascular dementia with more severe impairments in attentional functions. Her writing deficits may be accounted for by a model that incorporates an inhibitory mechanism, which is responsible for maintaining the appropriate case and inhibiting the inappropriate one, subsequent to case selection in writing. Failure of this mechanism would explain the pattern of case mixing shown by Pp.
This paper reports the results obtained from a writing task given to 23 Italian patients suffering from mild to moderate dementia of the Alzheimer's type (DAT). Spelling performance was tested with a task that taps the sub-word-level (spelling of regular words and nonwords), and the lexical route (spelling of regular and irregular words), in line with contemporary models of writing. Each patient's performance was classified according to the emergence of dissociated patterns of damage between regular words and nonwords and between regular and irregular words. The 23 DAT patients span the whole spectrum of dysgraphic taxonomy; five showed the characteristic pattern of impairment of surface dysgraphia, two showed the characteristics of phonological dysgraphia, while a mixed pattern (i.e. better performance on regular words compared to irregular words and regular nonwords) emerged in seven cases. Three patients presented undifferentiated writing disorders, two were completely agraphic, while four patients showed only minimal or no writing defects. The rate of dissociated impairments in the lexical and the sub-word-level routine is very similar to that observed after acute focal brain damage, which contradicts the hypothesis that degenerative brain damage selectively impairs writing performance along the lexical-semantic route. To test the hypothesis that surface sub-word-level processing abilities are affected only during the evolution of the disease, nine patients were tested longitudinally after an interval of 6-12 months. Once again, the data showed high variability across subjects, and do not seem to support involvement of the sub-word-level spelling routine only at a late stage in the development of the disease.
We assessed writing abilities in a cohort of 31 patients with a diagnosis of DAT (in two subgroups, with minimal [MMSE 24-28] and mild [MMSE 16-23] levels of dementia), and 10 matched controls. Central aspects of writing were assessed by both written and oral spelling to dictation of 72 single words varying in frequency (high or low) and predictability of sound-to-spelling correspondences (predictable, unpredictable and irregular). All subjects achieved better scores on high, as compared to low, frequency words. The performance of both patient groups was significantly affected by degree of predictability, and was equivalent in the written and oral spelling conditions. Phonologically acceptable alternative spellings (e.g. 'wade'-->WAID) constituted the majority of errors. More peripheral processes in writing were assessed by copying and cross-case transcription of single letters. Subjects were more successful at copying within case than transcribing across case. Performance was also better--substantially so for the mild DAT group--when the target response in either task was an upper- rather than a lower-case letter. There was considerable heterogeneity in performance on the spelling and the letter tasks. Some patients (even in the more affected DAT group) were unimpaired on both tasks, suggesting that dysgraphia is not a constant feature in early DAT. When writing deficits do become apparent, in the earliest stages of the disease the pattern is most likely to be one of mild surface dysgraphia, a form of central dysgraphia; impairments in more peripheral aspects of writing tend to emerge once the disease has progressed beyond the minimal stage.
The relationship between measures (of size or function) on one side of the brain, in relation to the difference between the two sides on that measure, are important components of theories of hemispheric asymmetry. For example, it has been concluded that increasing lateralization (e.g., of hand skill or planum temporale area) occurs at the expense of the non-dominant hemisphere. Here it is demonstrated that such relationships could merely be a necessary consequence of relating components of a laterality index to the index (L - R)/(L + R) itself, or indeed to L - R. An alternative approach (using random data to exemplify the null hypothesis) is presented together with an application to data on hand skill from 12,782 11-year-olds in a cohort study. This demonstrates a symmetry hitherto undocumented of maximal hand skill in left and right hands in left- and right-hand writers respectively, the point of the maximum falling short of the population mean for relative hand skill in either case. If degrees of laterality are what is genetically determined, this suggests that selection is present for a function (perhaps language) associated with a greater magnitude of lateralization than is represented by hand skill.
The mechanism of mirror writing was investigated using legal Chinese characters and illegal pseudocharacters. It was found from the results of right-handers in experiment 1 that the performance of normal writing and mirror writing for legal characters was better than that of normal writing and mirror writing for pseudocharacters. A reasonable explanation for this character-superiority effect is that normal engrams and mirror engrams exist only for legal characters but not for pseudocharacters. Further analysis revealed that the character-superiority effect took place in normal writing only when the right hand was used and the same effect was observed in mirror writing only when the left hand was used. It seemed that the normal engrams used in normal writing were stored in the left hemisphere while the mirror engrams used in mirror writing were stored in the right hemisphere. The results in experiment 2 from the left-handers showed the similar pattern as that of the right-handers. The mirror-engram hypothesis seems to be the best mechanism to account for the performance difference of right hand and left hand in mirror writing.
This study examined the grammatical content of written sentences elicited from 96 Parkinson's patients, 30 Parkinson's with dementia patients and 167 control subjects. Parkinson's patients without dementia or with mild dementia presented no impairments in sentence length, syntactic complexity or amount of information content. Moderately demented Parkinson's patients showed reduced sentence length and information content but normal syntactic complexity. This pattern of results provides evidence that lexical-semantic content is more susceptible to decline than syntactic structure with the progression of dementia in Parkinson's disease.
As students proceed through late elementary and junior high school, academic demands change drastically. The altered requirements may give rise to seemingly new learning disorders or may aggravate or modify a preexisting recognized dysfunction. This article discusses common areas of disability in this age group, developmental complications, and academic performance delays, and presents guidelines for assessment and management.
Since the beginning of this century it has been documented that cerebellar lesions induce speech deficits but these were thought to result from lack of motor coordination in the muscular activity needed for phonation. The pure motor nature of the cerebellum has been challenged on different grounds, and cerebellar activation has been documented in language-related tasks independently from motor activity. This chapter reviews the available evidence in favor of a cerebellar contribution to linguistic processing, focusing mainly on clinical observations in patients. The clinical findings are discussed in the light of recent theories on cerebellar functions.
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