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PubMed2004

Disentangling the web: neologistic perseverative errors in jargon aphasia.

This article explores the relationship between the neologisms and perseverative errors produced by KVH, a man with severe neologistic jargon aphasia. Detailed examination of KVH's level of language processing breakdown revealed mild difficulties with phonological encoding and severe difficulties accessing the lexical form of the word. Many of KVH's neologisms contained phonemes perseverated from previous neologisms, suggesting an integral relationship between the production of neologisms and the perseveration of phonemes. Furthermore, KVH's patterns of whole word (total)and phonological (blended) perseverations reflected his proposed underlying language processing deficits, consistent with recent literature on perseveration (e.g.,Cohen and Dehaene, 1998). However, the simple binary distinction oftotalandblendedperseveration is proposed to be somewhat limited for understanding the underlying nature of KVH's complex neologistic errors. Possible explanations regarding the mechanisms underlying the production of KVH's neologistic and perseverative errors also are discussed.

Aged↗
PubMed2005

Improved naming after TMS treatments in a chronic, global aphasia patient--case report.

We report improved ability to name pictures at 2 and 8 months after repetitive transcranial magnetic stimulation (rTMS) treatments to the pars triangularis portion of right Broca's homologue in a 57 year-old woman with severe nonfluent/global aphasia (6.5 years post left basal ganglia bleed, subcortical lesion). TMS was applied at 1 Hz, 20 minutes a day, 10 days, over a two-week period. She received no speech therapy during the study. One year after her TMS treatments, she entered speech therapy with continued improvement. TMS may have modulated activity in the remaining left and right hemisphere neural network for naming.

Aphasia↗
PubMed2005

Sign language aphasia following right hemisphere damage in a left-hander: a case of reversed cerebral dominance in a deaf signer?

Recent lesion studies have shown that left hemisphere lesions often give rise to frank sign language aphasias in deaf signers, whereas right hemisphere lesions do not, suggesting similar patterns of hemispheric asymmetry for signed and spoken language. We present here a case of a left-handed, deaf, life-long signer who became aphasic after a right-hemisphere lesion. The subject exhibits deficits in sign language comprehension and production typically associated with left hemisphere damaged signers. He also exhibits evidence of local versus global deficits similar to left-hemisphere lesioned hearing patients. This case represents reversed lateralization for sign language and also may represent reversed lateralization for visuo-spatial abilities in a deaf signer.

Aphasia↗
PubMed2005

Atypical progressive supranuclear palsy underlying progressive apraxia of speech and nonfluent aphasia.

Progressive supranuclear palsy (PSP) is a clinicopathological entity typically presenting as an akinetic rigid syndrome with early falls, axial rigidity, vertical supranuclear gaze palsy and levodopa resistance. Pathological features consist of tau deposition in neuronal and glial cells located mainly in subcortical and brainstem structures. Rare cases with the pathological diagnosis of atypical PSP have been described in which neocortical tau deposition is more widespread than what is usually seen in typical PSP. Progressive nonfluent aphasia (PNFA) is a syndrome characterized by spontaneous nonfluent speech and early preserved comprehension of language. Apraxia of speech (AOS) is a motor speech disorder that may be a feature of PNFA. We report the clinical and pathological findings of four cases that presented with features most consistent with PNFA predominated by AOS. Pathological features in these four cases included the typical features of PSP subcortically and in brainstem structures, but combined with tau-positive neuronal and glial pathology in the neocortex. Comprehensive semiquantitative analyses of tau burden including neurofibrillary tangles and pretangles, coiled bodies, tufted astrocytes and threads were undertaken in the four cases of atypical PSP and compared to 10 cases of typical PSP. Semiquantitative analysis demonstrated that in atypical PSP, the pathology shifts from subcortical grey and brainstem regions, commonly affected in typical PSP, towards neocortical regions. This shift in pathology accounts for the presentation of PNFA and AOS observed in our patients, as well as the lack of classic features of PSP. These cases demonstrate that atypical PSP can present as AOS and PNFA without the classic features of PSP.

Adult↗
PubMed2005

Cognitive and neural mechanisms underlying reading and naming: evidence from letter-by-letter reading and optic aphasia.

We report detailed analysis of language performance in a patient, RMI, a 55-year-old man who presented with a homonymous hemianopsia, optic aphasia, and alexia without agraphia (with letter-by-letter reading) acutely after stroke. MRI showed infarct in the left occipital and medial temporal lobe and hypoperfusion of the entire posterior cerebral artery territory, including the splenium. Extensive language testing revealed severely impaired picture naming and oral reading, with relatively spared tactile naming and recognition of orally spelled words, consistent with impaired access to lexical and semantic representations from vision. In addition, he had a milder deficit in accessing lexical representations for output from all input modalities. RMI's execution of various language tasks provided considerable insight into the mechanisms that underlie oral reading. His performance indicated that both semantic access and orthographic to phonologic conversion mechanisms were partially intact. When information from these two impoverished systems was coupled (the picture of an object presented with its written name), his ability to read/name improved significantly, consistent with the hypothesis that partially accessed semantic information from vision can combine with partially accessed sublexical orthographic to phonologic conversion mechanisms to access phonological representations for output. Furthermore, his written word and picture recognition improved to normal at a time when magnetic resonance perfusion imaging demonstrated reperfusion of the splenium. We interpret these results, as well as results from previous studies in the literature, within a model of the neural regions critical for various cognitive processes underlying reading.

Aphasia↗
PubMed2006

From progressive nonfluent aphasia to corticobasal syndrome: a case report of corticobasal degeneration.

In a previous report, we presented longitudinal clinical, cognitive and anatomical data of a right-handed woman, whose clinical picture evolved from progressive nonfluent aphasia with apraxia of speech to corticobasal syndrome (CBS) in the last stage of the disease. The patient died at age 57 and pathological examination revealed severe atrophy in the left frontal operculum and left premotor area. On histological examination, there was diffuse tau-positive pathology in gray and white cortical hemispheric gray and white matter, basal ganglia and substantia nigra, compatible with corticobasal degeneration (CBD). This case demonstrates the clinical overlap between frontotemporal lobar degeneration and CBD. In this case, early motor speech impairment predicted earlier and more accurately than CBS the presence of underlying tau-pathology and CBD.

Aphasia, Primary Progressive↗
PubMed2006

Non-fluent progressive aphasia, depression, and OCD in a woman with progressive supranuclear palsy: neuroanatomical and neuropathological correlations.

This paper details the case of a 64-year-old woman who presented to the psychiatry service with worsening mood in the context of a diagnosis of obsessive-compulsive disorder (OCD). On further examination she was found to have clinical findings consistent with frontotemporal lobar degeneration of the non-fluent progressive aphasia subtype. At post-mortem she was found to have progressive supranuclear palsy. We argue, in retrospect, that her OCD was likely prodromal to the development of her dementia. This case highlights the fact that frontotemporal lobar degeneration/progressive supranuclear palsy (FTLD/PSP) and other "tauopathies" represent a complex group of neurodegenerative disorders that may masquerade for many years as refractory psychiatric disorders.

Activities of Daily Living↗
PubMed2006

Functional imaging before and after constraint-induced language therapy for aphasia using magnetoencephalography.

Five patients with chronic aphasia underwent functional imaging using magnetoencephalography (MEG) before and after constraint-induced language therapy (CILT). Patients who responded well to CILT exhibited a greater degree of late MEG activation in posterior language areas of the left hemisphere and homotopic areas of the right hemisphere prior to therapy than those who did not respond well. Response to CILT, however, was positively correlated with the degree of pre-therapy MEG activity within posterior areas of the right hemisphere only on an individual basis.

Aged↗
PubMed2004

Preservation of reasoning in primary progressive aphasia: further differentiation from Alzheimer's disease and the behavioral presentation of frontotemporal dementia.

Primary Progressive Aphasia (PPA) is a clinical dementia syndrome characterized by the gradual dissolution of language without impairment of other cognitive domains for at least the first 2 years of illness (M.-M. Mesulam, 1982, 2001). It is difficult to demonstrate the integrity of nonlanguage domains in PPA because most neuropsychological tests of memory, reasoning, and attention require language competence for their performance. In the present study, reasoning and cognitive flexibility were tested nonverbally in patients with PPA using a modified ten-item version of the Visual Verbal Test (Feldman & Drasgow, 1959). This test measures the ability to detect similarities among objects and to sort a single set of objects according to two different principles. The performance of PPA patients (n = 20) was compared with that of patients with dementia of the Alzheimer type (AD) (n = 20), patients with the comportmental/executive dysfunction variant of frontotemporal dementia (FTD) (n = 16), and cognitively intact controls (n = 20). Patients with PPA and controls performed similarly, detecting commonalities among objects and shifting from one sorting principle to another. In contrast, both AD and FTD subjects were significantly impaired on both measures. These results provide evidence of preserved reasoning in PPA, further differentiating this syndrome from other behaviorally focal dementia syndromes.

Aged↗
PubMed2000

Transient high altitude expressive aphasia.

Transient focal neurological deficits have been described in sojourners to high altitude. We present two cases of transient expressive aphasia in well-acclimatized high altitude climbers. We speculate that this type of transient focal neurological impairment may represent migraine aura, and we discuss other reports of transient focal neurological deficit at high altitude.

Adult↗
PubMed1978

Bilateral tactile aphasia: a tacto-verbal dysfunction.

Bilateral tactile aphasia was exhibited by a patient who was operated upon for a left parieto-occipital haematoma. Neuropsychological investigation established the following points: (1) the patient, in whom no expressive or receptive dysphasia could be found, misnamed objects when they were presented to him tactually, whereas he almost always gave the correct name when they were presented visually or auditorily; (2) the naming disturbance was identical when the object was presented to the left hand or to the right hand; (3) not only did the patient have no sensory deficit, but he could give unquestionable proof of correct tactile identification by using the objects presented to him tactually; (4) the defect appeared in the verbo-tactile as well as in the tacto-verbal direction; (5) it was not restricted to the name of the object since the patient was unable to describe, without making dysphasic errors, the morphology or usage of objects presented to him tactually; (6) the tacto-verbal dysfunction did not result from a tacto-visual impairment. Computerized tomography scans showed that: (1) there was no evidence suggesting a lesion of the right hemisphere, nor of the corpus callosum; (2) the left lesion involved the angular gyrus, the posterior part of the second temporal convolution, the inferior longitudinal fasciculus, the geniculostriate fibres and some fibres of the tapetum.

Agnosia↗
PubMed1987

The double dissociation of short-term memory for lists and sentences. Evidence from aphasia.

We document the sentence and list repetition skills of 3 aphasic patients. Cases 1 and 2 were classified as span-impaired conduction aphasics and Case 3 was classified as a span-preserved transcortical sensory aphasic. We found that repetition by the span-impaired cases was facilitated by increasing the 'meaningfulness' of lists, whereas this had no effect on the performance of the span-preserved case. The patients' ability to repeat sentences was contrasted with their ability to repeat three words contained in the sentences, and a double dissociation was obtained: the span-impaired cases were better at repeating sentences than in repeating three word lists whereas the span-preserved case showed the opposite pattern of difficulty. The repetition of sentences containing a known and unknown vocabulary was tested in the span-preserved case. His repetition of sentences containing words which he still knew was significantly better than his ability to repeat sentences containing a vocabulary of words which he had 'forgotten' as a consequence of his aphasia. The patients' ability to repeat complete, incomplete, and nonsense sentences was contrasted: overall the span-impaired patients were somewhat better at repeating complete sentences than incomplete sentences. Finally we examined the patients' ability both to comprehend and repeat word strings in an abbreviated 'naming from description' task: the span-impaired patients were able to comprehend three word strings, but unable to repeat them, the span-preserved case was able to repeat but not comprehend. On the basis of these double dissociations we conclude that the evidence indicates two dissociable short-term memory systems, one a relatively passive phonological store subserving list repetition, the other a dynamic, anticipatory, and integrative memory system which underpins sentence repetition.

Adult↗
PubMed1988

Acute transcortical mixed aphasia. A carotid occlusion syndrome with pial and watershed infarcts.

Four of 1,200 consecutive patients with their first stroke showed acute transcortical mixed aphasia (TMA) characterized by nonfluent speech with impaired naming, semantic paraphasias, echolalia, impaired comprehension, good repetition, reading, and writing on dictation. All 4 had left internal carotid artery (ICA) occlusion with ipsilateral anterior pial territory infarction (precentral-central sulcus artery territory) and watershed infarction between the middle and posterior cerebral artery territories, which spared and 'isolated' the perisylvian speech areas. Although rare, acute TMA is highly suggestive of infarction due to ICA occlusion, in that it is probably related to simultaneous embolism (anterior pial infarction) and haemodynamic insufficiency (posterior watershed infarction).

Acute Disease↗
PubMed1990

Heterogeneity in progressive aphasia due to focal cortical atrophy. A clinical and PET study.

Six patients with a slowly progressive aphasic disorder due to degenerative cortical disease are reported. The length of history varied from 18 months to 6 years. In 5 patients, there was a naming deficit, associated in 4 of these with some abnormalities in comprehension. One patient had a progressive history of reduced speech output, which was associated with an orofacial dyspraxia. All patients were studied neuropsychologically, and in addition regional cerebral metabolism was measured using position emission tomography. A localized left hemisphere deficit was found in all patients except 1, who had the longest history and the most profound clinical deficit, and who also had right hemisphere hypometabolism. The major brunt of the deficit was in the anterior portion of the left temporal lobe in all but 1 case, the patient with poor speech output who had a more severe left posterior frontal deficit. These findings suggest that in progressive aphasia due to focal cortical atrophy there is heterogeneity in the clinical presentation and anatomical site of the deficit, with possible implications for the underlying aetiology.

Adult↗
PubMed1992

Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy.

We report five patients with a stereotyped clinical syndrome characterized by fluent dysphasia with severe anomia, reduced vocabulary and prominent impairment of single-word comprehension, progressing to a stage of virtually complete dissolution of the semantic components of language. A marked reduction in the ability to generate exemplars from restricted semantic categories (e.g. animals, vehicles, etc.) was a consistent and early feature. Tests of semantic memory demonstrated a radically impoverished knowledge about a range of living and man-made items. In contrast, phonology and grammar of spoken language were largely preserved, as was comprehension of complex syntactic commands. Reading showed a pattern of surface dyslexia. Autobiographical and day-to-day (episodic) memory were relatively retained. Non-verbal memory, perceptual and visuospatial abilities were also strikingly preserved. In some cases, behavioural and personality changes may supervene; one patient developed features of the Kluver-Bucy Syndrome. Radiological investigations have shown marked focal temporal atrophy in all five patients, and functional imaging by single positron emission tomography and positron emission tomography (one case) have implicated the dominant temporal lobe in all five. In the older literature, such cases would have been subsumed under the rubric of Pick's disease. Others have been included in series with progressive aphasia. We propose the term semantic dementia, first coined by Snowden et al. (1989), to designate this clinical syndrome.

Adult↗
PubMed1996

Quantification of cortical atrophy in a case of progressive fluent aphasia.

A patient with a rapidly developing fluent progressive aphasia was tested prospectively up to the time of death and examined neuropathologically. Severe impairment in accessing semantic skills with substantially intact phonological, syntactic and discourse skills was found. Some social behavioural difficulties were also noted. This case presented a unique opportunity to relate this significant language impairment to the pattern of neurodegeneration, a difficult task in most neuropathological studies of severe end-stage dementia. A detailed neuropathological examination revealed focal atrophy with neuronal loss without neuronal inclusions (Pick bodies, Lewy bodies, neurofibrillary tangles or senile plaques) or neuronal changes (shrinkage or swelling). In addition, spongiform degeneration (confined to layer two of the cortex) and gliosis were detected at atrophic sites. To establish the amount of tissue loss and pathology associated with the focal language deficit, volume analyses were performed and compared with two age- and sex-matched, neurologically normal controls. Both the left and right angular gyri and Brodmann's area 37 showed marked volume reduction compared with controls. The predominant language impairment seen in this case is likely to reflect these marked changes in the posterior parieto-temporal areas. The milder unilateral atrophy was concentrated in the right temporal lobe as well as the right hemisphere homologue of Broca's area. Recent work suggests a relationship between such unilateral changes and the social behavioural difficulties which were noted in this case. The hippocampus and other gyri such as the supramarginal gyrus showed no volume loss compared with controls correlating with the relative preservation of other language skills.

Aged↗
PubMed1996

Neurolinguistic and follow-up study of an unusual pattern of recovery from bilingual subcortical aphasia.

We report on the neuropsychological and neurolinguistic features of a bilingual patient, E.M., who presented with an uncommon pattern of aphasic deficit consequent to subcortical lesions mainly involving the left basal ganglia. Not only are reports of bilingual subcortical aphasia rare, but E.M.'s deficit is particularly uncommon for it concerns the most used mother tongue (Venetian) much more than a less practiced second language (standard Italian). In this patient, the linguistic deficit in mother tongue production has been observed in spontaneous speech and in cross language translation tasks, where an asymmetrical paradoxical performance has been revealed. Indeed, unlike neurologically intact subjects, E.M. has more difficulties when translating into her mother tongue than into her second language. Although E.M.'s mother tongue is prevalently an oral language, the asymmetrical translation pattern is similar in written and oral translation tasks, thus ruling out the possibility that the deficit simply reflects differences between written and oral language codes. Finally, another remarkable feature of E.M.'s impairment is its stability over almost 5 years from the stroke. We propose that this unusual type of recovery in E.M. is related to the higher degree of automatization of the first language with respect to the second one. This proposal fits with the role of basal ganglia in automatized motor and cognitive performance.

Aged↗
PubMed1999

Electrophysiological manifestations of open- and closed-class words in patients with Broca's aphasia with agrammatic comprehension. An event-related brain potential study.

This paper presents electrophysiological data on the on-line processing of open- and closed-class words in patients with Broca's aphasia with agrammatic comprehension. Event-related brain potentials were recorded from the scalp when Broca patients and non-aphasic control subjects were visually presented with a story in which the words appeared one at a time on the screen. Separate waveforms were computed for open- and closed-class words. The non-aphasic control subjects showed clear differences between the processing of open- and closed-class words in an early (210-375 ms) and a late (400-700 ms) time-window. The early electrophysiological differences reflect the first manifestation of the availability of word-category information from the mental lexicon. The late differences presumably relate to post-lexical semantic and syntactic processing. In contrast to the control subjects, the Broca patients showed no early vocabulary class effect and only a limited late effect. The results suggest that an important factor in the agrammatic comprehension deficit of Broca's aphasics is a delayed and/or incomplete availability of word-class information.

Adult↗