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Dystypia: isolated typing impairment without aphasia, apraxia or visuospatial impairment.

We report a 60-year-old right-handed Japanese man who showed an isolated persistent typing impairment without aphasia, agraphia, apraxia or any other neuropsychological deficit. We coined the term 'dystypia' for this peculiar neuropsychological manifestation. The symptom was caused by an infarction in the left frontal lobe involving the foot of the second frontal convolution and the frontal operculum. The patient's typing impairment was not attributable to a disturbance of the linguistic process, since he had no aphasia or agraphia. The impairment was not attributable to the impairment of the motor execution process either, since he had no apraxia. Thus, his typing impairment was deduced to be based on a disturbance of the intermediate process where the linguistic phonological information is converted into the corresponding performance. We hypothesized that there is a specific process for typing which branches from the motor programming process presented in neurolinguistic models. The foot of the left second frontal convolution and the operculum may play an important role in the manifestation of 'dystypia'.

Agraphia↗

Clinical and pathological overlap between frontotemporal dementia, primary progressive aphasia and corticobasal degeneration: the Pick complex.

A substantive overlap between the clinical syndromes of frontal lobe dementia (FLD), frontotemporal dementia (FTD), or primary progressive aphasia (PPA), and corticobasal degeneration syndrome (CBDS) has been demonstrated in a population of 55 patients followed for more than 3 years in a cognitive neurology clinic. Patients presenting with the personality behavior disorder (FLD) often develop progressive aphasia (PA) and vice versa. CBDS is often associated with FLD and PA, and the extrapyramidal-apractic syndrome of CBDS often appears in FLD and PPA. The histopathological variations do not predict the clinical phenotype. The term Pick complex is suggested to indicate that these clinical and pathological variations are related and they were first described by Pick as clinical manifestations of fronotemporal atrophy. This term will avoid the confusion of using FLD or FTD or for the whole complex and also for the personality behavioral presentation. The relationship of the various clinical presentations has been strengthened by the discovery of chromosome 17 linkage in families manifesting them.

Aphasia, Primary Progressive↗

Progressive nonfluent aphasia and subsequent aphasic dementia associated with atypical progressive supranuclear palsy pathology.

We describe a right-handed man who developed progressive nonfluent aphasia and apraxia of speech beginning at age 71. By age 74 he had behavioral changes, aphasic dementia, as well as mild parkinsonism; extraocular movements were normal except for mild limitation of upgaze. Serial neuropsychometric testing and single photon emission computed tomography (SPECT) scans showed progressive changes reflecting left>right cerebral hemisphere dysfunction. Neuropathologic examination revealed findings characteristic of progressive supranuclear palsy (PSP) except that the cortical pathology was more widespread than is typical of PSP. We conclude that the clinical manifestations in this case were more similar to the syndrome of progressive nonfluent aphasia with subsequent aphasic dementia and mild parkinsonism, rather than those of PSP. Hence, PSP can present clinically as an atypical dementing syndrome dominated by progressive aphasia/apraxia of speech.

Aged↗

Aphasia and thalamotomy: important issues.

Patients may present with classical symptoms suggesting aphasia following thalamotomy (repetition, comprehension, fluency and naming abnormalities). They may also present with 'freezing of speech', and this symptom should not be considered as a speech disorder or a symptom of Parkinson's disease progression, without careful testing to rule out language deficits, particularly dysfluency. There are important issues related to all language complications of thalamotomy, including (1) the time course of problems following surgery, (2) the impact of preexistingspeech problems, (3) the importance of the size and location of lesions, (4) the potential circuits important in the pathogenesis of a thalamic language disturbance and (5) whether laterality makes a difference (left- versus right-sided thalamic lesions). As more centers switch from thalamotomy to deep brain stimulation, the issues regarding aphasia will need to be addressed.

Aphasia↗

Global aphasia-with and without hemiparesis. A linguistic and CT scan study.

The location and size of brain lesions were compared between two groups of patients showing persisting global aphasia according to the criteria of the Aachen Aphasia test (AAT). The first group comprised 8 patients suffering from severe hemiparesis for more than 18 months. The 10 patients of the second group showed either no hemiparesis at all, or motor disturbance at the onset of the disorder with complete remission within 1 year. The sizes of the brain lesions were determined by means of CT scans. The location of the lesions was defined by a statistical model of the brain with labelled anatomical structure, which allowed an interindividual comparison. In the CT scans, the infarcted areas of the brains of patients with hemiparesis always extended to the wall of the lateral ventricle, thus including the whole corona radiata with the pyramidal tract. The infarcted areas of the patients without hemiparesis and with transient hemiparesis spared parts of the deep white matter. The data obtained via CT scans are of prognostic value for patients with transient hemiparesis, since determination of the extent of the lesion by means of CT permits an initial estimate on the possible later degree and quality of the motor recovery.

Aphasia↗

A case of aphasia following left thalamic hemorrhage.

In a patient without fluent speech, good comprehension, aphasia and frequent neologisms, computerized tomography (CT scan) demonstrated a left posterior thalamic hemorrhage sparing other language areas. We discuss the existence and the anatomical and pathogenetic mechanisms of thalamic aphasia.

Aged↗

Mixed transcortical aphasia: clinical features and neuroanatomical correlates. A possible role of the right hemisphere.

This paper reports the clinical features of 2 patients affected by mixed transcortical aphasia. Both of them had extensive damage of the left hemisphere. Cerebral blood flow measurement showed a marked decrease in left-hemisphere flow. In particular, speech areas were found to be morphologically and functionally damaged. The present findings, together with a critical review of the literature, served as a starting point for a discussion of linguistic features, diagnostic criteria and classical neuroanatomical interpretation of mixed transcortical aphasia. The possible contribution of the right hemisphere in determining this aphasic syndrome is suggested.

Aged↗

Pathogenesis of aphasia in deep-seated lesions: likely role of cortical diaschisis.

In order to study the pathophysiology of language disorders due to deep-seated left-hemisphere lesions not involving the cortex, a population of 43 right-handed stroke patients (29 aphasic) presenting with such lesions was studied clinically and by regional cerebral blood flow measurements (two-dimensional xenon-133 inhalation method). Most of the patients were studied sequentially between the 1st and 3rd months after stroke. Cortical diaschisis did not account for the whole clinical picture: the remote effect on the cortex could explain the occurrence of aphasia and its severity but not the type of aphasia. Furthermore, the clinical improvement observed in most cases was not accompanied by the disappearance of the diaschisis.

Adult↗

Grammaticality judgment in aphasia: deficits are not specific to syntactic structures, aphasic syndromes, or lesion sites.

We examined the abilities of aphasic patients to make grammaticality judgments on English sentences instantiating a variety of syntactic structures. Previous studies employing this metalinguistic task have suggested that aphasic patients typically perform better on grammaticality judgment tasks than they do on sentence comprehension tasks, a finding that has informed the current view that grammatical knowledge is relatively preserved in agrammatic aphasia. However, not all syntactic structures are judged equally accurately, and several researchers have attempted to provide explanatory principles to predict which structures will pose problems to agrammatic patients. One such proposal is Grodzinsky and Finkel's (1998) claim that agrammatic aphasics are selectively impaired in their ability to process structures involving traces of maximal projections. In this study, we tested this claim by presenting patients with sentences with or without such traces, but also varying the level of difficulty of both kinds of structures, assessed with reference to the performance of age-matched and young controls. We found no evidence that agrammatic aphasics, or any other subgroup, are selectively impaired on structures involving traces: Some judgments involving traces were made quite accurately, whereas other judgments not involving traces were made very poorly. Subgroup analyses revealed that patient groups and age-matched controls had remarkably similar profiles of performance across sentence types, regardless of whether the patients were grouped based on Western Aphasia Battery classification, an independent screening test for agrammatic comprehension, or lesion site. This implies that the pattern of performance across sentence types does not result from any particular component of the grammar, or any particular brain region, being selectively compromised. Lesion analysis revealed that posterior temporal areas were more reliably implicated in poor grammaticality judgment performance than anterior areas, but poor performance was also observed with some anterior lesions, suggesting that areas important for syntactic processing are distributed throughout the left peri-sylvian region.

Adolescent↗

ERP effects of subject-verb agreement violations in patients with Broca's aphasia.

This article presents electrophysiological data on on-line syntactic processing during auditory sentence comprehension in patients with Broca's aphasia. Event-related brain potentials (ERPs) were recorded from the scalp while subjects listened to sentences that were either syntactically correct or contained violations of subject-verb agreement. Three groups of subjects were tested: Broca patients (n = 10), nonaphasic patients with a right-hemisphere (RH) lesion (n = 5), and healthy aged-matched controls (n = 12). The healthy, control subjects showed a P600/SPS effect as response to the agreement violations. The nonaphasic patients with an RH lesion showed essentially the same pattern. The overall group of Broca patients did not show this sensitivity. However, the sensitivity was modulated by the severity of the syntactic comprehension impairment. The largest deviation from the standard P600/SPS effect was found in the patients with the relatively more severe syntactic comprehension impairment. In addition, ERPs to tones in a classical tone oddball paradigm were also recorded. Similar to the normal control subjects and RH patients, the group of Broca patients showed a P300 effect in the tone oddball condition. This indicates that aphasia in itself does not lead to a general reduction in all cognitive ERP effects. It was concluded that deviations from the standard P600/SPS effect in the Broca patients reflected difficulties with on-line maintaining of number information across clausal boundaries for establishing subject-verb agreement.

Acoustic Stimulation↗

Diagnosis: schizophrenia versus posterior aphasia.

Schizophrenia and posterior aphasia are easily cross-diagnosed, primarily because of similarities in verbal output, i.e., vagueness, looseness of association, and apparent confusion. Tape-recorded interviews with 8 posterior aphasics and 10 "loose" schizophrenics were transcribed and analyzed to provide guidelines for the clinician to differentiate the two conditions by monitoring verbal expression. Six major differentiating characteristics were identified. The authors present hypotheses that emphasize differing neuroanatomical loci and neuropsychological mechanisms to explain the differences in verbal output in schizophrenia and posterior aphasia.

Aphasia↗

Acquired epileptic aphasia. Diagnostic aspects of progressive language loss in preschool children.

A case of acquired epileptic aphasia presenting as language delay and hyperactivity is presented and clinical aspects reviewed. Acquired epileptic aphasia is a diffuse neurologic process of unknown etiology that presents in preschool children. Although progressive language loss and seizures are the major manifestations, deficits in attention and socialization may be noted. The electroencephalogram is usually diagnostic. While almost all (80%) children develop seizures, approximately half present as language delay without seizures. Recognition of the progressive and global nature of the language dysfunction may permit earlier diagnosis and intervention.

Aphasia↗

The career success of an adult with a learning disability: a psychosocial study of amnesic-semantic aphasia.

B.I. is a 39-year-old, intellectually gifted (IQ = 130) man with learning disabilities who, without known cause, demonstrated symptoms of amnesic-semantic aphasia at age 13. This led to placement in a public school class for students with mild mental retardation and to his dropping out of school after repeating Grade 9. His aphasia is associated with a severe deficit in speech comprehension, poor reading and writing, spatial confusion, and episodic memory loss. We studied the remarkable behavioral and cognitive adjustments that have enabled him to lead a fulfilling life and become a highly successful business executive. Implications are discussed in the context of patterns of successful functioning and current views of the neuropsychological and neurological bases of such disorders.

Adaptation, Psychological↗

Aphasia owing to subcortical brain infarcts in childhood.

The aim of this study was to further define the clinical features of subcortical aphasia in children with deep brain infarcts and to define the sequelae associated with childhood strokes. We retrospectively studied nine children with left subcortical brain infarcts who presented with acquired language disorder and underwent language investigations based on standardized tests. Stroke in these patients involved the left internal capsule, lenticular or thalamic nuclei, or a combination of these. Early aphasic manifestations following the deep cerebral infarcts affected language expression. These included mutism, nonfluent speech, word finding difficulties, and phonemic and semantic paraphasia. Speech comprehension was generally more preserved. All patients subsequently improved, although variably; sequelae such as dysfluency, word finding difficulties, and written language learning impairment could be detected through standardized tests in six of them (all younger than 6 years at the time of the infarct). Two of the three remaining patients (both older than 6 years at the time of the infarct) had a full recovery. Our study confirms the concept of childhood subcortical aphasia, depicts the linguistic profile in these patients, and sustains the indication of systematic formal language assessment during the follow-up of all children with subcortical infarct involving the dominant hemisphere.

Adolescent↗

Slowly progressive aphasia with striatal involvement.

We have described four patients with slowly progressive aphasia with striatal involvement occurring at different stages in the course of the illness. There were two males and two females, and their ages ranged from 68 to 76 (mean: 72) years. The extrapyramidal signs included tremors, bradykinesia, rigidity, and focal dystonia, and one had weakness resembling stroke. There is a heterogeniety among patients with slowly progressive aphasia and the clinical features correspond to the functional anatomy of the areas involved rather than to the pathology.

Activities of Daily Living↗

Intensive language training enhances brain plasticity in chronic aphasia.

BACKGROUND: Focal clusters of slow wave activity in the delta frequency range (1-4 Hz), as measured by magnetencephalography (MEG), are usually located in the vicinity of structural damage in the brain. Such oscillations are usually considered pathological and indicative of areas incapable of normal functioning owing to deafferentation from relevant input sources. In the present study we investigated the change in Delta Dipole Density in 28 patients with chronic aphasia (>12 months post onset) following cerebrovascular stroke of the left hemisphere before and after intensive speech and language therapy (3 hours/day over 2 weeks). RESULTS: Neuropsychologically assessed language functions improved significantly after training. Perilesional delta activity decreased after therapy in 16 of the 28 patients, while an increase was evident in 12 patients. The magnitude of change of delta activity in these areas correlated with the amount of change in language functions as measured by standardized language tests. CONCLUSIONS: These results emphasize the significance of perilesional areas in the rehabilitation of aphasia even years after the stroke, and might reflect reorganisation of the language network that provides the basis for improved language functions after intensive training.

Adult↗

A randomized, double-blind trial of bromocriptine efficacy in nonfluent aphasia after stroke.

The authors assessed the efficacy of bromocriptine in nonfluent aphasia after stroke in a 16-week, randomized, double-blind, placebo-controlled clinical trial conducted from June 2002 to April 2004. In all 38 patients after 4 months of treatment, improvement in both the bromocriptine and placebo treatment groups was observed (p < 0.001). The analysis of repeated-measures analysis of variance revealed bromocriptine did not improve nonfluent aphasia.

Adult↗

Trying to tell a tale: discourse impairments in progressive aphasia and frontotemporal dementia.

OBJECTIVE: To assess discourse in patients with frontotemporal dementia (FTD). METHODS: The authors asked patients with progressive nonfluent aphasia (PNFA), patients with semantic dementia (SemD), and nonaphasic patients with a disorder of social comportment and executive functioning (SOC/EXEC) to narrate the story of a wordless children's picture book. RESULTS: The authors found significant discourse impairments in all three groups of patients. Moreover, there were qualitatively important differences between the groups. Patients with PNFA had the sparsest output, producing narratives with the fewest words per minute. Patients with SemD had difficulty retrieving words needed to tell their narratives. Though not aphasic, patients with SOC/EXEC had profound difficulty organizing their narratives, and they could not effectively express the point of the story. This deficit correlated with poor performance on a measure of executive resources requiring an organized mental search. In addition, a correlation of narrative organization with cortical atrophy in patients with SOC/EXEC was significant in right frontal and anterior temporal brain regions. CONCLUSIONS: Impaired day-to-day communication in nonaphasic frontotemporal dementia patients with a disorder of social comportment and executive functioning is due in part to a striking deficit in discourse organization associated with right frontotemporal disease. Difficulty with discourse in progressive aphasia is due largely to the language impairments of these patients.

Aged↗