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Enhancing communication in jargon aphasia: a small group study of writing therapy.

People with jargon aphasia have severely disordered and incomprehensible speech that may be resistant to therapeutic intervention. In this study, we treated written output and examined whether it assisted communication for these clients. In stage one of the study, anagram sorting, delayed copying and lexical decision tasks were used to investigate the residual knowledge of written words in a group of ten people with jargon aphasia. Evidence of the presence of orthographic knowledge was taken as an indication that writing might be a useful focus for therapy. This hypothesis was explored in stage two with six clients. A personally useful vocabulary was selected for each, and copying, word completion and written picture-naming tasks were used in therapy to improve written production of these words. The clients made progress in written naming. However, they showed little change on a 'message' assessment that tested their ability to use the written words to convey messages. Stage three targeted communicative writing. Here, three of the clients received 'message therapy', which encouraged them to relate treated words to functional messages and to communicate them to a partner. The clients improved on the message assessment and observation of their communication and reports from relatives suggested that they made functional use of writing in a range of communication settings.

Aged↗

Writing with voice: an investigation of the use of a voice recognition system as a writing aid for a man with aphasia.

BACKGROUND: People with aphasia may experience difficulties that prevent them from demonstrating in writing what they know and can produce orally. Voice recognition systems that allow the user to speak into a microphone and see their words appear on a computer screen have the potential to assist written communication. AIM: This study investigated whether a man with fluent aphasia could learn to use Dragon NaturallySpeaking to write. METHODS & PROCEDURES: A single case study of a man with acquired writing difficulties is reported. A detailed account is provided of the stages involved in teaching him to use the software. The therapy tasks carried out to develop his functional use of the system are then described. Outcomes included the percentage of words accurately recognized by the system over time, the quantitative and qualitative changes in written texts produced with and without the use of the speech-recognition system, and the functional benefits the man described. OUTCOMES & RESULTS: The treatment programme was successful and resulted in a marked improvement in the subject's written work. It also had effects in the functional life domain as the subject could use writing for communication purposes. CONCLUSIONS: The results suggest that the technology might benefit others with acquired writing difficulties.

Aphasia↗

Written communication in undifferentiated jargon aphasia: a therapy study.

A subject, R.M.M., with a 2-year history of jargon aphasia is described. At the beginning of this study she had minimal meaningful spoken output and showed little awareness of her speech despite having relatively well-preserved auditory comprehension. Her spoken output had proved resistant to earlier periods of therapy. In contrast, R.M.M.'s written output showed some ability to access orthographic information and monitoring of this modality was shown by an acute awareness of her errors. A 3-stage therapy programme is described. This was designed to improve R.M.M.'s writing of single words and to encourage use of writing as an alternative means of communication. The initial stage of therapy aimed to increase R.M.M.'s access to written word forms by use of picture stimuli. She showed significant improvement in writing treated items in response to pictures both immediately after therapy and at re-assessment 6 weeks later. Despite the acquisition of these skills, R.M.M. failed to use them in communicative contexts. A second stage of therapy replicated the results of the first and sought to facilitate R.M.M.'s functional use of her written vocabulary by asking her to write words to spoken questions. She again showed improved written naming of the treated items and could now produce written names appropriately in a questionnaire-type assessment. Generalization of this ability extended to items that had not been trained in this way. Functional use of writing in everyday communication remained absent, however. The final stage of therapy made explicit the potential links between items which R.M.M. could now write and functional messages which they might convey. She again showed significant changes in the acquisition of new vocabulary and, encouragingly, progress was also seen in her use of the strategy in functional communication. R.M.M.'s speech is almost entirely incomprehensible. It has remained unchanged for 2 years and has not responded to therapy. Relatively well-preserved auditory comprehension and good monitoring of written output allowed therapy to effectively target a small written vocabulary. Despite significant progress in the acquisition of new items, transfer of this skill to functional communication was initially absent. Further therapy which specifically targeted the impairment causing this failure was needed before functional use was seen. The potential for treating written output in cases of jargon aphasia which have been resistant to therapy for spoken language is discussed.

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Drawing together: evaluation of a therapy programme for severe aphasia.

This paper reports a therapy study that aims to promote communicative drawing in a group of seven people with severe and long-standing aphasia. Therapy was conducted on individual and group bases over 12 weeks and entailed a range of techniques which are described in some detail. Treatment was evaluated using a novel generative drawing assessment in which subjects were required to draw absent items in response to photographic and conversational cues. Pre- and post-therapy assessments of untreated skills, such as comprehension, naming and gesture, were also conducted. The results indicated that, as a group, the subjects' drawing improved. Unchanged performance on the other assessments indicated that the gains were specific to the content of therapy and could not be attributed to spontaneous recovery. Pre- and post-therapy interviews with carers (i.e. relatives and close friends of the individuals) suggested that the effects of therapy were also being felt in the home. These results have important implications for therapy with people with severe aphasia.

Aged↗

Normative data on the Korean version of the Western Aphasia Battery.

The study aimed to describe the properties of the Korean version of the Western Aphasia Battery (hereinafter K-WAB) and to present the normative data of normal individuals and patients. The K-WAB contains the same test contents and structure as the original WAB and the general test administration method was maintained. We administered the K-WAB to 224 normal adults in seven age groups (1524, 2534, 3544, 4554, 5564, 6574, and 75 years or older), in five educational levels (0, 16, 79, 1012, and 13 years or more) and by gender. The age and educational levels were influential to the K-WAB performance. Accordingly, we formed six subgroups of the normal: two age groups (1574, and 75 years or older groups) by three educational groups (0, 16, and 7 years or more). Two hundred thirty-eight patients were also evaluated using the K-WAB. The highest aphasia quotient (AQ), language quotient (LQ), and cortical quotient (CQ) were achieved by 1574 age group with 7 or more years of education (M=97.11,M=95.51,M=95.57, respectively). Based on the receiver operating curve (ROC) analyses on AQ, LQ and CQ values, cut-off scores to optimally differentiate between the normal and the aphasic individuals were also provided.

Adolescent↗

Speech fluency in crossed aphasia.

Three strongly right-handed patients developed fluent aphasia after right hemisphere infarction documented by computerized tomography. For these patients and for other reported cases of crossed aphasia suitable for analysis, the correlation between fluency and infarct localization was similar to that of right-handed aphasics with left-sided lesions. Right hemisphere language representation in most crossed aphasics probably mirrors that normally present in the language-dominant left hemisphere. Two of these patients showed concomitant hemispatial inattention and visuoconstructive impairment. Right hemisphere language dominance therefore does not preclude ipsilateral specialization for visuospatial functions.

Adult↗

Aphasia and neglect after subcortical stroke. A clinical/cerebral perfusion correlation study.

Sixteen patients with unilateral subcortical haemorrhagic or ischaemic stroke, confirmed by CT, were evaluated for the presence of aphasia and neglect. Compared with patients without neuropsychological deficits, left brain-damaged aphasic and right brain-damaged neglect patients showed a significantly greater reduction of cortical perfusion on N,N,N1-trimethyl-N1-(2)-hydroxy-3-methyl-5-(I-123) iodobenzyl-1,3-propanediamine 2 HCl I-123 (HIPDM) and single photon emission computerized tomography (SPECT). These results suggest that major cortical derangement is the crucial factor for the appearance of aphasia or neglect after a subcortical stroke. These remote effects, which are related to the size of the subcortical lesion, are interpreted in terms of interruption of neural connections (diaschisis).

Adult↗

Slowly progressive aphasia in three patients. The problem of accompanying neuropsychological deficit.

Three patients are described presenting with a slowly progressive aphasic disorder associated with degenerative cortical disease. The symptoms began in the presenium and the length of illness was 4 to 5 years. The language disorder corresponded in all patients to a severe form of amnesic aphasia but a moderate to marked semantic breakdown was also found. Formal language examination was complemented by extensive neuropsychological testing. This revealed a severe deficit in language-dependent cognitive tasks. The patients were given a follow-up language and neuropsychological examination. In addition to the deterioration of language functions, a significant decline was observed in nonverbal intelligence tasks even though their level of performance still remained within the normal range. Follow-up with standardized intelligence tests might detect a trend towards generalized dementia in similar cases. This would mean that these patients should be considered as presenting with slowly progressive aphasia preceding generalized dementia.

Aphasia↗

Cerebral blood flow in progressive aphasia without dementia. Case report, using 133xenon inhalation, technetium 99m hexamethylpropyleneamine oxime and single photon emission computerized tomography.

We report a case of progressive aphasia without clinical signs of intellectual or behavioral impairment, satisfying Mesulam's clinical criteria of primary progressive aphasia, as 4 yrs of extensive psychometric testing and radiological imaging, comprising CT and MRI, failed to detect evidence of relevant involvement outside the left perisylvian regions. Cranial CT was normal but MRI showed multiple bilateral lesions in the deep white matter. Cerebral blood flow (CBF) studies by single photon emission computerized tomography, however, showed an initial frontotemporal focus of hypoperfusion that progressively extended to include most of the ipsilateral hemisphere and the contralateral frontal lobe. This suggests that CBF imaging may yet be the most sensitive technique in revealing subclinical injury in the degenerative brain diseases of focal onset.

Administration, Inhalation↗

Transcortical sensory aphasia: revisited and revised.

Transcortical sensory aphasia (TSA) is characterized by impaired auditory comprehension with intact repetition and fluent speech. We induced TSA transiently by electrical interference during routine cortical function mapping in six adult seizure patients. For each patient, TSA was associated with multiple posterior cortical sites, including the posterior superior and middle temporal gyri, in classical Wernicke's area. A number of TSA sites were immediately adjacent to sites where Wernicke's aphasia was elicited in the same patients. Phonological decoding of speech sounds was assessed by auditory syllable discrimination and found to be intact at all sites where TSA was induced. At a subset of electrode sites where the pattern of language deficits otherwise resembled TSA, naming and word reading remained intact. Language lateralization testing by intracarotid amobarbital injection showed no evidence of independent right hemisphere language. These results suggest that TSA may result from a one-way disruption between left hemisphere phonology and lexical-semantic processing.

Adult↗

Semantic impairment in stroke aphasia versus semantic dementia: a case-series comparison.

Different neuropsychological populations implicate diverse cortical regions in semantic memory: semantic dementia (SD) is characterized by atrophy of the anterior temporal lobes whilst poor comprehension in stroke aphasia is associated with prefrontal or temporal-parietal infarcts. This study employed a case-series design to compare SD and comprehension-impaired stroke aphasic patients directly on the same battery of semantic tests. Although the two groups obtained broadly equivalent scores, they showed qualitatively different semantic deficits. The SD group showed strong correlations between different semantic tasks--regardless of input/output modality--and substantial consistency when a set of items was assessed several times. They were also highly sensitive to frequency/familiarity and made coordinate and superordinate semantic errors in picture naming. These findings support the notion that amodal semantic representations degrade in SD. The stroke aphasia group also showed multimodal deficits and consistency across different input modalities, but inconsistent performance on tasks requiring different types of semantic processing. They were insensitive to familiarity/frequency--instead, tests of semantic association were influenced by the ease with which relevant semantic relationships could be identified and distractors rejected. In addition, the aphasic patients made associative semantic errors in picture naming that SD patients did not make. The aphasic patients' picture naming performance improved considerably with phonemic cues suggesting that these patients retained knowledge that could not be accessed without contextual support. We propose that semantic cognition is supported by two interacting principal components: (i) a set of amodal representations (which progressively degrade in SD) and (ii) executive processes that help to direct and control semantic activation in a task-appropriate fashion (which are dysfunctional in comprehension-impaired stroke aphasic patients).

Adult↗

Tau and alpha-synuclein inclusions in a case of familial frontotemporal dementia and progressive aphasia.

Recent studies have shown that neurofibrillary tangles are frequently accompanied by alpha-synuclein inclusions in sporadic and familial Alzheimer disease, in Down syndrome, in progressive supranuclear palsy, and Parkinsonism dementia complex of Guam. Here we report the cases of 2 brothers with familial progressive aphasia who developed features of frontotemporal dementia with predominant tau pathology but also alpha-synuclein pathology. The 2 patients' brains revealed abundant tau pathology in the hippocampus and basal ganglia, whereas tau and alpha-synuclein aggregates coexisted only in the nucleus basalis of Meynert, the only region where alpha-synuclein was present. In this brain region, abundant Lewy bodies, Lewy neurites, and tau inclusions were found; the pathology was more abundant in the older than in the younger brother. Sarkosyl-insoluble tau extracted from brains of the 2 patients showed the presence of tau filaments that contained 3 major tau bands of 60, 64, and 68 kDa on Western blot analysis. These bands contained mainly tau with 3 and 4 repeats and no amino-terminal inserts and tau with 4 repeats and one amino-terminal insert. No mutations were identified in the tau, alpha-synuclein, beta-synuclein, or parkin genes. We think that this is the first report showing a specific colocalization of neurofibrillary tangles and Lewy bodies in a family with progressive aphasia.

Aphasia↗

Continuous focal spikes during REM sleep in a case of acquired aphasia (Landau-Kleffner syndrome).

We report a girl 3 years and 6 months old with onset of aphasia at age 3 years and 3 months. There was no evidence of brain damage and there were no seizures. The neuropsychological evaluation showed that the girl tended to be right-handed, that aphasia was global and that other higher cortical functions seemed to be preserved. Isolated spikes and spikes-and-wave were recorded during wake over the right temporal region with rare independent contralateral abnormalities. During polysomnography (PSG), the physiological patterns of sleep were preserved and right temporal epileptiform discharges were significantly increased in all sleep stages. Maximal activation was obtained at sleep onset and during rapid eye movement (REM) sleep periods, when focal abnormalities became continuous and spread contralaterally. Repeat PSGs showed that the activation profile retained this particular trait, although subclinical discharges tended to increase during slow wave sleep (SWS). This pattern of subclinical temporal status epilepticus during REM sleep differs from the characteristic activation profile found in the syndrome of continuous spikes-and-waves during SWS. However, this profile was transient and all epileptiform changes disappeared during clinical recovery at 18 months of follow-up.

Anticonvulsants↗

An investigation of sensory deficits underlying the aphasia-like behavior of macaques with auditory cortex lesions.

Bilateral auditory cortex lesions in Japanese macaques result in an aphasia-like deficit in which the animals are unable to discriminate two forms of their coo vocalizations. To determine whether this deficit is sensory in nature, two monkeys with bilateral lesions were tested for their ability to discriminate frequency and frequency change. The results indicated that although the animals were able to discriminate between sounds of different frequencies, they were unable to determine whether a sound was changing in frequency. Because the animals' coo vocalizations differ primarily in the predominant direction of their frequency change and not in their absolute frequency content, the aphasia-like deficit of animals with bilateral auditory cortex lesions appears to be a sensory disorder.

Animals↗

Acquired epileptiform aphasia in children (Landau-Kleffner syndrome).

The association of a language disorder with epilepsy is frequent in children, but there is usually no causal relationship. In acquired epileptiform aphasia (AEA), the so-called Landau-Kleffner syndrome, there is increasing evidence that the language disorder is directly caused by epileptic discharges in critical language areas and must be viewed as a special kind of epileptic aphasia. This is based on a review of the published cases of AEA over the last 30 years and on the analogies that can be made between AEA and other epileptic syndromes, mainly benign partial epilepsy with centrotemporal spikes. AEA can start early in development and present as developmental dysphasia. It is only one among other cognitive or behavioral disturbances that can be epileptic manifestations of some particular epileptic syndromes, for example, epilepsy with continuous spike waves during slow sleep, which probably has the same pathophysiology as AEA. AEA must be seen, at least in some cases, as a particular form of resistant epilepsy. AEA is an important model because it suggests that isolated cognitive and behavioral disturbances can be epileptic manifestations in children.

Adult↗

Transient aphasia as late-onset seizure after embolization for dural arteriovenous malformation.

Tc-99m HMPAO single photon emission computed tomography was performed in a patient who had experienced aphasia for 3 days. The patient had a medical history of subarachnoid hemorrhage and intracranial hemorrhage. Both of these hemorrhages were related to a dural arteriovenous malformation that was embolized after the onset of the cerebral vascular events. Focal hyperperfusion was seen in the left temporal lobe adjacent to a perfusion defect corresponding to the old cerebral hemorrhage. The finding was interpreted as the focus of the patient's epilepsy. His aphasia resolved after the administration of anticonvulsant medication for several days.

Aphasia↗

The unknown source of John Hughlings Jackson's early interest in aphasia and epilepsy.

The National Hospital for Paralysis and Epilepsy in London (founded 1859) was the scene of great discoveries in the new specialty of neurology, carried out in great part by John Hughlings Jackson (1834-1911). The clinicians Jonathan Hutchinson and Charles Edward Brown-Sequard are typically identified as Jackson's mentors. This paper discusses the previously neglected role of Jabez Spence Ramskill (1824-1897), founding physician of the National Hospital. Ramskill appears to have been significant in providing the opportunity and context that led Jackson to develop his theories concerning higher cerebral function disorders. As assistant physician to Ramskill at the National Hospital, Jackson was provided with a vast caseload of epileptic, hemiplegic, and aphasic patients. Ramskill and Jackson both published papers on aphasia in the London Hospital Reports in 1864. Consideration of the similarities and differences between these 2 papers highlight significant issues in the clinical and theoretical development of understanding language organization in the brain. The early writings of Jackson and case notes of Ramskill document a close link between the 2 and indicate the debt that Jackson had to Ramskill for providing him with the opportunities to develop his original ideas on epilepsy and aphasia.

Aphasia↗

Vasectomy in men with primary progressive aphasia.

OBJECTIVE: To study the frequency of vasectomy in men with primary progressive aphasia (PPA). BACKGROUND: PPA is a dementia syndrome in which aphasia emerges in relative isolation during the initial stages of illness. On the basis of a clinical observation in a patient who dated the onset of symptoms to the period after a vasectomy, and because of the curious sharing of the tau protein exclusively by brain and sperm, vasectomy rates were examined in men with PPA. METHOD: This study used a case control design. Forty-seven men with PPA and 57 men with no cognitive impairment (NC) between 55 and 80 years of age were surveyed about a history of vasectomy. RESULTS: The age-adjusted rate of vasectomy in PPA patients (40%) was higher than in NC (16%, P=0.02). There was a younger age at onset for the patients with vasectomy (58.8 vs. 62.9 y, P=0.03). CONCLUSIONS: Vasectomy may constitute one risk factor for PPA in men. Potential mechanisms mediating risk include vasectomy-induced immune responses to sperm, which shares antigenic epitopes with brain. Antisperm antibodies can also develop in women and become risk factors for PPA.

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