Shifting of global aphasia to Wernicke's aphasia in a patient with intact motor function: a case report.

BACKGROUND: Global aphasia without hemiparesis (GAWH) is a rare stroke syndrome characterized by the dissociation of motor and language functions. Here, we present a case of GAWH with the patient later regaining speech fluency. CASE PRESENTATION: A 73-year-old man was admitted to our emergency department immediately after an episode of syncope. On arrival, we noted his global aphasia but without any focal neurologic signs. Computed tomography (CT) perfusion scans showed a large hypodense region over his left perisylvian area. Under the impression of acute ischaemic stroke, he received recombinant tissue plasminogen activator (rtPA) injection and was treated as an inpatient. The patient was later discharged with GAWH status and received regular speech rehabilitation. After 14 months of rehabilitation, the patient gradually recovered his language expression ability. The degree of aphasia was evaluated with the Concise Chinese Aphasia Test (CCAT), and we obtained brain single photon emission computed tomography (SPECT) scans to assess cerebral blood flow. CONCLUSION: A patient with severe impairments of Broca's and Wernicke's areas was able to talk fluently despite being unintelligible. SPECT revealed relative high level of radioactivity uptake in the right frontal lobe, suggesting the deficits in speech fluency could have been compensated by the right hemisphere. Although this is a single case demonstration, the results may strengthen the role of the right hemisphere in GAWH patients and suggests additional study that examines the possible benefits of stimulating activity at right homologous regions for recovering language function after global aphasia.

Dementia with aphasia and mirror phenomenon: examination of the mechanism using neuroimaging and neuropsychological findings: a case report.

BACKGROUND: Aphasia often appears in persons living with dementia; however, aphasia and the mirror phenomenon are rarely present at the same time. CASE PRESENTATION: Here, we report a case of fluent conversation with a person in a mirror or a magazine, and examine the underlying mechanism using brain imaging and neuropsychological findings. We found that the appearance of the mirror phenomenon may be associated with a visuospatial dysfunction caused by a decreased function of the posterior region of the right temporal and parietal lobe. Moreover, active talking to a person in a mirror or a person in a magazine could be associated with disinhibition caused by a decline in bilateral frontal lobe function. CONCLUSIONS: This case represents a very valuable and interesting presentation because it is the first report of a long-term follow-up of the course of dementia using neurological imaging, and of the neuropsychological analysis of the mechanism of conversation with a mirror image combined with aphasia.

[The language of children and young people with 22q11 deletion syndrome]. / Lenguaje de niños y jóvenes con síndrome de deleción 22q11.

INTRODUCTION: The 22q11 deletion syndrome (S22q11) is one of the most prevalent genetic disorders, resulting in multiple systemic and neuropsychological features. AIM: To describe the language profile in a sample of Spanish subjects with S22q11. PATIENTS AND METHODS: A sample of 30 Spanish participants with S22q11 aged between 5 years and 21 years and 11 months (mean: 12.14 ± 4.20 years) was evaluated using standardized tests and a questionnaire administered to parents. RESULTS: Almost half of the subjects obtained better results in expressive language than in comprehensive language and the majority obtained a higher score in language content than in language memory. The results suggest that people with S22q11 present language difficulties that improve with age to a certain level and subsequently stabilize. A specific profile is observed that suggests that pragmatic difficulties are a consequence of this language profile and not only of social difficulties already described in this pathology. CONCLUSIONS: In the sample of the present study, children and young people with S22q11 present specific language and pragmatic disorders. More than half of the study participants did not obtain significant differences between the level of expressive and receptive language. Most presented semantic fluency difficulties. The type and degree of impairment in pragmatic skills suggest that the basic problem may be related to their language difficulties.

TITLE: Lenguaje de niños y jóvenes con síndrome de deleción 22q11.Introducción. El síndrome de deleción 22q11 (S22q11) es uno de los trastornos genéticos más prevalentes, y presenta múltiples alteraciones sistémicas y neuropsicológicas. Objetivo. Describir el perfil de lenguaje y pragmática asociado a este síndrome. Pacientes y métodos. Se evaluó una muestra de 30 participantes españoles con S22q11 de edades comprendidas entre 5 años, y 21 años y 11 meses (media: 12,14 ± 4,2 años) mediante pruebas estandarizadas y un cuestionario administrado a los padres. Resultados. Casi la mitad de la muestra obtuvo mejores resultados en el lenguaje expresivo que en el comprensivo, y la mayoría logró una mayor puntuación en el contenido del lenguaje que en la memoria del lenguaje. Los resultados sugieren que las personas con S22q11 presentan dificultades de lenguaje que mejoran con la edad hasta cierto nivel y, posteriormente, se estabilizan. Se observa un perfil específico que sugiere que las dificultades pragmáticas son consecuencia de este perfil de lenguaje y no sólo de dificultades sociales ya descritas en esta patología. Conclusiones. En la muestra del presente estudio, los niños y jóvenes con S22q11 presentan alteraciones específicas del lenguaje y la pragmática. Más de la mitad de los participantes del estudio no obtuvieron diferencias significativas entre el nivel de lenguaje expresivo y el receptivo. La mayoría presentó dificultades de fluencia semántica. El tipo y el grado de las alteraciones que presentan en las habilidades pragmáticas sugieren que el problema básico podría estar relacionado con sus dificultades lingüísticas.

[Improved instrumental activities of daily living in acquired savant syndrome patient after developing interest in drawing].

We performed examinations of a 73-year-old, right-handed man who developed herpes simplex encephalitis, with cognitive dysfunction including severe Wernicke's aphasia. Although he had never previously been interested in arts, use of a coloring book, recommended by his wife, led him to start drawing. A few years after the onset of brain disease, the patient began to copy pictures of landscapes. The lesion was in the left hemisphere and his work showed a strongly realistic tendency, thus we think that this case demonstrated characteristics of acquired savant syndrome. Along with the increase in drawing ability, instrumental activities of daily living (IADL), such as shopping and use of public transport, were also considerably improved in this patient. On the other hand, results of neuropsychological tests, such as the Standard Language Test of Aphasia, were not improved. We concluded that a sense of accomplishment from the drawing activity and communication with supporters might have led to improvement of IADL in this case.

Book talk and aphasia: the power of a book.

BACKGROUND: Reading is most often affected in aphasia and this has an impact on most aspects of everyday life. Being able to read makes a significant difference to how well a person can participate in society. In this study, people with aphasia recount their experiences of being in a book club. AIMS: This small scale, exploratory study employs a qualitative approach to investigate how persons with aphasia (PWA) and a librarian experience participating in a book club. The aim was to explore their overall experiences of participation, including their views regarding the design of the book club. The research questions were: How did participants experience participating in a book club? How did participants experience the structure and the content of the book club? METHODS & PROCEDURES: Three men and one woman with aphasia took part in a book club, which was led by a librarian and met once a week for 9 weeks. The group read a book that had been adapted to suit adults who are not very skilled readers or who have a poor knowledge of the Swedish language; it was also available in an audio version. Data were collected through observations, interviews and field notes, and were analysed thematically to find patterns across data and across participants. OUTCOMES & RESULTS: The analysis showed that, despite their language difficulties, the participants experienced the book club as highly rewarding, possibly because the focus was on the content of the book and not on each individual's reading ability. The overarching theme identified in the data was 'Empowerment through a joint reading experience'. This encompassed three sub-themes: 'Structure and flexibility', 'Enjoyment of reading' and 'Fruitful discussions'. CONCLUSIONS & IMPLICATIONS: The PWA experienced the activity as positive and encouraging despite their language difficulties. What yielded the positive experience were the joint literary discussions. The project also showed that a dedicated group leader (here the librarian) and a flexible structure based on the situation and abilities of each individual were crucial for the encouraging findings.

Category specific recall in acute stroke: a case with letter speech.

Category selective recall in spontaneous speech after stroke has been reported only rarely. We recently described three cases demonstrating transient number speech in the acute stage of left hemispheric stroke and hypothesized a link with multilingualism and mathematical proficiency. In this report, we describe a similar case with a transient episode of utterances of randomly selected letters. Like in the three previous cases, this episode was preceded by a brief stage of mutism and ultimately evolved to Wernicke's aphasia over a period of days. This phenomenon is reviewed with reference to linguistic models and neuroanatomic and neurophysiological correlates.

Auditory, Phonological, and Semantic Factors in the Recovery From Wernicke's Aphasia Poststroke: Predictive Value and Implications for Rehabilitation.

Background. Understanding the factors that influence language recovery in aphasia is important for improving prognosis and treatment. Chronic comprehension impairments in Wernicke's aphasia (WA) are associated with impairments in auditory and phonological processing, compounded by semantic and executive difficulties. This study investigated whether the recovery of auditory, phonological, semantic, or executive factors underpins the recovery from WA comprehension impairments by charting changes in the neuropsychological profile from the subacute to the chronic phase. Method. This study used a prospective, longitudinal observational design. Twelve WA participants with superior temporal lobe lesions were recruited 2 months post-stroke onset (2 MPO). Language comprehension was measured alongside a neuropsychological profile of auditory, phonological, and semantic processing and phonological short-term memory and nonverbal reasoning at 3 poststroke time points: 2.5, 5, and 9 MPO. Results. Language comprehension displayed a strong and consistent recovery between 2.5 and 9 MPO. Improvements were also seen for slow auditory temporal processing, phonological short-term memory, and semantic processing but not for rapid auditory temporal, spectrotemporal, and phonological processing. Despite their lack of improvement, rapid auditory temporal processing at 2.5 MPO and phonological processing at 5 MPO predicated comprehension outcomes at 9 MPO. Conclusions. These results indicate that recovery of language comprehension in WA can be predicted from fixed auditory processing in the subacute stage. This suggests that speech comprehension recovery in WA results from reorganization of the remaining language comprehension network to enable the residual speech signal to be processed more efficiently, rather than partial recovery of underlying auditory, phonological, or semantic processing abilities.

Transcortical Sensory Aphasia after Left Frontal Lobe Infarction: Loss of Functional Connectivity.

BACKGROUND: The underlying mechanism of transcortical sensory aphasia (TSA) caused by lesions occurring in the left frontal lobe remains unclear. We attempted to investigate the mechanism with the use of functional MRI (fMRI). METHODS: We studied 2 patients with TSA after a left frontal infarction identified by diffusion-weighted MRI. As control subjects, a patient with transcortical motor aphasia and a healthy normal adult were chosen. The Korean version of Western Aphasia Battery was performed initially and at 3 months post stroke. We performed fMRI using verb generation and sentence completion tasks. Resting-state fMRI (rs-fMRI) was also obtained for network-level analysis initially and at 3 months post stroke. RESULTS: The results of diffusion- and perfusion-weighted MRI revealed no diffusion-perfusion mismatch. Initial fMRI in patients with TSA showed no reversed inter-/intrahemispheric activation patterns. rs-fMRI showed significantly decreased resting-state functional connectivity in the language network in patients with TSA compared with the control subjects. Follow-up rs-fMRI studies showed improvement in functional connectivity along with the recovery of patients' language function. CONCLUSION: Our data showed that the auditory comprehension deficits in patients with frontal lobe infarcts is attributed to difficulty accessing the posterior language area due to functional disconnection between language centers in the acute stage of stroke.

Arterial spin labelling shows functional depression of non-lesion tissue in chronic Wernicke's aphasia.

Behavioural impairment post-stroke is a consequence of structural damage and altered functional network dynamics. Hypoperfusion of intact neural tissue is frequently observed in acute stroke, indicating reduced functional capacity of regions outside the lesion. However, cerebral blood flow (CBF) is rarely investigated in chronic stroke. This study investigated CBF in individuals with chronic Wernicke's aphasia (WA) and examined the relationship between lesion, CBF and neuropsychological impairment. Arterial spin labelling CBF imaging and structural MRIs were collected in 12 individuals with chronic WA and 13 age-matched control participants. Joint independent component analysis (jICA) investigated the relationship between structural lesion and hypoperfusion. Partial correlations explored the relationship between lesion, hypoperfusion and language measures. Joint ICA revealed significant differences between the control and WA groups reflecting a large area of structural lesion in the left posterior hemisphere and an associated area of hypoperfusion extending into grey matter surrounding the lesion. Small regions of remote cortical hypoperfusion were observed, ipsilateral and contralateral to the lesion. Significant correlations were observed between the neuropsychological measures (naming, repetition, reading and semantic association) and the jICA component of interest in the WA group. Additional ROI analyses found a relationship between perfusion surrounding the core lesion and the same neuropsychological measures. This study found that core language impairments in chronic WA are associated with a combination of structural lesion and abnormal perfusion in non-lesioned tissue. This indicates that post-stroke impairments are due to a wider disruption of neural function than observable on structural T1w MRI.

Ability to solve riddles in patients with speech and language impairments after stroke.

INTRODUCTION: Successful riddle solving requires recognition of the meaning of words, attention, concentration, memory, connectivity and analysis of riddle content, and sufficiently developed associative thinking. OBJECTIVE: The aim of the study was to determine the ability to solve riddles in stroke patients who do or do not have speech and language disorders (SLDs), to determine the presence of SLDs in relation to the lesion localization, as well as to define the relationship between riddle-solving and functional impairment of a body side. METHODS: The sample consisted of 88 patients. The data used included age, sex, educational level, time of stroke onset, presence of an SLD, lesion localization, and functional damage of the body side. The patients were presented with a task of solving 10 riddles. RESULTS: A significant SLD was present in 38.60% of the patients. Brain lesions were found distributed at 46 different brain sites. Patients with different lesion localization had different success in solving riddles. Patients with perisylvian cortex brain lesions, or patients with Wernicke and global aphasia, had the poorest results. The group with SLDs had an average success of solved riddles of 26.76% (p = 0.000). The group with right-sided functional impairments had average success of 37.14%, and the group with functional impairments of the left side of the body 56.88% (p = 0.002). CONCLUSION: Most patients with SLDs had a low ability of solving riddles. Most of the patients with left brain lesions and perisylvian cortex damage demonstrated lower ability in solving riddles in relation to patients with right hemisphere lesions.

Case Studies Illustrating Focal Alzheimer's, Fluent Aphasia, Late-Onset Memory Loss, and Rapid Dementia.

Many dementia subtypes have more shared signs and symptoms than defining ones. We review 8 cases with 4 overlapping syndromes and demonstrate how to distinguish the cases. These include focal cortical presentations of Alzheimer's disease (AD; posterior cortical atrophy and corticobasal syndrome [CBS]), fluent aphasia (semantic dementia and logopenic aphasia), late-onset slowly progressive dementia (hippocampal sclerosis and limbic predominant AD) and rapidly progressive dementia (Creutzfeldt-Jakob disease and limbic encephalitis). Recognizing the different syndromes can help the clinician to improve their diagnostic skills, leading to improved patient outcomes by early and accurate diagnosis, prompt treatment, and appropriate counseling and guidance.

Effect of Donepezil on Wernicke Aphasia After Bilateral Middle Cerebral Artery Infarction: Subtraction Analysis of Brain F-18 Fluorodeoxyglucose Positron Emission Tomographic Images.

Aphasia is one of the most common neurologic deficits occurring after stroke. Although the speech-language therapy is a mainstream option for poststroke aphasia, pharmacotherapy is recently being tried to modulate different neurotransmitter systems. However, the efficacy of those treatments is still controversial. We present a case of a 53-year-old female patient with Wernicke aphasia, after the old infarction in the territory of left middle cerebral artery for 8 years and the recent infarction in the right middle cerebral artery for 4 months. On the initial evaluation, the Aphasia Quotient in Korean version of the Western Aphasia Battery was 25.6 of 100. Baseline brain F-18 fluorodeoxyglucose positron emission tomographic images demonstrated a decreased cerebral metabolism in the left temporoparietal area and right temporal lobe. Donepezil hydrochloride, a reversible acetylcholinesterase inhibitor, was orally administered 5 mg/d for 6 weeks after the initial evaluation and was increased to 10 mg/d for the following 6 weeks. After the donepezil treatment, the patient showed improvement in language function, scoring 51.0 of 100 on Aphasia Quotient. A subtraction analysis of the brain F-18 fluorodeoxyglucose positron emission tomographic images after donepezil medication demonstrated increased uptake in both middle temporal gyri, extended to the occipital area and the left cerebellum. Thus, we suggest that donepezil can be an effective therapeutic choice for the treatment of Wernicke aphasia.

Biparietal variant of Alzheimer's disease: a rare presentation of a common disease.

Alzheimer's disease (AD) is a clinically heterogeneous disease that may have atypical presentations with focal cortical syndromes and relatively preserved episodic memory. The posterior variant of AD has two subtypes: occipitotemporal, presenting with visuoperceptive impairment, and biparietal, presenting with visuospatial dysfunction and apraxia. We report a case of a 51-year-old woman with progressive limb apraxia and choreiform movements. Her neuropsychological evaluation was compatible with dementia, and revealed ideomotor and ideational limb apraxia, severe visuoconstructive ability impairment, dyscalculia and posterior aphasia. Workup excluded metabolic, infectious, inflammatory or neoplastic causes, and hereditary conditions as Huntington's disease and familial AD. Cerebrospinal fluid biomarkers revealed ß-amyloid reduction and τ protein increase. Brain imaging showed marked biparietal atrophy and hypoperfusion, and widespread cortical ß-amyloid deposition. Biparietal variant of AD was diagnosed and acetylcholinesterase inhibitor treatment induced clinical stabilisation. AD may present with atypical features and a high clinical suspicion is necessary for an early diagnosis.

Should pantomime and gesticulation be assessed separately for their comprehensibility in aphasia? A case study.

BACKGROUND: Gesticulation (gestures accompanying speech) and pantomime (gestures in the absence of speech) can each be comprehensible. Little is known about the differences between these two gesture modes in people with aphasia. AIMS: To discover whether there are differences in the communicative use of gesticulation and pantomime in QH, a person with severe fluent aphasia. METHODS & PROCEDURES: QH performed two tasks: naming objects and retelling a story. He did this once in a verbal condition (enabling gesticulation) and once in a pantomime condition. For both conditions, the comprehensibility of gestures was analysed in a forced-choice task by naïve judges. Secondly, a comparison was made between QH and healthy controls for the representation techniques used. OUTCOMES & RESULTS: Pantomimes produced by QH for naming objects were significantly more comprehensible than chance, whereas his gesticulation was not. For retelling a story the opposite pattern was found. When naming objects QH gesticulated much more than did healthy controls. His pantomimes for this task were simpler than those used by the control group. For retelling a story no differences were found. CONCLUSIONS & IMPLICATIONS: Although QH did not make full use of each gesture modes' potential, both did contribute to QH's comprehensibility. Crucially, the benefits of each mode differed across tasks. This implies that both gesture modes should be taken into account separately in models of speech and gesture production and in clinical practice for different communicative settings.

Phonological therapy in jargon aphasia: effects on naming and neologisms.

BACKGROUND: Jargon aphasia is one of the most intractable forms of aphasia with limited recommendation on amelioration of associated naming difficulties and neologisms. The few naming therapy studies that exist in jargon aphasia have utilized either semantic or phonological approaches, but the results have been equivocal. Moreover, the effect of therapy on the characteristics of neologisms is less explored. AIMS: This study investigates the effectiveness of a phonological naming therapy (i.e., phonological component analysis-PCA) on picture-naming abilities and on quantitative and qualitative changes in neologisms for an individual with jargon aphasia (FF). METHODS & PROCEDURES: FF showed evidence of jargon aphasia with severe naming difficulties and produced a very high proportion of neologisms. A single-subject multiple probe design across behaviours was employed to evaluate the effects of PCA therapy on the accuracy for three sets of words. In therapy, a phonological components analysis chart was used to identify five phonological components (i.e. rhymes, first sound, first sound associate, final sound and number of syllables) for each target word. Generalization effects-change in per cent accuracy and error pattern-were examined comparing pre- and post-therapy responses on the Philadelphia Naming Test, and these responses were analysed to explore the characteristics of the neologisms. The quantitative change in neologisms was measured by change in the proportion of neologisms from pre- to post-therapy and the qualitative change was indexed by the phonological overlap between target and neologism. OUTCOMES & RESULTS: As a consequence of PCA therapy, FF showed a significant improvement in his ability to name the treated items. His performance in maintenance and follow-up phases remained comparable with his performance during the therapy phases. Generalization to other naming tasks did not show a change in accuracy, but distinct differences in error pattern (an increase in proportion of real word responses and a decrease in proportion of neologisms) were observed. Notably, the decrease in neologisms occurred with a corresponding trend for increase in the phonological similarity between the neologisms and the targets. CONCLUSIONS & IMPLICATIONS: This study demonstrated the effectiveness of a phonological therapy for improving naming abilities and reducing the amount of neologisms in an individual with severe jargon aphasia. The positive outcome of this research is encouraging, as it provides evidence for effective therapies for jargon aphasia and also emphasizes that use of the quality and quantity of errors may provide a sensitive outcome measure to determine therapy effectiveness, in particular for client groups who are difficult to treat.

Ictal inner speech jargon.

We report on the case of a woman with jargon aphasic seizures who provided a careful written report of inner speech jargon occurring during her seizures. This inner speech jargon description is an unusual finding since in most aphasic disorders, patients also suffer from anosognosia. This case report may suggest that jargon could also involve inner speech and could be innerly detected as such. It provides an argument supporting the idea that common mechanisms may underlie both "overt" and "covert" production of jargon during aphasia.

[FMRI-study of speech perception impairment in post-stroke patients with sensory aphasia].

The aim of the study was to find neurophysiological correlates of the primary stage impairment of speech perception, namely phonemic discrimination, in patients with sensory aphasia after acute ischemic stroke in the left hemisphere by noninvasive method of fMRI. For this purpose we registered the fMRI-equivalent of mismatch negativity (MMN) in response to the speech phonemes--syllables "ba" and "pa" in odd-ball paradigm in 20 healthy subjects and 23 patients with post-stroke sensory aphasia. In healthy subjects active brain areas depending from the MMN contrast were observed in the superior temporal and inferior frontal gyri in the right and left hemispheres. In the group of patients there was a significant activation of the auditory cortex in the right hemisphere only, and this activation was less in a volume and intensity than in healthy subjects and correlated to the degree of preservation of speech. Thus, the method of recording fMRI equivalent of MMN is sensitive to study the speech perception impairment.

Stroke rehabilitation using noninvasive cortical stimulation: aphasia.

Poststroke aphasia results from the lesion of cortical areas involved in the motor production of speech (Broca's aphasia) or in the semantic aspects of language comprehension (Wernicke's aphasia). Such lesions produce an important reorganization of speech/language-specific brain networks due to an imbalance between cortical facilitation and inhibition. In fact, functional recovery is associated with changes in the excitability of the damaged neural structures and their connections. Two main mechanisms are involved in poststroke aphasia recovery: the recruitment of perilesional regions of the left hemisphere in case of small lesion and the acquisition of language processing ability in homotopic areas of the nondominant right hemisphere when left hemispheric language abilities are permanently lost. There is some evidence that noninvasive cortical stimulation, especially when combined with language therapy or other therapeutic approaches, can promote aphasia recovery. Cortical stimulation was mainly used to either increase perilesional excitability or reduce contralesional activity based on the concept of reciprocal inhibition and maladaptive plasticity. However, recent studies also showed some positive effects of the reinforcement of neural activities in the contralateral right hemisphere, based on the potential compensatory role of the nondominant hemisphere in stroke recovery.

Prolonged ictal aphasia: a diagnosis to consider.

Aphasia is a common symptom encountered by clinical neurologists. It is usually caused by strokes or lesions involving language regions of the brain, yet prolonged aphasia is rarely the sole manifestation of a simple partial status epilepticus. We report six patients, who suffered from prolonged ictal aphasia. All but one patient had a structural lesion in the left hemisphere, only three suffered from clinical seizures during or shortly prior to the aphasic episode. All patients had ictal patterns on the electroencephalogram (EEG), four of whom had periodic lateralized epileptiform discharges, and five showed frequent recurrent electrographic seizures during the aphasic state. The aphasia lasted several days in all patients, and it resolved after administration of antiepileptic drug treatment. In conclusion, prolonged ictal aphasia is a rare but important treatable cause of aphasia. Surface EEG recordings should be obtained in all patients with unexplained prolonged aphasia to diagnose this rare but treatable entity.