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Acute flare of multiple sclerosis usually presents with sensorimotor deficits in limbs or one side of the face, optic neuritis, internuclear ophthalmoplegia, and/or cerebellar signs and symptoms. Isolated aphasia is observed only in a handful of cases. Herein, we present a case of a patient who presented with isolated transcortical motor aphasia. Initial thought was that the patient was having a cerebrovascular accident as he had a history of uncontrolled hypertension. It was only later found on magnetic resonance imaging (MRI) of the brain that the patient had demyelinating lesions compatible with his new symptoms. He exhibited an excellent response to intravenous methylprednisolone therapy and was discharged with outpatient evaluation for immunotherapy.

Background: Neurolinguists are increasingly inclined to study the language behavior of patients with aphasia (PWAs) to discover more about the relationship between the brain and language. Objectives: This study investigated the production of synthetic and root compound nouns in the PWAs to discover how these lexemes were processed. Methods: Using a confrontation naming task, four PWAs (two patients with Broca aphasia and two patients with transcortical motor aphasia) named 80 random black and white drawings of simple and compound nouns. They also repeated the nouns through an auditory repetition task. Compound nouns were of two root and synthetic types. Root nouns belonged to the noun-noun, and synthetic compounds belonged to the noun-verb category. Results: There was a significant difference between the affected components in naming and repetition of compound nouns. Moreover, there was a significant difference between naming and repetition of simple and compound nouns. There was no significant difference between naming and repetition of root and synthetic nouns. Conclusions: PWAs process compound nouns through the dual-route model. They cannot retrieve the phonological forms of compound nouns, but they retain their knowledge of word-formation, indicating the modularity of linguistic ability. Morphological structure plays a role in word processing.

Background Crossed aphasia (CA) refers to aphasia following a right-hemispheric lesion in right-handed individuals. It has been suggested that the prevalence of CA differs with language, although its worldwide incidence, as reported by most studies, is less than 3%. Objective To find the incidence of CA in the Bengali language. Methods From 2016 to 2018, in a hospital located in a Bengali-speaking area of eastern India, 515 cases of first-ever stroke were documented, out of which 208 patients presented with aphasia (40.38%) according to their scores on the Bengali version of the Western Aphasia Battery. Results Among the patients with aphasia, 14 (6.73%; 8 men and 6 women) presented with CA. Of these, 10 were diagnosed with Broca aphasia and four with transcortical motor aphasia. No patient presented with Wernicke aphasia. Conclusions The relatively high incidence of CA in our study suggests that bi-hemispheric language representation may be more prevalent in Bengali speakers than in speakers of other languages. The absence of crossed Wernicke aphasia in our study participants may represent a left-hemispheric advantage for receptive language abilities in Bengali speakers. Further studies are required to clarify whether idiosyncrasies in the Bengali language may be responsible for the differential brain representation of language seen in our study participants.

Objective Linguistic problem is common in Huntington's disease (HD) patients. It has been studied before in native speakers of alphabetic languages, such as English. As a hieroglyphic language, Chinese differs from alphabetic languages in terms of phonology, morphology, semantics and syntax. We aimed to investigate the linguistic characteristics of manifest HD in native speakers of Mandarin. Meanwhile, we expected to explore the linguistic differences associated with cortical or subcortical pathology.Methods Five HD patients and five Alzheimer's disease (AD) patients matched in age, gender, disease course and educational level were enrolled. All the participants were Mandarin native speakers. All finished history inquiry, physical examination, basic test, genetic test and neuropsychological assessment. Language evaluation was performed by Aphasia Battery of Chinese.Results HD patients had a mean disease course of 5.4±2.97 (range, 2-10) years. They showed a linguistic disorder close to transcortical motor aphasia. They exhibited prominent phonological impairment, as well as slight semantic and syntactic abnormality. Tonic errors were found in speech. Character structural errors and substitutions were detected in writing. In comparison, AD patients showed a more severe linguistic impairment, characterized by global aphasia with more semantic errors. Conclusion Mandarin-speaking HD patients have a transcortical motor aphasia-like disturbance with prominent phonological impairment, whereas AD patients have a more severe global aphasia with salient semantic impairment.

Purpose: the objective of this paper is to verify the effect of speech therapy intervention program in patients with non-fluent aphasia due to stroke in language tasks related to verbal fluency in semantic and phonological categories. Methods: Patients with aphasia due to stroke were selected to take part in this study. Two groups were formed: diagnosed patients with Broca/transcortical motor aphasia (GA), and a control group (healthy individuals). GA took a fluency verbal task (FAS, other complementary categories: phonological /p/ /l/ and semantic: “fruits” and “names”). These patients were all engaged in a language intervention program developed by the authors of this study. GA received speech therapy sessions (ten sessions lasting for an hour once a week), following a specific language program. After the sessions, the patients were re-evaluated. Results: GA had statistical significant improvement in the verbal fluency task after the speech therapy program (p-value < 0,001). Conclusion: The speech language therapy program we proposed was efficient enough to show improvement in the results for GA in the verbal fluency task. RESUMO Objetivo: verificar o efeito do programa de intervenção fonoaudiológica em pacientes com afasia não fluente após AVC na tarefa de fluência verbal em categorias semânticas e fonológicas. Método: Foram analisados casos de pacientes com diagnóstico de afasia de Broca e transcortical motora, secundárias ao AVC. Os pacientes realizaram o teste de fluência verbal (FAS, categorias fonológicas /p/ /l/, e semânticas “frutas” e “nomes”) e foram submetidos à intervenção fonoaudiológica, que foi constituída por 10 sessões de terapia realizadas de forma individual, semanalmente, com duração de uma hora. O programa de intervenção fonoaudiológica foi desenvolvido pelos autores deste estudo, contou com a realização de atividades de linguagem visando estimular e desenvolver estratégias para melhorar o acesso da linguagem expressiva do paciente afásico. Após o término do período de intervenção, os pacientes foram reavaliados, tendo sido realizada novamente a prova de fluência verbal. Resultados: GA contou com dez participantes (nove com afasia de Broca e um com afasia transcortical motora). Foi observada melhora estatisticamente significante na tarefa de fluência verbal pós-intervenção fonoaudiológica (p < 0,001). Conclusão: Observamos que a intervenção fonoaudiológica de linguagem proposta foi eficiente para apresentar melhora, com resultado estatisticamente significante na prova de fluência verbal para GA.

Two male participants with chronic (> 2 years), non-fluent aphasia and their family members participated in script training using videoconferencing. Functional scripts were developed by people with aphasia (PWA) and their family members. Accuracy of scripts was measured by total target words produced per turn. Participant 1 with Broca’s aphasia produced scripts with 0% accuracy pre-treatment and 87.5% accuracy post-treatment. Participant 2 with Transcortical Motor aphasia produced scripts with 20.2% accuracy pre-treatment and 63.5% accuracy post-treatment. Pre- and post-questionnaires for communication effectiveness and the use of telepractice for speech therapy indicated improvements in answering yes/no questions, participating in conversations with strangers, and increasing confidence and satisfaction with technology delivered treatment. The use of videoconferencing to deliver script training appears beneficial for individuals with chronic aphasia.

Основни циљ истраживања је био да се упореди квалитет комуникативног живота између особа са Брокином и Транскортикалном моторном афазијом. Узорак је чинило 20 одраслих испитаника, који су подељени у две групе. У прву је било укључено 10 особа код којих је постављена дијагноза Брокине афазије, док је другу групу чинило 10 одраслих особа код којих је постављена дијагноза Транскортикалне моторне фазије. Дијагноза афазије је постављена на основу Бостонског дијагностичког теста за афазије. Процена нивоа квалитета комуникативног живота извршена је помоћу Скале квалитета комуникацијског живота (QCL). Резултати показују да пацијенти са Брокином и Транскортикалном моторном афазијoм испољавају различит ниво квалитета комуникативног живота. The main cause of the research was to compare the quality of communication life among the people with Broca's aphasia and Transcortical motor aphasia,. The examination had included 20 adult examinees that had been divided further into two groups. The first group included 10 people which had been diagnosed with Broca's aphasia, and the second group included 10 adults with had been diagnosed with Transcortical motor aphasia. Aphasia was diagnosed based on the Boston diagnostic test for aphasia. The evaluation of the quality of communicative life was conducted under the Quality of communication life scale (QCL). Тhe results had shown that the patients with Broca's aphasia and Transcortical motor aphasia had different level of quality of communication life.

Background Understanding the pattern of non-linguistic abilities in aphasia has been a controversial question. We hypothesized that only some aphasia patients, particularly patients with fluent forms of aphasia and global aphasia, would present deficits in non-linguistic abilities. Methods & procedures We studied 200 vascular aphasia patients (119 men and 81 women; mean age = 57.37 years, SD = 15.56) at the Cognitive Communicative Speech Language Pathology Unit at the Clinical Hospital University of Chile (Santiago, Chile). The mean time post onset was 6.57 months (SD = 12.94). The Spanish versions of Western Aphasia Battery Revised (SWAB-R) and the Spanish version of the Boston Diagnostic Aphasia Examination (SBDAE) were administered. We used the SWAB-R Part 1 to determine the aphasia severity (Aphasia Quotient: AQ). SWAB-R Part 2 was used to study nonverbal abilities; a Non-Linguistic Quotient (NLQ) was calculated. The SBDAE was used in determining the type of aphasia. Outcomes & results Deficits were particularly evident in Global, Mixed non-fluent, and Transcortical Motor aphasia, followed by Wernicke and Transcortical Sensory aphasia. Deficits were mildest in Amnesic, Conduction, and Broca aphasia. Correlation between linguistic and non-linguistic deficits were found to be statistically significant. Conclusion Our results support previous studies: non-linguistic abilities can be affected in aphasia, but there is an important variability. Some aphasia patients can present non-linguistic deficits. Verbal and nonverbal deficits are significantly correlated, suggesting some communality in their brain organization.

Aim: To explore what is the relationship of the types of post-stroke aphasia with sex, age and stroke types. Methods: Retrospective analysis was administrated on data of 421 patients with acute stroke. Western battery aphasia was used to measure aphasiac type and aphasia quotient (AQ) score. The patients were divided into three age groups: young, middle-aged and elderly. The stroke types were classified into cerebral infraction (CI) and intracerebral hemorrhage (ICH). Results: All subjects were right-handed, which males and females accounted for 69.60% and 30.40%, respectively. There were 116 cases of Broca’s aphasia (85 males), 35 cases of Wernicke’s aphasia (20 males), 15 cases of conductive aphasia (10 males), 63 cases of transcortical motor aphasia (50 males), 11 cases of transcortical sensory aphasia (8 males), 27 cases of transcortical combined aphasia (13 males), 73 cases of anomic aphasia (47 males) and 81 cases of global aphasia (60 males). Male patients (69.60%) have a significantly higher morbidity of aphasia than that of females (30.40%) after stroke (χ2 = 11.57, P = 0.003), especially those under 65 years old (73.38%). For people 65 years and older, the morbidity of female (42.97%) tends to increase with age. Sex has no significant influence on the types of aphasia (χ2 = 13.84, P = 0.054). Broca’s aphasia is the most common type inboth male and female (29.01%, 24.22%, respectively). The distribution of aphasic types has no obvious difference among three age groups (χ2 = 14.94, P= 0.382). Aphasia induced by CI (306 cases) is more common than that by ICH (115 cases), but there was no difference in distribution of types of aphasia (χ2 = 13.23, P = 0.067). Conclusions: Male patients have a significantly higher level of morbidity of aphasia than females after stroke and a lower average age of onset than females. Broca’s aphasia is the most common one in both male and female. Broca’s aphasia, global and anomic aphasia are the most common aphasic types in both CI and ICH patients, except the female with ICH.

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.

Introduction: The neuropharmacology of aphasia is an area of cognitive neuroscience. In this article we review what is known about these domain, especially with regard to treating aphasia with drugs. Neurotransmitters can improve language function in certain patients with aphasia. We discuss which neurotransmitters work for which language functions in which patients.Method: PubMed and Science databases were searched to identify studies investigated effects of drugs on language function in aphasia in peer-reviewed journals between 2000 and 2015. Results: Studies show that Catecholamines seem especially promising for nonfluent aphasias. Dopamine agonists, in particular bromocriptine, improve verbal fluency in transcortical motor aphasia. Norepinephrine affects prefrontal functions and seems to relieve symptoms of depression and improve overall recovery of function, including language, following stroke. Amphetamines potentiate norepinephrine to promote general recovery, and have been shown to improve language performance in some patients with nonfluent aphasia.Conclusion: studies have been looking at the possibility of manipulating brain chemistry for functional gain in patients with aphasia, but, to date, no overwhelming evidence has emerged to support routine use of drugs as either a complementary or alternative treatment for aphasia. Studies have been largely anecdotal with small numbers of patients and varying types of aphasia. Improvements due to spontaneous recovery have been difficult to separate from treatment benefits.

医学部神経内科学教室 内山真一郎教授退任記念特別号

Patients with chronic lithium toxicity typically present with symptoms of agitation, confusion, tremor, ataxia, and hyperreflexia. In rare cases, however, lithium toxicity can present with transient cognitive and language deficits. We report the case of a lithium-toxic patient who presented with transient transcortical motor aphasia, which resolved 2 days after discontinuation of lithium. This is the first report of focal transcortical motor aphasia in a patient with lithium toxicity. Given the narrow therapeutic range of lithium, clinicians should be aware of the possibility of focal aphasia as a rare manifestation of lithium toxicity.

An 8-year-old boy underwent a resection for focal cortical dysplasia at the left supplementary motor area (SMA) for the treatment of intractable epilepsy. The manifestations of SMA syndrome, such as transient mutism and right hemiparesis, resolved within a few weeks. Verbal disfluency and impaired executive function, accompanied by impulsivity and distractibility, persisted for more than 12 months. The verbal and behavioral problems caused serious difficulties in the school life of the patient, until they became less evident at 18 months after surgery. Tractography performed 18 months after surgery revealed a defect in the subportion of fronto-parietal association fibers within the left superior longitudinal fascicles. Verbal influency can persist with unusually long duration after resection of SMA during childhood. Although not discernible on the routine neuroimaging, white matter damage beneath the SMA region could result in serious disabilities in executive function. These complications should be recognized for the prediction and assessment of deficits in children after surgical intervention involving this region.

A 74-year-old right-handed woman without cognitive impairment suddenly developed nonfluent aphasia. Brain MRI showed acute infarction in the right frontal lobe and insula without involvement of the corpus callosum. A neurological examination demonstrated not only transcortical motor aphasia, but also ideomotor apraxia and right hand predominant constructional apraxia (CA). To date, right hand predominant CA has only been reported in patients with corpus callosum lesions. The right hand predominant CA observed in our patient may be associated with the failure to transfer information on the spatial structure from the right hemisphere to the motor cortex of the left hemisphere.

A 78-year-old man with sudden-onset right-sided weakness and mutism had no history of fever, seizures, or headache. On examination, abulia, transcortical motor aphasia, and right dense hemiplegia were noted. Contrast-enhanced brain MRI suggested meningitis (figure 1) and ruled out stroke (figure 2). On CSF analysis, lymphocytic (94%) pleocytosis (163 cells/mm3), abundant Gram-positive cocci, and hyperproteinorrachy (48 mg/dL) with normal CSF:serum glucose ratio were noted. After 14 days of ceftriaxone plus vancomycin and recovery of hemiparesis (MRC 4/5), speech (improved fluency), and CSF–pleocytosis resolution (2 cells/mm3), the patient was discharged. Localized meningitis is rare. We found 2 reports: one stroke mimic1 and another associated with necrotizing vasculitis.2

Background: Recent studies imply that executive functions (EF) are closely related to our ability to comprehend and produce language. A number of findings suggest that functional communication and language recovery in aphasia depend not only on intact language abilities but on EF as well. Some patients with transcortical motor aphasia (TMA) show language deficits only in tasks in which conflicting representations must be resolved by executive processes. In line with these results, others have proposed that TMA should be referred to as “dysexecutive aphasia”. EF in aphasia have mostly been studied using neuropsychological tests, therefore there is a need for systematic experimental investigations of these skills.Aims: 1. To investigate EF in TMA, and to test whether executive dysfunctions are specific to TMA. 2. To experimentally measure different components of EF: updating working memory representations and inhibition of prepotent responses.Methods & Procedures: Five individuals with TMA, five patients wi...

For a long time, in a localizationist view of brain functioning, a combination of symptoms called "frontal syndrome" has been interpreted as the direct result of damages involving the frontal lobe(s). The goal of this review is to challenge this view, that is, to move to a hodotopical approach to lesion mapping, on the basis of new insights provided by intraoperative electrostimulation mapping investigations in patients who underwent awake surgery for cerebral tumors. These original data reported in the last decade break with the traditional dogma of a modular and fixed organization of the central nervous system, by switching to the concepts of cerebral connectivity and plasticity - i.e., a brain organization based on dynamic interrelationships between parallel distributed networks. According to this revisited model, "frontal symptoms" can be generated by tumor or electrostimulation not only of the frontal lobes, but also of cortical and subcortical (white matter pathways/deep gray nuclei) structures outside the frontal lobes: especially, stimulation of the superior longitudinal fascicle may elicit speech production disorders, syntactic disturbances, involuntary language switching or phonemic paraphasia (arcuate fascicle), stimulation of the inferior fronto-occipital fascicle can generate semantic paraphasia or deficit of cross-modal judgment, stimulation of the subcallosal fasciculus may elicit transcortical motor aphasia, while stimulation of the striatum induces preservations. On the other hand, it is also possible to perform extensive right or left frontal lobectomy in patients who continue to have a normal familial, social and professional life, without "frontal syndrome". Therefore, this provocative approach may open the door to a renewal in the modeling of brain processing as well as in its clinical applications, especially in the fields of cerebral surgery and functional rehabilitation. These findings illustrate well the need to reinforce links between cognitive neuroscience and clinical neurology/neurosurgery.

Background: While border-zone infarcts (BZI) account for about 10% of strokes, studies on related aphasia are infrequent. The aim of this work was to redefine specifically their early clinical pattern and evolution. Methods: We prospectively studied consecutive patients referred to our stroke unit within a 2-year period. Cases of aphasia in right-handed patients associated with a MRI confirmed left-sided hemispheric BZI were included. These patients had a standardized language examination in the first 48 h, at discharge from stroke unit and between 6 and 18 months later. Results: Eight patients were included. Three had anterior (MCA/ACA), two posterior (MCA/PCA), two both anterior and posterior, and one bilateral BZI. All our patients initially presented transcortical mixed aphasia, characterized by comprehension and naming difficulties associated with preserved repetition. In all patients, aphasia rapidly improved. It fully recovered within a few days in three patients. Initial improvement was marked, although incomplete in the five remaining patients: their aphasias specifically evolved according to the stroke location toward transcortical motor aphasia for the three patients with anterior BZI and transcortical sensory aphasia for the two patients with posterior BZI. All patients made a full language recovery within 18 months after stroke. Conclusions: We report a specific aphasic pattern associated with hemispheric BZI, including an excellent long-term outcome. These findings appear relevant to (i) clinically suspect BZI and (ii) plan rehabilitation and inform the patient and his family of likelihood of full language recovery.

Background The frequency and type of central nervous system involvement in primary Sjogren's syndrome (pSS) remain controversial. Brain magnetic resonance imaging (MRI) abnormalities in pSS are usually discrete hyperintense areas in the white matter. Tumefactive brain lesions have been rarely reported. Case report We describe a 31-year-old woman who exhibited transcortical motor aphasia, hemiparesis and partial motor seizures as the initial manifestation of pSS. Brain MRI revealed a large frontoparietal lesion extending into the corpus callosum. The patient had spontaneous recovery and developed sicca symptoms 6 months after onset. Primary SS was diagnosed on the basis of clinical features, abnormal Schirmer test findings, high levels of anti-La/SSB antibodies and positive salivary gland biopsy results. Conclusion The present case suggests that a pseudotumoral brain lesion can occur as an initial symptom of pSS.

A classical tenet of clinical neurology proposes that cerebellar disorders may give rise to speech motor disorders (ataxic dysarthria), but spare perceptual and cognitive aspects of verbal communication. During the past two decades, however, a variety of higher-order deficits of speech production, e.g., more or less exclusive agrammatism, amnesic or transcortical motor aphasia, have been noted in patients with vascular cerebellar lesions, and transient mutism following resection of posterior fossa tumors in children may develop into similar constellations. Perfusion studies provided evidence for cerebello-cerebral diaschisis as a possible pathomechanism in these instances. Tight functional connectivity between the language-dominant frontal lobe and the contralateral cerebellar hemisphere represents a prerequisite of such long-distance effects. Recent functional imaging data point at a contribution of the right cerebellar hemisphere, concomitant with language-dominant dorsolateral and medial frontal areas, to the temporal organization of a prearticulatory verbal code ('inner speech'), in terms of the sequencing of syllable strings at a speaker's habitual speech rate. Besides motor control, this network also appears to be engaged in executive functions, e.g., subvocal rehearsal mechanisms of verbal working memory, and seems to be recruited during distinct speech perception tasks. Taken together, thus, a prearticulatory verbal code bound to reciprocal right cerebellar/left frontal interactions might represent a common platform for a variety of cerebellar engagements in cognitive functions. The distinct computational operation provided by cerebellar structures within this framework appears to be the concatenation of syllable strings into coarticulated sequences.

The defining symptoms of transcortical motor aphasia (TCMA) are nonfluent verbal output with relatively preserved repetition. Other symptoms, such as naming difficulties, agrammatic output, or even some paraphasias, may occur, but these are not cardinal symptoms defining TCMA and are not necessary for the diagnosis. The core anatomy involved in TCMA is a lesion of the medial frontal cortex, especially the left presupplementary motor area (pre-SMA) and adjacent Brodmann’s area 32; a lesion of the left posterior inferior frontal cortex, especially pars opercularis and ventral lateral premotor cortex; or a lesion of the pathways between these frontal structures. TCMA occasionally has been reported with a lesion of the left basal ganglia, the left thalamus, or the ascending dopaminergic pathways. From a cognitive standpoint, TCMA can be conceptualized as a disorder of intention, in other words, as a disorder of initiation and continuation of spoken language that is internally motivated. The medial frontal cortex provides the impetus to speak; this impetus to speak is conveyed to lateral frontal structures through frontal–subcortical pathways where it activates various language production mechanisms. The influence of the ascending dopaminergic pathways may occur either through their heavy connections with the pre-SMA region or through their influence on the basal ganglia. The influence of the basal ganglia and thalamus probably occurs through their connections with the medial frontal cortex. Assessments for TCMA should involve a thorough evaluation of conversational or narrative language output and repetition. New treatments are available that attempt to engage right-hemisphere intention mechanisms with left-hand movements and may be effective in TCMA. Although dopamine agonists have also shown some positive effects in increasing verbal output in TCMA, trials have been small, and some caution must be exercised in interpreting these findings.

Introduction The symptomatology of transcortical motor aphasia (TMA) or dynamic aphasia (Luria & Tsvetkova, 1970) allows the clinician to investigate “the particularly interesting stage in transition from initial thought to final verbal expression” and “to study in pure form the lack of speech initiative” (Rubens, 1976,p. 302). The symptoms described in the literature include: difficulty initiating speech, limited spontaneous speech, intact repetition, good auditory comprehension. In contrast, agrammatic sentence production is associated with Broca’s aphasia. Language data from a participant presenting with TMA and also agrammatic symptoms who showed a good recovery are analyzed. The aim of this presentation is to tease apart the observed language features to arrive at a better understanding of the nature of specific symptoms and their impact on language processing. Methods Participant GS: a 17-year-old carpenter’s apprentice, suffered a massive stroke. A complete occlusion of the left anterior-cerebral-artery was diagnosed. Procedure From 4 weeks to 10 months post onset GS was administered standardized language tests and tasks covering all linguistic levels. A matched control person (MH) was also tested. Results Selected results and examples are given in Table 1. GS’s initiation of spontaneously produced speech is captured by his slow rate of production and verbal output for texts in comparison to control MH. As shown in Table 1, specific syntactic features showed impairment. At six months his aphasia had evolved into a mild impairment. Discussion The utterances produced by a classical TMA patient are usually grammatical. In part, this applies to G.S. However, he also reveals the following difficulties: simplification of syntactic structure, substitutions and omissions of articles, frequent omission of prepositions, verb agreement and selection errors, and reversal of subject and object noun phrases various sentence and discourse level tasks. GS’s difficulties initiating spontaneous speech in comparison to the relative sparing of speech evoked through external stimulation – especially repetition of spoken language, visual confrontation naming and good auditory comprehension skills can be distinguished from the agrammatic symptomatology by classifying GS as presenting with the third type of transcortical motor aphasia put forward by Freedman, Alexander, & Naeser (1984): “TMA during recovery from Broca’s aphasia”. Kleist (1934) provides support for this third type of TMA; he stressed that patients with ‘adynamic language’ revealed difficulty producing sentences to the point of agrammatism. Based on analyses of GS’s initial symptoms and the evolution of his language skills, those features associated with TMA can be distinguished from the Broca’s agrammatic symptoms. The agrammatic deficits are considered in part to stem from an Impairment of automatic processing. Due to the long latencies in initiating his utterances those categories particularly affected in GS, namely the omission of articles and prepositions, are exactly those features put forward by Whitaker (1976) to be automatically processed. On the other hand, the reversal errors are associated with an impairment of the linear scheme of the phrase observed in dynamic aphasia. In summary, despite an initial massive lesion, GS showed a remarkable recovery as shown by his language profile at six months post onset.

Production of complex discourse—lengthy, open-ended utterances and narratives—requires intact basic language operations, but it also requires a series of learned procedures for construction of complex, goal-directed communications. The progression of clinical disorders from transcortical motor aphasia to dynamic aphasia to discourse impairments represents a progression of procedural deficits from basic morpho-syntax to complex grammatical structures to narrative and a progression of lesions from posterior frontal to polar and/or lateral frontal to medial frontal. Two cases of impaired utilization of language exemplify the range of impairments from clearly aphasic agrammatic, nonfluency to less and less “aphasic” and more and more executive impairments from transcortical motor aphasia to dynamic aphasia to narrative discourse disorder. The clinical phenomenology of these disorders gradually comes to be more accurately defined in the terminology of executive deficits than that of aphasia. The executive deficits are, in turn, based on impairments in various components of attention. Specific impairments in energizing attention and setting response criteria associated, respectively, with lesions in superior medial and left ventrolateral frontal regions may cause defective recruitment of the procedures of complex language assembly. (JINS, 2006,12, 236–247.)

We report a patient (KAS) who presented with pure dynamic aphasia in the context of progressive supranuclear palsy (PSP). KAS had the hallmark propositional language impairment in the context of preserved naming, reading, repetition and comprehension skills. The severity of KAS's propositional language deficit was demonstrated to be comparable to other dynamic aphasic patients. Remarkably, despite virtually abolished propositional speech, KAS was unimpaired on word and sentence level generation tasks that required a single response. This dissociation was further investigated on two discourse level generation tasks that required the generation of multiple connected sentences. Quantitative production analysis and novelty measures demonstrated that her performance was extremely reduced and characterised by a lack of novel words and sentences and a tendency to perseverate. This pattern of performance suggests that there may be two subtypes of dynamic aphasia. Patients with the more documented first subtype have language-specific deficits, fail word and sentence level generation tests and have left inferior frontal gyrus lesions. Patients with the second subtype, like KAS, pass word and sentence level generation tests and fail discourse level generation tests. They have a verbal and non-verbal generation deficit and bilateral frontal and subcortical damage. Our findings are discussed with reference to executive functioning accounts of dynamic aphasia and models of speech production. We interpret our patients' impairment as being underpinned by a deficit in one set of mechanisms involved in discourse generation; namely the generation of a fluent sequence of novel thought.

Language after frontal injury is usually not overtly aphasic, i.e, the essential instrumentalities of language ? phonology, lexical-semantics, and most aspects of grammar ? are intact. Patients with frontal lobe lesions are impaired at level of assembly of complex language. This impairment has three prototypical forms although there is substantial overlap between the prototypes, both in the language procedures recruited and in the clinical phenomenology. Transcortical motor aphasia is impairment at the level of complex sentence construction. Dynamic aphasia is impairment at the level of schema assembly. Discourse disorder is an impairment of script assembly, reflected as deficient narrative capacity. One model for understanding these disorders is the “action planning” model. The capacity to utilize language instrumentalities to produce goal-directed communications requires the on-line recruitment of a number of procedures (schemas) at various levels of complexity. These procedures are learned within cultural constraints across the lifespan. They may be the single most practiced and over-learned capacities of humans.

Transcortical motor aphasia (TCMA) is an acquired impairment of language expression that occurs following neurologic damage that affects left frontal cortex and spares perisylvian regions. In some individuals with TCMA, verbal expression is rendered nonfluent due to difficulty spontaneously initiating and elaborating upon verbal messages. Nonfluency arises from impaired activation of intended messages and inhibition of competing verbal expressions. This impairment of the intentional aspects of language expression can be termed 'adynamia.' Because adynamic forms of TCMA occur infrequently, few systematic treatment investigations have been reported for this condition. Behavioral treatments have been proposed to engage intact frontal regions to improve the ability to initiate spontaneous verbal expression. Some data suggest that nonsymbolic limb movements performed in the context of speaking activities, a form of what Luria termed gestural reorganization, may improve the adynamic verbal expression. (1) In addition, the influence of pharmacologic treatment with bromocriptine, a dopaminergic agonist, has been considered for its effects on verbal nonfluency in aphasia. Individuals classified as TCMA are more likely to benefit than those with other forms of nonfluent aphasia, suggesting an influence of bromocriptine on circuits necessary to activate spontaneous language. Additional studies are warranted that contrast behavioral and pharmacologic interventions to determine optimal conditions to improve verbal expression in adynamic forms of aphasia.