Cognitive dysfunction in patients with spinocerebellar ataxia type 6

The aim of this study was to assess the cognitive functions of patients with spinocerebellar ataxia type 6 (SCA6). We examined 13 patients with genetically confirmed SCA6 and 13 healthy control subjects matched for age, years of education, global cognitive status, and intellectual ability. We administered verbal memory (word recall and word recognition), executive function (digit span, category and letter fluency, Frontal Assessment Battery, and Trail Making Test-A and B), and visuospatial construction (figure copying) tests. We found that the patients with SCA6 had significantly lower scores on the demanding word recall and letter fluency tests compared to the control subjects, while word recognition was well preserved in the patients with SCA6. The other executive functions tested, as well as visuospatial construction, were preserved in the SCA6 group. However, although memory encoding and storage processes were preserved, the retrieval of memorized information concerning frontal function might be selectively affected in patients with SCA6 compared to in control subjects. The impaired word recall and letter fluency noted in patients with SCA6 were interpreted as being related to a word-retrieval disability. Such dysfunctions may be attributed to damage in the frontal-cerebellum circuit owing to SCA6.     

The cognitive spectrum in neurodegenerative Langerhans cell histiocytosis

Clinical spectrum of cognitive troubles complicating neurodegenerative Langerhans cell histiocytosis (ND-LCH) is poorly known. The aim of this study is to evaluate cognitive functions in ND-LCH. The cognitive functions of a series of eight adult patients (7 males and 1 female; mean age 26 years IQ 25–75; range 20–33) suffering from clinical and/or radiological ND-LCH were evaluated using the following tests: (1) forward/backward digit and spatial span tasks of the WAIS-R scale and the Corsi block task, (2) the French version of the free and cued selective reminding test, (3) verbal fluency tests, (4) the Frontal Assessment Battery (FAB), (5) backward measurement of the verbal and visuospatial memories of the WAIS-R scale, (6) the Rey complex figure test, (7) the trail making tests A and B, (8) digit symbol and symbol search of the WAIS-IV scale, and (9) the Stroop test. Episodic (i.e. autobiographical or personal) memory free recall, categorical verbal fluency, phonological verbal fluency, visuospatial processing skills, attention, speed of processing, and sensitivity to interference were impaired in ND-LCH patients. In contrast, verbal and visuospatial short-term memories (i.e. immediate memories or forward span tasks) were preserved in all patients. Adult ND-LCH patients suffer from a severe but dissociated dysexecutive syndrome, mostly affecting executive strategies and relatively sparing short-term memory. Our study supports the need of assessing executive functions using comprehensive cognitive evaluation in ND-LCH patients for early diagnosis.     

Verbal fluency and executive dysfunction in amyotrophic lateral sclerosis (ALS)

Neuropsychological investigations of amyotrophic lateral sclerosis (ALS) patients have revealed variable results on specific tests, despite a similar overall cognitive profile of predominantly executive dysfunction with some evidence of memory impairment. The most striking and consistent deficit is found using tests of verbal fluency. The current investigation explored why verbal fluency is particularly sensitive to the impairment in ALS, by investigating some of the underlying cognitive processes: (i) intrinsic response generation; (ii) phonological loop functions; and (iii) simple word retrieval. Twenty-two ALS patients and 25 healthy controls were investigated. The battery included: (i) written and spoken letter-based fluency, category fluency, design fluency; (ii) the Phonological Similarities effect and Word Length Effect; and (iii) computerised sentence completion and confrontational naming. The tests were designed to control for motor speed and to accommodate for the range of disabilities that are present in ALS patients. Significant impairments were found on some tests of intrinsic response generation, namely the Written Verbal Fluency Test, Category Fluency Test (generation of animal names) and Design Fluency Test. Phonological loop functions appeared to be intact with evidence of both the Phonological Similarities and Word Length Effects, but the ALS patients displayed significantly reduced working memory capacity. No deficits were found on tests of simple word retrieval. The findings indicate that verbal fluency impairments in ALS patients result from a higher order dysfunction, implicating deficits in the supervisory attentional system or central executive component of working memory, and are not caused or exaggerated by an impairment in phonological loop functions or in primary linguistic abilities. The study also demonstrates the importance of controlling for differences in motor speed, which may have served to exaggerate the presence of cognitive deficits in ALS patients reported by some other studies.     

Cognitive consequences of early phase of literacy.

The effect of the degree of illiteracy (complete or incomplete) on phonological skills, verbal and visual memory and visuospatial skills is examined in 97 normal Brazilian adults who considered themselves illiterate, and 41 Brazilian school children aged 7 to 8 years, either nonreaders or beginning readers. Similar literacy effects were observed in children and in adults. Tasks involving phonological awareness and visual recognition memory of nonsense figures distinguish the best nonreaders and beginning readers. Children performed better than adults at oral repetition of short items and figure recall, and adults better than children at semantic verbal fluency, digit span, and word list recall. A principal component analysis of the correlations between tasks showed that phonological awareness/reading, phonological memory/oral repetition, and semantic verbal memory/fluency tasks, generated different components. The respective role of culturally based preschool activities and literacy on the cognitive functions that are explored in this study is discussed.     

Learning, memory, and executive control in individuals with obstructive sleep apnea syndrome

A range of neuropsychological deficits have been identified in individuals with obstructive sleep apnea syndrome (OSAS) and have been related to disruptions in function of the frontal cortex of the brain. We hypothesized that impairments in the use of strategic, frontally-mediated processes that facilitate learning and memory would be associated with deficits in the long-term episodic memory of verbal material (i.e., word lists). We evaluated 28 adults with OSAS and 24 controls (ranging from 28 to 60 years of age) using the California Verbal Learning Test. General executive abilities were assessed using the Wisconsin Card Sorting Test, Letter fluency, and Category fluency. Individuals with OSAS exhibited poorer recall across learning trials, less efficient use of semantic clustering, and poorer use of semantic cues. Retention of previously encoded information and recognition, however, were intact. With the exception of letter fluency, deficits were not observed in general executive control. Results are discussed within the context of disruptions in the interactions between long-term memory and executive abilities that are subserved by frontal and distal brain regions.     

Learning,Memory, and Executive Control in Individuals With Obstructive Sleep Apnea Syndrome

A range of neuropsychological deficits have been identified in individuals with obstructive sleep apnea syndrome (OSAS) and have been related to disruptions in function of the frontal cortex of the brain. We hypothesized that impairments in the use of strategic, frontally-mediated processes that facilitate learning and memory would be associated with deficits in the long-term episodic memory of verbal material (i.e., word lists). We evaluated 28 adults with OSAS and 24 controls (ranging from 28 to 60 years of age) using the California Verbal Learning Test. General executive abilities were assessed using the Wisconsin Card Sorting Test, Letter fluency, and Category fluency. Individuals with OSAS exhibited poorer recall across learning trials, less efficient use of semantic clustering, and poorer use of semantic cues. Retention of previously encoded information and recognition, however, were intact. With the exception of letter fluency, deficits were not observed in general executive control. Results are discussed within the context of disruptions in the interactions between long-term memory and executive abilities that are subserved by frontal and distal brain regions.     

Arithmetic fact retrieval and working memory in schizophrenia.

Despite its importance in every-day life and vocational rehabilitation, arithmetic ability has rarely been investigated in schizophrenic patients. Those few studies reporting arithmetic deficits in schizophrenia, however, administered complex calculation tasks which drew not only on arithmetic abilities, but also on working memory resources known to be impaired in schizophrenia. In the present study, arithmetic abilities and working memory functions were investigated in schizophrenic patients (n=24) and healthy control subjects (n=24). Arithmetic fact retrieval was assessed in single-digit multiplication and corresponding division problems using a result verification task which minimized working memory demands. Problem size and the disparity of the proposed result were manipulated. The storage component of working memory was tested with a digit span forward task and the executive control component with a digit span backward as well as with verbal fluency tasks. Schizophrenic patients performed worse than controls only in the executive tasks. Digit span forward was preserved. In the arithmetic tasks, groups did not differ from each other, and a similar pattern of task manipulations was obtained. Hence, despite the executive control deficit retrieval of arithmetic facts is preserved in schizophrenia. Moreover, the same underlying cognitive processes as in control subjects are involved.     

Longitudinal study of cognitive and psychiatric functions in spinocerebellar ataxia types 1 and 2

The role of the cerebellum in cognition, both in healthy subjects and in patients with cerebellar diseases, is debated. Neuropsychological studies in spinocerebellar ataxia type 1 (SCA1) and type 2 (SCA2) demonstrated impairments in executive functions, verbal memory, and visuospatial performances, but prospective evaluations are not available. Our aims were to assess progression of cognitive and psychiatric functions in patients with SCA1 and SCA2 in a longitudinal study. We evaluated at baseline 20 patients with SCA1, 22 patients with SCA2 and 17 matched controls. Two subgroups of patients (9 SCA1, 11 SCA2) were re-evaluated after 2 years. We tested cognitive functions (Mini Mental State Examination, digit span, Corsi span, verbal memory, attentional matrices, modified Wisconsin Card Sorting Test, Raven Progressive Matrices, Benton test, phonemic and semantic fluency), psychiatric status (Scales for Assessment of Negative and Positive Symptoms, Hamilton Depression and Anxiety Scales), neurological conditions (Scale for Assessment and Rating of Ataxia), and functional abilities (Unified Huntington Disease Rating Scale–part IV). At baseline, SCA1 and SCA2 patients had significant deficits compared to controls, mainly in executive functions (phonemic and semantic fluencies, attentional matrices); SCA2 showed further impairment in visuospatial and visuoperceptive tests (Raven matrices, Benton test, Corsi span). Both SCA groups had higher depression and negative symptoms, particularly apathy, compared to controls. After 2 years, motor and functional disability worsened, while only attentive performances deteriorated in SCA2. This longitudinal study showed dissociation in progression of motor disability and cognitive impairment, suggesting that in SCA1 and SCA2 motor and cognitive functions might be involved with different progression rates.     

Verbal fluency performance in dementia of the Alzheimer's type: a meta-analysis

A meta-analysis of 153 studies with 15,990 participants was conducted to compare the magnitude of deficits upon tests of phonemic and semantic fluency for patients with dementia of the Alzheimer's type (DAT) relative to healthy controls. As has been found for patients with focal temporal cortical lesions (but not for patients with focal frontal cortical lesions), DAT patients were significantly more impaired on tests of semantic relative to phonemic fluency (r=0.73 and 0.57, respectively). Thus, since phonemic and semantic fluency are considered to impose comparable demands upon executive control processes such as effortful retrieval, but the latter is relatively more dependent upon the integrity of semantic memory, these results suggest that the semantic memory deficit in DAT reflects a degradation of the semantic store. Also supporting this conclusion, confrontation naming, a measure of semantic memory that imposes only minimal demands upon effortful retrieval, was significantly more impaired than phonemic fluency (r=0.60 versus 0.55, respectively). However, since semantic fluency was also significantly more impaired than confrontation naming (r=0.73 versus 0.61), deficits in semantic memory and effortful retrieval may be additive. Semantic, but not phonemic fluency, was significantly more impaired than measures of verbal intelligence and psychomotor speed. Thus, the semantic memory deficit in DAT qualifies as a differential deficit, but executive dysfunction as indexed by phonemic fluency does not constitute an additional isolated feature of the disorder. Dementia severity was not significantly related to the relative magnitude of deficits upon phonemic and semantic fluency.     

Working memory after severe traumatic brain injury.

The aim of the present study was to assess the functioning of the different subsystems of working memory after severe traumatic brain injury (TBI). A total of 30 patients with severe chronic TBI and 28 controls received a comprehensive assessment of working memory addressing the phonological loop (forward and backward digit span; word length and phonological similarity effects), the visuospatial sketchpad (forward and backward visual spans), and the central executive (tasks requiring simultaneous storage and processing of information, dual-task processing, working memory updating). Results showed that there were only marginal group differences regarding the functioning of the two slave systems, whereas patients with severe TBI performed significantly poorer than controls on most central executive tasks, particularly on those requiring a high level of controlled processing. These results suggest that severe TBI is associated with an impairment of executive aspects of working memory. The anatomic substrate of this impairment remains to be elucidated. It might be related to a defective activation of a distributed network, including the dorsolateral prefrontal cortex.     

Impaired semantic knowledge underlies the reduced verbal short-term storage capacity in Alzheimer's disease

A decrease in verbal short-term memory (STM) capacity is consistently observed in patients with Alzheimer's disease (AD). Although this impairment has been mainly attributed to attentional deficits during encoding and maintenance, the progressive deterioration of semantic knowledge in early stages of AD may also be an important determinant of poor STM performance. The aim of this study was to examine the influence of semantic knowledge on verbal short-term memory storage capacity in normal aging and in AD by exploring the impact of word imageability on STM performance. Sixteen patients suffering from mild AD, 16 healthy elderly subjects and 16 young subjects performed an immediate serial recall task using word lists containing high or low imageability words. All participant groups recalled more high imageability words than low imageability words, but the effect of word imageability on verbal STM was greater in AD patients than in both the young and the elderly control groups. More precisely, AD patients showed a marked decrease in STM performance when presented with lists of low imageability words, whereas recall of high imageability words was relatively well preserved. Furthermore, AD patients displayed an abnormal proportion of phonological errors in the low imageability condition. Overall, these results indicate that the support of semantic knowledge on STM performance was impaired for lists of low imageability words in AD patients. More generally, these findings suggest that the deterioration of semantic knowledge is partly responsible for the poor verbal short-term storage capacity observed in AD.     

Developmental aspects of linguistic and mnestic abilities in normal children

The Peabody Picture Vocabulary Test-Revised (PPVT-R), Boston Naming Test (BNT), Paired Associate Learning of the Wechsler Memory Scale (PAL), and verbal fluency tests were administered to 241 normal children aged 6-12 years. Normative data were compiled for the BNT, PAL, and verbal fluency tests. A Principal Components Factor Analysis with Varimax Rotation was conducted to determine whether the tests evaluated similar or differential functions. Three factors emerged, accounting for 67.7% of the variance: Factor 1 contained loadings from two semantic fluency measures (animals and food), Factor 2 contained the PPVT-R and the BNT, and Factor 3 contained two measures from the PAL (easy and hard associations). In children, the BNT relates more to word knowledge than to retrieval or fluency, and verbal memory appears to be relatively independent of these linguistic functions.     

老年遗忘型轻度认知损害患者语言工作记忆损害特点

目的 探讨老年遗忘型轻度认知损害(aMCI)患者语言工作记忆损害的特点及机制.方法 采用语言工作记忆检查软件对30例老年aMCI患者进行视觉语言工作记忆及词语流畅性和数字广度测试等神经心理学检查,并选择30名健康老人作对照.结果 aMCI患者的视觉语义工作记忆测试成绩正确率低于对照组,差异具有统计学意义(79.83%±3.22%与87.00%±1.93%,t=-1.03,P=0.002);视觉语音工作记忆测试成绩也低于对照组,但差异无统计学意义(78.92%±8.60%与86.80%±2.14%,t=-2.34,P=0.060);逆序数字广度测试(1.53±0.86与3.63±0.56,t=-1.23,P=0.027)和词语流畅性测试分值均低于对照组(22.96±2.31与31.53±3.72,t=-1.08,P=0.004),差异具有统计学意义.结论 老年aMCI患者的视觉语义性语言工作记忆受损,语音性语言工作记忆相对保留;逆序数字广度和词语流畅性测试成绩亦显著降低.     

Is semantic fluency differentially impaired in schizophrenic patients with delusions?

The study of cognitive deficits in schizophrenia has recently focused upon semantics: the study of meaning. Delusions are a plausible manifestation of abnormal semantics because by definition they involve changes in personal meaning and belief. A symptom-based approach was used to investigate semantic and phonological fluency in a group of schizophrenic patients subdivided into those with delusions and those with no current delusions. The results demonstrated that deluded patients only were differentially impaired on a test of semantic fluency in comparison to phonological fluency. All subjects showed the same decline in performance over the time course of both tests indicating that retrieval speed in schizophrenia is no different from that of normal controls. Further analysis of word associations in two semantic categories (animals and body parts), revealed that deluded subjects have a more idiosyncratic organisation for animals. The findings of reduced semantic fluency production and poor logical word associations may represent a disorganised storage of semantic information in deluded patients, which in turn affects efficient access.     

Verbal memory and verbal fluency in adolescents with schizophrenia spectrum disorders

Aim: Although impaired verbal memory and verbal fluency are frequently found in adults with schizophrenia, there has been a paucity of studies investigating adolescents with schizophrenia. Thus, the aim of the present study was to investigate the main subcomponents of verbal memory and verbal fluency in adolescents with schizophrenia spectrum disorders. Methods: Verbal learning and memory and verbal fluency was assessed in 21 adolescents with schizophrenia spectrum disorders (mean age, 15.4 years) compared with 28 healthy adolescents (mean age, 15.1 years). Results: The patient group performed significantly below healthy controls on measures of learning, delayed recall and on a frequency estimation task. No differences between the groups were found for measures of recognition, retention, implicit memory, or susceptibility to interference. Although they had impaired delayed recall the patients remembered most of what they actually learned. The patient group was impaired on phonological and semantic fluency, but there were no differences between the groups with respect to clustering or switching on the fluency tasks, when controlling for total output. There was no disproportionate impairment in semantic, as compared to phonological fluency, in the patient group. Conclusions: Adolescents with schizophrenia spectrum disorders exhibit impairments in verbal learning and verbal fluency, which might have an impact on the individual's everyday functioning.     

Mixed speech dominance in the intracarotid sodium amytal procedure: Validity and criteria issues

The study of cognitive deficits in schizophrenia has recently focused upon semantics: the study of meaning. Delusions are a plausible manifestation of abnormal semantics because by definition they involve changes in personal meaning and belief. A symptom-based approach was used to investigate semantic and phonological fluency in a group of schizophrenic patients subdivided into those with delusions and those with no current delusions. The results demonstrated that deluded patients only were differentially impaired on a test of semantic fluency in comparison to phonological fluency. All subjects showed the same decline in performance over the time course of both tests indicating that retrieval speed in schizophrenia is no different from that of normal controls. Further analysis of word associations in two semantic categories (animals and body parts), revealed that deluded subjects have a more idiosyncratic organisation for animals. The findings of reduced semantic fluency production and poor logical word associations may represent a disorganised storage of semantic information in deluded patients, which in turn affects efficient access.     

Lexicality effects in word and nonword recall of semantic dementia and progressive nonfluent aphasia

Background: Verbal working memory is an essential component of many language functions, including sentence comprehension and word learning. As such, working memory has emerged as a domain of intense research interest both in aphasiology and in the broader field of cognitive neuroscience. The integrity of verbal working memory encoding relies on a fluid interaction between semantic and phonological processes. That is, we encode verbal detail using many cues related to both the sound and meaning of words. Lesion models can provide an effective means of parsing the contributions of phonological or semantic impairment to recall performance.

Methods & Procedures: We employed the lesion model approach here by contrasting the nature of lexicality errors incurred during recall of word and nonword sequences by three individuals with progressive nonfluent aphasia (a phonological dominant impairment) compared to that of two individuals with semantic dementia (a semantic dominant impairment). We focused on psycholinguistic attributes of correctly recalled stimuli relative to those that elicited a lexicality error (i.e., nonword → word OR word → nonword).

Outcomes & Results: Patients with semantic dementia showed greater sensitivity to phonological attributes (e.g., phoneme length, wordlikeness) of the target items relative to semantic attributes (e.g., familiarity). Patients with PNFA showed the opposite pattern, marked by sensitivity to word frequency, age of acquisition, familiarity, and imageability.

Conclusions: We interpret these results in favour of a processing strategy such that in the context of a focal phonological impairment patients revert to an over-reliance on preserved semantic processing abilities. In contrast, a focal semantic impairment forces both reliance on and hypersensitivity to phonological attributes of target words. We relate this interpretation to previous hypotheses about the nature of verbal short-term memory in progressive aphasia.     

Fonctions cognitives sous-jacentes aux déficits de fluence verbale dans la schizophrénie : revue de la littérature

Individuals with schizophrenia usually show impairment on various cognitive functions, including long-term memory, executive functions and language. Compared to healthy controls, their performance is impaired in verbal fluency tests. These tests require participants to generate in a given time as many words as they can, belonging to a given category (semantic fluency), or beginning with a specific letter (orthographic fluency). Various cognitive functions are involved in verbal fluency tests: psychomotor speed, executive functions, language, long-term verbal memory and semantic memory. The purpose of the present study was to clarify the functional origin of verbal fluency deficits in schizophrenia through a literature review. Nineteen articles were selected in Pubmed and PsycINFO after initial screening and detailed review. They were formally analyzed with regard to general performance, cognitive strategies used in semantic and orthographic fluency tests and, underlying cognitive origin of deficits. Results show that individuals with schizophrenia produce fewer words than healthy controls in both types of fluency tests. Their impairment is more apparent in semantic than in orthographic fluency tests. Results of studies reviewed also show that individuals with schizophrenia adopt the same clustering (i.e. production of related words within a subcategory) and switching (i.e. ability to shift between clusters when a subcategory is exhausted) strategies than healthy controls, but less efficiently. Several hypotheses, such as the impairment of executive functions, semantic memory or speed of information processing, were put forward to account for this finding. Interestingly, the few studies in which researchers performed an analysis of the semantic relationships between words produced in fluency tests showed a less semantic coherence among people with schizophrenia than in healthy controls. Such a difference could be explained by disorganization of semantic memory or impairment in the activation of conceptual representations in semantic memory. Studies in which correlations and regression analyzes were performed allow for clarifying the cognitive origin underlying verbal fluency deficits in schizophrenia. The links between these deficits and information processing speed as well as working memory are well established. These two cognitive domains also appear to be strong predictors of performance in semantic and orthographic fluency tasks in schizophrenia. Individuals with schizophrenia usually present with a significant slowdown in the speed of information processing. Such a slowdown is likely to account for their poor performance in verbal fluency tests, which require the independent and rapid production of words. Working memory disorders are also core cognitive symptoms of schizophrenia. Working memory is involved in verbal fluency tests since they involve “strategic” activation of verbal information in long-term memory, inhibition of inappropriate words, switching between clusters, etc. However, the concept of working memory also partially encompasses the notion of executive functions so that the results reported in the present literature review, according to which impairment of verbal fluency in schizophrenia results from working memory deficits but not from executive functions deficits, are difficult to interpret. Results are also less clear-cut in regard to verbal long-term memory and to language abilities. Finally, numerous studies had shown that individuals with schizophrenia present specific deficits in the organization of semantic memory. However, the impact of this deficit on verbal fluency was explored in one study only. To conclude, verbal fluency tests are sensitive to various neurocognitive conditions and are helpful for differential diagnosis. Further studies are still needed to clarify the functional origin of verbal fluency deficits in schizophrenia, in particular the differential role of executive functions and working memory as well as the impact of semantic memory impairment.     

Phonological short-term store impairment after cerebellar lesion: a single case study

The cerebellum is a recent addition to the growing list of cerebral areas involved in the multifaceted structural system that sustains verbal working memory (vWM), but its contribution is still a matter of debate. Here, we present a patient with a selective deficit of vWM resulting from a bilateral cerebellar ischemic lesion. After this acute event, the patient had impaired immediate and delayed word-serial recall and auditory-verbal delayed recognition. The digit span, however, was completely preserved. To investigate the cerebellar contribution to vWM, four experiments addressing the function of different vWM phonological loop components were performed 18 months after the lesion, and results were compared with normative data or, when needed, with a small group of matched controls. In Experiment 1, digit span was assessed with different presentation and response modalities using lists of digits of varying lengths. In Experiment 2, the articulatory rehearsal system was analyzed by measurement of word length and articulatory suppression effects. Experiment 3 was devoted to analyzing the phonological short-term store (ph-STS) by the recency effect, the phonological similarity effect, short-term forgetting, and unattended speech. Data suggested a possible key role of the semantic component of the processed material, which was tested in Experiment 4, in which word and nonword-serial recall with or without interpolating activity were analyzed. The patient showed noticeably reduced scores in the tasks that primarily or exclusively engaged activity of the ph-STS, namely those of Experiment 3, and good performance in the tests that investigated the recirculation of verbal information. This pattern of results implicates the ph-STS as the cognitive locus of the patient's deficit. This report demonstrates a cerebellar role in encoding and/or strengthening the phonological traces in vWM.     

How does linguistic knowledge contribute to short-term memory? Contrasting effects of impaired semantic knowledge and executive control

Background: Linguistic knowledge makes an important contribution to verbal STM. Some theories, including Baddeley's original conception of the episodic buffer, hold that harnessing linguistic knowledge to support STM is executively demanding. However, some recent evidence suggests that the linguistic contribution does not depend on executive resources.

Aims: In this study we tested the hypothesis that activation of language representations is automatic and that executive control is most important when the material to be remembered is incompatible with this automatic activation.

Methods & Procedures: Word list recall was tested in three patients with transcortical sensory aphasia (TSA) following stroke. All had preserved word repetition and digit span but poor comprehension associated with impaired executive control. They were compared with two semantic dementia (SD) patients with degraded semantic representations but intact executive control. Patients repeated word lists that varied in their semantic and syntactic resemblance to meaningful sentences.

Outcomes & Results: The executively impaired TSA patients showed large benefits of semantic and syntactic structure, indicating that their executive deficits did not interfere with the normal linguistic contribution to STM. Instead they showed severe deficits in repetition of scrambled word lists that did not follow usual syntactic rules. On these, the patients changed the word order to better fit their existing knowledge of syntactic structure. In contrast, the SD patients had no problems repeating words in unusual sequences but their semantic knowledge degradation led to frequent phonological errors due to a loss of “semantic binding”, the process by which semantic knowledge of words helps to constrain their phonological representation.

Conclusions: These findings suggest that linguistic support for STM consists of (a) automatic activation of semantic and syntactic knowledge and (b) executive processes that inhibit this activation when it is incompatible with the material to be remembered.