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We report a patient with asymmetric Bálint's syndrome (predominantly right-sided oculomotor apraxia and simultanagnosia and optic ataxia for the right hemispace), and multimodal agnosia (apperceptive visual agnosia and bilateral associative tactile agnosia) with accompanying right hemianopia, bilateral agraphesthesia, hemispatial neglect, global alexia with unavailable kinesthetic reading, and lexical agraphia for kanji (Japanese morphograms), after hemorrhage in the left parieto-occipito-temporal area. The coexistence of tactile agnosia, bilateral agraphesthesia, and ineffective kinesthetic reading suggests that tactile-kinesthetic information can be interrupted because of damage to the fiber connection from the parietal lobe to the occipito-temporal area, leading to these tactually related cognitive impairments.

Several studies have shown that people with Alzheimer's disease (AD) tend to use more pronouns than healthy aged adults when referring to entities during discourse. This referential behavior has been associated with the decrease of cognitive abilities, such as lexical retrieval difficulties or reduced abilities in working memory. However, the influence of certain important discourse factors on the referential choices made by people with AD has yet to be established. This study examines referential choices made at three discourse stages during narrative discourse (the introduction of a referent, the maintaining of the referent in focus, and the shift from one referent in focus to another). These referential choices are examined in increasingly complex referential contexts. In addition, this study investigates the relationships between referential choices and various cognitive abilities. To do so, the narrative discourses of 21 people with AD and 21 healthy adults were elicited using a newly developed storytelling in sequence task. The analyses focused on the production of three major referential expressions (indefinite expressions, definite expressions and pronouns) which are expected to vary according to discourse stage and the referential complexity of the stories. The results show that AD participants produce significantly fewer of the referential expressions expected at the introduction and shift stages than healthy aged adults produce. Nevertheless, the variation in the categories of referential expressions produced by the AD participants between the discourse stages is similar to that produced by the healthy aged adults, suggesting a preserved sensitivity to the factors manipulated in the task (i.e., discourse stages and referential complexity). This study also highlights the fact that different cognitive competences, especially executive abilities, are greatly involved in referential choices. The results add further evidence that referential choices rely on a variety of cognitive skills, depending on the discourse context in which they are made.

Introduction: Language dysfunction has recently been suggested to be one route to alexithymia, an impairment in recognizing and communicating one's own emotions. Neuropsychological evidence is needed to investigate the possibility that acquired language problems could underlie acquired alexithymia., Method: This project examined data from a large group of chronic stroke patients ( N = 118) to test whether self-reported or behavioral measures of language and communication problems were associated with alexithymia. We also examined the impact of hemisphere of damage on alexithymia., Results: We found no differences in alexithymia levels for patients with observed language impairments on brief tests of picture naming, comprehension, and reading vs unimpaired patients. However, self-reported communication difficulties were found to be associated with higher scores of alexithymia, even after controlling for depression and anxiety. Patients with left- versus right-hemisphere damage did not differ in their alexithymia scores., Conclusions: We found partial support for the language hypothesis of alexithymia. We discuss potential reasons for the discrepant findings between the self-report and objective language measures and suggest that self-report measures may be more sensitive to milder, more pragmatic language impairments, as opposed to the severe structural language impairments measured by the cognitive screening tests.

Introduction: Pure word deafness is a rare neurological disorder linked with an inability to comprehend speech. The precise localization of damage is still unclear., Case Presentation: A 72-year-old woman presented with acute verbal contact disturbances, disorientation, and anxiety. In the neurological examination a slight right hemiparesis was found, together with a pure word deafness (PWD) in neuropsychological tests. Neuroimages confirmed bilateral ischemic lesions in the superior temporal gyrus (STG) acute in the left hemisphere., Conclusions: Our case suggested that bilateral, symmetric lesions in the region of STG could cause pure world deafness. The diversity and severity of symptoms confirm the necessity of pure world deafness classification for subtypes.

Background: The relationship between spontaneous speech and formal language testing in people with brain tumors (gliomas) has been rarely studied. In clinical practice, formal testing is typically used, while spontaneous speech is less often evaluated quantitatively. However, spontaneous speech is quicker to sample and may be less prone to test/retest effects, making it a potential candidate for assessing language impairments when there is restricted time or when the patient is unable to undertake prolonged testing., Aim: To assess whether quantitative spontaneous speech analysis and formal testing detect comparable language impairments in people with gliomas. Specifically, we addressed (a) whether both measures detected comparable language impairments in our patient sample; and (b) which language levels, assessment times, and spontaneous speech variables were more often impaired in this subject group., Method: Five people with left perisylvian gliomas performed a spontaneous speech task and a formal language assessment. Tests were administered before surgery, within a week after surgery, and seven months after surgery. Performance on spontaneous speech was compared with that of 15 healthy speakers., Results: Language impairments were detected more often with both measures than with either measure independently. Lexical-semantic impairments were more common than phonological and grammatical impairments, and performance was equally impaired across assessment time points. Incomplete sentences and phonological paraphasias were the most common error types., Conclusions: In our sample both spontaneous speech analysis and formal testing detected comparable language impairments. Currently, we suggest that formal testing remains overall the better option, except for cases in which there are restrictions on testing time or the patient is too tired to undergo formal testing. In these cases, spontaneous speech may provide a viable alternative, particularly if automated analysis of spontaneous speech becomes more readily available in the future. These results await replication in a bigger sample and/or other populations.

Herpes simplex virus encephalitis (HSVE) commonly presents with severe amnesia due to virus-mediated destruction of key regions in the temporal lobes, although language and executive impairment has been described. Little is known however of the long-term cognitive changes in these patients, including changes that may happen with cortical reorganization. We describe a patient with HSVE who presented with a highly unusual late-onset language syndrome, which may reflect distal cortical changes after her original injury.

The herpes simplex encephalitis (HSE) patient reported in this study presented a left hemisphere lesion limited to the left insula and to the left anterior parahippocampal region. The patient was followed longitudinally, focusing on the aphasia type, the language recovery, and the integrity of semantic representations. The language deficit was of fluent type, without phonological impairment, and showed a good but incomplete recovery after four months. A semantic impairment was possible at the onset, but recovered quickly and did not present a disproportionate impairment of living categories.

We report the case of a patient with ischemic lesion of the left cerebellum, who showed specific deficits in processing past versus future tense of action verbs. These findings confirm, in the presence of cerebellar damage, previous results obtained with transcranial magnetic stimulation in healthy subjects and suggest a specificity of the left cerebellum for preparation of responses to the past tense of action verbs. As part of the procedural brain, the cerebellum could play a role in applying the linguistic rules for selection of morphemes typical of past and future tense formation.

Objective: The present research investigates language and praxis abilities in patients with Alzheimer's disease in order to study the relationship between these two cognitive domains., Method: The experimental evaluation of patients and control group performance was designed to permit a direct comparison of linguistic abilities (i.e., verb and noun naming and sentence comprehension) and praxic abilities (i.e., gesture execution for complex movements). Moreover, for the first time, action comprehension was explored using the Action Sequence Comprehension., Results and Conclusion: Analyses of variance (ANOVAs) and correlational analyses showed that a direct relationship may exist between language impairment and apraxia in patients with Alzheimer's disease. In addition, the production and comprehension of both language and action were equally impaired in patients, providing further evidence for a spectrum of concomitant linguistic and praxis deficits in Alzheimer's disease. Finally, the ability to correctly comprehend action semantics was related more directly to verb production ability than to noun production.

In the present research, we investigated the processing of Italian mass and count nouns and of their semantic and morphosyntactic attributes in people with neurodegenerative disease. The performance of a group of 26 Italian participants with Alzheimer's disease was studied in a semantic judgment task and a syntactic judgment task. Results were analyzed by means of mixed-effect models, revealing an interaction between task and stimulus category: The probability for correct responses to mass stimuli was significantly lower than that for count stimuli, but only in the semantic task. These findings confirm the major semantic impairment in dementia and suggest that mass nouns have particular features that make them more prone to impairment than count nouns for a progressively degenerating brain.

Verbal fluency tasks are commonly used in cognitive and developmental neuropsychology in assessing executive functions, language skills as well as divergent thinking. Twenty-two typically developing children and 22 children with ADHD between the ages of 8 and 12 years were examined using verbal fluency tasks, prepotent response inhibition, and working memory tests. The clinical group showed impaired inhibitory and spatial working memory processes. We used different qualitative analyses of verbal fluency tasks to explore the lexical and executive strategies (word clustering and switching), and the temporal properties of the responses. Children with ADHD had a leeway in applying relevant lexical or executive strategies related to difficulties in strategy using. The reduced efficiency of children with ADHD in semantic fluency task is based on suboptimal shifting between word clusters and is related to the lack of ability of producing new clusters of items. The group difference appeared at the level of accessing and/or activating common words; however, the executive process of searching the lexicon extensively is intact.

The purpose of this study was to evaluate immediate auditory and visual memory processes in learning disability subtypes of 40 children born preterm. Three subgroups of children were examined: (a) primary language disability group (n = 13), (b) perceptual-motor disability group (n = 14), and (c) no learning disability diagnosis group without identified language or perceptual-motor learning disability (n = 13). Between-group comparisons indicate no significant differences in immediate auditory or visual memory performances between language and perceptual-motor learning disability groups. Within-group comparisons revealed that both learning disability groups performed significantly lower on a task of immediate memory when the mode of stimulus presentation and mode of response were visual.

Developmental absence (agenesis) of the corpus callosum (AgCC) is a common brain malformation that presents in isolation or in association with other conditions. Variability in aspects of neuropsychological functioning has been reported in small samples of individuals. This study aimed to systematically review the literature to characterize the profile and severity of cognitive impairments in individuals with AgCC across development. A mean general intellectual ability was calculated and neuropsychological functioning was qualitatively examined. We discuss methodological limitations of the literature, inherent heterogeneity of the causes and comorbidities of AgCC, and the developmental aspect of AgCC in opposition to "split-brain patients."

This paper for the first time reports detailed neurolinguistic findings in a patient with Neuro-Sweet syndrome. In this patient the presenting symptoms of central nervous system (CNS) involvement primarily consisted of a selective grammar deficit restricted to spontaneous speech. On MRI a left prefrontal ischemic stroke (superior part BA 6) and two small subcortical left parietal infarctions were found. Neurolinguistic analyses, however, did not reveal a profile consistent with any observations of agrammatism caused by structural damage to the language areas critically involved in grammatical processing. It is hypothesized that selectively distorted grammar might reflect disruption of the frontosubcortical network involved in language processing. Prefrontal neurobehavioral abnormalities associated with functional disruption of the inferior medial frontal regions as demonstrated by SPECT, additionally suggest that agrammatic symptoms may be linked to a higher-level cognitive disorder following encephalopathic CNS involvement.

The Floating Harbor syndrome is a rare genetic disease characterized by a triad of clinical signs: specific dysmorphic facial features, short stature with delayed bone age, and language and speech disorders. These signs are, in most cases, associated with borderline normal intelligence to moderate delay concerning intellectual functioning. We report an extensive neuropsychological evaluation for an adult female patient and show, in particular, a severe visuospatial impairment. We discuss this deficit in the light of the previous reported cases and suggest that visuospatial abilities should be explored more systematically.

The profile of HIV-associated neurocognitive disorders (HAND) has classically been characterized as "subcortical," but questions have arisen as to whether aging with HIV in the antiretroviral therapy era has subtly shifted the expression of HAND into a more "cortical" disorder (e.g., decay of semantic memory stores). We evaluated this hypothesis by examining semantic fluency and its component processes (i.e., clustering and switching) in 257 individuals across four groups stratified by age (<40 and ≥50 years) and HIV serostatus. Jonckheere-Terpstra tests revealed significant monotonic trends for the combined effects of HIV and aging on overall semantic (and letter) fluency and switching, but not cluster size, with greatest deficits evident in the older adults with HIV infection. Within the older HIV-infected cohort, poorer switching was uniquely associated with self-reported declines in instrumental activities of daily living and deficits in learning and executive functions, but not semantic memory. Results suggest that HIV infection and aging may confer adverse additive effects on the executive components of semantic fluency (i.e., switching), rather than a degradation of semantic memory stores (i.e., cluster size), which is a profile that is most consistent with combined frontostriatal neuropathological burden of these two conditions.

This longitudinal study assessed cognition in Williams syndrome (WS) over a 5 year period using the same test battery over the two occasions of testing. The aim was to explore whether absolute levels of ability and relative cognitive strengths and weaknesses remain consistent over time. 27 participants with WS were assessed using the Woodcock Johnson Test of Cognitive Ability--Revised (WJ-R COG, Woodcock & Johnson, 1989, 1990). Results suggested some developmental progress over time, but at a slower rate than typically developing peers. Cognitive strengths and weaknesses were consistent, at least on those abilities assessed using the WJ-R COG.

Commensurate with the hypothesized neural dissociation between verb and noun generation, research in HIV infection shows that, relative to noun fluency, action (verb) fluency is disproportionately impaired, more strongly related to executive dysfunction, and more sensitive to declines in everyday functioning. However, whether the neurobiological correlates of HIV-associated deficits in verb and noun generation are separable have not heretofore been investigated. The present study examined the biomarker correlates of action and noun fluency in 74 participants with HIV infection. Biomarkers of viral burden, neuroaxonal damage, macrophage activation, neuroprotection, inflammation, and astrocytosis were measured in plasma and cerebrospinal fluid (CSF). Deficits in action, but not noun generation, were significantly associated with higher CSF levels of S100beta, a marker of astrocyte activation, even after controlling for antiretroviral therapy, current immune compromise, and general cognitive impairment. Concurrent validity for the frontal systems hypothesis of verb generation was provided by post hoc analyses demonstrating that S100beta was also associated with measures of executive functions, but not semantic memory or psychomotor speed. Overall, these findings suggest that HIV-associated impairment in action fluency, and executive dysfunction more generally, may reflect astrocytosis (i.e., elevated S100beta). Complementing the literature in HIV and other clinical populations with frontal systems involvement, these data also support the possible neurobiological dissociation of noun and verb generation.

Recognizing the specific speech act (Searle, 1969) that a speaker performs with an utterance is a fundamental feature of pragmatic competence. However, little is known about neurocognitive mediation of speech act comprehension. The present research examined the extent to which people with Parkinson's disease (PD) comprehend specific speech acts. In the first experiment, participants read conversational utterances and then performed a lexical decision task (decide whether a target string of letters was a word). Consistent with past research, nonimpaired participants performed this task more quickly when the target string was the speech act associated with the preceding utterance. In contrast, people with PD did not demonstrate this effect, suggesting that speech act activation is slowed or is not an automatic component of comprehension for people with PD. In a second study, participants were given unlimited time to indicate their recognition of the speech act performed with an utterance. PD participants were significantly poorer at this task than were control participants. We conclude that a previously undocumented language disorder exists in PD and that this disorder involves a selective deficit in speech act comprehension. Frontostriatal systems (the systems impaired in PD) likely contribute to normal speech act comprehension.

The purpose of this article is to present current evidence regarding the role of semantic memory in everyday actions. First we describe key models of everyday actions. We then discuss current evidence regarding the role of semantic memory in everyday actions. We reviewed articles reporting on the execution and representation of everyday actions in populations with semantic-memory deficits and single-object use in patients with semantic dementia. Although the evidence is sparse, the general conclusion of this review is that semantic memory seems necessary to support everyday actions. Finally, future challenges and research perspectives are discussed.

We report the case of a 69-year-old woman with cerebral infarction in the left anterior cingulate cortex and corpus callosum. She showed hyperlexia, which was a distinctive reading phenomenon, as well as ambient echolalia. Clinical features also included complex disorders such as visual groping, compulsive manipulation of tools, and callosal disconnection syndrome. She read words written on the cover of a book and repeated words emanating from unrelated conversations around her or from hospital announcements. The combination of these two features due to a focal lesion has never been reported previously. The supplementary motor area may control the execution of established subroutines according to external and internal inputs. Hyperlexia as well as the compulsive manipulation of tools could be interpreted as faulty inhibition of preexisting essentially intact motor subroutines by damage to the anterior cingulate cortex reciprocally interconnected with the supplementary motor area.

We present the case of a right-handed patient who received surgical treatment for a left frontal WHO grade II glioma invading the left inferior and middle frontal gyri, the head of the caudate nucleus, the anterior limb of the internal capsule and the anterior insula, in direct contact also with the anterior-superior part of the lentiform nucleus. The tumor resection was guided by direct electrical stimulation on brain areas, while the patient was awake. Adding a narrative production task to the neuropsychological assessment, we compared pre-, peri- and post-surgical language skills in order to analyze the effects of the tumor infiltration and the consequences of the left IFG resection, an area known to be involved in various language and cognitive processes. We showed that the tumor infiltration and its resection did not lead to the severe impairments predicted by the localization models assigning a significant role in language processing to the left frontal lobe, notably Broca's area. We showed that slow tumor evolution - the patient had been symptom-free for a long time - enabled compensatory mechanisms to process most language functions endangered by the tumor infiltration. However, a subtle fragility was observed in two language devices, i.e., reported speech and relative clauses, related to minor working memory deficits. This case study of a patient speaking without Broca's area illustrates the efficiency of brain plasticity, and shows the necessity to broaden pre-, peri-, post-surgery language and cognitive assessments.

Cluster analysis of test scores on expressive phonology and comprehension of words and sentences in 7-9-year-old children with preschool diagnosis of Autistic Disorder yielded 4 clusters. Cluster 1 (N = 11): phonology and comprehension both low; Cluster 2 (N = 4): phonology low, near average comprehension; Cluster 3 (N = 40): average phonology, comprehension low to low average; Cluster 4 (N = 7): average or better phonology and comprehension. The clusters support two major types of language disorders in autism driven by impaired expressive phonology, each divisible by comprehension ability. The clusters refute a single language disorder in autism and are consonant with earlier-defined clinical subtypes.

HIV infection is associated with deficits in category fluency, but the underlying cognitive mechanisms of such impairments have not been determined. Considering the preferential disruption of the structure and function of frontostriatal circuits in HIV disease, the present study evaluated the hypothesis that HIV-associated category fluency deficits are driven by impaired switching. Study participants were 96 HIV-infected individuals and 43 demographically comparable healthy comparison volunteers who were administered a standard measure of animal fluency and an alternating category fluency task (i.e., fruits and furniture) in a randomized order. Consistent with prior research on letter fluency, HIV infection was associated with greater impairments in switching, but not semantic clustering within the animal fluency task. Moreover, a significant interaction was observed whereby the HIV-associated deficits in switching were exacerbated by the explicit demands of the alternating fluency task. Across both fluency tasks, switching demonstrated generally small correlations with standard clinical measures of executive functions, working memory, and semantic memory. Collectively, these findings suggest that HIV-associated category fluency deficits are driven by switching impairments and related cognitive abilities (e.g., mental flexibility), perhaps reflecting underlying neuropathology within prefrontostriatal networks.

In addition to various lexical errors, the writing of patients with deep dysgraphia may include a large number of segmental spelling errors, which increase towards the end of the word. Frequently, these errors involve deletion of two or more letters resulting in so-called "fragment errors". Different positions have been brought forward regarding their origin, including rapid decay of activation in the graphemic buffer and an impairment of more central (i.e., lexical or semantic) processing. We present data from a patient (M.D.) with deep dysgraphia who showed an increase of segmental spelling errors towards the end of the word. Several tasks were carried out to explore M.D.'s underlying functional impairment. Errors affected word-final positions in tasks like backward spelling and fragment completion. In a delayed copying task, length of the delay had no influence. In addition, when asked to recall three serially presented letters, a task which had not been carried out before, M.D. exhibited a preference for the first and the third letter and poor performance for the second letter. M.D.'s performance on these tasks contradicts the rapid decay account and instead supports a lexical-semantic account of segmental errors in deep dysgraphia. In addition, the results fit well with an implemented computational model of deep dysgraphia and segmental spelling errors.

In this study the verbal fluency procedure was used to investigate the organization of semantic and phonological representations in children with Down syndrome (DS) and typically developing children, matched pairwise for receptive vocabulary age. Productivity was found to be significantly reduced in the DS group in both the semantic and the phonological tasks. However, group differences in the number of clusters as opposed to cluster size suggest that this may reflect less efficient retrieval strategies rather than differences in the organization of linguistic representations. Together the findings point to executive deficits in Down syndrome rather than deviant language processes.

To test conflicting hypotheses regarding amnesic H.M.'s language abilities, this study examined H.M.'s sentence production on the Language Competence Test (Wiig & Secord, 1988). The task for H.M. and 8 education-, age-, and IQ-matched controls was to describe pictures using a single grammatical sentence containing prespecified target words. The results indicated selective deficits in H.M.'s picture descriptions: H.M. produced fewer single grammatical sentences, included fewer target words, and described the pictures less completely and accurately than did the controls. However, H.M.'s deficits diminished with repeated processing of unfamiliar stimuli and disappeared for familiar stimuli-effects that help explain why other researchers have concluded that H.M.'s language production is intact. Besides resolving the conflicting hypotheses, present results replicated other well-controlled sentence production results and indicated that H.M.'s language and memory exhibit parallel deficits and sparing. Present results comport in detail with binding theory but pose problems for current systems theories of H.M.'s condition.

Patients with 'refractory semantic access deficits' demonstrate several unique features that make them important sources of insight into the organization of semantic representations. Here we attempt to replicate several novel findings from single-case studies reported in the literature. Patient UM-103 displays the cardinal features of a 'refractory semantic access deficit' and showed many of the same effects of semantic relatedness reported in the literature. However, when probing concrete and abstract words, this patient revealed very different patterns of performance compared to two previously reported patients. We discuss the implications of our data for models of semantic organization of abstract and concrete words.

Creatine transporter deficit (CT1) is an inherited metabolic disorder that causes mental retardation, epilepsy, speech, language and behavioral deficits. Until now, no treatment has been proven to be successful for this condition. We describe 1-year follow-up study of a child, aged 9.6 years, with CT1 defect, on oral supplementation with L-arginine, a precursor of creatine synthesis. Under supplementation, he showed a noticeable improvement of neurological, language and behavioral status and an increase of brain creatine and phosphocreatine documented with magnetic resonance spectroscopy. The results suggest that children with CT1 disorder show some residual adaptive plasticity for certain functions even at quite an advanced age. Further trials with higher L-arginine dosages and more protracted treatment are encouraged.

The role of slowing of processing speed in verbal memory impairment in patients with schizophrenia was investigated. Forty-one patients with schizophrenia and 41 healthy control subjects were administered a verbal memory task involving free recall of three lists of words, which varied in their degree of semantic organization. Standard processing speed tests were administered as well. Regression analyses were conducted on the number of words recalled in each list. A global processing speed measure was a significant predictor of the recall of each list in patients. Patients were very significantly impaired in the recall of the three lists relative to healthy controls. However, when the processing speed measure was entered in the regression, the significance of diagnosis was considerably reduced for one of the lists, with no semantic organization, and eliminated for the other two lists which contained semantic organization. These findings suggest that slowing in processing speed is an important contributor to verbal memory impairment in patients with schizophrenia. The possible role of various specific slowing functions is discussed.

Relatively little is known about the neuropsychological profile of late-stage semantic dementia. This article provides a detailed assessment of patient MK who, despite her very severe semantic impairments, remained cooperative to testing and, unusually, did not show additional behavioral/personality changes. Although MK's initial presentation was typical of semantic dementia (SD), her performance began to deviate from the normal pattern. She developed impairments of single word repetition and regular word reading, and began to produce phonological errors in picture naming and spontaneous speech. These deficits might suggest that late-stage SD includes an independent disorder of phonology. An alternative possibility, however, is that phonological processing cannot proceed normally in the face of profound semantic degradation. A series of experiments supported the latter explanation of MK's deficits. In picture naming, MK showed little effect of progressive phonological cueing, did not reveal an increased sensitivity to word length or phonological complexity and continued to show a high degree of item-specific consistency in both accuracy and errors: she tended to produce the same erroneous phonemes for each item. She remained sensitive to the effects of phonological similarity in immediate serial recall. Letter substitution errors in regular word reading were more common for lower frequency letters (e.g., Q, Z). These letters also produced more item errors in immediate serial recall, suggesting that a frequency-graded loss of letter knowledge, rather than separate orthographic and phonological deficits, accounted for the deficits in both of these tasks. These findings are discussed in terms of theories that posit strong interactivity between phonology and semantics.

The retention of first-order theory of mind (ToM) despite severe loss of grammar has been reported in two patients with left hemisphere brain damage (Varley & Siegal, 2000; Varley, Siegal, & Want, 2001). We report a third, and more detailed, case study. Patient PH shows significant general language impairment, and severe grammatical impairment similar to that reported in previous studies. In addition we were able to show that PH's impairment extends to grammatical constructions most closely related to ToM in studies of children (embedded complement clauses and relative clauses). Despite this, PH performed almost perfectly on first-order false belief tasks and on a novel nonverbal second-order false belief task. PH was also successful on a novel test of "ToM semantics" that required evaluation of the certainty implied by different mental state terms. The data strongly suggest that grammar is not a necessary source of structure for explicit ToM reasoning in adults, but do not rule out a critical role for "ToM semantics." In turn this suggests that the relationship observed between grammar and ToM in studies of children is the result of an exclusively developmental process.

We describe a patient who came to neurological attention because of his at-risk status for the +16 exon 10 splice mutation in the tau gene (microtubule associated protein tau, MAPT), which had given rise to progressive behavioural disturbances in two of his siblings. The patient began to exhibit early signs of behavioural disturbance at around the age of symptom onset in both of his siblings. Although he did not spontaneously complain of difficulties in the domain of language, he met clinical, radiological and neuropsychological criteria for semantic dementia. On the assumption that his illness is mediated by the same pathological process as those of his siblings, we propose that this clinical picture represents the earliest changes of a semantic impairment - a phase of the illness that is often retrospectively described by patients and their relations, but has never previously been documented at first hand. Although typical of semantic dementia in many respects, the illness had several interesting and atypical features that emerged on detailed testing: first, he exhibited no insight into his difficulties; secondly, progression over a twelve-month interval was unusually slow; thirdly, he evinced a striking and consistent advantage for nonliving over living concepts; fourthly, a differential impairment of distinctive over shared knowledge did not emerge except when items that he could still name were compared with those for which he was anomic. Finally, the availability of post mortem pathological analysis from the brains of both of his affected siblings allowed us to attribute his illness to a specific pathological process which is considered unusual for patients with this clinical phenotype.

The present study investigated auditory attention skills in a sample of children with non-chronic otitis media with effusion (OME). Twenty children with repeated episodes of OME but not found in the need for myringotomy and insertion of ventilating tubes were compared to 20 control children with no known episodes of OME based on parental reports and medical records. Mean age during assessment was 9 years, and none of the children showed signs of impaired language functions and with normal general cognitive abilities. They were assessed with dichotic listening CV-syllables (DLCV-108) free recall and directed attention tasks. The control children showed the expected right ear advantage during free recall and the directed right condition, and demonstrated a shift toward a left ear advantage during the directed left. The children with a history of OME showed a predominant right ear advantage across all three tasks. Although some change in ear accuracy occurred across tasks, impaired auditory attention skills were found following a history of middle ear infections. These results replicate those reported earlier from a sample of children with persistent otitis media with effusion, and suggest that treatment with ventilating tubes does not appear to make any difference in the development of auditory attention skills, whereas occurrence of OME must be considered when testing auditory attentional skills as a part of a neuropsychological assessment.

This study examined executive dysfunction and its relation to language ability in verbal school-age children with autism. Participants were 37 children with autism and 31 nonautistic comparison participants who were matched on age and on verbal and nonverbal IQ but not on language ability, which was lower in the autism group. Children with autism exhibited deficits compared to the comparison group across all 3 domains of executive function that were assessed including working memory (Block Span Backward; Isaacs & Vargha-Khadem, 1989), working memory and inhibitory control (NEPSY Knock-Tap; Korkman, Kirk, & Kemp, 1998), and planning (NEPSY Tower; Korkman et al., 1998). Children with autism were less developed than the comparison group in their language skills, but correlational analyses revealed no specific association between language ability and executive performance in the autism group. In contrast, executive performance was positively correlated with language ability in the comparison group. This pattern of findings suggest that executive dysfunction in autism is not directly related to language impairment per se but rather involves an executive failure to use of language for self-regulation.

This article describes an investigation into the residual writing skills of a severely dysgraphic patient (DA). We found that they were powerfully influenced by a number of lexical variables (lexicality, frequency, imageability, length and geminates). His error pattern was characterized by semantic, lexical, substitution, deletion errors and fragment responses that preserved the first letter. Thus, DA's written spelling was characterized by both deep dysgraphic and graphemic output buffer effects. It is proposed that this pattern of performance represents a new"putative functional syndrome."

In this paper we present the case of a child with early left thalamic vascular damage who subsequently developed a language disorder. At 3 years and 8 months, her language was poor and unintelligible and showed phonetic, phonological and morpho-syntactic disorders. She did not exhibit any signs of mental retardation. After specific speech therapy, she improved in all linguistic skills. Given the lack of reports on thalamic lesions in children, this paper describes the effect of a thalamic injury in the earliest phases of language acquisition in a child who showed consistent phonological disorders. This case seems to confirm early hemispheric specialisation and the importance of a timely therapy.

The aim of this study was to investigate digit span performance in dyslexia. It was hypothesised that differences would be found in accordance with subgrouping by language comprehension and mathematic skills, and by analyses of how the digit span scores were attained. Two digit span tasks were given to a group of dyslexic children and controls (n = 57), mean age 12.62 (SD = 1.43). The tasks were "Digit Span" of the WISC-R, and "Digit Span 2," where the use of back-up strategies like finger counting and lip reading were restricted. As expected, the digit span scores were significantly lower in the dyslexia group than in the control group. Restrictions of back-up strategies did not alter the scores in the control group, while the scores were lowered in the dyslexia group. Further analyses of longest digit span, serial recall, and serial position indicated different retrieval patterns in the subgroups. The subgroup with good language comprehension and mathematic skills (n = 12), showed impaired serial recall especially in backward recall. The subgroup with good language comprehension skills, but with mathematics impairment (n = 9), showed impaired serial recall in both forward and backward recall. The subgroup with language impairments (n = 16), recalled fewer digits than the two other subgroups. The findings were discussed in relation to the "Phonological loop" of the Multi Component Model of Working Memory, and implications for intervention were discussed.

The Sensory/Functional Theory, until recently the received explanation of category-specific semantic deficits, has been shown to be at variance with various facts that have emerged about the nature of these deficits. In this context, Rosazza, Imbornone, Zorzi, Farina, Chiavari, and Cappa (2003: The Heterogeneity of Category-Specific Semantic Disorders: Evidence from a New Case. Neurocase, 9, 189-202.) report the case study of a patient, MA, with a purported category-specific semantic deficit for living things compared to nonliving things, and an associated modality-specific impairment that differentially affected visual/perceptual compared to functional/associative knowledge. While acknowledging that the Sensory/Functional Theory cannot account for the existence of category-specific semantic deficits, Rosazza and colleagues (2003) contend that "...the presence of a more severe loss of specific visual rather than functional knowledge could support an interpretation according to the Sensory/Functional Theory" [sic] (p. 200). Our comments are divided into two parts. First, we point out that there is an asymmetry between evidence and theory: if there is clear evidence that "disconfirms" a given theory, evidence that is consistent with the theory cannot be argued to support the theory. Second, we argue that the performance profile of MA could potentially be relevant to other interpretations of category-specific deficits but that theoretical interpretations of the performance profile of patient MA are undermined by a lack of methodological rigor, as well as the generally weak data associated with the case.

We report the performance of a herpes simplex encephalitis patient, EA, who shows a dissociation between poor performance for living things and spared performance for sensory quality categories, when appropriate measures are taken to control for baseline differences among categories in task difficulty and when we eliminate the contribution of visual processing deficits in the recognition of objects. This pattern of performance is problematic for theories of category-specific deficits that are based on the assumption that these deficits reflect selective damage to modality-specific semantic subsystems but is consistent with domain-specific accounts and theories based on the assumption that the structure of conceptual knowledge reflects the correlational structure among object properties.

We report a new case of category-specific semantic impairment, affecting living entities, in a patient with traumatic brain damage. In the present investigation we attempted to replicate as closely as possible the testing procedures which have been developed by Caramazza and Shelton (1998) to evaluate EW, a patient with a selective semantic disorder for the animal category. The results in our patient indicated a different performance profile, characterised by a more extensive semantic disorder for living entities, and by a more severe loss of specific visual rather than functional knowledge. These findings concur with other evidence indicating that category-specific semantic disorders are heterogeneous, reflecting different mechanisms of impairment, most likely associated with different neurobiological underpinnings.

We describe a 76-year-old man (ADY) with dynamic aphasia in the setting of a degenerative frontal lobe dementia: primary progressive dynamic aphasia. He displayed a striking paucity of propositional speech despite intact speech production, and preserved singing and prosody. Vocal expression in the verbal and musical domains was investigated in a series of neuropsychological experiments based on novel language and musical tasks that were designed to establish the nature and specificity of the verbal output deficit. The features of the language disorder indicated that the speech output pathway was disrupted at the early stage of generation of a new pre-verbal message. In contrast, tests of musical output demonstrated that the generation of new musical ideas was unimpaired. The domain-specificity of dynamic aphasia may result from the disruption of specific cognitive processes necessary for the creation of verbal messages, as well as selective damage of brain regions involved in language production.

In recent years research has focused on the exact nature of the linguistic skills that individuals with Williams syndrome (WS) exhibit. This work has resulted in controversial positions, with an increasing number of studies casting doubt on previous claims of superior linguistic competence for individuals with WS. This study investigated morphosyntactic knowledge in Hebrew-speaking adolescents with WS. The participants 'performance was compared to 2 groups of typically developing mental age-matched controls. Participants and controls were tested on experimental tasks designed to investigate knowledge of morphology. The findings suggest that individuals with WS have good control over the basic consonantal root structure of Hebrew words. However, rather poor performance was evident on other morphological paradigms. We conclude that there is little evidence from Hebrew to support a selective preservation of grammatical competence in individuals with WS.

A group of adolescents with Williams syndrome (WS) was compared to matched groups of adolescents with Prader-Willi syndrome and nonspecific mental retardation on a task that tested the ability to distinguish different forms of nonliteral language. Participants listened to stories that ended in either a lie or an ironic joke. They were asked to decide the form of the nonliteral utterance and justify their responses. Almost none of the participants in any of the groups were able to correctly classify the ironic jokes, instead judging them to be lies because they did not correspond to reality. Their errors were similar to those made by younger normally developing children, but contrasted with those made by brain-damaged adults. These data are taken as further evidence that neurodevelopmental disorders are quite different from acquired brain disorders and require different neuropsychological models. The findings from this study also have important implications for considering the difficulties that adolescents with WS and other disorders will have in everyday social situations, especially among peers.

This study examines the memory functioning of 25 children who sustained a traumatic brain injury (TBI) and who had prior learning problems, 48 children with TBI who did not have prior learning problems, and 23 noninjured controls. The children with TBI and prior learning problems displayed significantly worse memory abilities than both the control participants and the children with TBI and no prior learning problems. They differed significantly from these 2 groups on measures of general memory, verbal memory, sound-symbol learning, and attention. The results suggest that children with premorbid learning problems who sustain TBI have less cognitive reserve and a lower threshold for the expression of cognitive impairments in areas that reflect preexisting learning and language problems, compared to children without premorbid learning problems.

The neuropsychological symptomatology is reported for a 44-year-old patient of normal intelligence, EE, after removal of a circumscribed left hemispheric tumor the major part of which was located in the angular gyrus and in the subcortical white matter. EE had a distinct and persistent short-term memory impairment together with an equally severe impairment in transcoding numbers. On the other hand, his performance was flawless in calculation tasks and in all other tests involving number processing. Impairments in language tests could be attributed to his short-term memory deficit, which furthermore was characterized by a strong primacy effect in the absence of a recency effect. His graphomotoric output was temporarily inhibited. The patient, with a strong left-sided dominance, manifested a bi-hemispherical activation of the Broca and Wernicke regions in a positron-emission-tomographic investigation when required to produce verbs which he was to derive from nouns. The findings in EE suggest that unilateral and restricted lateral parietal damage can result in a profound short-term memory deficit together with a transcoding deficit for stimuli extending over only a few digits or syllables in the absence of any symptoms of the Gerstmann syndrome.

Non-insulin-dependent diabetes mellitus is present in 7-10% of the aged. This disease appears to be associated with an acceleration of the aging process and results in compromised performance on learning and memory tasks. The present study used a verbal fluency test to examine semantic memory performance in two age groups (55-64 and 65-74 years) of diabetic subjects and controls. In addition, immediate and secondary memory were also examined using the digit symbol and digit span tests and a serial learning task. Results showed that digit symbol performance was poorer for older subjects and diabetics while serial learning was poorer only for diabetic subjects. However, the number of words generated on the verbal fluency test was similar for all groups. Qualitative analyses of the verbal output revealed that older subjects and diabetics produced the greatest number of previously recited words (repetitions). Repetitions may signal a failure to adequately monitor behavior which in turn could contribute to cognitive decline.