Impaired information integration contributes to communication difficulty in corticobasal syndrome.

OBJECTIVE: To investigate the cognitive and neural correlates of discourse impairment in corticobasal syndrome (CBS). BACKGROUND: Difficulty communicating is a frequent clinical manifestation in patients with CBS. However, the mechanisms underlying this disabling problem are not well understood. METHODS: Twenty patients with CBS and 8 healthy seniors narrated a picture story. Narratives were analyzed for maintenance of the narrative theme, identification of the overall point of the story (global connectedness), and connectedness between consecutive events (local connectedness). Discourse measures were correlated with performance on cognitive tasks and with cortical atrophy as determined by magnetic resonance imaging voxel-based morphometry. RESULTS: Patients with CBS referred to the narrative theme significantly less frequently than controls. Global connectedness was intact in only 6 of 20 CBS patients (30%), but preserved in all controls. Local connectedness was significantly diminished in patients relative to controls. Discourse performance in CBS was related to tasks requiring higher-order integration of visual material, but not to basic visuospatial/visuoperceptual, language, or memory function. Discourse impairment was directly related to atrophy in the right parietal lobe and bilateral dorsolateral prefrontal cortex. CONCLUSIONS: Our findings suggest that impaired information integration in CBS, related to parieto-frontal disease, interferes with patients' ability to narrate a coherent story.

Non-Fluent Speech in Frontotemporal Lobar Degeneration.

We investigated the cognitive and neural bases of impaired speech fluency, a central feature of primary progressive aphasia. Speech fluency was assessed in 35 patients with frontotemporal lobar degeneration (FTLD) who presented with progressive non-fluent aphasia (PNFA, n=11), semantic dementia (SemD, n=12), or a social and executive disorder without aphasia (SOC/EXEC, n=12). Fluency was quantified as the number of words per minute in an extended, semi-structured speech sample. This was related to language characteristics of the speech sample and to neuropsychological measures. PNFA patients were significantly less fluent than controls and other FTLD patients. Fluency correlated with grammatical expression but not with speech errors or executive difficulty. SemD and SOC/EXEC patients were also less fluent than controls. In SemD, fluency was associated with semantically limited content. In SOC/EXEC, fluency was associated with executive limitations. Voxel-based morphometry analyses of high-resolution MRI related fluency to gray matter volume in left inferior frontal, insula, and superior temporal regions for the entire cohort of FTLD patients. This region overlapped partially distinct atrophic areas in each FTLD subgroup. It thus appears to play a crucial role in speech fluency, which can be interrupted in different ways in different FTLD subgroups.

Continuity Clinic Practice Feedback Curriculum for Residents: A Model for Ambulatory Education.

BACKGROUND: There is an unmet need for formal curricula to deliver practice feedback training to residents. OBJECTIVE: We developed a curriculum to help residents receive and interpret individual practice feedback data and to engage them in quality improvement efforts. METHODS: We created a framework based on resident attribution, effective metric selection, faculty coaching, peer and site comparisons, and resident-driven goals. The curriculum used electronic health record-generated resident-level data and disease-specific ambulatory didactics to help motivate quality improvement efforts. It was rolled out to 144 internal medicine residents practicing at 1 of 4 primary care clinic sites from July 2016 to June 2017. Resident attitudes and behaviors were tracked with presurveys and postsurveys, completed by 126 (88%) and 85 (59%) residents, respectively. Data log-ins and completion of educational activities were monitored. Group-level performance data were tracked using run charts. RESULTS: Survey results demonstrated significant improvements on a 5-point Likert scale in residents' self-reported ability to receive (from a mean of 2.0 to 3.3, P < .001) and to interpret and understand (mean of 2.4 to 3.2, P < .001) their practice performance data. There was also an increased likelihood they would report that their practice had seen improvements in patient care (13% versus 35%, P < .001). Run charts demonstrated no change in patient outcome metrics. CONCLUSIONS: A learner-centered longitudinal curriculum on ambulatory patient panels can help residents develop competency in receiving, interpreting, and effectively applying individualized practice performance data.

Impairment of script comprehension in Lewy body spectrum disorders.

A disabling impairment of higher-order language function can be seen in patients with Lewy body spectrum disorders such as Parkinson's disease (PD), Parkinson's disease dementia (PDD), and dementia with Lewy bodies (DLB). We focus on script comprehension in patients with Lewy body spectrum disorders. While scripts unfold sequentially, constituent events are thought to contain an internal organization. Executive dysfunction in patients with Lewy body spectrum disorders may interfere with comprehension of this internal structure. We examined 42 patients (30 non-demented PD and 12 mildly demented PDD/DLB patients) and 12 healthy seniors. We presented 22 scripts (e.g., "going fishing"), each consisting of six events. Pilot data from young controls provided the basis for organizing associated events into clusters and arranging them hierarchically into scripts. We measured accuracy and latency to judge the order of adjacent events in the same cluster versus adjacent events in different clusters. PDD/DLB patients were less accurate in their ordering judgments than PD patients and controls. Healthy seniors and PD patients were significantly faster to judge correctly the order of highly associated within-cluster event pairs relative to less closely associated different-cluster event pairs, while PDD/DLB patients did not consistently distinguish between these event-pair types. This relative insensitivity to the clustered-hierarchical organization of events was related to executive impairment and to frontal atrophy as measured by volumetric MRI. These findings extend prior work on script processing to patients with Lewy body spectrum disorders and highlight the potential impact of frontal/executive dysfunction on the daily lives of affected patients.

fMRI evidence for strategic decision-making during resolution of pronoun reference.

Pronouns are extraordinarily common in daily language yet little is known about the neural mechanisms that support decisions about pronoun reference. We propose a large-scale neural network for resolving pronoun reference that consists of two components. First, a core language network in peri-Sylvian cortex supports syntactic and semantic resources for interpreting pronoun meaning in sentences. Second, a frontal-parietal network that supports strategic decision-making is recruited to support probabilistic and risk-related components of resolving a pronoun's referent. In an fMRI study of healthy young adults, we observed activation of left inferior frontal and superior temporal cortex, consistent with a language network. We also observed activation of brain regions not associated with traditional language areas. By manipulating the context of the pronoun, we were able to demonstrate recruitment of dorsolateral prefrontal cortex during probabilistic evaluation of a pronoun's reference, and orbital frontal activation when a pronoun must adopt a risky referent. Together, these findings are consistent with a two-component model for resolving a pronoun's reference that includes neuroanatomic regions supporting core linguistic and decision-making mechanisms.

Impairments of speech fluency in Lewy body spectrum disorder.

Few studies have examined connected speech in demented and non-demented patients with Parkinson's disease (PD). We assessed the speech production of 35 patients with Lewy body spectrum disorder (LBSD), including non-demented PD patients, patients with PD dementia (PDD), and patients with dementia with Lewy bodies (DLB), in a semi-structured narrative speech sample in order to characterize impairments of speech fluency and to determine the factors contributing to reduced speech fluency in these patients. Both demented and non-demented PD patients exhibited reduced speech fluency, characterized by reduced overall speech rate and long pauses between sentences. Reduced speech rate in LBSD correlated with measures of between-utterance pauses, executive functioning, and grammatical comprehension. Regression analyses related non-fluent speech, grammatical difficulty, and executive difficulty to atrophy in frontal brain regions. These findings indicate that multiple factors contribute to slowed speech in LBSD, and this is mediated in part by disease in frontal brain regions.

Preserved musical semantic memory in semantic dementia.

OBJECTIVE: To understand the scope of semantic impairment in semantic dementia. DESIGN: Case study. SETTING: Academic medical center. PATIENT: A man with semantic dementia, as demonstrated by clinical, neuropsychological, and imaging studies. MAIN OUTCOME MEASURES: Music performance and magnetic resonance imaging results. RESULTS: Despite profoundly impaired semantic memory for words and objects due to left temporal lobe atrophy, this semiprofessional musician was creative and expressive in demonstrating preserved musical knowledge. CONCLUSION: Long-term representations of words and objects in semantic memory may be dissociated from meaningful knowledge in other domains, such as music.

Speech errors in progressive non-fluent aphasia.

The nature and frequency of speech production errors in neurodegenerative disease have not previously been precisely quantified. In the present study, 16 patients with a progressive form of non-fluent aphasia (PNFA) were asked to tell a story from a wordless children's picture book. Errors in production were classified as either phonemic, involving language-based deformations that nevertheless result in possible sequences of English speech segments; or phonetic, involving a motor planning deficit and resulting in non-English speech segments. The distribution of cortical atrophy as revealed by structural MRI scans was examined quantitatively in a subset of PNFA patients (N=7). The few errors made by healthy seniors were only phonemic in type. PNFA patients made more than four times as many errors as controls. This included both phonemic and phonetic errors, with a preponderance of errors (82%) classified as phonemic. The majority of phonemic errors were substitutions that shared most distinctive features with the target phoneme. The systematic nature of these substitutions is not consistent with a motor planning deficit. Cortical atrophy was found in prefrontal regions bilaterally and peri-Sylvian regions of the left hemisphere. We conclude that the speech errors produced by PNFA patients are mainly errors at the phonemic level of language processing and are not caused by a motor planning impairment.