Semantic dementia (SD) implicates the anterior temporal lobes (ATL) as a critical substrate for semantic memory. Multi-modal semantic impairment can also be a feature of post-stroke aphasia (referred to here as &#x201C;semantic aphasia&#x201D; or SA) where patients show impaired regulatory control accompanied by lesions to the frontal and/or temporo-parietal cortices, and thus the two patient groups demonstrate qualitatively different patterns of semantic impairment [1]. Previous comparisons of these two patient groups have tended to focus on verbal receptive tasks. Accordingly, this study investigated nonverbal receptive abilities via a comparison of reality decision judgements in SD and SA. Pictures of objects were presented alongside non-real distracters whose features were altered to make them more/less plausible for the semantic category. The results highlighted a number of critical differences between the two groups. Compared to SD patients, SA patients: (1) were relatively unimpaired on the two alternative forced choice (2AFC) decisions despite showing a comparable degree of semantic impairment on other assessments; (2) showed minimal effects of the plausibility manipulation; (3) were strongly influenced by variations in the regulatory requirements of tasks; and (4) exhibited a reversed effect of familiarity&#x2013;i.e., better performance on less commonly encountered items. These results support a distinction between semantic impairments which arise from impaired regulatory processes (e.g., SA) versus those where degraded semantic knowledge is the causal factor (e.g., SD). SA patients performed relatively well because the task structure reduced the requirement for internally generated control. In contrast, SD patients performed poorly because their degraded knowledge did not allow the fine-grained distinctions required to complete the task.

Krist A. Noonan

Peter Garrard

Elizabeth Jefferies

Sheeba Eshan

Matthew A. Lambon Ralph

Demonstrating the Qualitative Differences between Semantic Aphasia and Semantic Dementia: A Novel Exploration of Nonverbal Semantic Processing

Objectives: To investigate cognitive components and mechanisms of learning and memory in primary progressive aphasia (PPA) using a simple clinical measure, the Three Words Three Shapes Test (3W3S).Background: PPA patients can complain of memory loss and may perform poorly in standard tests of memory. The extent to which these signs and symptoms reflect dysfunction of the left hemisphere language versus limbic memory network remains unknown.Methods: 3W3S data from 26 patients with a clinical diagnosis of PPA were compared with previously published data from patients with typical dementia of the Alzheimer type (DAT) and cognitively healthy elders.Results: PPA patients showed two bottlenecks in new learning. First, they were impaired in the effortless (but not effortful) on-line encoding of verbal (but not non-verbal) items. Second, they were impaired in the retrieval (but not retention) of verbal (but not non-verbal) items. In contrast, DAT patients had impairments also in effortful on-line encoding and retention of verbal and nonverbal items.Conclusions: PPA selectively interferes with spontaneous on-line encoding and subsequent retrieval of verbal information. This combination may underlie poor memory test performance and is likely to reflect the dysfunction of the left hemisphere language rather than medial temporal memory network.

Sandra Weintraub

Emily Rogalski

Emily Shaw

Sabrina Sawlani

Alfred Rademaker

Christina Wieneke

M.-Marsel Mesulam

Verbal and Nonverbal Memory in Primary Progressive Aphasia: The Three Words-Three Shapes Test

Primary progressive aphasia (PPA) corresponds to the gradual degeneration of language which can occur as nonfluent/agrammatic PPA, semantic variant PPA or logopenic variant PPA. We describe the clinical evolution of a patient with PPA presenting jargon aphasia as a late feature. At the onset of the disease (ten years ago) the patient showed anomia and executive deficits, followed later on by phonemic paraphasias and neologisms, deficits in verbal short-term memory, naming, verbal and semantic fluency. At recent follow-up the patient developed an unintelligible jargon with both semantic and neologistic errors, as well as with severe deficit of comprehension which precluded any further neuropsychological assessment. Compared to healthy controls, FDG-PET showed a hypometabolism in the left angular and middle temporal gyri, precuneus, caudate, posterior cingulate, middle frontal gyrus, and bilaterally in the superior temporal and inferior frontal gyri. The clinical and neuroimaging profile seems to support the hypothesis that the patient developed a late feature of logopenic variant PPA characterized by jargonaphasia and associated with superior temporal and parietal dysfunction.

Paolo Caffarra

Simona Gardini

Stefano Cappa

Francesca Dieci

Letizia Concari

Federica Barocco

Caterina Ghetti

Livia Ruffini

Guido Dalla Rosa Prati

Degenerative Jargon Aphasia: Unusual Progression of Logopenic/Phonological Progressive Aphasia?

When assessing for the presence of hemianesthesia, the examiner touches the body of the patients, and requests that they report verbally the location of the delivered tactile stimulus. Contralesional omissions of single tactile stimuli, however, might be due to either primary somatosensory deficits or to spatial attention impairment (i.e., neglect). In this preliminary study, we tested whether clinical assessment can be improved to differentiate between these two types of deficit by modifying the assessment procedure. K.L., a patient with left unilateral neglect, was asked to detect tactile stimuli delivered in two conditions: spatial attention distributed either to his left or to his right hand, and spatial attention focused only on his left hand. Note that K.L. did not receive double simultaneous tactile stimuli. In the distributed spatial attention condition, K.L. omitted most of the single tactile stimuli delivered to his left hand. In the focused attention condition, K.L. was asked to focus his spatial attention only on his left hand. Under this latter condition, his performance increased dramatically, suggesting that his omissions were not due to hemianesthesia, but rather reflected left tactile neglect. In line with the neuropsychological findings, voxel based analysis of his grey and white matter damage confirmed significant loss in areas associated with left-sided neglect, but sparing of the primary somatosensory cortex. This result suggests that standard somatosensory assessment and differential diagnosis between hemianesthesia and tactile neglect may be more accurate when neuropsychology-based procedures are incorporated in the standard neurological examination.

Marco Pitteri

Annalena Venneri

Francesca Meneghello

Konstantinos Priftis

How to Differentiate Hemianesthesia from Left Tactile Neglect: A Preliminary Case Report

Objective: To investigate impaired shifting of visuospatial attention in Alzheimer&#x2019;s disease (AD) compared with age-matched controls.Method: An attention shifting was examined in 20 AD patients and 10 age-matched normal subjects by choice reaction time (CRT) and covert orienting paradigm. Visuospatial functions tests were also performed. For covert orienting, a peripheral spatial cue method was used, with stimulus-onset (SOA) between the cue and the target time varying from 250 to 2100 ms.Results: The CRT showed no difference between the AD and normal groups. However, the RTs costs plus benefits were greater in the AD than normal group for two SOA conditions independent of dementia severity. Individual profiles in the time course of cue validity revealed two AD subgroups, i.e., a normal pattern for the cue validity of time course, and an abnormal, &#x2018;extinction-like&#x2019; pattern. The latter had a particular difficulty in performing visual construction and spatial attention.Conclusions: Focusing attention was relatively intact in AD. However, shifting of visuospatial attention was impaired in AD compared with normal controls. There was a subgroup whose deficits were not only in &#x2018;disengagement,&#x2019; but their voluntary shifting of attention was affected. These subgroups may show clinically severe visuospatial symptoms in more advanced stage.

Junichi Ishizaki

Kenichi Meguro

Nobuhiro Nara

Mari Kasai

Atsushi Yamadori

Impaired Shifting of Visuospatial Attention in Alzheimer&#x2019;s disease as Shown by the Covert Orienting Paradigm: Implications for Visual Construction Disability

Spelling&#x2013;a core language skill&#x2013;is commonly affected in neurological diseases such as stroke and Primary Progressive Aphasia (PPA). We present two case studies of the same spelling therapy (learning of phoneme-to-grapheme correspondences with help from key words) in two participants: one who had a stroke and one with PPA (logopenic variant). Our study highlights similarities and differences in the time course of each indivdual's therapy. The study evaluates the effectiveness and generalization of treatment in each case, i.e. whether the treatment affected the trained items and/or untrained items, and whether or not the treatment gains were maintained after the end of therapy. Both participants were able to learn associations between phonemes and graphemes as well as between phonemes and words. Reliable generalization to untrained words was shown only for the participant with post-stroke aphasia, but we were not able to test generalization to untrained words in the individual with PPA. The same spelling therapy followed a different time course in each case. The participant with post-stroke aphasia showed a lasting effect of improved spelling, but we were unable to assess maintenance of improvement in the participant with PPA. We discuss these differences in light of the underlying nature of each disease.

Kyrana Tsapkini

Argye E. Hillis

Spelling Intervention in Post-Stroke Aphasia and Primary Progressive Aphasia

The role of biomarkers in predicting pathological findings in the frontotemporal dementia (FTD) clinical spectrum disorders is still being explored. We present comprehensive, prospective longitudinal data for a 66 year old, right-handed female who met current criteria for the nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA). She first presented with a 3-year history of progressive speech and language impairment mainly characterized by severe apraxia of speech. Neuropsychological and general motor functions remained relatively spared throughout the clinical course. Voxel-based morphometry (VBM) showed selective cortical atrophy of the left posterior inferior frontal gyrus (IFG) and underlying insula that worsened over time, extending along the left premotor strip. Five years after her first evaluation, she developed mild memory impairment and underwent PET-FDG and PiB scans that showed left frontal hypometabolism and cortical amyloidosis. Three years later (11 years from first symptom), post-mortem histopathological evaluation revealed Pick's disease, with severe degeneration of left IFG, mid-insula, and precentral gyrus. Alzheimer&#x2019;s disease (AD) (CERAD frequent/Braak Stage V) was also detected. This patient demonstrates that biomarkers indicating brain amyloidosis should not be considered conclusive evidence that AD pathology accounts for a typical FTD clinical/anatomical syndrome.

Francesca Caso

Benno Gesierich

Maya Henry

Manu Sidhu

Amanda LaMarre

Miranda Babiak

Bruce L. Miller

Gil D. Rabinovici

Eric J. Huang

Giuseppe Magnani

Massimo Filippi

Giancarlo Comi

William W. Seeley

Maria Luisa Gorno-Tempini

Nonfluent/Agrammatic PPA with In-Vivo Cortical Amyloidosis and Pick&#x2019;s Disease Pathology

New Insights from a Not-So-Neglected Field: Hemispatial Neglect

Perseverative behavior, manifest as re-cancelling or re-visiting targets, is distinct from spatial neglect. Perseveration is thought to reflect frontal or parietal lobe dysfunction, but the neuroanatomical correlates remain poorly defined and the interplay between neglect and perseveration is incompletely understood. We enrolled 87 consecutive patients with diffusion-weighted, perfusion-weighted imaging, and spatial neglect testing within 24 hours of right hemisphere ischemic stroke. The degrees of spatial neglect and perseveration were analyzed. Perseveration was apparent in 46&#x25; (40/87) of the patients; 28&#x25; (24/87) showed perseveration only; 18&#x25; (16/87) showed both perseveration and neglect; and 3&#x25; (3/87) showed neglect only. Perseverative behaviors occur in an inverted &#x201C;U&#x201D; shape: little neglect was associated with few perseverations; moderate neglect with high perseverations; and in severe neglect targets may not enter consciousness and perseverative responses decrease. Brodmann areas of dysfunction, and the caudate and putament, were assessed and volumetrically measured. In this study, the caudate and putamen were not associated with perseveration. After controlling for neglect, and volume of dysfunctional tissue, only Brodmann area 46 was associated with perseveration. Our results further support the notion that perseveration and neglect are distinct entities; while they often co-occur, acute dorsolateral prefrontal cortex ischemia is associated with perseveration specifically.

Jonathan T. Kleinman

Jeffery C. DuBois

Melissa Newhart

Disentangling the Neuroanatomical Correlates of Perseveration from Unilateral Spatial Neglect

We describe the clinical manifestations and the lesion patterns of five patients with somatoparaphrenia, the denial of ownership for a paralyzed limb, who showed the rare dissociation from anosognosia for hemiplegia. Similar cases have been only occasionally cited in the literature with scanty descriptions of their symptoms and no detailed anatomical assessment. All patients had extrapersonal and at least mild personal neglect. The lesions pattern was mainly subcortical, with a significant involvement of the right thalamus, the basal ganglia and the internal capsule. A formal comparison between the anatomical pattern previously associated with anosognosia in a study performed in 2005 by Berti and colleagues, and the lesion distribution of each patient clearly shows that our pure somatoparaphrenic patients had a sparing of most of the regions associated with anosognosia for hemiplegia. The behavioral dissociation between SP and anosognosia for hemiplegia, together with this new anatomical evidence, suggests that motor awareness is not sufficient to build up a sense of ownership and therefore these two cognitive abilities are at least in part functionally independent and qualitatively different.

Paola Invernizzi

Martina Gandola

Daniele Romano

Laura Zapparoli

Gabriella Bottini

Eraldo Paulesu

What is Mine? Behavioral and Anatomical Dissociations between Somatoparaphrenia and Anosognosia for Hemiplegia

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