This paper reports a case study of acquired surface alexia in Spanish and discusses the most suitable tests to detect this syndrome in a writing system that is very regular for reading at the segmental and supra-segmental levels. Patient MM has surface alexia characterized by quantitatively good performance in reading words and pseudowords; accurate but slow and syllabic reading of words, nonwords and sentences; good performance in lexical decision tasks including words and nonwords; errors in lexical decision with pseudohomophones; and homophone confusions. This pattern of reading can be interpreted as a disorder in the lexical reading route and overdependence on the non-lexical route. We discuss nonlexical impairments and the interpretation of alexia and suggest tasks to identify surface alexia in a shallow orthography.
Background: A cross-culturally valid nonverbal assessment of semantic knowledge is needed. Accurately identifying impairment of object semantics is important for diagnosis of several disorders, including distinguishing semantic variant primary progressive aphasia (svPPA), a neurodegenerative condition characterised by progressive impairment in word comprehension, from logopenic and nonfluent agrammatic variants, which are not associated with impaired object semantics. However, current assessments require culturally specific knowledge. Aims: We developed a cross-culturally valid short form of the Pyramids and Palm Trees Test to assess object semantic memory. We investigated its clinical utility in differentiating the semantic variant of primary progressive aphasia, from the logopenic and nonfluent agrammatic variants. Areas of atrophy associated with poor performance were identified. Methods & Procedures: Fourteen items that rely on knowledge of objects' defining features were selected from the original 52-item version. The full and short forms were administered to healthy individuals in the US (N = 18), Argentina (N = 20), and Greece (N = 12) and performance was compared. Seventy-eight individuals with primary progressive aphasia in the US completed the short form. Behavioural performance of the svPPA group (N = 24) was compared to other variants. Atlas-based analysis identified regions where atrophy correlated with poor performance in 39 individuals with primary progressive aphasia who had high-resolution magnetic resonance imaging (MRI) scans. Outcomes & Results: Control performance was classified as normal on the short form significantly more often than on the full version. Across groups with primary progressive aphasia, the group with semantic variant performed significantly worse than the groups with logopenic or nonfluent agrammatic variants. Volume in left anterior and inferior temporal cortex correlated with performance. Conclusions: The short-form Pyramids and Palm Trees Test is a clinically relevant, cross-culturally valid assessment of nonverbal object semantics. It can be used to identify semantic impairments, with poor performance associated with atrophy of the temporal lobes.
Processing of nouns and action verbs can be differentially compromised following lesions to posterior and anterior/motor brain regions, respectively. However, little is known about how these deficits progress in the course of neurodegeneration. To address this issue, we assessed productive lexical skills in a patient with posterior cortical atrophy (PCA) at two different stages of his pathology. On both occasions, he underwent a structural brain imaging protocol and completed semantic fluency tasks requiring retrieval of animals (nouns) and actions (verbs). Imaging results were compared with those of controls via voxel-based morphometry (VBM), whereas fluency performance was compared to age-matched norms through Crawford’s t-tests. In the first assessment, the patient exhibited atrophy of more posterior regions supporting multimodal semantics (medial temporal and lingual gyri), together with a selective deficit in noun fluency. Then, by the second assessment, the patient’s atrophy had progressed mainly toward fronto-motor regions (rolandic operculum, inferior and superior frontal gyri) and subcortical motor hubs (cerebellum, thalamus), and his fluency impairments had extended to action verbs. These results offer unprecedented evidence of the specificity of the pathways related to noun and action-verb impairments in the course of neurodegeneration, highlighting the latter’s critical dependence on damage to regions supporting motor functions, as opposed to multimodal semantic processes.
Above and beyond the critical contributions of left perisylvian regions to language, the neural networks supporting pragmatic aspects of verbal communication in native and non-native languages (L1s and L2, respectively) have often been ascribed to the right hemisphere (RH). However, several reports have shown that left-hemisphere activity associated with pragmatic domains (e.g., prosody, indirect speech, figurative language) is comparable to or even greater than that observed in the RH, challenging the proposed putative role of the latter for relevant domains. Against this background, we report on an adult bilingual patient showing preservation of pragmatic verbal skills in both languages (L1: Spanish, L2: English) despite bilateral damage mainly focused on the RH. After two strokes, the patient sustained lesions in several regions previously implicated in pragmatic functions (vast portions of the right fronto-insulo-temporal cortices, the bilateral amygdalae and insular cortices, and the left putamen). Yet, comparison of linguistic and pragmatic skills with matched controls revealed spared performance on multiple relevant tasks in both her L1 and L2. Despite mild difficulties in some aspects of L2 prosody, she showed no deficits in comprehending metaphors and idioms, or understanding indirect speech acts in either language. Basic verbal skills were also preserved in both languages, including verbal auditory discrimination, repetition of words and pseudo-words, cognate processing, grammaticality judgments, equivalent recognition, and word and sentence translation. Taken together, the evidence shows that multiple functions of verbal communication can be widely spared despite extensive damage to the RH, and that claims for a putative relation between pragmatics and the RH may have been overemphasized in the monolingual and bilingual literature. We further discuss the case in light of previous reports of pragmatic and linguistic deficits following brain lesions and address its relation to cognitive compensation in bilingual patients.
Posterior cortical atrophy (PCA) is a rare neurodegenerative syndrome characterized by initial predominant visuoperceptual deficits followed by a progressive decline in other cognitive functions. This syndrome has not been as thoroughly described as other dementias, particularly from a neuropsychological evolution perspective with only a few studies describing the evolution of its cognitive progression. In this investigation we review the literature on this rare condition and we perform a 7-year neuropsychological and neuroradiological follow-up of a 64-year-old man with PCA. The subject's deficits initially appeared in his visuoperceptual skills with later affectation appearing in language and other cognitive functions, this being coherent with the patient's parieto-temporal atrophy evolution.
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