Benson’s Disease or Posterior Cortical Atrophy, Revisited

Background: D. Frank Benson and colleagues first described the clinical and neuropathological features of posterior cortical atrophy (PCA) from patients in the UCLA Neurobehavior Program. Objective: We reviewed the Program’s subsequent clinical experience with PCA, and its potential for clarifying this relatively rare syndrome in comparison to the accumulated literature on PCA. Methods: Using the original criteria derived from this clinic, 65 patients with neuroimaging-supported PCA were diagnosed between 1995 and 2020. Results: On presentation, most have visual localization complaints and related visuospatial symptoms, but nearly half had memory complaints followed by symptoms of depression. Neurobehavioral testing showed predominant difficulty with visuospatial constructions, Gerstmann’s syndrome, and Balint’s syndrome, but also impaired memory and naming. On retrospective application of the current Consensus Criteria for PCA, 59 (91%) met PCA criteria with a modification allowing for “significantly greater visuospatial over memory and naming deficits.” There were 37 deaths (56.9%) with the median overall survival of 10.3 years (95% CI: 9.6–13.6 years), consistent with a slow neurodegenerative disorder in most patients. Conclusion: Together, these findings recommend modifying the PCA criteria for “relatively spared” memory, language, and behavior to include secondary memory and naming difficulty and depression, with increased emphasis on the presence of Gerstmann’s and Balint’s syndromes.

The Use of Written Naming and Repetition to Treat Naming Deficits in Aphasia

Purpose The purpose of the study was to examine the use of a combined written naming + repetition (WN + REP) approach and an REP-only approach to treat naming deficits in a group of individuals with aphasia. Method Six individuals participated in the study. A multiple-baseline design across behaviors was used. A WN + REP approach, an REP approach, and an untreated condition were presented. Three 10-item treatment lists and one 10-item untreated list were created. Each 10-item treatment list was evenly divided so that 5 items were treated using the WN + REP approach and 5 items were treated using the REP approach. Results Visual analyses of treatment data revealed improvements in 4 of the 6 participants. Meaningful treatment effect sizes were obtained for 5 of the 6 participants in at least 1 of the approaches across the treatment lists. Slightly more participants obtained meaningful effect sizes in the WN + REP approach than in the REP approach. Use of writing as a strategy to help with naming the pictures verbally increased over the duration of treatment in 5 of the 6 participants. Conclusions The findings revealed that both approaches improved spoken naming accuracy in aphasia. Although the WN + REP approach resulted in more meaningful effect sizes than the REP approach, there was no overwhelming advantage in using 1 or the other approach. However, the WN + REP approach promoted writing as a functional strategy that could be used when naming the picture verbally was not successful.

A new test for action verb naming: Normative data from 290 Italian adults

Verbs and nouns can be selectively impaired, suggesting that they are processed, at least in part, by distinct neural structures. While several tests of object naming are available, tasks involving action verb naming with normative data are lacking. We report the construction and standardization of a new test for the assessment of picture naming of actions. The test includes 50 stimuli, rigidly controlled for several confounding variables. Normative data on 290 Italian subjects pooled across homogenous subgroups for age, sex and education are reported. Multiple regression analyses revealed that age and education significantly correlated with the subject’s score. In particular, increasing age negatively affected performance, while the performance increased with a higher education. In the clinical practice, the availability of equivalent scores will help the comparison with performance in the picture naming of objects. This test allows investigating action naming deficits in aphasic patients, in Parkinson’s disease patients and in further neurodegenerative disorders, in which a specific impairment of action verbs is expected, filling a gap in the clinical neuropsychological assessment. [The material for the test is available from the authors on request.]

The Use of One or Three Semantic Associative Primes in Treating Anomia in Aphasia

Purpose The aims of the study were to investigate the use of semantic associative relationships as primes in treating naming deficits, or anomia, in aphasia and to determine if differential treatment effects would be found if 1 or 3 primes were presented. Method Ten individuals with varying degrees of anomia participated in the study. A single-subject, A-B treatment design with a cross-over component was used. A 1-PRIME condition (use of 1 prime) was compared with a 3-PRIMES condition (use of 3 primes) for each participant. Results Visual analyses of treatment data revealed improvements in 8 of 10 participants. Meaningful effect sizes were obtained in at least 1 of the conditions for 6 of the 10 participants. Slightly more participants demonstrated meaningful effect sizes in the 3-PRIMES condition than in the 1-PRIME condition. Correlation analyses revealed a positive correlation between the number of teaching episodes and 3-PRIMES probe performance. Conclusions The results support a protocol that uses semantic associative primes to increase naming accuracy in aphasia. The 3-PRIMES condition was slightly more beneficial than the 1-PRIME condition in terms of improving naming abilities in these participants, but there was no overwhelming advantage in using one or the other condition.

Transient aphasias after left hemisphere resective surgery

OBJECT Transient aphasias are often observed in the first few days after a patient has undergone resection in the language-dominant hemisphere. The aims of this prospective study were to characterize the incidence and nature of these aphasias and to determine whether there are relationships between location of the surgical site and deficits in specific language domains. METHODS One hundred ten patients undergoing resection to the language-dominant hemisphere participated in the study. Language was evaluated prior to surgery and 2–3 days and 1 month postsurgery using the Western Aphasia Battery and the Boston Naming Test. Voxel-based lesion-symptom mapping was used to identify relationships between the surgical site location assessed on MRI and deficits in fluency, information content, comprehension, repetition, and naming. RESULTS Seventy-one percent of patients were classified as aphasic based on the Western Aphasia Battery 2–3 days postsurgery, with deficits observed in each of the language domains examined. Fluency deficits were associated with resection of the precentral gyrus and adjacent inferior frontal cortex. Reduced information content of spoken output was associated with resection of the ventral precentral gyrus and posterior inferior frontal gyrus (pars opercularis). Repetition deficits were associated with resection of the posterior superior temporal gyrus. Naming deficits were associated with resection of the ventral temporal cortex, with midtemporal and posterior temporal damage more predictive of naming deficits than anterior temporal damage. By 1 month postsurgery, nearly all language deficits were resolved, and no language measure except for naming differed significantly from its presurgical level. CONCLUSIONS These findings show that transient aphasias are very common after left hemisphere resective surgery and that the precise nature of the aphasia depends on the specific location of the surgical site. The patient cohort in this study provides a unique window into the neural basis of language because resections are discrete, their locations are not limited by vascular distribution or patterns of neurodegeneration, and language can be studied prior to substantial reorganization.