A 'Mini Linguistic State Examination' to classify primary progressive aphasia.

There are few available methods for qualitatively evaluating patients with primary progressive aphasia. Commonly adopted approaches are time-consuming, of limited accuracy or designed to assess different patient populations. This paper introduces a new clinical test-the Mini Linguistic State Examination-which was designed uniquely to enable a clinician to assess and subclassify both classical and mixed presentations of primary progressive aphasia. The adoption of a novel assessment method (error classification) greatly amplifies the clinical information that can be derived from a set of standard linguistic tasks and allows a five-dimensional profile to be defined. Fifty-four patients and 30 matched controls were recruited. Five domains of language competence (motor speech, phonology, semantics, syntax and working memory) were assessed using a sequence of 11 distinct linguistic assays. A random forest classification was used to assess the diagnostic accuracy for predicting primary progressive aphasia subtypes and create a decision tree as a guide to clinical classification. The random forest prediction model was 96% accurate overall (92% for the logopenic variant, 93% for the semantic variant and 98% for the non-fluent variant). The derived decision tree produced a correct classification of 91% of participants whose data were not included in the training set. The Mini Linguistic State Examination is a new cognitive test incorporating a novel and powerful, yet straightforward, approach to scoring. Rigorous assessment of its diagnostic accuracy confirmed excellent matching of primary progressive aphasia syndromes to clinical gold standard diagnoses. Adoption of the Mini Linguistic State Examination by clinicians will have a decisive impact on the consistency and uniformity with which patients can be described clinically. It will also facilitate screening for cohort-based research, including future therapeutic trials, and is suitable for describing, quantifying and monitoring language deficits in other brain disorders.

Do You Read How I Read? Systematic Individual Differences in Semantic Reliance amongst Normal Readers.

The extent to which meaning is involved in reading aloud has proven an area of longstanding debate, and current computational models differ on this dimension. The connectionist triangle model proposes that normal individuals rely on semantic information for correct reading of words with atypical spelling-sound relationships, but to varying degrees. This proposed individual difference would account for the varying stage of decline at which patients with semantic dementia first show the reading impairment known as surface dyslexia. Recent neuroimaging data has provided validation of this view, showing that individual differences in degree of semantic reliance during exception word reading predict the amount of activation in left anterior temporal regions associated with semantic processing. This study aimed to establish the cognitive correlates of individual differences in semantic reliance during exception word reading. Experiment 1 used a subgrouping approach with 32 participants and found larger imageability and semantic priming effects specifically for exception word reading amongst high relative to low semantic reliance readers. High semantic reliance readers also tended to read nonwords more slowly than low semantic reliance readers. A second experiment used a regression approach with 129 readers and confirmed the relationship of degree of semantic reliance both to imageability effects in exception word reading and speed of nonword reading. Further, while the performance of the higher semantic readers revealed no significant association with semantic processing tasks, there was a negative relationship with rhyme processing tasks. We therefore speculate that differences in phonological abilities may be responsible for varying degrees of semantic reliance in reading aloud. This proposal accords with the results of functional imaging showing that higher semantic reliance during exception word reading corresponds to lower activation in left pre-central gyrus, an area associated with direct spelling sound mapping and phonological processing. Our results therefore establish the nature of systematic individual differences in degree of semantic involvement amongst normal readers, and suggest directions for future neuroimaging and computational modeling research to uncover their origins.

Neuropsychological Thoughts, Then and Now: A Tribute to Oscar Marin.

This brief paper, inspired by an invitation to acknowledge and celebrate Oscar Marin's great contributions to cognitive neurology and neuropsychology, reviews the case of a patient, T.P., who had significant deficits of naming, reading, and spelling. I first studied and reported this patient 35 years ago, in 1979, when I was significantly influenced by the work of Oscar Marin and his colleagues. I have recently had the unusual opportunity to do some brief reassessment of T.P.'s current (2015) cognitive abilities, and to reassess the interpretations that I had given to her pattern of impairment in the initial studies. I suggest that advances over the last decade or so-in theorizing about, and connectionist modeling of, reading and spelling disorders-enable a more coherent account of T.P.'s acquired anomia, dyslexia, and dysgraphia, and the relationships among them.

What lies beneath: a comparison of reading aloud in pure alexia and semantic dementia.

Exaggerated effects of word length upon reading-aloud performance define pure alexia, but have also been observed in semantic dementia. Some researchers have proposed a reading-specific account, whereby performance in these two disorders reflects the same cause: impaired orthographic processing. In contrast, according to the primary systems view of acquired reading disorders, pure alexia results from a basic visual processing deficit, whereas degraded semantic knowledge undermines reading performance in semantic dementia. To explore the source of reading deficits in these two disorders, we compared the reading performance of 10 pure alexic and 10 semantic dementia patients, matched in terms of overall severity of reading deficit. The results revealed comparable frequency effects on reading accuracy, but weaker effects of regularity in pure alexia than in semantic dementia. Analysis of error types revealed a higher rate of letter-based errors and a lower rate of regularization responses in pure alexia than in semantic dementia. Error responses were most often words in pure alexia but most often nonwords in semantic dementia. Although all patients made some letter substitution errors, these were characterized by visual similarity in pure alexia and phonological similarity in semantic dementia. Overall, the data indicate that the reading deficits in pure alexia and semantic dementia arise from impairments of visual processing and knowledge of word meaning, respectively. The locus and mechanisms of these impairments are placed within the context of current connectionist models of reading.