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Primary progressive aphasia (PPA) is a neurodegenerative disease characterised by a progressive decline in language and speech as the first clinical manifestation, which mostly spares other cognitive functions. However, the linguistic impairment of PPA shows different features than that resulting from cerebrovascular diseases. The major difference between the linguistic manifestations of PPA and the traditional classification of aphasias has led to the development of new, more specific methods of language assessment. Among the currently available tools, there has been great interest in the Progressive Aphasia Severity Scale (PASS). This quick and easy-to-use clinical tool allows to collect significant information from caregivers about the communicative, linguistic, and functional difficulties of patients affected by PPA. In addition to monitoring the severity and progression of deficits in 13 different language domains, this scale integrates the classic "clinically reported" assessment with a "caregiver-reported" analysis of the daily experience of the patient, which provides a better understanding of how the disease affects the quality of life of both the patient and the caregiver. In the present contribution, the PASS was translated and adapted into Italian according to the international guidelines for the cross-cultural adaptation of self-report measures. This version of the PASS can help clinicians and researchers in the diagnosis of PPA in Italian clinical populations. Furthermore, it could be particularly useful for the long-term evaluation of the disease, in order to monitor its evolution, and might represent an optimal means to verify the efficacy of speech/language therapy in delaying the progression of the disease., (© 2021. Fondazione Società Italiana di Neurologia.)

An important issue in research on language is how concepts are represented and associated with each other in the brain. Many investigations have focused on language ambiguity and the phenomenon of homonymy in which a single lexical item, presenting the same form, is related to different meanings. Our study aims to test the hypothesis that weak association of meaning characterizing homonyms may be especially prone to brain damage. To verify this hypothesis a test of attribution of the meaning of homonymous words, the Humpty Dumpty (HD) test, was applied to 50 patients with Alzheimer's disease (AD) and 50 healthy subjects. Results show that AD patients are impaired in the HD test in an early phase of disease and that performance correlates with naming ability and phonological fluency. The data are in keeping with a growing body of literature that supports dual impairment to the semantic system in AD, i.e., to semantic knowledge and active processing and access to the semantic field. The evaluation of the ability to resolve homonymous ambiguity, using the HD test, may provide a useful and quick clinical tool to detect the anomalies of the semantic network linked to either a loss of the core system where meaning of words is stored or an impairment of the access to an intact semantic representation.

Primary progressive aphasia (PPA) is characterized by an isolated progressive impairment of word use and comprehension reflecting the distribution of pathological processes within the left hemisphere. We used proton magnetic resonance spectroscopy (1H-MRS) to study in vivo the integrity of axonal fibers connecting perisylvian language areas in 11 patients with PPA, 11 subjects with Alzheimer's disease, and 22 controls. Brain metabolites (N-acetylaspartate, myoinositol, choline, creatine) were measured bilaterally within a volume of interest located in the central portion of the superior longitudinal fasciculus, a long associative bundle connecting Broca's area with Wernicke's area, and other language regions of the temporal lobe. In the PPA group, there was an asymmetrical N-acetylaspartate to creatine ratio reduction compared with Alzheimer's disease and controls, with greater changes on the left side. The myoinositol to creatine ratio was increased in the PPA group bilaterally compared with controls. The choline to creatine ratio did not differ among the three groups. These results indicate an asymmetrical focal axonal injury within the language network in PPA. The marked difference in the distribution of N-acetylaspartate to creatine between PPA and Alzheimer's disease suggests that proton magnetic resonance spectroscopy may help to differentiate between these two conditions.