Logopenic aphasia (logopenic variant primary progressive aphasia (lvPPA)) is considered to be the third type of primary progressive aphasia (PPA). The other two types of PPA are progressive nonfluent aphasia… Click to show full abstract
Logopenic aphasia (logopenic variant primary progressive aphasia (lvPPA)) is considered to be the third type of primary progressive aphasia (PPA). The other two types of PPA are progressive nonfluent aphasia (nonfluent/agrammatic variant PPA) and semantic dementia (semantic variant PPA (svPPA)). We previously reported on a female patient with taupositive PPA, which was suspected based on F-THK-5351 positron emission tomography (PET); the patient had negative amyloid-β deposition and was diagnosed with lvPPA. The images of C-Pittsburgh compound B PET and F-THK-5351 PET are shown in Figure 1. Eventually, she was diagnosed with frontotemporal lobar degeneration (FTLD). We followed up with this patient after our previous study and report our results here. We obtained written informed consent from the patient and her spouse to publish this report, including clinical and imaging data and cognitive estimations. The present case involves a 64-year-old, righthanded woman. We previously reported her life and medical history, symptoms, and imaging data. Her symptoms fulfilled the criteria for a clinical diagnosis of lvPPA, as described by Gorno-Tempini et al., but speech (phonologic) errors were not observed in her spontaneous speech and naming. The findings of F-fluorodeoxyglucose PET did not fulfil the criteria described by Gorno-Tempini et al. for imagingsupported lvPPA. However, the findings of FTHK-5331 PET fulfilled the criteria for lvPPA with definite pathology, as described by Gorno-Tempini et al. At baseline, we observed surface dyslexia on the Japanese Adult Reading Test in only one Kanji word out of 50 words on the test. Of note, surface dyslexia was not clearly observed in this case in contrast with a previously described patient with svPPA. In addition, impaired single-word comprehension, which was unclear at baseline, had been progressing based on the confrontation naming and single-word comprehension subtest of the Test of Lexical Processing in Aphasia (by the Japan Logopedics and Phoniatrics Association Committee on Speech and Language’s Subcommittee on Aphasia) (File S1). The patient’s motor speech was spared, and frank agrammatism was absent. The number of items in which confrontation naming was impaired had increased considerably, suggesting that the characteristics of lvPPA were getting stronger compared with those of typical svPPA. The patient could not be diagnosed with svPPA based on the criteria. At baseline, the total score of Neuropsychiatric Inventory (NPI), which was performed with the aid of the patient’s husband, was zero. However, her behavioural and psychological symptoms of dementia had worsen over time. Approximately 2 years after baseline, the results of NPI (frequency × severity), which was again performed with the aid of her husband, were depression (1 × 1), apathy (4 × 2), and eating behaviour abnormalities (4 × 2) (her husband claimed that she engaged in binge eating). The scores of all other subtests were zero. According to the patient’s husband, although her executive function in daily life was impaired, she managed to independently go out and come home. These findings fulfilled the criteria of probable behavioural variant of frontotemporal dementia (bvFTD). Approximately 3 years after baseline, her husband claimed that she occasionally displayed aggression towards him although she could hardly speak, often exhibited stereotyped behaviours (e.g. she puts on and takes off her clothes repeatedly), and often experienced urinary incontinence for which a nappy had to be used. At the time, the results of NPI (frequency × severity) were agitation/aggression (4 × 2), apathy (4 × 3), aberrant motor behaviour (3 × 3), and eating behaviour abnormalities (4 × 2). The scores of all other
               
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