Primary Progressive Aphasia has a Unique Signature Distinct from Dementia of the Alzheimer’s Type and Behavioral Variant Frontotemporal Dementia Regardless of Pathology

Abstract Background The Landscape Montage Technique was originally developed by Hisao Nakai, a Japanese psychiatrist, to pursue the possibility and application of a psychotherapeutic approach using drawing for patients with schizophrenia. Drawing was initially adopted to evaluate patients with an impaired ability for verbal expression, particularly for the diagnosis and treatment of patients with schizophrenia. Since its development, the Landscape Montage Technique has been utilized in various clinical settings throughout Japan. This study aimed to evaluate the psychiatric conditions of a patient diagnosed as having primary progressive aphasia using the Landscape Montage Technique at a 3-year follow-up. Case presentation We present the case of a 64-year-old, right-handed Japanese woman initially diagnosed as having logopenic variant primary progressive aphasia or logopenic aphasia. At a 3-year follow-up, logopenic aphasia progressed to behavioral variant frontotemporal dementia or frontotemporal dementia. According to her husband, she began to have speech difficulties approximately 5 years before her first visit. The results of neurocognitive tests suggested mild cognitive impairment or early stages of dementia. Her clinical dementia rating score was 0.5, suggesting a diagnosis of mild cognitive impairment. She had a Raven’s Colored Progressive Matrices score of 31 out of 36, which indicated a nonverbal cognitive ability that was greater than the 90th percentile for her age. The Japanese Standard Language Test of Aphasia, which was performed at two points during the follow-up, indicated the possibility for a diagnosis of primary progressive aphasia given the progression of her aphasia. Based on her clinical symptoms and Japanese Standard Language Test of Aphasia results, a diagnosis of logopenic variant primary progressive aphasia was established. Magnetic resonance imaging revealed severe predominant left frontal and anterior temporal atrophy, as well as bilateral parietal atrophy. Amyloid beta deposition was negative. At the 3-year follow-up, logopenic variant primary progressive aphasia had progressed to behavioral variant frontotemporal dementia. However, the Landscape Montage Technique allowed for the diagnosis of behavioral variant frontotemporal dementia only 2 years after baseline. Conclusions The present study showed that the Landscape Montage Technique can be useful for diagnosing behavioral variant frontotemporal dementia that starts as logopenic variant primary progressive aphasia at earlier stages.

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Automated profiling of spontaneous speech in primary progressive aphasia and behavioral-variant frontotemporal dementia: An approach based on usage-frequency

Cortex ◽

10.1016/j.cortex.2020.08.027 ◽

2020 ◽

Vol 133 ◽

pp. 103-119 ◽

Cited By ~ 1

Author(s):

Vitor C. Zimmerer ◽

Chris J.D. Hardy ◽

James Eastman ◽

Sonali Dutta ◽

Leo Varnet ◽

...

Keyword(s):

Frontotemporal Dementia ◽

Primary Progressive Aphasia ◽

Spontaneous Speech ◽

Behavioral Variant Frontotemporal Dementia ◽

Progressive Aphasia ◽

Primary Progressive ◽

Usage Frequency

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P3-487: THE FRONTOTEMPORAL LOBAR DEGENERATION MODULE (FTLD-MOD) OF THE UNIFORM DATA SET: PERFORMANCE ON LANGUAGE AND BEHAVIOR MEASURES IN PRIMARY PROGRESSIVE APHASIA (PPA) AND BEHAVIORAL VARIANT FRONTOTEMPORAL DEMENTIA (BVFTD)

Alzheimer s & Dementia ◽

10.1016/j.jalz.2018.06.1851 ◽

2006 ◽

Vol 14 (7S_Part_24) ◽

pp. P1308-P1308

Author(s):

Tamar Gefen ◽

Merilee Teylan ◽

Lilah M. Besser ◽

Sandra Weintraub

Keyword(s):

Frontotemporal Dementia ◽

Frontotemporal Lobar Degeneration ◽

Primary Progressive Aphasia ◽

Data Set ◽

Behavioral Variant Frontotemporal Dementia ◽

Progressive Aphasia ◽

Primary Progressive ◽

And Behavior

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Multidimensional Apathy in Behavioral Variant Frontotemporal Dementia, Primary Progressive Aphasia, and Alzheimer Disease

Journal of Geriatric Psychiatry and Neurology ◽

10.1177/0891988720924716 ◽

2020 ◽

pp. 089198872092471

Author(s):

Ratko Radakovic ◽

Shuna Colville ◽

Denise Cranley ◽

John M. Starr ◽

Suvankar Pal ◽

...

Keyword(s):

Alzheimer Disease ◽

Frontotemporal Dementia ◽

Primary Progressive Aphasia ◽

Future Research ◽

Self Awareness ◽

Behavioral Variant Frontotemporal Dementia ◽

Progressive Aphasia ◽

Primary Progressive ◽

Patient Groups ◽

Rating Discrepancy

Apathy is prevalent in dementia, such as behavioral variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), and Alzheimer disease (AD). As a multidimensional construct, it can be assessed and subsumed under a Dimensional Apathy Framework. A consistent apathy profile in bvFTD and PPA has yet to be established. The aim was to explore apathy profiles and awareness in bvFTD, PPA, and AD. A total of 12 patients with bvFTD, 12 patients with PPA, 28 patients with AD, and 20 matched controls, as well as their informants/carers, were recruited. All participants completed the Dimensional Apathy Scale (DAS), assessing executive, emotional, and initiation apathy subtypes, a 1-dimensional apathy measure, depression measure, and functional and cognitive screens. Apathy subtype awareness was determined through DAS informant/carer and self-rating discrepancy. Apathy profile comparison showed patients with bvFTD had significantly higher emotional apathy than patients with AD ( P < .01) and significantly higher apathy over all subtypes than patients with PPA ( Ps < .05). Additionally, patients with bvFTD had significantly lower awareness for emotional apathy ( P < .01) when compared to patients with AD and PPA. All patient groups had significant global apathy over all subtypes compared to controls. The emergent apathy profile for bvFTD seems to be emotional apathy (indifference or emotional/affective neutrality), with lower self-awareness in this subtype. Further, lower self-awareness for executive apathy (lack of motivation for planning, organization, or attention) differentiates bvFTD from PPA. Future research should investigate the cognitive and neural correlates as well as the practical impact of apathy subtypes.

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