Progression of logopenic variant primary progressive aphasia to apraxia and semantic memory deficits

Abstract

BackgroundDue to the nature of neurodegenerative disorders, patients with primary progressive aphasia develop cognitive impairment other than aphasia as the disorder progresses. The progression of logopenic variant primary progressive aphasia (lvPPA), however, has not been well described. In particular, praxic disorders and semantic memory deficits have rarely been reported.Case presentationsWe report three patients in the initial stage of lvPPA who subsequently developed apraxia in the middle stage and developed clinically evident semantic memory deficits in the advanced stages.ConclusionsThe present case series suggests that some patients with lvPPA develop an atypical type of dementia with apraxia and semantic memory deficits, suggesting that these cases should be classified as a type of early-onset Alzheimer’s disease.

Degeneração Lobar Frontotemporal: Polêmicas E Desafios

“…Alterações comportamentais como declínio na conduta social, regulação emocional, embotamento afetivo e perda da capacidade de insight, semelhantes às da apresentação comportamental são encontradas em estágios tardios da APP (FUNAYAMA et al, 2013). Isso reforça a ideia de que as síndromes não são processos estanques, pois no decorrer da evolução do processo degenerativo a atrofia pode se estender a outras áreas antes não afetadas pela doença.…”

Pica after Acquired Brain Injury and in Degenerative Diseases is Associated with Temporal Lobe Dysfunction and its Related Semantic Memory Deficits

“…In previous reports, pica that occurs in degenerative diseases is associated with semantic dementia [9][10][11] and early-onset Alzheimer's disease [13,14]. In our facility, the Cognitive Dysfunction Clinic associated with Ashikaga Red Cross Hospital, Tochigi, Japan, 19 patients with advanced-stage degenerative diseases presented with pica during the period from January 2007 to August 2014.…”

“…Previous case reports of pica in individuals with acquired brain injury and degenerative diseases had a lesion or degeneration in temporal lobe [9][10][11][12][13][14]. From clinical observations, Morris et al [15] suggested that a failure to recognize objects might account for the eating of inedible objects.…”

“…Based on clinical observations, most of the individuals with early-onset Alzheimer's disease did not show episodic memory impairment at the onset of the disease, the epitome of typical Alzheimer's disease. Instead, they often showed progressive aphasia, such as logopenic variant primary progressive aphasia [13] and progressive transcortical sensory aphasia [14], as an initial symptom. These cases suggested that dysfunction at the left temporoparietal cortices for logopenic variant primary progressive aphasia [13] or the left posterior temporal cortex for progressive transcortical sensory aphasia [14] might contribute to the development of pica.…”

“…Instead, they often showed progressive aphasia, such as logopenic variant primary progressive aphasia [13] and progressive transcortical sensory aphasia [14], as an initial symptom. These cases suggested that dysfunction at the left temporoparietal cortices for logopenic variant primary progressive aphasia [13] or the left posterior temporal cortex for progressive transcortical sensory aphasia [14] might contribute to the development of pica. Hypofunction within these areas is compatible with our findings on the neural basis for pica after acquired brain injury [15], even if slightly different areas are involved.…”