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Print version ISSN 0004-282X
Arq. Neuro-Psiquiatr. vol.70 no.7 São Paulo July 2012
A patient with primary progressive aphasia developing dementia due to Alzheimer's disease
Uma paciente com afasia progressiva primária devido à doença de Alzheimer
Thais Stvan Vaz; Paulo Henrique Ferreira Bertolucci; Fabricio Ferreira de Oliveira
Department of Neurology and Neurosurgery, Escola Paulista de Medicina, Federal University of São Paulo (UNIFESP), São Paulo SP, Brazil
Patients with primary progressive aphasia (PPA) often evolve to frontotemporal lobar degeneration (FTLD) syndromes1, but the logopenic form of PPA has been found to have Alzheimer's disease (AD) pathology and in vivo biomarkers suggestive of AD2. We have reported the case of a female right-handed patient with four years of schooling, who first sought assistance at the age of 63 years-old for impairments in her speaking fluency. Her daughter mentioned that two years earlier the patient began to mumble, struggling to complete sentences, often saying things out of context, with clear perception that she meant to say something else. Semantic defects were often present, when asking for a spoon and pointing to a fork, or changing the names of her children though she knew who they were. In the previous six months, the patient progressively lost the ability to read, currently writing with difficulty. Lately, she began to show difficulties locating herself in space. Most household tasks were effortlessly performed, but she could no longer answer the phone because she could not communicate or take notes, and neither shop, since her calculating skills had been lost.
Neurological examination was largely normal. The neuropsychiatric testing displayed the following results: a 12-item neuropsychiatric inventory score of 17 (mostly for anxiety and sleep disorders), with a score of 8 for caregiver distress; Clinical Dementia Rating of 1.0; score of 9 on the Mini-Mental State Examination (orientation to time 2/5; orientation to space 3/5; immediate memory 1/3; calculations 0/5; recent memory 0/3; language 3/9); score of 1 on the 15-point Clock Drawing Test; she was able to complete one item of the Rey-Osterrieth Complex Figure Test, but was not able to recall it; score of 3 on the Boston Naming Test (15-item short version); abstract thinking was seriously impaired, while the patient was not able to read simple sentences, and scored 2 for animals and 4 for fruits in one minute for verbal fluency tests. She was independent for activities of daily living (ADL), whereas partially independent for instrumental ADL. Magnetic resonance (MR) studies of the brain showed a pattern compatible with both PPA and AD (Fig 1 and 2).
Difficulties with language constitute the most prominent clinical feature of PPA, while cognition usually remains unimpaired at first1. Our patient has deficits in language, memory, praxis, and gnosis. This clinical picture is more likely compatible with AD, since there is global cognitive impairment and there are no behavioral changes typical of FTLD. Even though histopathologic evidence is unavailable, clinical data point to the possibility that this patient initially presented with the logopenic variant of PPA, showing anomic hesitant speech, impaired word retrieval, and sentence repetition deficits among its core features (Table)1. This variant is present in up to 30% of patients with PPA, and it usually progresses to AD1,3. However, features that differentiate the PPA variants in the early stages may lose their distinctiveness as the degeneration advances4.
Language impairment may be the initial feature of AD5, while PPA may evolve to several dementia syndromes. This report emphasizes the need of recognizing not only FTLD and semantic dementia, but also dementia due to AD as a result of the evolution of PPA syndromes.
1. Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology 2011;76:1006-1014. [ Links ]
2. Deramecourt V, Lebert F, Debachy B, et al. Prediction of pathology in primary progressive language and speech disorders. Neurology 2010;74:42-49. [ Links ]
3. Harciarek M, Kertesz A. Primary progressive aphasias and their contribution to the contemporary knowledge about the brain-language relationship. Neuropsychol Rev 2011;21:271-287. [ Links ]
4. Rogalski E, Cobia D, Harrison TM, Wieneke C, Weintraub S, Mesulam MM. Progression of language decline and cortical atrophy in subtypes of primary progressive aphasia. Neurology 2011;76:1804-1810. [ Links ]
5. Ortiz KZ, Bertolucci PHF. Alterações de linguagem nas fases iniciais da doença de Alzheimer. Arq Neuropsiquiatr 2005;63:311-317. [ Links ]
Thais Stvan Vaz
Universidade Federal de São Paulo (UNIFESP)
Escola Paulista de Medicina
Departamento de Neurologia e Neurocirurgia
Rua Botucatu 740
04023-900 São Paulo SP - Brasil
Received 03 January 2012
Received in final for 16 February 2012
Accepted 23 January 2012
Conflict of interest: There is no conflict of interest to declare.