Acta Neurol Belg DOI 10.1007/s13760-015-0446-8


A patient with agrammatic primary progressive aphasia developing frontotemporal dementia Fabricio Ferreira de Oliveira • Lucas Amorim Vieira de Barros • Paulo Henrique Ferreira Bertolucci

Received: 23 December 2014 / Accepted: 13 February 2015  Belgian Neurological Society 2015

Keywords Aphasia  Frontotemporal dementia  Speech disorders  Disability evaluation  Atrophy

This report focuses on the specific aspects of the neurodegenerative syndrome that started with progressive aphasia and ultimately led to dementia with parkinsonian features suggesting basal nuclei dysfunction.

Introduction There are three recognized subtypes of primary progressive aphasia syndromes [1]: the logopenic variant, the agrammatic (or non-fluent) variant, and semantic dementia. The logopenic variant of primary progressive aphasia, with hesitant anomic speech, word retrieval and sentence repetition deficits, features left posterior perisylvian or parietal atrophy with Alzheimer’s disease pathology, and may evolve to dementia due to Alzheimer’s disease in advanced stages [2]. By comparison, apraxia of speech is one of the initial features of the agrammatic variant, which typically involves the left posterior frontoinsular region, is a better predictor of a tauopathy and may evolve to corticobasal degeneration in later stages, while the semantic variant of primary progressive aphasia develops early involvement of the anterior temporal lobes (predominantly in the dominant hemisphere for language) and usually evolves to the classical form of semantic dementia or to behavioural variant frontotemporal dementia [1].

F. F. de Oliveira (&)  L. A. V. de Barros  P. H. F. Bertolucci Departamento de Neurologia e Neurocirurgia, Escola Paulista de Medicina, Universidade Federal de Sa˜o Paulo (UNIFESP), Rua Botucatu 740, Vila Clementino, Sa˜o Paulo, SP 04023-900, Brazil e-mail: [email protected] L. A. V. de Barros e-mail: [email protected] P. H. F. Bertolucci e-mail: [email protected]

Case report A 74-year-old right-handed female patient with 4 years of schooling started to develop progressive telegraphic speech, non-fluent aphasia with naming impairments and phonemic distortions, along with impaired comprehension of complex sentences. She could obey simple commands, and no memory symptoms were reported. The patient was a widower, and lived alone by then. Later on, she developed frank apraxia of speech and frequent exchanges between ‘‘yes’’ and ‘‘no’’ responses. A computed tomography of the brain showed no evidence of stroke or mass lesions such as tumours; the diagnosis of agrammatic primary progressive aphasia ensued. A year later, she became functionally dependent and could not leave the house alone anymore, running the risk of losing herself. Neuropsychiatric symptoms included severe apathy, irritability, night-time behaviour and appetite disturbances; thus, the diagnosis of frontotemporal dementia was established. By the time she received the diagnosis of dementia, the most remarkable neuropsychiatric results were as follows: •

• • • •

Mini-Mental State Examination: 20 (losing 2 points in orientation, 5 points in calculations, 1 point in recall, 1 point in writing a sentence and 1 point in copy design) Category verbal fluency test in 1 min: 7 for animals, and 6 for fruits 15-point Clock Drawing Test: 10 Digit span—forward: 4 Digit span—backward: 4


Acta Neurol Belg Fig. 1 Magnetic resonance imaging of the brain—axial flair sequences (a, b) and coronal T2 sequences (c, d) showing left perisylvian atrophy

Magnetic resonance imaging of the brain showed left perisylvian atrophy (Fig. 1), while a brain SPECT scan showed hypoperfusion of the frontal lobes and of the left temporoparietal regions (Fig. 2). Lobar hypoperfusion was particularly prominent in the left hemisphere when compared with the right hemisphere. Two years after the initial symptoms started, the patient developed progressive rigidity of the right limbs, bradykinesia, postural instability and an apraxic gait. Ideomotor apraxia was more noticeable when testing the right hand. These asymmetrical parkinsonian features suggested the diagnostic possibility of corticobasal degeneration [1, 3]. The last score on the Mini-Mental State Examination was 15 (losing 3 points in orientation, 5 points in calculations, 1 point in recall, 1 point in repetition, 2 points in verbal command, 1 point in written command, 1 point in writing a sentence and 1 point in copy design), and the last score on the 15-point Clock Drawing Test was 3.


Discussion In the age range of 70–79 years-old, prevalence of dementia in our environment varies from 2.0 % to 32.9 % [4]. It is important to recognize frontotemporal dementia syndromes in the elderly, particularly along the evolution of agrammatic primary progressive aphasia. Descriptions of actual cases of patients that have been followed from the frontal dementia stage to the corticobasal degeneration stage are unusual in the literature. Perisylvian atrophy is an unspecific finding that may be related to several neurodegenerative disorders, but neuroimaging exams are useful to exclude vascular or neoplastic lesions. Impairment in phonologic processing is a typical feature of corticobasal degeneration, and seems to be more remarkable for patients with perisylvian atrophy in the dominant hemisphere for language [5]. Not all patients

Acta Neurol Belg Fig. 2 Brain SPECT scan— rendering showing hypoperfusion of the frontal lobes and of the left temporoparietal regions (a, b) in comparison with the right hemisphere (c, d)

with corticobasal degeneration start with agrammatic primary progressive aphasia and further develop frontotemporal dementia, as much as not all patients with agrammatic primary progressive aphasia develop frontotemporal dementia or corticobasal degeneration. Nevertheless, linguistic impairment in these neurodegenerative syndromes may be a continuum between mild phonologic impairment and severe aphasia.

Conclusions Primary progressive aphasia is a neurodegenerative condition characterized by progressive loss of specific language functions with initial sparing of other cognitive domains. Throughout the course of this disease, patients may develop frontotemporal lobar degeneration syndromes, particularly frontotemporal dementia and, ultimately, corticobasal degeneration. Neuroimaging findings may be unspecific, requiring proper neuropsychiatric assessments for a suitable diagnosis. Considering that no effective treatments are available for these conditions, it is important to bear in mind that this pattern of evolution may require close family support and lead to greater caregiver

distress, consisting on an important issue in public healthcare. Acknowledgments We acknowledge the financial support from CAPES—Coordenac¸a˜o de Aperfeic¸oamento de Pessoal de Nı´vel Superior. We also thank the patient and her caregiver for agreeing with the publication of this case report. Conflict of interest related to this study.

The authors declare no conflicts of interest

Informed consent The patient and her caregiver agreed with the publication of this case report, and signed the informed consent form Ethical standards This study was supported by CAPES—Coordenac¸a˜o de Aperfeic¸oamento de Pessoal de Nı´vel Superior (BRAZIL).

References 1. De Oliveira FF, Correia Marin SM, Ferreira Bertolucci PH (2013) Communicating with the non-dominant hemisphere: implications for neurological rehabilitation. Neural Regen Res 8:1236–1246. doi:10.3969/j.issn.1673-5374.2013.13.009 2. Vaz TS, Bertolucci PHF, Oliveira FF (2012) A patient with primary progressive aphasia developing dementia due to Alzheimer’s disease. Arq Neuropsiquiatr 70:551–552. doi:10.1590/ S0004-282X2012000700016


Acta Neurol Belg 3. Laforce R Jr (2013) Behavioral and language variants of frontotemporal dementia: a review of key symptoms. Clin Neurol Neurosurg 115:2405–2410. doi:10.1016/j.clineuro.2013.09.031 4. Fagundes SD, Silva MT, Thees MFRS, Pereira MG (2011) Prevalence of dementia among elderly Brazilians: a systematic


review. Sao Paulo Med J 129:46–50. doi:10.1590/S151631802011000100009 5. Graham NL, Bak T, Patterson K, Hodges JR (2003) Language function and dysfunction in corticobasal degeneration. Neurology 61:493–499. doi:10.1212/01.WNL.0000081230.09863.ED

A patient with agrammatic primary progressive aphasia developing frontotemporal dementia.

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