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Primary progressive aphasia: A dementia of the language network

Afasia progressiva primária: uma demência da rede de linguagem

ABSTRACT

Primary progressive aphasia (PPA) is a clinical syndrome diagnosed when three core criteria are met. First, there should be a language impairment (i.e., aphasia) that interferes with the usage or comprehension of words. Second, the neurological work-up should determine that the disease is neurodegenerative, and therefore progressive. Third, the aphasia should arise in relative isolation, without equivalent deficits of comportment or episodic memory. The language impairment can be fluent or non-fluent and may or may not interfere with word comprehension. Memory for recent events is preserved although memory scores obtained in verbally mediated tests may be abnormal. Minor changes in personality and behavior may be present but are not the leading factors that bring the patient to medical attention or that limit daily living activities. This distinctive clinical pattern is most conspicuous in the initial stages of the disease, and reflects a relatively selective atrophy of the language network, usually located in the left hemisphere. There are different clinical variants of PPA, each with a characteristic pattern of atrophy. The underlying neuropathological diseases are heterogeneous and can include Alzheimer's disease as well as frontotemporal lobar degeneration. The clinician's task is to recognize PPA and differentiate it from other neurodegenerative phenotypes, use biomarkers to surmise the nature of the underlying neuropathology, and institute the most fitting multimodal interventions.

Key words:
dementia; language; network; frontotemporal; progranulin; tau

RESUMO.

A afasia progressiva primária (APP) é uma síndrome clínica diagnosticada quando três critérios centrais são preenchidos. Inicialmente deve haver comprometimento da linguagem (afasia) que interfere com o uso ou a compreensão das palavras. Em segundo lugar, os métodos diagnósticos devem determinar que a doença é neurodegenerativa e, portanto, progressiva. Por último, a afasia deve emergir de forma relativamente isolada, sem alterações equivalentes de comportamento ou memória episódica. O comprometimento da linguagem pode ser de tipo fluente ou não fluente, e pode ou não interferir com a compreensão de palavras. A memória para eventos recentes está preservada, embora escores de memória em testes verbais possam ser anormais. Alterações discretas de personalidade e comportamento podem estar presentes, mas não são os fatores que levam o paciente ao atendimento médico ou que o limitam em suas atividades de vida diária. Este padrão distinto é mais evidente nas fases iniciais da doença e reflete a atrofia relativamente seletiva da rede de linguagem, geralmente localizada no hemisfério cerebral esquerdo. Há diferentes variantes clínicas da APP, cada uma com padrão de atrofia característico. Os substratos neuropatológicos são heterogêneos e podem incluir a doença de Alzheimer e a degeneração lobar frontotemporal. A tarefa do clínico é reconhecer a APP e diferenciá-la de outros fenótipos neurodegenerativos, utilizar biomarcadores para inferir a natureza da neuropatologia subjacente e instituir as intervenções multimodais cabíveis.

Palavras-chave:
demência; linguagem; rede; frontotemporal; progranulina; tau

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REFERENCES

  • Franceschi F. Gliosi perivasculare in un caso de demenza afasica. Annali di Neurologia 1908;26:281-290.
  • Pick A. Ueber die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Medizinische Wochenschrift 1892;17:165-167.
  • Pick A. Zur Symptomatologie der linksseitigen Schlaffenlappenatrophie. Monatsschrift für Psychiatrie und Neurologie 1904;16:378-388.
  • Rosenfeld M. Die partielle Grosshirnatrophie. Journal of Psychology and Neurology 1909;14:115-130.
  • Sérieux P. Sur un cas de surdité verbale pure. Revue de Medecine 1893;13:733-750.
  • Mesulam M-M. Primary progressive aphasia: a 25 year retrospective. Alzheimer Dis Assoc Disord. 2007;21:S8-S11.
  • Mesulam MM. Slowly progressive aphasia without generalized dementia. Ann Neurol 1982;11:592-598.
  • Mesulam MM. Primary progressive aphasia--differentiation from Alzheimer's disease [editorial]. Ann Neurol1987;22:533-534.
  • Mesulam M-M, Weintraub S. Spectrum of primary progressive aphasia. In: Rossor MN, ed. Unusual Dementias. London: Baillière Tindall, 1992: 583-609.
  • Mesulam M-M. Primary Progressive Aphasia. Ann Neurol2001;49:425-432.
  • Mesulam M-M. Primary Progressive Aphasia. In: FTD-Pick Conference, London, Ontario, 2002.
  • Weintraub S, Rubin NP, Mesulam MM. Primary progressive aphasia. Longitudinal course, neuropsychological profile, and language features. Arch Neurol 1990;47:1329-1335.
  • Broussolle E, Bakchine S, Tommasi M, et al. Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes. J Neurol Sci 1996;144:44-58.
  • Josephs KA, Duffy JR, Strand EA, et al. Characterizing a neurodegenerative syndrome: primary progressive apraxia of speech. Brain 2012;135:1522-1536.
  • Mesulam M-M, Wieneke C, Thompson C, Rogalski E, Weintraub S. Quantitative classification of primary progressive aphasia at early and mild impairment stages. Brain2012;135:1537-1553.
  • Rogalski EJ, Mesulam M-M. Clinical trajectories and biological features of primary progressive aphasia (PPA). Curr Alzh Res 2009;6:331-336.
  • Sapolsky D, Bakkour A, Negreira A, et al. Cortical neuroanatomic correlates of symptom severity in primary progressive aphasia. Neurology 2010;75:358-366.
  • Mesulam M-M, Weintraub S. Primary progressive aphasia and kindred disorders. Hand Clin Neurol 2008;89:573-587.
  • Gorno-Tempini ML, Hillis A, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology2011;76:1006-1014.
  • Gorno-Tempini ML, Brambati SM, Ginex V, et al. The logopenic/phonological variant of primary progressive aphasia. Neurology2008;71:1227-1234.
  • Senaha MLH, Caramelli P, Porto CS, Nitrini R. Verbal and non-verbal semantic impairment from fluent primary progressive aphasia to semantic dementia. Dement Neuropsychol 2007;2:203-211.
  • Folstein M, Folstein S, McHugh PR. Mini-mental state: a practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:189-198.
  • Osher J, Wicklund A, Rademaker A, Johnson N, Weintraub S. The Mini-Mental State Examination in behavioral variant frontotemporal dementia and primary progressive aphasia. Am J Alzheimers Dis Other Demen 2007;22:468-473.
  • Kertesz A. Western Aphasia Battery- Revised (WAB-R). Austin, Texas: Pro-Ed, 2006.
  • Mesulam M, Wieneke C, Rogalski E, Cobia D, Thompson C, Weintraub S. Quantitative template for subtyping primary progressive aphasia. Arch Neurol2009;66:1545-1551.
  • Weintraub S, Mesulam M-M, Wieneke C, Rademaker A, Rogalski EJ, Thompson CK. The Northwestern Anagram Test: measuring sentence production in primary progressive aphasia. Am J Alzheimers Dis Other Demen2009;24:408-416.
  • Thompson CK. Northwestern Assessment of Verbs and Sentences (NAVS). In. Evanston, IL: Northwestern University, 2011.
  • Kay JLR. Psycholinguistic Assesment of Language Processing in Aphasia. Hove: Lawrence Erlbaum Associates, 1992.
  • Dunn LA, Dunn LM. Peabody Picture Vocabulary Test-4: Pearson, 2006.
  • Mesulam M-M, Wieneke C, Hurley RS, et al. Words and objects at the tip of the left temporal lobe in primary progressive aphasia. Brain2012;in press.
  • Kaplan E, Goodglass H, Weintraub S. The Boston Naming Test. Philadelphia: Lea & Febiger, 1983.
  • Howard D, Patterson K. Pyramids and Palm Trees: A Test of Symantic Access From Pictures and Words. Bury St. Edmonds, Suffolk, UK: Thames Valley Test Company, 1992.
  • Weintraub S, Rogalski E, Shaw E, et al. Verbal and nonverbal memory in primary progressive aphasia: the Three Words-Three Shapes Test. Behav Neurol. 2013;26:67-76.
  • Wicklund A, Johnson N, Weintraub N. Preservation of reasoning in primary progressive aphasia: Further differentiation from Alzheimer's disease and the behavioral presentation of frontotemporal dementia. J Clin Exp Neuropsychol 2004;26:347-355.
  • Benton A, Hamsher K, Varney N, Speen O. Contributions to Neuropsychological Assessment, Second ed. New York: Oxford University Press, 1998.
  • Kertesz A, Nadkarni N, Davidson W, Thomas AW. The Frontal Behavioral Inventory in the differential diagnosis of frontotemporal dementia. J Int Neuropsychol Soc 2000;6:460-468.
  • Gorno-Tempini ML, Dronkers NF, Rankin KP, et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol2004; 55:335-346.
  • Sonty SP, Mesulam M-M, Thompson CK, et al. Primary progressive aphasia: PPA and the language network. Ann Neurol2003;53:35-49.
  • Sonty SP, Mesulam M-M, Weintraub S, Johnson NA, Parrish TP, Gitelman DR. Altered effective connectivity within the language network in primary progressive aphasia. J Neurosci 2007;27:1334-1345.
  • Mesulam M, Wicklund A, Johnson N, et al. . Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Ann Neurol2008;63:709-719.
  • Knibb JA, Xuereb JH, Patterson K, Hodges JR. Clinical and pathological characterization of progressive aphasia. Ann Neurol2006;59:156-165.
  • Leyton CE, Villemange VL, Savage S, et al. Subtypes of progressive aphasia: application of the international consensus criteria and validation using b-amyloid imaging. Brain2011;134:3030-3043.
  • Gefen T, Gasho K, Rademaker A, et al. Clinically concordant variations of Alzheimer patology in aphasic versus amnestic dementia. Brain2012;135:1554-1565.
  • Rogalski E, Rademaker A, Helenewski I, et al. APOE e4 is a susceptibility factor in amnestic but not aphasic dementias. Alzheimer Dis Assoc Disord.2011;25:159-163.
  • Rabinovici GD, Jagust WJ, Furst AJ, et al. Ab amyloid and glucose metabolism in three variants of primary progressive aphasia. Ann Neurol2008;64:388-401.
  • Mandell AM, Alexander MP, Carpenter S. Creutzfeldt-Jacob disease presenting as isolated aphasia. Neurology1989;39:55-58.
  • Rademakers R, Baker M, Gass J, et al. Phenotypic variability associated with progranulin haploinsufficiency in patients with the common 1477C-T (Arg493X) mutation: an international initiative. Lancet Neurol 2007;6:857-868.
  • Munoz DG, Ros R, Fatas M, Bermejo F, Yebenes JGd. Progressive nonfluent aphasia associated with a new mutation V363I in tau gene. Am J Alzheimers Dis Other Demen2007;22:294-299.
  • Simón-Sánchez J, Dopper EGP, Cohn-Hokke PE, et al. The clinical and pathological phenotype of C9ORF72 hexanucleotide repeat expansions. Brain2012;135:723-735.
  • Mesulam M, Johnson N, Krefft TA, et al. Progranulin mutations in primary progressive aphasia. Arch Neurol2007;64:43-47.
  • Gliebus G, Bigio E, Gasho K, et al. Asymmetric TDP-43 distribution in primary progressive aphasia with progranulin mutation. Neurology2010;74:1607-1610.
  • Rogalski E, Weintraub S, Mesulam M-M. Are there susceptibility factors for primary progressive aphasia? BrainLang (in press).
  • Rogalski E, Johnson N, Weintraub S, Mesulam M-M. Increased frequency of learning disability in patients with primary progressive aphasia and their first degree relatives. Arch Neurol2008;65:244-248.
  • Alberca R, Montes E, Russell E, Mesulam M-M. Left hemicranial hypoplasia in two patients with primary progressive aphasia. Arch Neurol2004;61:265-268.
  • Gitelman DR, Nobre AC, Sonty S, Parrish TB, Mesulam M-M. Language network specializations: An analysis with parallel task design and functional magnetic resonance imaging. NeuroImage 2005;26: 975-985.
  • Hodges J, Graham N, Patterson K. Charting the progression in semantic dementia: implications for the organization of semantic memory. Memory 1995;3:463-495.
  • Lambon Ralph MA, Cipolotti L, Manes F, Patterson K. Taking both sides: do unilateral anterior temporal lobe lesions disrupt semantic memory? Brain2010;133:3243-3255.
  • Mesulam M-M, Rogalski E, Wieneke C, et al. Neurologyof anomia in the semantic subtype of primary progressive aphasia. Brain2009; 132:2553-2565.
  • Schwartz MF, Kimberg DY, Walker GM, et al. Anterior temporal involvement in semantic word retrieval: voxel-based lesion-symptom mapping evidence from aphasia. Brain2009;132:3411-3427.
  • Rogalski E, Cobia D, Harrison TM, et al. Anatomy in language impairments in primary progressive aphasia. J Neurosci2011;31:3344- 3350.
  • Thompson CK, Cho S, Hsu C-J, et al. Dissociations between fluency and agrammatism in primary progressive aphasia. Aphasiology 2011; 26:20-43.
  • Weintraub S, Morhardt DJ. Treatment, education and resources for non Alzheimer dementia: one size does not fit all. Alzheimer Care Quarterly 2005:201 -214.
  • Reed DA, Johnson NA, Thompson C, Weintraub S, Mesulam M-M. A clinical trial of bromocriptine for tretment of primary progressive aphasia. Ann Neurol2004;56:750.
  • Johnson NA, Rademaker A, Weintraub S, GItelman D, Wieneke C, Mesulam M-M. Pilot trial of memantine in primary progressive aphasia. Alzheimer Dis Assoc Disord 2010;24:308.

Publication Dates

  • Publication in this collection
    Jan-Mar 2013

History

  • Received
    05 Dec 2012
  • Accepted
    08 Feb 2013
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