Abstract
INTRODUCTION:Dementias are a group of neurocognitive disorders, and in people under 65 years of age, frontotemporal dementia stands out, a heterogeneous neurodegenerative syndrome that has two major variants: behavioral and primary progressive aphasia. In the latter, three variants are described: non-fluent, semantic and logopenic, which require up-to-date knowledge in practice for their differentiation and understanding. The objective is to carry out a narrative review on the three clinical variants of primary progressive aphasia, delving into diagnosis, evolution, imaging characteristics and management.
MATERIALS AND METHODS:Narrative review article based on the state of the art in biomedical literature on frontotemporal dementia, primary progressive aphasia and its variants.
RESULTS:The compromise of language and other cognitive functions, as well as the imaging findings, are heterogeneous in the three variants. Semiologically, non-fluent progressive primary aphasia is characterized by apraxia of speech, the logopenic variant by failures in the nomination and the semantic variant by failures in the meaning of the message. Imaging involvement in non-fluent progressive primary aphasia is mainly frontoinsular and cortico-subcortical; in the semantic variant it is usually temporary on the dominant side; and in the logopenic variant, temporo-parietal alterations prevail. There is no specific treatment, but some pharmacological options can be linked with language rehabilitation processes / techniques.
CONCLUSION:although Frontotemporal dementia is an heterogenous disorder, there are important clinical and imagenologic features that are useful to the diagnostic approach in the clinical field.
References
Grupo Gestión Integrada para la Salud Mental, Subdirección de Enfermedades No Trasmisibles. Boletín de salud mental n.o 3: Demencia [Internet]. Ministerio de Salud; 2017. Disponible en: https://www.minsalud.gov.co/sites/rid/Lists/Biblioteca-Digital/RIDE/VS/PP/ENT/Boletin-demencia-salud-mental.pdf
Zurique C, Cadena MO, Zurique M, Camacho PA, Sánchez M, Hernández S, et al. [Prevalence of dementia in the elderly in Latin America: A systematic review]. Rev Esp Geriatr Gerontol. 2019;54(6):346-55, doi: 10.1016/j.regg.2018.12.007.
Pradilla AG, Vesga A, León-Sarmiento FE. Estudio neuro-epidemiológico nacional (Epineuro) colombiano. Rev Panam Salud Pública. 2003;14:104-11.
García-Cifuentes E, Jaramillo-Jiménez A, Aguillón D, Gómez-Vega M, Vélez-Hernández JE, Cano Gutiérrez C, et al. Prevenir la demencia: un reto para la salud pública en Colombia. Acta Neurol Colomb. 2019;35(4):208-10, doi: 10.22379/24224022269.
Ramírez L, Velila L, Quiroz Y, Lopera F, Giraldo M. Demencia frontotemporal: variante temporal derecha, reporte de dos casos. Acta Neurol Colomb. 2016;32(1):54-60.
Gutiérrez MA, Valdés B, Cordido F, Anguita G, Vallejo P, Molina P, et al. Enfermedad neurodegenerativa: demencia frontotemporal. Sanid Mil. 2020;76(1):39-40, doi: 10.4321/s1887-85712020000100008.
Dottori M, Sedeño L, Caro MM, Alifano F, Hesse E, Mikulan E, et al. Biomarcadores asequibles de demencia frontotemporal: un estudio de clasificación basado en la conectividad de redes cerebrales. Cuad Neuropsicol. 2019;13(3):103-30. doi: 10.7714/CNPS/13.3.208.
Sepúlveda-Ibarra C. Correlato entre hallazgos neurológicos y sintomatología neuropsiquiátrica en la demencia fronto-temporal de variante conductual: revisión sistemática. Rev Chil Neuro-Psiquiatr. 2020;58(1):40-9, doi: 10.4067/S0717-92272020000100040.
Custodio N, Becerra-Becerra Y, Cruzado L, Castro-Suárez S, Montoya-Bedoya S., Campuzano-Cortina S., Gómez-Ruiz I., et al. Montesinos R, Bardales Y, et al. Nivel de conocimientos sobre demencia frontotemporal en una muestra de médicos que evalúan regularmente a pacientes con demencia en Lima-Perú. Rev Chil Neuro-Psiquiatr. 2018;56(2):77-88, doi: 10.4067/s0717-92272018000200077.
Lillo P, Leyton C. Demencia frontotemporal, cómo ha resurgido su diagnóstico. Rev Med Clin Las Condes. 2016;27(3):309-18.
Young JJ, Lavakumar M, Tampi D, Balachandran S, Tampi RR. Frontotemporal dementia: latest evidence and clinical implications. Ther Adv Psychopharmacol. 2018;8(1):33-48, doi: 10.1177/2045125317739818.
Matallana D, Montañés P. Dementia and creativity: the emergence of pictorial activity in a patient with primary progressive aphasia (PPA). Rev Colomb Psiquiatr. 2010;39(1):211-23.
Botha H, Duffy JR, Whitwell JL, Strand EA, Machulda MM, Schwarz CG, et al. Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech. Cortex. 2015;69:220-36, doi: 10.1016/j.cortex.2015.05.013.
Henry ML, Hubbard HI, Grasso SM, Mandelli ML, Wilson SM, Sathishkumar MT, et al. Retraining speech production and fluency in non-fluent/agrammatic primary progressive aphasia. Brain. 2018;141(6):1799-814, doi: 10.1093/brain/awy101.
Mestres JS. Afasia progresiva primaria: Aspectos clínicos y diagnósticos. Inf Psiquiatr. 2017;(228):13-23.
Cabello PLR, Siguero AM. Demencia fronto-temporal variante conductual: la visión del médico general y de familia. Med Gen. 2018;7(2):7.
Tabernero ME, Politis DG. Reconocimiento facial de emociones básicas y su relación con la teoría de la mente en la variante conductual de la demencia frontotemporal. Interdisciplinaria. 2016;(1):21-39.
Olney NT, Spina S, Miller BL. Frontotemporal dementia. Neurol Clin. 2017;35(2):339-74, doi: 10.1016/j.ncl.2017.01.008.
Cope TE, Wilson B, Robson H, Drinkall R, Dean L, Grube M, et al. Artificial grammar learning in vascular and progressive non-fluent aphasias. Neuropsychologia. 2017;104:201-13, doi: 10.1016/j.neuropsychologia.2017.08.022.
Custodio N, Castro S, Herrera-Pérez E, Lira D, Núñez del Prado L, Cortijo P, et al. Afasias progresivas primarias: las afasias de lenta evolución a demencia. Rev Neuropsiquiatr. 2013;75(3):93-100, doi: 10.20453/rnp.v75i3.1227.
Mandelli ML, Vilaplana E, Brown JA, Hubbard HI, Binney RJ, Attygalle S, et al. Healthy brain connectivity predicts atrophy progression in non-fluent variant of primary progressive aphasia. Brain. 2016;139(Pt 10):2778-91, doi: 10.1093/brain/aww195.
Santos-Santos MA, Mandelli ML, Binney RJ, Ogar J, Wilson SM, Henry ML, et al. Features of patients with nonfluent/ agrammatic primary progressive aphasia with underlying progressive supranuclear palsy pathology or corticobasal degeneration. JAMA Neurol. 2016;73(6):733-42, doi: 10.1001/jamaneurol.2016.0412.
Solís CA, Salvador-Cruz J, Marcos-Ortega J. Alteraciones de la lectura en un caso de afasia primaria progresiva no fluente. Cuad Neuropsicol. 2019;13(2):62-72, doi: 10.7714/CNPS/13.2.206.
Martínez-Cuitiño M, Soriano F, Formoso J, Borovinsky G, Ferrari J, Pontello N, et al. Semantic processing of concrete and abstract concepts in semantic variant - primary progressive aphasia. Rev Investig Logop. 2018;8(1):63-76.
Meyer AM, Getz HR, Brennan DM, Hu TM, Friedman RB. Telerehabilitation of anomia in primary progressive aphasia. Aphasiology. 2016;30(4):483-507, doi: 10.1080/02687038.2015.1081142.
Weder ND, Aziz R, Wilkins K, Tampi RR. Frontotemporal dementias: a review. Ann Gen Psychiatry. 2007;6:15, doi: 10.1186/1744-859X-6-15.
Botha H, Josephs KA. Primary progressive aphasias and apraxia of speech. Continuum (Minneap Minn). 2019;25(1):101-27, doi: 10.1212/CON.0000000000000699.
Meier EL, Breining BL, Sheppard SM, Goldberg EB, Tippett DC, Tsapkini K, et al. White matter hyperintensities contribute to language deficits in primary progressive aphasia. Cogn Behav Neurol. 2020;33(3):179-91, doi: 10.1097/WNN.0000000000000237.
Taylor-Rubin C, Croot K, Nickels L. Is word learning enough? Improved verb phrase production following cueing of verbs and nouns in primary progressive aphasia. Cortex. 2021;139:178-97, doi: 10.1016/j.cortex.2021.02.022.
Tippett DC, Hillis AE, Tsapkini K. Treatment of primary progressive aphasia. Curr Treat Options Neurol. 2015;17(8):362, doi: 10.1007/s11940-015-0362-5.
Afasia progresiva primaria - Síntomas y causas - Mayo Clinic [Internet]. [Consultado 2020 Sept. 4]. Disponible en: Disponible en: https://www.mayoclinic.org/es-es/diseases-conditions/primary-progressive-aphasia/symptoms-causes/syc-20350499
Warren JD, Rohrer JD, Rossor MN. Clinical review. Fronto temporal dementia. Br Med J. 2013;347:f4827, doi: 10.1136/bmj.f4827.
Boeve BF. Parkinson-related dementias. Neurol Clin. 2007;25(3):761-81, doi: 10.1016/j.ncl.2007.04.002.
Ringholz GM, Greene SR. The relationship between amyotrophic lateral sclerosis and frontotemporal dementia. Curr Neurol Neurosci Rep. 2006;6(5):387-92, doi: 10.1007/s11910-996-0019-6.
Andrade-Calderón P, Salvador-Cruz J, Sosa-Ortiz AL. Positive impact of speech therapy in progressive non-fluent aphasia. Acta Colomb Psicol. 2015;18(2):101-14, doi: 10.14718/ACP.2015.18.2.9.
Hazelton JL, Irish M, Hodges JR, Piguet O, Kumfor F. Cognitive and affective empathy disruption in non-fluent primary progressive aphasia syndromes. Brain Impair. 2017;18(1):117-29, doi: 10.1017/BrImp.2016.21.
Vandenberghe R. Classification of the primary progressive aphasias: principles and review of progress since 2011. Alzheimers Res Ther. 2016;8(1):16, doi: 10.1186/s13195-016-0185-y.
Matías-Guiu JA, García-Ramos R, Cabrera-Martín MN, Moreno-Ramos T. Logopenic progressive aphasia associated with idiopathic Parkinson's disease. Neurologia. 2015;30(8):521-4, doi: 10.1016/j.nrl.2014.01.007.
García CG, Lancho MCP. Demencia frontotemporal: programa de intervención psicológica en afasia progresiva primaria fluente. Rev Infad Psicol. 2019;1(1):297-308.
Pérez Lancho MC, García Bercianos S. Caracterización del lenguaje en las variantes lingüísticas de la afasia progresiva primaria. Rev Signos. 2020;53(102):198-218, doi: 10.4067/S0718-09342020000100198.
Quang H, Wong S, Husain M, Piguet O, Hodges JR, Irish M, et al. Beyond language impairment: Profiles of apathy in primary progressive aphasia. Cortex. 2021;139:73-85, doi: 10.1016/j.cortex.2021.02.028.
Garcin B, Lillo P, Hornberger M, Piguet O, Dawson K, Nestor PJ, et al. Determinants of survival in behavioral variant fron-totemporal dementia. Neurology. 2009;17;73(20):1656-61, doi: 10.1212/WNL.0b013e3181c1dee7.
Aguillón-Solís C, Salvador-Cruz J, Marcos-Ortega J. Alteraciones de la lectura en un caso de afasia primaria progresiva no fluente. Cuad Neuropsicol. 2019;13(2), doi: 10.7714/CNPS/13.2.206.
Soriano FG, Zamora DJ, Ferrari JC, Martínez-Cuitiño M. Efecto de concretud inverso en Afasia Progresiva Primaria-variante semántica: Estudio longitudinal de un paciente. Rev Iberoam Psicol. 2019;12(2):51-60, doi: 10.33881/2027-1786.rip.12205.
Bisenius S, Neumann J, Schroeter ML. Validating new diagnostic imaging criteria for primary progressive aphasia via anatomical likelihood estimation meta-analyses. Eur J Neurol. 2016;23(8):e52-3, doi: 10.1111/ene.13046.
Barrios YC, Hernández OG, Rojas ACC, Soler JFM. Diferenciación clínica, patológica y genética entre demencia fronto temporal (DFT) y esclerosis lateral amiotrófica (ELA) (Original). Rev Científica Olimp. 2018;15(51):154-66.
Harciarek M, Kertesz A. Primary progressive aphasias and their contribution to the contemporary knowledge about the brain-language relationship. Neuropsychol Rev. 2011;21(3):271-87, doi: 10.1007/s11065-011-9175-9.
University of Washington. Neuroscience coloring book [Internet]. Neuroscience for Kids. Disponible en: https://faculty.washington.edu/chudler/introb.html
Lombardi J, Mayer B, Semler E, Anderl-Straub S, Uttner I, Kassubek J, et al. Quantifying progression in primary progressive aphasia with structural neuroimaging. Alzheimers Dement. 2021;17(10):1595-609, doi: 10.1002/alz.12323.
Matías-Guiu JA, García-Ramos R. Primary progressive aphasia: from syndrome to disease. Neurologia. 2013;28(6):366-74, doi: 10.1016/j.nrl.2012.04.003.
Jiménez de la Peña MM, Gómez Vicente L, García Cobos R, Martínez de Vega V. Correlación neurorradiológica de las afasias. Mapa cortico-subcortical del lenguaje. Radiologia. 2018;60(3):250-61, doi: https://doi.org/10.1016/j.rx.2017.12.008
Battista P, Capozzo R, Rizzo G, Zecca C, Anastasia A, De Blasi R, Logroscino G. Early pathological gambling in cooccurrence with semantic variant primary progressive aphasia: a case report. Clin Interv Aging. 2019;14:727-33, doi: https://doi.org/10.2147/CIA.S197484.
Huey ED, Putnam KT, Grafman J. A systematic review of neurotransmitter deficits and treatments in frontotemporal dementia. Neurology. 2006;66(1):17-22, doi: 10.1212/01.wnl.0000191304.55196.4d.
Cardarelli R, Kertesz A, Knebl JA. Frontotemporal dementia: a review for primary care physicians. Am Fam Physician. 2010;82(11):1372-7.
Kertesz A, Morlog D, Light M, Blair M, Davidson W, Jesso S, et al. Galantamine in frontotemporal dementia and primary progressive aphasia. Dement Geriatr Cogn Disord. 2008;25(2):178-85, doi: 10.1159/000113034.
Paek EJ, Murray LL, Newman SD. Effects of concurrent action and object naming treatment on naming skills and functional brain activation patterns in primary progressive aphasia: An fMRI study with a case-series design. Brain Lang. 2021;218:104950, doi: 10.1016/j.bandl.2021.104950.
Themistocleous C, Webster K, Tsapkini K. Effects of tDCS on sound duration in patients with apraxia of speech in primary progressive aphasia. Brain Sci. 2021;11(3):335, doi: 10.3390/brainsci11030335.
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.