Multidisciplinary Approach to Diagnosis of Primary Progressive Aphasia in a Younger Middle Aged Patient

Authors: Robert Krause

Abstract:

Primary progressive aphasia (PPA) is a neurodegenerative disease similar to frontotemporal and semantic dementia, while having a different clinical image and anatomic pathology topography. Nonetheless, they are often included under an umbrella term: frontotemporal lobar degeneration (FTLD). In the study, examples of diagnosing PPA are presented through the multidisciplinary lens of specialists from different fields (neurologists, psychiatrists, clinical speech therapists, clinical neuropsychologists and others) using a variety of diagnostic tools such as MR, PET/CT, genetic screening and neuropsychological and logopedic methods. Thanks to that, specialists can get a better and clearer understanding of PPA diagnosis. The study summarizes the concrete procedures and results of different specialists while diagnosing PPA in a patient of younger middle age and illustrates the importance of multidisciplinary approach to differential diagnosis of PPA.

Keywords: Primary progressive aphasia, etiology, diagnosis, younger middle age.

References:

[1] Mesulam MM, Grossmann M, Hillis A, Kertesz A, Weintraub S. The core and halo of primary progressive aphasia and semantic dementia. Ann Neurol 2003; 54 (suppl 5): S11–S14.
[2] Neary D, Snowden J, Mann D. Frontotemporal dementia.Lancet Neurol. 2005 Nov; 4 (11): 771–780. Review.
[3] Miller BL, Diehl J, Freedman M, Kertesz A, Mendez M, Rascovsky K. International approaches to frontotemporal dementia diagnosis: From social cognition to neuropsychology. Ann Neu- rol 2003; 54 (suppl 5): S7–S10.
[4] Kertesz A, Hillis A, Munoz DG. Frontotemporal degeneration, Pick‘s disease, Pick complex, and Ravel. Ann Neurol 2003; 54 (suppl 5): S1–S2.
[5] Neary D, Frontotemporal Degeneration, Pick Disease and Corticobasal Degeneration Arch. Neurol 1997; 54: 1425–1427.
[6] Munoz D, Dickson DW, Bergeon C, Mackenzie IRA, Delacourte A, Zhukareva V, The neu- ropathology and biochemistry of frontotemporal dementia Ann Neurol 2003; 54 (suppl 5): S24– S28.
[7] Gorno – Tempini, M.L. et.al. (2011) Classification of primary progressive aphasia and its variants. Neurology, 76 (2011), pp. 1006-10
[8] Baba Y, Tsuboi Y, Baker MC, Uitti RJ, Hutton ML, Dickson DW, Farrer M, et al. The effect of tau genotype on clinical features in FTDP-17. Parkinsonism Relat Disord. 2005 Jun; 11 (4): 205–208.
[9] Kobayashi K, Hayashi M, Kidani T, Ujike H, Iijima M, Ishihara T, Nakano H, Sugimori K, Shimazaki M, Kuroda S, Koshino Y. Pick‘s disease pathology of a missense mutation of S305N of frontotemporal dementia and parkinsonism linked to chromosome 17: another phenotype of S305N. Dement Geriatr Cogn Disord 17: 2004, 293–297,
[10] Poorkaj P, Grossman M, Steinbart E, Payami H, Sadovnick A, Nochlin D, Tabira T, Trojanowski JQ, Borson S, Galasko D, Reich S, Quinn B, Schellenberg G, Bird TD. Frequency of tau gene mutations in familial and sporadic cases of non- Alzheimer dementia. Archives of Neurology 58: 383–387, 2001
[11] Gauthier S. Alzheimer disease. Third edition. Informa UK Ltd. 2007: 169–170.
[12] Grossman, M. Primary progressive aphasia: clinicopathological correlations. Nature Reviews Neurology. 2010, 6, 88¬97.
[13] Mesulam, M. M. Primary progressive aphasia. A dementia of the language network. Dementia&Neuropsychologia 2013 March; 7(1):2¬9.
[14] Mesulam, M. M. Slowly progressive aphasia without generalized dementia. Annals of Neurology 1982; 11:592¬598.
[15] Kirschner, H. S. Frontotemporal dementia and primary progressive aphasia, a review. Neuropsychiatric Disease and Treatment. 2014, 10, 1045¬1055.
[16] Gorno¬tempini, M.L., hillis, A., Weintraub, S., et al. Classification of primary progressive aphasia and its variants. Neurology 2011; 76:1006¬1014.
[17] Sajjadi, S. A., Patterson, K., Arnold, R. J., Watson, P. C., Nestor, P. J. Primary progressive aphasia: a tale of two syndromes and the rest. Neurology. 2012 May 22;78(21):1670¬7.
[18] Mesulam, M. M, Weintraub, S. Spectrum of primary progressive aphasia. In: Rossor MN, ed. Unusual Dementias. London: BaillièreTindall, 1992: 583¬609.