A Dementia with Lewy Bodies Patient Presents Primary Progressive Aphasia
DOI:
https://doi.org/10.12970/2311-1917.2021.09.04Keywords:
Dementia with Lewy bodies (DLB), primary progressive aphasia (PPA), the agrammatic variant (PPA-G), the semantic variant (PPA-S), the logopenic variant (PPA-L)Abstract
Dementia with Lewy bodies (DLB) is a popular cause of dementia that clinically manifests as dementia with any combination of parkinsonism, psychosis, delusion, hallucination and pareidolia and rapid eye movement sleep behavior disorder (RBD). However, aphasia is not its suggestive diagnostic feature. Another hand, primary progressive aphasia (PPA) is a neurological syndrome in which language capabilities become slowly and progressively impaired. PPA can be divided into three variants: the agrammatic variant (PPA-G), the semantic variant (PPA-S), and the logopenic variant (PPA-L). Mesulam suggested frequent associations between PPA-G and frontotemporal lobar degeneration (FTLD), between the PPA-S and FTLD, and between PPA-L and AD.
The PPA-L is characterized by fluent but sparse spontaneous speech caused by word finding difficulty and severely impaired sentence repetition. Accumulated evidence suggests that a wide variety of disorders including DLB can develop PPA. We report a DLB patient with PPA-L.
References
McKeith I, Mintzer J, Aarsland D, et al. Dementia with Lewy bodies. Lancet Neurol 2004; 3: 19-28. https://doi.org/10.1016/S1474-4422(03)00619-7
McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: fourth consensus report of the DLB consortium. Neurology 2017; 89: 88-100. https://doi.org/10.1212/WNL.0000000000004058
Mesulam MM. Primary progressive aphasia. Ann Neurol 2001; 49: 425-32. https://doi.org/10.1002/ana.91
Mesulam MM. Primary progressive aphasia–a language-based dementia. N Engl J Med 2003; 349: 1535-42. https://doi.org/10.1056/NEJMra022435
Gorno-Tempini ML, Dronkers NF, Rankin KP, et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 2004; 55: 335-46. https://doi.org/10.1002/ana.10825
Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology 2011; 76: 1006-14. https://doi.org/10.1212/WNL.0b013e31821103e6
Bonner MF, Ash S, Grossman M. The new classification of primary progressive aphasia into semantic, logopenic, or nonfluent/agrammatic variants. Curr Neurol Neurosci Rep 2010; 10: 484-90. https://doi.org/10.1007/s11910-010-0140-4
Harris JM, Gall C, Thompson JC, Richardson AM, et al. Classification and pathology of primary progressive aphasia. Neurology 2013; 81: 1832-9. https://doi.org/10.1212/01.wnl.0000436070.28137.7b
Caselli RJ, Beach TG, Sue LI, et al. Progressive aphasia with Lewy bodies. Dement Geriatr Cogn Disord 2002; 14: 55-8. https://doi.org/10.1159/000064925
Le Rhun E, Richard F, Pasquier F. Natural history of primary progressive aphasia. Neurology 2005; 65: 887-91. https://doi.org/10.1212/01.wnl.0000175982.57472.84
Teichmann M, Migliaccio R, Kas A, et al. Logopenic progressive aphasia beyond Alzheimer's–an evolution towards dementia with Lewy bodies. J Neurol Neurosurg Psychiatry 2013; 84: 113-4. https://doi.org/10.1136/jnnp-2012-302638
Watanabe H, Ikeda M, Mori E. Primary Progressive Aphasia as a Prodromal State of Dementia With Lewy Bodies: A Case Report. Front Neurol 2020; 11: 49. https://doi.org/10.3389/fneur.2020.00049
Yamada M, Komatsu J, Nakamura K, et al. Diagnostic Criteria for Dementia with Lewy Bodies: Updates and Future Directions. J Mov Disord 2020; 13: 1-10. https://doi.org/10.14802/jmd.19052
Abe K. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology 2018; 90: 298-299. https://doi.org/10.1212/WNL.0000000000004920
Clark HM, Utianski RL, Duffy JR, et al. Western Aphasia Battery-Revised Profiles in Primary Progressive Aphasia and Primary Progressive Apraxia of Speech. Am J Speech Lang Pathol 2020; 21: 498-510. https://doi.org/10.1044/2019_AJSLP-CAC48-18-0217
Kitajima K, Abe K, Takeda M, et al. Clinical impact of 11C-Pittsburgh compound-B positron emission tomography in addition to magnetic resonance imaging and single-photon emission computed tomography on diagnosis of mild cognitive impairment to Alzheimer's disease. Medicine (Baltimore) 2021; 100: e23969. https://doi.org/10.1097/MD.0000000000023969
Dubois B, Feldman HH, Jacova C, et al. Advancing research diagnostic criteria for Alzheimer's disease: the IWG-2 criteria. Lancet Neurol 2014; 113: 614-29. https://doi.org/10.1016/S1474-4422(14)70090-0
Grossman M. Primary progressive aphasia: clinicopathological correlations. Nat Rev Neurol 2010; 6: 88-97. https://doi.org/10.1038/nrneurol.2009.216
Volkmer A, Rogalski E, Henry M, et al. Speech and language therapy approaches to managing primary progressive aphasia. Pract Neurol 2020; 20: 154-161. https://doi.org/10.1136/practneurol-2018-001921
Ash S, McMillan C, Gross RG, et al. The organization of narrative discourse in Lewy body spectrum disorder. Brain Lang 2011; 119: 30-41. https://doi.org/10.1016/j.bandl.2011.05.006
Jokel R, Graham NL, Rochon E, et al. Word retrieval therapies in primary progressive aphasia. Aphasiology 2014; 28: 1038-68. https://doi.org/10.1080/02687038.2014.899306
Henry ML, Hubbard HI, Grasso SM, et al. Retraining speech production and fluency in non-fluent/agrammatic primary progressive aphasia. Brain 2018; 141: 1799-814. https://doi.org/10.1093/brain/awy101
Kindell J, Wilkinson R, Sage K, et al. Combining music and life story to enhance participation in family interaction in semantic dementia: A longitudinal study of one family’s experience. Arts and Health: an international journal for research, policy and practice. ISSN 1753-3015. https://doi.org/10.1080/17533015.2017.13422692017
