Objective: To investigate the a series of cases presenting with apparent logopenic variant primary progressive aphasia who then developed symptoms suggestive of PSP.

Background:
Deficits of language are prominent aspects of many neurodegenerative diseases. Consistent with the principal of selective vulnerability of brain networks in neurodegenerative disease, specific patterns of language disruption are associated with particular pathologies and patterns of anatomical spread. Tau pathology is most commonly associated with frontal perisylvian degeneration, a pattern of involvement which manifests as non-fluent variant progressive aphasia (nfvPPA) and apraxia of speech (AOS).

Method: We describe three cases of patients presenting with clinical presentations and ancillary tests consistent with tau-associated movement disorders and prominent early language impairment following a logopenic aphasia (lvPPA).

Results: Case 1: 69 year old woman presented with a two year history of word finding difficulties. There was mild receptive and mild-moderate expressive dysfunction with word finding difficulty in spontaneous speech and naming, frequent pauses and hesitations, and mild comprehension difficulty with increasing length and complexity, consistent with a diagnosis of lvPPA. Within 18 months she had developed a supranuclear gaze palsy, nuchal rigidity, bradykinesia, and postural instability.

Case 2: A 72 year old farmer presented with a three and a half year history of speech disturbance. There was difficulty with repetition of lengthy words and categorical fluency was better preserved than lexical fluency, suggestive of lvPPA. Within 2 years he developed square-wave jerks, initiation latency in saccades. He had an anteroflexed posture and a “gunslinger” gait. The syndrome suggested a PSP/CBS overlap syndrome. CSF biomarkers were not suggestive of amyloid pathology.

Case 3: A 76 year old cleric presented with a 2 year history of speech disturbance consistent with lvPPA. There were square-wave jerks and slow vertical saccades with a “round-the-houses” sign on downward saccades. There was axial rigidity, parkinsonism, and marked retropulsion.

Conclusion:
In each case, the patient went on to develop a motor syndrome consistent with PSP or a PSP/CBS overlap syndrome. Tauopathy-associated movement disorders can present language deficits with phenomenology suggestive of logopenic aphasia.

References: 1. Peterson KA, Patterson K, Rowe JB. Language impairment in progressive supranuclear palsy and corticobasal syndrome. J Neurol. 2021;268(3):796-809. doi:10.1007/s00415-019-09463-1.
2. Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76(11):1006-1014. doi:10.1212/WNL.0b013e31821103e6.

« Back to 2022 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/three-patients-with-psp-presenting-with-a-logopenic-pattern-of-aphasia-expanding-the-phenotype-of-language-impairment-in-psp/