Title:Early and Mild Phases of Primary Progressive Aphasia: A Case Series
Volume: 10
Issue: 2
Author(s): Jessica Powell, James Lendrum, Rosalind Huff, Christine Belden and Marwan N. Sabbagh
Affiliation:
Keywords:
Aphasia, dementia, mild cognitive impairment, neuropsychological assessment, primary progressive aphasia.
Abstract: Background: The early and mild phases of various neurodegenerative diseases, sometimes
described as Mild Cognitive Impairment (MCI), has been characterized as the transitional
state between normal cognition and dementia. It is described as having cognitive decline not severe
enough to cause functional impairment. MCI has been divided into amnestic and non-amnestic subtypes
with the amnestic subtype most commonly progressing to AD. The MCI phase of other dementias
such as DLB has also been described. There are very few reports summarizing this early
phase of Primary Progressive Aphasia.
Aims: Our aim is to contribute to the clinical characterization of the early and mild phases of clinically
suspect PPA in order to better describe the presenting features and neuropsychological profile.
Methods: This is a cross-sectional case series abstracted from our memory disorders clinic. We retrospectively
queried and analyzed the cases of 9 patients with a primary diagnosis of MCI and secondary
diagnosis of progressive aphasia. Acquired (non-degenerative) aphasias (e.g. stroke, mass)
were excluded.
Results: Of the 9 cases, 5 were non-amnestic MCI and 4 were amnestic MCI, all with language as
the primary domain. All eMCI cases were non-amnestic. Word finding difficulty was observed in 8
of the 9 cases and sentence repetition impairments in 8 of 8 tested.
Conclusion: PPA is a syndrome with an underlying progressive neurodegenerative etiology that
results in dementia. PPA, like other neurodegenerative conditions, can transition through an early
(i.e. MCI) phase prior to the dementia phase. The clinical description of this early stage of PPA is
predominantly characterized by word finding difficulty on observation, sentence repetition impairment
on neuropsychological testing, and a diagnosis of MCI or early MCI (eMCI) with language as
the primary impaired domain ± an amnestic component.
