Brain, Vol 121, Issue 1 115-126, Copyright © 1998 by Oxford University Press
M Schwarz, R De Bleser, K Poeck and J Weis
Primary progressive aphasia has been clinically defined as a progressive
language deficit leading to the dissolution of almost all language
functions with relative preservation of other cognitive functions until
late in the course of the disease. Two types of language impairment have
been described for primary progressive aphasia, which differ with respect
to the degree of fluency of spontaneous speech. Whereas some authors have
emphasized non-fluency as a defining characteristic of primary progressive
aphasia, others have proposed that phonemic rather than semantic
paraphasias in naming, both in the fluent and the non-fluent subtype,
should be used as a criterion to distinguish primary progressive aphasia
from slowly progressive aphasia in other forms of degenerative brain
disease. Patients with fluent speech and semantic dementia, as typically
seen in Alzheimer's disease, produce semantic paraphasias and
circumlocutions rather than phonemic errors in naming. This paper reports
the long-term follow-up of a patient with fluent aphasic speech, whose
language profile over a decade was similar to that of patients with
semantic dementia. Neuropathological examination revealed no evidence of
Alzheimer's disease. Pick's disease or Pick variant, but showed spongiform
changes of cortical layers (II and III) in temporal and, less severely, in
frontal gyri. The present case indicates that semantic dementia is not a
reliable indicator of probable Alzheimer's disease and supports the notion
that there are different subtypes of primary progressive aphasia which
cannot be defined by fluency or by the presence of phonemic paraphasia.
Progress in identifying the neuropathological correlates of these subtypes
in cases with lobar atrophy and spongiform changes should be expected from
hereditary variants of progressive disorder.
ARTICLES
A case of primary progressive aphasia. A 14-year follow-up study with neuropathological findings
Department of Neurology, RWTH Aachen, Germany.
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