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We report the clinical,neuroimaging,and neuropathologic features of progressive nonfluent aphasia(PNFA),a rare neurodegenerative syndrome most notable for its distinct language disturbance.Longitudinal observations of 3 patients revealed progressively telegraphic speech and writing,followed by gradual deterioration of sentence comprehension,and finally,preterminal mutism and dementia.Magnetic resonance imaging revealed cortical atrophy most pronounced in anterior regions of the left hemisphere.Functional neuroimaging demonstrated reduced cerebral activity most prominently in left frontal and temporal regions.At necropsy,microscopic pathology of brain was most consistent with the diagnosis of"dementia lacking distinctive histology"(DLDH).A review of published primary progressive aphasia cases with adequate clinical and histopathological descriptions reveals that the most common pathology underlying PNFA is DLDH.PNFA is one example of a family of clinical syndromes with similar underlying histopathology that affects different regions of the frontal lobe. |
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aphasia
aphasia,progressive
aphasia,progressive,primary
CAT scan,emission
CAT scan,emission,abnormal
cerebral cortical atrophy
dementia
familial
genetic neurologic disorders
lobar atrophy
MRI
MRI,abnormal
mutism
neuropathology
neuropathology,brain
spongy degeneration of brain
Wernicke's aphasia
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