A REVIEW OF PRIMARY PROGRESSIVE APHASIA ( MESULAM-SYNDROME) - (1982-1996)

The clinical syndrome of primary progressive aphasia (PPA) is defined as a-gradual onset and worsening of language dysfunction in patients w ithout any major alteration of the other cognitive functions, behaviou r and instrumental daily living activities. The main clinical trait of this syndrome is that spares the patient's autonomy for a long time, but finally turns into generalized dementia. Structural and functional brain imaging studies generally indicate cortical atrophy of the left temporal and/or frontal robe and hypoperfusion or hypometabolism loca lized in left hemisphere, respectively. In this article we present a r eview of 33 autopsy cases of PPA, published between 1982 and 1996. The outcome of this review is that the underlying neurohistopatholgy of t his focal cortical degeneration is very heterogeneous, suggesting that this disorder represent either atypical forms of Pick disease, Alzhei mer type dementia, focal cortical spongiosis, Creutzfelt-Jakob disease , dyspahsic dementia, astrocytic gliosis, focal neuronal achromasia or frontal lobe dementia.