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Abstract
Among 158 cases of organic dementia in a prospective study concerning both psychiatry
and regional cerebral blood flow there were 26 cases with a mainly frontal or fronto-temporal
dementia. Careful neuropathological investigation disclosed 20 cases of a mainly frontal
or fronto-temporal grey matter degeneration, in four of them compatible with Pick's
disease (2.5%) whereas 16 cases (10%) appeared to form a separate group without histological
Alzheimer features and therefore named 'frontal lobe degeneration of non-Alzheimer
type' (FLD). The remaining group of dementias of a clinically similar type proved
to consist of cases of Jakob-Creutzfeldt's and Alzheimer's disease with frontal predominance
and also a case of normal frontal cortex with a projected dysfunction caused by bilateral
thalamic infarctions. Also other similar conditions are accounted for from the literature.
The validity of the pathological changes described here, particularly with regard
to their severity and regional distribution as well as their tendency to spare certain
areas is attested by the clinical picture including neuropsychiatric symptoms and
the regional cerebral blood flow pattern, both consistently producing the picture
of a frontal and fronto-temporal disease of a progressive degenerative type. These
features are dealt with in the following papers by Gustafson (1987) and Risberg (1987).
FLD is in some morphological respects similar to other dementing disorders such as
the ALS dementia complex and progressive subcortical gliosis, though with both clinical
and clear-cut pathoanatomical differences. For the time being it seems safest to conclude
that we are faced with a hitherto not fully recognized if not a new type of dementia
caused by 'simple' neuronal degeneration of mainly the frontal or frontal and temporal
lobes. It makes up about 10% of organic dementias, a figure that would be higher in
purely clinical classifications due to the admixture of other frontal lobe disorders
or frontally projected dysfunction clinically simulating FLD of the pathoanatomical
type here described.