TY - JOUR
T1 - Dementia lacking distinctive histologic features
T2 - A common non-Alzheimer degenerative dementia
AU - Knopman, D. S.
AU - Mastri, A. R.
AU - Frey, W. H.
AU - Sung, J. H.
AU - Rustan, T.
PY - 1990/2
Y1 - 1990/2
N2 - From a series of 460 dementia patients referred to a regional brain bank, 14 (3%) patients had a pathologic diagnosis of primary degeneration of the brain involving multiple sites (frontoparietal cortex, striatum, medial thalamus, substantia nigra, and hypoglossal nucleus), with cell loss and astrocytosis. There were no neuronal inclusions and essentially no senile plaques. This entity, which we have termed “dementia lacking distinctive histology”(DLDH), presented with memory loss and personality changes, and led to death, usually within 2 to 7 years. Dysarthria and dysphagia were prominent in the later phases of the illness in most patients. The psychometric findings of some of the patients were consistent with a “frontal” lobe dementia. A few patients had prominent caudate atrophy on CT as well as neuropathologically. Eight of our patients had positive family histories for neurologic disease, mainly dementia. DLDH, in addition to Pick’s disease, is a major member of the frontallobe dementia group. In patients under age 70 years, the frontal lobe dementias represent an important diagnostic consideration.
AB - From a series of 460 dementia patients referred to a regional brain bank, 14 (3%) patients had a pathologic diagnosis of primary degeneration of the brain involving multiple sites (frontoparietal cortex, striatum, medial thalamus, substantia nigra, and hypoglossal nucleus), with cell loss and astrocytosis. There were no neuronal inclusions and essentially no senile plaques. This entity, which we have termed “dementia lacking distinctive histology”(DLDH), presented with memory loss and personality changes, and led to death, usually within 2 to 7 years. Dysarthria and dysphagia were prominent in the later phases of the illness in most patients. The psychometric findings of some of the patients were consistent with a “frontal” lobe dementia. A few patients had prominent caudate atrophy on CT as well as neuropathologically. Eight of our patients had positive family histories for neurologic disease, mainly dementia. DLDH, in addition to Pick’s disease, is a major member of the frontallobe dementia group. In patients under age 70 years, the frontal lobe dementias represent an important diagnostic consideration.
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U2 - 10.1212/wnl.40.2.251
DO - 10.1212/wnl.40.2.251
M3 - Article
C2 - 2300243
AN - SCOPUS:0025341975
SN - 0028-3878
VL - 40
SP - 251
EP - 256
JO - Neurology
JF - Neurology
IS - 2
ER -