NR ADQI
AU Duffy,P.; Mayeux,R.; Kupsky,W.
TI Familial Alzheimer's disease with myoclonus and 'spongy change'
QU Archives of Neurology 1988 Oct; 45(10): 1097-100
PT journal article
AB "Spongy change," or vacuolar change, was observed in the neocortex of temporal and frontal lobes in four of 27 patients with clinical and pathologic characteristics of Alzheimer's disease. All four cases had the autosomal dominant form of the disease, and aside from severe dementia, all developed myoclonus and became mute before death. The degeneration observed was unlike that seen in Creutzfeldt-Jakob disease because it lacked astrocytosis and had a different distribution of lesions. Moreover, the abundance of neurofibrillary changes and senile plaques was consistent with Alzheimer's disease. None of the other 23 cases were familial, and none developed myoclonus or mutism; three of them had minor vacuolation limited to the temporal lobe. The four cases with severe spongy change described are the first, to our knowledge, in which vacuolar or spongy change has been associated with the familial form of Alzheimer's disease.
MH Aged; Alzheimer Disease/complications/*genetics/pathology; Brain/pathology; Case Report; Female; Frontal Lobe/pathology; Human; Male; Middle Age; Mutism/complications; Myoclonus/*complications; *Nerve Degeneration; Neurofibrils/pathology; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.
AD Department of Neurology, Columbia University, College of Physicians and Surgeons, New York.
SP englisch
PO USA