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Subcortical neurofibrillary degeneration presenting as steele‐richardson‐olszewski and other related syndromes: A review of 90 pathologically verified cases
Author(s) -
de Bruin V. M. S.,
Lees A. J.
Publication year - 1994
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.870090402
Subject(s) - degeneration (medical) , pathology , neuroscience , medicine , psychology
We have reviewed 90 patients from the literature with histopathological features compatible with currently accepted criteria for the diagnosis of the Steele‐Richardson‐Olszewski syndrome (SROS). Only 62 patients (69%) had clinically definite SROS based on the criteria of Maher and Lees. Neurofibillary degeneration of subcortical structures with involvement of the internal pallidum, the subthalamic nucleus, and substantia nigra was considered essential for the pathological diagnosis. Thirty‐six cases (40%) had neocortical neurofibrillary change that bore no clear relationship to the degree of cognitive impairment (χ 2 = 9.293; p < 0.4107). Grumose degeneration of the dentate nucleus was present in 25 cases (28%), and occasionally there were other, less usual, findings such as Pick bodies and Lewy bodies.