TY - JOUR
T1 - Subacute diencephalic angioencephalopathy. A clinicopathological case study
AU - DeGirolami, Umberto
AU - Haas, Melvyn L.
AU - Richardson, Edward P.
N1 - Funding Information:
Th.s study was supported m part by USPHS training grant awards 2T1 NS5393 and 2T1 NS5075 and special fellowship award 1Fll NS2359 (Dr DeGlrolaml) from the National Instxtute of Neurological Diseases and Stroke Reprint requests should be addressed to E P Richardson, Jr, C S Kublk Laboratory for Neuropathology, Massachusetts General Hospital, Boston, Mass 02114 (USA)
PY - 1974/6
Y1 - 1974/6
N2 - A 60-year-old previously healthy man died with a progressive neurologic illness, characterized by deterioration of intellect and awareness, impaired ocular motility, and, terminally, widespread myoclonic jerks. The total course was about 11 weeks. The CSF showed a progressive increase in protein content (58 to 97 mg 100 ml), and the EEG showed diffuse slowing; laboratory investigations otherwise were unrevealing. Post-mortem examination showed extensive distinctive lesions in the thalamus and periaqueductal region bilaterally, accompanied by proliferative and inflammatory lesions in the walls of small blood vessels. These lesions were not those of subacute spongiform encephalopathy (Creutzfeldt-Jakob disease), and could not be classified with any of the known forms of human angiopathy. An infective process of an as yet unidentified type is a possibility.
AB - A 60-year-old previously healthy man died with a progressive neurologic illness, characterized by deterioration of intellect and awareness, impaired ocular motility, and, terminally, widespread myoclonic jerks. The total course was about 11 weeks. The CSF showed a progressive increase in protein content (58 to 97 mg 100 ml), and the EEG showed diffuse slowing; laboratory investigations otherwise were unrevealing. Post-mortem examination showed extensive distinctive lesions in the thalamus and periaqueductal region bilaterally, accompanied by proliferative and inflammatory lesions in the walls of small blood vessels. These lesions were not those of subacute spongiform encephalopathy (Creutzfeldt-Jakob disease), and could not be classified with any of the known forms of human angiopathy. An infective process of an as yet unidentified type is a possibility.
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U2 - 10.1016/0022-510X(74)90246-9
DO - 10.1016/0022-510X(74)90246-9
M3 - Article
C2 - 4829194
AN - SCOPUS:0016219230
SN - 0022-510X
VL - 22
SP - 197
EP - 210
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
IS - 2
ER -