-transparent.png){kind=logo}
An 83-year-old female with no personal or familial neurological history developed progressive gait and speech disturbance and left motor deficit. She suffered intractable seizures and died 3 months after the onset of neurological signs. Neuropathology showed severe spongiosis and gliosis in the cortex and basal ganglia, and diffuse cerebral amyloid angiopathy. Immunostaining for prion protein (PrP) showed intense PrP positivity in areas of confluent spongiosis and some granular staining in astrocytes. The cortical vessel walls stained positively for beta/A4 amyloid but not for PrP amyloid. Both types of amyloid were only observed in pericapillary parenchyma, in areas with severe spongiosis. There were only a few tangles and neuritic plaques in the temporal cortex; amyloid plaques were not present either by silver stains or immunostains. There was neither arteriopathic leukoencephalopathy nor cerebral hemorrhage. Immunoblot analysis of brain extracts revealed an abnormal proteinase K-resistant isoform of PrP. Association of Creutzfeldt-Jakob disease and Cerebral amyloid angiopathy in the absence of Alzheimer changes in unusual. The association of PrP and beta/A4 amyloid deposits could have been fortuitous in an 83-year-old patient. An etiopathogenic relationship between beta/A4 amyloid deposition and PrP accumulation may also be considered.
F Gray, and
F Chrétien, and
P Cesaro, and
J Chatelain, and
P Beaudry, and
J L Laplanche, and
J Mikol, and
J Bell, and
P Gambetti, and
J D Degos
August 2010,
Journal of the neurological sciences,
F Gray, and
F Chrétien, and
P Cesaro, and
J Chatelain, and
P Beaudry, and
J L Laplanche, and
J Mikol, and
J Bell, and
P Gambetti, and
J D Degos
July 2017,
The Australian and New Zealand journal of psychiatry,
F Gray, and
F Chrétien, and
P Cesaro, and
J Chatelain, and
P Beaudry, and
J L Laplanche, and
J Mikol, and
J Bell, and
P Gambetti, and
J D Degos
November 1985,
Journal of neurology, neurosurgery, and psychiatry,
F Gray, and
F Chrétien, and
P Cesaro, and
J Chatelain, and
P Beaudry, and
J L Laplanche, and
J Mikol, and
J Bell, and
P Gambetti, and
J D Degos
January 2016,
Swiss medical weekly,
F Gray, and
F Chrétien, and
P Cesaro, and
J Chatelain, and
P Beaudry, and
J L Laplanche, and
J Mikol, and
J Bell, and
P Gambetti, and
J D Degos
January 1992,
Acta neuropathologica,
F Gray, and
F Chrétien, and
P Cesaro, and
J Chatelain, and
P Beaudry, and
J L Laplanche, and
J Mikol, and
J Bell, and
P Gambetti, and
J D Degos
November 2008,
Acta neuropathologica,
F Gray, and
F Chrétien, and
P Cesaro, and
J Chatelain, and
P Beaudry, and
J L Laplanche, and
J Mikol, and
J Bell, and
P Gambetti, and
J D Degos
July 1992,
Neurosurgery clinics of North America,
F Gray, and
F Chrétien, and
P Cesaro, and
J Chatelain, and
P Beaudry, and
J L Laplanche, and
J Mikol, and
J Bell, and
P Gambetti, and
J D Degos
July 2010,
Hirosaki igaku = Hirosaki medical journal,
F Gray, and
F Chrétien, and
P Cesaro, and
J Chatelain, and
P Beaudry, and
J L Laplanche, and
J Mikol, and
J Bell, and
P Gambetti, and
J D Degos
March 1988,
Journal of the neurological sciences,
F Gray, and
F Chrétien, and
P Cesaro, and
J Chatelain, and
P Beaudry, and
J L Laplanche, and
J Mikol, and
J Bell, and
P Gambetti, and
J D Degos
December 2009,
Journal of neurology, neurosurgery, and psychiatry,
