An autopsied case of Creutzfeldt-Jakob disease with the lateral geniculate body lesion showing antagonizing correlation between periodic synchronous discharges and photically induced giant evoked responses.
We reported an necropsy finding of a patient with Creutzfeldt-Jakob disease (CJD) who showed photo-stimulated giant spikes that simultaneously suppressed periodic synchronous discharges (PSD) and the loss of pupillary light reflex during the course of the illness. The necropsy revealed extensive gray and white matter lesions, and both the lateral geniculate body (LGB) and pregeniculate body were primarily affected. The superior colliculus, optic nerve and tracts were not affected. The cerebral cortices particularly of the occipital lobe were severely damaged. The Gennari line, however, was spared from lesion. The primary involvement of the LGB has been reported infrequently in CJD, however, it appears to be associated with the unusual electroencephalograph (EEG) feature of the present case. The pregeniculate lesion contributed to the loss of pupillary reflex. This finding indicates that the visual pathway may be involved in the mechanism of the generation of PSD in CJD.
Aoki
T
T
Department of Neuropsychiatry, Kamazawa University School of Medicine, Japan.
Kobayashi
K
K
Jibiki
I
I
Muramori
F
F
Hayashi
M
M
Miyazu
K
K
Kobayashi
E
E
Koshino
Y
Y
Yamaguchi
N
N
D000200:Action Potentials; D000368:Aged; D007562:Creutzfeldt-Jakob Syndrome / Q000503:physiopathology*; D004569:Electroencephalography; D005074:Evoked Potentials, Visual / Q000502:physiology*; D005260:Female; D005829:Geniculate Bodies / Q000473:pathology*; D006801:Humans; D009778:Occipital Lobe / Q000473:pathology; D014795:Visual Pathways / Q000473:pathology*