We report the case of a 67-year-old woman living in Leon with no personal history of interest who reported a three-week history of progressive bilateral loss of visual acuity (VA).
The first exploration revealed a VA of 0.3 in right eye and 0.1 in left eye, with normal ocular tensions, biomicroscopy, ocular motility and funduscopic examination.
He presented alteration in the visual fields and slight alteration in the color test.
Magnetic resonance imaging (MRI) of the brain, visual evoked potentials (VEP) and electroretinogram (ERG) were performed, which were nonspecific.
During the following month, the patient presented behavior alteration, severe photoathetization, cognitive impairment, pseudo-synostic movements and myoclonus, with a rapidly progressive evolution, so a new MRI, individual electroencephalogram (EEG) was requested.
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EEG showed brain slowing and activity as triphasic, generalised seizures, and short periodicity waves.
MRI showed changes in the head of both caudal nuclei and upper portion of the lenticular nucleus on the right side.
SPECT showed bilateral and subcortical brain perfusion alterations in the right frontal and posterior temporal lobes of moderate intensity.
Lumbar puncture was performed with positive determination of 14-3-3 protein in cerebrospinal fluid (CSF).
In the following month there was a severe deterioration of the general state, to present akinetic mutism, generalized rigidity and myoclonus, producing the death two months after the beginning of the visual disorder.
In the autopsy study a massive neuronal loss and marked astrogliosis were observed in the areas with numerous cortical vacuums, sometimes confluent.
The suspicion of CJD was established for immunohistochemical study and the existence of PrP protein was demonstrated.
