This is a 73-year-old male patient from Santiago with no relevant eating habits.
She underwent colon cancer surgery (stage IIIC) in January 2014, others received three cycles of chemotherapy with oxaliplatin, last dose in July 2014.
The patient came to the Emergency Department of the Hospital del Salvador in August 2014, due to a progressive picture of approximately 7 months of evolution, which had started with insomnia due to a day of sleep plus 3 h.
The following months, the family members noticed it as aptuous, with lack of initiative, difficulty remembering new information and progressive gait instability until they stopped walking.
She began to present disorientation episodes and unresponsive episodes associated with complex visual disturbances.
Later he was unable to answer simply questions.
In his evaluation, at admission, a patient is objectified spontaneous vigil, disoriented in time and space, inattentive, with a non-fluent language, unaffected and without communicative intention.
The patient obeyed simple orders, and was able to follow his eyes for a short period of time.
The motor examination revealed increased muscle tone, spastic, with increased osteotendinous reflexes (TOR) alive, symmetrical and a bilateral indifferent plantar reflex.
The examination of cranial nerves, sensitive, and meningeal signs showed no abnormalities.
The general laboratory and CSF study was within the expected normal parameters.
Non-contrast brain computed tomography (CAT) was normal for age.
A rapidly progressive dementia syndrome v/s was considered as a non-syndromic diagnosis.
At the beginning of hospitalization she had an episode of tonic-clonic seizure, which was managed with benzodiazepines with good response.
The wakefulness electroencephalogram (EEG) showed an abnormal tracing, markedly asymmetric, characterized to the left by the continuous presence of trains of up to 4 theta waves of acute contours and occipital region.
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The possibility of being a non-convulsive status epilepticus was indicated as a loading dose of phenytoin.
The patient did not present clinical changes, so levetiracetam administration was added, again without response.
Nine electroencephalograms showed evolution of the anterior tracing towards a record characterized by the presence of periodic discharges lateralized to the left, composed of spike-wave complexes or double-point or triple-wave complexes.
Right-sided activity was essentially configured as triphasic wave morphology.
The following days the patient presented greater deterioration, being in a state of superficial murmur, not emitted language and only located painful stimuli.
Brain MRI showed increased diffuse signal at the neocortical level, with greater intensity in the left hemisphere and basal ganglia.
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Among laboratory tests performed in search of other possible causes of rapidly progressive dementia, the presence of positive anti-thyro peroxidase antibodies (anti-TPO) stood out.
This finding was treated with corticosteroids (methylprednisolone 1 g/day for 5 days) without improvement.
A new EEG was performed two weeks after the previous one, which showed a tracing characterized by the generalized presence of waves of triphasic morphology, with an approximate frequency of 1 cps, always of greater expression on the left hemisphere.
Occasional episodes of narrowing amplitude and some episodes with progressive increase in the frequency of repetition of complexes were observed.
Based on the clinical evolution and MRI findings, the diagnosis of probable CJD was proposed and anticonvulsant drugs were gradually withdrawn.
A biochemical study of 14-3-3 protein in CSF was performed by Western blot, measured at different stages of the clinical picture as previously performed14, showing a greater signal of this as the clinical deterioration progressed.
The patient developed akinetic mutism and myoclonus of the upper limbs.
After almost two months of hospitalization the patient died.
After consulting the family about the possibility of performing an autopsy, they decided not to perform this procedure.
