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Effrosyni Koutsouraki

Effrosyni Koutsouraki

Aristotle University, Greece

Title: The probable role of copper in the pathogenesis of spongiform encephalopathy: Case report with a co morbidity of Wilson and creutzfeldt-jakob disease


Biography: Effrosyni Koutsouraki


Copper is associated with the endogenous PrPC and PrPSc contributing to the pathogenesis of spongiform encephalopathy (CJD). A 44-year-old male with a history of inherited wilson's disease (hepatolenticular degeneration), mild liver injury for 12 years and bipolar psychosis during the last months, was admitted in our department due to speech and cognitive disturbances. Upon his admission, he had motor aphasia and psychomotor retardation without any other neurological signs and with Glasgow Coma Scale score 13/15. Laboratory examination including liver enzymes, copper and serum ammonia was normal. The brain MRI showed increased T2 signal in the caudate nuclei attributed to copper deposition in the context of Wilson's disease. In the electroencephalogram, periodic sharp discharges were found initially unilateral and then generalized.

His clinical condition was deteriorated rapidly presenting mutismus, myoclonic jerks, deterioration of consciousness, and within a few days the patient presented myoclonic status epilepticus without response to benzodiazepines or other antiepileptic treatment. He passed away a few days later.

The clinical picture, the positive 14-3-3 protein in the CSF and the new brain MRI that demonstrated elevated DWI signal not only in basal ganglia but also in brain cortex (cortical ribbon sign) made the diagnosis of CJD possible. The detection of PrPSc in CSF using the RT-QuIC method, which has 99.4 -100% specificity for CJD, made the diagnosis of CJD definite. This is the first reference to the Wilson and Creutzfeld-Jakob disease co-morbidity in case literature, which suggests the possible association of copper with the pathogenesis of CJD.