CREUTZFELDT-JAKOB DISEASE. REPORT OF ONE CASE

Desafíos en el diagnóstico de enfermedad de Creutzfeldt-Jakob. Caso.

Author
Ramírez, Marcos; Universidad de Chile

Gallardo, Andrés; Universidad de Chile

Vidal, Aarón; Intituto de Neurocirugía

Cornejo, Sebastián; Universidad de Chile

Ramírez, Darío; Hospital del Salvador

Medinas, Danilo; Universidad de Chile

Bustamante, Gonzalo; Hospital del Salvador

Pasquali, Renzo; Universidad de Chile

Hetz, Claudio; Universidad de Chile

Full text
http://www.revistamedicadechile.cl/ojs/index.php/rmedica/article/view/4023
Abstract
Creutzfeldt-Jakob disease has a higher incidence in Chile than in other countries. The postmortem pathological characterization of brain tissue is necessary to reach a definitive diagnosis. We report a 73 years old man with a history compatible with of a rapidly progressive dementia, in which the first electroencephalographic study showed a pattern consistent with non-convulsive status epilepticus. Besides discarding this diagnosis, it was necessary to rule out other causes of rapidly progressive dementia such as Hashimoto encephalopathy. Finally, the sustained clinical deterioration with no response to anticonvulsants and corticosteroids, the imaging studies, a serial electroencephalographic monitoring study and the detection of 14-3-3 protein in cerebrospinal fluid were the keys to achieve the diagnosis of the disease.
 
Creutzfeldt-Jakob disease has a higher incidence in Chile than in other countries. The postmortem pathological characterization of brain tissue is necessary to reach a definitive diagnosis. We report a 73 years old man with a history compatible with of a rapidly progressive dementia, in which the first electroencephalographic study showed a pattern consistent with non-convulsive status epilepticus. Besides discarding this diagnosis, it was necessary to rule out other causes of rapidly progressive dementia such as Hashimoto encephalopathy. Finally, the sustained clinical deterioration with no response to anticonvulsants and corticosteroids, the imaging studies, a serial electroencephalographic monitoring study and the detection of 14-3-3 protein in cerebrospinal fluid were the keys to achieve the diagnosis of the disease.
 
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