Rapidly progressive tetraplegia and cognitive deterioration during rehabilitation: A case of neurodegenerative disease

Abstract

Human prion diseases are fatal, progressive neurodegenerative disorders caused by neurolytic pathogen proteins, called prions. The most common human prion disease is sporadic Creutzfeldt-Jakob disease, with an approximate annual prevalence of 0.5-1 per million. The symptoms and signs include rapidly progressive dementia, ataxia, myoclonic seizures, akinetic mutism and other neurological and neurobehavioral disorders. The clinical spectrum of Creutzfeldt-Jakob disease is highly variable; therefore it can be difficult to diagnose premortem. This article describes a 78-year-old woman who initially presented with difficulty walking and balance disorder. As a result of the evaluation, the patient was transferred to rehabilitation clinic, with a diagnosis of cervical spinal stenosis. During hospitalization, she showed progressive decline in gait and balance and deteriorated rapidly. The patient was considered to be probable sporadic Creutzfeldt-Jakob disease after further investigations.