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.

Keywords

• Neurodegenerative disease,Creutzfeldt-Jakob disease,Rehabilitation

Rehabilitasyon sırasında hızlı ilerleyen tetrapleji ve bilişsel bozulma: Bir nörodejeneratif hastalık olgusu

Öz

İnsan prion hastalıkları, prionlar olarak adlandırılan nörolitik patojen proteinlerin neden olduğu ilerleyici nörodejeneratif hastalıklardır. En yaygın insan prion hastalığı sporadik Creutzfeldt-Jakob hastalığı olup, yıllık prevalansı yaklaşık milyonda 0.5-1'dir. Semptomlar ve bulgular; hızla ilerleyen demans, ataksi, miyoklonik nöbetler, akinetik mutizm ve diğer nörolojik ve nörodavranışsal bozuklukları içerir. Creutzfeldt-Jakob hastalığının klinik spektrumu oldukça değişkendir, bu nedenle premortem teşhis etmek zor olabilir. Bu makalede, başlangıçta yürüme zorluğu ve denge bozukluğu ile başvuran 78 yaşında bir kadın hasta anlatılmaktadır. Yapılan değerlendirme sonucunda hasta servikal spinal stenoz tanısı ile rehabilitasyon kliniğine transfer edildi. Hastanede yatışı sırasında, yürüme ve dengesi progresif olarak kötüye giderek bozuldu. İleri tetkiklerden sonra hastanın olası sporadik Creutzfeldt-Jakob hastalığı olduğu düşünülmüştür.

Anahtar Kelimeler

• Nörodejeneratif hastalık,Creutzfeldt-Jakob hastalığı,Rehabilitasyon

Kaynakça

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