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

Yıl 2019, Cilt: 3 Sayı: 1, 100 - 102, 27.01.2019

Sevgi İkbali Afşar Oya Umit Yemişçi

https://doi.org/10.28982/josam.454181

Öz

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.

Anahtar Kelimeler

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

• 1. Laverse E, Shah S, Mavra M. Sporadic Creutzfeldt-Jakob disease: early signs and pre-mortem diagnosis. BMJ Case Reports. 2009;2009:bcr07.2009.2105. doi:10.1136/bcr.07.2009.2105.
• 2. Johnson RT, Gibbs CJ Jr. Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies. N Engl J Med. 1998;339(27):1994-2004. Doi: 10.1056/NEJM199812313392707.
• 3. Gialanella B, Prometti P, Ferlucci C, Bertolinelli M. A case of very rapid progressive ataxia in rehabilitation setting. Aging Clin Exp Res. 2012;24(2):197-9.
• 4. Bekiesińska-Figatowska M, Kuczyńska-Zardzewiały A, Pomianowska B, Kajdana K, Szpak GM, Iwanowska B, et al. The value of magnetic resonance imaging in the early diagnosis of Creutzfeldt-Jakob disease - own experience. Pol J Radiol. 2012;77(1):63-7.
• 5. Lyytinen J, Sairanen T, Valanne L, Salmi T, Paetau A, Pekkonen E. Progressive Stroke-Like Symptoms in a Patient with Sporadic Creutzfeldt-Jakob Disease. Case Rep Neurol. 2010;2(1):12-8.
• 6. World Health Organization. Global surveillance, diagnosis and therapy of human transmissible spongiform encephalopathies: Report of a WHO consultation; Geneva, Switzerland. 9–11 February 1998; Geneva, Switzerland: World Health Organization; 1998.
• 7. Grant D, Loomis S. Progressive neurodegenerative disease in presumed spinal cord injury: case report of a patient with prion disease. J Spinal Cord Med. 2003;26(3):259-61.
• 8. Vitali P, Maccagnano E, Caverzasi E, Henry RG, Haman A, Torres-Chae C, et al. Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology. 2011;76(20):1711-9.
• 9. Shea YF, Chan TC, Chang RS, Hon FK, Kwan JS, Chu LW. Rapidly progressive ataxia during rehabilitation and a difficult road to diagnosis. J Am Geriatr Soc. 2014;62(8):1609-10.