Sporadic Creutzfeldt-Jacob Disease: Analysis of 16 Patients

Key Findings & Data:This study is a review of clinical findings and diagnostic procedures used for 16 patients diagnosed with sporadic CJD (sCJD) at Ankara University School of Medicine.

💡

Conclusion & Impact:Conclusion: It is important to consider diagnosis of sCJD in patients with rapidly progressive dementia.

🔬Design:Review & Systematic Survey

📍Scope:2015 Verileri

⚙️Variables:Domain Parameters

Abstract

Objectives: Creutzfeldt-Jacob Disease (CJD) is a rare neurodegenerative disorder. This study is a review of clinical findings and diagnostic procedures used for 16 patients diagnosed with sporadic CJD (sCJD) at Ankara University School of Medicine. Methods: Medical records of 16 patients who were diagnosed with sCJD between January 1990 and January 2015 were analyzed. Clinical features, periodic sharp and slow wave complexes (PSSW) in electroencephalography (EEG), finding of 14-3-3 protein in cerebrospinal fluid (CSF) and brain magnetic resonance imaging (MRI) of all patients were assessed. Results: Study included 16 patients with sCJD: 6 females (37.5%) and 10 males (62.5%), with mean age 64±9.5 years. All patients had dementia and myoclonus; 14 patients had extrapyramidal or pyramidal signs, 13 had cerebellar (n=10) and/or visual signs (n=6), and 10 patients had akinetic mutism. PSSW were present in EEG of 13 patients. Brain MRI of 13 patients showed abnormalities and 14-3-3 protein was detected in CSF of 6 of 7 patients. Within an average period of 3 months from the onset of signs, 9 hospitalized patients died. Conclusion: It is important to consider diagnosis of sCJD in patients with rapidly progressive dementia.

Özet

Amaç: Creutzfeldt–Jakob Hastalığı (CJH), demans ve nöropsikiyatrik bulguların görüldüğü, hızlı seyirli nadir bir nörodejeneratif hastalıktır. Bu yazıda, sporadik CJH (sCJH) tanısının klinik bulgu ve tanı yöntemleri gözden geçirilerek, Ankara Üniversitesi Tıp Fakültesi kliniğinde izlenmiş 16 olgu sunuldu. Gereç ve Yöntem: Bu çalışmada Ocak 1990–Ocak 2015 yılları arasında tanı konarak izlenmiş olan 16 sCJH olgusu geriye dönük olarak incelendi. Tüm hastaların yaş ve cinsiyetleri, demans ve/veya miyoklonus varlığı, piramidal, ekstrapiramidal, serebellar, görsel, akinetik mutizm açısından klinik özellikleri, elektroensefalografide (EEG) periyodik keskin dalga kompleksleri (PKDK) varlığı, BOS’ta 14.3.3 proteini varlığı ve kraniyal manyetik rezonans görüntüleme (MRG) bulguları kaydedildi. Bulgular: Çalışmaya 16 sCJH (6 [%37.5] kadın, 10 [%62.5] erkek, ortalama yaş 64±9.5 yıl) alındı. Hastaların hepsinde bunama belirtileri ve miyoklonus, 14’ünde ekstrapiramidal veya piramidal belirtiler, 13’ünde serebellar (n=10) ve/veya görsel belirtiler (n=6), 10’unda ise akinetik mutizm bulundu. Hastaların 13’ünün EEG’sinde PKDK, 13’ünün MRG’sinde sinyal artışı gösteren anormallikler vardı. Beyin omurilik sıvısı (BOS) incelemesi yapılan 7 hastanın 6’sında BOS’ta 14-3-3 proteini saptandı. Hastanede yatan 9 hasta, belirtilerin başlangıcından itibaren ortalama 3 ay (1–6 ay) içinde kaybedildi. Sonuç: Hızlı ilerleyen demans olgularında CJH tanısının akla gelmesi ve buna yönelik incelemelerin yapılması tanının konmasında önemlidir

🔖

Cite This Article

AYKAÇ, Ö., POLAT, B. S. A., ERDOĞAN, Ş., SORGUN, M. H., DELEN, F., TUNCAY, E., YİĞİT, A. (2016). Sporadic Creutzfeldt-Jacob Disease: Analysis of 16 Patients. Epilepsi, Cilt: 22 - Sayı: 2, 46–50. https://ajindex.com/articles/160708

📄 Full Text PDF Viewer

Open in New Tab ↗

↗New Tab

📄

PDF görüntüleyici hazırlanıyor...

References (14)

Brandel JP, Delasnerie-Lauprêtre N, Laplanche JL, Hauw JJ, Alpérovitch A. Diagnosis of Creutzfeldt-Jakob disease: effect of clinical criteria on incidence estimates. Neurology 2000;54(5):1095–9.
Gambetti P, Kong Q, Zou W, Parchi P, Chen SG. Sporadic and familial CJD: classification and characterisation. Br Med Bull 2003;66:213–39.
Jubelt B, Miller JR. Viral infections in Rowland LP: Merrit’s Textbook of Neurology. Williams and Wilkins 1995;142–79. Johnson RT. Prion diseases. Lancet Neurol 2005;4(10):635–42. Bosque P. Molecular types of Creutzfeldt-Jakob disease: the strange diversity of prions. Neurology 2005;65(10):1520–1.
Collins SJ, Lawson VA, Masters CL. Transmissible spongiform encephalopathies. Lancet 2004;363(9402):51–61.
Van Everbroeck B, Pals P, Dziedzic T, Dom R, Godfraind C, Sciot R, et al. Retrospective study of Creutzfeldt-Jakob disease in Belgium: neuropathological findings. Acta Neuropathol 2000;99(4):358-64.
Glatzel M, Stoeck K, Seeger H, Lührs T, Aguzzi A. Human prion diseases: molecular and clinical aspects. Arch Neurol 2005;62(4):545–52. Collinge J. Prion diseases of humans and animals: their causes and molecular basis. Annu Rev Neurosci 2001;24:519–50.
Steinhoff BJ, Räcker S, Herrendorf G, Poser S, Grosche S, Zerr I, et al. Accuracy and reliability of periodic sharp wave complexes in Creutzfeldt-Jakob disease. Arch Neurol 1996;53(2):162–6.
Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemann U, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 2009;132(Pt 10):2659–68.
Yiğit A, Şener Ö, Avunduk M, Çağlar İ, Bacacı K. CreutzfeldtJakob hastalığı: Vaka takdimi. Nöroloji 1992;19:101–4.
Yiğit A, Demirci S, Mutluer N. Creutzfeldt-Jakob hastalığı: İki vaka takdimi ve Türkçe literatürün gözden geçirilmesi. Türk Nörol Derg 1995;1:91–4.
Paterson RW, Torres-Chae CC, Kuo AL, Ando T, Nguyen EA, Wong K, et al. Differential diagnosis of Jakob-Creutzfeldt disease. Arch Neurol 2012;69(12):1578–82.
Shiga Y, Miyazawa K, Sato S, Fukushima R, Shibuya S, Sato Y, et al. Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology 2004;63(3):443–9.
Young GS, Geschwind MD, Fischbein NJ, Martindale JL, Henry RG, Liu S, et al. Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis. AJNR Am J Neuroradiol 2005;26(6):1551–62.
Ukisu R, Kushihashi T, Kitanosono T, Fujisawa H, Takenak