Hayriye KÜÇÜKOĞLU, Yavuz ALTUNKAYNAK, Belgin MUTLUAY, Nurhak DEMİR, Vedat ÖZMEN, Ayten DİRİCAN, Sevim BAYBAŞ, Musa ÖZTÜRK

Sporadik Creutzfeldt-jacob hastalığı: 5 olgu sunumu

Sporadic Creutzfeldt-Jakob Hastalığı (CJH), 50-70 yaş arasında görülen demansla ilişkili süreçlerde akla getirilmesi gereken hastalıklardan biridir. Klinik özellikleri, tipik elektroensefalografi (EEG) bulguları, laboratuar ve radyolojik görüntüleme özellikleri ile demansla giden diğer hastalıklardan ayırt edilebilir. Bu çalışmada klinik, EEG, laboratuar ve radyolojik özellikleri ile birlikte beş sporadic CJH olgumuzu sunmayı amaçladık. Sporadic CJH tanısı ile kliniğimizde takip edilmiş olan beş olgu değerlendirildi. Yaş ortalaması 63,8 olan hastaların ikisi kadın, üçü erkekti. Bütün hastalarda EEG kayıtları yapıldı. Beyin görüntüleme, bir hastada bilgisayarlı tomografi (BT), dört hastada manyetik rezonans görüntüleme (MRG) ile değerlendirildi. Üç hastada, beyin omurilik sıvısında (BOS) 14.3.3 protein, iki hastada nöron spesifik enolaz (NSE) bakıldı. Beyin biyopsisi bir hastada yapılabildi ve kesin CJH tanısı konuldu. Diğer olgularda biyopsi yapılamadığı için muhtemel sporadic CJH tanısı klinik özellikler, EEG bulguları, radyolojik ve laboratuar özellikleri ile konuldu. Demans bulguları olan hastaların dördünde EEG'de periyodik keskin ve yavaş dalga aktivitesi saptandı. İlk EEG kayıtları nonspesifik iken, tekrarlanan EEG kayıtlarında özgün bulgular saptandı. 14.3.3 proteini bakılan üç hastada da sonuç pozitif idi. Bazal gangliyonlarda hiperintensite beyin MRG tetkiki yapılan, dört hastadan birinde mevcuttu. Klinik özellikleri ile sporadic CJH düşünülen hastalarda, tekrarlayan EEG kayıtları yapmak tanıda yararlıdır.

Sporadic Creutzfeldt-jacob disease: Report of five cases

Sporadic Creutzfeldt-Jacob Disease (CJD) should be considered in the differential diagnosis of demential processes presenting between age of 50-70. This entity can be differentiated from other diseases with dementia using clinical features, typical electroencephalo- graphy (EEG) recordings, laboratory and radiological findings. We present 5 patients diagnosed as sporadic CJD with clinical, EEG, laboratory and radiological findings. We evaluated five patients, (3 females, 2 males, with a mean age of 63.8), diagnosed as sporadic CJD. All patients had multiple EEG recordings, 4 patients had magnetic resonance imaging (MRI) of the brain, 1 had cranial computaized tomography (CT). Cerebrospinal fluid (CSF) levels of 14.3.3 protein was assessed in 3 patients, neuron specific enolase (NSE) in 2 patients. One case had postmortem brain biopsy evaluation and diagnosed as definite CJD. The EEG recordings revealed periodic spike and slow wave activity in four patients with dementia. The findings in the initial EEG recordings were nonspecific, however, repeated recordings showed typical EEG findings. Protein 14.3.3 was positive in all three patients. Basal ganglia hyperintensity was seen in only one patient on the MRI of the brain. The patients with suspected CJD, should be followed-up with consecutive EEG recordings to support the diagnosis.

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