Fokal tutulum ve lateralizasyon gösteren bir sporadik Creutzfeldt-Jakob hastalığı olgusunda kranial MRG ve EEG özellikleri
Küçük Resim Yok
Tarih
2007
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Dergi Başlığı
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Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Bilimsel zemin: Sporadik Creutzfeldt-Jakob Hastalığı (s CJH)'nda klinik görünüm, hızlı ilerleyen demans ve yaygın myoklonilere ek olarak gelişen piramidal, ekstrapiramidal veya serebellar bulgular biçiminde şekillenir. Fokal tutulum ve lateralizasyon sık rastlanan bir klinik tablo değildir. Böyle olgularda fokal ve lateralizan bulguların radyolojik ve elektrofizyolojik olarak da saptanması ve sebat etmesi, klinisyenin yapısal bir lezyon araştırmasına ve tanıda gecikmeye neden olabilir. Amaç: Bu yazıda bir olgudan yola çıkarak s CJH'nin sebep olabildiği değişik klinik tabloların ve bunlar arasında da fokal tutulum ve lateralizasyon gösteren olguların varlığına dikkat çekmek ve noninvaziv, güvenilir birer tanı aracı olarak kranial MRG ve serial EEG çekimlerinin önemine vurgu yapmak amaçlanmıştır. Olgu: Bu olgu sunumunda sağ üst ekstremitede myoklonus, dengesizlik ve unutkanlık yakınmaları ile başvuran 48 yaşında bir erkek olgu bildirilmiştir. Kranial MRG ile FLAIR ve difüzyon ağırlıklı görüntülerde sol hemisfere lokalize kortikal ve subkortikal hiperintensiteler ve EEG'de sola lokalize periyodik keskin dalga deşarjları saptanmış, myoklonilerin yaygın hale gelmesi ve klinik tablonun kötüleşmesi ile beraber tekrarlanan tetkiklerde, bu lateralizan özelliğin ortadan kalktığı görülmüştür. Yorum: Kesin s CJH tanısı otopsi ile konur. Hastalığın klinik görünüm yelpazesi geniştir ve bazı olgular fokal beyin hasarını işaret eden lateralizan özellikler gösterebilirler. Bu olgularda serial EEG kayıtları, FLAIR ve difüzyon MRG gibi görüntüleme tetkiklerinin kullanılması hastalığın tanısı açısından önem taşımaktadır.
Scientific background: Clinical picture of sporadic Creutzfeldt-Jakob Disease (s CJD) is composed of pyramidal, extrapyramidal or cerebellar findings in addition to rapidly progressive dementia and myodonus. Focal involvement and lateralization are not common. Radiologic and electrophysiologic detection of prolonged or resistant focal and lateralizing findings may cause the clinician search for a structural lesion and this might result as a delay of the diagnosis. Objective: The importance of EEG recordings, FLAIR and diffusion MRI are emphasized in the medical literature in order to detect the atypical cases. Reminding the s CJD cases with focal involvement and lateralizing findings and emphasizing the importance of cranial MRI, serial EEG as non invazive and reliable diagnostic tools are aimed by this case presentation. Keywords: Sporadic Creutzfeldt-Cranial MRI and EEG Features in A Sporadic Creutzfeldt-Jakob Disease Case With Focal Involvement and Lateralization Scientific background: Clinical picture of sporadic Creutzfeldt-Jakob Disease (s CJD) is composed of pyramidal, extrapyramidal or cerebellar findings in addition to rapidly progressive dementia and myodonus. Focal involvement and lateralization are not common. Radiologic and electrophysiologic detection of prolonged or resistant focal and lateralizing findings may cause the clinician search for a structural lesion and this might result as a delay of the diagnosis. Objective: The importance of EEG recordings, FLAIR and diffusion MRI are emphasized in the medical literature in order to detect the atypical cases. Reminding the s CJD cases with focal involvement and lateralizing findings and emphasizing the importance of cranial MRI, serial EEG as non invazive and reliable diagnostic tools are aimed by this case presentation. Case: A 48 years old male patient reffered to our hospital with upper right myodonus, ataxia and forgetfulness. Cranial MRI, FLAIR and diffusion weighted images revealed cortical and subcortical hyperintensities in left hemisphere. Periodic sharp wave complexes (PSWC) localized to left hemisphere were detected by EEG. After generalizing of myodonus and worsening of clinical course, lateralizing findings have vanished. Conclusion: Autopsy is essential for the accurate diagnose of s CJD. The clinical spectrum of disease is comparatively extensive and some cases presented with focal or lateralized clinical findings which are conceived focal cerebral involvement. Serial EEG, FLAIR and. diffusion weighted cranial MRI play an important role in the clinical setting of CJD, contributing to formulation of an early diagnosis, especially in cases with this unusual clinical presentation.
Scientific background: Clinical picture of sporadic Creutzfeldt-Jakob Disease (s CJD) is composed of pyramidal, extrapyramidal or cerebellar findings in addition to rapidly progressive dementia and myodonus. Focal involvement and lateralization are not common. Radiologic and electrophysiologic detection of prolonged or resistant focal and lateralizing findings may cause the clinician search for a structural lesion and this might result as a delay of the diagnosis. Objective: The importance of EEG recordings, FLAIR and diffusion MRI are emphasized in the medical literature in order to detect the atypical cases. Reminding the s CJD cases with focal involvement and lateralizing findings and emphasizing the importance of cranial MRI, serial EEG as non invazive and reliable diagnostic tools are aimed by this case presentation. Keywords: Sporadic Creutzfeldt-Cranial MRI and EEG Features in A Sporadic Creutzfeldt-Jakob Disease Case With Focal Involvement and Lateralization Scientific background: Clinical picture of sporadic Creutzfeldt-Jakob Disease (s CJD) is composed of pyramidal, extrapyramidal or cerebellar findings in addition to rapidly progressive dementia and myodonus. Focal involvement and lateralization are not common. Radiologic and electrophysiologic detection of prolonged or resistant focal and lateralizing findings may cause the clinician search for a structural lesion and this might result as a delay of the diagnosis. Objective: The importance of EEG recordings, FLAIR and diffusion MRI are emphasized in the medical literature in order to detect the atypical cases. Reminding the s CJD cases with focal involvement and lateralizing findings and emphasizing the importance of cranial MRI, serial EEG as non invazive and reliable diagnostic tools are aimed by this case presentation. Case: A 48 years old male patient reffered to our hospital with upper right myodonus, ataxia and forgetfulness. Cranial MRI, FLAIR and diffusion weighted images revealed cortical and subcortical hyperintensities in left hemisphere. Periodic sharp wave complexes (PSWC) localized to left hemisphere were detected by EEG. After generalizing of myodonus and worsening of clinical course, lateralizing findings have vanished. Conclusion: Autopsy is essential for the accurate diagnose of s CJD. The clinical spectrum of disease is comparatively extensive and some cases presented with focal or lateralized clinical findings which are conceived focal cerebral involvement. Serial EEG, FLAIR and. diffusion weighted cranial MRI play an important role in the clinical setting of CJD, contributing to formulation of an early diagnosis, especially in cases with this unusual clinical presentation.
Açıklama
Anahtar Kelimeler
Nörolojik Bilimler
Kaynak
Türk Nöroloji Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
13
Sayı
3
