Cranial MRI and EEG Features in A Sporadic Creutzfeldt-Jakob Disease Case With Focal Involvement and Lateralization

Abstract

Scientific backround: Clinical picture of sporadic Creutzfeldt-Jakob Disease (s CJD) is composed of pyramidal, extrapyramidal or cerebellar findings in addition to rapidly progressive dementia and myoclonus. 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 myoclonus, 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 myoclonus 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.