Psychiatric and neuroimaging findings in Creutzfeldt-Jakob disease

Springer Science and Business Media LLC - Tập 5 - Trang 43-46 - 2003
Jennifer L. Martindale, Michael D. Geschwind, Bruce L. Miller1
1Department of Neurology, University of California, San Francisco, San Francisco, USA

Tóm tắt

Although Creutzfeldt-Jakob disease (CJD) is the prototypical rapidly progressive dementia, clinical heterogeneity in the disease can make diagnosis difficult. Creutzfeldt-Jakob disease affects multiple brain areas, which causes multifocal deficits that involve movement, cognition, and psychiatric status. Thorough neurologic, cognitive, and psychiatric examinations are necessary for observing its clinical features. Recent advances in neuroimaging techniques have allowed researchers and clinicians to discover imaging patterns that distinguish CJD from other neurologic diseases. This article discusses how these advances may make neuroimaging the most valuable noninvasive tool for diagnosing CJD, which helps to track the progression of the disease course and provides insight into clinical-anatomic correlations.

Tài liệu tham khảo

Will RG: Infectious and sporadic prion diseases. In Prion Biology and Diseases. Edited by Prusiner SB. Cold Spring Harbor: Cold Spring Harbor Laboratory Press; 1999:465–507. Prusiner SB: Genetic and infectious prion diseases. Arch Neurol 1993, 50:1129–1153. Will RG: Descriptive epidemiology of Creutzfeldt-Jakob disease (CJD) in six European countries, 1993–1995. EU Collaborative Study Group for CJD. Ann Neurol 1998, 43:763–767. Schroter A: Magnetic resonance imaging in the clinical diagnosis of Creutzfeldt Jakob disease. Arch Neurol 2000, 57:1751–1757. Finkenstaedt M: MR imaging of Creutzfeldt-Jakob disease. Radiology 1996, 199:793–798. Vrancken AF, Frijns CJ, Ramos LM: FLAIR MRI in sporadic Creutzfeldt-Jakob disease. Neurology 2000, 55:147–148. This case report highlights the use of FLAIR MRI in demonstrating signal intensity changes of the cerebral cortex in CJD. Fluid-attenuated inversion recovery imaging has played a critical role in showing the conspicuity of this "cortical ribboning" effect observed in CJD. Demaerel P: Diffusion-weighted MRI in sporadic Creutzfeldt-Jakob disease. Neurology 1999, 52:205–208. Bahn MM, Parchi P: Abnormal diffusion-weighted magnetic resonance images in Creutzfeldt-Jakob disease. Arch Neurol 1999, 56:577–583. Yee AS: Diffusion-weighted MRI of right-hemisphere dysfunction in Creutzfeldt-Jakob disease. Neurology 1999, 52:1514–1515. Kim HC: Diffusion-weighted MR imaging in biopsy-proven Creutzfeldt-Jakob disease. Korean J Radiol 2001, 2:192–196. This is one of the first publications to suggest the increased sensitivity of diffusion-wieghted MRI in showing classic changes in CJD. Kim suggests that DWI MRI allows for better detection of lesions in both the basal ganglia and cerebral cortices than FLAIR and T2 sequences. Matoba M: Creutzfeldt-Jakob disease: serial changes on diffusion-weighted MRI. J Comput Assist Tomogr 2001, 25:274–277. To date, few studies have analyzed serial MRI in patients with CJD, largely because of the rapid course of the disease. Matoba describes serial changes on DWI MRI in a patient with CJD, and proposes DWI MRI as a useful tool for tracking the progression of CJD through its clinical course. Murata T: Conspicuity and evolution of lesions in Creutzfeldt-Jakob disease at diffusion-weighted imaging. AJNR Am J Neuroradiol 2002, 23:1164–1172. The diagnostic use in DWI MRI has been suggested in the form of case reports. This is one of the first studies to look at a cohort of patients (n=13) to assess the sensitivity and serial changes shown in apparent diffusion coefficient maps. Haik S: Prion protein deposits match magnetic resonance imaging signal abnormalities in Creutzfeldt-Jakob disease. Ann Neurol 2002, 51:797–799. Haik describes two cases—one sporadic and one variant CJD—where FLAIR and DWI MRI sequences had been performed and semiquantitative analysis of gliosis and spongiform change, as well as immunohistochemistry of prion protein and glial markers, had been conducted at autopsy. This study describes a strong correlation of prion protein deposit location with the location of MRI signal abnormalities in the setting of an ongoing debate over the underlying pathology, which correlates with diffusion abnormalities on MRI. Zeidler M: The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet 2000, 355:1412–1418. Levy SR: Early evolution and incidence of electroencephalographic abnormalities in Creutzfeldt-Jakob disease. J Clin Neurophysiol 1986, 3:1–21. Steinhoff BJ: Accuracy and reliability of periodic sharp wave complexes in Creutzfeldt-Jakob disease. Arch Neurol 1996, 53:162–166. Zochodne DW: Creutzfeldt-Jakob disease without periodic sharp wave complexes: a clinical, electroencephalographic, and pathologic study. Neurology 1988, 38:1056–1060. Bortone E: Reliability of EEG in the diagnosis of Creutzfeldt-Jakob disease. Electroencephalogr Clin Neurophysiol 1994, 90:323–330.