OBJECTIVE: Sporadic Jakob-Creutzfeldt disease (sCJD) and dementia with Lewy bodies (DLB) have overlapping clinical symptoms that can lead to their misdiagnosis. We delineated the clinical overlap between sCJD and DLB, and assessed the value of magnetic resonance imaging (MRI) to differentiate between them. METHODS: Medical records, MRI, electroencephalogram (EEG) and cerebrospinal fluid (CSF) were reviewed from 56 sCJD and 30 DLB subjects. RESULTS: 46% of sCJD subjects met probable DLB criteria and 40% of DLB subjects met probable CJD criteria. A greater proportion of sCJD subjects had cerebellar signs (66% vs. 10%, p<0.001), myoclonus (64% vs. 30%, p=0.002), and visual symptoms (other than hallucinations) (61% vs. 7%, p<0.001), whereas more DLB subjects had hallucinations (70% vs. 39%, p=0.007) and fluctuations (57% vs. 23%, p=0.002). Cortical and/or basal ganglia MRI diffusion weighted imaging hyperintensities consistent with sCJD were seen in 96% of sCJD subjects but in none with DLB. Logistic regression in sCJD revealed that those meeting probable DLB criteria were more likely to have occipital lobe involvement on MRI (OR 1.4, p=0.058, model p=0.022). Parietal lobe involvement on MRI was a predictor of "Other Focal Cortical signs" (OR 1.9, p=0.021). EEG and CSF assessments lacked sensitivity for sCJD as 48% of sCJD patients had a negative EEG; 67% of the 36 sCJD patents with a CSF evaluation had a negative or inconclusive 14-3-3 result. Too few DLB patients had EEG or CSF to assess their utility. CONCLUSION: Sporadic CJD and DLB have significant symptom overlap. MRI helps differentiate these diseases and is related to the signs/symptoms observed in sCJD.
OBJECTIVE: Sporadic Jakob-Creutzfeldt disease (sCJD) and dementia with Lewy bodies (DLB) have overlapping clinical symptoms that can lead to their misdiagnosis. We delineated the clinical overlap between sCJD and DLB, and assessed the value of magnetic resonance imaging (MRI) to differentiate between them. METHODS: Medical records, MRI, electroencephalogram (EEG) and cerebrospinal fluid (CSF) were reviewed from 56 sCJD and 30 DLB subjects. RESULTS: 46% of sCJD subjects met probable DLB criteria and 40% of DLB subjects met probable CJD criteria. A greater proportion of sCJD subjects had cerebellar signs (66% vs. 10%, p<0.001), myoclonus (64% vs. 30%, p=0.002), and visual symptoms (other than hallucinations) (61% vs. 7%, p<0.001), whereas more DLB subjects had hallucinations (70% vs. 39%, p=0.007) and fluctuations (57% vs. 23%, p=0.002). Cortical and/or basal ganglia MRI diffusion weighted imaging hyperintensities consistent with sCJD were seen in 96% of sCJD subjects but in none with DLB. Logistic regression in sCJD revealed that those meeting probable DLB criteria were more likely to have occipital lobe involvement on MRI (OR 1.4, p=0.058, model p=0.022). Parietal lobe involvement on MRI was a predictor of "Other Focal Cortical signs" (OR 1.9, p=0.021). EEG and CSF assessments lacked sensitivity for sCJD as 48% of sCJD patients had a negative EEG; 67% of the 36 sCJD patents with a CSF evaluation had a negative or inconclusive 14-3-3 result. Too few DLB patients had EEG or CSF to assess their utility. CONCLUSION: Sporadic CJD and DLB have significant symptom overlap. MRI helps differentiate these diseases and is related to the signs/symptoms observed in sCJD.
Authors: I Zerr; M Pocchiari; S Collins; J P Brandel; J de Pedro Cuesta; R S Knight; H Bernheimer; F Cardone; N Delasnerie-Lauprêtre; N Cuadrado Corrales; A Ladogana; M Bodemer; A Fletcher; T Awan; A Ruiz Bremón; H Budka; J L Laplanche; R G Will; S Poser Journal: Neurology Date: 2000-09-26 Impact factor: 9.910
Authors: Carles Gaig; Francesc Valldeoriola; Ellen Gelpi; Mario Ezquerra; Sara Llufriu; Mariateresa Buongiorno; Maria Jesús Rey; Maria Jose Martí; Francesc Graus; Eduardo Tolosa Journal: Mov Disord Date: 2011-04-11 Impact factor: 10.338
Authors: H J Tschampa; M Neumann; I Zerr; K Henkel; A Schröter; W J Schulz-Schaeffer; B J Steinhoff; H A Kretzschmar; S Poser Journal: J Neurol Neurosurg Psychiatry Date: 2001-07 Impact factor: 10.154
Authors: S Haïk; J P Brandel; V Sazdovitch; N Delasnerie-Lauprêtre; K Peoc'h; J L Laplanche; N Privat; C Duyckaerts; J L Kemeny; N Kopp; A Laquerrière; M Mohr; J P Deslys; D Dormont; J J Hauw Journal: Neurology Date: 2000-11-14 Impact factor: 9.910
Authors: Michael D Geschwind; Jennifer Martindale; Deborah Miller; Stephen J DeArmond; Jane Uyehara-Lock; David Gaskin; Joel H Kramer; Nicholas M Barbaro; Bruce L Miller Journal: Arch Neurol Date: 2003-06
Authors: Ross W Paterson; Charles C Torres-Chae; Amy L Kuo; Tim Ando; Elizabeth A Nguyen; Katherine Wong; Stephen J DeArmond; Aissa Haman; Paul Garcia; David Y Johnson; Bruce L Miller; Michael D Geschwind Journal: Arch Neurol Date: 2012-12
Authors: Kyan Younes; Julio C Rojas; Amy Wolf; Goh M Sheng-Yang; Matteo Paoletti; Gianina Toller; Eduardo Caverzasi; Maria Luisa Mandelli; Ignacio Illán-Gala; Joel H Kramer; Yann Cobigo; Bruce L Miller; Howard J Rosen; Michael D Geschwind Journal: Ann Clin Transl Neurol Date: 2021-05-05 Impact factor: 4.511