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Table 1 Demographics, clinical data, and diagnostic findings at the time of diagnostic assessment

From: Clinical, neuropathological, and molecular characteristics of rapidly progressive dementia with Lewy bodies: a distinct clinicopathological entity?

 

rpDLB

CJD

rpAD

P-value

Demographics

Female – N/N (%)

8/22 (36.3)

49/100 (49.0)

16/31 (51.6)

0.499

Age at onset – years (IQR)

73.0 (70.5–77.0)

67.5 (62.0-75.3)

76.5 (69.0-80.5)

< 0.001

Disease duration – months (IQR)

7.5 (2.0–21.0)

3.5 (2.0-6.4)

8.0 (3.0–12.0)

0.033

Clinical characteristics - N/N (%)

RPD

22/22 (100)

98/100 (98.0)

31/31 (100)

0.584

Fluctuations

8/22 (36.3)

12/100 (12.0)

5/31 (16.1)

0.020

Visual hallucinations

7/22 (31.8)

12/100 (12.0)

11/31 (35.4)

0.005

Parkinsonism

11/22 (50.0)

33/100 (33.0)

13/31 (41.9)

0.273

RBD

1/22 (4.5)

2/100 (2.0)

0/31 (0)

0.462

Myoclonus

9/22 (40.9)

53/100 (53.0)

17/31 (54.8)

0.545

Cerebellar signs

0/22 (0)

66/100 (66.0)

4/31 (12.9)

< 0.001

Visual disturbances

0/22 (0)

26/100 (26.0)

3/31 (9.6)

0.003

Pyramidal signs

3/22 (13.6)

22/100 (22.0)

11/31 (35.4)

0.150

Akinetic mutism

5/22 (22.7)

28/100 (28.0)

15/31 (48.3)

0.065

Neuropsychiatric symptoms

17/22 (77.2)

45/100 (45.0)

18/31 (58.0)

0.018

Delirium onset

10/22 (45.4)

8/100 (8.0)

4/31 (12.9)

< 0.001

Seizures

4/22 (18.1)

5/100 (5.0)

11/31 (35.4)

< 0.001

Diagnostic findings suggestive of CJD - N/N (%)

EEG - PSWCs

4/19 (21.0)

39/93 (41.9)

10/28 (35.7)

0.224

Positive brain MRI

0/13 (0)

67/81 (82.7)

0/17 (0)

0.006

CSF 14-3-3 (> 23400 AU/ml)

1/12 (8.3)

85/100 (85.0)

6/18 (33.3)

< 0.001

CSF t-tau (> 1250 pg/ml)

4/12 (33.3)

94/99 (94.9)

6/14 (42.8)

< 0.001

CSF pathological biomarkers - N/N (%)

positive prion RT-QuIC

0/12 (0)

97/99 (97.9)

0/14 (0)

< 0.001

positive αSyn RT-QuIC

13/13 (100)

6/77 (7.7)

0/7 (0)

< 0.001

Aβ42/Aβ40 ratio (< 0.68)

5/9 (55.5)

14/72 (19.4)

7/7 (100)

< 0.001

p-tau181 (> 65 pg/ml)

3/9 (33.3)

21/72 (29.2)

7/7 (100)

0.001

CSF neurodegeneration biomarkers - median (IQR)

t-tau (pg/ml)

530 (195–1355)

6388 (2988–10440)

1034 (273–2156)

< 0.001

14-3-3 (AU/ml)

12,800 (4159–16500)

75,200 (30800–47000)

15,350 (7398–29800)

< 0.001

NfL (pg/ml)

3204 (2026–9550)

6200 (3702–10800)

1615 (1518–19700)

0.181

Clinical diagnosis

    

DLB [18]

7 probable,

9 possible, 6 not

12 probable,

33 possible, 55 not

9 probable,

8 possible, 14 not

-

sCJD [19]

2 probable,

7 possible, 13 not

99 probable,

1 possible, 0 not

12 probable,

9 possible, 10 not

-

  1. Bold values indicate statistically significant results. Visual disturbances include one or more of: visual loss, visual field defect, visual distortion, abnormal color vision, cortical blindness. Neuropsychiatric symptoms include one or more of: apathy, anxiety, depression, systematized delusions. Brain MRI was considered “positive” with high DWI or FLAIR signal in caudate/caudate-putamen/caudate-putamen-thalamus or at least two cortical areas (parietal, temporal or occipital). CSF 14-3-3 and t-tau values above cut-offs are indicative of CJD. Abnormal values of CSF p-tau181 and the Aβ42/Aβ40 ratio are indicative of AD pathological process
  2. Abbreviations αSyn, α-synuclein; Aβ, beta amyloid; AD, Alzheimer’s disease; CJD, Creutzfeldt-Jakob disease; CSF, cerebrospinal fluid; DLB, Dementia with Lewy bodies; DWI, diffusion weighted imaging; FLAIR, fluid-attenuated inversion recovery; IQR, interquartile range; EEG, electroencephalography; IQR, interquartile range; N, number; NfL, neurofilament light chain; PSWCs, generalized periodic sharp/wave complexes; p-tau181, phospho-tau181; RBD, REM sleep behavior disorder; RPD, rapidly progressive dementia; RT-QuIC, Real-Time Quaking-Induced Conversion; sCJD, sporadic Creutzfeldt-Jakob disease; t-tau, total tau
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Alzheimer's Research & Therapy

ISSN: 1758-9193