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1 with human prions, such as those involved in Creutzfeldt-Jakob disease.
2 disease in humans were diagnosed as variant Creutzfeldt-Jakob disease.
3 e correct histological diagnosis of sporadic Creutzfeldt-Jakob disease.
4 e hitherto recognized phenotypic spectrum of Creutzfeldt-Jakob disease.
5 he most common human prion disease, sporadic Creutzfeldt-Jakob disease.
6 ng autoimmune encephalopathies from sporadic Creutzfeldt-Jakob disease.
7 ce of human-to-human transmission of variant Creutzfeldt-Jakob disease.
8 ted into the diagnostic criteria of sporadic Creutzfeldt-Jakob disease.
9 disease, nephrogenic diabetes insipidus, and Creutzfeldt-Jakob disease.
10 abnormalities that closely resemble sporadic Creutzfeldt-Jakob disease.
11 me have met diagnostic criteria for sporadic Creutzfeldt-Jakob disease.
12 iduals resembled those of classical sporadic Creutzfeldt-Jakob disease.
13 observed in sporadic, iatrogenic and variant Creutzfeldt-Jakob disease.
14 nine homozygote to both sporadic and variant Creutzfeldt-Jakob disease.
15 arkinson's disease, motor neuron disease and Creutzfeldt-Jakob disease.
16 can be acquired, as is the case for variant Creutzfeldt-Jakob disease.
17 ts both between and within cases of sporadic Creutzfeldt-Jakob disease.
18 in a unique series of nine cases of variant Creutzfeldt-Jakob disease.
19 us system (CNS) in five of six patients with Creutzfeldt-Jakob disease.
20 evere acute respiratory syndrome, or variant Creutzfeldt-Jakob disease.
21 cephalopathy (BSE) prions causes new variant Creutzfeldt-Jakob disease.
22 tance of some sheep to scrapie and humans to Creutzfeldt-Jakob disease.
23 patitis C virus, enterovirus 70, and variant Creutzfeldt-Jakob disease.
24 d either inherited prion disease or sporadic Creutzfeldt-Jakob disease.
25 y of 80 to 90% for the diagnosis of sporadic Creutzfeldt-Jakob disease.
26 symmetric pattern of involvement in sporadic Creutzfeldt-Jakob disease.
27 ld lead to a more virulent strain of variant Creutzfeldt-Jakob disease.
28 00% (95% CI, 90 to 100) for the detection of Creutzfeldt-Jakob disease.
29 vities in the total white matter in sporadic Creutzfeldt-Jakob disease.
30 rved in experimental, acquired, and sporadic Creutzfeldt-Jakob diseases.
31 ases including Alzheimer's, Parkinson's, and Creutzfeldt-Jakob diseases.
32 nt diagnoses were Alzheimer's disease (18%), Creutzfeldt-Jakob disease (12%) and inflammatory disorde
33 kob disease (15 of 15 with definite sporadic Creutzfeldt-Jakob disease, 13 of 14 with probable sporad
34 ings were positive in 30 of 31 patients with Creutzfeldt-Jakob disease (15 of 15 with definite sporad
35 f 14 patients with the E200K genetic form of Creutzfeldt-Jakob Disease, 20 healthy carriers of this m
39 enotype individual can propagate the variant Creutzfeldt-Jakob disease agent and that the infectious
40 ssibility that the properties of the variant Creutzfeldt-Jakob disease agent could change after trans
41 s hypothalamic GT cells infected with the FU Creutzfeldt-Jakob disease agent, but not parallel mock c
43 Sc) that accumulates in the brain in variant Creutzfeldt-Jakob disease also contains a minority type
44 m human post-mortem samples, of both variant Creutzfeldt-Jakob disease and Alzheimer's disease patien
46 om donors who subsequently developed variant Creutzfeldt-Jakob disease and an asymptomatic red cell t
47 eases of humans and other animals, including Creutzfeldt-Jakob disease and bovine spongiform encephal
49 able at low levels in some cases of sporadic Creutzfeldt-Jakob disease and conversely, that the form
50 iform encephalopathies (variant and sporadic Creutzfeldt-Jakob disease and genetic forms of prion dis
51 is related to various human diseases such as Creutzfeldt-Jakob disease and Gerstmann-Straussler-Schei
52 e only in the urine of patients with variant Creutzfeldt-Jakob disease and had the typical electropho
53 und in all of the major subtypes of sporadic Creutzfeldt-Jakob disease and in a case of iatrogenic Cr
54 samples obtained from patients with variant Creutzfeldt-Jakob disease and in none of the 224 urine s
55 m nasal brushings was accurate in diagnosing Creutzfeldt-Jakob disease and indicated substantial prio
57 opagation of prions, the causative agents of Creutzfeldt-Jakob disease and other human prion diseases
59 tes in the brains of patients suffering from Creutzfeldt-Jakob disease and related conditions, such a
61 patients with and patients without sporadic Creutzfeldt-Jakob disease and tested them using RT-QuIC,
62 the clinicopathological diversity evident in Creutzfeldt-Jakob disease and whether different prion pr
64 kob disease, 13 of 14 with probable sporadic Creutzfeldt-Jakob disease, and 2 of 2 with inherited Cre
65 y this clinical syndrome is so distinct from Creutzfeldt-Jakob disease, and biomarkers of the early s
66 ion diseases, including sheep scrapie, human Creutzfeldt-Jakob disease, and cervid chronic wasting di
67 is centrally involved in the pathogenesis of Creutzfeldt-Jakob Disease, and its anatomical connection
68 tions can be detected early in the course of Creutzfeldt-Jakob Disease, and years before symptomatic
69 tions more common in the T/BG group included Creutzfeldt-Jakob disease, arbovirus, and Mycobacterium
71 gregates derived from patients with sporadic Creutzfeldt-Jakob disease are taken up and degraded by i
73 spongiform encephalopathies, such as variant Creutzfeldt-Jakob disease, are believed to result from i
74 , in brain samples from humans with sporadic Creutzfeldt-Jakob disease, as well as in rodents with ex
75 dt-Jakob disease and in a case of iatrogenic Creutzfeldt-Jakob disease associated with growth hormone
76 and may be clinically mistaken for sporadic Creutzfeldt-Jakob disease because of a negative family h
77 ges that allowed early diagnosis of probable Creutzfeldt-Jakob disease before the characteristic clin
78 PrP(TSE)) when exposed to medium spiked with Creutzfeldt-Jakob disease brain homogenate, resulting in
79 -fold dilution of 10% (wt/vol) human variant Creutzfeldt-Jakob disease brain homogenate, with >3,800-
81 ldt-Jakob disease, and 2 of 2 with inherited Creutzfeldt-Jakob disease) but were negative in 43 of 43
82 ular system is a defining feature of variant Creutzfeldt-Jakob disease, but that the biochemical isof
84 by this review, the transmission of variant Creutzfeldt-Jakob disease by transfusion has been confir
85 a precaution against transmission of variant Creutzfeldt-Jakob disease by transfusion of domestic blo
86 al prion protein that characterizes sporadic Creutzfeldt-Jakob disease can be found in certain brain
88 and findings were followed-up in six variant Creutzfeldt-Jakob disease cases with 9.4 T high-resoluti
92 rP(Sc) aggregates extracted from 60 sporadic Creutzfeldt-Jakob disease (CJD) and 6 variant CJD brains
94 between the invariably lethal prion disease Creutzfeldt-Jakob disease (CJD) and nonprion rapidly pro
95 Prion protein (PrP) plays a central role in Creutzfeldt-Jakob Disease (CJD) and other transmissible
97 typical forms of Alzheimer disease (AD) and Creutzfeldt-Jakob disease (CJD) are clinically distingui
98 ile cerebrospinal fluid (CSF) biomarkers for Creutzfeldt-Jakob disease (CJD) are established and part
101 ns from the brains of patients with sporadic Creutzfeldt-Jakob disease (CJD) bind to very low-density
102 uman brain extracts demonstrated that PrP in Creutzfeldt-Jakob disease (CJD) brains is cleaved by a c
104 pendent reports have claimed anticipation in Creutzfeldt-Jakob disease (CJD) caused by the c.598G > A
105 rm encephalopathy (BSE) to humans as variant Creutzfeldt-Jakob disease (CJD) has affected over 100 in
109 al, neurodegenerative diseases which include Creutzfeldt-Jakob disease (CJD) in humans and bovine spo
110 le neurodegenerative conditions that include Creutzfeldt-Jakob disease (CJD) in humans and bovine spo
112 ssible neurodegenerative disorders including Creutzfeldt-Jakob disease (CJD) in humans, is the conver
119 Early and accurate in vivo diagnosis of Creutzfeldt-Jakob disease (CJD) is necessary for quickly
122 First, we successfully propagated various Creutzfeldt-Jakob disease (CJD) isolates (sporadic, vari
124 pletely resistant to both kuru and classical Creutzfeldt-Jakob disease (CJD) prions (which are closel
125 s (PrPs) have shorter incubation periods for Creutzfeldt-Jakob disease (CJD) prions than mice express
126 More than two hundred individuals developed Creutzfeldt-Jakob disease (CJD) worldwide as a result of
128 ), frontotemporal lobar degeneration (FTLD), Creutzfeldt-Jakob disease (CJD), Alzheimer's disease (AD
129 myoclonic jerks develop in individuals with Creutzfeldt-Jakob disease (CJD), an incurable brain diso
130 nd deer with those in subjects with sporadic Creutzfeldt-Jakob disease (CJD), as well as CJD-affected
131 ses numerous neurological diseases including Creutzfeldt-Jakob disease (CJD), but the aggregation mec
132 e most common human prion disorder, sporadic Creutzfeldt-Jakob disease (CJD), in which prions are for
133 giform encephalopathy to people as a variant Creutzfeldt-Jakob disease (CJD), it becomes critical to
134 replication of the infectious agents causing Creutzfeldt-Jakob disease (CJD), scrapie, and bovine spo
135 Compared with a mouse model of transmissible Creutzfeldt-Jakob disease (CJD), the ataxia of Tg(A116V)
137 in different forms and molecular subtypes of Creutzfeldt-Jakob disease (CJD), we applied 3 different
146 sceptible to prions from humans with variant Creutzfeldt-Jakob disease (CJD); on second passage in Tg
147 re classified as either familial or sporadic Creutzfeldt-Jakob disease (CJD); there was no case of va
148 ation associated with one of these diseases [Creutzfeldt-Jakob disease (CJD)] that was exactly analog
149 rophic lateral sclerosis (FTD/ALS, n = 252), Creutzfeldt-Jakob disease (CJD, n = 239), Parkinson's di
150 maging to study the white matter in sporadic Creutzfeldt-Jakob disease compared to healthy control su
151 samples obtained from patients with variant Creutzfeldt-Jakob disease contained minute quantities of
152 b disease (to date, 1,147 cases of confirmed Creutzfeldt-Jakob disease deaths in the United Kingdom s
153 sorders: fatal familial insomnia or familial Creutzfeldt-Jakob disease, depending upon the presence o
156 Back-calculation analysis of the variant Creutzfeldt-Jakob disease epidemic in the United Kingdom
157 f growth hormone derived from a patient with Creutzfeldt-Jakob disease expressing prion protein valin
158 (E200K in humans), responsible for inherited Creutzfeldt-Jakob disease, finds that the mutation lower
159 urodegenerative disorders that include Kuru, Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinke
160 hether the strain characteristics of variant Creutzfeldt-Jakob disease had been modified by the host
163 fusion recipient, who did not die of variant Creutzfeldt-Jakob disease, has been identified with prio
166 vious definite and probable cases of variant Creutzfeldt-Jakob disease have been methionine homozygot
167 ephalopathy to humans in the form of variant Creutzfeldt Jakob disease, have raised concerns about th
168 long been used in the diagnosis of sporadic Creutzfeldt-Jakob disease; however, the characteristic w
169 in patients both with and without iatrogenic Creutzfeldt-Jakob disease; however, there is little info
170 orts (FTLD, ALS, Alzheimer disease, sporadic Creutzfeldt-Jakob disease, Huntington disease-like syndr
172 fatal neurodegenerative conditions including Creutzfeldt-Jakob disease in humans and scrapie and bovi
173 tal neurodegenerative disorders that include Creutzfeldt-Jakob disease in humans, scrapie in sheep an
174 fatal neurodegenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in sheep an
180 ithin the cohort of patients with iatrogenic Creutzfeldt-Jakob disease in the UK suggests that there
182 ections with variant, sporadic, and familial Creutzfeldt-Jakob disease, in the presence of blood comp
183 d with other transmission studies in variant Creutzfeldt-Jakob disease, including those on the spleen
184 t were negative in 43 of 43 patients without Creutzfeldt-Jakob disease, indicating a sensitivity of 9
185 r significant numbers of subclinical variant Creutzfeldt-Jakob disease individuals in at least the Un
186 modification, the identification of variant Creutzfeldt-Jakob disease infection in a PRNP 129 methio
190 of humans and animals, one of which (variant Creutzfeldt-Jakob disease) is known to be a zoonotic for
191 novel acquired human prion disease, variant Creutzfeldt-Jakob disease, is thought to result from ora
192 including sporadic, variant, and iatrogenic Creutzfeldt-Jakob disease; kuru; inherited prion disease
193 exia and paresthesia, pure dementia GSS, and Creutzfeldt-Jakob disease-like GSS); GSS may be more com
194 t disease phenotypes, identified as sporadic Creutzfeldt-Jakob disease (M/M2 sCJD) and sporadic fatal
195 n the white matter of patients with sporadic Creutzfeldt-Jakob disease, mainly in the left hemisphere
196 in many neurodegenerative diseases including Creutzfeldt-Jakob disease, motor neuron disease and Alzh
197 59) and compare these with cases of sporadic Creutzfeldt-Jakob disease (n = 170) in the United Kingdo
198 PrP(Sc) from autopsy-proved cases of variant Creutzfeldt-Jakob disease (n = 59) and compare these wit
200 re was no evidence of any death from variant Creutzfeldt-Jakob disease or from conditions that could
201 an occur as an idiopathic disorder (sporadic Creutzfeldt-Jakob disease) or can be acquired, as is the
202 rion protein disease with this feature after Creutzfeldt-Jakob disease, originally reported in 1920.
203 Risk assessments for transmission of variant Creutzfeldt-Jakob disease predicted that leukocyte reduc
206 analysis also improved detection of sporadic Creutzfeldt-Jakob disease prions in human nasal brushing
207 it was ineffective in slowing propagation of Creutzfeldt-Jakob disease prions in transgenic mice.
208 nerative diseases, and arguably, scrapie and Creutzfeldt-Jakob disease prions represent the best ther
210 Cumulatively, the data show that sporadic Creutzfeldt-Jakob disease PrP(Sc) is not a single confor
211 his case report from the Australian National Creutzfeldt-Jakob Disease Registry concerns a 61-year-ol
213 review of patients referred to the National Creutzfeldt-Jakob Disease Research & Surveillance Unit d
214 thought to confer susceptibility to sporadic Creutzfeldt-Jakob disease (rs1029273), all patients were
215 n clinic who were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to h
216 cerebrospinal fluid (CSF) tests for sporadic Creutzfeldt-Jakob disease (sCJD) are based on the detect
219 tal insomnia (sFI) and a subtype of sporadic Creutzfeldt-Jakob disease (sCJD) identified as sCJDMM2,
221 r 85% of human prion disease cases, sporadic Creutzfeldt-Jakob disease (sCJD) is the prevalent human
222 QuIC differentiated 94% of cases of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 from the sCJD MM2 p
226 ort a detailed study of a cohort of sporadic Creutzfeldt-Jakob disease (sCJD) VV1-2 type-mixed cases
227 ding, the most common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive
228 with the most common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive
229 ct subtypes have been identified in sporadic Creutzfeldt-Jakob disease (sCJD), based on the methionin
230 brains of two patients, who died of sporadic Creutzfeldt-Jakob disease (sCJD), contained either sCJD(
232 und PrP species present in control, sporadic Creutzfeldt-Jakob disease (sCJD), or variant CJD (vCJD)
233 the heart tissue of a patient with sporadic Creutzfeldt-Jakob Disease (sCJD), the most common form o
239 as diverse as Alzheimer's, Parkinson's, and Creutzfeldt-Jakob disease share a common pathogenetic me
242 fusivity within the white matter in sporadic Creutzfeldt-Jakob disease, suggesting possible primary i
243 s a single PrP(Sc) molecular type in variant Creutzfeldt-Jakob disease (termed type 2B), presumably r
244 PrP(Sc) was found in all 21 cases of variant Creutzfeldt-Jakob disease tested, irrespective of brain
245 A test that detects the specific marker for Creutzfeldt-Jakob disease, the prion protein (PrP(CJD)),
246 PrP 129M/V polymorphism protects people from Creutzfeldt-Jakob disease, the Sup35p polymorphisms were
249 , such as endotoxemia, sepsis, or iatrogenic Creutzfeldt-Jakob disease (to date, 1,147 cases of confi
251 rent understanding of the risks of (variant) Creutzfeldt-Jakob disease transmission via dental practi
252 ere totally consistent with those of variant Creutzfeldt-Jakob disease transmitted to 129 methionine/
253 maining patients, dementia with Lewy bodies, Creutzfeldt-Jakob disease, vascular or unclassified deme
255 he fatal neurodegenerative condition variant Creutzfeldt-Jakob disease (vCJD) and, based on recent hu
256 dy indicates a prototype blood-based variant Creutzfeldt-Jakob disease (vCJD) assay has sufficient se
257 truments from, a deceased carrier of variant Creutzfeldt-Jakob disease (vCJD) can be followed up to q
262 Person-to-person transmission of variant Creutzfeldt-Jakob disease (vCJD) has occurred through bl
265 We report a case of preclinical variant Creutzfeldt-Jakob disease (vCJD) in a patient who died f
266 ease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having gu
273 lity to, and clinical expression of, variant Creutzfeldt-Jakob disease (vCJD) is essential for future
275 cal variation in the distribution of variant Creutzfeldt-Jakob disease (vCJD) might indicate the tran
276 mples, from prion-affected patients (variant Creutzfeldt-Jakob disease (vCJD) n = 20, iatrogenic CJD
277 spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) prions are faithfully m
280 reasing concern since the reports of variant Creutzfeldt-Jakob disease (vCJD) transmission through bl
282 timates for the risk of transmitting variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion h
283 fication of possible transmission of variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion h
285 ) in urine of patients affected with variant Creutzfeldt-Jakob disease (vCJD), a prion disease typica
286 umans have since developed a variant form of Creutzfeldt-Jakob disease (vCJD), also mostly in the Uni
288 athy (BSE) and its human equivalent, variant Creutzfeldt-Jakob disease (vCJD), are caused by the same
289 has been described in cases of human variant Creutzfeldt-Jakob disease (vCJD), experimental ovine bov
290 vine spongiform encephalopathy (BSE)/variant Creutzfeldt-Jakob disease (vCJD), Nipah virus, several v
291 in lymphoid tissues of patients with variant Creutzfeldt-Jakob disease (vCJD), sheep with natural scr
296 en PrP(Sc) types was maintained when variant Creutzfeldt-Jakob disease was transmitted to wild-type m
297 tected in the urine of patients with variant Creutzfeldt-Jakob disease, we used the protein misfoldin
298 en implicated both in prion diseases such as Creutzfeldt-Jakob disease, where its monomeric cellular
299 sion and the appearance of a variant form of Creutzfeldt-Jakob disease, which has been linked to cons