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1 e correct histological diagnosis of sporadic Creutzfeldt-Jakob disease.
2 e hitherto recognized phenotypic spectrum of Creutzfeldt-Jakob disease.
3 he most common human prion disease, sporadic Creutzfeldt-Jakob disease.
4 ng autoimmune encephalopathies from sporadic Creutzfeldt-Jakob disease.
5 ce of human-to-human transmission of variant Creutzfeldt-Jakob disease.
6 ted into the diagnostic criteria of sporadic Creutzfeldt-Jakob disease.
7 disease, nephrogenic diabetes insipidus, and Creutzfeldt-Jakob disease.
8 abnormalities that closely resemble sporadic Creutzfeldt-Jakob disease.
9 d either inherited prion disease or sporadic Creutzfeldt-Jakob disease.
10 me have met diagnostic criteria for sporadic Creutzfeldt-Jakob disease.
11 iduals resembled those of classical sporadic Creutzfeldt-Jakob disease.
12 observed in sporadic, iatrogenic and variant Creutzfeldt-Jakob disease.
13 nine homozygote to both sporadic and variant Creutzfeldt-Jakob disease.
14 arkinson's disease, motor neuron disease and Creutzfeldt-Jakob disease.
15 can be acquired, as is the case for variant Creutzfeldt-Jakob disease.
16 ts both between and within cases of sporadic Creutzfeldt-Jakob disease.
17 in a unique series of nine cases of variant Creutzfeldt-Jakob disease.
18 us system (CNS) in five of six patients with Creutzfeldt-Jakob disease.
19 evere acute respiratory syndrome, or variant Creutzfeldt-Jakob disease.
20 cephalopathy (BSE) prions causes new variant Creutzfeldt-Jakob disease.
21 tance of some sheep to scrapie and humans to Creutzfeldt-Jakob disease.
22 such as bovine spongiform encephalopathy and Creutzfeldt-Jakob disease.
23 uman beings affected by sporadic and variant Creutzfeldt-Jakob disease.
24 bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease.
25 of patients affected by the E200K variant of Creutzfeldt-Jakob disease.
26 s for therapy, for example, in 'new variant' Creutzfeldt-Jakob disease.
27 y of 80 to 90% for the diagnosis of sporadic Creutzfeldt-Jakob disease.
28 symmetric pattern of involvement in sporadic Creutzfeldt-Jakob disease.
29 patitis C virus, enterovirus 70, and variant Creutzfeldt-Jakob disease.
30 00% (95% CI, 90 to 100) for the detection of Creutzfeldt-Jakob disease.
31 vities in the total white matter in sporadic Creutzfeldt-Jakob disease.
32 with human prions, such as those involved in Creutzfeldt-Jakob disease.
33 disease in humans were diagnosed as variant Creutzfeldt-Jakob disease.
34 rved in experimental, acquired, and sporadic Creutzfeldt-Jakob diseases.
35 ases including Alzheimer's, Parkinson's, and Creutzfeldt-Jakob diseases.
36 nt diagnoses were Alzheimer's disease (18%), Creutzfeldt-Jakob disease (12%) and inflammatory disorde
37 kob disease (15 of 15 with definite sporadic Creutzfeldt-Jakob disease, 13 of 14 with probable sporad
38 ings were positive in 30 of 31 patients with Creutzfeldt-Jakob disease (15 of 15 with definite sporad
39 f 14 patients with the E200K genetic form of Creutzfeldt-Jakob Disease, 20 healthy carriers of this m
42 enotype individual can propagate the variant Creutzfeldt-Jakob disease agent and that the infectious
43 s hypothalamic GT cells infected with the FU Creutzfeldt-Jakob disease agent, but not parallel mock c
45 Sc) that accumulates in the brain in variant Creutzfeldt-Jakob disease also contains a minority type
46 m human post-mortem samples, of both variant Creutzfeldt-Jakob disease and Alzheimer's disease patien
48 om donors who subsequently developed variant Creutzfeldt-Jakob disease and an asymptomatic red cell t
49 eases of humans and other animals, including Creutzfeldt-Jakob disease and bovine spongiform encephal
51 able at low levels in some cases of sporadic Creutzfeldt-Jakob disease and conversely, that the form
52 on is believed responsible for most cases of Creutzfeldt-Jakob disease and for initiating the mad cow
53 iform encephalopathies (variant and sporadic Creutzfeldt-Jakob disease and genetic forms of prion dis
54 is related to various human diseases such as Creutzfeldt-Jakob disease and Gerstmann-Straussler-Schei
55 e only in the urine of patients with variant Creutzfeldt-Jakob disease and had the typical electropho
56 und in all of the major subtypes of sporadic Creutzfeldt-Jakob disease and in a case of iatrogenic Cr
57 samples obtained from patients with variant Creutzfeldt-Jakob disease and in none of the 224 urine s
58 m nasal brushings was accurate in diagnosing Creutzfeldt-Jakob disease and indicated substantial prio
60 opagation of prions, the causative agents of Creutzfeldt-Jakob disease and other human prion diseases
62 tes in the brains of patients suffering from Creutzfeldt-Jakob disease and related conditions, such a
64 patients with and patients without sporadic Creutzfeldt-Jakob disease and tested them using RT-QuIC,
65 the clinicopathological diversity evident in Creutzfeldt-Jakob disease and whether different prion pr
67 kob disease, 13 of 14 with probable sporadic Creutzfeldt-Jakob disease, and 2 of 2 with inherited Cre
68 ion diseases, including sheep scrapie, human Creutzfeldt-Jakob disease, and cervid chronic wasting di
69 is centrally involved in the pathogenesis of Creutzfeldt-Jakob Disease, and its anatomical connection
70 tions can be detected early in the course of Creutzfeldt-Jakob Disease, and years before symptomatic
71 tions more common in the T/BG group included Creutzfeldt-Jakob disease, arbovirus, and Mycobacterium
73 gregates derived from patients with sporadic Creutzfeldt-Jakob disease are taken up and degraded by i
75 either Creutzfeldt-Jakob disease or variant Creutzfeldt-Jakob disease are transmitted by transfusion
76 spongiform encephalopathies, such as variant Creutzfeldt-Jakob disease, are believed to result from i
77 cluding bovine spongiform encephalopathy and Creutzfeldt-Jakob disease, are usually characterized by
78 , in brain samples from humans with sporadic Creutzfeldt-Jakob disease, as well as in rodents with ex
79 dt-Jakob disease and in a case of iatrogenic Creutzfeldt-Jakob disease associated with growth hormone
80 and may be clinically mistaken for sporadic Creutzfeldt-Jakob disease because of a negative family h
81 ges that allowed early diagnosis of probable Creutzfeldt-Jakob disease before the characteristic clin
82 PrP(TSE)) when exposed to medium spiked with Creutzfeldt-Jakob disease brain homogenate, resulting in
83 -fold dilution of 10% (wt/vol) human variant Creutzfeldt-Jakob disease brain homogenate, with >3,800-
85 ldt-Jakob disease, and 2 of 2 with inherited Creutzfeldt-Jakob disease) but were negative in 43 of 43
86 ular system is a defining feature of variant Creutzfeldt-Jakob disease, but that the biochemical isof
88 by this review, the transmission of variant Creutzfeldt-Jakob disease by transfusion has been confir
89 a precaution against transmission of variant Creutzfeldt-Jakob disease by transfusion of domestic blo
90 al prion protein that characterizes sporadic Creutzfeldt-Jakob disease can be found in certain brain
92 and findings were followed-up in six variant Creutzfeldt-Jakob disease cases with 9.4 T high-resoluti
97 rP(Sc) aggregates extracted from 60 sporadic Creutzfeldt-Jakob disease (CJD) and 6 variant CJD brains
99 demyelinating disease, Alzheimer's disease, Creutzfeldt-Jakob disease (CJD) and Gerstmann-Straussler
100 between the invariably lethal prion disease Creutzfeldt-Jakob disease (CJD) and nonprion rapidly pro
101 Prion protein (PrP) plays a central role in Creutzfeldt-Jakob Disease (CJD) and other transmissible
103 g disease in deer and elk, and Kuru disease, Creutzfeldt-Jakob disease (CJD) and variant CJD in human
104 typical forms of Alzheimer disease (AD) and Creutzfeldt-Jakob disease (CJD) are clinically distingui
105 ile cerebrospinal fluid (CSF) biomarkers for Creutzfeldt-Jakob disease (CJD) are established and part
108 ns from the brains of patients with sporadic Creutzfeldt-Jakob disease (CJD) bind to very low-density
109 uman brain extracts demonstrated that PrP in Creutzfeldt-Jakob disease (CJD) brains is cleaved by a c
111 pendent reports have claimed anticipation in Creutzfeldt-Jakob disease (CJD) caused by the c.598G > A
112 rm encephalopathy (BSE) to humans as variant Creutzfeldt-Jakob disease (CJD) has affected over 100 in
116 al, neurodegenerative diseases which include Creutzfeldt-Jakob disease (CJD) in humans and bovine spo
117 le neurodegenerative conditions that include Creutzfeldt-Jakob disease (CJD) in humans and bovine spo
119 ssible neurodegenerative disorders including Creutzfeldt-Jakob disease (CJD) in humans, is the conver
126 Early and accurate in vivo diagnosis of Creutzfeldt-Jakob disease (CJD) is necessary for quickly
129 First, we successfully propagated various Creutzfeldt-Jakob disease (CJD) isolates (sporadic, vari
131 pletely resistant to both kuru and classical Creutzfeldt-Jakob disease (CJD) prions (which are closel
132 s (PrPs) have shorter incubation periods for Creutzfeldt-Jakob disease (CJD) prions than mice express
133 More than two hundred individuals developed Creutzfeldt-Jakob disease (CJD) worldwide as a result of
134 ), frontotemporal lobar degeneration (FTLD), Creutzfeldt-Jakob disease (CJD), Alzheimer's disease (AD
135 myoclonic jerks develop in individuals with Creutzfeldt-Jakob disease (CJD), an incurable brain diso
136 nd deer with those in subjects with sporadic Creutzfeldt-Jakob disease (CJD), as well as CJD-affected
137 ses numerous neurological diseases including Creutzfeldt-Jakob disease (CJD), but the aggregation mec
138 e most common human prion disorder, sporadic Creutzfeldt-Jakob disease (CJD), in which prions are for
139 giform encephalopathy to people as a variant Creutzfeldt-Jakob disease (CJD), it becomes critical to
140 replication of the infectious agents causing Creutzfeldt-Jakob disease (CJD), scrapie, and bovine spo
141 Compared with a mouse model of transmissible Creutzfeldt-Jakob disease (CJD), the ataxia of Tg(A116V)
154 sceptible to prions from humans with variant Creutzfeldt-Jakob disease (CJD); on second passage in Tg
155 re classified as either familial or sporadic Creutzfeldt-Jakob disease (CJD); there was no case of va
156 ation associated with one of these diseases [Creutzfeldt-Jakob disease (CJD)] that was exactly analog
157 rophic lateral sclerosis (FTD/ALS, n = 252), Creutzfeldt-Jakob disease (CJD, n = 239), Parkinson's di
158 maging to study the white matter in sporadic Creutzfeldt-Jakob disease compared to healthy control su
159 samples obtained from patients with variant Creutzfeldt-Jakob disease contained minute quantities of
160 b disease (to date, 1,147 cases of confirmed Creutzfeldt-Jakob disease deaths in the United Kingdom s
161 sorders: fatal familial insomnia or familial Creutzfeldt-Jakob disease, depending upon the presence o
164 Back-calculation analysis of the variant Creutzfeldt-Jakob disease epidemic in the United Kingdom
165 f growth hormone derived from a patient with Creutzfeldt-Jakob disease expressing prion protein valin
166 urodegenerative disorders that include Kuru, Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinke
169 fusion recipient, who did not die of variant Creutzfeldt-Jakob disease, has been identified with prio
171 vious definite and probable cases of variant Creutzfeldt-Jakob disease have been methionine homozygot
172 ephalopathy to humans in the form of variant Creutzfeldt Jakob disease, have raised concerns about th
173 long been used in the diagnosis of sporadic Creutzfeldt-Jakob disease; however, the characteristic w
174 orts (FTLD, ALS, Alzheimer disease, sporadic Creutzfeldt-Jakob disease, Huntington disease-like syndr
175 fatal neurodegenerative conditions including Creutzfeldt-Jakob disease in humans and scrapie and bovi
176 tal neurodegenerative disorders that include Creutzfeldt-Jakob disease in humans, scrapie in sheep an
177 fatal neurodegenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in sheep an
183 ithin the cohort of patients with iatrogenic Creutzfeldt-Jakob disease in the UK suggests that there
185 ections with variant, sporadic, and familial Creutzfeldt-Jakob disease, in the presence of blood comp
186 d with other transmission studies in variant Creutzfeldt-Jakob disease, including those on the spleen
187 t were negative in 43 of 43 patients without Creutzfeldt-Jakob disease, indicating a sensitivity of 9
188 r significant numbers of subclinical variant Creutzfeldt-Jakob disease individuals in at least the Un
192 of humans and animals, one of which (variant Creutzfeldt-Jakob disease) is known to be a zoonotic for
193 novel acquired human prion disease, variant Creutzfeldt-Jakob disease, is thought to result from ora
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
199 also highly susceptible to a new variant of Creutzfeldt-Jakob disease (nvCJD) and natural sheep scra
200 35 deaths had been attributed to new variant Creutzfeldt-Jakob disease (nvCJD) in the United Kingdom,
201 re was no evidence of any death from variant Creutzfeldt-Jakob disease or from conditions that could
203 an occur as an idiopathic disorder (sporadic Creutzfeldt-Jakob disease) or can be acquired, as is the
204 rion protein disease with this feature after Creutzfeldt-Jakob disease, originally reported in 1920.
205 Risk assessments for transmission of variant Creutzfeldt-Jakob disease predicted that leukocyte reduc
208 it was ineffective in slowing propagation of Creutzfeldt-Jakob disease prions in transgenic mice.
209 nerative diseases, and arguably, scrapie and Creutzfeldt-Jakob disease prions represent the best ther
211 Cumulatively, the data show that sporadic Creutzfeldt-Jakob disease PrP(Sc) is not a single confor
212 phic codon in humans associated with variant Creutzfeldt-Jakob disease, pulls the N terminus into the
213 his case report from the Australian National Creutzfeldt-Jakob Disease Registry concerns a 61-year-ol
214 ders, the solution structure of the familial Creutzfeldt-Jakob disease-related E200K variant of human
216 review of patients referred to the National Creutzfeldt-Jakob Disease Research & Surveillance Unit d
217 thought to confer susceptibility to sporadic Creutzfeldt-Jakob disease (rs1029273), all patients were
218 n clinic who were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to h
219 cerebrospinal fluid (CSF) tests for sporadic Creutzfeldt-Jakob disease (sCJD) are based on the detect
221 tal insomnia (sFI) and a subtype of sporadic Creutzfeldt-Jakob disease (sCJD) identified as sCJDMM2,
223 QuIC differentiated 94% of cases of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 from the sCJD MM2 p
226 ding, the most common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive
227 with the most common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive
228 ct subtypes have been identified in sporadic Creutzfeldt-Jakob disease (sCJD), based on the methionin
229 brains of two patients, who died of sporadic Creutzfeldt-Jakob disease (sCJD), contained either sCJD(
231 und PrP species present in control, sporadic Creutzfeldt-Jakob disease (sCJD), or variant CJD (vCJD)
232 the heart tissue of a patient with sporadic Creutzfeldt-Jakob Disease (sCJD), the most common form o
237 as diverse as Alzheimer's, Parkinson's, and Creutzfeldt-Jakob disease share a common pathogenetic me
238 fusivity within the white matter in sporadic Creutzfeldt-Jakob disease, suggesting possible primary i
239 s a single PrP(Sc) molecular type in variant Creutzfeldt-Jakob disease (termed type 2B), presumably r
240 PrP(Sc) was found in all 21 cases of variant Creutzfeldt-Jakob disease tested, irrespective of brain
241 A test that detects the specific marker for Creutzfeldt-Jakob disease, the prion protein (PrP(CJD)),
242 PrP 129M/V polymorphism protects people from Creutzfeldt-Jakob disease, the Sup35p polymorphisms were
245 , such as endotoxemia, sepsis, or iatrogenic Creutzfeldt-Jakob disease (to date, 1,147 cases of confi
247 rent understanding of the risks of (variant) Creutzfeldt-Jakob disease transmission via dental practi
249 maining patients, dementia with Lewy bodies, Creutzfeldt-Jakob disease, vascular or unclassified deme
251 he fatal neurodegenerative condition variant Creutzfeldt-Jakob disease (vCJD) and, based on recent hu
252 dy indicates a prototype blood-based variant Creutzfeldt-Jakob disease (vCJD) assay has sufficient se
253 truments from, a deceased carrier of variant Creutzfeldt-Jakob disease (vCJD) can be followed up to q
258 Person-to-person transmission of variant Creutzfeldt-Jakob disease (vCJD) has occurred through bl
261 We report a case of preclinical variant Creutzfeldt-Jakob disease (vCJD) in a patient who died f
262 ease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having gu
263 relevant to the current outbreak of variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom,
270 lity to, and clinical expression of, variant Creutzfeldt-Jakob disease (vCJD) is essential for future
272 cal variation in the distribution of variant Creutzfeldt-Jakob disease (vCJD) might indicate the tran
273 mples, from prion-affected patients (variant Creutzfeldt-Jakob disease (vCJD) n = 20, iatrogenic CJD
274 spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) prions are faithfully m
277 reasing concern since the reports of variant Creutzfeldt-Jakob disease (vCJD) transmission through bl
279 fication of possible transmission of variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion h
280 timates for the risk of transmitting variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion h
284 umans have since developed a variant form of Creutzfeldt-Jakob disease (vCJD), also mostly in the Uni
286 athy (BSE) and its human equivalent, variant Creutzfeldt-Jakob disease (vCJD), are caused by the same
287 has been described in cases of human variant Creutzfeldt-Jakob disease (vCJD), experimental ovine bov
288 vine spongiform encephalopathy (BSE)/variant Creutzfeldt-Jakob disease (vCJD), Nipah virus, several v
289 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
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