Diagnosis of new variant Creutzfeldt-Jakob disease

Will, Robert; Zeidler, Martin; Stewart, G.; Macleod, Margaret-Ann; Ironside, James; Cousens, Simon; Mackenzie, J.; Estibeiro, K; Green, A. J. E.; Knight, Richard

doi:10.1002/1531-8249(200005)47:5<575::aid-ana4>3.0.co;2-w

Cited by 361 publications

(159 citation statements)

References 13 publications

(15 reference statements)

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“…Thus, our observations may not be relevant to humans carrying one or both valine alleles; however, all patients with vCJD reported to date have been M͞M at this position (27).…”

Section: Discussionmentioning

confidence: 87%

Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt– Jakob disease: Implications for human health

Lasmézas

Fournier²,

Nouvel³

et al. 2001

Proc. Natl. Acad. Sci. U.S.A.

156

117

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There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (BSE) has contaminated human beings, causing variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted BSE agent are unknown. We show that (i) BSE can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD to humans could be readily recognized pathologically, whether it occurs by the central or peripheral route. Strain typing in mice demonstrates that the BSE agent adapts to macaques in the same way as it does to humans and confirms that the BSE agent is responsible for vCJD not only in the United Kingdom but also in France. The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.

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“…Thus, our observations may not be relevant to humans carrying one or both valine alleles; however, all patients with vCJD reported to date have been M͞M at this position (27).…”

Section: Discussionmentioning

confidence: 87%

Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt– Jakob disease: Implications for human health

Lasmézas

Fournier²,

Nouvel³

et al. 2001

Proc. Natl. Acad. Sci. U.S.A.

156

117

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“…Sporadic fatal insomnia is a rare prion disease that has recently been recognized and is characterized by loss of sleep, oneiric stupor with autonomic/motor hyperactivity and somato-motor abnormalities, including pyramidal signs, myoclonus, dysarthria, dysphagia and ataxia 30 . In the new variant of Creutzfeldt-Jakob disease some patients demonstrated a combination of psychiatric symptoms, ataxia and severe sleep disorders 31 . Although still unrecognized sleep disorders in cerebellar ataxias have been a recent issue of interest, and growing evidence has proving the importance of their identification.…”

Section: Sleep Disorders In Sporadic Ataxiasmentioning

confidence: 99%

Sleep disorders in cerebellar ataxias

Pedroso

Braga‐Neto

Felı́cio

et al. 2011

Arq. Neuro-Psiquiatr.

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Cerebellar ataxias comprise a wide range of etiologies leading to central nervous system-related motor and non-motor symptoms. Recently, a large body of evidence has demonstrated a high frequency of non-motor manifestations in cerebellar ataxias, specially in autosomal dominant spinocerebellar ataxias (SCA). Among these nonmotor dysfunctions, sleep disorders have been recognized, although still under or even misdiagnosed. In this review, we highlight the main sleep disorders related to cerebellar ataxias focusing on REM sleep behavior disorder (RBD), restless legs syndrome (RLS), periodic limb movement in sleep (PLMS), excessive daytime sleepiness (EDS), insomnia and sleep apnea. Key words: sleep disorders, ataxias, diagnosis.Distúrbios do sono nas ataxias cerebelares RESUMO As ataxias cerebelares se caracterizam por uma enorme variedade de etiologias, cursando tanto com sintomas motores como também com sintomas não motores. Recentemente, várias evidências têm demonstrado uma frequência elevada de sintomas não motores nas ataxias cerebelares, especialmente nas ataxias espinocerebelares autossômicas dominantes (SCA). Dentre os sintomas não motores, estão os distúrbios do sono, que muitas vezes são sub-diagnosticados ou pouco valorizados. Nessa revisão, enfatizamos os principais distúrbios do sono relatados nas ataxias cerebelares, como transtorno comportamental do sono REM, síndrome das pernas inquietas, movimentos periódicos das pernas no sono, sonolência diurna excessiva, insônia e apnéia do sono. Palavras-chave: distúrbios do sono, ataxias, diagnóstico.

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“…Increased rates of early ataxia have been reported in DM-CJD, hGH-CJD and vCJD [10][11][12][13]. In DM-CJD, a possible influence of the graft location on the clinical onset has been suggested [14,15].…”

Section: ■ Abstract Cjd · Mri · Dementia · Cerebellar Disorder · Cnsmentioning

confidence: 99%