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New variant Creutzfeldt-Jakob disease: neurological features and diagnostic tests.
Lancet. 1997 Sep 27; 350(9082):903-7.Lct

Abstract

BACKGROUND

In April, 1996, ten cases of Creutzfeldt-Jakob disease (CJD) with an apparently new clinicopathological phenotype were published and it was suggested that these new variant cases (nvCJD) might be causally linked to bovine spongiform encephalopathy (BSE). There have now been 21 cases of nvCJD in the UK and one case in France. We report clinical features and diagnostic test results of the first 14 cases of nvCJD in the UK.

METHODS

Case ascertainment of CJD was mainly by direct referral from neurologists and neuropathologists. Clinical and investigate details were obtained by interview with patients' relatives and by examination of case notes. Ten cases in this report were examined while alive. Prion protein (PrP) gene analysis was carried out with informed consent from the patient or from a relative. The diagnosis of nvCJD was established histologically.

FINDINGS

Eight cases were women. Mean age at onset of symptoms was 29 (16-48) years and the median duration of illness was 14 (9-35) months. All patients had early psychiatric symptoms, most often depression, and 13 were seen by a psychiatrist early in the clinical course. Eight patients developed early sensory symptoms which were persistent and often painful. Neurological signs, including ataxia and involuntary movements, developed in all cases and towards the end of the illness, most had akinetic mutism. The electroencephalogram was abnormal in most patients but typical periodic complexes of CJD were not seen in any case. Cerebral imaging was usually normal or showed non-specific abnormalities; in two cases magnetic-resonance imaging scans showed high signal in the thalamus.

INTERPRETATION

Clinical features in these cases are similar and relatively distinct from other forms of CJD, suggesting that this is a new clinical phenotype consistent with a single strain of infectious agent. There is, however, some overlap with atypical cases of sporadic CJD, and the diagnosis of nvCJD remains dependent on neuropathological confirmation.

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Authors+Show Affiliations

Zeidler M
National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh.
Stewart GE
No affiliation info available
Barraclough CR
No affiliation info available
Bateman DE
No affiliation info available
Bates D
No affiliation info available
Burn DJ
No affiliation info available
Colchester AC
No affiliation info available
Durward W
No affiliation info available
Fletcher NA
No affiliation info available
Hawkins SA
No affiliation info available
Mackenzie JM
No affiliation info available
Will RG
No affiliation info available

MeSH

AdolescentAdultCreutzfeldt-Jakob SyndromeElectroencephalographyFemaleHumansMaleMiddle AgedPhenotypePrionsTomography, Emission-Computed, Single-PhotonTomography, X-Ray Computed

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

9314867

Citation

Zeidler, M, et al. "New Variant Creutzfeldt-Jakob Disease: Neurological Features and Diagnostic Tests." Lancet (London, England), vol. 350, no. 9082, 1997, pp. 903-7.
Zeidler M, Stewart GE, Barraclough CR, et al. New variant Creutzfeldt-Jakob disease: neurological features and diagnostic tests. Lancet. 1997;350(9082):903-7.
Zeidler, M., Stewart, G. E., Barraclough, C. R., Bateman, D. E., Bates, D., Burn, D. J., Colchester, A. C., Durward, W., Fletcher, N. A., Hawkins, S. A., Mackenzie, J. M., & Will, R. G. (1997). New variant Creutzfeldt-Jakob disease: neurological features and diagnostic tests. Lancet (London, England), 350(9082), 903-7.
Zeidler M, et al. New Variant Creutzfeldt-Jakob Disease: Neurological Features and Diagnostic Tests. Lancet. 1997 Sep 27;350(9082):903-7. PubMed PMID: 9314867.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - New variant Creutzfeldt-Jakob disease: neurological features and diagnostic tests. AU - Zeidler,M, AU - Stewart,G E, AU - Barraclough,C R, AU - Bateman,D E, AU - Bates,D, AU - Burn,D J, AU - Colchester,A C, AU - Durward,W, AU - Fletcher,N A, AU - Hawkins,S A, AU - Mackenzie,J M, AU - Will,R G, PY - 1997/10/7/pubmed PY - 1997/10/7/medline PY - 1997/10/7/entrez SP - 903 EP - 7 JF - Lancet (London, England) JO - Lancet VL - 350 IS - 9082 N2 - BACKGROUND: In April, 1996, ten cases of Creutzfeldt-Jakob disease (CJD) with an apparently new clinicopathological phenotype were published and it was suggested that these new variant cases (nvCJD) might be causally linked to bovine spongiform encephalopathy (BSE). There have now been 21 cases of nvCJD in the UK and one case in France. We report clinical features and diagnostic test results of the first 14 cases of nvCJD in the UK. METHODS: Case ascertainment of CJD was mainly by direct referral from neurologists and neuropathologists. Clinical and investigate details were obtained by interview with patients' relatives and by examination of case notes. Ten cases in this report were examined while alive. Prion protein (PrP) gene analysis was carried out with informed consent from the patient or from a relative. The diagnosis of nvCJD was established histologically. FINDINGS: Eight cases were women. Mean age at onset of symptoms was 29 (16-48) years and the median duration of illness was 14 (9-35) months. All patients had early psychiatric symptoms, most often depression, and 13 were seen by a psychiatrist early in the clinical course. Eight patients developed early sensory symptoms which were persistent and often painful. Neurological signs, including ataxia and involuntary movements, developed in all cases and towards the end of the illness, most had akinetic mutism. The electroencephalogram was abnormal in most patients but typical periodic complexes of CJD were not seen in any case. Cerebral imaging was usually normal or showed non-specific abnormalities; in two cases magnetic-resonance imaging scans showed high signal in the thalamus. INTERPRETATION: Clinical features in these cases are similar and relatively distinct from other forms of CJD, suggesting that this is a new clinical phenotype consistent with a single strain of infectious agent. There is, however, some overlap with atypical cases of sporadic CJD, and the diagnosis of nvCJD remains dependent on neuropathological confirmation. SN - 0140-6736 UR - https://www.unboundmedicine.com/medline/citation/9314867/New_variant_Creutzfeldt_Jakob_disease:_neurological_features_and_diagnostic_tests_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0140673697074722 DB - PRIME DP - Unbound Medicine ER -