Creutzfeldt-Jakob disease in a husband and wife

P Brown; L Cervenáková; L McShane; L G Goldfarb; K Bishop; F Bastian; J Kirkpatrick; P Piccardo; B Ghetti; D C Gajdusek

Creutzfeldt-Jakob disease in a husband and wife

P Brown, L Cervenáková, L McShane, L G Goldfarb, K Bishop, F Bastian, J Kirkpatrick, P Piccardo, B Ghetti, D C Gajdusek

Royal (Dick) School of Veterinary Studies

Research output: Contribution to journal › Article › peer-review

Abstract

A 53-year-old man died of sporadic Creutzfeldt-Jakob disease (CJD) after a 1.5-year clinical course. Four and a half years later, his then 55-year-old widow died from CJD after a 1-month illness. Both patients had typical clinical and neuropathologic features of the disease, and pathognomonic proteinase-resistant amyloid protein ("prion" protein, or PrP) was present in both brains. Neither patient had a family history of neurologic disease, and molecular genetic analysis of their PrP genes was normal. No medical, surgical, or dietary antecedent of CJD was identified; therefore, we are left with the unanswerable alternatives of human-to-human transmission or the chance occurrence of sporadic CJD in a husband and wife.

Original language	English
Pages (from-to)	684-8
Number of pages	5
Journal	Neurology
Volume	50
Issue number	3
Publication status	Published - Mar 1998

Keywords / Materials (for Non-textual outputs)

Animals
Blotting, Western
Brain
Creutzfeldt-Jakob Syndrome
Cricetinae
Disease Transmission, Infectious
Drug Resistance
Endopeptidases
Family Health
Humans
Male
Middle Aged
Mutation
Prions
Reference Values
Spouses

Cite this

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title = "Creutzfeldt-Jakob disease in a husband and wife",

abstract = "A 53-year-old man died of sporadic Creutzfeldt-Jakob disease (CJD) after a 1.5-year clinical course. Four and a half years later, his then 55-year-old widow died from CJD after a 1-month illness. Both patients had typical clinical and neuropathologic features of the disease, and pathognomonic proteinase-resistant amyloid protein ({"}prion{"} protein, or PrP) was present in both brains. Neither patient had a family history of neurologic disease, and molecular genetic analysis of their PrP genes was normal. No medical, surgical, or dietary antecedent of CJD was identified; therefore, we are left with the unanswerable alternatives of human-to-human transmission or the chance occurrence of sporadic CJD in a husband and wife.",

keywords = "Animals, Blotting, Western, Brain, Creutzfeldt-Jakob Syndrome, Cricetinae, Disease Transmission, Infectious, Drug Resistance, Endopeptidases, Family Health, Humans, Male, Middle Aged, Mutation, Prions, Reference Values, Spouses",

author = "P Brown and L Cerven{\'a}kov{\'a} and L McShane and Goldfarb, \{L G\} and K Bishop and F Bastian and J Kirkpatrick and P Piccardo and B Ghetti and Gajdusek, \{D C\}",

year = "1998",

month = mar,

language = "English",

volume = "50",

pages = "684--8",

journal = "Neurology",

issn = "0028-3878",

publisher = "Lippincott Williams and Wilkins",

number = "3",

}

TY - JOUR

T1 - Creutzfeldt-Jakob disease in a husband and wife

AU - Brown, P

AU - Cervenáková, L

AU - McShane, L

AU - Goldfarb, L G

AU - Bishop, K

AU - Bastian, F

AU - Kirkpatrick, J

AU - Piccardo, P

AU - Ghetti, B

AU - Gajdusek, D C

PY - 1998/3

Y1 - 1998/3

N2 - A 53-year-old man died of sporadic Creutzfeldt-Jakob disease (CJD) after a 1.5-year clinical course. Four and a half years later, his then 55-year-old widow died from CJD after a 1-month illness. Both patients had typical clinical and neuropathologic features of the disease, and pathognomonic proteinase-resistant amyloid protein ("prion" protein, or PrP) was present in both brains. Neither patient had a family history of neurologic disease, and molecular genetic analysis of their PrP genes was normal. No medical, surgical, or dietary antecedent of CJD was identified; therefore, we are left with the unanswerable alternatives of human-to-human transmission or the chance occurrence of sporadic CJD in a husband and wife.

AB - A 53-year-old man died of sporadic Creutzfeldt-Jakob disease (CJD) after a 1.5-year clinical course. Four and a half years later, his then 55-year-old widow died from CJD after a 1-month illness. Both patients had typical clinical and neuropathologic features of the disease, and pathognomonic proteinase-resistant amyloid protein ("prion" protein, or PrP) was present in both brains. Neither patient had a family history of neurologic disease, and molecular genetic analysis of their PrP genes was normal. No medical, surgical, or dietary antecedent of CJD was identified; therefore, we are left with the unanswerable alternatives of human-to-human transmission or the chance occurrence of sporadic CJD in a husband and wife.

KW - Animals

KW - Blotting, Western

KW - Brain

KW - Creutzfeldt-Jakob Syndrome

KW - Cricetinae

KW - Disease Transmission, Infectious

KW - Drug Resistance

KW - Endopeptidases

KW - Family Health

KW - Humans

KW - Male

KW - Middle Aged

KW - Mutation

KW - Prions

KW - Reference Values

KW - Spouses

M3 - Article

C2 - 9521256

SN - 0028-3878

VL - 50

SP - 684

EP - 688

JO - Neurology

JF - Neurology

IS - 3

ER -

Creutzfeldt-Jakob disease in a husband and wife

Abstract

Keywords / Materials (for Non-textual outputs)

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