TABLE 1. Human prion diseases

Etiologic categories Traditional namea
Infectious prion diseases Kuru
latrogenic CJD
Inherited prion diseases
Inherited prion disease GSSb
(P102L)
Inherited prion disease GSSc
(A117V)
Inherited prion disease Familial CJDd, FFIe
(D178N)
Inherited prion disease

(F198S)

GSS-nftf
Inherited prion disease Familial CJDg
(E200K)
Inherited prion disease GSS-nfth
(Q217R)
Inherited prion disease

(4 octarepeat insert)

Familial CJDi
Inherited prion disease

(5 octarepeat insert)

Familial CJDi
Inherited prion disease

(6 octarepeat insert)

Familial CJDi
Inherited prion disease

(7 octarepeat insert)

Familial CJDi
Inherited prion disease

(8 octarepeat insert)

Familial CJDi
Inherited prion disease

(9 octarepeat insert)

Familial CJDi
Sporadic prion disease CJD

a CJD, Creutzfeldt-Jakob disease; GSS, Gerstmann-Sträussler-Scheinker syndrome; GSS-nft, GSS with neurofibrillary tangles; FFI, fatal familial insomnia.

b Ref. 41.

c Refs. 22 and 44

d Refs. 6 and 37.

e Refs. 37 and 51.

f Refs. 21.

g Refs. 28, 29, 35 and 39.

h Ref. 42.

i Refs. 38, 54, and 57.

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published 2000