Table 4 - Sporadic CJD Untreated Patient Series
Supplemental Data: Tables e-1 to e-5
Table e-1: Sporadic CJD Untreated Patient Series
Country
|Author Year (Ref) [1] |Study type/
Cohort definition |Study
period |Detailed location |Number of patients [2]
Strength of diagnosis
Designation sCJD
Codon 129 genotype |Median age at onset [3]
(range)
% male |Median age at death (range) |Median duration (range) | | | | | | | | | | | |Argentina |Taratuto 1989(1) [4] |Unclear whether prospective or retrospective.
Case series, definition unclear. |1980 - 1987 |- |9
Definite
Series also included 1 iPD which has been removed from tabulated data
-
|60
(45-63)
M: 67% |-
- |7 months
(3.5-24) | |Australia
|Klug 2004(2)
Same cohort as/Supersedes
[Collins 2002a(3)]
Collins 2002b(4)
Boyd 2001(5)
Collins 1999(6)
|Retrospective & prospective
National surveillance study.
Cases identified by searching national death index and contacting all Australian neurologists and pathologists. iPD and iCJD from same study reported in relevant tables. |1970 – 2003
Retrospective 1970-1993.
Prospective from 1993.
|National |417
Definite: 244
Probable: 168
Possible: 5
Stated as sporadic
129MM: 20
129MV: 6
VV: 4 |-
-
M: 48% |66
(25-89) |4 months
(0.9-60) | |Austria & Germany
|Hainfellner 1998(7)
Same cohort as/Supersedes
[Hainfellner 1996a(8)]
Hainfellner 1996b(9) [5]
Radbauer 1998(10) [6]
Jellinger 1972(11)
Garzuly 1971(12)
Possible overlap
Kropp 1999(13) |Retrospective
Combination of series of 80 consecutive patients diagnosed Austria 1969-1995 (reported separately Hainfellner 1996) plus 30 consecutive patients from single German institution diagnosed 1983 –1995. Unable to separate datasets.
Aimed to explore coexistence of Alzheimer neuropathology. Also compared with matched controls. |1969 - 1995 |National Austria plus
Institute Neuropath
Munster |110
Definite
States no patient with unequivocal family history, assume sporadic
- |-
-
M: 50%
(No Alzheimer’s
Pathology)
n=98
-
-
M: 25%
n=12
(Alzheimer’s pathology)
|64
(27-80)
n=98
72
(64-82)
n=12 |5 months
(1-30)
n=110
| |Belgium
|Van Everbroeck 2004(14) |Prospective
Patients confirmed with definite CJD from series of 250 national referrals to reference laboratory for CSF testing during specified period.
Aimed to compare patients with definite CJD to 79 diagnosed with Alzheimer’s disease, dementia with Lewy bodies and vascular dementia. Patients with other diagnoses excluded. |1998 - 2001 |National referral laboratory |52
Definite
States 3 hereditary cases were excluded, assume sporadic
- |66
(31-82)
M: 58% |-
- |6 months
(1-36) | | |Pals 1999(15)
Same cohort as/Supersedes
Van Everbroeck 2000(16) [7]
Costa 1998(17)
|Retrospective
All Belgian patients identified from search of archives of all Belgian neuropathology laboratories. 12 patients referred from other countries were excluded. |1961 - 1998 |National |100
Definite: 86
Probable: 1
Pending: 13
96% sCJD
4% iPD
- |-
-
M: 33% |Mean
63 ( 9
(30-83) |4 months
(2-108) | |Brazil |Marchiori 1996(18) |Unclear whether retrospective or prospective
Patient series from single institution. |1974 - 1995 |Neurologic Clinic
Sao Paulo University School of Medicine |13
Definite: 9
Probable: 2
Pending: 2
Series also included 1 iPD which has been removed from tabulated data
- |62
(24-76)
M: 50%
|-
- |6 months
(3.5-34) | |Chile |Galvez 1983(19) [8]
Same cohort as/Supercedes
Galvez 1987(20)
Galvez 1982(21)
Galvez 1980(22)
|Cases treated by authors and also by direct contact with neurologists throughout country and review of published cases.
Paper also describes 13 iPD reported in relevant table. |1955 – 1983 [9]
|National |48
Definite: 38
Probable: 10
States no evidence of iPD, assume sporadic
- |-
-
M: 54% |Mean
54.9 ± 9.6
-
n=38 definite |Mean
6.8 ( 4.4 months
-
| |China
[In Chinese] |Guo 1989(23) |Unclear whether retrospective or prospective
Clinicopathological study. |- |Beijing and Qingdao |3
Definite
States no family history for 3 patients, assume sporadic. One patient with positive family history excluded from tabulated data.
- |56
(53-58)
M: 100% |-
- |9 months
(4-12.5) | |Finland |Kovanen 1993(24) [10]
Supercedes
Kovanen 1988(25) |Retrospective & prospective
All cases of sCJD in Finland in specified period.
Paper also describes 12 familial cases and contrasts clinical characteristics. iPD presented in relevant table. |1974 - 1989 |National |32
Definite
Stated as sCJD
- |62.5
(50-81)
M: 38% |63
(51-81) |4.5 months
(0.5-25) | |France |Alpérovitch 26)
Supersedes
Capek 2003(27)
d'Aignaux 2000(28)
Brandel 1994(29)
|Prospective
National surveillance study
Paper reports on all CJD in specified period. |1992 - 2002 |National |805
Definite & probable
Stated as sCJD
129MM: 515
129MV: 137
VV: 153
|Mean
68.4
(24-90)
M: 45% |-
- |6.5 months
(1-75) | | |Brown 1986(30)
Same cohort as/Supersedes
Brown 1987(31) [11]
Cathala 1985(32)
Brown 1979a(33)
Brown 1979b(34)
Brown 1979c(35)
Cathala 1979(36)
Overlap
Brown 1994b(37)
Aguglia 1987(38)
|Retrospective & prospective
Survey of all neurologists, neuropathologists & electroencephalographers in France covering 1968-1977. Prospective from 1978.
|1968 - 1982 |National |230
Definite
96% sCJD
4% iPD
- |Mean
61.5 ± 9.7
(19-83)
M: 45% |-
- |4 months
(?–120) | | |Aguglia 1987(38)
Possible overlap
Brown 1986(30) |Retrospective
20 patients with EEG paroxysmal activity seen in single institution. Criteria for case selection not clear.
|1963 - 1985 |Service d’explorations fonctionelles du systeme nerveux. Timone Marseille |20
95% sCJD
5% iPD
- |Mean
63 ± 9.5
(45-79)
- |-
- |Mean
5.4 ± 4.7 months
(1.4-18.4)
| |Germany |Tschampa 2002(39)
Possible overlap
Kropp 1999(13) |Confirmed cases sent to German reference centre.
Case control study of thalamic pathology.
|-
|National |21
Definite
Stated as sporadic
129MM: 12
129MV: 3
VV: 6
|-
-
M: 71% |65
(25-76) |6.8 months
(1.4-20.1) | |[In German] |Geiger 2002(40)
Supercedes
Krucke 1973(41) [12] |Retrospective
Case series |1954 - 1997 |-
Authors based Munich & Leipzig |22
Definite
Stated as sporadic
- |-
-
M: 46% |58
(32–79)
n=21 |4.5 months
(1-15) | | |Kropp 1999(13)
Supersedes
Meissner 2004(42) [13]
Poser 1999(43)
Otto 1998(44)
Possible overlap
Geiger 2002(40)
Hainfellner 1998(7)
|Prospective
All cases sporadic CJD from National surveillance reference centre in defined period.
Aim of study was to characterize Heidenhain variant. |1993 - 1997 |National
|169
Definite
Stated as sCJD
Heidenhain: 25
129MM: 11
Other:144 |Mean
Heidenhain
67.7
(51-76)
-
n=25
Other
65.7
(31-87)
-
n=144
|-
- |Mean
Heidenhain
5.7 months
(2.3-14.1)
n=25
Other
7.5 months
(1.3-32.4)
n=144 | |[In German] |Meyendorf 1973(45)
|Retrospective
Case series |1964 - 1968 |Munich
Neurology Clinic, Munich University
|3
-
States no family history for 3 patients, assume sporadic. One patient with positive family history not included.
- |64
(62-64)
M: 0% |-
- |4 months
(1-5) | |Hungary
[In Hungarian] |Majtenyi 1996(46) |Brains /cases collected in specified period. English summary suggests this represents all (definite) Hungarian patients during this period.
|1960 - 1996 |National |109
Definite
5.5–10% iPD
- |Mean
60
-
- |-
(41-?) |Mean
6.7 months
(?–60) | |India |Satishchandra 1991(47) |Retrospective
Survey/ direct contact with all neurologists, neurosurgeons, neuropathologists in India.
|1971 - 1990 |National
Cases reported from 8 centers, 70% cases from Bombay & Bangalore |30
Definite: 20
Probable: 10
States no familial cases, assume sporadic
- |Mean
54.6 ( 10.1
(34-76)
M: 60% |-
- |Mean
8 ( 10.2 months
(1-36)
n=26 | |Ireland |Horan 2004(48) |Retrospective & prospective
Retrospective clinical/pathological surveillance at two hospitals. 1980-1998. Prospective National surveillance from 1998. |Retrospective
1980 - 1998
Prospective
1999 - 2002 |Beaumont and Cork University hospitals, then national surveillance |43
Definite: 30
Probable: 13
94% sCJD
2% iPD
2% iCJD
2% FFI
Data reported on 37/43 patients[14]
-
|Mean
61
(33-79)
n=24
M: 64%
n=13 |Mean
61
(33-77)
n=13 |Mean
-
(1.5-66 months)
n=16
3.7 months
(1.4-9.3)
n=8
5 months
(2-12)
n=13
| |Italy |Puopolo 2003(49)
Supercedes
Arpino 1999(50) |Prospective
National surveillance all definite cases (neuropathologically confirmed) that died 1993-2000 |1993 - 2000 |National |382
Definite
Series also included 1 possible case that has been removed from the tabulated data
-
129MM: 151
129MV: 30
129VV: 27 |-
-
M: 43% |Mean
Male
65.3 ± 8.5
-
Female
67.3 ± 8.2
-
Combined
66.4
- |Mean
Male
5.4 ± 4.9 months
-
Female
7.0± 6.1 months
-
Combined
6.3 months
-
Median
129MM: 4
129MV: 7
129VV: 6 | | |Trabattoni 1990(51)
Supercedes
Lechi 1983(52)
|Unclear if prospective or retrospective
Cases evaluated by authors (unclear if all observed directly or from notes) in loosely defined period. Earliest patient predates period where notification of cases requested. |1975 - 1984
|Neurological clinics hospitals of Piacenza, Mantua, Modena & Parma |13
Definite: 11
Probable: 2
States no relationship between patients and no family history of neurological or psychiatric illness, assume sporadic
- |Mean
62.2 ± 7.6
(45-78)
M: 38% |-
- |Mean
4 months
(1-10) | |
|Masullo 1988(53)
Possible overlap
Brown 1994b(37) [15]
Lechi 1983(52) |Retrospective
Cases referred to authors’ institutes & from survey of the literature (1972-1986). Cannot separate data.
|1972 - 1986 |Istituto Neurologia, Universita Cattolica S. Cuore, Rome. Dipartmento di Biologia, Universita di Lechi. Divisione di Neurologia, S. Filippo Neri Hospital, Rome.
|79
Definite: 54
Probable: 25
State no familial cases, assume sporadic
- |-
-
M: 28% |62.5
(38-81)
n=78 |5 months
(1-18)
n=77 | | |Tabaton 1981(54) |Unclear if prospective or retrospective
Notification requested from neurologists, psychiatrists and neurosurgeons in the single centre. Survey of medical records did not reveal any additional cases.
|1974 - 1979 |Genoa |6
Definite: 5
Probable: 1
States no dementia observed in families, assume sporadic
- |-
-
M: 33% |62
(41-69) |5 months
(3-19) | |[In Spanish] |Alema 1971(55) |Retrospective
Consecutive cases some noted as published others as from archives. Unclear whether publication is source of data or incidental.
|1943 - 1970 |Ascoli, Latium, Naples Parma, Rome, Venice. |25
Definite
Excluded 2 familial cases, assume remainder sporadic. [16]
- |-
-
M: 48% |62
(40-71) |6 months
(1.5- 45) | |Japan |Shintaku 2004(56) |Series of 8 consecutive cases of sCJD identified from previous 35 years autopsy reports at 2 hospitals.
|1978 - 2003 |Osaka Red Cross Hospital;
National Cardiovascular Center, Suita |8
Definite
Stated as sporadic
- |70.5
(61-77)
M: 50% |-
- |17 months
(7-45) | | |Matsusue 2004(57) |Retrospective
Analysis of serial MRI images. Cohort definition unclear |- |-
(Authors fromTottori University, Yonago) |6
Definite
Stated as sporadic
129VV: 2 |72
(64-82)
M: 17% |-
- |23.5 months
(8-30) | | |Fukushima 2004(58) |Retrospective
MRI analysis of the 2 patients (out of 123) recognized as VV2 by the Japanese Surveillance Committee.
|- |National |2
Definite
Stated as sporadic
129VV: 2 |72
(69,75)
M: 50% |-
- |9 months
(7,11) | | |Akai 1989(59)
Possible overlap
Tsuji 1983a(60) |Patients with CJD in Yamanshi Prefecture identified by epidemiological survey.
Paper also describes 3 iPD patients reported in iPD table
|1976 - 1986 |Regional
Yamanashi Prefecture |6
Definite: 4
Probable:2
Stated as sCJD
- |-
-
M: 83% |59.5
(53-69) |10 months
(4-18) | |[In Japanese][17] |Mizutani 1984(61)
Supercedes
Yagishita 1981(62)
Possible Overlap
Shintaku 2004(56)
Matsusue 2004(57)
Akai 1989(59)
Tsuji 1983a(60)
|Review of autopsy cases in Japan |1961 - 1981 |- |97
Definite
State no familial cases, assume sporadic
- |-
-
- |55.6
- |17.6 months
- | | |Tsuji 1983a(60) [18]
Same cohort as/Supersedes
Tsuji 1983b(63)
[in Japanese]
Kondo 1982(64) |Nationwide survey of 902 neurologic and psychiatric institutions (51% reply rate). |1975 - 1978 |National |75
Definite: 38
Probable: 6
Alive: 31 (at time of survey)
States no iatrogenic transmission, 4 iPD not included as they died before 1975
- |Mean
55.7 ± 11.1
(25-77)
M: 44% |-
- |Mean
16.6 ( 12.8 months
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