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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