Volume 8: Variant CJD
Annex 2: Summary tables on the clinical investigative and pathological data on suspect or confirmed cases of CJD aged less than 50 years ¹

Table 1: YOUNG CASES 12/11/95

Age	Name	Hospital	Onset	Dod	Duration	Clinical Features	EEG	Genetics	Pathology	Other details	Class
17					>18	Facial and limb dysaesthesia, tremor. Behavioural change, ataxia, dysarthria, progressive cognitive impairment and myoclonus	Not typical		Biopsy Classical Plaques		1
19					11	Behavioural change. Global dementia plus spasticity. Cortical blindness, pyramidal & cerebellar signs. ?No myoclonus	Not typical		Classical Plaques		1
29					>7	Progressive memory impairment, & ataxia. Supranucleur gaze palsy, akinetic mute, myoclonus, cortical blindness, frontal and pyramidal signs.	Not typical		Biopsy Classical Plaques		1
29					>11	Behavioural change, ataxia and memory impairment. Mute, myoclonus, unresponsive, frontal and pyramidal signs.	Not typical		Nil		3
29					>20	Limb dysaesthesia, ataxia, wt loss, lethargy, dysphasia & dysarthria, memory impairment,& chorea. Now AM, myoclonus, frontal and pyramidal signs.	Not typical		Nil		3
30					7.5	Behavioural change, dysarthria, ataxia, lethargy, & memory impairment. Myoclonus, frontal and pyramidal signs.	Not typical		PM performed		3
38					6	Ataxia, dyscalculia, tremor, progressive memory impairment & myoclonus. AM, startle, paratonic rigidity and grasp.	Typical		PM performed		2
42					>6	Behavioural change, progressive memory impairment, dysphasia, tremor, myoclonus, & ataxia. Paratonic rigidity & frontal signs.	Not typical		Nil		4.1

CLASSIFICATION:

1 = Definite

2 = Probable

3 = Possible

4.1 = Other

Table 2: TABLES OF SUSPECT CASES OF CJD AGED <50 YEARS, SEPT 1995 - MAR 1996
DIAGNOSTIC CLASSIFICATION AT THE TIME OF TABLE COMPILATION AND EVENTUAL CLASSIFICATION

Date of Table	No. of suspect cases	Classification at date of compilation						Final Classification post March 20th 1996
		Def	Def+Plaques	GSS	Prob	Poss	Other	nvCJD	CJD	GSS	Other
27.09.95	5	2	0	0	1	0	2	3	2	0	0
12.11.95	8	0	3	0	1	3	1	6	2	0	0
14.12.95	10	0	3	0	1	3	3	7	2	0	1
07.01.96	9	1	3	0	0	3	2	7	2	0	0
16.01.96	10	1	5	2	0	0	2	7	2	0	1
29.01.96	11	1	7	1	0	0	2	7	2	1	1
07.02.96	12	1	7	1	0	0	3	8	2	1	1
11.03.96	14	1	8	1	0	0	4	10	2	1	1

Table 3:

CONFIRMED CASES OF CJD IN YOUNG PATIENTS WITHOUT CORTICAL PLAQUES (IMMUNOCYTOCHEMICAL STAINING IN EDINBURGH)

AGE	ONSET	DURATION	PATHOLOGY
16		16	CJD no plaques
38		6	CJD no plaques
38		5	CJD no plaques
36		6	CJD no plaques
41		12	CJD plaques in cerebellum

CJD IN ENGLAND AND WALES 1970-1979

AGE	ONSET	DURATION	PATHOLOGY
37		6	CJD
35		25	CJD
34		5	CJD
30		21	CJD
37		23	CJD

CJD IN ENGLAND AND WALES 1980-1989

AGE	ONSET	DURATION	PATHOLOGY
33		10	No PM
37		46	CJD
38		14	CJD
41		11	No PM
36		12	No PM
35		8	Biopsy - CJD

Table 4: AGE DISTRIBUTION OF CJD <44 YEARS

	<30	30-34	35-39	40-44
1970-79	0	1	4	2
1980-84	0	1	2	1
1985-89	0	0	3	3
1990-95	2	1	2	2
1996-	3 1	2	0	1

1 I confirmed case referred in 1995 is still alive.