National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral...

125
THE DIFFICULTIES IN MAKING A DIAGNOSIS OF CJD Richard Knight National CJD Surveillance Unit University of Edinburgh

Transcript of National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral...

Page 1: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

THE DIFFICULTIES IN MAKING A DIAGNOSIS OF CJD

Richard Knight

National CJD Surveillance Unit

University of Edinburgh

Page 2: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

TALK

GENERAL INTRODUCTION

WHAT TIME DOES IT TAKE TO DIAGNOSE SPORADIC CJD ?

COULD THIS TIME BE SHORTER ?

TWO ILLUSTRATIVE HISTORIES

CONCLUSIONS

Page 3: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

GENERAL INTRODUCTION

Page 4: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

PRELIMINARY COMMENT 1

PERSPECTIVE

Page 5: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

DIFFERENT PERSPECTIVES

Page 6: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

DIFFERENT PERSPECTIVES

Page 7: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

PRELIMINARY COMMENT 2

SETTING

Page 8: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4
Page 9: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

PRELIMINARY COMMENT 3

DISEASE TYPE

Page 10: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

CJD

Hans Creutzfeldt Alfons JakobHans Creutzfeldt Alfons Jakob

Page 11: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

DIFFERENT FORMS OF CJD

• GENETIC CJD

• IATROGENIC CJD

• SPORADIC CJD

• VARIANT CJD

Page 12: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

DIFFERENT FORMS: DIFFERING DIAGNOSTIC CONSIDERATIONS

• GENETIC CJD

• IATROGENIC CJD

• SPORADIC CJD

• VARIANT CJD

Page 13: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

DIFFERENT FORMS: DIFFERING DIAGNOSTIC CONSIDERATIONS

• GENETIC CJD

• IATROGENIC CJD

• SPORADIC CJD sCJD

• VARIANT CJD

Page 14: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

3 ESSENTIAL PROBLEMS IN DIAGNOSIS

• RARE DISEASE

• NO SIMPLE, NON-INVASIVE, CLINICAL TEST

• DISEASE: UNTREATABLE & FATAL

Page 15: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

3 ESSENTIAL PROBLEMS IN DIAGNOSIS

• RARE DISEASE

• NO SIMPLE, NON-INVASIVE, CLINICAL TEST

• DISEASE: UNTREATABLE & FATAL

Page 16: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

3 ESSENTIAL PROBLEMS IN DIAGNOSIS

• RARE DISEASE

• NO SIMPLE, NON-INVASIVE, CLINICAL TEST

• DISEASE: UNTREATABLE & FATAL

Page 17: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

THE ESSENTIAL PROBLEMS IN DIAGNOSIS

• RARE DISEASE

MOST DOCTORS HAVE LIMITED EXPERTISE

THE DIAGNOSIS IS INHERENTLY UNLIKELY

Page 18: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

THE ESSENTIAL PROBLEMS IN DIAGNOSIS

• RARE DISEASE

MOST DOCTORS HAVE LIMITED EXPERTISE

THE DIAGNOSIS IS INHERENTLY UNLIKELY

Page 19: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

THE ESSENTIAL PROBLEMS IN DIAGNOSIS

• NO SIMPLE, NON-INVASIVE, CLINICAL TEST

RELIANCE ON CLINICAL EVOLUTION OVER TIME

INVASIVE TESTS CARRY RISKS

Page 20: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

THE ESSENTIAL PROBLEMS IN DIAGNOSIS

• NO SIMPLE, NON-INVASIVE, CLINICAL TEST

RELIANCE ON CLINICAL EVOLUTION OVER TIME

INVASIVE TESTS CARRY RISKS

Page 21: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

THE ESSENTIAL PROBLEMS IN DIAGNOSIS

• DISEASE: UNTREATABLE & FATAL

WANT TO BE CERTAIN OF DIAGNOSIS BEFORE GIVING IT

DESIRE TO GIVE PEOPLE TIME NOT ‘USED’ UP BY TESTS

Page 22: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

THE ESSENTIAL PROBLEMS IN DIAGNOSIS

• DISEASE: UNTREATABLE & FATAL

WANT TO BE CERTAIN OF DIAGNOSIS BEFORE GIVING IT

DESIRE TO GIVE PEOPLE TIME NOT ‘USED’ UP BY TESTS

Page 23: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

ANOTHER PROBLEM

“I DON’T KNOW WHY IT WASN’T DIAGNOSED EARLIER.....

WHEN I READ ABOUT THE DISEASE, THE SYMPTOMS WERE TYPICAL.”

Page 24: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

ANOTHER PROBLEM

“I DON’T KNOW WHY IT WASN’T DIAGNOSED EARLIER.....

WHEN I READ ABOUT THE DISEASE, THE SYMPTOMS WERE TYPICAL.”

BUT THIS IS A RETROSPECTIVE VIEWPOINT

Page 25: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

1

2

3

4

5

6

X

PRESENTING SYMPTOMS MAY SUGGEST SEVERAL CAUSES

Page 26: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

1

2

3

4

5

6

X

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1

2

3

4

5

6

X

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1

2

3

4

5

6

X

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1

2

3

4

5

6

X

TIME

Page 30: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

TIME

SYMPTOMS DIAGNOSIS

Page 31: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHAT TIME DOES IT TAKE ?

SYMPTOMS DIAGNOSISof

sCJD

Page 32: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

‘DELAY’COULD THIS TIME BE SHORTER ?

SYMPTOMS DIAGNOSISof

sCJD

Page 33: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHAT TIME DOES IT TAKE TO DIAGNOSE SPORADIC CJD ?

Page 34: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

TIME

SYMPTOMS DIAGNOSISUK SPORADIC CJD

MEDIAN TIMEONSET-DIAGNOSIS

~3-4 MONTHS

Page 35: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY DOES IT TAKE THIS TIME ?

SYMPTOMS DIAGNOSIS

Page 36: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY DOES IT TAKE THIS TIME ?

SYMPTOMS DIAGNOSIS

‘SOMETHINGWRONG’

Page 37: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY DOES IT TAKE THIS TIME ?

GETMEDICAL

ADVICE

SYMPTOMS DIAGNOSIS

Page 38: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY DOES IT TAKE THIS TIME ?

SYMPTOMS DIAGNOSIS

OUTCOMEOF INITIAL

MANAGEMENT

Page 39: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY DOES IT TAKE THIS TIME ?

SYMPTOMS DIAGNOSIS

SEE SPECIALIST

Page 40: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY DOES IT TAKE THIS TIME ?

SYMPTOMS DIAGNOSIS

RESULTSOF SPECIALIST

TESTS

Page 41: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY DOES IT TAKE THIS TIME ?

SYMPTOMS DIAGNOSIS

CJD POSSIBLE

OR LIKELY

Page 42: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY DOES IT TAKE THIS TIME ?

SYMPTOMS DIAGNOSIS

CONSULTATIONWITH

SURVEILLANCE SYSTEM

Page 43: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY DOES IT TAKE THIS TIME ?

SYMPTOMS DIAGNOSIS

FINAL CLINICAL DIAGNOSIS

Page 44: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

TIME

SYMPTOMS DIAGNOSISUK SPORADIC CJD

MEDIAN TIMEONSET-DIAGNOSIS

~3-4 MONTHS

Page 45: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

COULD THIS TIME BE SHORTER ?

Page 46: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SYMPTOMS DIAGNOSIS

‘SOMETHING

WRONG’

SYMPTOMS MAY BE NON-SPECIFICSYMPTOMS MAY HAVE POSSIBLE EXPLANATION

SOME WAITING IS SENSIBLE & REASONABLE

TIME VARIES FROM PATIENT TO PATIENT

GETMEDICAL

ADVICE

Page 47: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SYMPTOMS DIAGNOSIS

‘SOMETHING

WRONG’

SYMPTOMS MAY BE NON-SPECIFICSYMPTOMS MAY HAVE POSSIBLE EXPLANATION

SOME WAITING IS SENSIBLE & REASONABLE

TIME VARIES FROM PATIENT TO PATIENT

GETMEDICAL

ADVICE

Page 48: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SYMPTOMS DIAGNOSIS

‘SOMETHING

WRONG’

SYMPTOMS MAY BE NON-SPECIFICSYMPTOMS MAY HAVE POSSIBLE EXPLANATION

SOME WAITING IS SENSIBLE & REASONABLE

TIME VARIES FROM PATIENT TO PATIENT

GETMEDICAL

ADVICE

Page 49: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SYMPTOMS DIAGNOSIS

‘SOMETHING

WRONG’

SYMPTOMS MAY BE NON-SPECIFICSYMPTOMS MAY HAVE POSSIBLE EXPLANATION

SOME WAITING IS SENSIBLE & REASONABLE

TIME VARIES FROM PATIENT TO PATIENT

GETMEDICAL

ADVICE

Page 50: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SYMPTOMS DIAGNOSIS

‘SOMETHING

WRONG’

GETMEDICAL

ADVICE

MEDIAN TIME: 2 ¼ WEEKS

[1 DAY-SEVERAL MONTHS]

UK SPORADIC CJD 2006

Page 51: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SYMPTOMS DIAGNOSIS

‘SOMETHING

WRONG’

GETMEDICAL

ADVICE

SEE SPECIALIST

MEDIAN TIME: 6-8 WEEKS

[1 DAY-SEVERAL MONTHS]

UK SPORADIC CJD 2006

Page 52: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SYMPTOMS DIAGNOSIS

‘SOMETHING

WRONG’

GETMEDICAL

ADVICE

SEE SPECIALIST

MEDIAN TIME: 8-12 WEEKS

[FEW WEEKS –SEVERAL MONTHS]

UK SPORADIC CJD 2006

CJD POSSIBLE OR LIKELY

Page 53: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SYMPTOMS DIAGNOSIS

‘SOMETHING

WRONG’

GETMEDICAL

ADVICE

SEE SPECIALIST

MEDIAN TIME: 12-16 WEEKS

[FEW WEEKS –SEVERAL MONTHS]

UK SPORADIC CJD 2008

CJD POSSIBLE OR LIKELY

REFERRAL TONCJDSU

Page 54: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

TIME

SYMPTOMS DIAGNOSISUK SPORADIC CJD

MEDIAN TIMEONSET-DIAGNOSIS

~3-4 MONTHS

Page 55: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

THIS TIME TO DIAGNOSIS IS NOT VERY DIFFERENT FROM THAT FOR MANY NEUROLOGICAL DISEASES

WHY IS IT A PARTICULAR PROBLEM IN sCJD ?

Page 56: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

THIS TIME TO DIAGNOSIS IS NOT VERY DIFFERENT FROM THAT FOR MANY NEUROLOGICAL DISEASES

WHY IS IT A PARTICULAR PROBLEM IN sCJD ?

Page 57: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

sCJD IS TYPICALLY VERY RAPIDLY PROGRESSIVE

Page 58: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

DURATION OF ILLNESS IN sCJD(n=1084, UK)

0

10

20

30

40

50

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70

80

90

100

0-4 5- <8 8- <12 12- <18 18-24 >24

Perc

en

tag

e o

f ca

se

s

Duration of illness in months

Page 59: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

DURATION OF ILLNESS IN sCJD(n=1084, UK)

0

10

20

30

40

50

60

70

80

90

100

0-4 5- <8 8- <12 12- <18 18-24 >24

Perc

en

tag

e o

f ca

se

s

Duration of illness in months

Page 60: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

RATE OF PROGRESSION IN sCJD

RAPID: TYPICAL

• DIAGNOSTIC PROCESS TAKES TIME

• INCREASING & SEVERE DISABILITY & NO DIAGNOSIS

• DIAGNOSIS MAY BE ONLY SHORTLY BEFORE DEATH

Page 61: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SPORADIC CJD in the UKMEDIAN TIME IN MONTHS

2004 2006 2008

ONSET-DEATH 4.5 4.5 4.0

ONSET-DIAGNOSIS 3.95 3.97 3.69

Page 62: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

THERE IS NO SIMPLE, NON-INVASIVE, CLINICAL DIAGNOSTIC TEST

Page 63: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

THERE IS NO SIMPLE, NON-INVASIVE, CLINICAL DIAGNOSTIC TEST

THERE ARE TESTS THAT ARE HELPFUL IN SUPPORTING THE DIAGNOSIS

Page 64: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

AIDS TO DIAGNOSIS FOR sCJD

• EEG

• CSF 14-3-3 PROTEIN TEST

• BRAIN MRI

• PASSAGE OF TIME

Page 65: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

AIDS TO DIAGNOSIS FOR sCJD

• EEG

• CSF 14-3-3 PROTEIN TEST

• BRAIN MRI

• PASSAGE OF TIME

Page 66: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

AIDS TO DIAGNOSIS FOR sCJD

• EEG

• CSF 14-3-3 PROTEIN TEST

• BRAIN MRI

• PASSAGE OF TIME

Page 67: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

AIDS TO DIAGNOSIS FOR sCJD

• EEG

• CSF 14-3-3 PROTEIN TEST

• BRAIN MRI

NONE OF THESE SHOW ABNORMALITIES UNIQUE TO sCJD

Page 68: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

OTHER DIAGNOSES POSSIBLESOME OF WHICH ARE POTENTIALLY TREATABLE

CERTAIN BLOOD TESTS

MRI SCANS

SPINAL FLUID EXAMINATIONS

ALL TAKE TIME TO DO

MAY TAKE TIME FOR RESULTS

FATAL & UNTREATABLE ILLNESSES

Page 69: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

AIDS TO DIAGNOSIS FOR sCJD

• EEG

• CSF 14-3-3 PROTEIN TEST

• BRAIN MRI

• PASSAGE OF TIME

Page 70: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

VITAL FOR DIAGNOSIS OF/EXCLUSION OF

OTHER DISEASES

• EEG

• CSF 14-3-3 PROTEIN TEST

• BRAIN MRI

• PASSAGE OF TIME

Page 71: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

DURATION OF ILLNESS IN sCJD(n=1084, UK)

0

10

20

30

40

50

60

70

80

90

100

0-4 5- <8 8- <12 12- <18 18-24 >24

Perc

en

tag

e o

f ca

se

s

Duration of illness in months

Page 72: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

RATE OF PROGRESSION IN sCJD

SLOWER: ATYPICAL

• OFTEN ATYPICAL CLINICAL FEATURES

• DIAGNOSTIC AIDS (EEG, CSF, MRI) MAY BE LESS HELPFUL

• RARE FORMS OF ALREADY RARE ILLNESS

Page 73: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SYMPTOMS DIAGNOSIS

‘SOMETHING

WRONG’

GETMEDICAL

ADVICE

SEE SPECIALIST

CJD POSSIBLE OR LIKELY

REFERRAL TONCJDSU

DIFFICULT TO SEE HOW COULD (OR SHOULD) TO REDUCE TIME HERE

Page 74: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SYMPTOMS DIAGNOSIS

‘SOMETHING

WRONG’

GETMEDICAL

ADVICE

SEE SPECIALIST

CJD POSSIBLE OR LIKELY

REFERRAL TONCJDSU

WHAT ABOUT HERE ?

Page 75: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY SPECIALIST DIAGNOSIS TAKES TIME

• NON-SPECIFIC PRESENTATION

• RARE ILLNESS: INHERENTLY UNLIKEY

• RARE ILLNESS: UNFAMILIARITY WITH IT

• FATAL & UNTREATABLE: WANT TO BE CERTAIN

• NO SIMPLE DIAGNOSTIC TESTSFATAL & UNTREATABLE ILLNESSES

Page 76: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY SPECIALIST DIAGNOSIS TAKES TIME

• NON-SPECIFIC PRESENTATION

• RARE ILLNESS: INHERENTLY UNLIKEY

• RARE ILLNESS: UNFAMILIARITY WITH IT

• FATAL & UNTREATABLE: WANT TO BE CERTAIN

• NO SIMPLE DIAGNOSTIC TESTSFATAL & UNTREATABLE ILLNESSES

Page 77: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY SPECIALIST DIAGNOSIS TAKES TIME

• NON-SPECIFIC PRESENTATION

• RARE ILLNESS: INHERENTLY UNLIKEY

• RARE ILLNESS: UNFAMILIARITY WITH IT

• FATAL & UNTREATABLE: WANT TO BE CERTAIN

• NO SIMPLE DIAGNOSTIC TESTSFATAL & UNTREATABLE ILLNESSES

Page 78: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY SPECIALIST DIAGNOSIS TAKES TIME

• NON-SPECIFIC PRESENTATION

• RARE ILLNESS: INHERENTLY UNLIKEY

• RARE ILLNESS: UNFAMILIARITY WITH IT

• FATAL & UNTREATABLE: WANT TO BE CERTAIN

• NO SIMPLE DIAGNOSTIC TESTSFATAL & UNTREATABLE ILLNESSES

Page 79: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY SPECIALIST DIAGNOSIS TAKES TIME

• NON-SPECIFIC PRESENTATION

• RARE ILLNESS: INHERENTLY UNLIKEY

• RARE ILLNESS: UNFAMILIARITY WITH IT

• FATAL & UNTREATABLE: WANT TO BE CERTAIN

• NO SIMPLE DIAGNOSTIC TESTSFATAL & UNTREATABLE ILLNESSES

Page 80: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHY SPECIALIST DIAGNOSIS TAKES TIME

• NON-SPECIFIC PRESENTATION

• RARE ILLNESS: INHERENTLY UNLIKEY

• RARE ILLNESS: UNFAMILIARITY WITH IT

• FATAL & UNTREATABLE: WANT TO BE CERTAIN

• NO SIMPLE DIAGNOSTIC TESTSFATAL & UNTREATABLE ILLNESSES

Page 81: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

AWARENESS OF DISEASE

IN THE UK (& ELSEWHERE)

BSE & VARIANT CJD

HAVE INCREASED AWARENESS

OF CJD IN GENERAL

Page 82: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

WHAT DO UK NATIONAL SURVEILLANCE DATA

TELL US ABOUTSPECIALIST DIAGNOSIS ?

Page 83: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

REFERRALS TO NCJDRSU:SUSPECTED CASES

[NOT ALL TURN OUT TO HAVE CJD]

Page 84: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

NUMBER OF REFERRALS TO THE UK NCJDSU 1996-2009

0

20

40

60

80

100

120

140

160

180

199

6

199

7

199

8

199

9

200

0

200

1

200

2

200

3

200

4

200

5

200

6

200

7

200

8

200

9

Page 85: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

NUMBER OF REFERRALS TO THE UK NCJDSU 1996-2009

0

20

40

60

80

100

120

140

160

180

199

6

199

7

199

8

199

9

200

0

200

1

200

2

200

3

200

4

200

5

200

6

200

7

200

8

200

9

Page 86: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

NUMBER OF REFERRALS TO THE UK NCJDSU 1996-2009

0

20

40

60

80

100

120

140

160

180

199

6

199

7

199

8

199

9

200

0

200

1

200

2

200

3

200

4

200

5

200

6

200

7

200

8

200

9

Page 87: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

NUMBER OF REFERRALS TO THE UK NCJDSU 1996-2009

0

20

40

60

80

100

120

140

160

180

199

6

199

7

199

8

199

9

200

0

200

1

200

2

200

3

200

4

200

5

200

6

200

7

200

8

200

9

Page 88: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SPORADIC CJD CASES

ACTUAL CONFIRMED CASES

Page 89: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

UK NCJDSU: NUMBER OF sCJD CASES*1996-2009

0

10

20

30

40

50

60

70

80

90

199

6

199

7

199

8

199

9

200

0

200

1

200

2

200

3

200

4

200

5

200

6

200

7

200

8

200

9

*DEFINITE & PROBABLE

Page 90: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

REFERRAL (SUSPECT) RATES VARIED & FELL

INCREASING NUMBER OF CONFIRMED CASES

WHAT DOES THIS TELL US?

Page 91: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

NUMBER OF REFERRALS TO THE NCJDSU & UK sCJD CASES

1996-2009

0

20

40

60

80

100

120

140

160

180

199

6

199

7

199

8

199

9

200

0

200

1

200

2

200

3

200

4

200

5

200

6

200

7

200

8

200

9

Number of Referrals Number of sCJD cases

Page 92: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

NUMBER OF REFERRALS TO THE NCJDSU & UK sCJD CASES

1996-2009

0

20

40

60

80

100

120

140

160

180

199

6

199

7

199

8

199

9

200

0

200

1

200

2

200

3

200

4

200

5

200

6

200

7

200

8

200

9

Number of Referrals Number of sCJD cases

Page 93: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

NUMBER OF REFERRALS TO THE NCJDSU & UK sCJD CASES

1996-2009

0

20

40

60

80

100

120

140

160

180

199

6

199

7

199

8

199

9

200

0

200

1

200

2

200

3

200

4

200

5

200

6

200

7

200

8

200

9

BETTER RECOGNITION OF CJD CASES BY NEUROLOGISTSREDUCTION IN REFERRAL OF NON-CASES

Page 94: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

UK CLINICIANS HAVE IMPROVED IN DIAGNOSIS OF SPORADIC CJD

AWARENESSEDUCATION

DIAGNOSTIC TESTS*

*CSF 14-3-3 and BRAIN MRI

Page 95: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SPORADIC CJD in the UKONSET-NOTIFICATION

0 0.5 1 1.5 2 2.5 3 3.5 4

2008

1998

1990

MEDIAN TIMES IN MONTHS

Page 96: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SPORADIC CJD in the UKONSET-NOTIFICATION

0 0.5 1 1.5 2 2.5 3 3.5 4

2008

1998

1990

MEDIAN TIMES IN MONTHS

Page 97: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SPORADIC CJD in the UKONSET-NOTIFICATION

0 0.5 1 1.5 2 2.5 3 3.5 4

2008

1998

1990

MEDIAN TIMES IN MONTHS

Page 98: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SPORADIC CJD in the UKONSET-NOTIFICATION

0 0.5 1 1.5 2 2.5 3 3.5 4

2008

1998

1990

MEDIAN TIMES IN MONTHS

Page 99: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

UK CLINICIANS HAVE IMPROVED IN DIAGNOSIS OF SPORADIC CJD

BUT

NOT IMPACTED GREATLY ON

TIME TO NOTIFICATION

Page 100: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

UK CLINICIANS HAVE IMPROVED IN DIAGNOSIS OF SPORADIC CJD

BUT

NOT IMPACTED GREATLY ON

TIME TO NOTIFICATION

AND IN TIME TO DIAGNOSIS

Page 101: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SOME FURTHER INDIRECT EVIDENCE THAT

THE DIAGNOSIS OF SPORADIC CJD IS DIFFICULT

FATAL & UNTREATABLE ILLNESSES

Page 102: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SUSPECTED sCJD REFERRALS SEEN BY NCJDSU in 2008NUMBER DEFINITE or PROBABLE AT TIME SEEN BY NCDSU STAFF

TOTAL: 85

STILL ?

~55% sCJD

NOT CJD

Page 103: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SUSPECTED sCJD REFERRALS SEEN BY NCJDSU in 2008NUMBER DEFINITE or PROBABLE AT TIME SEEN BY NCDSU STAFF

TOTAL: 85

STILL ?

~55% sCJD

NCJDSU STAFF VERY EXPERIENCED IN CJDSTILL UNCERTAIN IN NEARLY 1/4

NOT CJD

Page 104: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SUSPECTED sCJD REFERRALS SEEN BY NCJDSU in 2008NUMBER DEFINITE or PROBABLE AT TIME SEEN BY NCDSU STAFF

13/19 CASES STILL UNCERTAIN AT TIME NCJDSU VISITEDWERE IN FACT sCJD

68% FINALLYDEFINITE or PROBABLE

sCJD

Page 105: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SUSPECTED sCJD REFERRALS TO NCJDSU in 2008FINAL OUTCOME

TOTAL: 135

~64%*

~33%* DEF or PROB

REFERRED SUSPECTS TO NATIONAL SURVEILLANCE

~1/3 TURN OUT NOT TO BE CJD OF ANY KIND

Page 106: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

OTHER DIAGNOSES POSSIBLE(SOME POTENTIALLY TREATABLE)

PASSAGE OF TIME AS DIAGNOSTIC TEST ITSELF

FATAL & UNTREATABLE ILLNESSES

Page 107: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

TWO ILLUSTRATIVE HISTORIES

Page 108: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

83 yr old Man (Definite sCJD)

• 1st seeking advice 3 weeks

• Referred neurologist 4 weeks

• Seen by neurologist 5 weeks

• Admitted to hospital 6 weeks

• Brain MRI 6 ½ weeks

• Notified NCJDSU 8 weeks

• Seen NCJDSU 9 weeks

• Died 11 weeks

Page 109: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

Male 83 (Definite sCJD)CJD first suggested

• 1st seeking advice 3 weeks

• Referred neurologist 4 weeks

• Seen by neurologist 5 weeks

• Admitted to hospital 6 weeks

• Brain MRI 6 ½ weeks

• Notified NCJDSU 8 weeks

• Seen NCJDSU 9 weeks

• Died 11 weeks

Page 110: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

67 yr old Female (Definite sCJD)

• 1st saw a doctor 8 weeks

• Hospital admission 14 weeks

• Referred neurologist 14 weeks

• Seen by neurologist 15 weeks

• Notified to NCJDSU 16 weeks

• Seen by NCJDSU 16 weeks

• Died 20 weeks

Page 111: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

67 yr old Female (Definite sCJD)

• 1st saw a doctor 8 weeks

• Hospital admission 14 weeks

• Referred neurologist 14 weeks

• Seen by neurologist 15 weeks

• Notified to NCJDSU 16 weeks

• Seen by NCJDSU 16 weeks

• Died 20 weeks

Very non-specific early symptoms No cognitive problems until 12 weeks

Page 112: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

67 yr old Female (Definite sCJD)CJD first suspected

• 1st saw a doctor 8 weeks

• Hospital admission 14 weeks

• Referred neurologist 14 weeks

• Seen by neurologist 15 weeks

• Notified to NCJDSU 16 weeks

• Seen by NCJDSU 16 weeks

• Died 20 weeks

Page 113: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

CONCLUSIONS

Page 114: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

CLINICAL DIAGNOSIS IS DIFFICULT

• NON-SPECIFIC PRESENTATION

• OTHER DISEASES NEED TO BE EXCLUDED

• NO SIMPLE DIAGNOSTIC TEST

• PASSAGE OF TIME MAY BE IMPORTANT

Page 115: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

SPORADIC CJD in the UKMEDIAN TIME IN MONTHS

2004 2006 2008

ONSET-DEATH 4.5 4.5 4.0

ONSET-DIAGNOSIS 3.95 3.97 3.69

Page 116: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

COULD THIS TIME BE SHORTER ?

SYMPTOMS DIAGNOSIS

Page 117: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

COULD THIS TIME BE SHORTER ?

SYMPTOMS DIAGNOSIS

PROBABLY NOT AS THINGS STAND

SIMPLE DIAGNOSTIC TEST WOULD HELP

Page 118: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

COULD THIS TIME BE SHORTER ?

SYMPTOMS DIAGNOSIS

PROBABLY NOT AS THINGS STAND

SIMPLE DIAGNOSTIC TEST WOULD HELP

Page 119: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

DIAGNOSIS TAKES TIME

UNCERTAINTY

Page 120: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

DIAGNOSIS TAKES TIME

UNCERTAINTY

AFFECTS

PATIENT

FAMILY & FRIENDS

CLINICAL STAFF

Page 121: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

DIAGNOSIS TAKES TIME

UNCERTAINTY

AFFECTS

PATIENT

FAMILY & FRIENDS

CLINICAL STAFF

Page 122: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

STAFF OF THE NCJDSUREFERRING DOCTORS

PATIENTS & THEIR FAMILIES

Page 123: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4
Page 124: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

STAFF OF THE NCJDSUREFERRING DOCTORS

PATIENTS & THEIR FAMILIES

Page 125: National CJD Surveillance Unit University of Edinburgh 2011.pdf · cjd possible or likely referral to ncjdsu. time symptoms diagnosis uk sporadic cjd median time onset-diagnosis ~3-4

STAFF OF THE NCJDSUREFERRING DOCTORS

PATIENTS & THEIR FAMILIES