Progressive Supranuclear Palsy: A Survey of the Disease Course

(supplement)

 

Pilar Santacruz, M.A. § , Bob Uttl, Ph.D. ª , Irene Litvan, M.D. ¨ , & Jordan Grafman, Ph.D.§

 

§ Cognitive Neuroscience Section, NINDS, NIH

ª Henry M. Jackson Foundation and Cognitive Neuroscience Section, NINDS, NIH

¨ Neuropharmacology Unit, Henry M. Jackson Foundation and Medical Neurology Branch, NINDS, NIH

 

The following table (Table 1) shows, for the living patients only, Kaplan-Meier product estimates of the proportions of PSP patients affected with each symptom/sign by yearly intervals starting 5 years prior to diagnosis. The symptoms and signs are ordered by problem area and by proportion of patients who experienced the symptoms at the time of diagnosis (i.e., 0 years from diagnosis). The shaded portion represents the clinical stage of the disease – from the estimated time of the principal symptoms onset until 5 years after the diagnosis (the median principal symptom onset to diagnosis time lag was 2.1 years, thus, the symptom onset can be placed approximately 2 years ahead of diagnosis). To illustrate, the table shows that problems with balance appeared in 16% of patients 4 years prior to diagnosis, 28% of patients 3 years prior to diagnosis, 41% of patients 2 years prior to diagnosis (about the time of principal symptoms onset), 55% of patients 1 year prior to diagnosis, 72% of patients at the time of diagnosis, and 96% of patients who survived for 5 years following the diagnosis.

 

The stressed typography in the table permits one to see in a glance how various symptoms progress during the course of the disease because increasing proportions of patients affected with each symptom are shown in increasingly large font sizes, in underlined and in bold print according to the following scheme:

p < .25 8pt font

.25 £ p < .50 10pt font, underlined

.50 £ p < .75 10pt font, underlined, bold

.75 £ p 12pt font, underlined, bold

 

 

 

 

Table 1

Kaplan-Meier product limit estimates of proportions of PSP patients affected with each symptom/sign as a function of time elapsed from the diagnosis, for the living patients only. Table also includes proportion of patients affected by each symptom (separately for living and deceased samples).

Yearly intervals prior and after the diagnosis

Living

Deceased

Symptom/Sign

-4

-3

-2

-1

0

1

2

3

4

5

M

SD

M

SD

Motor/Physical

Loss of balance

.16

.28

.41

.55

.72

.81

.85

.91

.95

.96

.91

.29

.97

.18

Falls

.12

.22

.33

.50

.69

.79

.87

.89

.93

.95

.90

.30

.95

.22

Deteriorating handwriting

.10

.17

.27

.41

.63

.77

.86

.92

.95

.96

.94

.25

.98

.13

Slow to perform daily activities

.08

.11

.23

.36

.58

.73

.82

.87

.92

.94

.89

.31

.95

.21

Fatigue

.07

.10

.18

.29

.46

.61

.69

.76

.78

.80

.74

.44

.80

.40

Slurred speech

.04

.10

.14

.26

.43

.63

.74

.83

.87

.87

.82

.38

.86

.35

Loss of strength

.03

.09

.16

.27

.43

.55

.59

.60

.65

.69

.63

.48

.80

.40

Stiffness in the neck

.06

.08

.13

.24

.40

.52

.61

.70

.76

.80

.67

.47

.84

.36

Dizziness

.07

.11

.15

.24

.36

.42

.44

.45

.48

.51

.46

.50

.64

.48

Coughing when eating

.02

.03

.06

.13

.32

.48

.63

.74

.81

.84

.74

.44

.91

.28

Difficulty swallowing

.03

.03

.07

.10

.28

.45

.58

.72

.77

.85

.72

.45

.96

.18

Back or neck pain or injury

.09

.11

.13

.18

.24

.33

.38

.41

.44

.50

.41

.49

.44

.50

Muscle cramps or spasms

.06

.09

.11

.16

.24

.29

.35

.40

.46

.49

.39

.49

.48

.50

Shakes, tremors or jerks

.06

.07

.11

.13

.22

.31

.39

.43

.46

.51

.40

.49

.44

.50

Abnormal hand posture

.02

.04

.08

.12

.21

.33

.42

.47

.51

.54

.44

.50

.50

.50

Abnormal leg posture

.01

.02

.04

.08

.17

.22

.27

.33

.35

.43

.32

.47

.41

.49

Loss of consciousness

.01

.01

.01

.02

.05

.06

.08

.09

.09

.12

.09

.28

.07

.26

Vision

Can't move his/her eyes down

.02

.06

.09

.19

.48

.65

.74

.81

.87

.90

.79

.41

.96

.21

Difficulty to read

.03

.07

.14

.26

.44

.65

.74

.82

.84

.91

.81

.39

.91

.29

Blurred vision

.05

.09

.17

.31

.44

.54

.60

.67

.68

.77

.65

.48

.80

.41

Double vision

.05

.08

.10

.19

.32

.39

.45

.48

.48

.55

.44

.50

.57

.50

Personality/Emotion

Changes in personality

.10

.17

.23

.33

.46

.56

.60

.65

.69

.72

.64

.48

.72

.45

Depression

.08

.12

.17

.27

.44

.56

.60

.64

.64

.66

.63

.48

.70

.46

Losing control of emotions

.04

.06

.12

.16

.27

.35

.42

.47

.52

.55

.45

.50

.54

.50

Lack of emotions

.04

.06

.09

.17

.25

.33

.39

.47

.55

.59

.45

.50

.52

.50

Excessive anger

.03

.04

.07

.11

.18

.20

.23

.25

.25

.28

.24

.43

.32

.47

Cognitive

Difficulty concentrating

.04

.07

.14

.22

.39

.54

.64

.72

.77

.78

.73

.44

.76

.43

Word finding difficulty

.04

.08

.11

.19

.35

.53

.61

.68

.73

.76

.69

.46

.73

.45

Failing memory

.04

.06

.10

.15

.25

.37

.48

.53

.61

.63

.53

.50

.57

.50

Other

Changed sleeping patterns

.08

.11

.15

.26

.38

.49

.57

.66

.67

.71

.62

.49

.77

.42

Difficulty in sleeping

.05

.10

.13

.20

.31

.41

.44

.51

.53

.58

.48

.50

.57

.50

Bowel/bladder function

.05

.08

.10

.15

.30

.44

.49

.66

.69

.75

.60

.49

.78

.41

Eating too quickly

.03

.07

.09

.15

.25

.33

.39

.40

.42

.44

.40

.49

.35

.48

Eating too much at once

.01

.05

.08

.13

.22

.34

.40

.44

.46

.50

.43

.50

.36

.48

Ringing or loss of hearing

.06

.10

.13

.16

.21

.25

.26

.30

.32

.32

.30

.46

.27

.44

Loss of sensation anywhere

.01

.03

.04

.06

.10

.14

.15

.19

.21

.21

.19

.39

.25

.44

Tingling or burning

.03

.05

.05

.08

.10

.15

.20

.22

.23

.27

.22

.41

.19

.40

 

Figure 1. The figure 1 shows selected data from Table 1; It shows Kaplan-Meier product estimates of the proportions of PSP patients affected with each symptom/sign by yearly intervals starting 5 years prior to diagnosis. Only data for two leading symptoms and signs from each problem area in Table 1 are shown in the figure.

 

 

 

 

 

Author notes

The contribution of the first two authors was equal and their names appear in the order determined by a coin flip. Pilar Santacruz was supported by a grant from La Caixa, Barcelona, Catalonia, Spain during her two year fellowship in the Cognitive Neuroscience Section.

 

For the full report see: Santacruz P, Uttl B, Litvan I, Grafman J. Progressive supranuclear palsy: A survey of the disease course. Neurology, 1998; June, in press.

 

We were required to remove the Table 1 from our manuscript as a condition of Neurology publishing the manuscript.