1. Osteoporosis in Adults with Cerebral Palsy
AAPMR November 2014
Christina Marciniak, Joelle Gabet, JungWha Lee and Nicole Wyoscki
The Rehabilitation Institute of Chicago and Northwestern University

2. No authors have any relevant disclosures

3. Background: Children with CP and Bone Mineral Density
Low bone mineral density (BMD) has consistently been shown for children with cerebral palsy compared to non-disabled, and differences are increased as children age (Henderson, 2005)
Predictors of BMD:
Level of ambulation
Nutritional status
Body mass index (Henderson,2004)
Distal femur site reference values and techniques have been developed.
Longitudinal studies of children with CP and subsequent rate of change of BMD in adulthood have not been reported.
Studies in children with osteoporosis have shown response to bisphosphonates, but long term safety is unknown.

4. IN children with CP, fractures have been reported in 16-26%
Upper Limb – 14%
Axial – 4%
Sixty-six percent of patients had spastic quadriplegia, of whom 83% were nonambulatory.
Risk Factors:
anticonvulsant therapy
spastic quadriplegia nonambulatory, osteopenic
Femur 48%
Restrospective study of 156 children with CP with fracturesCompared to age and gender matched children, fractures group showed a statistically significant difference for
anticonvulsant therapy (P=0.001), CP pattern (P=0.005),
ambulatory status (P=0.001), and osteopenia (P=0.001)
Percentage of children
With CP and fractures:
12-26%
(Leet, Henderson
Tibia – 27%
Foot – 7%
Presedo, J Pediatr Orthop. 2007

5. Dual Energy X-ray Absorptiometry
BMD = grams/centimeter squared
Relative Risk of hip fracture increases by 2.5 for every SD decrease in femoral neck BMD.
T score = SD more or less than a young adult with same sex
Osteopenia = T score between -2.5 to -1
Osteoporosis is less than or equal to -2.5 (expert consensus)
Recommendations based on T scores and fracture risk (FRAX)
FRAX index used to calculate the 10 year pobabiliy of hip fracture and major osteoporotic fracture (clinical vertebral, hip forearm or proximal hureral fracture)

6. T vs. Z scores
Z Score = Number of standard deviations more or less than a same age reference mean
Z scores are used in adults less than 50
Treatment recommendations have been based on Z:- 2.0 and significant fracture

7. BACKGROUND: ADULTS WITH CP and BMD
King (2003) L-spine 48 non-ambulatory children/ adults
- Ages years (19 adults)
- Average z =2.37
- 58% had z scores lower than expected.
Yoon (2012) : 38 adults (mean age 35 years) Only 6 subjects with spastic quadriplegia, 12 non-ambulatory.
Average T score L spine and Hip -1.5 (No Z scores reported)
BMD was not associated with gender, age and subtype of CP.
BMI correlated with BMD of L spine and femur
No difference in L spine for walker vs. non-walker
Higher ambulatory function positively correlated with BMD of femur.
Yoon – no difference with quadriplegia vs diplegia
Living well

8. Current Screening recommendations
National Osteoporosis Foundation:
Woman 65 and old and men 70 and older
Younger peri-menopausal and men with risk factors
Adults with fracture after age 50
Adults with medical condition or medication associated with low bone mass or bone loss
Do NOT list CP under neurological conditions with risk for osteoporosis, though do list impaired transfers and mobility
Canadian Guidelines:
Women and men 65 years and older
Women and men with risk factors
Younger men or women (under 50) with a disease or condition associated with low bone mass or bone loss/ Fragility fractures
List SCI, MS, Muscular dystropy, stroke

9. Changes in BMD with age
Adults with CP lose function at an earlier age.

10. OUR Study Objective: Design: Retrospective review study
To identify factors associated with low bone mineral density (BMD) in adults with cerebral palsy (CP)
To assess for longitudinal BMD changes 
Design: Retrospective review study
Participants: Adults with CP seen in an adult physiatry clinic over a two 1/2-year period and who had at least one dual-energy X-Ray absorptiometry (DXA) scan to assess bone health status over a 7 year time frame
Recommendations for scans clinically were made in adults GMFCS III IV and V, and I and II with fractures or frequent falls.

11. Hypothesis: Osteoporosis in Individuals with CP
Low bone mineral density will be seen with high frequency in adults with CP.
Z scores will decrease with age
Lower mobility levels will correlate with lower BMD at hip sites, but not L spine locations.

12. Measures: Bone Mineral Density Baseline and change
3 sites: L spine, total hip and femoral neck
T scores
Z scores
Demographic
Age
Sex
Body Mass index
Gross Motor Function Classification Scale (I-V)
Level of ambulation (none vs exercise or community) /transfers
CP Type: pattern (e.g. hemi- vs. quadriplegia)
and tone (spastic, dystonic, ataxic or mixed)

13. Results 97 adults seen in time frame
5 patients unable to obtain due movement disorder/cognitive
3 declined
18 had DXA at other facility
Others GMFCS I – III or Dxa performed outside of time frame
42 had at least one DXA performed at our institution.
17 had two DEXAs performed
2 had had interventions following initial study, leaving 15 for analysis
Of the 42, 83% were less than age 50 years

14. Results: DemoGraphics
Age at first DEXA, years M (range) 38.9 ( ) <50 (n=35) , years M 34.2 years 50 or greater (n=7) , years M 62.2 years Sex, N (%) Male/Female 24 (57.1) / 18 (42.9) Anthropometrics Weight, (kg) M (±SD) 59.4 (19) BMI, (kg/m2) M(±SD) 59.4 (19) Median (Range) 23.2 ( ) Under Weight 8 (19.05) Normal weight 20 (47.6) Overweight 7 (16.7) Obese 7 (16.7) Race, N (%) Caucasian 29 (69.1) African American 12 (28.6) Asian 1 (2.4)
M = mean
N = number

15. Results: Functional and Medical
GMFCS Level
PATTERN

16. Results: Osteoporosis and Fractures
Osteoporosis/Osteopenia by T score criteria:
ALL subjects had at least one site with osteoporosis/osteopenia in LE
Osteopenia – 14 (33%)
Osteoporosis – 27 (64%)
Fractures
Seven patients (16.7 %) with fractures
12 fractures (5 with 2 fractures)
Foot/Toes: 4
Femur – 1
Tibia/fibula – 3
Spine – 3
Rib – 1
Lower BMD at the spine, left and right total hip, and
right femoral neck sites was associated with quadriplegia.
Lower BMD at the left total hip and right and left femoral neck
sites was associated with needing assistance with transfers and no ambulation.

17. RESULTS: FUNCTIONal and Medical
Seizures, N (%) Current Treatment-yes Smoking Status, N (%) Yes No Menopausal Status (No), N (%) Thyroid Replacement, N (%)
4 (9.52)
2 (4.76)
40 (95.24)
12/18 females

18. Results: Mean T and Z Scores
Note: DXA could not be performed at all sites to due prior procedures/positioning/contractures.
Site
Number of studies
(All/<50)
T score
Z- score
Z-score <50 yr
(range)
Lumbar Spine
38/32
-1.98
-1.69
-1.82
Total Hip R
27/27
-1.94
-1.60
-1.88
Total Hip L
34/27
-1.80
-1.50
-1.53
Femoral
Neck R
33/27
-2.38
-2.05
Neck L
35/29
-2.02
-1.56
-1.66

19. SPINE T scores by Age vs GMFCS
No relationship between osteoporosis presence and GMFCS P = 1.0
Note: incidence low

20. Spine Z- scores by age and GMFCS

21. Left Femoral Neck T scores
Higher GMFCS level is associated with osteoporosis at left femoral neck (p= 0.021).

22. Left Femoral Neck Z score vs age and GMFCS

23. Left Total Hip T scores
Osteoporosis at the L total hip site was not associated with GMFCS (P=.449)

24. Left total hip Z score vs age

25. T scores spine first to next dexa
Percent change per year mean = - 4% per year

26. Z scores (SPINE) from 1st to NEXT DXA
Percent change per year -10%

27. Left Total Hip T score from first to next DEXA
Percent change per year -5%

28. Left total hip Z score Dexa 1 to 2
Left total hip z score change per year -6%

29. Limitations Limitations - largely non-ambulatory population
Not always easy to obtain BMD in this population
Positioning/Contractures
Prior surgeries (hip and spine)
Movement disorders/cognition
Best location to identify who is at risk has not been identified.

30. Conclusions
Lower than expected-for-age BMD was found to be very frequent in adults with CP with mobility limitations
Low BMD is present at both lumbar spine and hip sites.
Quadriplegia, transfer assistance and no ambulation is associated with the presence of osteoporosis, at multiple sites
GMFCS did not correlate with spinal osteoporosis
90 percent of bone mass is achieved by aroung 18 in women and 20 in males, peak at around age 30

31. Thank you!