1. Hereditary Spastic Paraparesis How can Physiotherapy help?
Meredith Wynter
Senior Physiotherapist
CP Health, Royal Children’s Hospital

2. Overview
HSP
Spasticity
Treatments for children
Physio approaches

3. HSP Many different names
Hereditary spastic paresis/ paraplegia
Familial spastic paraplegia
Strumpell-Lorrain disease: first described 1880.
Characteristically: progressive spasticity of legs (hams, quads, calves)
Spasticity >> weakness
10-60% sensory involvement
25-45% asymptomatic
Prevalence 2-6/100,000. Rare

4. Classification systems
Genetic markers
Age of onset:
Type 1 : Early – slower progression
Type 2: > 35y, more rapidly progressive disease, muscle weakness, sensory loss, urinary involvement more marked
Onset varies infancy - > 80yo.

5. Clinical presentation – pure HSP
Delay in walking
Leg stiffness, urinary disturbance (urgency, hypertonic bladder), premature wear of shoes
Cardinal signs:
spasticity, hyperreflexia, extensor plantars, weakness in pyramidal distribution (legs)
Family history
Circumducting gait
Weakness: iliopsoas, tib ant, hams.
Can have discrepancy between severe spasticity and mild / absent muscle weakness.
“wheelchair bound patient from spasticity, but normal strength”

6. SPASTICITY Major clinical feature
Generally a major cause of discomfort or functional limitations
Many resources for treatment of spasticity
Long term secondary effects of spasticity can become prime disability
Leads to contractures ( paediatric)
Pathologic condition of soft tissue
Stiffness, fixed shortening, loss of range

7. Spasticity in cerebral palsy has both neurophysiological and musculoskeletal components……our modern endeavours in treatment are designed to `equalise the race’ between bone and muscle growth
Flett 2003

8. SPASTICITY Physiotherapy Oral medications Orthotics
Orthopaedic surgical interventions (Multilevel surgery)
Electrical stimulation
Botulinum Toxin Injections
Selective Dorsal Rhizotomy ( SDR)
Intrathecal Baclofen Infusion ( ITB)

9. Physiotherapy Motor Control Task training Stretching Strengthening
Electrical stimulation
Serial Casting
Splinting

10. Treatment with Botulinum Toxin
Many treatments world wide
Gold standard
Safe
Reversible
Helps with growth related contracture
Improves function

11. Botulinum Toxin Type A What is it? How does it work?
A purified form of the neurotoxin responsible for botulism found to be effective in reducing spasticity - CP, ABI, SCI
How does it work?
Temporarily blocks neuromuscular conduction by inhibiting the release of acetylcholine
Partial paralysis of targeted spastic muscle(s)

12. Assessment Activities Strength, range of movement, gait video
Participation
Goals, patient and medical
Maintenance of skeleton and muscles
Caution for excessive weakness

13. How is it used? Intramuscular injection for Focal spasticity

14. Calf injection sites

15. Why treat spasticity with BTX-A?
improves walking
reduces pain and discomfort
Ease of care and hygiene
Enhance the effects of therapy
Avoid early or repeated surgery / delay surgery
Assist in prevention of contracture
improved tolerance to serial casts
improved tolerance to orthoses and splinting

16. Active physiotherapy program
muscle length and flexibility
serial casting commencing ~ 2 to 3 weeks post injection if required (earlier in acute ABI)
strengthening
targeted motor training
functional skills
splinting and orthotic intervention
home and school program
aim to achieve carry over beyond pharmacological effects of BTX

17. Exercise Something enjoyable Gym training Swimming Cycling
strengthen and stretch
Gym training
Swimming
Cycling
Yoga / Pilates
Martial arts, karate etc
Horse riding
Rock climbing

18. Thank you meredith_wynter@health.qld.gov.au
15 minutes stretching every day
Enjoyable physical activity