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Hereditary Spastic Paraparesis How can Physiotherapy help?
Meredith Wynter Senior Physiotherapist CP Health, Royal Children’s Hospital
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Overview HSP Spasticity Treatments for children Physio approaches
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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
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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.
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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”
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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
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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
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SPASTICITY Physiotherapy Oral medications Orthotics
Orthopaedic surgical interventions (Multilevel surgery) Electrical stimulation Botulinum Toxin Injections Selective Dorsal Rhizotomy ( SDR) Intrathecal Baclofen Infusion ( ITB)
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Physiotherapy Motor Control Task training Stretching Strengthening
Electrical stimulation Serial Casting Splinting
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Treatment with Botulinum Toxin
Many treatments world wide Gold standard Safe Reversible Helps with growth related contracture Improves function
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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)
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Assessment Activities Strength, range of movement, gait video
Participation Goals, patient and medical Maintenance of skeleton and muscles Caution for excessive weakness
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How is it used? Intramuscular injection for Focal spasticity
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Calf injection sites
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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
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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
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Exercise Something enjoyable Gym training Swimming Cycling
strengthen and stretch Gym training Swimming Cycling Yoga / Pilates Martial arts, karate etc Horse riding Rock climbing
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Thank you meredith_wynter@health.qld.gov.au
15 minutes stretching every day Enjoyable physical activity
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