Hereditary Spastic Paraparesis How can Physiotherapy help?

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Hereditary Spastic Paraparesis How can
Physiotherapy help?

• Meredith Wynter
• Senior Physiotherapist
• CP Health, Royal Childrens 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 gtgt 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 gt 35y, more rapidly progressive disease,
  muscle weakness, sensory loss, urinary
  involvement more marked
• Onset varies infancy - gt 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
componentsour 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?
• 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
• strengthen and stretch
• Gym training
• Swimming
• Cycling
• Yoga / Pilates
• Martial arts, karate etc
• Horse riding
• Rock climbing

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Thank youmeredith_wynter_at_health.qld.gov.au

• 15 minutes stretching every day
• Enjoyable physical activity