Hereditary Spastic Paraparesis How can Physiotherapy help? Meredith Wynter Senior Physiotherapist CP...

Hereditary Spastic Paraparesis

How can Physiotherapy help?Meredith Wynter

Senior Physiotherapist

CP Health, Royal Children’s Hospital

Overview

• HSP

• Spasticity

• Treatments for children

• Physio approaches

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

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.

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”

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

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

SPASTICITY• Physiotherapy • Oral medications • Orthotics• Orthopaedic surgical interventions (Multilevel

surgery)• Electrical stimulation• Botulinum Toxin Injections• Selective Dorsal Rhizotomy ( SDR)• Intrathecal Baclofen Infusion ( ITB)

Physiotherapy

• Motor Control

• Task training

• Stretching

• Strengthening

• Electrical stimulation

• Serial Casting

• Splinting

Treatment with Botulinum Toxin

• Many treatments world wide

• Gold standard

• Safe

• Reversible

• Helps with growth related contracture

• Improves function

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)

Assessment

• Activities

• Strength, range of movement, gait video

• Participation

• Goals, patient and medical

• Maintenance of skeleton and muscles

• Caution for excessive weakness

How is it used? Intramuscular injection

for Focal spasticity

Calf injection sites

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

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

Exercise

• Something enjoyable– strengthen and stretch

• Gym training • Swimming• Cycling• Yoga / Pilates• Martial arts, karate etc• Horse riding• Rock climbing

Thank youmeredith_wynter@health.qld.gov.au

15 minutes stretching every day

Enjoyable physical activity