Folie 1

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Intrathecal Baclofen and Botulinum Toxin Two
therapeutic options in HSP
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Clinic
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Clinical characteristics of HSP
HSP clinically very heterogeneous disease
Pure
Complex
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Clinical characteristics of HSP

• Spasticity
• Weakness
• Instability
• Pain in the LL

Pure
Complex
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Definition Spasticity
Spasticity is an increase in muscle tone due to
hyperexcitability of the stretch reflex and is
characterized by a velocity dependend increase in
tonic stretch reflexes (Lance 1980)
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Spastic Paraparesis
Lesion of CST
Therapieleitfaden Spastik Dystonien, 2.
Auflage, Uni-Med Verlag
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Spastic Paraparesis
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Damage to CST, RSP, EPS
Immediate consequences
Delayed consequences
Rearrangement of spinal reactivity
Paralyse/Paresis
Spasticity
Immobilization in shortened position
Contracture
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Positive symptoms

• velocity dependend increase of muscle tone

• repetitive stretch reflexe clonus

• synergistic mass movements

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Negative symptoms

• Reduced Coordination

• Paresis

• reduced force

• slowed movements

• Muscle pain and cramps

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Movement Disorder Laboratory
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Movement Disorder Laboratory
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Healthy control
HSP
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Treatment of HSP
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Therapeutic options
Physiotherapy
Activation of residual functions
Prevention of secondary changes
Spasmolytic drugs
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Drug treatment of spasticity

• Benzodiazepine , Baclofen
• Presynaptic Inhibition
• Tizanidine, Memantine, Tolperison
• Interaction with excitatory transmitters
• Botulinum-Toxin-Injection
• Inhibition of presynaptic Ach release
• Dantrolen
• Peripheral mechanism

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Intrathecal Baclofen in HSP
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ITB pump systems
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Intrathecal Baclofen in HSP

• ITB test injection of 50 µg Baclofen

• Gait analysis and clinical examination
• at baseline and after 3 h

• Multi-disciplinary assessment with
• Physiotherapist and Neurosurgeons

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Intrathecal Baclofen in HSP
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Before ITB
After ITB
right
left
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Baseline
ITB test
6 months after implantation
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Responder
Non- Responder
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Botulinum-Toxin in HSP
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Botulinum Toxin
Therapieleitfaden Spastik Dystonien, 2.
Auflage, Uni-Med Verlag
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Botulinum Toxin
Selection of spastic muscles
Injektion
Spasticity
Fuctional Improvement
Paresis
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Botulinumtoxin EMG-guided injection
EMG device
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Functional improvement
Prä Botulinum Toxin
Post Botulinum Toxin
100 U Tibialis posterior muscle 50 U M. Medial
gastrocnemius muscle
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Functional improvement
Prä Botulinum Toxin
Post Botulinum Toxin
60 U Flexor carpi radialis muscle 60 U Flexor
carpi ulnaris muscle 20 U Flexor pollicis longus
muscle
60 U Flexor digitorum superficialis muscle 60 U
Flexor digitorum profundus muscle 10 U
Lumbricales muscles
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Pain
Krampfartiger Muskelschmerz in den Fingerbeugern
Prä Botulinum Toxin
Post Botulinum Toxin
60 U Flexor digitorum superficialis muscle 60 U
Flexor digitorum profundus muscle 10 U
Lumbricales muscles
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Hygienic Indications
50 U Flexor digitorum superficialis muscle 50 U
Flexor digitorum profundus muscle
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G. Deuschl, H. Stolze, M. Witt
G. Stevanin, I. Nelson, N. Bouslam, S. Forlani,
C. Depienne, A. Durr, A. Brice
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Muchas gracias