Spasticity

PAC4462 Integrated Therapeutics – Neurology and

Oncology

FCHS 2022

.
fchs.ac.ae
 Spasticity
Definition
• Condition of performing involuntary contractile
movements (ie. increased muscle tone)
– Generalized rigidity (hypertonicity)

• Arise from lesions interfering with motoneurone

activity
– Parkinsonism – lesions in basal ganglia
– Cerebral palsy – lesions to parts of cortex
– Rigidity on one (hemiplegia) or both sides of body
– lesions to midbrain
– Rigidity in muscles innervated below a lesion
(monoplegia is the paralysis of any one limb) –
damage to spinal cord
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 Spasticity
Causes

1.Multiple sclerosis (MS), Amyotrophic Lateral Sclerosis (ALS) /

Motoneurone disease

2.Spinal cord injury: Birth, accident, chronic back pain

3.Stroke, cerebral vascular disease (Covered already)

4.Local injury or inflammation: eg. arthritis

5.Cerebral palsy

6.Tetanus

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 Amyotrophic Lateral Sclerosis (ALS)
Moto-neurone disease

• Motor neuron disease (UK) / Amyotrophic Lateral Sclerosis

(ALS) (USA)
• 50% of patients die within 18 months after diagnosis
• Only 20% survive 5 years and 10% live longer than 10 years
• Persons with ALS who go on a ventilator may live for many
years. Improved treatment is allowing ALS patients to live
longer than before.

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Amyotrophic Lateral Sclerosis (ALS)
Symptoms
• tripping, stumbling and falling
• loss of muscle control
• loss of strength in hands and arms
• difficulty speaking, swallowing and/or breathing
• chronic fatigue
• muscle twitching and/or cramping

ALS is characterized by both upper and lower motor neuron

damage. Symptoms of upper motor neuron damage include
stiffness (spasticity), muscle twitching (fasciculations), and
muscle shaking (clonus). Symptoms of lower motor neuron
damage include muscle weakness and muscle shrinking
(atrophy).

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Amyotrophic Lateral Sclerosis (ALS)
Diagnosis:
Process of elimination - Findings inconsistent with ALS include:
• Neuroimaging, EMG, clinical or other evidence of an alternative
disease explaining signs or symptoms
• Lack of progression to other body regions
• Sphincter abnormalities
• Sensory dysfunction
• Visual decline, dementia
 Definite ALS
 LMN (lower motor neuron) and UMN (upper motor neuron) signs in three to four
regions
 Evidence of progression
 Probable ALS
 LMN and UMN signs in at least two regions 
 Evidence of progression
 Possible ALS
 LMN and UMN in one region OR UMN in two regions
 LMN and UMN signs with no evidence of progression
 Suspected ALS
 LMN signs in two to three regions 6
Amyotrophic Lateral Sclerosis (ALS)
Causes
• 10% of ALS cases appear to be hereditary
• This is called familial ALS - defined as two or more cases in the
same bloodline
• Autosomal dominant, meaning that if a parent has ALS, their
children have a 50% chance of inheriting the defective gene
• While the risk of inheriting the defective gene is 50% for each
child of an affected person, not all people with the defective gene
will develop the disorder
• Twenty percent of familial ALS cases have a specific gene defect
in the SOD (super oxide dismutase) gene
• 90% of ALS cases have no familial link, and they are called
sporadic ALS.

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Amyotrophic Lateral Sclerosis (ALS)
Causes
• ALS occurs in all races and all around the world.
• Men get ALS more than women (1.5 to 1.0 ratio.)
• The incidence of ALS is 2 per 100,000.
• The average age of onset is 55 years.
• 80% of ALS cases begin between the ages of 40 & 70.
• Patients with ALS diagnosed between the ages of 20 and 40
have a higher chance of surviving for 5 years. 

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Amyotrophic lateral Sclerosis (ALS)

British physicist:
Dr Steven Hawking
British actor: David Niven (8 January 1942 –
14 March 2018

Lou Gehrig
Lou Gehrig’s
Disease Diagnosed
in 1939 and died in
1941 aged 41.
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Spasticity
Amyotrophic Lateral Sclerosis (ALS):
• Caused by oxidative stress due to accumulation of
reactive oxygen species
• Oxidative stress causes neurons to become more
susceptible to excitotoxic damage

• Fatal debilitating disease resulting from progressive

degeneration of motor neurons
• Following diagnosis, life expectancy is only 5 years

• Defective superoxide dismutase (SOD).

• SOD breaks down reactive oxygen species

• Defective SOD leads to oxidative stress

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 Excitotoxicity
Fig. 34.1
Rang et al.

Accumulation
of reactive
oxygen
species
which
stimulates
excitatory
glutamate
release
leading to
motor
neurons
excitation
and muscles
contraction

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Amyotrophic Lateral Sclerosis (ALS)
Treatment

Riluzole
• Inhibits release and postsynaptic action of
glutamate
• Slows excitotoxicity
• Retards deterioration of patients with ALS
• All patients are started on Riluzole at the time of
diagnosis (British medical journal 2020)

Amyotrophic lateral sclerosis - symptoms, diagnosis and treatment | BMJ best practice [online] Available
from: https://bestpractice-bmj-com.ezproxy.rgu.ac.uk/topics/en-gb/330?q=Amyotrophic lateral
sclerosis&c=suggested [Accessed 2/25/2020 2020]
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 Amyotrophic Lateral Sclerosis
(ALS)
• ALS is a progressive disease of the motor system and, as
such, produces symptoms primarily relating to the location
and degree of weakness.
• The exact treatment needs are almost never identical from
patient to patient and will change with time for individual
patients.
– Nutritional and respiratory support is critical, with the actual intervention
required dependent on the status of the patient.
– Those with significant weakness of bulbar musculature may require
treatment for sialorrhoea, communication difficulties, and secretions.
– Patients with weakness in the extremities may require treatment aimed
at maintaining mobility, therapy for spasticity, or devices aimed at
maintaining upper extremity function.
– Mental status changes that may require treatment include depression
and anxiety
Amyotrophic lateral sclerosis - symptoms, diagnosis and treatment | BMJ best practice [online] Available from: 
https://bestpractice-bmj-com.ezproxy.rgu.ac.uk/topics/en-gb/330?q=Amyotrophic lateral sclerosis&c=suggested [Accessed 2/25/2020
Tetanus

• Arises when a wound is contaminated with spores of

Clostridium tetani (soil)
• Bacilli multiply at site of infection and produce the protein
neurotoxin: tetanospasmin
• Toxin diffuses along axons of motoneurones to CNS
• Interferes with inhibitory neuronal mechanisms by
preventing release of transmitter

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 Effects of tetanus
• Muscles become hypertonic
• Violent reflex spasms to weak sensory stimuli
• Lock-jaw
• Death occurs as a consequence of spastic paralysis of
respiratory muscles

Treatment of tetanus:
• Artificial ventilation of lungs
• Prolonged use of muscle relaxants:
• Protection for 5-10 years by immunization with
tetanus toxoid
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