AMN, Physiotherapy & Exercise

Hayley Steele & Claire EvansAdvanced NeuroPhysiotherapist

From Optimal Rehab

Goals

• Our goal is to try to give you the best possible quality of life.

• Keeping you as active as possible in your environment

• Get you the help you need• Assess your symptoms early

AMN

• Adrenomyeloneuropathy (AMN) is a form of X-linked adrenoleukodystrophy (X-ALD)

• AMN, is a disorder of fatty acid beta oxidation which results in the accumulation of very long fatty acids in tissues throughout the body.

• The most severely affected tissues are the myelin in the central nervous system and the adrenal cortex.

• AMN patients generally have spinal cord dysfunction and impaired nerve conduction, which leads to the initial symptoms that include difficulties in walking or a change in the walking pattern.

• The average age at which symptoms first appear is 28, but onset can occur anywhere from the second to the fifth decades of life.

• As yet there is no cure, however management of relapses and symptoms is ever evolving and changing.

AMN• AMN can be broken down into two general clinical forms: • AMN with cerebral involvement (the spinal cord and brain

are both affected), and • AMN without cerebral involvement (only the spinal cord is

affected and not the brain).• Of patients with AMN, approximately 54% have normal

brain function, while 46% have brain involvement of varying degrees.

• This can be assessed by a Magnetic Resonance Image scan (MRI)which is used to produce detailed images of the brain.

Myelin and the CNS

• Myelin is an electrically insulating material that forms a layer, the myelin sheath around the nerve fibre which conducts electrical impulses.

• It is essential for the proper functioning of the nervous system

• Myelin looks like strings of sausages• The main purpose of a myelin

layer is to increase the speed at which the impulses move along the nerve fibre.

CNS

• The central nervous system (CNS) is the part of the nervous system consisting of the brain and spinal cord.

• The central nervous system is so named because it integrates information it receives from, and coordinates and influences the activity of, all parts of the body.

Symptoms

• Difficulty in walking/change in walking pattern: this is the most common initial symptom of patients with AMN

• Spastic paraparesis: a gradual, weakness and stiffness of the legs; in AMN this is often specific to the lower limbs.

• Ataxia: loss of the ability to coordinate muscle movement-this can cause balance problems

• Hypertonia: excessive muscle tone leading to stiffness.

• Muscle spasms• Mild peripheral neuropathy

Physiotherapy• Physiotherapy can assist in different ways.• Detailed assessment;• -strength• -muscle tone• -stiffness• -mobility• -posture• -balance• -exercise tolerance• -Pain• Sensory problems

Strength• Physio can can give you help and advice on specific

exercises. • Muscle weakness in arms & legs

uses more energy which increases fatigue.• You may need to exercise certain muscles such as the

hamstrings, quadriceps or calf muscles.• This can be done through

using weights or your own body.• A graduated programme can

prevent fatigue whilst you are exercising

Muscle tone

• Muscle tone is the muscle's resistance to a slow stretch during its resting state.

• It helps to maintain the body’s posture and posture.• If a sudden pull occurs on the muscle the body will

automatically increase the muscles tension, which is a reflex which helps guard against danger and helps to maintain your balance.

• If the muscle tone is increased this can be referred to as spasticity and may prevent normal movement of the body

Muscle Stiffness

• Increased tone (resistance in the muscle) can mean muscles are slow to relax, and this can cause stiffness.

• Depending on the muscles affected, this stiffness can make it difficult to perform delicate movements with the hands and fingers, or make larger movements difficult, which can affect walking, for example.

• Stiffness & spasticity in the muscles increases fatigue

• Physio can advise you onspecific stretches

Spasticity

• Your physio can assess for spasticity, stiffness and advise on;

• Stretches for the lower limbs• Splints-such as arch supports, patella strap• Exercises• Positioning• Medication (via GP)• Botox

Mobility

• Weakness and spasticty in the lower limbs can cause difficulties with mobility and balance.

• They can lead to instability when walking• They can make the hips difficult to lift when walking• Poor mobility can make exercise more difficult.• Physio can assess your gait and need for walking aids

such as a stick, rollator or zimmer frame.

Physio & Splints

• Physio can assess your walking pattern and advise on specialist splints and other devices to help you such as FES.

• There are many different different types of splints which can help lift your foot up such as an

‘AFO’,‘foot up’‘dyna ankle’‘mus mate’

Functional Electrical Stimulation

• Odstock dropped foot stimulator

• Picks up your foot when walking

• Additional benefits over splint but commitment to set it up

• Surface electrodes on lower leg

• Controlled by foot switch

• Available on NHS at specialist centres.

Posture

• Poor posture results from certain muscles tightening up or shortening whilst others lengthen or become weak.

• Physio can assess your posture and advise on the correct postural exercises.

• Core stability exercises will improve posture, movementefficiency and balance

Balance• Balance is an ability to maintain the line of gravity of a body

within its base.• Maintaining balance requires coordination of input from

multiple sensory systems including the vestibular and visual systems.

• AMN can cause you to fall or trip due to lack of self correction.• AMN can cause proprioception

and sensory problems which affect balance- proprioception is the body’s ability to know whereit is in space.

Physio & Balance

• Physio can assess balance and give you very specific exercises to work on this including ‘Cawthorne Cooksey exercises’.

• We can give you exercise to work on proprioception

• Using a mirror to exercise may help• Using light touch to increase proprioception through

the arms• Swimming and cycling are good for increasing

strength and stability and thus balance

• Pain can felt as heavy legs, legs of lead, general feeling of stiffness, back, neck and shoulder pain.

• It can be also due to poor alignment of muscles and joints from spasticity eg spasticity around the knee muscles can pull the knee cap out of line.

• Neuropathic pain may be felt as abnormal sensations such as pins and needles or burning.

• Physio can assess your pain and advise on exercises to relieve you pain, pain relief devises and medication.

• We can advise you on gentle exercise which can help

Other factors than affects your symptoms

• Nutrition- need a balanced diet, try to limit weight loss & gain.

• Stress- try and prioritise daily tasks- do the most tasks important first-if you get rid of dust it only makes room for more dust!’

• Heat has an effect on fatigue-e.g.. infections, over-exertion,over-heated room all cause bodytemp to rise

Fatigue Management

Keep Cool

Keep a DiaryRegular exercise

Consider posture

Consider your environment

Who can help?• GP

• Physiotherapist

• Occupational Therapist

• Nurse

• Dietician

• Clinical Psychologist

• Occupational Health Advisor at work

Occupational Therapist• Personal Care

- Use equipment to prevent bending and over exertion- Home assessment- advice on stairs, rails, seat heights-Set out clothes the night before

• Domestic Tasks- Arrange kitchen for easy access- Cook in bulk- kitchen equipment

• Leisure Time- Avoid over-committing- Prioritise-Relaxation techniques

Dietician• No food is a miracle cure

• Large, hot meals can worsen fatigue-They take longer to digest & uses more energy

• Caffeine/ Sugary snacks have initial pick-me-up effect but lead to tiredness later on

• Advice on supplements & vitamins

Occupational Health• Most Employers are legally obliged to make reasonable

adjustments to allow you to continue working

• Work station assessment to improve posture at desk- which uses less muscles.

• Liaise with your boss about need for regular rests

• Could work hours be adapted?E.g.. Day shifts only

Shorter working days

See ‘Working with MS’ www.mssociety.org.uk

MDT Assessment• Some hospital offer an MDT assessment- where you will be

assessed in a holistic specialist team members.

• Identify symptoms and advise on specific medication that may help spasticity, fatigue and pain.

• They can also advise on treatments such as botox, Intrathecalbaclofen pumps, splints and co ordinate these treatments together for optimal effect.

• Identify need for onward referral to other health professionals

Benefits of exercise

Helps maintain mobility6

Helps maintain mobility

Increases Fitness

Reduces Risk of

Comorbidities

Reduces Depression Improves

Quality of Life2,

Improves

Fatigue

Management

And!!!

1. Improve and/or maintain muscle activity2. Exercise improves balance & stability3. Improves Posture4. Improves Joint range of movement 5. Improves ability to carry out activities of daily living.6. Maintaining independent functional ability7. Teaches Self- management techniques.8. Maintain cardiovascular fitness9. Improve overall health and wellbeing through treatment

techniques and education.

Benefits of physio in AMN

• Advice on:-muscle strengthening and stretchingspasticity and Botoxsplintsbalance and walking & transferswalking aidsFES, functional electrotherapypacing and fatiguecore stability ex’s

Key Points• Get assessed early by the MDT.

• Follow self management programmes:--Diary - Rests and adapting activities- Exercise and Diet- Make activities more energy efficient and use appropriate

walking aids and splints to help you.

• Aggravating factors need to be identified and treated.

• Review medication.

Any Questions?

Claire Evans & Hayley SteeleAdvanced neuro Physiotherapists

Optimal Rehab 07747 63233507535 705168

Email: admin@optimalrehab.co.uk