Neurological Facts

Motor Neurone Disease

Melanie WorthingtonRegional Care Development AdviserLancashire & CumbriaMotor Neurone Disease Association

Tel: 08453 751841

MND Connect: 08457 626262

Neurological Facts• 10 million people in UK have a neurological condition

• Account for 20% of acute hospital admissions

• Third most common reason for visit to GP

• 850,000 carers

• 69% of primary care budget is spent on long term conditions(Department of Health)

• MND – over 5,000 people in UK

• Parkinson’s Disease – 120,000

• Multiple Sclerosis – 100,000

What is Motor Neurone Disease

Motor Neurone Disease

• Every person develops the disease in a different way

• Symptoms experienced depends on the area of nervous system affected

• 90% - 95% of people have the sporadic form (out of the blue)

• 5-10% Familial – 200-300 people

• Adult Illness – most people are over 50

• Average survival 2-5 years from first symptoms.

• From diagnosis 14 months average.

• No cure but symptom management and medication that may improve quality or prolong life

• Onset and progression is variable – can progress swiftly

Who does it affect ?

• Relatively uncommon• Annual incidence of 2 in 100,000• Prevalence 5-7 per 100,000• More common in men but over 65 yrs

becomes more even• GPs can expect to see 1 or 2 cases during

their career

What is Motor Neurone Disease?• Upper motor neurones (UMN)

originate in the base of the cortex of the brain : Spasticity

• Lower motor neurones (LMN) originate in the spinal cord: Wasting/Weakness

• Act as transmitters that provide a chain of command for voluntary movement to muscles throughout the body

• In MND this chain of command is broken as neurones degenerate

Causes of MND

Sporadic – 90%

• Risk factors: genetic, environmental and lifestyle factors that may tip the balance:

- mechanical/electrical trauma- Military service- High levels of exercise- Agricultural chemicals and

heavy metals

Evidence is often circumstantial

and conflicting

Familial – 5-10%

•Rare•Research found genetic faults•SOD 1, FUS, VCP and TDP-43 genes•Ubiquilin protein gene•Chromosone 9

Types of Motor Neurone Disease

Amyotrophic LateralSclerosis (ALS)

65 - 66% of cases (onset)

• involves UMNs and LMNs• muscle weakness – often• develops in hands and • feet first, spasticity,• hyperactive reflexes

Progressive BulbarPalsy (PBP)20% of cases (onset)

•involves UMNs and LMNs• dysarthria• dysphagia • emotional lability• progressive weakness in upper limbs/neck/ shoulder girdle

Progressive Muscular Atrophy (PMA)7.5% - 10% of cases

• predominantly LMNs affected (may start in small muscles of hand)• muscle wasting, weakness• fasciculation(may in time develop UMN involvement and may eventually develop some speech problems)

Primary LateralSclerosis (PLS)2% of cases

• rare• UMNs only• muscle weakness• stiffness• balance• dysarthria• does not shorten survival

Course of Disease

• Onset and progression variable

• Is always progressive with no remissions

• Usually affects both the upper and lower motor neurones

• 90% develop some bulbar symptoms

• Death often through respiratory failure

Site of Onset

• Limb (usually distal)

• Bulbar

• Respiratory

Early Symptoms

Depend on area of nervous system affected:

• stumbling• foot drop• loss of dexterity• weakened grip• cramps• change of voice quality• slurred speech• early swallowing difficulties• muscle wasting • fatigue

Diagnosis of Motor Neurone Disease

Diagnosis

• On average, it takes 14 months from first symptoms to diagnose MND

• First signs and symptoms often subtle and non-specific, similar to other diseases

• Person often not referred to a neurologist directly

• No definitive diagnostic test

How is MND Diagnosed?

• Interpretation of clinical symptoms and signs

• Investigations to exclude other causes

• MRI

• Lumbar puncture

• Lack of definitive test problematic

Effects of Motor Neurone Disease

Effects of MND

• Progressive muscle weakness and wasting

• Loss of weight• Fasciculation, cramp

and spasticity• Dysarthria-slurred

effortful speech• Saliva and Mucus

Problems

• Dysphagia - poor swallow due to weakness and paralysis of bulbar muscles

• Respiratory muscle weakness

• emotional lability• Cognitive changes

Clues to respiratory muscle involvement in MND

•Breathlessness - on minimal exertion - on lying flat• Poor sleep• Excessive daytime sleepiness• Headaches on awakening• Excessive nocturnal sweating

Psychosocial Impact

• Multiple losses: physical loss, loss of control, role, independence, self image, self esteem and confidence

• Financial • Home environment• Communication difficulties• Increasing isolation and dependence on carers• Anxiety, Fear, Anger• Knowledge of own impending deterioration and death

Cognitive changes

• MND has been traditionally viewed at a disease affecting the motor system with no compromise of cognitive abilities• Recent research shows that 25% or more show some cognitive changes in the frontal lobe region• 3-5% will have fronto-temporal dementia (FTD)

What isn’t affected by MND

• Senses: touch, taste, sight, smell and hearing

• Bowel and bladder function

• Sexual function and sexuality

• Eye Muscles

• Heart muscles

Treatments and Interventions

Aims of Management

•Control of symptoms • Promote independence and control – usually supported at home as much as possible

• Plan appropriate interventions

• Enable person with MND and family to live as full a life as possible

Treatments/interventions in MNDMultidisciplinary

approach

Sensitive Management

Nutritional support

PEG/RIG

Respiratory care

Disease modifying therapy

Pharmaceutical management of

symptoms

Rehabilitation medicine

Palliative care

Person with MND

Life Prolonging Interventions

• Riluzole only drug to have beneficial effect on survival : 3-4 months

• Respiratory care: Non-invasive ventilation (NIV)

• To improve quality of life.• Median survival extended 205

days (Miller et all 2009).

Multidisciplinary approach

End of Life Decisions

• Advanced Care Planning• Advanced decision to refuse treatment

(ADART)• Advanced Statment of wishes and

preferences• Preferred Priorities of Care (PPC)• Withdrawal of treatments• Tissue donations

MND Association Support

Provided to plwMND, families, carers and professionals

• Standards of Care

• Regional Care Development Advisers

• Association Visitors and Volunteers

• Equipment Loan

• Financial Support

• Care Information

• MND Connect

• Local Branch Network

• Care Centre Programme

• Education/Training

• www.mndassociation.org

Motor Neurone Disease• Melanie Worthington

RCDA Lancashire and CumbriaTel: 08453 751841 melanie.worthington@mndassociation.org

• Preston MND Care & Research Centre Royal Preston Hospital

Tel: 01772 522545

• MND Connect: 08457 626262• mndconnect@mndassociation.org