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Neurological Facts

Motor Neurone Disease Melanie Worthington Regional Care Development Adviser Lancashire & Cumbria Motor 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

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Neurological Facts

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  1. Motor Neurone DiseaseMelanie WorthingtonRegional Care Development AdviserLancashire & CumbriaMotor Neurone Disease AssociationTel: 08453 751841MND Connect: 08457 626262

  2. 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

  3. What is Motor Neurone Disease

  4. 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

  5. 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

  6. 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

  7. Causes of MND

  8. Familial – 5-10% • Rare • Research found genetic faults • SOD 1, FUS, VCP and TDP-43 genes • Ubiquilin protein gene • Chromosone 9 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

  9. Types of Motor Neurone Disease

  10. Progressive Bulbar • Palsy (PBP) • 20% of cases (onset) • involves UMNs and • LMNs • dysarthria • dysphagia • emotional lability • progressive • weakness in upper • limbs/neck/ • shoulder girdle • Amyotrophic Lateral • Sclerosis (ALS) • 65 - 66% of cases (onset) • involves UMNs and • LMNs • muscle weakness – often • develops in hands and • feet first, spasticity, • hyperactive reflexes

  11. 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 Lateral • Sclerosis (PLS) • 2% of cases • rare • UMNs only • muscle weakness • stiffness • balance • dysarthria • does not shorten • survival

  12. Courseof 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

  13. Site of Onset • Limb (usually distal) • Bulbar • Respiratory

  14. 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

  15. Diagnosis of Motor Neurone Disease

  16. 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

  17. How is MND Diagnosed? • Interpretation of clinical symptoms and signs • Investigations to exclude other causes • MRI • Lumbar puncture • Lack of definitive test problematic

  18. Effects of Motor Neurone Disease

  19. 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 Effects of MND

  20. Clues to respiratory muscle involvement in MND • Breathlessness • - on minimal exertion • - on lying flat • • Poor sleep • • Excessive daytime sleepiness • • Headaches on awakening • • Excessive nocturnalsweating

  21. 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

  22. 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)

  23. 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

  24. Treatments and Interventions

  25. 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

  26. Treatments/interventionsin MND Multidisciplinary approach Palliative care Sensitive Management Person with MND Nutritional support PEG/RIG Rehabilitation medicine Pharmaceutical management of symptoms Respiratory care Disease modifying therapy

  27. 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).

  28. Multidisciplinary approach

  29. 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

  30. MND Association Support

  31. 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

  32. Motor Neurone Disease • Melanie WorthingtonRCDA Lancashire and CumbriaTel: 08453 751841 melanie.worthington@mndassociation.org • Preston MND Care & Research Centre Royal Preston HospitalTel: 01772 522545 • MND Connect: 08457 626262 • mndconnect@mndassociation.org

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