Week 8 : MS, MND and GBS Flashcards
definition of MS
- Multiple sclerosis is a demyelinating disease with widespread effects in the central nervous system.
- The name comes from the ‘sclerosed’ plaques of scar tissue at ‘multiple’ sites in the CNS.
- First described in 1860s by Professor Jean Charcot as ‘sclerose en plaques’.
epidemiology of MS
caucasians
adult immigrants
temperate climates - northern Europe, North America and australasia
23000 Aus
Clinical features of MS
• Characterised by signs and symptoms of widespread CNS involvement
• A relapsing and remitting course
• Most common in young adults from 20-40 years
– Peak age of onset 20-25 years
– Later adult onset (>50) in only 5% – Childhood onset rare (2%)
• More common in females (2:1)
Aetiology of MS
Exact causation is unknown • Probably multifactorial
–Abnormal immune response to myelin (autoimmune)
–Genetic predisposition
–Environmental exposure (to viruses eg varicella, measles, rubella, herpes simplex)
–Age of individual at exposure
Pathophysiology of MS
- Demyelination (disintegration) of the myelin sheath caused by an inflammatory and destructive process – the axon being partly or completely denuded
- Destruction of the myelin sheath disrupts the normal transmission of nerve impulses
- The axons themselves are preserved initially – some loss of axons may occur particularly in large chronic plaques
Sites of plaques
- Close relationship to veins
- Grey-white boundary in cerebrum • Periventricular regions
- Cerebellar white matter
- Optic nerves
- Brain stem
- Spinal cord (especially cervical)
- Can be anywhere
4 types of MS
RRMS
secondary -progressive MS
Primary progressive MS
Progressive - Relapsing MS
Relapsing remitting MS
- most common type of MS with sporadic exacerbations, neurologically stable between exacerbations
Secondary- Progressive MS (SPMS)
- Over a period of a few decades, approx two-thirds of people with
RRMS will progress to SPMS. SPMS is characterised by a gradual, progressive decline in function while exacerbations become less frequent
• Primary -Progressive MS (PPMS)
- Approx 10 – 15% of people with MS begin with PPMS which is characterised by having no relapses (exacerbations) or remissions - men are affected about as often as women
- rate of progression is similar to SPMS
• Progressive – Relapsing MS (PRMS)
- Progressive symptoms from onset with rare relapses - Accounts for 5% of MS patients
Diagnosis of MS
• Based on demonstration of clinical, neurophysiological or radiological lesions spread over time and space (anatomical localisation)
• Two attacks and clinical evidence of two separate lesions or clinical of one and paraclinical of other.
• Neurophysiological:
– Evoked potential studies – visual, auditory
• Radiological:
– MRI shows disease in 90% cases
• Other tests:
– CSF examination by lumbar puncture – shows oligoclonal bands (more common in well-established cases)
Sensory - Motor dysfunction in MS
• Sensory
– Posterior column (impaired propriception and vibration); Lhermitte’s sign – cervical flexion causes ‘shock’ sensation in upper limbs
– Spinothalamic tract – dysaesthesia (burning sensation)
– Dorsal root entry zone ( loss of all sensory modalities for that root distribution)
• Motor
– Weakness (monoparesis & paraparesis most common) – Spasticity (central or spinal mediated)
– Cerebellar ( truncal ataxia, limb ataxia, dysarthria, intention tremor
Cranial Nerve impairment in MS
Optic neuritis – sudden loss of vision, pain on extraocular movement, central scotomas
– May be early sign which disappears or persistent • Oculomotor abnormalities
– IIIrd or IVth cranial nerves most common
– Visuovestibular and brainstem intranuclear connections involved
• Trigeminal neuralgia diagnostic in young adult
• Facial weakness common
• Subclinical dysphagia present, often with dysarthria
• Vertigo (vestibular); occasional hearing loss
Urinary incontinence in MS
• Bladder dysfunction is an early sign
• Detrusor sphincter dyssynergia and Detrusor
hyperreflexia are indicative of a pontine and
cervical spinal cord lesion, respectively
• Complaints are of frequency and urgency and
failure to empty bladder
Sexual Dysfunction in MS
Affects 80% of males and 61% of females with MS • In females, can involve:
– Perineal sensory loss, weakness of pelvic floor, hyperreflexic bladder, adductor spasticity, depression, loss of self-esteem
• In males, can involve:
– Erectile dysfunction, generalised weakness and spasticity
– Impotence is related to specific lumbar spinal cord damage not duration of disease
Fatigue in MS
Occurs daily, interferes with physical and social function and worsens with heat
• Heat in form of hot weather, overheated room or immersion in hot weather increases fatigue
• Four types of fatigue described by Shapiro et al (1987):
– Fatigue following physical exertion
– Nerve impulse fatigue
– Fatigue related to depression (associated with sleep disturbances)
– Lassitude – an abnormal sense of tiredness of unknown aetiology
Cognitive and Affective disorders in MS
More common with advanced disease
• Attentional deficits (visual and auditory) can impact on memory
• High incidence of intellectual deterioration later
• Depression more common in MS than comparable medical disorders
• Stress exacerbates symptoms; emotional distress increases with exacerbations
• Personality change and psychosis in some late-stage patients
Medical management in MS
• Management of the acute attack
– IV methlyprednisolone to decrease inflammation effects
• Treatment of the underlying disease – Immunomodulatory agent
• Beta interferon 1b (Betaseron)
• Beta interferon 1a (Avonex, Rebif) – Immune reaction inhibitor
• Glatiramer acetate (Copaxone)
• Management of symptoms
– Spasticity – Baclofen (Lioresal); Tizanidine (Zanaflex) – Depression / fatigue – (Zoloft, Prozac, amphetamine)
Complementary management in MS
- Exercise
- Diet, food supplements
- Stress management strategies • Lifestyle changes
Physiotherapy assessment of MS
Impairments
– Motor – strength, tone, co-ordination, ROM
– Sensory – sensation, proprioception, etc
– Visual / Cranial nerves – swallowing, speech, – Bowel/ bladder/ sexual
– Cognitive, affective
• Balance, mobility, transfers / ADL
• Fatigue, endurance
• Cardiovascular and respiratory status
• Psychosocial status/ vocational status
• Physical environment / community resources
• Outcome measures – Expanded Disability Status Scale; FIM
Aims of physiotherapy in MS
restorative therapy
– Optimise performance of everyday activities and
skills/ Maximize functional ability
– Target disuse weakness, spasticity, pain, incontinence, cardiopulmonary deconditioning; preserve musculoskeletal integrity
Aims of physiotherapy in MS compensatory approach
– Prevent unnecessary disability and handicap
– Improve individuals quality of life
– Ensure that interventions are relevant to person’s needs and desires; collaborate to set meaningful goals
– Manage fatigue
– Teach use of mobility aids, transfers; modify environment
What is MND
- Motor neurone disease damages both upper and lower motor neurones.
- The UMNs affected in MND originate in the motor cortex of the brain. These UMN axons descend by means of the corticobulbar and corticospinal tracts to synapse with LMNs in the brainstem and spinal cord (anterior horn cells)
- MND is a progressive, degenerative disorder of cortical, brainstem and spinal motor neurones
incidence and aetiology of MND
• Each year, about 1 in 50,000 Australians develops MND.
• It is slightly more common in men than women
• Peak age of onset is between 50 to 70 years of age
• Approx 5-10% have the familial form; they often present
earlier in life
• Cause is unknown however research has looked at
viruses, toxins, genetic factors and immune factors
Clinical presentation of MND
The clinical presentation tends to be incidious and
depends on the part of the CNS affected
– Lower motor neurone degeneration - main features are
weakness, wasting and fasciculation of the muscles
– Upper motor neurone degeneration leads to weakness and
muscle wasting but the muscles become spastic
– Due to neural plasticity involving enlargement of surviving
motor neurones, up to 50% of motor neurones can be lost before weakness becomes apparent
Types of MND
There are 4 main types of MND:
– Amyotrophic lateral sclerosis (ALS) – Progressive muscular atrophy (PMA) – Progressive bulbar palsy (PBP)
– Primary lateral sclerosis (PLS)
Symptoms of MND
• Early symptoms are mild with pattern of symptoms
varying from person to person they can include: – Cramps
– Slurred speech
– Swallowing difficulties
– Slowed movement
– Muscles stiffness, twitching or jerking – Difficulty walking or holding objects
– Emotional responses trigger easily
Diagnosis of MND
Can be difficult initially because it resembles several other conditions.
• Clinical diagnosis is confirmed by electromyography (EMG) revealing widespread evidence of denervation due to anterior horn cell damage. Typically showing spontaneous fibrillations and fasciculations with giant spikes with voluntary movement.
management of MND
pharmacology
• Requires a multidisciplinary approach
• Symptomatic Drug Treatment
– Anticholinergics to reduce saliva secretion
– Baclofen or dantolene for spasticity – Quinine for cramp
– Anitdepressants
– Laxatives for constipation
– Opiates for terminal relief of dyspnoea – Anti-inflammatories for pain
management of MND
- Speech pathology for dysphagia management and communication aids and advice for carers for management of dysarthria
- Occupational therapy for assistance with ADLs and advise regarding modifications to the home and wheelchair prescription
- Medical input regarding medications and if assisted ventilation is required later in the disease.
overall physiotherapy management
• Based on patient’s goals
• Maintenance of mobility and ADLs (prevention of disuse
atrophy and overuse injury)
• Maintenance of muscle strength and length
• Prevention and minimisation of secondary problems
(includes contractures, DVT, decubitus ulcers and
respiratory infections)
• Energy conservation
• Provision of walking aids and orthoses as indicated
Exercise and MND
- Combine formal exercise with enjoyable physical activities
- More concentric than eccentric exercises
- Moderate resistance has been shown to be better than high resistance exercises
- Muscles with less than antigravity strength will not improve, focus on stronger muscles
- Monitor for overwork weakness and include adequate rest periods
- Anecdotally, overwork exercise can lead to a loss of muscle strength
phases of MND
Phase 1 - independant
– Stage 1 – mild weakness and independent with ADLs
(clumsiness but walking)
– Stage 2 – moderate weakness, difficulty with stairs (difficulty
raising arms, buttoning clothing but walking)
– Stage 3 – severe weakness, decreased independence in ADLs
(severe selective weakness in ankles, wrists and hands, moderately decreased independence with ADLs. Fatigues walking long distances, slightly decreased respiratory effort)
Phase II of MND - partially independent
– Stage 4 – Likely to have shoulder pain and oedema in the
hand. Severe lower limb weakness with or without spasticity
(able to perform ADLs but fatigues easily).
– Stage 5 – Severe lower limb weakness. Moderate to severe
upper limb weakness. (increasingly dependent in ADLs,
possible skin breakdown due to decreased mobility)
Phase III of MND - dependent
– Stage 6 – Bed ridden (completely dependent in ADLs)
Physio mgmt during stage 1 MND
– Increase activity if sedentary
– Begin exercise programme including ROM exercises, stretches, yoga, Tai Chi
– Add strengthening programme of gentle resistance exercises
physio mgmt during stage 2 MND
– Stretching to avoid contractures
– Continue cautious strengthening
– Consider orthotic support (AFO, thumb, wrist splints) – Adaptive equipment to facilitate ADLs (involve OT)
Physio mgmt during stage 3 MND
– Continue stage 2 programme as tolerated, monitor fatigue and
adjust programme accordingly
– Maintain independence for as long as possible through
pleasurable activities such as walking, etc
– Deep breathing exercises and postural drainage if needed
– Wheelchair prescription to reduce fatigue and maintain function
for as long as possible
Physio mgmt during stage 4 MND
– Heat & massage to control spasm and pain
– Preventative anti oedema measures (TED stockings, etc) – Active assisted ROM exercises
– Encourage isometric contractions as tolerated
– Consider motorised wheelchair for independent mobility
physio mgmt stage 5 MND
– Teach family and carers transfer and turning techniques
– Involve OT for home modifications and assistance with ADLs – Electric hospital bed and anti-pressure mattress
– Home mechanical ventilation
physio mgmt stage 6 MND
– Soft diet, tube feeds or percutaneous gastrostomy (PEG)
– Medication, suction for excess saliva
– Electronic speech aids for dysarthria
– For breathing difficulty; clear airways, tracheostomy, ventilator. – Medications to decrease impact of dyspnoea.
Background of GBS
- Classic lower motor neuron disorder
- Demyelinating neuropathy with ascending weakness.
- Subtypes
- Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) • Acute Motor Axonal Neuropathy (AMAN)
- Acute Motor-Sensory Axonal Neuropathy (AMSN)
- Miller Fisher Syndrome
- Acute panautonomic neuropathy
- Pure sensory GBS
- Other less common variants
- First documented cases of ascending weakness/paralysis occurred in 1859 and by 1916 three French physicians Guillian, Barre and Strohl reported on soldiers with motor weakness, areflexia, CSF protein changes and diminished deep tendon reflexes.
aetiology of GBS
- Unknown aetiology
- Post infectious immune mediating disease that targets peripheral nerves
- Autoimmune condition
- Viral or bacterial triggers -
- Respiratory or GI illnesses are common cause
- Less common – pregnancy, dengue fever, surgical procedures, insect bites, Bell’s Palsy.
Pathophysiology of GBS
- Exact pathogenesis is unclear despite being the most common cause of acute paralysis.
- Abnormal immune response destruction of Schwann cells leading to demyelination/inflammation NCV slowed/blocked.
- After 2-3 weeks the Schwann cells begin to proliferate and there is reduction in inflammation accompanied by remyelination.
Epidemiology of GBS
- Reported throughout the world with nil racial preponderance identified.
- In Australia, North America and Western Europe most people with GBS display demyelinating polyneuropathy whereas in northern China 65% of people with GBS have axonal pathology.
- Male to female ratio is 1.5:1 in most parts of the world.
- Occurs in all age groups from infancy to older age however infants at lowest risk. •Two common peaks for age of onset are 15-35 yrs and then again at 50-75 yrs.
Presentation of GBS
- Weakness – bilateral
- Reduced or absent reflexes
- Sensory disturbances
- Pain – shoulders, back, buttocks and thighs
Cranial nerve involvement in GBS
•Facial droop •Diplopia
•Dysarthria •Dysphagia •Opthalmoplegia •Pupillary disturbance
nerves 3-7 and 9-12
autonomic changes in GBS
- Tachycardia or bradycardia
- Facial flushing
- Paroxysmal or orthostatic hypertension •Anhidrosis and/or diaphoresis •Urinary retention
Respiratory changes in GBS
- SOBOE
* SOB at rest •Slurred speech •Dysphagia
Diagnosis of GBS
- Difficult to diagnose due to vague and unrelated nature of symptoms •Physical exam
- Muscle strength testing
- Muscle activity testing
- Reflex testing (especially knee jerks) •NCV testing
- Lumbar puncture
- Lung function
MDT mgmt of GBS
Doctors – ED/ICU/Neurologist Physiotherapy
Occupational Therapy Speech/Language Pathologist Social Worker
Nursing
medical mgmt of GBS
Plasmaphoresis IVIG
Supportive care Ventilatory Support
Autonomic dysfunction
Prevention of secondary infections Prevention of DVT/PE
physiotherapy mgmt of GBS
Maintain clear airway and prevent lung infection Maintain joint ROM
Joint preservation
Prevention of pressure area formation
Maintain peripheral circulation
Psychological support for patient and family
pain management
OT mgmt of GBS
- Pressure relief/positioning •Posture/upper body strengthening •Equipment/aid prescription
- Promotion of independence in self care
speech/language pathology - GBS
Management of dysphagia Management of dysarthria
Prognosis of GBS
Approx. 9/10 people with GBS survive. 75-90% will recover completely.
10-15% will have some ongoing disability.
Prognosis appears to correlate with timing of symptoms easing however can take more than 2 years to fully recover.
