Week 8 : MS, MND and GBS Flashcards
(57 cards)
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