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