Neuroscience Clinical lecture MS and GB

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NeuroscienceClinical lecture MS and GB

• Reference
• Lindsay and Bone Neurology and Neurosurgery
  Illustrated

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Multiple sclerosis

• INTRODUCTION
• demyelinating disease primarily of the white
  matter of the central nervous system
• highly variable disease both between and within
  patients
• characterised by periods of exacerbation
  (worsening) and remissions and widespread CNS
  involvement due to the disseminating nature of MS
• disseminated in time (due to relapses) and space
  (due to varied anatomical location

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Overview of pathology

• cause is unknown but there are theories of
  pathogenesis
• include immune deficiency, hereditary / genetic
  factors, viruses, biochemical factors and
  environment eg temperate climate
• Common pathological findings are lesions called
  plaques within the white matter of the brain and
  spinal cord
• vary in size (1mm to a few cm) and are scattered
  throughout the CNS but are present in a
  perivenous distribution ie. close to veins
• Recent lesions show destruction of myelin with
  relative sparing of the axon

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• breakdown of the blood brain barrier with
  interstitial oedema and infiltration by
  lymphocytes and mononuclear cells
• Astrocyte proliferation occurs
• Older lesions are relatively acellular and bare
  axons within plaques are surrounded by astrocytes
• inflammation and demyelination affects nerve
  conduction leading to deficits as propogation of
  impulses changes eg slow conduction, inability
  to conduct fast trains of impulses, conduction
  block, spontaneous firing
• Some remyelination may occur, but it is rarely
  complete.

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Clinical onset and course

• These are highly variable and range from
• Acute sudden and severe onset with dramatic
  recovery and long phase of remission
• Relapsing progressive or chronic progressive
• Relapsing remitting with progressive disability
• Benign abrupt onset but with good remission and
  long periods without relapses

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Diagnosis

• Diagnosis of MS is difficult and is made on the
  basis of
• Clinical examination
• CSF changes gammaglobulin, oligoclonal bands
• Neurophysiological investigation eg sensory
  evoked responses such as delayed visual evoked
  responses
• CT scan / MRI more sensitive than CT scans to
  detect multiple lesions

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Signs and symptoms

• Vary depending on the disease process AND
  location of plaques
• Vague symptoms at onset headache, lack of
  energy, aches in limbs, depression
• More specific symptoms at onset sensory changes
  (numbness and paraesthesias), visual changes eg
  from retrobulbar neuritis such as blurred vision,
  limb weakness / clumsiness, vertigo, ataxia
  (incoordination), sphincter disturbances,
  trigeminal neuralgia (facial pain)
• Sensorimotor changes may reflect involvement of
  the spinal cord or brain (UMNL), cerebellum,
  basal ganglia or ascending / descending tracts
  within the brainstem
• Other symptoms include emotional and cognitive
  symptoms such as attentional deficits, mood
  changes, personality changes and emotional
  lability Lhermittes sign (sudden neck flexion
  causes shock like pain in limbs) Uhhtoffs sign
  (visual changes following increase in temperature
  eg from a hot bath or exercise) fatigue

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• Long term the patient may remain independently
  mobile and working or may be wheelchair bound
  with severe disability. At any one time
  approximately 1/3 of patients will be in the
  remitting stage and will have minimal disability,
  1/3 are slowly deteriorating and 1/3 are stable
  but disabled having had the disease for some time.

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Long term

• There is no cure for MS and little evidence for
  the majority of treatments
• Medical management includes steroids
  (anti-inflammatory eg methylprednisolone) and
  immunosuppressive therapy as well as supportive
  therapy based on signs and symptoms eg for
  treatment of spasticity, bladder problems, pain,
  seizures, fatigue, neurobehavioural disorders
• Therapy such as physiotherapy based on signs and
  symptoms
• A range of other treatments have been tried and
  include relaxation / yoga, hyperbaric oxygen
  therapy, diet, body cooling

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Rehabilitation

• The diagnosis of MS is associated with many
  emotions, stress and fear of severe disability.
• for those newly diagnosed is to be educated about
  the disease process
• based on the needs of the patient and resources
  available to improve quality of life
• support services are available including
  self-help and support groups within the community
• The Multiple Sclerosis Centre provides a range of
  services including periodic reviews, treatment
  programs including hydrotherapy, relaxation
  classes, wheelchair / seating advice and support.

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Physiotherapy

• A physiotherapists role in the management of MS
  is to maximise function particularly after a
  relapse
• General aims of rehabilitation are to
• Prevent disability and handicap
• Assess and treat movement disorders to maximise
  function such as walking, climbing stairs, moving
  between positions (rolling, lie to sit, sit to
  stand), upper limb function (reach, grasp,
  manipulation, writing) and to return to or
  maintain functional activity such as work,
  recreation and ADL
• Assess and treat balance problems to prevent or
  minimise falls

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• Maintain general fitness and aerobic capacity,
  muscle length and strength and general posture
• Educate about movement strategies and modify the
  environment for both patient and their carers and
  the availability and use of aids and equipment
  such as walking aids
• Educate about the disease process and factors
  that can worsen symptoms such as fatigue and heat
• Educate about safety for patient and carers
  (including back care)
• Improve quality of life

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Guillain Barre

• INTRODUCTION
• Guillain Barre is a demyelinating disease of the
  peripheral nervous system.
• It is an acute inflammatory post-infectious
  polyneuropathy as it generally occurs after an
  infection such as viral illness or after
  immunisation.

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Pathology and prognosis

• More than half of the patients with GB note a
  flu-like illness 2-4 weeks prior to symptoms.
• may cause a cell mediated immunological reaction
  (decreased supressor T-cell response) and
  segmental demyelination of peripheral nerves
• Axonal damage occurs if the process is severe.
• There is perivascular infiltration of lymphocytes
  within nerve roots and damage to Schwann cells
  and myelin.
• Myelin is removed by macrophages
• remyelination/regeneration will occur if axon
  damage and nerve cell death do not occur.

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• Acute onset - change from normal function to
  bedridden and on a ventilator within 2-3 days.
• Other patterns of onset are stuttering or gradual
  over weeks.
• Recovery usually begins about three weeks after
  onset, with a plateau phase of about 10-14 days.
  
• The illness duration is usually less than 12
  weeks and prognosis is generally good.
• Residual deficits in mobility and pulmonary
  function are seen in approximately 20 of
  patients. Some patients suffer from relapse of
  the disease.
• Approximately 10 of patients die mainly from the
  cardiorespiratory symptoms of the disease.

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Common investigations

• Clinical assessment
• Nerve conduction studies / EMG
• CSF investigation raised protein and
  gammaglobulin
• Viral studies

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Signs and symptoms

• These reflect the generalised LMNL with
• Sensory changes at onset - distal paraesthesia
  (feet and hands)
• Pain
• Weakness often begins distally and rapidly
  ascending or begins as generalised weakness
  weakness has a symmetrical distribution
• Low tone and loss of reflex activity
• Cranial nerve involvement eg problems with
  swallowing, speech, vision, hearing, facial
  movements
• Autonomic changes eg fluctuating BP, sinus
  tachycardia,, urinary retention, cardiac
  arrythmias
• Patient may require tracheostomy and ventilatory
  support
• Complications include respiratory problems, DVT,
  autonomic dysfunction, pain and anaemia

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Early management

• In severe cases patient managed in ICU,
• may include plasmaphoresis, corticosteriods and
  immune globulins and symptomatic therapy
• The general aims of management in the acute stage
  are to maintain respiratory function and prevent
  / manage complications. The physiotherapist will
  be involved with
• respiratory care
• management of the musculoskeletal system (which
  may include positioning and splinting such as
  resting splints, muscle stretch and gentle
  strengthening)

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• early mobilisation once medically stable (eg
  moving around the bed, sitting on the edge of the
  bed, standing on a tilt table)
• skin care
• awareness of fatigue, cardiac problems and
  unstable BP, pain
• education and support
• At this stage nurses and other allied health
  staff will be involved with patient care and will
  depend on specific needs of the patient.

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rehabilitation

• multi-disciplinary team approach
• Rehabilitation programs vary
• general aims are to maximise function and quality
  of life by reducing disability

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Physiotherapy

• A physiotherapists role in the management of GB
  is to maximise function. General aims of
  rehabilitation are to
• Maintain clear chest
• Maintain joint range and improve muscle strength
  / length
• Retrain balance
• Teach functional skills eg moving in bed,
  transfers, gait (including use of walking aids
  and splints such as ankle foot orthoses (AFO) if
  required), upper limb
• Educate patient, family and carers
• Note that neuropathies may occur due to other
  causes such as diabetes, alcoholism, HIV,
  peripheral vascular disease and hereditary causes.