Introduction “It is an acquired ,frequently severe ,monophasic autoimmune illness of Peripheral Nervous System(PNS )” 10/14/2010 4
Alternative Names Landry-Guillain-Barre-Strohl Syndrome Post-Infectious Polyneuropathy Acute Idiopathic Polyneuropathy
After the advent of OPV ,today GBS is almost the only inflammatory Polyneuropathy and most frequent cause of acute flaccid paralysis in general medical practice 10/14/2010 6
Approximately 85% patients recover spontaneously while 10% patients need hospitalization Its prevalence has been reported to vary from region to region 10/14/2010 7
Epidemiology According to more recent study ,GBS occurs throughout the world with a medium incidence of 1.3 cases per 100,000 population Males are more commonly affected than females Peaks in young adults and in elderly
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Sub-types of GBS 10/14/2010 10
Acute Inflammatory Demyelinating Polyneuropathy(AIDP) Prevalent in western countries (90% of the GBS cases) Adults are affected more than the children First attack appears directed against a component of Schwann cell
AIDP cont’d… Cause of flaccid paralysis & sensory disturbances is the block of conduction ,whereas axonal connection remains intact Recovery is most often rapid as remyelination occurs In the severe forms of AIDP ,when axonal damage occurs , the rate of recovery is slower ,& the degree of residual disability greater
Primary Acute Motor Axonal Neuropathy (AMAN) Prevalent in China & Mexico with seasonal prevalence Children and young subjects are affected more than adults First attack appears directed against the axolemma & Nodes of Ranvier
AMAN cont’d… Axonal damage is the prominent pathological alteration Recovery takes place when axon regeneration is complete and it is rapid when lesion is localized 10/14/2010 15
AMAN cont’d… Usual Electro diagnostic features; In case of primary axonal damage there is reduced amplitude of compound action potential (without conduction slowing or prolongation of distal latencies)
Acute Motor-Sensory Axonal Neuropathy(AMSAN) Very rare Closely related to AMAN Adults are mostly affected
AMSAN cont’d.. First attack is directed at motor nodes of Ranvier ,but also affects Sensory nerve and roots Axonal damage is severe Recovery is slow and often incomplete
Miller-Fisher Syndrome Adults ,young subjects and children are affected Involves PNS & CNS structures Pathological features resemble that of AIDP
MFS cont’d… Characterized by rapidly evolving of Triad ; Variable opthalmoplegia (often with pupillary paralysis) Ataxia Tendon areflexia (without weakness) Recovery can be rapid
Etiology (Predisposing or Antecedent events in GBS )
Pregnancy and GBS Even though maternal GBS is very rare there may be approximately 6% chances of GBS development during pregnancy Cause is idiopathic
Malignancy and GBS GBS has also been described in association with malignancy ,in these instances GBS may be considered in a secondary event
Pathogenesis Classic studies in man and experimental animals and several lines of evidence support on immunological basis for demyelination of peripheral nerves in GBS patients
Pathology DEMYELINATION is the main type of pathophysiological lesion Characteristics of GBS is the “Segmental Demyelination ” with mononuclear cell infiltration in spinal roots ,proximal nerve trunks ,distal nerves and autonomic ganglia
Pattern of re-myelination
In GBS primary demyelination of CNS is not found. Other changes such as degeneration of spinal posterior tract are secondary to pathology in the PNS 10/14/2010 27
Clinical features Rapidly evolving areflexic ascending motor paralysis of the extremities ,up to the tetraparesis Reduced or absent deep tendon reflexes Mild sensory symptoms
Diagnosis Observation of the patients symptoms and evaluation of the medical history provide the basis for the diagnosis of GBS ,although no single observation is suitable to make the diagnosis 10/14/2010 32
Diagnosis cont’d… Past medical history Laboratory findings a. Lumbar puncture an elevated level of protein without an in the no. of WBC in the CSF is he characteristic of GBS b. Electromyogram show the loss of individual nerve impulses due to the disease ‘s characteristic slowing of nerve responses c. NCS these signals are characteristically slowed in GBS 10/14/2010 33
DISABILITY CRITERIA In most studies, the primary outcome measure used disability scale, where: 0 = normal 1 = symptoms but able to run 2 = unable to run 3 = unable to walk unaided 4 = bed-bound 5 = needing ventilation 6 = dead 10/14/2010 34
Management
MEDICATION 10/14/2010 36
Medical management IVIg Plasmapheresis 10/14/2010 37
PLASMAPHERESIS 10/14/2010 38
a- Pain NSAID Acetaminophen with Hydrocodone b-Unpleasant sensations such as painful tingling Tricyclic antidepressants Anti convulsants Corticosteroids ,which often effectively treat the symptoms of autoimmune disorder actually worsen GBS and should not be used 10/14/2010 39
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“The physiotherapist was a most welcome person ,as ,despite the discomfort endured to have ‘dead’ limbs stretched and repositioned ,this left me comfortable for several hours.” Clark,1985 10/14/2010 42
Physiotherapy Management
ACUTE PHASE It is a phase when patient is admitted in hospital GOAL: Respiratory care Prevention from Decubitus Ulcer Prevention from Contracture formation Prevention from DVT Maintain peripheral circulation Assist in swallowing (feeding)
RESPIRATORY MANAGEMENT If patient is on ventilator , Suctioning can be done (if required) Huffing-coughing
Areas which are most affected in different positions PREVENTION FROM DECUBITUS ULCER By Repositioning By Devices (e.g. pneumatic gloves) Through diet 10/14/2010 52 PREVENTION FROM DECUBITUS ULCER
PREVENTION FROM DVT Begin ambulation as soon as possible Anticoagulant as a prophylactic Rx Active pumping ex’s Keep lower extremities elevated
FROM CONTRACTURES Generalized ROM ex’s Spinal movements should be included e.g. Double knee-and-hip flexion, Knee rolling and Neck movements with due care of tracheal tubes 10/14/2010 54
Double knee-and-hip flexion 10/14/2010 55
Knee rolling 10/14/2010 56
NECK MOVEMENTS 10/14/2010 57
10/14/2010 58 Risk of hypotension is reduced by ; Ensuring that turning is gentle Avoiding any intervention if CVP is below 5cmH 2 O Acclimatization to the upright posture with a tilt table Risk of bradycardia is reduced by oxygenation before and after suction
ASSIST IN SWALLOWING By positioning Keep head upright with slight extension (elevated-45degree)
PHASE II (when patient maintain his respiration) 10/14/2010 60
Pain management-TENS ACBT’S Stretching Strengthening and endurance ex’s Paced breathing Aerobic ex’s Energy conservation Improve swallowing 10/14/2010 61
Interventions for Strengthening may include; PROM AAROM AROM ARROM By means of EMG biofeedback PNF Rhythmic Initiation Rhythmic Stabilization Repeated contractions Hold Relax 10/14/2010 64
EMG BIOFEEDBACK 10/14/2010 65
position for ex’s: sitting or prone on elbow To keep the chewed food inside the mouth patient must be able to hold their lips closed ,can improved by ex’s of facial muscles & tongue movements Improve Swallowing 10/14/2010 66
Tongue movements 10/14/2010 67
As the swallowing continues ,the hyoid bone and larynx moves upward. To stimulate the muscles that elevate the larynx use quick ice and stretch .Give the stretch diagonally down to the right and. then to the left. 10/14/2010 68
PHASE III (when patient have good strength of muscles) 10/14/2010 69
Strengthening Stretching Improve gripping Balancing (Tai chi) Hydrotherapy Gait training Prevention from medical complications and sequlae 10/14/2010 70
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T A I C H I 10/14/2010 74
To improve gripping 10/14/2010 75
SPEECH THERAPY Stimulation of the laryngeal muscles with quick ice followed by stretch and resistance to the motion of laryngeal elevation Promote controlled exhalation during speech with resisted breathing exercises 10/14/2010 76
HOME PROGRAM Breathing ex’s Stretching ex’s Walking Jogging
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SEQUELAE GBS may leave sequelae that are unpredictable Most serious residual disability was found distally in the legs Residual severe neurological deficits Muscle aches and cramps
PROGNOSIS The length of time and the amount of effort required to bring about the best possible recovery varies among individuals and is related primarily to the severity of the symptoms. About 30% of persons affected with GBS have some degree of residual weakness after three years. 3 - 5% may suffer a relapse many years later. 1- 5% of cases are fatal, usually due to respiratory or cardiac complications. Most people, however, are able to recover completely and lead normal lives.
REFRENCES Guillain-Barre syndrome: pathological, clinical, and therapeutical aspects By Silvia Iannello PNF in practice –An illustrated guide Adler ,Beckers ,Buck Therapeutic exercises Kisner http://neurologychannel.com/guillain http://en.wikipedia.org/wiki/guillain-barre-syndrome/ 10/14/2010 81
GOLDEN WORDS “Your main occupation should be — in fairness to yourself, in fairness to your parents, in fairness to the state – to devote your attention to your studies.” (Mohammad Ali Jinnah-March 21 ,1948) 10/14/2010 82