Transcript Guillain-Barre Syndrome

Guillain-Barre Syndrome
DR. INTEKHAB AHMAD
25 JAN 2010
Definition
It is an acute inflammatory
demyelinating polyneuropathy
leading to progressive muscle weakness &
areflexia.
Guillain Barre Syndrome
Commonest cause of rapid-onset flaccid
paralysis since polio decline
 Occurs as an autoimmune response
following a GI or respiratory infection
 Potentially severely debilitating disorder
affecting 1-3 per 100,000
 10% die from associated complications
 A further 10% suffer from long term
neurological sequelae and physical
dependence
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History
Guillain, Barre and Strohl first
described a disease affecting French
soldiers ( motor weakness, areflexia and
CSF abnormalities) in 1916
 Descriptions date back to 1859 when
Landry described ascending paralysis
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Aetiology
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All ages affected with bimodal distribution
towards young adults and the elderly
Slight male preponderance
Children less severely affected
Most commonly occurs within a month of GI
or resp upset.
Commonest organism is campylobacter
Others inc EBV, CMV, HIV, herpes &
mycoplasma
Have been reports of association with
vaccines, surgery, epidurals, bone
marrow and organ transplantation, SLE,
lymphoma, sarcoidosis
 Pregnancy and OCP confer some
protection
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Pathogenesis
Immunologically mediated nerve injury
 Inflammatory cell infiltrates are seen in
association with the demyelination, which is
regarded as the primary pathological process
 Precise mechanism of sensitisation not known
 Antibodies formed against nonself antigens
(inf agents,vacc) but misdirected to host
nerve tissue (neural target- gangliosides) due
to molecular mimicry leading to demyelination
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Figure 1. Structural components of peripheral nerves. In the endoneurial compartment (En), a
single Schwann cell envelops several unmyelinated axons, and another Schwann cell provides
multiple wrappings of plasma membrane forming the myelin sheath of a myelinated axon. The
portion of a myelinated axon myelinated by a single Schwann cell is called the internode, and
internodes are separated by nodes of Ranvier. Schwann cells associated with both unmyelinated
and myelinated axons are covered with a continuous basal lamina (BL). Capillaries (Cap) are
present within the endoneurial compartment, and collagen fibers (Col) run primarily longitudinally
between the axons. The axons, Schwann cells, collagen, and endoneurial fluid are bundled into a
fascicle by the perineurium (Pe). The perineurium consists of several layers of flattened
perineurial cells connected by tight junctions and covered internally and externally by a basal
lamina. The layers of perineurial cells are separated by collagen fibers (Col) oriented obliquely.
Several fascicles are bundled together by the epineurium (Ep) to form a nerve. The epineurium
consists primarily of fibroblasts, collagen fibers (Col), and elastic fibers. The epineurium between
fascicles is termed the interfascicular epineurium, and that encompassing all of the fascicles is
termed the epifascicular epineurium. Arterioles (A) and veins are oriented primarily longitudinally
within the epineurium.
Distributed throughout the nerve length but
focused at nerve roots, spinal nerves and
major plexuses
 Macrophages actively strip myelin from
bodies of schwann cells and axons
 Underlying immune response is complex
 Effectiveness of plasma exchange and IgG is
thought to be blocking of demyelinating
antibodies
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VARIANTS
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Several distinct clinical pictures described
 Acute inflammatory demyelinating
polyradiculopathy (AIDP)
 Acute motor axonal neuropathy (AMAN)
 Acute motor sensory axonal neuropathy
(AMSAN)
 Miller-Fisher syndrome ( ataxia, areflexia
and opthalmoplegia ) which may be
accompanied by limb weakness, ptosis and
facial and bulbar palsy
Clinical presentation
Classical picture is that of ascending limb
weakness with areflexia, although a purely
sensory variant has been well documented
 Features of GBS include
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Progressive motor weakness, usually ascending
from the legs
Areflexia
Facial palsy and bulbar weakness
Sensory symptoms—mainly subjective
Bladder/bowel—in severe cases
 Severe
pain esp girdle
 Resp muscle weakness
 Autonomic dysfunction( over or
underactivity of the SNS or PSNS)
Features required for diagnosis
defined by national institute of neurological and
communicative diseases and strokes
Progressive muscle weakness of more
than one limb
 Areflexia or marked hyporeflexia
 CSF cell counts of no more than 10
 Features highly suggestive
 Features required to rule out other
diagnosis
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Doughtful diagnosis
Definite sensory level
 Marked, persistent asymmetry of motor
weakness
 Severe & persistent bladder/bowel
dysfunction
 CSF cell count > 50
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Monitoring
Cardiac
 Blood pressure
 Vital capacity measured three times a
day
 Bulbar function monitored to prevent
aspiration
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Investigations
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In over 90% patients CSF protein is raised (
>0.4g/l) within a week of onset of symptoms
Level does not correlate with clinical findings
Nerve conduction studies demonstrate
reduced conduction velocity
Liver and renal function may be impaired
Antiganglioside antibody should be searched
for
SIADH may occur in association with
GBS
 Stool cultures for campylobacter
 Ecg’s for QT, T and ST abnormalities
 Head CT to exclude raised ICP and
other pathology prior to LP
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Treatment
Major challenge
 Outcome excellent if complications
treated or avoided
 Prevented by meticulous attention to
detail
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Specific treatment-disease
modifying modalities
Plasma exchange
 Immunoglobin
 Both should be used when patient nonambulatory or resp decompensation
occurs
 Both have been examined in RCT and no
difference in efficacy demonstrated
between them
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Plasma exchange
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2 RCT showed reduction in ventilation and
reduced time to motor recovery
Mortality was not altered
Most effective within 7 days of onset
3-5 exchanges of 1-2 plasma volumes each
over 1-2 weeks
Ffp more complications than albumin
CI include CVS instability, sepsis and
haemostatic problems
Side effects are hypotension,
hypocalcaemia,coagulation abnormalities
and sepsis
IV Immunoglobin
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0.4mg/kg daily for 5-6 days
Easier administration
Fewer side effects
Commence tx within 2 weeks of onset of
symptoms
CI include IgA deficiency(anaphylaxis)
Renal function may deteriorate
Severe congestive cardiac failure major
contraindication
RCT has suggested as effective as plasma
exchange
Steroids
No place in the treatment of GBS
 RCT have shown no advantage
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CSF filtration
Few case reports
 When plasmapheresis and IgG have
failed
 Logistics difficult!
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Supportive care
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Respiratory,
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25% require ventilated
Physio and VC monitoring in the spont breathing.
If less than 15ml/kg or rising pCO2, mechanical
ventilation likely
Monitoring of bulbar function for prevention of
aspiration
Non-invasive ventilation often not useful as does
not eliminate the problem of not being able to
clear secretions due to poor cough
Early tracheostomy should be considered
Supportive care
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Cardiovascular
 Full invasive monitoring
 Care with induction of anaesthesia as leads
to hypotension and arrhythmias
 Care with suxamethonium may lead to
arrhythmias
 Instability may be worsened by other drugs
 Autonomic instability common
Supportive care
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Nutrition, fluid and electrolytes
Paralytic ileus common
Tpn may be required
Energy and fluid requirements are reduced
in these patients
Physiotherapy
DVT prophylaxis
Sepsis survellience
Psychological care
analgesia
Prognosis
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Death in up to 25% of those who require
ICU has been reported often from
autonomic abnormalities
Approx 16% patients suffer permanent
disability
Those who require ventilation, improve
after more than 3 weeks, not improved
within 1 month have greater risk of poorer
outcome
Gradual improvement may occur over
18months -2years
Recurrence n 2-5% cases
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