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| {{Guillain-Barré syndrome}} | | {{Guillain-Barré syndrome}} |
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| {{CMG}}; {{AE}} [[Priyamvada Singh|Priyamvada Singh, MBBS]] [mailto:psingh13579@gmail.com] | | {{CMG}}; {{AE}} {{Fs}} |
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| ==Overview== | | ==Overview== |
| There are several types of GBS, but unless otherwise stated, GBS refers to the most common form, [[acute inflammatory demyelinating polyneuropathy]] (AIDP). It is frequently severe and usually exhibits as an [[ascending paralysis]] noted by weakness in the legs that spreads to the upper limbs and the face along with complete loss of [[deep tendon reflexes]]. The other less common variants involve [[Miller Fisher syndrome]], [[acute motor axonal neuropathy]] (AMAN), [[acute motor sensory axonal neuropathy]](AMSAN), acute panautonomic neuropathy and [[Bickerstaff's brainstem encephalitis]] (BBE).
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| ==Classification== | | ==Classification== |
| ===Acute inflammatory demyelinating polyneuropathy===
| | Guillain barre syndrome may be classified according to the underlying pathophysiology into four groups: |
| * Commonest form of GBS, and the term is often used synonymously with GBS.
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| * Caused by an auto-immune response directed against [[Schwann cell]] membranes.
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| * Commonly preceded by a bacterial or viral infection.
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| * [[Campylobacter jejuni]] is the commonest causative agent (positive in approximately 2 out of 5 patients).
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| * Peripheral nerve demyelination is present. Symptoms generally resolve with remyelination.
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| ===Acute Motor Axonal Neuropathy (AMAN) <ref name="McKhann1991">{{Cite journal|author=McKhann GM, Cornblath DR, Ho T, ''et al'' |year=1991 |month= |title=Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China |journal=Lancet |volume=338 |issue=8767 |pages=593–7 |pmid=1679153 |url= |accessdate= |quote= |doi=10.1016/0140-6736(91)90606-P }}</ref>===
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| * Also known as '''Chinese paralytic syndrome'''
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| * Prevalent in China and Mexico.
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| * The disease may have seasonal variations.
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| * Recovery can be rapid.
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| * Due to auto-immune response directed against the [[axoplasm]] and [[nodes of Ranvier]] of [[peripheral nervous system|peripheral nerves]].
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| * Anti-GD1a antibodies<ref name="Ho1995">{{Cite journal|author=Ho TW, Mishu B, Li CY, ''et al'' |title=Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies |journal=Brain |volume=118 ( Pt 3) |issue= 3|pages=597–605 |year=1995 |pmid=7600081 |doi= 10.1093/brain/118.3.597}}</ref> are present. [[Anti-ganglioside antibodies#Anti-GD3|Anti-GD3]] antibodies are commonly found associated with it.
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| ===Acute Motor Sensory Axonal Neuropathy (AMSAN)===
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| * It is similar to [[AMAN]] however unlike AMAN it also affects sensory nerves <ref name="pmid12391383">{{cite journal |author=Winer JB |title=Treatment of Guillain-Barré syndrome |journal=[[QJM : Monthly Journal of the Association of Physicians]] |volume=95 |issue=11 |pages=717–21 |year=2002 |month=November |pmid=12391383 |doi= |url=http://qjmed.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=12391383 |accessdate=2012-02-19}}</ref>.
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| * It is probably due to an auto-immune response directed against the [[axoplasm]] of peripheral nerves.
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| * Recovery is slow and often incomplete.<ref name="Griffin1995">{{Cite journal|author=Griffin JW, Li CY, Ho TW, ''et al'' |title=Guillain–Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases |journal=Brain |volume=118 ( Pt 3) |issue= 3|pages=577–95 |year=1995 |pmid=7600080 |doi= 10.1093/brain/118.3.577}}</ref>
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| ===Pure Sensory===
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| * Rapid onset of sensory loss and [[areflexia]] in a symmetric pattern.
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| * Lumbar puncture studies show albuminocytologic dissociation as seen with other GBS
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| * Electromyography show characteristic signs of a [[demyelination]].
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| * Prognosis is usually good.
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| ===Miller Fisher Syndrome===
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| * Accounts for approximately 5% of GBS cases
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| * Unlike Acute inflammatory demyelinating polyneuropathyit manifests as a descending [[paralysis]]
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| * It usually affects the [[human eye|eye]] muscles first and presents with the triad of [[ophthalmoplegia]] (external) <ref name="pmid17130419">{{cite journal |author=Kimoto K, Koga M, Odaka M, Hirata K, Takahashi M, Li J, Gilbert M, Yuki N |title=Relationship of bacterial strains to clinical syndromes of Campylobacter-associated neuropathies |journal=[[Neurology]] |volume=67 |issue=10 |pages=1837–43 |year=2006 |month=November |pmid=17130419 |doi=10.1212/01.wnl.0000244468.22377.6b |url=http://www.neurology.org/cgi/pmidlookup?view=long&pmid=17130419 |accessdate=2012-02-19}}</ref>, [[ataxia]] and [[areflexia]] <ref name="pmid13334797">{{cite journal |author=FISHER M |title=An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia) |journal=[[The New England Journal of Medicine]] |volume=255 |issue=2 |pages=57–65 |year=1956 |month=July |pmid=13334797 |doi=10.1056/NEJM195607122550201 |url=http://www.nejm.org/doi/abs/10.1056/NEJM195607122550201?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmed |accessdate=2012-02-19}}</ref>.
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| * The ataxia predominantly affects the gait and trunk, with the limbs relatively spared.
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| * [[Anti-ganglioside antibodies#Anti-GQ1b|Anti-GQ1b]] antibodies are present in 90% of cases <ref name="pmid8413947">{{cite journal |author=Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I |title=Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies |journal=[[Neurology]] |volume=43 |issue=10 |pages=1911–7 |year=1993 |month=October |pmid=8413947 |doi= |url= |accessdate=2012-02-19}}</ref>.
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| ===Acute Panautonomic Neuropathy===
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| * Most rare variant of GBS
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| * Prognosis may be poor because of its association with [[encephalopathy]] and cardiovascular involvement, and associated [[cardiac arrhythmia|dysrhythmias]].
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| * Initial symptoms of lethargy, fatigue, headache can be present.
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| * Common symptoms include impaired sweating, lack of tear formation, [[photophobia]], dryness of nasal and oral mucosa, itching and peeling of skin, [[nausea]], [[dysphagia]], and constipation or alternating with diarrhea.
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| * Autonomic symptoms like orthostatic hypotension, lightheadedness, blurring of vision, abdominal pain, diarrhea, dryness of eyes, and disturbed [[micturition]] can be seen.
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| * [[Parasympathetic]] impairment (abdominal pain, vomiting, constipation, ileus, urinary retention, dilated unreactive pupils, loss of accommodation) may also be observed.
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| ===Bickerstaff's Brainstem Encephalitis (BBE)===
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| * Another rare variant of GBS
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| * Associated with acute onset ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or [[Plantar reflex|Babinski's sign]]. * It can have monophasic or remitting-relapsing progression.
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| * Neuroimaging with [[Magnetic resonance imaging]] (MRI) plays a critical role in the diagnosis.
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| * Large, irregular hyperintense lesions located mainly in the brainstem, especially in the [[pons]], [[midbrain]] and [[medulla]] can be seen.
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| * Prognosis is good despite the initial severe presentation
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| * It is commonly found associated with axonal Guillain–Barré syndrome suggesting some linking between the two disorders
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| {| class="wikitable" | | {| class="wikitable" |
| ! style="background: #4479BA; text-align: center;" |Subtypes | | ! style="background: #4479BA; text-align: center;" |Subtypes |