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| Wikidoc
| | <ref name="pmid26223336">{{cite journal| author=Modi S, Dharaiya D, Schultz L, Varelas P| title=Neuroleptic Malignant Syndrome: Complications, Outcomes, and Mortality. | journal=Neurocrit Care | year= 2016 | volume= 24 | issue= 1 | pages= 97-103 | pmid=26223336 | doi=10.1007/s12028-015-0162-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26223336 }} </ref> |
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| Acute Inflammatory Demyelinating Polyradiculoneuropathy
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| ==Introduction:== | |
| *Classified under the eponym Guillain-Barre syndrome (GBS)
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| *Other variants of GBS include Acute Motor Axonal Neuropathy (AMAN), Acute Motor and Sensory Axonal Neuropathy (ASMAN), and the Miller Fisher syndrome (MFS).
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| *AIDP is acute monophasic immune-mediated polyradiculoneuropathy provoked by a preceding infection
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| ==Epidemiology:== | |
| *Mean age of onset of 40 years affecting slightly more males than females of all ages, races and nationalities
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| *Worldwide incidence of GBS ranges from 0.6 to 4.0/100,000 people
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| ==Etiology:== | |
| *AIDP is most common form of GBS in North America, Europe and most of the developed world representing about 85% to 90% of cases
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| *⅔ patients give history of antecedent respiratory tract or GI tract infection
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| *Campylobacter infection is most commonly observed in upto 30% cases
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| *Campylobacter associated GBS has worse prognosis, manifests slow recovery and with greater residual neurologic disability.
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| *Other precipitants include EBV, CMV, mycoplasma, pneumonia, and influenza-like illnesses
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| *Also has an association with HIV infection; predominantly in those who are not profoundly immunocompromised
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| ==Pathogenesis:== | |
| *Molecular Mimicry: auto-antibodies cross-react with peripheral nerve components because of sharing of cross-reactive epitopes
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| *Immune response can be directed towards myelin or axon of peripheral nerve
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| *Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP): When directed against epitopes in myelin or schwann cell membrane; cellular + humoral immune responses are involved
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| *Progression of disease for about two weeks
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| *Nadir of disease reaches 4 weeks after initial symptoms in 90% patients
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| *If disease progression is more than 8 weeks, it is classified as chronic inflammatory demyelinating polyradiculoneuropathy or CIDP
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| ==Clinical Features:== | |
| *Weakness starts in the legs (90%)
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| *Decreased or absent reflexes in affected arms or legs (90%)
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| *Paresthesias accompanying weakness (>80%)
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| *Dysautonomia (~70%)
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| *Pain due to nerve root inflammation, typically in the back and extremities (two thirds of patients)
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| *Facial nerve Palsies (>50%)
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| *Oropharyngeal weakness (50%)
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| *Severe respiratory muscle weakness requiring ventilatory support (10%-30%)
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| *Oculomotor weakness (15%)
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| *Weakness begins in arms and facial muscles (10%)
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| *SIADH (5%; more in hospitalized patients)
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| *Unusual clinical features of GBS include: papilledema, facial myokymia, hearing loss, meningeal signs, vocal cord paralysis, and mental status changes.
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| *Posterior reversible encephalopathy syndrome, also called reversible posterior leukoencephalopathy syndrome has been associated with GBS in children and adults likely related to acute hypertension from dysautonomia
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| ==Electrodiagnostic findings:== | |
| Earliest findings:
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