Guillain-Barre SyndromeGuillain-Barre Syndrome

December 7, 2009December 7, 2009

Lisa Rose-Jones, MDLisa Rose-Jones, MD

GBSGBS

Eponym that encompasses acute immune-Eponym that encompasses acute immune-mediated polyneuropathiesmediated polyneuropathies

Peripheral nerve myelin is target of an Peripheral nerve myelin is target of an immune attackimmune attack

Starts at level of nerve root=conduction Starts at level of nerve root=conduction blocks & muscle weaknessblocks & muscle weakness

Eventually get widespread patchy Eventually get widespread patchy demyel= increased paralysisdemyel= increased paralysis

PathophysiologyPathophysiology

Usually postinfectiousUsually postinfectiousImmune-mediated: infectious agents thought to Immune-mediated: infectious agents thought to induce Ab production against specific induce Ab production against specific gangliosides/glycolipidsgangliosides/glycolipidsLymphocytic infiltration of spinal roots/peripheral Lymphocytic infiltration of spinal roots/peripheral nerves & then macrophage-mediated, multifocal nerves & then macrophage-mediated, multifocal stripping of myelinstripping of myelinResult: defects in the propagation of electrical Result: defects in the propagation of electrical nerve impulses, with eventual conduction block nerve impulses, with eventual conduction block and flaccid paralysis and flaccid paralysis

Clinical Features:Clinical Features:

Progressive, fairly symmetric muscle Progressive, fairly symmetric muscle weaknessweakness

-typically starts in proximal legs-typically starts in proximal legs

-10% will 1-10% will 1stst develop weakness in face develop weakness in face or armsor arms

-severe resp muscle weakness in 10--severe resp muscle weakness in 10-30% pts30% pts

-oropharyngeal weakness in ~ 50%-oropharyngeal weakness in ~ 50%

Clinical Features:Clinical Features:

Absent or depressed DTRAbsent or depressed DTR

Often prominent severe pain in lower backOften prominent severe pain in lower back

Common to have paresthesias in hands Common to have paresthesias in hands and feetand feet

Dysautonomia is very common: Dysautonomia is very common: tachycardia, urinary retention, tachycardia, urinary retention, hypertenison alternating w/ hypotension, hypertenison alternating w/ hypotension, ileusileus

Diagnosis:Diagnosis:

Albuminocytologic dissociation: elevated Albuminocytologic dissociation: elevated CSF protein w/ normal WBC (80-90% pts)CSF protein w/ normal WBC (80-90% pts)

Electromyography (EMG) helps confirm Electromyography (EMG) helps confirm diagnosis = prolonged or absent F wavesdiagnosis = prolonged or absent F waves

NINDS Expert Consensus:NINDS Expert Consensus:

Req’d Features for dx: Req’d Features for dx: 1.1. Progressive weakness of > than 1 limbProgressive weakness of > than 1 limb2.2. AreflexiaAreflexia

Supportive Features:Supportive Features:~progression of Sx over days to 4 weeks~progression of Sx over days to 4 weeks~relative symmetry~relative symmetry~CN involv esp b/l facial n weakness~CN involv esp b/l facial n weakness~autonomic dysfunction ~EMG features~autonomic dysfunction ~EMG features~elev CSF protein w/ cell count ,10 mm3~elev CSF protein w/ cell count ,10 mm3

GBS=heterogenous syndrome w/ GBS=heterogenous syndrome w/ variant formsvariant forms

Think of Think of AIDPAIDP as the traditional form as as the traditional form as described previously, accts for 85-90% described previously, accts for 85-90%

Miller Fisher SyndromeMiller Fisher Syndrome: opthalmoplegia, : opthalmoplegia, ataxia, and areflexia (5%). GQ1b antibody. ataxia, and areflexia (5%). GQ1b antibody. Only 1/4Only 1/4thth w/ extremity weakness w/ extremity weakness

AMANAMAN: selective involv of motor nerves, DTRs : selective involv of motor nerves, DTRs are preserved, more common in Japan/China, are preserved, more common in Japan/China, almost all preceded by Campylobacter infxnalmost all preceded by Campylobacter infxn

AMSANAMSAN: more severe form of AMAN +sensory: more severe form of AMAN +sensory

DDx of Polyneuropathy:DDx of Polyneuropathy:

Arsenic poisoningArsenic poisoningN-Hexane (glue sniffing)N-Hexane (glue sniffing)VasculitisVasculitisLyme DiseaseLyme DiseaseTick paralysisTick paralysisSarcoidosisSarcoidosisLeptomeningeal DzLeptomeningeal DzParaneoplastic DzParaneoplastic DzCritical IllnessCritical Illness

Supportive CareSupportive Care

Monitor Resp status closely (follow NIFs), Monitor Resp status closely (follow NIFs), up to 30% may req ventilatory support up to 30% may req ventilatory support

In severe cases, intrarterial monitoring In severe cases, intrarterial monitoring may be necessary given the gisngifcant may be necessary given the gisngifcant blood pressure fluctuationsblood pressure fluctuations

Neuropathic pain plagues most, often Neuropathic pain plagues most, often managed w/ Gabapentin or managed w/ Gabapentin or CarbamazepineCarbamazepine

Disease Modifying TreatmentDisease Modifying Treatment

IVIG : typically given for 5 d at 0.4 gram/kg/d IVIG : typically given for 5 d at 0.4 gram/kg/d (may need to extend course depending on (may need to extend course depending on response)response)Plasmapheresis: usually 4-6 treatments over 8-Plasmapheresis: usually 4-6 treatments over 8-10 days10 days

The choice b/w plasma exchange and IVIG is dep The choice b/w plasma exchange and IVIG is dep on availability, pt contraindications, etc. Because on availability, pt contraindications, etc. Because of ease of administration, IVIG is frequently of ease of administration, IVIG is frequently preferred. The cost and efficacy of the 2 preferred. The cost and efficacy of the 2 treatments are comparable.treatments are comparable.Glucocorticoids have NO ROLE!!Glucocorticoids have NO ROLE!!

Outcomes:Outcomes:

65% can walk independently @ 6 mos65% can walk independently @ 6 mosOverall, 80% usually recover completelyOverall, 80% usually recover completely5-10% have prolonged course w/ 5-10% have prolonged course w/ incomplete recovery, ~3% wheelchair incomplete recovery, ~3% wheelchair boundboundApprox 5% die despite ICU careApprox 5% die despite ICU care2% will develop chronic relapsing Chronic 2% will develop chronic relapsing Chronic Inflammatory Demyelinating Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)Polyradiculoneuropathy (CIDP)

REFERENCES:REFERENCES:

Uptodate 2009.Uptodate 2009.

Plasmapheresis and acute Guillain-Barre syndrome. The Guillain-Barre Plasmapheresis and acute Guillain-Barre syndrome. The Guillain-Barre Syndrome Study Group. Neurology 1984; 2:1296.Syndrome Study Group. Neurology 1984; 2:1296.

Ropper, AH. The Guillain-Barre Syndrome. N Engl J Med 1992; 326:1130.Ropper, AH. The Guillain-Barre Syndrome. N Engl J Med 1992; 326:1130.

Sumner, AJ. The physiologic basis for symptoms in Guillain-Barre Syndrome. Sumner, AJ. The physiologic basis for symptoms in Guillain-Barre Syndrome. Ann Neurol 1981; 9 Suppl:28.Ann Neurol 1981; 9 Suppl:28.