See also: [[Weakness]] See: [Harrison’s - GBS](x-devonthink-item://D7D7DEE3-8F19-43B8-9F8B-9722C906DAB6?page=3541) - acute, acquired, inflammatory *demyelinating polyradiculoneuropathy* (**AIDP**) caused by autoimmune attack on peripheral nerves/nerve roots - most common cause of acute progressive generalised weakness in the ED - often precedes diarrhoea illness, especially *Campylobacter jejuni*. CMV is second most common infection associated with GBS. EBV, mycoplasma pneumoniae, [[HIV]], and h flu also associated # clinical features - progressive ascending weakness with loss of deep tendon reflexes - proximal and distal limb muscles are involved, as well as truncal and respiratory muscles - cranial nerve involvement is common, with facial nerve palsy occuring in up to 70% of cases - sensory symptoms are variable; paraesthesias and severe pain in some patients - autonomic dysregulation occurs in some patients, can be fatal due to severe fluctuations in BP and cardiac arrhythmias # investigations - [[Lumbar puncture#GBS|Lumbar puncture]]: CSF findings of ==high CSF protein and normal glucose and cell count.== (albuminocytologic dissociation) - mild CS pleuocytosis is common, however presence of CSF leucocytosis should promot careful consideration of alternative dxs such as lymphoma or HIV ## Harrison’s notes - ↑ CSF protein level (1–10 g/L \[100–1000 mg/dL]) without accompanying pleocytosis. - The CSF is often normal when symptoms have been present for ≤48 h; by the end of the first week, the level of protein is usually elevated. - A transient increase in the CSF white cell count (10–100/μL) occurs on occasion in otherwise typical GBS; however, a sustained CSF pleocytosis suggests an alternative diagnosis (viral myelitis) or a concurrent diagnosis such as unrecognised HIV infection, leukemia or lymphoma with infiltration of nerves, or neurosarcoidosis. - EDx (electrodiagnostic) features are mild or absent in the early stages of GBS and lag behind the clinical evolution. In AIDP, the earliest features are prolonged F-wave latencies, prolonged distal latencies, and reduced amplitudes of compound muscle action potentials (CMAPs), probably owing to the predilection for involvement of nerve roots and distal motor nerve terminals early in the course. Later, slowing of conduction velocity, conduction block, and temporal dispersion may be appreciated > Laboratory tests are helpful primarily to exclude mimics of GBS. CSF pleocytosis is seen with poliomyelitis, acute flaccid myelitis, and Lyme and CMV polyradiculitis. EDx features may be minimal early in GBS, and the CSF protein level may not rise until the end of the first week. If the diagnosis is strongly suspected, treatment should be initiated without waiting for evolution of the characteristic EDx and CSF findings to occur. GBS patients with risk factors for HIV or with CSF pleocytosis should have a serologic test for HIV. # complications - respiratory failure if phrenic nerve involvement - [[FVC]] of 10-12 mL/kg (<30% of predicted) is generally considered to be an indication for intubation and assisted ventilation (also for myasthenia gravis; normal FVC is 60-70) - paCO2 > 50 also may indicated intubation - do NOT use sux - NIV is not recommended for GBS, especially if there is significant bulbar weakness # treatment - [[IVIG]] - 2g/kg - plasmapheresis maybe - do NOT use steroids