How GBS is tested on Step 1

A recent infection, then rapidly rising weakness without reflexes.

TriggerCampylobacter, CMV, EBV, Zika, influenza; rarelyvaccinesMimicryAntibodies and T cells cross-react with nervegangliosidesDemyelinationSchwann cell myelin damaged; conduction slows orblocksWeaknessAscending, symmetric, areflexic; peaks within about4 weeksRecoveryRemyelination over weeks to months; most recover
GBS course. Miller Fisher variant: ophthalmoplegia, ataxia, and areflexia with anti-GQ1b antibodies.

Diagnosis and treatment

Support the diagnosis, then protect breathing.

StepFinding or action
CSFHigh protein, normal white cell count (may be normal in the first week)
Nerve conduction studiesSlowed conduction, conduction block, or reduced amplitudes
MonitoringForced vital capacity and negative inspiratory force; cardiac telemetry
TreatmentIVIG or plasma exchange; supportive care and DVT prevention
Not helpfulCorticosteroids

A high CSF cell count suggests another cause, such as HIV or Lyme disease.

Upper vs lower motor neuron signs

These signs localize every weakness question.

Upper motor neuronCortex, corticospinal tractSpastic weaknessHyperreflexia, clonusBabinski sign presentLittle atrophyLower motor neuronAnterior horn, nerve root, nerveFlaccid weaknessHyporeflexia or areflexiaFasciculationsMarked atrophyvs
UMN vs LMN. GBS is a peripheral (LMN) disorder; MS and stroke are UMN disorders.

Spinal cord and motor neuron disorders

Which tracts are hit determines the findings.

DisorderLesionFeatures
Amyotrophic lateral sclerosisCorticospinal tract and anterior horn (UMN + LMN)Mixed signs, no sensory loss; SOD1 in some familial cases; riluzole
Spinal muscular atrophyAnterior horn (LMN); SMN1 geneFloppy infant, tongue fasciculations
PolioAnterior horn (LMN)Asymmetric flaccid paralysis after febrile illness
Subacute combined degenerationDorsal columns, corticospinal tractsVitamin B12 deficiency; ataxia, spasticity
Tabes dorsalisDorsal columnsTertiary syphilis; sensory ataxia, Romberg sign
SyringomyeliaCentral cord, anterior white commissureCape-like loss of pain and temperature
Brown-SéquardHemisectionIpsilateral weakness and vibration loss, contralateral pain loss

Friedreich ataxia (GAA repeat) combines spinocerebellar and dorsal column degeneration with cardiomyopathy.

Worked example

Read the CSF.

functions Worked example — weakness after diarrhea

Two weeks after a bout of bloody diarrhea, a 30-year-old has tingling feet and weakness that climbs from the legs to the arms over 4 days. Reflexes are absent. CSF protein is 180 mg/dL with 2 white cells/µL. What is the diagnosis, the likely trigger, and the priority?

  1. Ascending symmetric weakness with areflexia suggests acute peripheral nerve demyelination.
  2. High CSF protein with a normal cell count is albuminocytologic dissociation.
  3. Bloody diarrhea before onset suggests Campylobacter jejuni, which mimics nerve gangliosides.
  4. The priority is to monitor vital capacity and start IVIG or plasma exchange.

Answer. Guillain-Barré syndrome after Campylobacter jejuni; monitor breathing and give IVIG or plasma exchange.