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Neuromuscular

Guillain–Barré Syndrome

Diagnosis, variants, and when to treat

Guillain–Barré syndrome (GBS) is an acute, ascending, areflexic weakness. Respiratory and autonomic failure are the main threats.

1.Presentation

Ascending, symmetric weakness; areflexia. Paresthesias. Preceding infection 1–4 weeks (e.g. Campylobacter, CMV). Miller Fisher variant.[+]

2.Diagnosis

CSF: albuminocytologic dissociation. NCS: demyelinating or axonal. LP and EMG support; diagnosis often clinical when classic.[+]

3.Respiratory and Autonomic

FVC and NIF (MIP) q4–6h. Intubate when FVC <20 ml/kg or NIF >−30 (or rapid fall). Autonomic: labile BP, arrhythmia, ileus, retention.[+]

4.Treatment

IVIG 0.4 g/kg/day × 5 days or plasmapheresis (5 exchanges). Start within 2–4 weeks. Equally effective; IVIG often logistically easier.[+]

Steroids are not first-line. No proven benefit as primary treatment.[+]

Admit for monitoring. Weakness can progress over days. Respiratory and autonomic failure can develop quickly.

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Frequently asked questions

What CSF findings support a diagnosis of Guillain-Barré syndrome?

The classic CSF finding in GBS is albumino-cytologic dissociation — elevated protein (typically >45 mg/dL, often 100–1000 mg/dL) with a normal or only minimally elevated white cell count (<10 cells/µL, predominantly mononuclear). This pattern develops over the first week of illness: CSF protein is normal in approximately 50% of patients within the first 3 days, rises in 75% by the end of week 1, and exceeds 80–90% by week 2. A normal CSF protein early in the course does not exclude GBS. WBC >50 cells/µL should prompt evaluation for alternative diagnoses including HIV seroconversion, Lyme, sarcoidosis, or carcinomatous meningitis.

When should IVIG versus plasmapheresis be used in Guillain-Barré syndrome?

IVIG and plasma exchange are equally effective for GBS — neither is superior on disability at 4 weeks (Hughes Cochrane review). IVIG 0.4 g/kg/day for 5 days is the most common first-line treatment because it is more widely available, easier to administer, and does not require central venous access. Plasma exchange (5 sessions over 1–2 weeks, total exchange volume ~250 mL/kg) is preferred when IVIG is contraindicated (IgA deficiency, severe renal failure, hypercoagulable state) or when cardiac instability makes the volume load of IVIG hazardous. Combining IVIG with plasmapheresis offers no additional benefit and is not recommended. Treatment should begin within 2–4 weeks of symptom onset; benefit is greatest when started in patients still able to walk independently.

What respiratory monitoring is required in Guillain-Barré syndrome?

Bedside pulmonary mechanics should be checked every 4 hours during the progressive phase. Intubate when any of the 20/30/40 criteria are met: forced vital capacity <20 mL/kg, maximum inspiratory pressure (NIF) less negative than -30 cmH2O, or maximum expiratory pressure <40 cmH2O. Other indications for intubation include rapid decline (>30% drop in FVC in 24 hours), bulbar dysfunction with aspiration risk, or autonomic instability with cardiovascular collapse. Elective intubation in a controlled setting is strongly preferred over emergent intubation after respiratory failure. Approximately 20–30% of GBS patients require mechanical ventilation; the Erasmus GBS Respiratory Insufficiency Score (EGRIS) can help risk-stratify at admission.

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