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Guillain-Barre syndrome (GBS): The areflexic ascending symmetric neuromuscular disease

Guillain-Barre syndrome is an autoimmune disorder of the myelin sheath characterized by an acute/subacute onset of progressive peripheral polyneuropathy, symmetrical distribution, ascending pattern of loss of power and areflexia. GBS has a 5% mortality risk, with one-third of patients requiring endotracheal intubation and ICU admission. Even non-ventilated patient can have prolonged admissions of up to 5 weeks. Recovery is also slow, with 50-95% of patients taking a year to return to baseline functional status.

Areflexia and ascending symmetrical weakness are the hallmarks of GBS

The loss of deep tendon reflexes in the patient with acute loss of symmetric…

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  Oh Me Nerves! A Neurology Medley

Our job in the ED is to rule out brain stem CVA (acute onset) or acute spinal cord compression (MRI spine). The plantar reflexes in patient with GBS should be down going unless prior history of CVA/UMN disorder).

 Guillain-Barré Syndrome – Third time’s the charm

Diagnosis of early acute GBS is often missed due to the failure to recognize key features essential in the diagnosis of the disease. This is often due to the minimizing the paresthesia, discounting the existence of the complaint, not being vigilant to check deep tendon reflexes, or misinterpreting the complaints of leg, back, or sciatic pain.

 Guillain–Barré syndrome after SARS-CoV-2 vaccination in a patient with previous vaccine-associated Guillain–Barré syndrome

GBS should be considered in the differential diagnosis of a patient who presents with symmetric, ascending muscle weakness, with sensory deficits, after vaccination against influenza and SARS-CoV-2.

 Tasty Morsels of Critical Care 031 | Guillain-Barre Syndrome Part 2

The question at this stage remains as to which expensive treatment you should choose. There does not seem to be an advantage of one over the other so I think I would go for the one that does not need a 12F catheter in my neck. Turning to supportive treatment there are a few important principles at play. Firstly is the question of when do we intubate these folk. This can be split into two parts

 Those Who Cannot Do...Might Have Guillain-Barre Syndrome

The mainstay of treatment is intravenous immunoglobulin (IVIG) which has mostly supplanted plasma exchange. IVIG is convenient to give, widely available and has few side effects. IVIG is most effective when started within two weeks of symptom onset.

Articles of Interest

Guillain-Barre's less evil twin - CIDP!

CIDP, or chronic inflammatory demyelinating polyradiculoneuropathy, is an immune-mediated polyneuropathy which presents similarly to Guillain-Barré Syndrome (GBS). However, it is not as dangerous as GBS.

An Approach to Guillain-Barre Syndrome

2/3 of patients experience an infection in the month before onset (30% Campylobacter jejuni). Autoimmune destruction of Schwann cells causing demyelination of peripheral nerves and motor fibers.

Atypical Guillain-Barré in the Emergency Department

Guillain Barré Syndrome (GBS), although an uncommon diagnosis in the emergency department (ED), usually presents as one of the more common chief complaints—weakness. In this report we present an unusual case of weakness, initially seen in the ED and sent home only to return with worsening symptoms and ultimately found to be GBS.

What Emergency Physicians Should Know About Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) is a rare and potentially catastrophic paralyzing disorder of the peripheral nerves.1 It is also known as Landry’s ascending paralysis, postinfectious polyneuropathy, and acute inflammatory demyelinating polyneuropathy.

Resources

Core EM

Acute, immune-mediated demyelinating polyneuropathy characterized by an ascending flaccid paralysis and most often preceded by infection.

EmCrit

Cell count is generally normal in GBS (<5 cells/uL), or at the most mildly elevated (<50 cells/uL). Elevated cell count >50 or increased neutrophils in CSF suggest an alternative diagnosis (e.g., HIV, Lyme, leptomeningeal carcinomatosis, or sarcoidosis).

Life in the Fastlane

Guillain-Barré Syndrome (GBS) is the most common and most severe acute paralytic neuropathy, consisting of multiple variants with distinct clinical and pathological features.

Maimonides Emergency Medicine

Acute monophasic paralyzing illness approx 88% provoked by a preceding infection. 50% have facial palsies or oropharyngeal weakness.