Bored and... MOONWALK: Guillain Barre Syndrome

mj2 mj1

It’s a beautiful night in the ED in 1986. Bad fashion is in, EM docs are still cowboys who heal the world, and yours truly is about to be born. Most importantly, Michael Jackson is performing in your city tonight and while you are disappointed that you are working instead of dancing with the King of Pop, you don’t know that propofol will be his dangerous downfall in 2009, so you are still happy. Then–like a thriller!–MJ is in a stretcher in your ED waiting to be seen! He tells you he’s been having trouble with his footwork for the last few days – his legs feel weak and he has less control over them. He also noted some tingling feelings in his hands and feet. He tried to moonwalk during his show tonight and almost fell backwards! Otherwise, he’s generally been feeling well since recovering from a mild flu 2 weeks ago.

You are concerned about...?

Guillian Barre syndrome!

What exam findings are typical?

–Motor weakness, starting from the feet and ascending; should be in more than one limb.

–Reflexes decreased or absent

-May have mild sensory symptoms like hand and feet parasthesias, but motor weakness > sensory symptoms

How do you make the diagnosis?

History of preceding viral illness or GI infection with Campylobacter jejuni
Exam suggestive of progressive weakness of more than one limb and areflexia
CSF with high protein, WBC < 10, normal glucose.

What's your favorite MJ song?

Mine is Billie Jean.

mj3

What is the most important thing to frequently assess? How do you do this?

–Respiratory function: do this by measuring vital capacity (anything low should raise suspicion), or as a cowboy approach, ask the patient to take one breath then count out loud as far as they can to 25, keeping track of any changes.

–Indications for intubation: any difficulty breathing, difficulty speaking or swallowing, PaO2 < 70, decreasing trend in work of breathing/NIF/vital capacity; vital capacity < 15 should definitely be intubated and < 20 should be monitored in the ICU.

-Also watch out for autonomic dysfunction which common in GBS

Treatment? Disposition?

-Consult neurology and admit, usually to ICU. Even if ventilating adequately, decompensation may occur, and patient should be in a monitored setting.

-Treatment is IVIG and plasmapheresis

-Mortality is 5%; most people recover but it may take months of rehabilitation

References

Andrus P, Jagoda A. Chapter 166. Acute Peripheral Neurologic Lesions. In:Tintinalli JE, Stapczynski J, Ma O, Cline DM, Cydulka RK, Meckler GD, T. eds.Tintinalli’s Emergency Medicine: A Comprehensive Study Guide, 7e. New York, NY: McGraw-Hill; 2011.

Josh Farkus. Blog post, “Five pearls for the dyspneic patient with guillian barre syndrome or myasthenia gravis.” PulmCrit Blog. Feb 22, 2015