Dr. Mooers treated us to the fascinating case of a female patient in her 70s who was ultimately diagnosed with Guillain-Barré syndrome.
What is Guillain-Barré syndrome (GBS)?
GBS is an autoimmune, inflammatory polyradiculoneuropathy: it is primarily demylinating. It can present as one of the following:
Rapid, acute onset of generalized weakness
Rapid progression of generalized weakness over several days
Rapid progression of generalized weakness over weeks
GBS occurs when some inciting event (classically, infection--most commonly, Campylobacter jejuni) activates the immune system, which then attacks myelin in peripheral nerves and spinal roots secondary to molecular mimicry.
About 50% of patients with GBS have antibodies against gangliosides, which are found in peripheral nerves. This entire cascade results in impaired nerve conduction.
Presentation
The classic presentation involves progressive, symmetric ASCENDING muscle weakness with diffuse areflexia. Paresthesias are seen in about 80% of patients but it is important to note that true sensory deficits are rare.
Other common symptoms include low back pain (often common in the initial phase, secondary to nerve root inflammation), dysautonomia (common and can result in life-threatening blood pressure changes and arrhythmias), facial nerve palsies and oropharyngeal weakness.
There are also variants of GBS which include:
Paraplegic variant-weakness limited to the legs
Pharyngeal-cervical-brachial variant- displays relative sparing of the legs
Miller Fisher variant-multiple cranial neuropathies and ataxia
Diagnosis
GBS is a clinical diagnosis but other studies should be done to rule out other diagnoses and suggest GBS:
CSF analysis- can reveal a classic pattern of a highly elevated protein level with a normal or mildly elevated leukocyte count (albuminocytologic dissociation) in 60% of patients
EMG- This confirms the diagnosis when it shows a predominantly demyelinating pattern but results may initially be normal.
MRI of the lumbar spine- Not mandatory but may reveal evidence of spinal root enhancement.
Management
Management focuses on supportive care: patients should be closely monitored for respiratory failure with serial negative inspiratory force (NIFs) measurements and for dysautonomia with monitoring of blood pressures and heart rates. Prognosis is good in 80% of patients. However, relapse occurs in 6% and requires repeated treatment.
Additional treatments (plasmapheresis or intravenous immune globulin) may be trialed in patients who experience severe progressive symptoms. plasmapheresis or intravenous immune globulin. Both are equally effective and shorten the time to recovery by as much as 50%. Plasmapharesis has a faster onset of action but is less convenient and potentially requires longer hospitalization.
Steroids are not beneficial in GBS and may even slow the recovery.
**Make sure to check on D2L for the whole powerpoint including a real cool review of diaphragmatic dysfunction!**
References:
Fokke C, Van den Berg B, Drenthen J, et al. Diagnosis of Guillain-Barre syndrome and validation of Brighton criteria. Brain 2014:137;33-43.
McCool D, Tzelepis G. Dysfunction of the Diaphragm. N Engl J Med 2012;366:932-942.
McCool D, Manzoor K, Minami T. Disorder of the Diaphragm. Clin Chest Med 2018;39:345–360
Minami T, Manzoor K, McCool D. Assessing Diaphragm Function in Chest Wall and Neuromuscular Diseases. Clin Chest Med 2018;38:335-344.
Billings M, Aitken M, Benditt J. Bilateral Diaphragm Paralysis: A Challenging Diagnosis. Resp Care 2008;53:1368-1371.
Ricoy J, Rodriguez-Nunez N, Alvarez-Dobano, et al. Diaphragm dysfunction. Pulmonol 2019;25(4):223-235
Yuki N, Hartung H. Guillain–Barré syndrome. N Engl J Med. 2012;366(24):2294-2304.
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