Critical Care Medicine-Neurologic Disorders>>>>>Neuromuscular Disorders
Question 3#

A 56-year-old man with essential thrombocytosis (baseline approximately 800 000) and prior renal cell carcinoma status post left nephrectomy 5 years before presentation presents with progressive bilateral lower extremity weakness and ascending paresthesia over the course of 7 days. He has mild proximal weakness in his bilateral legs, but is able to ambulate, and decreased sensation in his bilateral legs to mid-shin. No reflexes noted on examination. Additional testing including a lumbar puncture, demonstrating elevated protein with no white blood cells, and nerve conduction study, demonstrating dispersion of motor and sensory nerve responses with conduction block, confirms the diagnosis of GBS.

What is the most appropriate treatment?

a. Intravenous immunoglobulin (IVIg) at 2 g/kg in divided doses of 0.4 g/kg/d for 5 days
b. Plasmapheresis (PLEX) exchange volume 250 mL/kg for five sessions on alternating days
c. Intravenous methylprednisolone at 1 g/day for 5 days
d. PLEX exchange volume 250 mL/kg for five sessions on alternating days followed by IVIg at 2 g/kg in divided doses of 0.4 g/kg/d for 5 days

Correct Answer is B

Comment:

Correct Answer: B

There are several types of GBS, classified by the part of the peripheral nerve involved in this disease. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common etiology, but there is also an axonal type, which typically results in worse recovery. This patient presents with the stereotypical presentation of GBS: ascending paresthesias, progressive ascending weakness, and loss of deep tendon reflexes. Confirmatory testing includes lumbar puncture, which demonstrates a cytoalbuminologic gap and nerve conduction study suggestive of demyelinating features. Although not presented in the case, there are a number of disease-related complications including labile blood pressure, arrhythmias, gastroparesis, urinary retention, and syndrome of inappropriate antidiuretic hormone (SIADH). Treatment of GBS has two treatments: IVIg and PLEX. The American Academy of Neurology recommendations for treatment of GBS in ambulatory patients within 2 weeks of neuropathic symptoms advocates for PLEX. Another reason for use of PLEX over IVIg in this patient is the complications of treatment. IVIg can results in thrombosis because of hyperviscosity. Given his underlying thrombocytosis, IVIg could place him at even higher risk worsening thrombocytosis and in turn increase the risk of bleeding especially with platelet counts over 1 000 000. Studies showed that sequential treatment with PLEX followed by IVIg does not have a greater benefit than either treatment given alone. In addition, AAN guideline does not recommend steroids for the treatment of GBS patients (Level A, Class 1). 

References:

  1. Yuki N, Hartung HP. “Guillain-Barre syndrome.” N Engl J Med. 2012;366:2294-2304.
  2. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Lancet. 1997;349:225-230.