How Should Guillain-Barré Syndrome Be Managed in the ICU?

Guillain-Barré syndrome (GBS) is a rapidly progressive motor, sensory, and autonomic neuropathic disorder that may present to critical care with acute respiratory failure or bulbar palsy. Correct diagnosis and implementation of therapy usually results in favorable outcomes. This chapter considers management of GBS in the intensive care unit (ICU).

Pathogenesis

GBS is a prototype of a postinfectious autoimmune disease. Most patients develop GBS 1 to 3 weeks after a microbial infection. Histopathologically, GBS can be divided into acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy based on the site of involvement of the inflammatory process within the peripheral nerve ( Fig. 66-1 ). Infections such as Campylobacter jejuni or cytomegalovirus induce the development of antibodies that subsequently bind to target antigens on the peripheral nerves as a result of molecular mimicry. These autoantibodies attach to the outer surface of Schwann cells or axonal membranes at the nodes of Ranvier, resulting in activation of the compliment system. This subsequently leads to the detachment of the paranodal myelin, resulting in motor conduction failure and muscle weakness.

Diagnosis

The presentation of GBS and related conditions can be heterogeneous, making the clinical diagnosis at times challenging. Classically, GBS presents with a rapidly progressive weakness of the extremities with variable involvement of the bulbar and respiratory muscles. There are localized subtypes of GBS that tend to involve only a specific group of muscles ( Table 66-1 ). These include (1) the pharyngeal-cervical-brachial subtype with involvement of the bulbar and proximal upper limb muscles; (2) the paraparetic subtype; and (3) the bifacial variant, which presents with isolated facial weakness. Miller Fisher syndrome (MFS), which presents with ophthalmoplegia, ataxia, and areflexia, is a variant of GBS. MFS may present in incomplete form as acute ophthalmoplegia or acute ataxic neuropathy. Bickerstaff brainstem encephalitis, which presents with hypersomnolence, ophthalmoplegia, and ataxia, is a central nervous system subtype of MFS. Some of these patients go on to have involvement of other groups of muscles and thus phenotypically resemble the classical type of GBS. Pharyngeal-cervical-brachial weakness, MFS, and Bickerstaff encephalitis are often misdiagnosed as having brainstem stroke, myasthenia gravis, botulism, or Wernicke encephalopathy ( Table 66-2 ).

Table 66-1

Clinical Features of Guillain-Barré Syndrome, Miller Fisher Syndrome, and Their Subtypes

	Pattern of Weakness	Ataxia	Hypersomnolence
Guillain-Barré syndrome	Four limbs	Yes
• Pharyngeal-cervical-brachial weakness	Bulbar/cervical/upper limbs
Incomplete form
• Acute pharyngeal weakness	Bulbar
• Bifacial weakness with paraesthesias	Facial
• Paraparetic Guillain-Barré syndrome	Lower limbs
Miller Fisher syndrome	Ophthalmoplegia	Yes
Incomplete forms
• Acute ophthalmoparesis	Ophthalmoplegia
• Acute ataxic neuropathy	No weakness	Yes
• Acute ptosis	Ptosis
• Acute mydriasis	Paralytic mydriasis
Central nervous system subtype
• Bickerstaff brainstem encephalitis	Ophthalmoplegia		Yes
Incomplete form
• Acute ataxic hypersomnolence	No weakness		Yes

Table 66-2

Differential Diagnosis of Guillain-Barré Syndrome, Miller Fisher Syndrome, and Their Subtypes

Guillain-Barré Syndrome

•

Acute spinal cord disease
•

Carcinomatous or lymphomatous meningitis
•

Myasthenia gravis
•

Critical illness neuropathy
•

Thiamine deficiency
•

Corticosteroid-induced myopathy
•

Toxins (e.g., neurotoxic shellfish poisoning)
•

Acute hypophosphatemia
•

Prolonged use of neuromuscular blocking drugs
•

Tick paralysis
•

West Nile poliomyelitis
•

Acute intermittent porphyria

Miller Fisher Syndrome, Bickerstaff Brainstem Encephalitis, and Pharyngeal-Cervical-Brachial Weakness

•

Basilar artery occlusion
•

Myasthenia gravis
•

Wernicke encephalopathy
•

Botulism
•

Brainstem encephalitis
•

Diphtheria
•

Tick paralysis

Paraparetic Variant

•

Lumbosacral plexopathy
- •
  
  Diabetic
- •
  
  Neoplastic
- •
  
  Inflammatory (e.g., sarcoidosis)
- •
  
  Infective (e.g., cytomegalovirus, Lyme disease)
•

Lesions of cauda equine

Bifacial weakness with paraesthesias

•

Lyme disease
•

Sarcoidosis

Nerve conduction studies and cerebrospinal fluid (CSF) analysis are not always conclusive, especially at admission, and the diagnosis should be based on clinical grounds ( Table 66-3 ). A lumbar puncture is performed primarily to rule out infectious processes, such as Lyme disease, or malignancies, such as lymphoma. Albuminocytologic dissociation (elevation in CSF protein [>0.55 g/L] without an elevation in white blood cells) is present in approximately 50% of patients with GBS during the first week of illness. Brain and spinal imaging studies are unhelpful. A history of antecedent infectious symptoms such as sore throat, cough, or diarrhea is useful for the clinical diagnosis as well as the presence of distal paraesthesias immediately before, and at the onset of, weakness or ataxia.

Table 66-3

Diagnostic Criteria for Guillain-Barré Syndrome, Miller Fisher Syndrome and Their Subtypes

Core Clinical Features	Supportive Features
• Relatively symmetric pattern of limb and/or motor cranial nerve weakness ^a^,^b • Monophasic illness pattern and interval between onset and nadir of weakness between 12 hours and 28 days and subsequent plateau • Absence of identifies alternative diagnosis	• Antecedent infectious symptoms ^c • Presence of distal paraesthesias before or at the onset of weakness • Cerebrospinal fluid albuminocytologic dissociation ^d
1. Guillain-Barré syndrome
• Weakness in all four limbs ^a^,^e^,^fand areflexia/hyporeflexia ^g	• Neurophysiologic evidence of neuropathy
1.1 Pharyngeal-cervical-brachial weakness
• Oropharyngeal weakness and neck weakness and arm weakness and arm areflexia/hyporeflexia ^a^,^b^,^h • Absence of leg weakness and ataxia ⁱ^,^j	• Neurophysiologic evidence of neuropathy • Presence of anti-GT 1a or anti-GQ1b
1.2 Paraparetic Guillain-Barré syndrome
• Leg weakness and areflexia/hyporeflexia ^a • Absence of arm weakness	• Neurophysiologic evidence of neuropathy
1.3 Bifacial weakness with paraesthesias
• Facial weakness and areflexia/hyporeflexia ^a • Absence of ophthalmoplegia and limb weakness
2. Miller Fisher syndrome
• Ophthalmoparesis and ataxia and areflexia/hyporeflexia ^a^,^b^,^k^,^l • Absence of limb weakness ^mand hypersomnolence	• Presence of anti-GQ1b antibodies
2.1 Bickerstaff brainstem encephalitis
• Hypersomnolence and ophthalmoparesis and ataxia ^a^,^b^,ⁿ • Absence of limb weakness ^m	• Presence of anti-GQ1b antibodies