Updated: 9/10/2019

Guillain-Barre Syndrome

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Snapshot
  • A 35-year-old man presents to the emergency department for difficulty with walking. His symptoms began approximately 1 week ago and has progressively worsened. He has noticed some lightheadedness with standing up from a seated position and some numbness in the bilateral lower extremities. Physical examination is notable for 4/5 power and decreased sensation to light touch and pinprick in the bilateral lower extremity and absent patellar and ankle reflexes. A lumbar puncture is performed, which demonstrates a cell count of 1/uL, protein is 135 mg/dL, and glucose is 65 mg/dL. He is admitted to the neurology floor for frequent negative inspiratory pressure and vital capacity measures and intravenous immunoglobulin. 
Introduction
  • Definition
    • an immune-mediated polyneuropathy that has many variant forms
      • acute inflammatory demyelinating polyneuropathy
      • chronic inflammatory demyelinating polyneuropathy
      • Miller Fisher syndrome
        • ophthalmoplegia
        • ataxia
        • areflexia
  • Epidemiology
    • risk factors
      • recent infection
        • Campylobacter jejuni 
          • most commonly identified
        • Mycoplasma pneumoniae 
        • HIV
        • Ebstein-Barr virus
        • Zika virus
  • Pathogenesis
    • a previous infection triggers an immune response that cross-reacts with parts of the peripheral nerve via molecular mimicry
      • damages the Schwann cells
  • Prognosis
    • improved outcomes with either plasmapheresis or intravenous immunoglobulin
Presentation
  • Symptoms/physical exam
    • classic presentation
      • typically ascending (and fairly symmetric) muscle weakness along depressed or absent reflexes
    • other findings may include
      • autonomic dysfunction
      • peripheral cranial nerve palsies
        • cranial nerve III-XII
    • Miller Fisher variant
      • ophthalmoplegia
      • ataxia
      • areflexia
Imaging
  • MRI
    • indication
      • typically used to rule out other causes of weakness 
    • findings
      • in acute cases, may demonstrate enhancement and thickening of the
        • intrathecal spinal nerve roots
        • cauda equina
Studies
  • Spirometry
    • to ensure adequate respiratory function
    • intubate early if necessary
  • Lumbar puncture
    • albuminocytologic dissociation 
      • elevated protein
      • normal white blood cell count
  • Nerve conduction studies 
    • slowed conduction velocity
    • reduced amplitude in compound muscle action potentials
Differential
  • Acute myelopathy
    • differentiating factors
      • spinal cord lesion on MRI
Treatment
  • Conservative
    • acute supportive care
      • indication
        • to acutely manage complications of Guillain-Barre syndrome, such as
          • respiratory failure
            • managed with intubation
          • hypotension
            • managed with fluids or phenylephrine
          • atrioventricular blocks and asystole
            • managed with atropine or cardiac pacing
  • Medical
    • plasmapheresis 
      • indication
        •  hastens neurological recovery
    • intravenous immunoglobulin (IVIG)
      • indication
        • hastens neurological recovery
    • note that plasmapheresis and IVIG are equally effective
Complications
  • Respiratory failure
  • Autonomic instability
  • Complications from IVIG and plasmapheresis

 

35 year old banker presents to the ED after hitting his head when he tripped while climbing the subway stairs. He has noted his legs feeling heavy over the last 5 days and reported trouble pushing himself up afterthe fall. Rnausea, vomiting, and diarrhea 3 days ago (admits to eating discount sushi). Exam is remarkable for symetric 3/5 lower and upper extremity weakness, absent ankle and patellar reflexes and 1+ biceps reflex. He is only able to count to 10 in one breath. Routine labs, chest x-ray, and head CT are unrevealing.
 

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Questions (3)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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