top of page

THE MORNING REPORT

The Best in FOAM Education

  • Alexon Munson-Catt, MD

Guillain Barre Syndrome

Case presentation

26-year-old female, history of hypothyroidism, multiple psychiatric conditions presents with weakness. 3 weeks prior, patient was admitted for UTI and gastroenteritis, and discharged with flagyl and levoquine. Approximately 2weeks ago, patient began to feel weakness around knees and decreased sensation. She has fallen multiple times secondary to weakness and trouble with coordination. Of note, she started to develop decreased sensation of lower extremities around the same time, and now complains of decreased sensation to finger tips.


Vitals: 36.8; 107; 104/71; 16; 97% RA

  • General: Alert, oriented, no acute distress, anxious demeanor​

  • Cardiac: tachycardic, normal S1/S2, no m/r/g, warm extremities, peripheral pulses 2+ throughout​

  • Pulmonary: clear to auscultation bilaterally​

  • Neuro: CN 2-12 intact, 5/5 strength all myotomes, decreased sensation to light touch in bilateral L3-S1, 0+ reflexes in all DTRs except for LUE which was 2+, intact bilateral finger to nose and heel to shin, + romberg, unsteady gait

Differential Diagnosis

  • Guillian Barre Syndrome​

  • Hypokalemic periodic paralysis​

  • Transverse myelitis​

  • Lyme paralysis​

  • Multiple sclerosis​

  • Subacute Combined Degeneration​

Guillain Barre Syndrome

  • Variants

    • Acute Inflammatory Demyelinating Polyneuropathy​

    • Miller Fisher Syndrome​

    • Acute motor axonal neuropathy​

    • Acute motor and sensory axonal neuropathy​

    • Pharyngeal-Cervical-Brachial variant​

    • Bickerstaff brainstem encephalitis​

    • Facial diplegia with paresthesia variant

Acute Inflammatory Demyelinating Polyneuropathy

  • Epidemiology: most common cause of GBS in North America and Europe (~90%)​

  • Pathophysiology: diffuse inflammatory demyelination of nerves​

  • Clinical presentation: classic GBS manifestation​

  • Diagnosis: Clinical, Lumbar tap

  • Treatment: IVIG, plasmapheresis​

  • Prognosis: full recovery over several weeks to months

Miller Fisher Syndrome

  • Epidemiology: Second most common cause of GBS in the United States (~10%)​

  • Pathophysiology: Anti-GQ1b antibodies​

  • Clinical presentation: classic triad: areflexia, external ophthalmoplegia, cerebellar ataxia. Extremity weakness may occur. Usually no development of respiratory failure​

  • Diagnosis: lumbar tap, albuminocytologic dissociation​

  • Treatment: IVIG, plasmapheresis

GBS epidemiology

  • Most common cause of generalized neuromuscular paralysis​

  • Causes: infection is involved in 75% cases (campylobacter jejuni, mycoplasma pneumonia, hemophilus influenzae, CMV, EBV, VZV, HAV, HEV, HIV, Zika virus, influenza A, Covid-19, checkpoint inhibitors, immunizations

GBS presentation

  • Preceding trigger approximately 5-28 days​

  • Sensory disturbances: often first symptom. paresthesias especially in fingers and toes​

  • Ascending paralysis: symmetric. Speed of progression correlates with disease severity. 25% of patients develop respiratory failure​

  • Cranial nerve involvement is common​

  • Dysautonomia: BP lability, sympathetic and or parasympathetic activation, constipation/diarrhea, urinary retention​

  • Physical findings: weakness, loss of DTRs (~90%)

GBS disease course progression

  • Typically worsens over ~2 weeks​

  • 80% of patients nadir by 2 weeks​

  • 90% of patients nadir by 4 weeks​

  • Typically monophasic disease progression

Diagnosis

  • CSF examination: albuminocytologic dissociation (typically elevated 100-1000). Sensitivity of ~50% by week 1 and ~80% by week 2​

  • MRI: Contrast enhancement of spinal nerve roots are sensitive but not specific​

  • EMG: most sensitive and specific test. May be normal initially​

  • Ganglioside serology: long turnaround time and many antibodies that we never learned about

Diagnostic Criteria

  • Required features include:​

    • Progressive weakness of the arms and/or legs, ranging from minimal weakness of the legs to total paralysis of all four limbs, and including the trunk, bulbar and facial muscles, and external ophthalmoplegia.​

    • Areflexia or decreased deep tendon reflexes in weak limbs.​

  • Supportive features include:​

    • Symptom progression over days to four weeks​

    • Relatively symmetric, bilateral symptoms​

    • Pain in the trunk or limbs​

    • Cranial nerve symptoms or signs​

    • Autonomic dysfunction​

    • Sensory dysfunction that is mild​

    • No fever at symptom onset​

    • CSF with elevated protein and normal to mildly elevated leukocyte count (usually <5 cells/mm3)​

    • Electrodiagnostic abnormalities consistent with GBS​

    • Recovery starting two to four weeks after progression halts

GBS treatment

IVIG vs Plasmapheresis​

  • Begin treatment based off of clinical diagnosis. No need to wait for confirmatory studies​

  • IVIG and Plasmapheresis seem to be equally effective​

  • Combination therapy appear to be equivalent to monotherapy​

  • IVIG – safer and easier to perform. 0.4g/kg/day​

  • Plasmapheresis – typically reserved for patients with IVIG contraindications

Dysautonomia management​

  • May have fluctuating sympathetic / parasympathetic hyperactivity​

  • Fluctuations are short lived so avoid aggressive hemodynamic treatment​

  • ** avoid beta blockers (may increase risk of bradycardia)​

  • Hypertension – avoid treatment unless end organ damage or persistent severe hypertension . First line treatment: remove stimulus. Second line treatment: short acting agents (nicardipine / clevidipine infusions)​

  • Hypotension – fluid administration, low dose vasopressors​

  • Bradycardia – brady cardia ACLS treatment​

  • Urinary retention - foley

Pulmonary Function Tests

  • Forced Vital Capacity (FVC)​

    • Normal ~60cc/kg​

    • Value below 30cc/kg suggests risk of atelectasis or hypoventilation​

  • Negative Inspiratory Force (NIF)​

    • Measures strength of inspiratory muscles​

    • Does NOT add statistically independent / useful information beyond measurement of FCV​

  • Utility​

    • Triaging for admission: ICU vs. Floors​

    • Tracking trajectory

Intubation

  • Criteria​

    • Same as other patients​

    • Use ventilatory and oxygenation status, disease progression trajectory, evidence of respiratory fatigue, FVC​

  • Patients are prone to extreme vagal reactions​

  • AVOID succinylcholine

References

Farkas, J. EMCrit A. Guillain Barre Syndrome (GBS) - EMCRIT Project. EMCrit Project. https://emcrit.org/ibcc/gbs/. Published March 3, 2023.


Alexon Munson-Catt, MD

Comments


bottom of page