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
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