A 19 year old female with a past medical history of anxiety, depression, bulimia (resolved 5 years ago) presents with weakness. She states that her weakness began two days ago, starting with her feet and slowly ascending up her legs. Initially she noticed tingling in her feet in the morning followed by difficulty grasping a pen in class. It progressed to hand numbness, prompting the patient to seek care. She is accompanied by her mother who states that there has been a change in the patient’s voice and that she has been having difficulty walking. She had an upper respiratory infection 3 weeks ago treated with a Z-pack. She denies recent travel, hiking, tick bites, rash, vision changes, and dysphagia.
Afebrile with vital signs stable
Fluent speech with hoarse voice
CN2-12 intact. Able to sustain upward gaze. Bilateral dysmetria with finger to nose testing present
4/5 upper extremity strength, 3/5 lower extremity strength
Absent biceps, triceps, brachioradialis, patellar, achilles reflexes
Normal tone, no atrophy
Severely ataxic, wide based gait
No ticks present on skin exam
LP, encephalitis and tick panel
Obtain NIF (-30, -38 post IVIG)
Follow Up/Clinical Course:
Admitted to PICU
IVIG daily x5 days
NIF increased to -60, never required intubation or supplemental oxygen
CSF unremarkable (Protein 18, WBC 1)
11 days inpatient
2 weeks outpatient rehab
Discovered in 1916 by Drs Guillain, Barre, and Strohl who observed albuminocytological dissociation in two independent cases.
2/3 of patients experience an infection in the month before onset (30% Campylobacter jejuni). Autoimmune destruction of Schwann cells causing demyelination of peripheral nerves and motor fibers.
Take Away #1
LP will show Albumin-cytological dissociation of CSF (protein >45, WBC <10). MRI may show enhancement of anterior spinal nerve roots.
Take Away #2
NIF has not been shown to be more effective than FVC, and is more uncomfortable.
Jeremy Hardin, MD is a current first year resident at Stony Brook Emergency Medicine.
Edited by Bassam Zahid, MD