30 - Guillain-Barre Syndrome Flashcards
Case 3
- 26 y/o white male with progressive limb weakness and numbness
- Noted numbness in palms 11 days ago with hands feeling “swollen and prickly”.
- 2 days later, same feelings under toes.
- 3 days later has difficulty climbing stairs, rising from a chair, cannot wash hair because he cannot keep arms over head.
- Constipation
- Numbness inside mouth and foods taste salty
So far we know that there is both sensory and motor neuropathy plus autonomic (constipation)
Seems to involve both upper and lower extremities
What is important to focus on when thinking about a diagnosis for this patient?
It is an ACUTE onset
Think: PAT PAT
Describe the past medical history of this patient
- No health problems
- Severe GI “flu” with diarrhea for 3 days one month ago
Some viral or bacterial etiology could have caused this
Medications and allergies
- Denies medications
- Denies allergies to environment, foot, drugs
Describe the social history of this patient
- Denies tobacco and alcohol
- Works as a custodian
- Volunteer fireman
Work place exposure to toxic cleaners?
Describe the physical exam in this patient
- Appears ill and is profusely diaphoretic
- BP: 140/110; Pulse: 116; Resp: 18, Temp: 98.0
- Lungs are clear to auscultation
- Cardiac exam normal
- Orientated x 3
- Unable to stand on toes, but can stand on heels (indicates a CNS problem)
- Unable to arise from a chair without pushing up with his arms
- Hand grips are weak bilateral
- Decreased touch, temperature, vibration below knees with profound loss of proprioception in toes bilateral
- Normal pin sensation
- Knee and reflexes are 0/4 bilateral
- Babinski sign is downgoing (normal)
BOTH large and small fiber neuropathy
What is on the differential list for this case?
Acute
- PAT PAT
Chronic
- A DUMB MMedIcAl BLmOG to CHARt Infections
Things that are possible…
- Bacterial meningitis-raised neutrophils
- Viral meningitis-raised lymphocytes
- Subarachnoid hemorrhage-elevated RBC
- Guillain-Barre-normal WBC
- Multiple sclerosis-raised lymphocytes
What else would you want to get for this patient?
- A1c
- CBC
- CMP
- Tox screen
- Lumbar puncture
CBC and CMP were normal. Went ahead with a lumbar puncture…
** KNOW THIS ***
Lumbar puncture results
- WBC = 1
- RBC = 0
- Glucose = 67
- Protein = 88
Normal levels
- WBC =
What is the significance of the CSF findings?
- Elevated protein in the absence of white cells (so-called albumino-cytologic dissociation) suggests a demyelinating polyneuropathy
- Elevated protein in the absence of white cells (so-called albumino-cytologic dissociation) suggests a demyelinating polyneuropathy
Are the pateints signs and symptoms localized to the CNS or PNS?
There are signs and symptoms of both
- Ability to stand on heels but not toes suggests a problem with the spinal cord (CNS)
- However, hyporeflexia, negative Babinski, and distal sensory loss in a stocking-glove distribution more strongly suggest PNS problem
What would you expect muscle tone to be?
Flaccid due to lower motor nerve dysfunction due to a peripheral neuropathy
Why are the reflexes diminished?
- Hyporeflexia is seen with a peripheral neuroapthy and suggests a problem with the sensory neuron, dorsal column motor neuron, synapsis or muscle
Why was he constipated, tachycardic and diaphoretic?
All are signs of autonomic nerve dysfunction of the autonomic fibers of the PNS
What is the most likely diagnosis?
Guillain-Barre syndrome
This is acute inflammatory demyelinating polyneuropathy (AIDP)
In this case, since it is a young healthy guy and the onset was so acute, I think PAT PAT would take precedence over the other slower onset
What is the prognosis?
Good
Need to monitor - they are susceptible to cardiac and respiratory (ventilation) issues
There is a good chance of a full recovery and only 35% will have residual effects
Most of the motor/sensory/autonomic functions will come back, but not all of these symptoms will
What is the treatment?
Filter the blood (plasmaphoresis)
IV immunoglobulins
Close medical support for respiratory and cardiac
What might nerve conduction studies show?
Demyelinating etiology
- Prolonged distal sensory and motor latencies
- Diffusely decreased nerve conduction velocities
- Findings are due to severe demyelination of peripheral nerves, which slows conduction of action potentials
What about the diarrhea?
Suggests a previous bacterial infection or viral gastroenteritis, which precedes syndrome in 50% of patients
Guillain-Barre syndrome is commonly preceded by this
How does Guillain-Barre differ from multiple sclerosis?
- G-B is acute with PNS demyelination
- MS is chronic, with exacerbations and remissions, and is due to CNS demyelination
