Recurrent AIDP vs CIDP vs sensory GBS variant 2/4/2019

Thank you Julie for presenting a case of a 27yo F with a history of Guillain Barre syndrome 2 years ago, requiring 1 year of rehab but now with full neurological recovery, presenting with numbness and tingling of her lower extremities and her hands. Her symptoms are described as “ants crawling” on her skin, which started at her feet but became progressively more superior. Also endorses involvement of her hands as well later on. She has no recent illnesses and she has no illicit drug use. Her first episode of GBS started out very similarly, hence she was concerned.

EMG revealed a primarily demyelinating polyneuropathy affecting motor > sensory nerves predominantly in the lower extremities… but wait, does it even fit her symptoms, which are primarily sensory (although mild subjective weakness)???


We will use this case to illustrate that there is a spectrum of Guillain Barre!

GBS

Epidemiology

  • All age groups affected but risk increases with more advanced age
  • M > F slightly
  • Most commonly associated with after an episode of campylobacter jejuni infection.
  • Other culprits: VMC, EBV, HIV, Zika virus, Mycoplasma.
  • Rare cases of GBS can happen after a triggering event, i.e. immunization, surgery, BMT.

Pathophysiology

  • Immune response to a preceding infection that cross-reacts with peripheral nerve leading to demyelination (a form of molecular mimicry)
  • Note that there are two forms of peripheral neuropathy:
    • Axonal
      • Pathophys: Disruption of the axon itself, typically presents with motor >>> sensory deficits.
      • DDx: HIV, amyloidosis, B12 deficiency, Lyme disease, hypothyroidism, critical illness polyneuropathy, variant of GBS
    • Demyelinating
      • Pathophys: Disruption of the myelin sheath, slowing nerve conduction velocity. Both sensory and motor deficits would be present.
      • DDx: GBS (classic), hereditary, infectious, drugs, certain monoclonal gammopathies.
  • Picture1.jpg

Variants of GBS include but are not limited to…

  • AIDP (Acute Inflammatory Demyelinating Polyradiculoneuropathy)
    • Most common form, (85-90%)
    • Disease activity usually nadirs by 4 weeks
    • Progressive ascending symmetric weakness due to inflammatory demyelination
    • 2-5% may develop CIDP
  • CIDP (Chronic Inflammatory Demyelinating Polyradiculoneuropathy)
    • Disease progression or relapses lasting longer than 8 weeks
    • Most of the time does not have an antecedent/preceding event.
  • Miller Fisher Variant
    • 10-20% of cases
    • Typical presentation: ophthalmoplegia, ataxia, areflexia.
    • 25% will develop some extremity weakness
    • 85-90% of pts with MFS will have GB1b antibodies positivity, strongly associated with involvement of oculomotor nerves.
    • If presence of encephalitis: Bickerstaff encephalitis, possibility a spectrum of anti-GQ1b antibody syndrome.
  • Acute motor axonal neuropathy (AMAN): Axonal form of GBS
    • More rapid progression motor wise but sensory and DTR usually preserved.
    • Most cases preceded by campylobacter infection as well
    • Frequent in Asia and esp in Hong Kong, more prevalent in the summer
    • More severe form is AMSAN (Acute motor and sensory axonal neuropathy)
  • Pharyngeal-cervical-brachial variant
    • “Localized” version of GBS
  • Pure Sensory GBS
    • Primarily sensory deficits with mild motor deficits.

Presentation

  • Timing: Progresses over 2 weeks, most cases start improving by 4 weeks. IF post-infection, sx can develop after days to weeks.
  • Progressive symmetric ascending muscle weakness starting in the lower extremities and then spread upward.
  • 10% of the time, muscle weakness starts in arms or facial muscles.
  • Absent to depressed DTR seen in all cases
  • Respiratory muscle weakness requiring ventilator support in 10-30% of cases
  • 80% of patients will experience paresthesias of the hands and feet accompanying motor weakness.
  • Neuropathic pain.
  • Dysautonomia in 70% of cases (tachycardia, HTN/Hypotension, bradycardia, ileus, urinary retention, arrhythmias, can also see SIADH.

Diagnosis

  • CSF: LP recommended to support dx
    • Elevated protein (45-200 typically but can be as high as >1000 mg/dL) with nrl WBC (albuminocytologic dissociation), seen in 50-66% in the 1st week after onset of sx, >75% of pts in the 3rd
    • CSF cell count typically nrl, < 5 cells/mm3, in a minority of cases can expect to see mild elevation.
  • EMG: Valuable for confirming dx but can be nrl early on the disease course.
  • GQ1b antibody: associated with Miller Fisher variant
  • MRI: Spinal MRI may reveal enhancement of spinal nerve roots and cauda aquina.

Management

  • Secure airway!
    • Predictors of respiratory failure: inability to cough, stand, lift the head, lift the elbows, and liver enzyme elevation.
    • Avoid succinylcholine in intubation, increased risk of hyperkalemia.
  • Supportive care, DVT prophylaxis, bladder/bowel care, PT/OT, pain control
  • Plasma exchange/IVIG is indicated in most cases, speed up recovery.
    • Plasma exchange usually more effective when started within 7 days of sx onset. 4-6 tx over 8 days.
    • IVIG: 0.4g/kg per day x 5 days.
    • IVIG and plasma exchange also recommended for ambulatory patients who are still not seeing improvement of sx after 5 weeks.
  • No role of steroids!

Prognosis

  • Full motor recovery around 60% at 1 year. Recovery can take several years.
  • 2-5% develop CIDP
  • 3-7% mortality rate, 20% of patients who become ventilator dependent die of complications.

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s