Guillain-Barre Syndrome Disease

BASICS

DESCRIPTION

A group of demyelinating diseases causing acute progressive weakness, usually an ascending paralysis. 30% have respiratory paralysis requiring mechanical ventilation, but complete or substantial recovery is the rule.

System(s) affected: Nervous
Genetics: Not directly genetically inherited. presumed to be individual's idiosyncratic response to preceding infection, which may have a genetic basis
Incidence/Prevalence in USA: 0.6-1.9/100,000, non-seasonal, non-epidemic
Predominant age: All ages
Predominant sex: Male > Female (1.5:1)

SIGNS AND SYMPTOMS

Acute, progressive weakness of all 4 limbs, symmetric and usually ascending
Areflexia associated with the muscle weakness
Pain common, especially back, legs, and extremities, may be severe, not predictive of disease course
Gait disorder common in all age groups: the most common presentation in children
Dysesthesias, paresthesias (often perioral), pain in band distribution, decreased position and vibratory sensation common
Autonomic neuropathy (50%) with hypo- and hypertension, labile BP, arrhythmias (atrial and ventricular), ileus, and/or urinary retention
Respiratory muscle paralysis 30% in untreated disease. Neck muscle weakness, dysphagia, shoulder weakness predictors of impending respiratory failure.
Cranial nerve involvement 50%: usually facial weakness, 10–20% ocular involvement
GBS divided into 3 disease forms:
- AMAN (acute motor axonal neuropathy): pure motor involvement, previous Campylobacter jejuni infection, axonal injury, poorer prognosis for recovery
- AMSAN (acute motor-sensory axonal neuropathy): both motor and sensory involvement
- AIDP (acute inflammatory demyelinating polyradiculopathy)
Important variants:
- Miller-Fisher syndrome (5%): ataxia, ophthalmoplegia, and areflexia
- Bulbar variant: dysphagia, dysarthria, drooling with descending weakness
- Other variants: only lower extremity weakness; pharyngeal-cervical-brachial muscle weakness; pure sensory forms. Upper extremity weakness may occur before lower extremity weakness; proximal reflexes may be lost before distal. Cranial nerves frequently involved.

CAUSES

Autoimmune destruction of myelin and/or axon by anti-ganglioside antibodies. Activated T-cells, macrophages, and increased matrix metalloproteinases may also play a role.
Upper respiratory or diarrheal illness within previous 1–3 weeks in 50–70%, including Campylobacter jejuni (30–40%), CMV (13%), EBV (10%), Mycoplasma pneumoniae (5%), HIV
Vaccinations: swine flu (but not other influenza vaccine) and rabies
Malignancies (lymphoma, especially Hodgkin's)
Surgery
Drugs: gold, penicillamine, streptokinase, captopril, danazol, IV heroin, IV gangliosides

RISK FACTORS

Antecedent infection, especially C. jejuni, which has more severe course and higher residual disability. Recent CMV predicts delay in early recovery.

DIAGNOSIS

LABORATORY

CSF: elevated protein with normal cell count and pressure. Protein normal in 50% of cases in first week of illness, becoming elevated within 3 weeks.
Blood: CBC, electrolytes, liver and renal function studies; SLE and PAN screen; HIV testing; if appropriate, Lyme disease and sarcoidosis testing
Urine: acute intermittent porphyria
Imaging: MRI spinal cord if any evidence of cord lesion or myelopathy
Other: consider heavy metal testing; review medications (e.g., chemotherapeutics, nitrofurantoin, megavitamins especially pyridoxine); if previous diarrheal illness, consider C. jejuni antibodies

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS

Multifocal inflammatory cell infiltration of peripheral nerves with patchy demyelination and/or axonal degeneration

SPECIAL TESTS

N/A

IMAGING

To exclude other causes, especially spinal cord lesions/cord compression

DIAGNOSTIC PROCEDURES

Nerve conduction studies: Evidence of multifocal demyelination (decreased amplitude, prolonged distal latencies, decreased CMAP, F-wave abnormal, and conduction block) in motor and sensory nerves. Important to test several different nerves. May be normal early in disease.
Lumbar puncture: elevated protein with normal cell count

TREATMENT

APPROPRIATE HEALTH CARE

Hospitalization with monitoring of respiratory function and elective intubation for impending respiratory failure
IVIG/plasmapheresis for all patients too weak to walk, rapid progression, or poor prognostic indicators
Intensive care monitoring for dysautonomia and arrhythmias as indicated

GENERAL MEASURES

Serial measurements FVC and oximetry with intubation for respiratory distress or FVC <20 mL/kg (<1L in adults). FVC best measurement (oximetry/ABGs normal until respiratory failure has occurred; peak flow measures air flow resistance not respiratory muscle strength).
Intubation for difficulty swallowing/aspiration from bulbar palsy
Cardiac monitoring for arrhythmias: Tachyarrhythmias usually do not require treatment; bradyarrhythmias – atropine; Sinus arrest and complete heart block – temporary pacemaker
Hypertension: often does not need treatment. Small doses beta blockers if needed. Avoid vasodilators including calcium channel blockers (may precipitate hypotension).
Hypotension: Trendelenburg position and volume expansion (IV fluids)
Pain common, can be severe and recalcitrant: analgesics including opioids; TCAs, carbamazepine, TENS helpful adjuncts
Constipation/ileus from autonomic neuropathy: laxatives, enemas
Urinary retention from autonomic neuropathy: catheterization
Prevent complications of immobilization, e.g., pneumonia, decubitus ulcers, DVT/PE, contractures
Depression: frequent reminders that recovery is the rule; antidepressants.

SURGICAL MEASURES

Tracheostomy for prolonged intubation

ACTIVITY

No limitation. Prevent contractures and assist with weakness.

DIET

No special diet. Enteral feedings if intubated.

PATIENT EDUCATION

Important to stress expectation of full/significant recovery

FOLLOW UP

PREVENTION/AVOIDANCE

N/A

POSSIBLE COMPLICATIONS

Paralysis, permanent weakness
Respiratory failure, mechanical ventilation
Hypotension, hypertension, labile BP
Cardiac arrhythmias
Ileus
Urinary retention
Aspiration, pneumonia, sepsis
DVT, PE
Psychiatric problems including depression

EXPECTED COURSE AND PROGNOSIS

Untreated

3 phases of illness:
- Initial progression phase 24 hours – 3 weeks, usually 10–14 days: highest risk of death and complications during this phase
- Plateau phase: same approximate duration as initial phase
- Recovery phase: generally 1–6 months, up to 2 years. No further improvement after 2 years.
2–5% mortality (most often from dysautonomia or complications), 50% complete recovery, 50% some residual disability with 5% severe permanent disability. Relapses may occur both before and after recovery.
Poor prognostic signs:
- Rapid progression to severe disease (<7 days)
- Mechanical ventilation
- Nerve conduction studies: CMAP <10% or mean distal motor amplitude <20%
- AMAN (see Differential Diagnosis)
- Preceding C. jejuni infection
- Age over 60 years

MISCELLANEOUS

ASSOCIATED CONDITIONS

Chronic inflammatory demyelinating polyneuropathy (CIDP)

AGE-RELATED FACTORS

Pediatric: Disease tends to be milder
Geriatric: Worse prognosis >60 years
Others: N/A

PREGNANCY

No data

OTHER NOTES

N/A

ABBREVIATIONS

GBS = Guillain-Barre syndrome
AIDP = acute inflammatory demyelinating polyradiculopathy
AMAN = acute motor axonal neuropathy
AMSAN = acute motor-sensory axonal neuropathy
CMAP = compound muscle action potential
CIDN = chronic inflammatory demyelinating neuropathy
DVT = deep venous thrombosis
PE = pulmonary embolism
IVIG = intravenous immunoglobulin
IgA = immunoglobulin A
SLE = systemic lupus erythematosus
PAN = polyarteritis nodosa
CMV = cytomegalovirus
EBV = Ebstein-Barr virus
HIV = human immunodeficiency virus

Clinical Investigations

ROLE OF HOMOEOPATHY

Holistic healing that treats the person, not just the disease. Experience gentle, lasting wellness with expert constitutional care.