Guillain-Barré syndrome (GBS)

Definition

Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyradiculoneuropathy of autoimmune etiology. The diagnosis of Guillain-Barré syndrome is established in the presence of increasing weakness and areflexia in more than one limb. It should exclude other neurological diseases accompanied by peripheral paresis: poliomyelitis, acute period of trunk stroke, toxic lesions of the CNS, and others. Treatment of patients with Guillain-Barré syndrome is carried out in the hospital, as the patient may require ventilator support.

General information

Guillain-Barré syndrome (GBS) is an acute demyelinating inflammatory polyneuropathy of autoimmune etiology. The characteristic features of the disease are peripheral paralysis and protein-cell dissociation in the liquor (in most cases). At present, four main clinical variants are distinguished within GBS:

• classic form of GBS – acute inflammatory demyelinating polyradiculoneuropathy (up to 90% of cases)
• axonal form of GBS – acute motor axonal neuropathy. The characteristic feature of this form of GBS is an isolated lesion of motor fibers. In acute motor and sensory axonal neuropathy, both motor and sensory fibers are affected (up to 15%)
• Miller-Fisher syndrome is a form of GBS characterized by ophthalmoplegia, cerebellar ataxia, and areflexia with mild paresis (up to 3%).

In addition to the above-mentioned forms of Guillain-Barré syndrome, several other atypical forms of the disease have recently been identified – acute sensory neuropathy, and acute cranial polyneuropathy, which are quite rare.

Clinical picture of Guillain-Barré syndrome

The first manifestations of Guillain-Barré syndrome are usually muscle weakness and/or sensory disorders (numbness, paresthesias) in the lower extremities, which spread to the upper extremities after a few hours (days). In some cases, the disease is manifested by pain in the muscles of the limbs and lumbosacral region. Very rarely, the first manifestation is lesions of the peripheral nerves (oculomotor disorders, impaired phonation, and swallowing). The degree of motor disorders in Guillain-Barré syndrome varies considerably – from minimal muscle weakness to tetraplegia. Paresis is usually symmetrical and more pronounced in the lower extremities.

Hypotonia and a significant decrease (or complete absence) of tendon reflexes are typical. Respiratory failure develops in 30% of cases. Disorders of superficial sensitivity manifest as mild to moderate hypo- or hyperkinesia of polyneuritic type. Approximately half of patients have disorders of deep sensitivity (sometimes up to its complete loss). Peripheral nerveslesions, detected in most patients, are manifested by paresis of mimic muscles and bulbar disorders. The most frequent autonomic disorders are cardiac arrhythmias, arterial hypertension, sweating disorders, GI and pelvic organ dysfunctions (urinary retention).

Diagnosis

When collecting anamnesis, it is necessary to pay attention to the presence of provoking factors, as in more than 80% of cases, the development of GBS is preceded by certain diseases and conditions (transferred infections of the gastrointestinal tract, upper respiratory tract, vaccination, surgical interventions, intoxication, tumor). The neurological examination aims to identify and assess the severity of the main symptoms of Guillain-Barré syndrome – sensory, motor, and autonomic disorders.

It is necessary to conduct general clinical studies (general urine analysis, general blood analysis), biochemical blood analysis (blood gas composition, serum electrolyte concentration), liquor examination, serologic studies (if infectious etiology of the disease is suspected), as well as electromyography, the results of which are essential to confirm the diagnosis and determine the form of GBS. In severe cases of the disease (rapid progression, bulbar disorders), daily BP monitoring, ECG, pulse oximetry, and external respiratory function (spirometry, peak flowmetry) should be performed.

The presence of progressive muscle weakness in more than one limb and the absence of tendon reflexes (areflexia) is necessary to confirm the diagnosis. The presence of persistent pelvic disorders marked persistent asymmetry of paresis, polymorphonuclear leukocytes, and a precise level of sensory disorders should raise doubts about the diagnosis of Guillain-Barré syndrome. In addition, several features exclude the diagnosis of GBS, including recent diphtheria, symptoms of lead intoxication or evidence of lead intoxication, the presence of sensory disturbances alone, and porphyrin metabolism disorders.

Differential diagnosis

First of all, a neurologist differentiates Guillain-Barré syndrome from other diseases that also manifest peripheral paresis (poliomyelitis), as well as other polyneuropathies. Polyneuropathy in acute intermittent porphyria may resemble Guillain-Barré syndrome but is usually accompanied by a variety of psychopathologic symptoms (hallucinations, delusions) and marked abdominal pain. Symptomatology similar to the signs of GBS is possible in extensive brainstem strokes with the development of tetraparesis, which in the acute period takes the features of peripheral. The main differences between myasthenia gravis and GBS are variability of symptoms, absence of sensory disorders, and characteristic changes in tendon reflexes.

Treatment

All patients diagnosed with Guillain-Barré syndrome should be hospitalized in a hospital with an intensive care and intensive care unit. In about 30% of cases of GBS, due to the development of severe respiratory failure, there is a need for a ventilator, the duration of which is determined individually, focusing on the vital signs, restoration of swallowing, and cough reflex. Disconnection from the ventilator is carried out gradually with a mandatory stage of intermittent forced ventilation.

In severe cases with severe paresis, proper care is of particular importance to prevent complications associated with prolonged immobility of the patient (infections, bedsores, pulmonary embolism). It is necessary to have periodic (at least once every 2 hours) changes in the patient’s position, skin care, control of bladder and bowel functions, passive exercises, and prevention of aspiration. In persistent bradycardia with the threat of asystole may require the installation of a temporary pacemaker.

As a specific therapy for Guillain-Barré syndrome aimed at controlling the autoimmune process, pulse therapy with class G immunoglobulin and plasmapheresis are currently used. The effectiveness of each method is relatively equal, so their simultaneous use is considered inappropriate. Membrane plasmapheresis significantly reduces the severity of paresis and the duration of ventilation. 

It should be remembered about contraindications to plasmapheresis (infections, clotting disorders, liver failure) and possible complications (electrolyte disturbance, hemolysis, allergic reactions). Class G immunoglobulin, as well as plasmapheresis, reduces the duration of stay on a ventilator. Possible side effects: nausea, headaches, muscle aches, fever.

Symptomatic therapy in Guillain-Barré syndrome corrects acid-base and water-electrolyte balance disorders, corrects blood pressure levels, and prevents deep vein thrombosis and thromboembolism. Surgical intervention may be necessary for tracheostomy in case of prolonged ventilation (more than 10 days) and gastrostomy in case of severe and prolonged bulbar disorders.

All these treatment and diagnostic options are available in more than 400 hospitals worldwide (https://doctor.global/results/diseases/guillain-barre-syndrome-gbs). For example, Lumbar puncture can be performed in these countries at following approximate prices:

Israel$295 in 11 clinics

Turkey$502 in 14 clinics

China$1,748  in 7 clinics

Germany$1,756  in 22 clinics

United States$3,225  in 12 clinics.

Prognosis

Most patients diagnosed with Guillain-Barré syndrome show complete functional recovery within 6-12 months. Persistent residual symptoms persist in about 7-15% of cases. The recurrence rate of GBS is about 4%, and lethality is 5%. Possible causes of death are respiratory failure, pneumonia or other infections, and pulmonary embolism. The probability of death depends significantly on the age of the patient: in children under 15 years of age, it does not exceed 0.7%, while in patients over 65 years of age, it reaches 8%.

Prevention

There are no specific methods of prevention for Guillain-Barré syndrome. However, the patient should be advised that immunizations are prohibited for the first year after the onset of the disease, as any immunization can cause a relapse. Further immunization is permitted, and the need for immunization must be justified. In addition, developing Guillain-Barré syndrome within six months of any vaccination is a contraindication to future vaccine use.