Guillain-Barré Syndrome

Guillain-Barré Syndrome

Guillain-Barré syndrome is a post-infectious demyelinating polyneuropathy, the most common demyelinating lesion. It is characterized by peripheral paralysis of the muscles of the extremities and protein-cell dissociation in the CSF while maintaining superficial sensitivity. May have an ascending character involving the muscles of the face, pharynx, larynx (Landry’s ascending palsy). Frequency – 0.6-1.9 cases per 100,000 population. The predominant gender is male. Etiology. There is an opinion about the autoimmune nature of the disease. Develops subsequently or during the following conditions:

• • Infectious diseases
  • Upper respiratory tract infections
  • Infectious mononucleosis
  • Cytomegalovirus infection
  • herpetic infection
  • Influenza A
  • Mycoplasma infection
  • Mumps
  • HIV infection
  • Lymphoma (especially Hodgken’s)
  • Vaccination, serum sickness
  • Operational intervention.

Pathogenesis. Selective demyelination of the roots of the spinal cord, apparently of an autoimmune nature. Against the background of immune disorders, edema, inflammatory cell infiltration and diffuse primary segmental demyelination appear, primarily in the anterior roots and proximal sections of the spinal nerves, plexuses, nerves of the extremities and autonomic nodes. Pathomorphology

• • Segmental demyelination of peripheral nerves, in severe cases, axonal degeneration
  • Lymphocytic and monocytic infiltration of the myelin sheath and perivascular area.

Clinical picture

• • As a rule, after 2 weeks, after a viral infection or immunization, weakness of the muscles of the distal lower extremities suddenly develops. In 65% of cases, the disease manifests itself within 3 weeks after an infectious disease.
  • Ascending progressive muscle weakness continues for 2-4 weeks with the transition to the diaphragm and muscles innervated by cranial nerves. The patient’s condition remains stable for approximately 4 weeks.
  • Symmetrical distal flaccid paresis that begins in the legs, then spreads to the arms within a few days
  • Bulbar disorders – bilateral paresis of the muscles of the face and oropharynx (difficulty swallowing)
  • Respiratory muscle paralysis (5-10% of cases)
  • Loss of sensation in the type of socks and gloves (varies, in some cases quickly disappears)
  • Decrease and then loss of deep tendon reflexes
  • Autonomic disorders
  • Inadequate ADH secretion (urinary retention)
  • Arrhythmias
  • BP fluctuations.

Special Studies

• • Electromyography – a significant decrease in the amplitude of the muscle response when stimulating the distal parts of the peripheral nerve. Nerve impulse conduction is slow
  • Lumbar puncture. An increase in protein content, sometimes significant (>10 g/l), begins a week after the onset of the disease, for a maximum of 4-6 weeks. Differential Diagnosis
  • Intoxications leading to impaired neuromuscular transmission
  • Heavy metal poisoning (lead, arsenic)
  • Poisoning by industrial substances (acrylamide, carbon disulfide, trichlorethylene, rapeseed oil, organophosphorus compounds)
  • Intoxication when taking drugs: gold products, hydralazine, disulfiram, glutethimide, diphenin, nitrofurantoin, dapsone, metronidazole, isoniazid, pyridoxine when taken more than 2 g / day
  • Alcohol intoxication
  • Neuropathy in diabetes mellitus, porphyria, polyarthritis nodosa, rheumatoid arthritis
  • Vitamin B12 or folic acid deficiency
  • Congenital polyneuropathies – Charcot-Marie-Tooth disease, metachromatic leukodystrophy, adrenoleukodystrophy, Dezherche-Sott neuritis
  • Nerve damage in cancer
  • Infectious diseases
  • Acute poliomyelitis
  • Diphtheria (complicated by paralysis)
  • Botulism.

Treatment

• • According to indications – mechanical ventilation (in 10-23% of cases), tracheostomy
  • In severe cases, plasmapheresis
  • Sufficient fluid intake to maintain diuresis at the level of 1-1.5 l / day under the control of the concentration of serum electrolytes
  • Physiotherapy to prevent contractures
  • Prednisolone up to 80-120 mg / day orally and the introduction of y-globulin / in – only in chronic progressive or recurrent course
  • Posyndromic therapy
  • Heparin 5,000 units s / c 2 r / day – for the entire period of bed rest.

Complications

• • During illness: paralysis, respiratory failure and aspiration, arterial hypertension or hypotension, arrhythmias, urinary retention, depression
  • The consequences of the development of the chronic stage: chronic inflammatory demyelinating polyradicular neuropathy.

Course and forecast

• • Characterized by an acute onset and a progressive course. Usually, the progression of the process continues for 2-4 weeks, then gradually, approximately within a year, there is a restoration of functions.
  • In 7-22% of patients, residual neurological deficits are detected (weakness, decreased reflexes)
  • 10% relapse within one year of recovery or develop severe complications
  • Mortality – within 3%
  • The prognosis for unclear etiology is favorable in about 50% of cases.

Synonyms

• • Guillain-Barré-Stroll Syndrome
  • Polyradiculoneuritis acute primary idiopathic
  • Infectious idiopathic polyneuropathy
  • Radiculoanglionitis
  • Landry-Guillet-on-Barre syndrome
  • Acute demyelinating polyradiculoneuropathy / names – Barre

See also Botulism, Diphtheria, Poliomyelitis, Diabetes insipidus, Sugar diabetes, Porphyria, Rheumatoid arthritis, Hypovitaminosis B/2, ICD alcoholism. G61.0 Guillain-Barreux syndrome