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From Guillain-Barre syndrome

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Guillain-Barre syndrome

Guillain-Barre syndrome (GBS) is also called as acute idiopathic polyradiculoneuritis. This is the most common aquired demyelinating disease. This disease is characterized by the inflammatory disorder of the peripheral nerves. The incidence of Guillain-Barre syndrome is one in 100,000. The life time risk for any one individual getting Guillain-Barre syndrome (GBS) is 1/1000. All age group may affected by GBS. Both sexes are equally affected. The disease is characterized by weakness of limbs and numbness and tingling sensation over the limbs, body and the face. Mild cases of Guillain-Barre syndrome may present only with ataxia (difficulty in walking and balancing), whereas severe cases may present with difficulty in respiration due to paralysis the respiratory muscles and cranial nerves.

What causes Guillain-Barre Syndrome?

  • The actual cause for Guillain-Barre syndrome is still not known. It may be an autoimmune disease; in which antibodies are directed against the peripheral myelin (myelin is the sheath covering of the peripheral nerves and is responsible for faster conduction of nerve signals).
  • A mild respiratory or gastrointestinal infection may be present 1-3 weeks before the onset of neurological symptoms in 60% of the patients.
  • Infection with Campylobacter jejuni is the most often found association with Guillain-Barre syndrome.
  • Viral infection, minor surgery, immunization, mycoplasma infection may also be a preceding event in Guillain-Barre syndrome.
  • High frequency of occurrence of this Guillaine-Barre syndrome is found in the following conditions than in the normal population: Hodgkin's disease, lymphoma, systemic lupus erythematosis.

What are the symptoms of Guillaine-Barre syndrome?
  • Most of the patients present with numbness and tingling of the limbs in the initial phase of the disease.
  • The major complaint is the weakness of limbs which will be mostly symmetrical. Both proximal and distal parts of the limbs are affected. The lower limbs are affected first. Then the weakness spreads to trunk, respiratory muscles, upper limbs, neck muscles and cranial muscles.
  • Sometimes the weakness may progress to complete paralysis of the body leading to respiratory difficulty and death in a few days.
  • About 50% of the patients, complaint of pain and aching discomfort of the hip, thigh and back muscles.
  • Facial muscle weakness may be present in 50% of the cases. Facial muscle weakness usually comes after the upper arm weakness.
  • Difficulty in passing urine (urinary retention) occurs in 15% of the patients.

How do you diagnose Guillaine-Barre syndrome?
Most of the time, a thorough neurological examination with laboratory investigation will clinch the diagnosis of Guillaine-Barre syndrome.

Features required for diagnosis of Guillain-Barre syndrome (GBS) :
  • Progressive motor weakness of more than one limb: From minimal weakness with or without ataxia (difficulty if balancing) to complete paralysis, which may include cranial nerves. Proximal muscles are more affected than distal ones.
  • Areflexia(loss of muscle stretch reflexes)-usually universal

Features strongly supportive of diagnosis of Guillain-Barre syndrome (GBS) :
  • Progression of the motor weakness in the patient peaks at 2 weeks in 50%, by 3 weeks in 80% and by 4 weeks in 90%.
  • There is relative symmetry of weakness in the body. That is, both half of the body are equally affected.
  • Mild sensory symptoms-mild tingling and numbness in hand and feet.
  • Cranial nerve involvement: Facial weakness in 50%, usually bilateral.
  • Oropharyngeal muscles (muscles of the oral cavity and throat) may be affected.
  • Recovery of the patient starts usually by 2-4 weeks after the progression stops, but may be delayed by months
  • Tachycardia (increase in heart rate), postural hypotension
  • Fever, sensory loss with pain, bladder paralysis (urinary retention)
  • Lumbar puncture is done and the Cerebrospinal fluid (brain fluid) is examined. Albuminocytological dissociation (elevated CFF protein) is the most important finding in Guillain-Barre syndrome.
  • Nerve conduction studies shows that, 80% of the patient have nerve conduction slowing or block at some time of their illness.

What are the other conditions that mimic Guillain-Barre syndrome?
  • Current hexa carbon abuse: volatile solvents, glue sniffing
  • Acute intermittent porphyria- It is a disorder of porphyrin metabolism (disorder of the red blood cells). Here CSF protein is not elevated .Recurrent painful abdominal crisis are common. Urine analysis is helpful in diagnosing this condition.
  • Recent diphtheretic infection: Diptheretic polyneuropathy has a longer latency and slower crescendo of symptoms.
  • Lead neuropathy: It usually present with upper limb weakness and wrist drop.
  • Poliomyelitis: Usually asymmetric. Patient will have signs of meningeal irritation like headache, neck pain etc.
  • Toxic neuropathy: due to the usage of drugs like nitrofurantoin, dapsone, thallium or arsenic.
  • Tick paralysis: May causing ascending motor neuropathy without sensory symptoms. Careful examination of the scalp must be done to see for ticks.
  • Chronic immune demyelinating polyradiculoneuropathy: Here the symptoms are present for greater than 2 months. Progressive symmetrical proximal and distal weakness is present. Cranial nerves are usually spared. Balance is commonly affected. Need for respiratory support is rare. Electro diagnosis and nerve biopsy are indicative of demyelination. CSF findings are similar to Guillaine-Barre syndrome. Most cases respond to treatment with methylprednisolone (steroid).
  • Miller fisher variant of Guillaine Barre syndrome includes ataxia, areflexia and opthalmoplegia.

What are the treatment options available for Guillain-Barre syndrome?
  • Immunoglobulins may be helpful.
  • In severe cases, early plasmapheresis (filtration of the blood using special filters to separate the toxic products that causes damages to the myelin) hastens the recovers and reduces the residual defect.
  • Mechanical ventilation will be necessary in 30% of the patients, who have respiratory muscle paralysis.
  • Measures to prevent aspiration are carried out.
  • Deep vein thrombosis should be prevented in to prolonged bed ridden patients
  • Steroids are not helpful.
  • In case of facial muscle weakness, the patient will have difficulty in closing the eyes. So, the eyes must be protected to prevent exposure keratitis.( which may lead to blindness)

What is the prognosis of Guillain-Barre syndrome?
Majority of the patients recover completely or with minimal residual defect. Recovery may not be complete for several months.35% of untreated patients have residual weakness and atrophy of the muscles. Recurrence of Guillain-Barre syndrome after achieving maximal recovery is 2%.

Did you know?
The life time risk for any one individual getting Guillain-Barre syndrome (GBS) is 1/1000. All age group may affected by GBS. Both sexes are equally affected. The disease is characterized by weakness of limbs and numbness and tingling sensation over the limbs, body and the face. Mild cases of Guillain-Barre syndrome may present only with ataxia Guillain-Barre syndrome

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