Guillain-Barre syndrome

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Guillain-Barre syndrome

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A 19-year-old football player is brought to the emergency department by his roommate after he complained of lower extremity weakness. He initially had “tingling” of the toes, but he attributed it to an “intense practice” several days ago. The patient also reports he has not voided urine in the last 5 days. Four weeks ago, the patient had a febrile diarrheal illness that resolved after several days. Temperature is 37.0°C (98.6°F), pulse is 44/min, respirations are 14/min, and blood pressure is 128/74 mmHg. Physical examination reveals absent ankle and knee reflexes but intact sensation to light touch and pain. A lumbar puncture is performed. Which of the following cerebrospinal fluid findings is most likely to be seen in this patient?  

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Guillain-Barré syndrome is named after two neurologists- Georges Guillain and Jean Barré, and it’s a demyelinating disease of the peripheral nervous system, which includes all of the neurons that extend beyond the brain and the spinal cord.

Guillain-Barré, or GBS for short, is also called acute inflammatory demyelinating polyneuropathy.

Neurons are made up of three main parts.

The dendrites, which are little branches that receive signals from other neurons, the soma or cell body, which has all of the neuron’s main organelles, and the axon, which transmits the signal to the next neuron in the series.

For peripheral nerves, the cell body can either be located in the spinal cord, where it’s called a spinal nerve, or the brain, where it’s called a cranial nerve.

Myelin is the protective sheath that surrounds the axons of the peripheral neurons, allowing them to quickly send electrical impulses.

This myelin is produced by Schwann cells, which are a group of cells that support neurons.

In Guillain-Barré syndrome, demyelination happens when the immune system inappropriately attacks and destroys the myelin, which makes communication between neurons break down, ultimately leading to all sorts of sensory, motor, and cognitive problems.

The cause of Guillain- Barré syndrome is unknown, but it’s known to develop after a bacterial infection, like Campylobacter jejuni and Mycoplasma pneumoniae, or a viral infection, like cytomegalovirus and Epstein-Barr virus.

Specifically, some strains of Campylobacter jejuni have a particular kind of oligosaccharides on their membrane, that are identical to gangliosides found on the surface of motor neurons.

So as a result, immune cells mistakenly attack and destroy the gangliosides, damaging the neurons.

This is called molecular mimicry, because from the perspective of the immune cells, a host substance is mimicking a foreign protein.

Fuentes

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Guillain–Barré syndrome: pathogenesis, diagnosis, treatment and prognosis" Nature Reviews Neurology (2014)
  6. "Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome" The Lancet Neurology (2008)
  7. "Guillain–Barré Syndrome" New England Journal of Medicine (2012)
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