Q 2.8. Acute and chronic inflammatory demyelinating polyradiculoneuropathy (Guillen-Barre syndrome). Treatment. Gloria’s Battle with Guillain-Barré Syndrome

Q 2.8. Acute and chronic inflammatory demyelinating polyradiculoneuropathy (Guillen-Barre syndrome). Treatment. Gloria’s Battle with Guillain-Barré Syndrome

Gloria’s Battle with Guillain-Barré Syndrome

Gloria, a 30-year-old woman, had been recovering from a severe viral infection in her upper respiratory tract about a month ago. She thought she was on the mend, but then something strange happened. Two days ago, she began experiencing tingling sensations in her legs. It felt like pins and needles, and she dismissed it as a passing discomfort.

 

However, things took a sudden turn. Within 12 hours, the strange sensations spread to her arms as well. Gloria became alarmed when she noticed weakness setting in. Her legs felt heavy, and she struggled to walk. By the next day, she couldn’t even get out of bed. Her arms were equally weak, and she described the pain in all her extremities as excruciating.

 

Gloria’s worried family rushed her to the hospital. After a thorough examination and some tests, the doctors suspected Guillain-Barré Syndrome (GBS). Here’s what they explained to her:

 

What is GBS?

  • GBS is an autoimmune disorder where the immune system mistakenly attacks the peripheral nerves.

  • It often follows an infection, like Gloria’s recent viral illness.

  • The immune response damages the myelin sheath around nerves, leading to weakness and paralysis.

 

Symptoms:

  • Tingling and numbness: Gloria’s initial sensations in her legs.

  • Muscle weakness: Progressing rapidly, affecting both legs and arms.

  • Pain: The intense discomfort she felt in all her limbs.

 

Neurological Examination Findings:

The doctors performed a thorough neurological examination to pinpoint the issue. Here’s what they observed:

  • Lower Motor Neuron Syndrome. Gloria exhibited signs consistent with a lower motor neuron syndrome:

    • Diminished Reflexes: Her deep tendon reflexes (like the knee jerk) were absent or significantly reduced.

    • Flaccid Paralysis: Her muscle tone was decreased, leading to weakness and limpness.

 

  • Weakness Distribution: The weakness started in her legs and progressed to her arms, indicating a symmetrical pattern. This bilateral involvement is typical of GBS.

 

Diagnosing Guillain-Barré Syndrome (GBS)

 

Clinical Assessment:

  • The diagnosis of GBS begins with a thorough clinical evaluation by a healthcare provider.

  • The doctor will take a detailed medical history and perform a physical examination to assess symptoms, reflexes, and muscle strength.

 

Laboratory Tests:

  • Spinal Tap (Lumbar Puncture):

    • A small amount of fluid is withdrawn from the spinal canal in the lower back.

    • The cerebrospinal fluid (CSF) obtained is tested for specific changes that commonly occur in people with GBS.

    • Findings: Elevated protein levels without pleocytosis (an increase in white blood cells), which is a non-specific finding seen in many conditions that mimic GBS.

 

  • Electromyography (EMG) and Nerve Conduction Studies Key findings include:
    • Prolonged distal latencies.

    • Slowed motor and sensory nerve conduction velocities.

    • Temporal dispersion of waveforms.

    • Decreased SNAP (sensory nerve action potential) and CMAP (compound muscle action potential) amplitudes.

    • Prolonged or absent F-waves (indicating demyelination).

    • Reduced recruitment and rapid firing of motor units in weak muscles (early EMG findings).

! Note that EMG abnormalities may take 3-4 weeks to develop and can be normal in acute presentations.

Imaging Studies:

  • MRI of the Spine:

    • Used to exclude other causes of symptoms and evaluate the spinal cord and nerve roots.

    • Contrast-enhanced MRI is essential if GBS is suspected, as non-contrast sequences are essentially normal.

 

Treatment:

There is no cure for GBS, but two treatments can help speed recovery and reduce severity:

  • Plasma Exchange (Plasmapheresis):

    • Removes plasma (liquid portion of blood) and separates it from blood cells.

    • Blood cells are then returned to the body, and the body manufactures more plasma.

    • Plasmapheresis may remove harmful antibodies contributing to the immune attack on peripheral nerves.

  • Immunoglobulin Therapy:

    • Intravenous injections of immunoglobulins (healthy antibodies from blood donors).

    • High doses can block damaging antibodies associated with GBS.

  • Medications are given to relieve pain and prevent blood clots.

  • Physical therapy and training with adaptive devices are crucial during recovery.

 

Prognosis:

Recovery from GBS varies, but most people experience this general timeline:

  • Progressive worsening of symptoms for about two weeks.

  • Symptoms plateau within four weeks.

  • Recovery begins, lasting 6 to 12 months (sometimes up to three years).

 

Gloria’s prognosis depends on the severity. Most people recover fully, but it can take weeks to months. Some may have lingering weakness or fatigue. Fortunately, with prompt treatment, Gloria has a good chance of regaining her strength and mobility.

References: 

(1) Guillain-Barre syndrome – Diagnosis and treatment – Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/guillain-barre-syndrome/diagnosis-treatment/drc-20363006.

(2) Guillain-Barré syndrome – Diagnosis – NHS. https://www.nhs.uk/conditions/guillain-barre-syndrome/diagnosis/.

(3) Guillain-Barré syndrome | Radiology Reference Article – Radiopaedia.org. https://radiopaedia.org/articles/guillain-barre-syndrome-2.

(4) Diagnosis of Guillain-Barré syndrome and what are its different treatment options?. https://www.msn.com/en-us/health/condition/Guillain-Barré-syndrome/hp-Guillain-Barré-syndrome?source=conditioncdx.

(5) Guillain-Barre Syndrome in Children – Children’s Hospital of Philadelphia. https://www.chop.edu/conditions-diseases/guillain-barre-syndrome-children.

(6) Guillain-Barré Syndrome | Cedars-Sinai. https://www.cedars-sinai.org/health-library/diseases-and-conditions/g/guillain-barre-syndrome.html.

(7) How to diagnose Guillain-Barre syndrome | Medmastery. https://www.medmastery.com/guides/neurology-diseases-clinical-guide/how-diagnose-guillain-barre-syndrome.

(8) Guillain-Barré syndrome laboratory tests – wikidoc. https://www.wikidoc.org/index.php/Guillain-Barr%C3%A9_syndrome_laboratory_tests.

(9) Guillain-Barré Syndrome | American Journal of Neuroradiology. https://bing.com/search?q=guillain+barre+syndrome+imaging.

(10) How Doctors Diagnose Guillain-Barré Syndrome – Verywell Health. https://www.verywellhealth.com/how-doctors-diagnose-guillain-barre-syndrome-2488657.

(11) Guillain-Barré Syndrome | American Journal of Neuroradiology. https://www.ajnr.org/ajnr-case-collections-diagnosis/guillain-barr%C3%A9-syndrome.

Verified by Dr. Petya Stefanova