SÍNDROME DE GUILLAIN BARRÉ (Video Aula) - Rogério Souza

Summary

This video provides an in-depth overview of Guillain-Barré Syndrome (GBS), a rare autoimmune condition causing rapid muscle weakness due to nerve damage. The instructor, Rogério Souza, explains the disease's clinical presentation, pathophysiology, and treatment options. He highlights the progressive, symmetrical nature of GBS, the typical symptoms such as progressive paresthesia, paralysis, and respiratory failure, and the diagnostic methods. The video also explores the disease's historical discovery, its association with infections, and the importance of early diagnosis and specialized physiotherapy for recovery. Additionally, he touches on severe cases like Landry's paralysis and GBS-related autonomic dysfunctions.

Takeaways

• 😀 Guillain-Barré Syndrome (GBS) is an acute inflammatory polyneuropathy that primarily affects motor, sensory, and autonomic nerves, with symptoms progressing from distal to proximal areas of the body.
• 😀 The disease was first described in 1916 by Georges Guillain, Jean Alexandre Barré, and André Stöl, but earlier similar cases were noted by Jean-Baptiste Landré.
• 😀 GBS is the leading cause of acute flaccid paralysis globally, occurring in both children and adults, with an incidence of 1-2 cases per 100,000 people worldwide.
• 😀 The condition is triggered by infections, especially Campylobacter jejuni, and is associated with autoimmune mechanisms where the body's immune system attacks its own nerves.
• 😀 Symptoms of GBS include progressive tingling (paresthesia), muscle weakness (paresis), or complete paralysis (plegia), along with absent or diminished reflexes (hyporeflexia).
• 😀 Autonomic dysfunction in GBS can lead to issues like blood pressure fluctuations, arrhythmias, gastrointestinal disturbances, and respiratory failure due to diaphragm paralysis.
• 😀 Diagnosis of GBS is clinical and supported by tests like electroneuromyography, which shows slowed nerve conduction, and lumbar puncture, which reveals high protein levels in cerebrospinal fluid without an increase in white blood cells.
• 😀 The pathophysiology involves autoimmune attacks on the myelin sheath or axons of nerves, causing demyelination or axonal damage, often triggered by molecular mimicry following infections.
• 😀 Treatment for GBS includes intravenous immunoglobulin (IVIG), plasmapheresis, corticosteroids, and specialized physical therapy to stimulate recovery and nerve regeneration.
• 😀 The prognosis for GBS is generally good with early diagnosis and treatment, though severe cases may lead to more significant long-term disability. The syndrome can also present in more severe forms, such as Landré's variant, with worse outcomes.

Q & A

What is Guillain-Barré Syndrome?

Guillain-Barré Syndrome is an acute inflammatory polyneuropathy, characterized by demyelination, which affects the peripheral nervous system. It primarily causes motor, sensory, and autonomic dysfunction, and the symptoms typically start distally, in the feet and hands, progressing proximally.

What are the main clinical features of Guillain-Barré Syndrome?

The key clinical features of Guillain-Barré Syndrome include progressive, symmetrical muscle weakness, sensory changes such as paresthesia (tingling), reduced or absent reflexes, and in some cases, respiratory failure, dysphagia, and autonomic dysfunction.

Who discovered Guillain-Barré Syndrome and when?

Guillain-Barré Syndrome was described in 1916 by Georges Guillain, Jean Alexandre Barré, and André Strohl. The disease is named after Guillain and Barré.

What is the difference between Guillain-Barré Syndrome and Landry's Paralysis?

Landry's Paralysis, described by Jean Baptiste Landry in the 19th century, is a more severe form of what is now known as Guillain-Barré Syndrome. The main difference is that Landry did not perform lumbar puncture, which was not available at the time, and it involved fatal progressive weakness affecting all four limbs, including respiratory muscles.

What are the potential causes of Guillain-Barré Syndrome?

Guillain-Barré Syndrome is an autoimmune disorder that can be triggered by bacterial or viral infections, such as Campylobacter jejuni infection. It can also occur after certain vaccinations, though these cases are rare.

How does Guillain-Barré Syndrome affect the nervous system?

In Guillain-Barré Syndrome, the immune system mistakenly attacks the peripheral nervous system. Autoantibodies target the myelin sheath and sometimes the axons of nerves, leading to nerve dysfunction, motor weakness, and sensory changes. This process is called molecular mimicry.

What is the role of lumbar puncture in diagnosing Guillain-Barré Syndrome?

Lumbar puncture is essential for diagnosing Guillain-Barré Syndrome as it typically reveals elevated protein levels with a normal white blood cell count, a phenomenon known as albuminocytologic dissociation. This finding helps distinguish Guillain-Barré from other conditions.

What are some common complications of Guillain-Barré Syndrome if not treated properly?

If left untreated, Guillain-Barré Syndrome can lead to complications such as respiratory failure due to diaphragm paralysis, permanent disability, pressure sores, deformities, muscle contractures, and autonomic dysfunction.

What are the different types of Guillain-Barré Syndrome?

Guillain-Barré Syndrome can manifest in three main forms: a purely demyelinating form, an axonal form, and a mixed form involving both demyelination and axonal damage. A specific variant, called Miller Fisher Syndrome, is characterized by ocular palsy, ataxia, and areflexia.

What is the typical treatment for Guillain-Barré Syndrome?

Treatment for Guillain-Barré Syndrome includes intravenous immunoglobulin therapy (IVIG), plasma exchange (plasmaferesis), and corticosteroids. Physical therapy is also crucial for recovery, especially to promote nerve regeneration and prevent complications.