Landry-Guillain-Barre-Strohl syndrome

Landry-Guillain-Barr -Strohl Syndrome Landry-Guillain-Barr -Strohl syndrome, commonly known as Guillain-Barr syndrome (GBS), is a rare neurological disorder in which the body's immune system mistakenly attacks the peripheral nerves. This condition can lead to muscle weakness, numbness, and even paralysis. The syndrome is named after the French physicians Jean-Baptiste-Octave Landry, Georges Guillain, and Jean Alexandre Barr , who first described the condition in the early 20th century.

Clinical Features

The primary symptoms of Landry-Guillain-Barr -Strohl syndrome include:

• Muscle Weakness: Typically begins in the legs and can ascend to the arms and face.
• Numbness and Tingling: Often starts in the toes and fingers.
• Paralysis: In severe cases, paralysis can occur, affecting breathing and requiring mechanical ventilation.
• Autonomic Dysfunction: May include fluctuations in blood pressure, heart rate irregularities, and difficulties with bladder control.

Pathophysiology

The exact cause of Landry-Guillain-Barr -Strohl syndrome is not fully understood, but it is believed to be an autoimmune disorder. The immune system attacks the myelin sheath of peripheral nerves, leading to nerve damage. This can be triggered by infections, such as Campylobacter jejuni or Cytomegalovirus, and occasionally by vaccinations or surgery.

Diagnosis

Diagnosis of Landry-Guillain-Barr -Strohl syndrome is based on clinical examination and supportive tests, including:

• Lumbar Puncture: Elevated protein levels in cerebrospinal fluid with normal white blood cell count.
• Electromyography (EMG): Shows nerve conduction slowing or blockage.
• Nerve Conduction Studies (NCS): Demonstrates demyelination of peripheral nerves.

Treatment

Treatment for Landry-Guillain-Barr -Strohl syndrome focuses on reducing the severity of symptoms and speeding recovery. Common treatments include:

• Plasmapheresis: Removes antibodies from the blood that are attacking the nerves.
• Intravenous Immunoglobulin (IVIG): Provides the body with normal antibodies to counteract the immune attack.
• Supportive Care: Includes physical therapy, pain management, and respiratory support if needed.

Prognosis

The prognosis for individuals with Landry-Guillain-Barr -Strohl syndrome varies. Most patients recover fully, although some may experience lingering weakness or fatigue. The recovery period can range from weeks to years, and early treatment is associated with better outcomes.

Epidemiology

Landry-Guillain-Barr -Strohl syndrome is rare, with an incidence of approximately 1-2 cases per 100,000 people per year. It can affect individuals of any age, but it is more common in adults and slightly more prevalent in males.

See Also

• Autoimmune Disorders
• Peripheral Neuropathy
• Myelin Sheath
• Asbury, A. K., & Cornblath, D. R. (1990). Assessment of current diagnostic criteria for Guillain-Barr syndrome. Annals of Neurology, 27(S1), S21-S24.
• Hughes, R. A., & Cornblath, D. R. (2005). Guillain-Barr syndrome. The Lancet, 366(9497), 1653-1666.

NIH genetic and rare disease info

• NIH info on Landry-Guillain-Barre-Strohl syndrome

Landry-Guillain-Barre-Strohl syndrome is a rare disease.