Morvan's syndrome

Morvan's syndrome is a rare, life-threatening autoimmune disease first described by French physician Augustin Marie Morvan in 1890 as "La choree fibrillaire. The condition is characterized by multiple, irregular contractions of the long muscles, cramping, weakness, pruritus, hyperhidrosis, insomnia, and delirium. Morvan's syndrome typically presents with a slow insidious onset over months to years.

Signs and symptoms

Morvan's syndrome is characterized by the following signs and symptoms:

• Multiple irregular muscle contractions
• Cramping
• Weakness
• Pruritus (itching)
• Hyperhidrosis (excessive sweating)
• Insomnia
• Delirium

Causes

Morvan's syndrome is an autoimmune disease in which the body's immune system mistakenly attacks its own healthy tissues. The exact cause of the autoimmune reaction is still unknown, but it has been associated with the presence of antibodies against certain potassium channel proteins.

Diagnosis

Diagnosis of Morvan's syndrome is typically based on clinical presentation, with additional laboratory tests, electromyography, MRI, and cerebrospinal fluid analysis to confirm the diagnosis and rule out other conditions with similar symptoms. The presence of specific antibodies can also be tested in blood and cerebrospinal fluid samples.

Treatment

Treatment for Morvan's syndrome generally focuses on managing the autoimmune aspect of the disease. Common treatments include:

• Immunosuppressive therapy: Medications such as azathioprine, mycophenolate mofetil, or cyclosporine may be used to suppress the immune system and reduce inflammation.
• Corticosteroids: Prednisone or other corticosteroids may be used to reduce inflammation and help control symptoms.
• Intravenous immunoglobulin (IVIG): IVIG treatment can help to modulate the immune system and may be effective in treating Morvan's syndrome.
• Plasma exchange: This procedure involves removing harmful antibodies from the blood, which may help to reduce symptoms and improve the patient's condition.

Prognosis

The prognosis for Morvan's syndrome varies, with approximately 90% of cases spontaneously going into remission. However, the remaining 10% of cases can lead to death. The prognosis depends on the severity of the disease, the presence of complications, and the patient's response to treatment.

Epidemiology

Morvan's syndrome is a rare condition with few reported cases worldwide. Due to its rarity and the lack of large-scale epidemiological studies, the exact prevalence and incidence of the disease remain unknown.

History

Morvan's syndrome was first described by French physician Augustin Marie Morvan in 1890, when he observed patients presenting with multiple irregular contractions of the long muscles, cramping, weakness, pruritus, hyperhidrosis, insomnia, and delirium. He coined the term "La choree fibrillaire" to describe the condition.

Summary

Morvan's syndrome is a rare, life-threatening autoimmune disease named after the nineteenth century French physician Augustin Marie Morvan. La choree fibrillaire was first coined by Morvan in 1890 when describing patients with multiple, irregular contractions of the long muscles, cramping, weakness, pruritus, hyperhidrosis, insomnia, and delirium.It normally presents with a slow insidious onset over months to years.Approximately 90% of cases spontaneously go into remission, while the other 10% of cases lead to death.

See also

• Autoimmune disease
• Neuromyotonia
• Lambert-Eaton syndrome

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