Harlequin syndrome

Harlequin syndrome
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Flushing on one side of the face
Complications	N/A
Onset	N/A
Duration	N/A
Types	N/A
Causes	Autonomic nervous system dysfunction
Risks	N/A
Diagnosis	N/A
Differential diagnosis	N/A
Prevention	N/A
Treatment	Symptomatic treatment
Medication	N/A
Prognosis	N/A
Frequency	N/A
Deaths	N/A

Harlequin syndrome is a rare neurological disorder characterized by unilateral flushing and sweating of the face. This condition is caused by a dysfunction of the autonomic nervous system, specifically affecting the sympathetic nervous system.

Presentation

Individuals with Harlequin syndrome typically present with asymmetrical flushing and sweating, which occurs on one side of the face, neck, and sometimes the upper chest. This phenomenon is often triggered by exercise, heat, or emotional stress. The unaffected side of the face remains pale and dry.

Causes

Harlequin syndrome is believed to result from damage or dysfunction of the sympathetic nervous system pathways that control sweating and blood vessel dilation. This can occur due to various reasons, including:

• Surgical procedures involving the thoracic region
• Trauma
• Tumors affecting the sympathetic chain
• Idiopathic causes, where no specific cause is identified

Diagnosis

The diagnosis of Harlequin syndrome is primarily clinical, based on the characteristic presentation of unilateral flushing and sweating. Additional tests, such as thermoregulatory sweat testing or imaging studies, may be conducted to rule out other conditions or identify underlying causes.

Treatment

Treatment for Harlequin syndrome is generally symptomatic. In cases where the condition is caused by an identifiable underlying issue, addressing that issue may alleviate symptoms. For example, surgical removal of a tumor may resolve the syndrome. In other cases, beta-blockers or other medications may be used to manage symptoms.

Prognosis

The prognosis for individuals with Harlequin syndrome varies depending on the underlying cause. In many cases, the condition is benign and does not significantly impact the individual's quality of life.

See also

• Autonomic nervous system disorders
• Horner's syndrome
• Ross syndrome

External links

• Article on Harlequin syndrome