Flammer syndrome

Flammer syndrome
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Flammer syndrome is a recently described clinical entity comprising a complex of clinical features caused mainly by dysregulation of the blood supply which has previously been called vascular dysregulation. It can manifest itself in many symptoms such as cold hands and feet and is often associated with low blood pressure. In certain cases it is associated with or predisposes for the development of diseases such as a normal tension glaucoma. Flammer syndrome is named after the Swiss ophthalmologist Josef Flammer.

Most of the symptoms of Flammer syndrome result from an impaired regulation of the blood supply. How these symptoms affect the patient depends on which organ's or body part's blood supply is inhibited. Typical symptoms of Flammer syndrome are cold hands or feet, a low blood pressure, occasional white and red patches on the face or neck, and migraine-like pain or a feeling of pressure behind the upper eyelid. In addition, there are symptoms not directly resulting from dysregulations of the blood supply such as a prolonged time needed to fall asleep, a reduced feeling of thirst, high sensitivity not only to cold but also to odors, vibrations, psychological stress or certain medications (e.g., calcium antagonists, beta blockers) etc. Pain and muscle spasms are common. People with Flammer syndrome are usually very precise, highly motivated and successful in their professional life.

Scientific studies have shown that in many patients with glaucoma the cause of the disease are perfusion issues of the optic nerve and retina while intraocular pressure is normal. Many patients with normal tension glaucoma have a basic constitution, which corresponds to the Flammer syndrome. Some of its features were formerly summarized under the term "primary vascular dysregulation syndrome".

Signs of Flammer syndrome may include:

People with Flammer syndrome typically respond excessively to mental or physical stimuli such as stress or exposure to cold. The disease is more prevalent in the following groups of people: in women compared to men, in people with lower blood pressure, in Asians compared with Caucasians, it is more frequent among academics than among blue collar-workers. People with Flammer syndrome are usually highly motivated in their professional life, they sleep poorly and they have a decreased sensation of thirst.

The diagnosis of Flammer's syndrome is primarily based on the patient's history with its typical features as well as the findings of nail fold capillaroscopy. This test shows an excessive constriction (narrowing) in the smallest blood vessels (capillaries) of the fingers in response to being exposed to cold as a stimulus. Patients with vasospastic symptoms as they are characteristic for Flammer syndrome have shown a unique gene expression when their lymphozytes underwent gene expression profiling.

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