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Frey's syndrome | |
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Other names | Auriculotemporal syndrome, Baillarger's syndrome, Dupuy’s syndrome, Frey-Baillarger syndrome |
Redness associated with Frey's syndrome | |
Specialty | Neurology |
Symptoms | Redness and sweating of cheek area when salivating |
Causes | Damage to auriculotemporal nerve |
Diagnostic method | Starch-iodine test |
Frequency | 30–50% (after parotidectomy) |
Frey's syndrome (also known as Baillarger's syndrome, Dupuy's syndrome, auriculotemporal syndrome, [1] or Frey-Baillarger syndrome) is a rare neurological disorder resulting from damage to or near the parotid glands responsible for making saliva, and from damage to the auriculotemporal nerve often from surgery. [1] [2]
The symptoms of Frey's syndrome are redness and sweating on the cheek area adjacent to the ear (see focal hyperhidrosis). They can appear when the affected person eats, sees, dreams, thinks about, or talks about certain kinds of food which produce strong salivation. [3] Observing sweating in the region after eating a lemon wedge may be diagnostic. [2]
Signs and symptoms include erythema (redness or flushing) and sweating in the cutaneous distribution of the auriculotemporal nerve, usually in response to gustatory stimuli. There is sometimes pain in the same area, often burning in nature. Between attacks of pain, there may be numbness or other altered sensations (anesthesia or paresthesia). This is sometimes termed "gustatory neuralgia".
Frey's syndrome often results as a complication of surgeries of or near the parotid gland or due to injury to the auriculotemporal nerve, which passes through the parotid gland in the early part of its course. The auriculotemporal branch of the mandibular branch (V3) of the trigeminal nerve carries parasympathetic fibers to the parotid salivary gland and sympathetic fibers to the sweat glands of the scalp. As a result of severance and inappropriate regeneration, the parasympathetic nerve fibers may switch course to a sympathetic response, resulting in "gustatory sweating" or sweating in anticipation of eating, instead of the normal salivary response. [2] It is often seen with patients who have undergone endoscopic thoracic sympathectomy, a surgical procedure wherein part of the sympathetic trunk is cut or clamped to treat sweating of the hands or blushing. The subsequent regeneration or nerve sprouting leads to abnormal sweating and salivation. It can also include discharge from the nose when smelling certain food.
Rarely, Frey's syndrome can result from causes other than surgery, including accidental trauma, local infections, sympathetic dysfunction, and pathologic lesions within the parotid gland. [4] An example of such rare trauma or localized infection can be seen in situations where a hair follicle has become ingrown and is causing trauma or localized infection near or over one of the branches of the auriculotemporal nerve.
Diagnosis is made based on clinical signs and symptoms and a starch-iodine test, also known as the Minor test. The affected area of the face is painted with iodine which is allowed to dry, then dry corn starch is applied to the face. The starch turns blue on exposure to iodine in the presence of sweat. [5]
Cochrane reviews of interventions to either prevent [7] or treat [8] Frey's syndrome have found little or no evidence to support their effectiveness or safety, and conclude that further clinical trials are needed.
The condition is rare, although the exact incidence is unknown. [9]
The disorder most often occurs as a complication of the surgical removal of a parotid gland (parotidectomy). The percentage of individuals who develop Frey syndrome after a parotidectomy is controversial and reported estimates range from 30 to 50 percent. In follow-up examinations, approximately 15 percent of affected individuals rated their symptoms as severe. Frey syndrome affects males and females in equal numbers.
It is named after Łucja Frey-Gottesman. [10] The disorder was first reported in medical literature by Baillarger in 1853. A neurologist from Poland, Dr. Lucja Frey, provided a detailed assessment of the disorder and coined the term "auriculotemporal syndrome" in 1923. [11]
The parasympathetic nervous system (PSNS) is one of the three divisions of the autonomic nervous system, the others being the sympathetic nervous system and the enteric nervous system. The enteric nervous system is sometimes considered part of the autonomic nervous system, and sometimes considered an independent system.
Saliva is an extracellular fluid produced and secreted by salivary glands in the mouth. In humans, saliva is around 99% water, plus electrolytes, mucus, white blood cells, epithelial cells, enzymes, and antimicrobial agents.
Dysgeusia, also known as parageusia, is a distortion of the sense of taste. Dysgeusia is also often associated with ageusia, which is the complete lack of taste, and hypogeusia, which is a decrease in taste sensitivity. An alteration in taste or smell may be a secondary process in various disease states, or it may be the primary symptom. The distortion in the sense of taste is the only symptom, and diagnosis is usually complicated since the sense of taste is tied together with other sensory systems. Common causes of dysgeusia include chemotherapy, asthma treatment with albuterol, and zinc deficiency. Liver disease, hypothyroidism, and rarely, certain types of seizures can also lead to dysgeusia. Different drugs can also be responsible for altering taste and resulting in dysgeusia. Due to the variety of causes of dysgeusia, there are many possible treatments that are effective in alleviating or terminating the symptoms. These include artificial saliva, pilocarpine, zinc supplementation, alterations in drug therapy, and alpha lipoic acid.
The salivary glands in many vertebrates including mammals are exocrine glands that produce saliva through a system of ducts. Humans have three paired major salivary glands, as well as hundreds of minor salivary glands. Salivary glands can be classified as serous, mucous, or seromucous (mixed).
The facial nerve, also known as the seventh cranial nerve, cranial nerve VII, or simply CN VII, is a cranial nerve that emerges from the pons of the brainstem, controls the muscles of facial expression, and functions in the conveyance of taste sensations from the anterior two-thirds of the tongue. The nerve typically travels from the pons through the facial canal in the temporal bone and exits the skull at the stylomastoid foramen. It arises from the brainstem from an area posterior to the cranial nerve VI and anterior to cranial nerve VIII.
The glossopharyngeal nerve, also known as the ninth cranial nerve, cranial nerve IX, or simply CN IX, is a cranial nerve that exits the brainstem from the sides of the upper medulla, just anterior to the vagus nerve. Being a mixed nerve (sensorimotor), it carries afferent sensory and efferent motor information. The motor division of the glossopharyngeal nerve is derived from the basal plate of the embryonic medulla oblongata, whereas the sensory division originates from the cranial neural crest.
The parotid gland is a major salivary gland in many animals. In humans, the two parotid glands are present on either side of the mouth and in front of both ears. They are the largest of the salivary glands. Each parotid is wrapped around the mandibular ramus, and secretes serous saliva through the parotid duct into the mouth, to facilitate mastication and swallowing and to begin the digestion of starches. There are also two other types of salivary glands; they are submandibular and sublingual glands. Sometimes accessory parotid glands are found close to the main parotid glands.
Endoscopic thoracic sympathectomy (ETS) is a surgical procedure in which a portion of the sympathetic nerve trunk in the thoracic region is destroyed. ETS is used to treat excessive sweating in certain parts of the body, facial blushing, Raynaud's disease and reflex sympathetic dystrophy. By far the most common complaint treated with ETS is sweaty palms. The intervention is controversial and illegal in some jurisdictions. Like any surgical procedure, it has risks; the endoscopic sympathetic block (ESB) procedure and those procedures that affect fewer nerves have lower risks.
Xerostomia, also known as dry mouth, is dryness in the mouth, which may be associated with a change in the composition of saliva, or reduced salivary flow, or have no identifiable cause.
Parotitis is an inflammation of one or both parotid glands, the major salivary glands located on either side of the face, in humans. The parotid gland is the salivary gland most commonly affected by inflammation.
The otic ganglion is a small parasympathetic ganglion located immediately below the foramen ovale in the infratemporal fossa and on the medial surface of the mandibular nerve. It is functionally associated with the glossopharyngeal nerve and innervates the parotid gland for salivation.
The auriculotemporal nerve is a sensory branch of the mandibular nerve (CN V3) that runs with the superficial temporal artery and vein, and provides sensory innervation to parts of the external ear, scalp, and temporomandibular joint. The nerve also conveys post-ganglionic parasympathetic fibres from the otic ganglion to the parotid gland.
Pleomorphic adenoma is a common benign salivary gland neoplasm characterised by neoplastic proliferation of epithelial (ductal) cells along with myoepithelial components, having a malignant potentiality. It is the most common type of salivary gland tumor and the most common tumor of the parotid gland. It derives its name from the architectural Pleomorphism seen by light microscopy. It is also known as "Mixed tumor, salivary gland type", which refers to its dual origin from epithelial and myoepithelial elements as opposed to its pleomorphic appearance.
The tympanic nerve is a branch of the glossopharyngeal nerve found near the ear. It gives sensation to the middle ear, the Eustachian tube, the parotid gland, and mastoid air cells. It gives parasympathetic to supply to the parotid gland via the otic ganglion and the auriculotemporal nerve.
Compensatory hyperhidrosis is a form of neuropathy. It is encountered in patients with myelopathy, thoracic disease, cerebrovascular disease, nerve trauma or after surgeries. The exact mechanism of the phenomenon is poorly understood. It is attributed to the perception in the hypothalamus (brain) that the body temperature is too high. The sweating is induced to reduce body heat.
Sialadenitis (sialoadenitis) is inflammation of salivary glands, usually the major ones, the most common being the parotid gland, followed by submandibular and sublingual glands. It should not be confused with sialadenosis (sialosis) which is a non-inflammatory enlargement of the major salivary glands.
The salivatory nuclei are two parasympathetic general visceral efferent cranial nerve nuclei - the superior salivatory nucleus and the inferior salivatory nucleus - that innervate the salivary glands. Both are located in the pontine tegmentum of the brainstem.
Łucja Frey or Łucja Frey-Gottesman was a Polish-Jewish physician and neurologist, known for describing the syndrome later named after her. She was one of the first female academic neurologists in Europe. Frey perished during the Holocaust in 1942 in Lwów ghetto aged 53.
Salivary gland diseases (SGDs) are multiple and varied in cause. There are three paired major salivary glands in humans: the parotid glands, the submandibular glands, and the sublingual glands. There are also about 800–1,000 minor salivary glands in the mucosa of the mouth. The parotid glands are in front of the ears, one on side, and secrete mostly serous saliva, via the parotid ducts, into the mouth, usually opening roughly opposite the second upper molars. The submandibular gland is medial to the angle of the mandible, and it drains its mixture of serous and mucous saliva via the submandibular duct into the mouth, usually opening in a punctum in the floor of mouth. The sublingual gland is below the tongue, on the floor of the mouth; it drains its mostly mucous saliva into the mouth via about 8–20 ducts, which open along the plica sublingualis, a fold of tissue under the tongue.
A parotidectomy is the surgical excision (removal) of the parotid gland, the major and largest of the salivary glands. The procedure is most typically performed due to neoplasms (tumors), which are growths of rapidly and abnormally dividing cells. Neoplasms can be benign (non-cancerous) or malignant (cancerous). The majority of parotid gland tumors are benign, however 20% of parotid tumors are found to be malignant. Parotidectomy is performed mostly by oral and maxillofacial surgeon and ENT surgeon.