Freys syndrome (also known as Baillargers syndrome, Dupuys syndrome, auriculotemporal syndrome, 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.The symptoms of Freys 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. Observing sweating in the region after eating a lemon wedge may be diagnostic. Signs and symptoms
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". Causes
Freys 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.
Chloroquine retinopathy is a form of toxic retinopathy (damage of the retina) caused by the drugs chloroquine or hydroxychloroquine, which are sometimes used in the treatment of autoimmune disorders such as rheumatoid arthritis and systemic lupus erythematosus. This eye toxicity limits long-term use of the drugs. Presentation
The risk of toxicity is low for individuals without complicating conditions during the first five years of treatment using less than 6.5 mg/kg/day of hydroxychloroquine or 3 mg/kg/day of chloroquine, and/or cumulative doses of less than 1000 gram and 460 gram (total dose), respectively. Some physicians suggest that lean body weight is more accurate when calculating daily dosage.Most patients are routinely given 400 mg of hydroxychloroquine daily (or 250 mg chloroquine). This dose is considered acceptable. Early stage
The earliest signs of toxicity include bilateral paracentral visual field changes (best detected with a red test object) and a subtle granular depigmentation of the paracentral RPE. Advanced stage
With continued drug exposure, there is progressive development of a bilateral atrophic bulls-eye maculopathy and paracentral scotomata, which may in severe cases ultimately spread over the entire fundus, causing widespread retinal atrophy and visual loss. Pathophysiology
Both agents bind to melanin pigment in the RPE, and this may serve to concentrate the drugs or to prolong their adverse effects. Diagnosis
Patients and their primary care physicians must be made fully aware of the ophthalmic risks and the need for regular screening examinations to detect retinal toxicity at an early stage.