Riley-Day syndrome

Overview also known as familial nerve disorder, a kind of congenital anomaly syndrome feeling. Cause pathogenesis is still unknown. Implicit an autosomal genetic. Possible to the nervous system, autonomic nervous system, especially congenital dysfunction. Clinical features of a variety of autonomic dysfunction, no tear, abnormal sweating, skin erythema, dysphagia, Sporadic high fever and tongue Cao mushroom equipment such as nipple missing. Nearly all occurred in Ashkenazic Jews. When he was unable to shed tears, cornea pain receded, the lack of taste, intramuscular activities incompatible, swallowing and dysarthria. Orthostatic hypotension, hypertension, can not explain the fever, emotional instability, abnormal sweating, cyclical vomiting. often a lung infection. 50% of patients with corneal ulcer, tongue and the lack of Lei Ye fungiform papilla, spinal kyphosis, occasionally Charcoat joints, Romberg's patients. Patients with percutaneous injection of histamine often without feeling no pain reaction; Right norepinephrine sensitive, Few intravenous infusion can cause severe hypertension; A 2.5% acetyl choline, Mao lye pilocarpine eye drops caused retinoscopy disorders, and the right to a renal gland-pupil reaction was normal. Urinary homovanillic acid and vanillylmandelic acid was significantly increased. Differential diagnosis (1) acute self neuropathy (acute autonomous neuropathy ) acute onset, the clinical manifestations of blurred vision, pupillary light reflex and regulate abnormal sweating less, no tears, Positional hypotension, urinary retention, and so on. Most cases in a few months or a few weeks after the resumption of their own. A 2.5% acetyl choline Drip often caused Miosis, percutaneous injection of histamine after normal reaction. (2) Sjogree's syndrome characterized by tears, saliva secretion was decreased drying performance for keratitis, mouth dry, chapped mucosa, parotid swelling, associated with rheumatoid arthritis, and the skin dry and sweat-free, and the lack of acid, hepatosplenomegaly, and so on.