Decreased or absent production of tears, saliva, and sweat

Introduction Acute total autonomic dysfunction is more common in children and adults. It is characterized by extensive or incomplete paralysis of the autonomic nerves, such as blurred vision, abnormal pupillary response to light and regulation, large pupils, and decreased or disappeared secretion of tears, saliva and sweat. And urinary retention, impotence, gastrointestinal dysfunction (nausea, vomiting, constipation, bloating or diarrhea) and abnormal body temperature regulation. The cause of this symptom is unclear, and there is currently a tendency to believe that the disease is associated with an abnormal immune response following viral infection.

Cause

(1) Causes of the disease

The cause is not yet clear. It has been found that this disease occurs after infectious mononucleosis and dysentery, and in some cases, the antibody titer of Epstein-Barr virus is increased. Some people think that this disease is an autoimmune autonomic neuropathy, similar to a neurotropic virus infection, but it is currently considered to be an abnormal immune response to autonomic ganglia or postganglionic nerve fibers.

(two) pathogenesis

There is currently a tendency to believe that the disease is associated with an abnormal immune response following viral infection.

1. Peripheral nerve autoimmune dysfunction: Cochlear nerve biopsy at home and abroad shows that the myelin fiber and unmyelinated fiber are significantly reduced in this disease, accompanied by axonal degeneration, the lesion mainly occurs in small fibers, and inflammatory demyelination The sheath performed consistently. Some patients with peripheral nerve sensation, dyskinesia, cerebrospinal fluid protein quantitative increase, normal cell number, good prognosis. Therefore, some people think that this disease is a special type of Guillain-Barre syndrome.

2. Viral infection: In recent years, the direct invasion of autonomic ganglia and post-ganglionic fibers by certain neurotropic viruses has been found to be an important mechanism leading to this disease. Besnard et al (2000) reported that a patient with intestinal obstruction as the main manifestation was positive for EB virus in hemorrhage and cerebrospinal fluid by PCR, and further detected by RNA probe in situ hybridization in the small intestine muscle layer, mesenteric lymph node and stomach. Department of Epstein-Barr virus.

Pathological changes of the disease: mainly occurs in the peripheral or central autonomic nervous system, directly affecting the function of the peripheral ganglia or postganglionic sympathetic and parasympathetic nerves. It is characterized by the disappearance of neurons, the proliferation of collagen fibers in the nodules, demyelination, and axonal degeneration. The sural nerve biopsy has no abnormality during the onset of the disease, but patients with autonomic dysfunction still exist after several years of onset. It can be seen that the number of unmyelinated nerve fibers is increased compared with normal, accompanied by regenerative changes.

an examination

Related inspection

EEG examination, lacrimal duct imaging, lacrimal examination, lacrimal gland examination

Laboratory examination

Cerebrospinal fluid routine examination showed increased protein.

2. Other auxiliary inspections

(1) X-ray examination has gastrointestinal muscle tension relaxation.

(2) The sweating reaction of pilocarpine subcutaneous injection test is often weakened.

(3) Eye drops are often caused by eye drops of 2.5% methacholine (acetylcholine), while intradermal injection of histamine is normal.

Differential diagnosis

Should be identified with Guillain-Barre syndrome, diabetes, or alcoholic neuropathy, familial autonomic dysfunction.

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