pure autonomic failure
Introduction
Introduction to pure autonomic failure Pure autonomic failure (PAF) is a widespread loss of isolated autonomic function, primarily impairing sympathetic ganglia neurons. Autonomic dysfunction is caused by dysfunction of the visceral function of the patient, resulting in serious physical discomfort, serious harm to the patient's physical and mental health, and seriously affecting the normal work and life of the patient. basic knowledge The proportion of illness: 0.0003% Susceptible people: no specific people Mode of infection: non-infectious Complications: cough, myasthenia gravis
Cause
Cause of pure autonomic failure
Posterior membrane acetylcholine receptor lesions (45%)
Ultrastructural studies have found that the disease is mainly caused by lesions of the postsynaptic membrane acetylcholine receptor (AChR). AChR lesions cause post-sympathetic ganglion neuronal damage. Lead to pure autonomic nervous function, which induces the disease.
Immune mechanism disorder (25%)
There are no other pathological changes and infection factors or neuroinflammation. The antibodies produced by the autoimmune system cross-immunize with the nervous tissues, causing damage to the neurons after sympathetic ganglia, leading to pure autonomic nerve failure, thereby inducing the disease.
Prevention
Pure autonomic nervous failure prevention
It is recommended to avoid taking cola, coffee, chocolate, wine, tea, regular diet before going to bed, and pay attention to improve the sleeping environment, which is beneficial to reduce the excitement of the brain. You can take oral oryzanol tablets + vitamin B1 tablets + Anshen Bunao for conditioning, sleep difficulties Oral: Decoction tablets. Pay attention to the light diet, regular quantitative, and eat less foods with high cholesterol.
Complication
Pure autonomic failure Complications, cough, myasthenia gravis
Myasthenia gravis is caused by disease progression and insufficient anticholinesterase drugs. Clinical manifestations of swallowing, coughing, respiratory distress, difficulty, and even stop. Physical examination showed dilated pupils, sweating, bloating, normal bowel sounds and improved symptoms after neostigmine injection.
Cholinergic crisis accounts for 1.0% to 6.0% of the number of crisis cases due to an excess of anticholinesterase. In addition to the common features of muscle weakness, the patient's pupils are narrowed, sweating, muscles are beating, bowel sounds are hyperthyroidism, and the symptoms of the muscles are increased.
The rumination crisis is caused by infection, poisoning and electrolyte imbalance, and can be temporarily relieved by the application of anti-cholinesterase drugs, followed by a critical state of aggravation.
Symptom
Pure autonomic failure symptoms Common symptoms Abdominal muscle weakness, cognitive neurological loss
Pure autonomic dysfunction is more common in middle-aged recessive onset, more women, no family history. The performance is the same as Shy-Drager syndrome, and early manifestations are mostly paroxysmal syncope. Sexual erectile dysfunction is a prominent early manifestation. Orthostatic hypotension is an early and significant sign. When changing from a supine position to a sitting position or an upright position, the blood pressure systolic blood pressure is reduced by 2.7 kPa (20 mmHg) or the diastolic blood pressure is decreased by 1.3 kPa (10 mmHg) or more. Involuntary heart rate increases. Cardiac autonomic dysfunction leads to arrhythmia or changes in cardiac output. Abnormal gastrointestinal function, diarrhea, constipation, abdominal pain or fecal incontinence. Urinary dysfunction includes urinary fistula, frequent urination, urgency or incontinence. Abnormal sweating. Abnormal pupil (small but lightly unresponsive). Bladder symptoms are relatively mild, with no brain and peripheral nerve damage. Although the symptoms can be quite severe, it does not seem to shorten life.
Examine
Pure autonomic failure test
Physical examination: Shenqing, Yuli, bilateral pupils, etc., with a diameter of 6.0 mm, disappeared from light reflex, and the whole body pain decreased; the upper limb muscle strength was 4, the lower limb muscle strength was 3, the limb muscle tension was low, and the tendon reflex disappeared. .
There was no abnormality in cervical spine MR and lumbar puncture. Cranial CT, MRI, no intracranial vascular lesions or loss.
Electromyography showed neurogenic damage to the upper and lower limbs.
Diagnosis
Diagnosis and diagnosis of pure autonomic nerve failure
Diagnosis can be based on medical history, clinical symptoms, and laboratory findings.
Similar to Shy-Drager (SDS), which has not yet developed brain symptoms, it is difficult to identify clinically. The plasma norepinephrine (NE) is normal in the SDS, the PAF is lower than normal, and the indirect sympathetic tyramine is in the SDS (SDS). Post-ganglionic sympathetic neurons are normal) cause NE release and elevated blood pressure, PAF is weakened by post-ganglionic neuronal damage, and the identification of autonomic failure with peripheral neuropathy is to find evidence of etiology and peripheral nerve damage.
