Shay-Drager syndrome
Introduction
Introduction to Xiay-Dreig Syndrome Schei-Dragger Syndrome (SDS), also known as progressive autonomic failure, idiopathic orthostatic hypotension, neurogenic orthostatic hypotension, was first introduced by Shy and Drager in 1960. Reported autopsy cases, the idea is that the disease is a neurodegenerative disease. The disease is a rare disease of extensive degeneration of the central nervous system including the autonomic nervous system. The patients are sporadic. The clinical manifestations include dyspareunia, impotence, other autonomic dysfunctions, and other autonomic dysfunctions. Cone system, extrapyramidal, cerebellar ataxia and other symptoms of the somatic nervous system. Some people think that OPCA is accompanied by autonomic symptoms such as orthostatic hypotension, dizziness or syncope, impotence, incontinence, etc. It is considered to be the Shay-Dreig syndrome, which is currently considered to be a type of multiple system atrophy (MSA). basic knowledge The proportion of illness: 0.001% - 0.005% Susceptible people: the elderly Mode of infection: non-infectious Complications: impotence, no sweating
Cause
The cause of Xiay-Dreig syndrome
Dysfunction (30%):
It is thought to be due to degeneration of the central nervous system or the surrounding autonomic nervous system, which in turn leads to dysfunction of the central or peripheral autonomic nervous system.
Conduction function defects (30%):
It has been reported that the conduction function of the system is deficient, which makes the synthesis of the norepinephrine insufficient. Some authors point out that the metabolic disorder of catecholamines forms the basis of autonomic and extrapyramidal diseases, and it is found that dopa decarboxylase and high vanillic acid are also reduced.
Sensory aberrations (25%):
It has also been reported that baroreceptors in the lower limbs and visceral blood vessel walls are dysfunctional, resulting in a small arterial reflex contraction disorder and a decrease in venous return flow.
Pathological changes: chronic autonomic nervous system dysfunction or with somatic neurological dysfunction, manifesting multiple system atrophy (MSA); changes in central and peripheral nervous systems found in autopsy are not consistent, in autonomic ganglia Changes in the nucleus of the spinal cord, brainstem, cerebellum, cortex, and basal ganglia, especially in the dorsal and substantia nigra of the putamen, the locus nucleus, the lower olive nucleus, and the dorsal vagus nucleus obvious.
The above changes indicate that the autonomic function may be changed first, resulting in repeated episodes of hypotension, resulting in cerebral hypoxia, and pathological changes in the nervous tissue are secondary to hypoxia.
Prevention
Shay-Dreiger Syndrome Prevention
Pay attention to nutrition, enhance physical fitness, take general strong agents and various vitamins, or apply appropriate Chinese medicine such as Buzhong Yiqi Pills, or Shengmai Powder to increase or decrease treatment, and appropriately strengthen physical exercise.
Complication
Complications of Xiay-Dreig syndrome Complications, impotence, no sweat
Often accompanied by impotence, no sweat, and bladder rectal dysfunction, or cerebellum, extrapyramidal dysfunction.
Symptom
Symptoms of Xiay-Dreig's Syndrome Common Symptoms Constipation Diarrhea Sweating Abnormal Eyes Dizziness Reflex Hyperthyroidism Sputum Urinary Disorder Defecation Disorder Ataxia
The disease is a multi-system-affected degenerative disease characterized by autonomic neurological symptoms. It is clinically characterized by orthostatic hypotension and other autonomic dysfunction, accompanied by degeneration of the cerebellum, basal ganglia or spinal motor neurons. Nerve abnormalities.
The onset of the disease can be insidious, from several months to several years, the elderly can reach more than 10 years, from the middle-aged and old age, the onset age is 55 years old, about 65% are male, no family history, the course of disease is progressive, Autonomic symptoms often appear first, with somatic neurological symptoms in the months or years, and a few patients with somatic neurological symptoms earlier than autonomic symptoms.
Upright hypotension
Early clinical manifestations include dizziness when standing up suddenly or standing for too long. Patients often have blurred vision and fatigue. Patients often see erectile syncope. Due to extensive degeneration of autonomic nerves, baroreflex arcs are blocked. When standing upright Can not produce reflex heart rate and peripheral arterioles contraction, and the emergence of orthostatic hypotension, orthostatic hypotension is the most common symptoms, normal blood pressure in the supine position, but also higher than normal, blood pressure decreased significantly when standing up [systolic blood pressure decreased more than 4.0 ~6.7kPa (3050mmHg)], the standing and lying blood pressure often differs by 4.0/2.67kPa (30/20mmHg) within 2min. Because the sympathetic tone is low, the patient's standing pulse rate does not change, no pale, nausea Symptoms such as vomiting and sweating, according to statistics, accounted for 11% of patients with orthostatic hypotension.
2. Other autonomic dysfunction
Such as the bladder, rectal sphincter disorders cause urination, defecation disorders, including frequent urination, urgency, urinary retention or incontinence, diarrhea and constipation alternate, sexual desire disorder can occur several years before the appearance of other nervous system symptoms, sweating abnormalities, can be Excessive sweating, followed by less sweat or dry skin, no sweat, in addition to rare skin temperature abnormalities, Horner syndrome, iris atrophy, etc., the disease mostly begins with the medulla, gradually develops upwards, so the order of symptoms is first Impotence, loss of libido, dysuria, dizziness, vertigo, syncope, orthostatic hypotension and other autonomic dysfunction symptoms, and finally cerebellar ataxia, brain stem damage symptoms and pyramidal tract.
3. Cerebellar dysfunction such as intentional tremor
Limbs, torso ataxia, poor rotation, nystagmus, unclear speech, dysarthria.
The three main signs of orthostatic hypotension, autonomic dysfunction and cerebellar symptoms are clinical core symptoms.
It can also be combined with abnormalities of the somatic nerve caused by degeneration of extrapyramidal, basal ganglia or spinal motor neurons. Later, there may be pyramidal tract damage, such as increased muscle tone, hyperreflexia, pathological signs, etc.; Muscular atrophy and fasciculation, EMG shows degenerative changes of anterior horn cells; Parkinson's syndrome can also be manifested by substantia nigra, or cranial nerve spasm.
Laryngeal stridor occurs at night due to weak laryngeal muscle strength and may be accompanied by apnea. A considerable number of patients have difficulty swallowing. In advanced stage, due to autonomic nervous function, they may have apnea during sleep. Death, common emotional instability in the later stages of the disease, depression, late manifestations of mental decline or even dementia, bedridden.
Examine
Examination of Xiay-Dreig syndrome
1.24h urine norepinephrine and adrenaline excretion can be determined below normal.
2. Some patients have reduced aldosterone secretion.
3. Blood pressure measurement in the lying position and the upright position.
4. Autonomic nerve examination sweat test, skin scratch test, cold test pressure test weakened or disappeared; 1% adrenaline or 3% cocaine eye drops, showing abnormal pupillary response.
5. MRI examination has a differential diagnosis significance.
6. Electromyography has a differential diagnosis.
Diagnosis
Diagnostic identification of Xiay-Dreig syndrome
diagnosis
1. The symptoms of Xiay-Dreig syndrome are diversified and easy to be misdiagnosed. The main basis for diagnosis is:
(1) Progressive autonomic dysfunction with sporadic adult latent onset, clinical manifestations of orthostatic hypotension, standing systolic blood pressure decreased by 4 to 6.67 kPa (30 to 50 mmHg), and diastolic blood pressure decreased compared with supine position 2.67kPa, and heart rate changes little, impotence or amenorrhea, sweating disorder, dysuria and pupil changes.
(2) Parkinson's syndrome.
(3) The cerebellum sign appears.
(4) A pyramidal tract sign appears.
(5) Exclude other diseases.
Among the above five items, (1) must be, (2), (3) have one item, (4), (5) as a reference.
2. Clinical diagnosis can be divided into 3 types
(1) Progressive autonomic dysfunction.
(2) Progressive autonomic dysfunction with Parkinson's syndrome.
(3) progressive autonomic dysfunction with cerebellar sign.
Differential diagnosis
It should usually be differentiated from the following diseases.
1. Identification with general syncope
(1) simple syncope: there are obvious incentives for syncope, such as pain, fear, emotional tension, air pollution, fatigue, etc., there are short-term prodromal symptoms before fainting, such as dizziness, nausea, paleness, sweating, etc.; syncope is most common Occurs in the upright position, but does not have the special relationship of the above-mentioned obvious straight steric position change; blood pressure drops when syncope, heart rate slows down and weak, pale and lasts until the late syncope, recovery is faster, no obvious aftereffects.
(2) urinary syncope: occurs when urinating, or at the end of urination, causes reflex blood pressure drop and syncope, most often occurs when the patient wakes up at midnight to urinate, or in the morning or nap to urinate.
(3) Cardiac syncope: Cardiovascular diseases, such as atrioventricular block, tachycardia, valvular heart disease, myocardial infarction, etc., can cause syncope due to lack of blood output, causing syncope, ECG examination abnormal.
2. Identification with tremor paralysis
Autonomic symptoms are quite common in Parkinson's disease. Due to the damage of the dorsal nucleus of the vagus nerve, patients often have intractable constipation, or the stomach and small intestines are weakened, but the disease has no orthostatic hypotension, and does not invade the rectum and bladder sphincter. When the urinary dysfunction is caused by factors such as prostatic hypertrophy, in order to further confirm the diagnosis, the electromyogram of the perineal muscle of the patient is performed, and the normal electromyogram is performed.
3. Other
Diseases to be identified secondary to some diseases that cause orthostatic hypotension, such as adrenal insufficiency, chronic alcoholism, diabetes, spinal cord spasm, syringomyelia, porphyria, primary amyloidosis, acute infectious diseases Recovery period, encephalitis and sympathetic trunk resection or post-injury syndrome should be noted. Some patients receive certain antihypertensive drugs, such as chlorpromazine, guanethidine and other drugs, suddenly standing from the lying position. Syncope can occur.
