Shiy-Drager Syndrome in the Elderly
Introduction
Introduction to the elderly Xiay-Dreig syndrome Xiay-Dreig syndrome is a rare, progressive neurological degenerative disease that occurs in middle-aged and elderly people. It is characterized by progressive paroxysmal syncope, orthostatic hypotension, and sexual function. Autonomic dysfunction and motor dysfunction such as obstacles and no sweat. The main pathological changes are the autonomic nervous system, extensive degeneration of the pyramidal tract, basal ganglia and cerebellum. basic knowledge The proportion of illness: 0.002% Susceptible people: the elderly Mode of infection: non-infectious Complications: brain atrophy, hypotension, ataxia
Cause
The cause of the elderly Xiay-Dreig syndrome
(1) Causes of the disease
Schaeff-Dreiger syndrome is a more common degenerative disease in multiple system atrophy, often indistinguishable from hereditary cerebellar ataxia or Parkinson's superposition syndrome, except in the extensive central nervous system. In addition to autonomic nervous system degeneration, there are significant degenerative changes in the anterior horn of the spinal cord, pyramidal tract, cerebellum, and basal ganglia.
(two) pathogenesis
The manifestations of autonomic dysfunction are mainly caused by efferent adrenergic neurological failure, circulating norepinephrine levels are normal, patients with normal norepinephrine and tyramine have normal reactivity, but plasma catecholamine concentration There was no corresponding increase in erect position, and circulating dynamics studies confirmed that patients had significant impairment of cardiovascular function. The positron emission tomography (PET) study found that cerebral blood flow was associated with metabolic abnormalities.
Prevention
Elderly Xiay-Dreig Syndrome Prevention
For the treatment of the cause, prevent complications.
Complication
Elderly Xiay-Dreig syndrome complications Complications, brain atrophy, hypotension, ataxia
Can be complicated by brain atrophy, impotence, hypotension and ataxia.
Symptom
Symptoms of the elderly Xiay-Dreig syndrome Common symptoms Abdominal pain response, diarrhea, ataxia, loss of blood pressure, weakness, hypotension, motor dysfunction, male sexual dysfunction, muscle atrophy
1. More than 50 to 60 years old, the male and female are basically equal.
2. Autonomic dysfunction is a common and outstanding performance
(1) Early manifestations often have paroxysmal syncope.
(2) Male erectile dysfunction is a prominent early manifestation.
(3) 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). Above, and lack of reactive heart rate, cardiac autonomic dysfunction leads to arrhythmia or changes in cardiac output.
(4) gastrointestinal dysfunction, diarrhea, constipation, abdominal pain or fecal incontinence.
(5) urinary dysfunction including urinary fistula, frequent urination, urgency or incontinence.
(6) Abnormal sweating.
(7) Abnormal pupil (small but lightly unresponsive).
3. Patients with abnormal motor function may have motor dysfunction in addition to the above performance.
(1) Cerebellar damage: limb ataxia or trunk balance disorder.
(2) Cone beam involvement: increased muscle tone, muscle weakness, hyperreflexia or pathological reflex.
(3) Extrapyramidal lesions: various dyskinesias such as myotonia, bradykinesia, or tremors, such as Parkinson's syndrome.
(4) The anterior horn of the spinal cord damages muscle atrophy or fasciculation.
Examine
Examination of the elderly Xiay-Dreig syndrome
Blood potassium and blood calcium are normal.
1. Neuroimaging CT or MRI showed cerebellar atrophy. MRI showed that abnormal signals in the nucleus of the nucleus have important diagnostic value in multi-system atrophy.
2. Positron emission tomography studies of cerebral blood flow and metabolic status revealed low metabolic status.
Diagnosis
Diagnostic identification of the elderly Xiay-Dreig syndrome
Diagnostic criteria
1. In the middle and old age, the disease progressed slowly.
2. Autonomic dysfunction is the main symptom, such as paroxysmal syncope, orthostatic hypotension, sexual dysfunction, etc. The characteristic signs are that when the body position falls from the supine position to the upright position, the blood pressure drops, and the lack of reactive heart rate increases. .
3. Some patients have motor dysfunction such as increased muscle tone, tremor, decreased exercise, ataxia or sputum reflex changes.
4. Neuroimaging examination CT or MRI showed cerebellar atrophy, and MRI showed abnormal signal of shell nuclear.
5. Exclude orthostatic hypotension caused by other diseases or causes.
Differential diagnosis
1. Other causes of autonomic failure are other chronic chronic autonomic dysfunction with chronic multiple peripheral neuropathy (caused by multiple causes), high cervical spinal cord disease, familial Riley-Day autonomic function Obstacles and severe tetanus.
2. Cardiac dysfunction caused by heart disease caused by cardiac dysfunction or decreased cardiac output caused by syncope or orthostatic hypotension needs to be differentiated from this disease.
3. Drug-induced orthostatic hypotension The use of certain drugs in the elderly may lead to orthostatic hypotension. Commonly used drugs include beta blockers, calcium channel blockers and chlorpromazine. Some patients have orthostatic hypotension. Caused by water loss.
4. The main point of identification of Parkinson's disease is that the dyskinesia of Shy-Drager syndrome often lacks resting tremor, and the performance of cerebellar involvement and the symptomatic treatment against Parkinson's disease are not good.
