What is multisystem atrophy?
Multisystem atrophy is a degenerative disease of the central nervous system involving the extrapyramidal, cerebellar, autonomic and pyramidal tracts. Depending on the clinical presentation, it was previously known as substantia nigra striata degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome, respectively.
What are the clinical manifestations of multisystem atrophy?
There are three clinical manifestations – Parkinson’s symptoms, cerebellar ataxia, and autonomic dysfunction. In the early stages of the disease, the clinical manifestations may have their own focus, and as the disease progresses, the various clinical manifestations may overlap and combine, eventually presenting the full range of clinical phases.
What are the autonomic dysfunctions of multiple system atrophy?
1, urinary disorders – including urinary frequency, urinary urgency, urinary incontinence, unclean urine, frequent nocturia.
2, defecation disorders – constipation, diarrhea or fecal incontinence.
3. sexual dysfunction – especially for male patients, mainly erectile dysfunction
4, postural hypotension – a drop in systolic blood pressure ≥ 25 mmHg or diastolic blood pressure ≥ 10 mmHg after standing for 3 minutes, often accompanied by dizziness, blurred vision, blackness, or even leading to syncope, usually with little change in heart rate.
Abnormal sweating and pupillary abnormalities may also be present.
What are the consequences of multisystem atrophy?
Multisystem atrophy itself is not fatal, but it can cause severe motor and autonomic dysfunction. In advanced stages, patients may have significant limitations in movement, difficulty with speech and swallowing, and urinary and bowel incontinence. They are highly susceptible to co-infections of all kinds.
Does multisystem atrophy affect intelligence?
Usually not.
How do I differentiate between multiple system atrophy and Parkinson’s disease?
The differentiation of the two diseases has been a major problem in neurology because the clinical presentation is very similar. The following features of multisystem atrophy can be distinguished.
1. poor response to medroxyprogesterone.
2. frequent early manifestation of abnormal autonomic function.
3. rapid progression of the disease. Electromyography and magnetic resonance imaging of the anal sphincter can also help to differentiate the two. The final differential diagnosis still needs to be made by a neurologist, so follow-up is essential.
How is multisystem atrophy treated?
To date, there is no effective treatment and limited medications are available for symptomatic treatment only, such as Medrobac and Tubotone. Non-pharmacological treatment may have a role in improving quality of life.
What are the non-pharmacological treatments for postural hypotension?
Recommendations: sleeping with the head in a high position, small and frequent meals, increased salt intake (not advisable if the patient is at risk of heart or kidney failure), prudent exercise (swimming; no exertion in the morning; careful rising in the morning, immediately after meals and after exercise), physical exercises (plank flexion). Consider the use of elastic stockings, tights or abdominal bands, and drink a moderate amount of water (350 ml) in the morning.
Avoid: sudden head lifting (especially when waking up), prolonged lying, straining during urination or defecation, high ambient temperature (e.g. hot baths), excessive straining, rapid meals, overeating, alcohol, drugs with vascular decompression, anxiety, fear or hyperventilation.
What is the cross-legged flexion method?
The specific method is to sit with legs crossed in a squat, or sit with one lower limb flexed, raise the other foot on a chair, and then repeatedly do the body leaning forward, as a way to increase the pressure in the abdominal and thoracic cavities and increase the amount of blood returned to the heart. Increase the mean arterial pressure. The best recommended time to train is in the afternoon.
What do I need to pay attention to when taking TubeTone (Midodrine)?
1. The medication should be administered during the daytime whenever possible.
2. the first dose should be taken 30-60 minutes before waking up and the 2nd and/or 3rd dose given before lunch or in the late afternoon.
3. Do not take this medication within 4 hours before bedtime to avoid recumbent hypertension.
How to avoid recumbent hypertension?
Add a moderate amount of food before bedtime; avoid taking Tubotone 4 hours before bedtime; sleep in a head-high position.
Non-pharmacological treatment of urinary and bowel disorders
1. Treatment of coexisting prostatic hyperplasia in men or pelvic floor muscle relaxation in women.
2. treatment of coexisting urinary tract infections.
3. acidification of urine.
4, intermittent (when residual urine >100 ml) or permanent retention of ureter, or suprapubic fistula.
5, avoid urological surgery.
Can patients with multiple system atrophy smoke?
Nicotine directly affects the central nervous system, increasing the release of acetylcholine, norepinephrine, dopamine, 5-HT, etc. in the brain, causing a marked increase in transient symptoms. Therefore, patients with this disease should avoid smoking.