Multiple system atrophy (MSA)
Multiple system atrophy is a rare disease in which nerve cells in several parts of the brain die off at the same time.
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Medicines commonly prescribed for Multiple system atrophy (MSA)
For informational purposes only. Always consult a doctor before using any medicine.
Dosage form: INJECTABLE, 0.05 mgActive substance: baclofenManufacturer: Novartis Farmaceutica S.A.Prescription requiredDosage form: TABLET, 5 mgActive substance: midodrineManufacturer: Cheplapharm Arzneimittel GmbhPrescription requiredDosage form: INJECTABLE PERFUSION, 10 mgActive substance: baclofenManufacturer: Novartis Farmaceutica S.A.Prescription required
Multiple system atrophy is a rare disease in which nerve cells in several parts of the brain die off at the same time. Hence the name: it is not one system that fails but several. A person loses steadiness and precision of movement, and along with them the control of blood pressure, bladder and bowel. There is still no way to halt the process, but almost every symptom can be eased, and how early that work starts decides how many years are spent without falls, without a catheter and without choking at night.
What actually breaks down
A protein called alpha-synuclein builds up in the cells that surround the nerve cells. The same protein collects in Parkinson's disease, but there it gathers inside the nerve cells themselves, while here it settles in the supporting cells, so the loss is wider and faster. Three territories are affected: the one that makes movement smooth, the cerebellum, and the brainstem centres that hold blood pressure steady and run the bladder and bowel.
Two forms are described. In the parkinsonian form stiffness and slowness come first; in the cerebellar form it is unsteadiness and slurred speech. Over time the picture usually blends. Disturbances of the automatic nervous system are present in both: without them the diagnosis is not made.
The illness mostly begins after the age of fifty and hardly ever before thirty. It is not passed on to children: cases in which MSA affects several members of one family have barely been described, so relatives do not need to be tested.
Where it usually starts
The first thing to fail is normally not the muscles but the centres that run the body automatically, and this can happen several years before walking becomes visibly difficult.
- Blood pressure dropping on standing: vision darkens, ears ring, the neck and shoulders ache as though a coat hanger were pulling on them. In severe cases the person faints. The dizziness is worse in the morning, after meals, in hot weather and after a hot shower.
- Bladder: at first frequent and sudden urges, getting up at night, leaking. Later the opposite: a weak stream, incomplete emptying, urine left behind, which brings a risk of infection and of complete retention.
- In men, erectile dysfunction, often the very first sign of all.
- Stubborn constipation, a dry mouth and dry eyes, disturbed sweating: soaking palms one day, completely dry skin the next.
Taken singly, every one of these symptoms occurs in a great many healthy people and is explained by something else entirely. What is telling is the combination: falling blood pressure plus bladder trouble plus a changed walk in someone over fifty.
What happens to movement
In the parkinsonian form the muscles become tight, movements small and slow, the stride shortens and handwriting shrinks. There may be tremor, but it is usually irregular and appears on movement rather than at rest as in Parkinson's disease. There is an even more revealing detail: levodopa, which in Parkinson's disease gives clear improvement for years, here helps weakly and briefly, or not at all.
In the cerebellar form the walk becomes wide and unsteady, the hand misses the cup and starts to shake just as it reaches the target, speech turns broken and slurred, and the eyes jerk when looking to the side.
Posture deserves a separate mention: the head tips forward until the chin nearly rests on the chest, and the trunk leans to one side. This is characteristic of MSA and uncommon in the diseases it resembles. Falls do occur, but unlike in progressive supranuclear palsy they do not appear in the first year.
Sleep, breathing and what is rarely mentioned
Some of the features have nothing to do with blood pressure or walking, and people usually do not count them as part of the illness at all.
- Restless dreaming sleep: the person talks, shouts, fights back and sits up as if acting out a dream, and may injure themselves or hit whoever is sleeping beside them. This often begins many years before everything else.
- Night-time stridor, a high whistling sound on breathing in. It happens because the vocal cords stop opening properly, and it is not a variety of snoring but a narrowing of the airway.
- Pauses in breathing during sleep, daytime sleepiness, morning headache.
- Choking, especially on liquids, a feeling that food is stuck in the throat, drooling.
- Sudden laughing or crying that does not match the mood. This is a fault in how the signal travels, not emotional instability, and it helps if the family knows that.
- Icy, blotchy blue hands and feet, and poor tolerance of both heat and cold.
When help is needed urgently
Call an ambulance (112 across much of Europe) if any of these appear:
- noisy, whistling breathing on breathing in, a feeling of not getting enough air, blue lips;
- choking with an inability to cough, speak or breathe;
- a faint with a blow to the head, or the person does not come round;
- a seizure.
See a doctor the same day, without waiting for the next booked appointment, if:
- several hours pass with no urine despite a full-feeling bladder: this is urinary retention and it is relieved with a catheter;
- fever and a cough follow episodes of choking on food: this is how pneumonia from food entering the airway begins;
- the urine turns cloudy and smelly, with pain and shivering;
- faints become more frequent, or the person starts falling without warning.
How the diagnosis is reached
There is no single test that confirms MSA. The neurologist assembles the diagnosis from the whole set of signs, and the investigations serve to confirm some things and rule out others.
- Measuring blood pressure lying down and then standing, at one minute and at three. It is the simplest test and the one most often skipped, yet it is exactly what demonstrates the drop in pressure without which the diagnosis is not made.
- MRI of the brain: shrinkage of the putamen, the middle cerebellar peduncles and the pons, and sometimes a cross-shaped pattern on a slice through the pons. These findings do not appear straight away, and a normal scan early on does not rule the diagnosis out.
- Bladder ultrasound after passing urine, to see how much is left behind.
- A trial of levodopa, to judge whether there is a response and whether it lasts.
What does not make the diagnosis: routine blood tests and a CT scan of the head are normal in MSA, and a normal result excludes nothing. A scan of the dopamine transporter shows the loss of the same nerve cells as in Parkinson's disease and cannot tell the two apart, although it is often requested in exactly that hope.
Full certainty comes only from examining brain tissue, and therefore only after death. While the person is alive the diagnosis is treated as probable and is revisited: in the first year or two MSA is frequently taken for Parkinson's disease, and the picture becomes clear when levodopa has failed to work and the automatic nervous system is affected more severely than parkinsonism would explain.
What can be done to help
No medicine stops the nerve cells dying. Everything done is aimed at symptoms, and a good deal can be done.
- Blood pressure: sleep with the head of the bed raised, drink more water, do not cut down on salt unless a doctor has specifically said so, wear compression stockings and an abdominal binder, and get up in two stages, sitting first. The medicines used are those that raise the tone of the blood vessels and a hormone that holds on to salt; they are adjusted carefully, because lying down these patients often have high blood pressure instead.
- Bladder: learning intermittent self-catheterisation, plus medicines that calm an overactive bladder or make emptying easier.
- Stiffness: levodopa is tried and kept on if it gives any improvement at all.
- Speech and swallowing: a speech and language therapist works out exactly what the person chokes on and adjusts the texture of food and the techniques that lower the risk. When swallowing is no longer safe, feeding through a tube into the stomach is discussed.
- Movement: physiotherapy for balance and strength, occupational therapy so that dressing, cooking and washing stay independent for longer, and a stick, frame or grab rails chosen before they are desperately needed.
- Stridor and pauses in breathing at night: a machine that delivers air under pressure through a mask; if the narrowing of the larynx is severe, an operation on the windpipe is considered.
- Constipation, pain in stiff muscles, broken sleep and low mood are treated in their own right: they spoil daily life badly and respond well.
What is worth deciding early
MSA moves faster than Parkinson's disease, and the need for help grows over a few years rather than decades. That is an unwelcome but solid reason to discuss the important things while the person still speaks clearly and decides for themselves.
The matters are practical ones: how to alter the home so there are no steps and there is something to hold on to in the shower; who in the family takes on what, and whether it is all quietly landing on one person; which documents are worth signing, including power of attorney and a written statement of what the person does and does not want regarding assisted breathing and tube feeding. It is worth finding out in advance where the nearest palliative care team is: they do not come in only for the last days, but when there are more symptoms than a neurology appointment has time to work through.
Whoever is doing the caring needs their own breaks and their own doctor. Carer burnout is neither weakness nor a rarity but the predictable result of a load that never stops.
Online consultation
A remote appointment is useful when you need to work out what to do with a diagnosis already made, or with a confusing set of symptoms. The doctor will go through whether your complaints fit the picture of MSA or point elsewhere, explain which investigations are worth having and in what order, help with blood pressure, bladder and sleep between neurology visits, and tell you how to act when faints or choking happen.
It helps to have ready: a list of your medicines with doses, blood pressure readings taken lying and standing over several days, reports and scans if any tests have been done, and a short list of what has changed over the past six months. An online consultation does not replace a face-to-face neurological examination and is not suitable for an emergency: difficulty breathing, a faint with injury or an inability to pass urine all need care in person.
This material is for information only and does not replace medical advice.
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