The larger clumps seen under the microscope, called glial cytoplasmic inclusions, may actually be the brain’ s attempt to solidify the tiny, toxic clumps floating around inside the cell into a hard, relatively harmless form.
The same protein, alpha-synuclein, accumulates in the brain cells in Parkinson’ s disease, but in somewhat different parts of the brain. Also, in MSA, the initial accumulation and cell loss appear to be in the glia, the electrically inactive supporting cells of the brain. In Parkinson’ s disease, on the other hand, the problem starts in the neurons, the electrically active cells.
At what age does MSA start?
The average age at which the symptoms of MSA begin is only 53. This is younger than the averages of 59 for Parkinson’ s and 63 for PSP.
What happens to someone with MSA?
All three kinds of MSA feature important balance problems that can eventually require assistance in walking or even a wheelchair. All three types can also display difficulty in the aspect of thinking called executive function. This is what allows us to organize information by categories, understand abstractions and instructions, create and follow a plan, and inhibit inappropriate actions. These abilities often decline to some extent in people with MSA.
Other features occur in all three types of MSA. Perhaps the most important is difficulty swallowing, which obliges one to modify the diet to exclude thin liquids or tough solids. All three types of MSA can also have difficulties in moving the eyes, which can interfere with reading, and in speaking, which may require speech therapy and communication devices.
People with MSA of the parkinsonian type tend to get more muscle stiffness and general slowness. Those with the cerebellar type develop a coarse tremor when moving the limbs and difficulty aiming their limb movements or walking in a straight line, much like someone who is drunk. Those people with MSA that emphasizes the autonomic problem have a variety of symptoms, including urinary urgency and incontinence, constipation, erectile disturbance in men, lightheadedness or even fainting upon standing, noisy or irregular breathing, sleep disruption, and difficulties with temperature regulation. Most people with MSA have some combination of the three types rather than a pure form.
27
Some other problems with movement can occur in MSA. The hands can gradually assume abnormal, fixed postures called dystonia. This can be prevented to some degree by stretching exercises and can be treated in some cases by botulinum toxin( Botox) injections into the hands or forearms. Some people with MSA hold their heads bent forward to an extreme degree, a condition called antecollis. This too may respond to Botox injected into the neck muscles, although care must be taken not to exacerbate the swallowing problems via leakage of the Botox to the immediately adjacent muscles. Another issue in some people is sudden, rapid jerks of a limb or of the trunk. These issues can be annoying but are almost never large enough to interfere with normal movement. This is called myoclonus and can be treated with medication.
Finally, a tremor, which is not nearly as prominent as in most people with Parkinson’ s, can occur in MSA. This tremor generally happens when the limb is in use rather than at rest( as in Parkinson’ s). The MSA tremor may respond to medication but usually is too mild to require treatment.
Some people with MSA develop obstructive sleep apnea. This is where the upper airway tends to close during sleep, causing insomnia and sometimes insufficient blood oxygen levels. It can be treated by wearing a mask during sleep that connects to a kind of air pump that keeps the airway open, called continuous positive airway pressure( CPAP). This is usually best managed by a specialist in sleep medicine or pulmonary medicine rather than a neurologist.