Multiple system atrophy - cerebellar subtype
MSA-C; Cerebellar multiple system atrophy; Olivopontocerebellar atrophy; OPCA; Olivopontocerebellar degenerationMultiple system atrophy - cerebellar subtype (MSA-C) is a rare disease that causes areas deep in the brain, just above the spinal cord, to shrink (atrophy). MSA-C used to be known as olivopontocerebellar atrophy (OPCA).
Causes
MSA-C can be passed down through families (inherited form). It can also affect people without a known family history (sporadic form).
Researchers have identified certain genes that are involved in the inherited form of this condition.
The cause of MSA-C in people with the sporadic form is not known. The disease slowly gets worse (is progressive).
MSA-C is slightly more common in men than in women. The average age of onset is 54 years.
Symptoms
Symptoms of MSA-C tend to start at a younger age in people with the inherited form. The main symptom is clumsiness (ataxia) that slowly gets worse. There may also be problems with balance, slurring of speech, and difficulty walking.
Ataxia
Uncoordinated movement is due to a muscle control problem that causes an inability to coordinate movements. It leads to a jerky, unsteady, to-and-fr...
Read Article Now Book Mark ArticleOther symptoms may include:
- Abnormal eye movements
- Abnormal movements
- Abnormal sweating
- Bowel or bladder problems
- Difficulty swallowing
Difficulty swallowing
During pregnancy, it is harder for a woman's immune system to fight infections. This makes a pregnant woman more likely to get the flu and other dis...
Read Article Now Book Mark Article - Cold hands and feet
- Lightheadedness when standing
- Headache while standing that is relieved by lying down
Headache
A headache is pain or discomfort in the head, scalp, or neck. Serious causes of headaches are rare. Most people with headaches can feel much better...
Read Article Now Book Mark Article - Muscle stiffness or rigidity, spasms, tremor
Muscle stiffness
Spasticity is stiff or rigid muscles. It may also be called unusual tightness or increased muscle tone. Reflexes (for example, a knee-jerk reflex) ...
Read Article Now Book Mark ArticleTremor
A tremor is a type of shaking movement. A tremor is most often noticed in the hands and arms. It may affect any body part, including the head, tong...
Read Article Now Book Mark Article - Nerve damage (neuropathy)
- Problems in speaking and sleeping due to spasms of the vocal cords
Spasms of the vocal cords
Spasmodic dysphonia is difficulty speaking due to spasms (dystonia) of the muscles that control the vocal cords.
Read Article Now Book Mark Article - Sexual function problems
Exams and Tests
A thorough medical and nervous system examination, as well as a symptom review and family history are needed to make the diagnosis.
There are genetic tests to look for the causes of some forms of the disorder. But, no specific test is available in many cases. An MRI of the brain may show changes in the size of affected brain structures, especially as the disease gets worse. But it is possible to have the disorder and have a normal MRI.
MRI of the brain
A head MRI (magnetic resonance imaging) is an imaging test that uses powerful magnets and radio waves to create pictures of the brain and surrounding...
Read Article Now Book Mark ArticleOther tests such as positron emission tomography (PET) may be done to rule out other conditions. These may include swallowing studies to see if a person can safely swallow food and liquid.
Positron emission tomography (PET)
A positron emission tomography (PET) scan is a type of imaging test. It uses a radioactive substance called a tracer to look for disease in the body...
Read Article Now Book Mark ArticleTreatment
There is no specific treatment or cure for MSA-C. The aim is to treat the symptoms and prevent complications. This may include:
- Tremor medicines, such as those for Parkinson disease
Parkinson disease
Parkinson disease results from certain brain cells dying. These cells help control movement and coordination. The disease leads to shaking (tremors...
Read Article Now Book Mark Article - Speech, occupational and physical therapy
- Ways to prevent choking
- Walking aids to help with balance and prevent falls
- Devices to treat sleep apnea (such as CPAP)
- Treatment for low blood pressure
Support Groups
More information and support for people with MSA-C and their families can be found at:
- Defeat MSA Alliance -- defeatmsa.org/patient-programs/
- Mission MSA -- missionmsa.org/resource-library/
Outlook (Prognosis)
MSA-C slowly gets worse, and there is no cure. The outlook is generally poor. But, it may be years before someone is very disabled.
Possible Complications
Complications of MSA-C include:
- Choking
- Infection from inhaling food into the lungs (aspiration pneumonia)
Aspiration pneumonia
Pneumonia is inflammation (swelling) and infection of the lungs or large airways. Aspiration pneumonia occurs when food or liquid is breathed into th...
Read Article Now Book Mark Article - Injury from falls
- Nutrition problems due to difficulty swallowing
When to Contact a Medical Professional
Contact your health care provider if you have any symptoms of MSA-C. You will need to be seen by a neurologist. This is a specialist who treats nervous system problems.
References
Jancovic J. Parkinson disease and other movement disorders. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 96.
Jellinger KA. Neuropathology of movement disorders. In: Winn HR, ed. Youmans and Winn Neurological Surgery. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 104.
Ma MJ. Biopsy pathology of neurodegenerative disorders in adults. In: Perry A, Brat DJ, eds. Practical Surgical Neuropathology: A Diagnostic Approach. 2nd ed. Philadelphia, PA: Elsevier, 2018:chap 27.
Walsh RR, Krismer F, Galpern WR, et al. Recommendations of the global multiple system atrophy research roadmap meeting. Neurology. 2018;90(2):74-82. PMID: 29237794 pubmed.ncbi.nlm.nih.gov/29237794/.
Brain structures - illustration
The structures of the brain include the brainstem, consisting of the spinal cord, the medulla oblongata, the pons and the midbrain; the cerebellum; the cerebrum (one half, or hemisphere shown), and the diencephalon.
Brain structures
illustration
Review Date: 6/13/2024
Reviewed By: Joseph V. Campellone, MD, Department of Neurology, Cooper Medical School at Rowan University, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.