Subacute combined degeneration
Causes of Subacute combined degeneration
Subacute combined degeneration is caused by a vitamin B12 deficiency. It mainly affects the spinal cord. But its effects on the brain and the peripheral (body) nerves are the reason for use of the term "combined." At first, the covering of the nerves (myelin sheath) is damaged. Later, the entire nerve cell is affected.
Doctors do not know exactly how a lack of vitamin B12 damages the nerves. It is possible the lack of this vitamin causes abnormal fatty acids to form around cells and nerves.
A person has a high risk of this condition if vitamin B12 cannot be absorbed from the intestine or if a person has:
- Pernicious anemia
- Disorders of the small intestine, including Crohn's disease
- Problems absorbing nutrients, which can occur after gastrointestinal surgery
Subacute combined degeneration Symptoms
These symptoms slowly get worse and are usually felt on both sides of the body.
Other symptoms include:
- Clumsiness, stiff, or awkward movements
- Change in mental state such as memory problems, irritability, apathy, confusion, or dementia
- Decreased vision
- Unsteady gait and loss of balance
Tests and Exams
The health care provider will perform a physical exam. The exam usually shows muscle weakness and sensation problems on both sides of the body, especially in the legs. Knee jerk reflexes are often decreased. Muscles may develop spasticity. There may be reduced senses of touch, pain, and temperature.
Mental changes range from mild forgetfulness to severe dementia or psychosis. Severe dementia is uncommon, but in some cases, it is the first symptom of the disorder.
An eye exam may show damage to the optic nerve, a condition called optic neuritis. Signs of nerve inflammation may be seen during a retinal exam. There may also be abnormal pupil responses, decreased visual acuity, and other changes.
Blood teststhat may be ordered include:
Treatment of Subacute combined degeneration
Early treatment improves the chance of a good outcome.
Vitamin B12 is given, usually by injection into a muscle. Injections are often given once a day for a week, then weekly for about 1 month, and then monthly. Vitamin B12 supplements either by injection or high dose pills must continue throughout life to prevent symptoms from returning.
How well a person does depends on how long they had symptoms before receiving treatment. If treatment is received within a few weeks, complete recovery may be expected. If treatment iss delayed for longer than 1 or 2 months, full recovery may not be possible.
Untreated, the disorder results in progressive and irreversible damage to the nervous system.
Complications can include permanent, progressive loss of nerve and mental functions.
When to Contact a Health Professional
Call your health care provider if abnormal sensations, muscle weakness, or other symptoms of subacute combined degeneration develop. This is particularly important if you or a family member has had pernicious anemia or other risk factors.
Prevention of Subacute combined degeneration
Some vegetarian diets may be low in vitamin B12. Taking a supplement can prevent the disorder.
Katri B, Koontz D. Disorders of the peripheral nerves. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradley's Neurology in Clinical Practice. 6th ed. Philadelphia, Pa: Elsevier Saunders; 2012:chap 76.
|Review Date: 5/28/2013
Reviewed By: Luc Jasmin, MD, PhD, FRCS (C), FACS, Department of Neurosurgery at Cedars-Sinai Medical Center, Los Angeles CA; Department of Surgery at Los Robles Hospital, Thousand Oaks CA; Department of Surgery at Ashland Community Hospital, Ashland OR; Department of Surgery at Cheyenne Regional Medical Center, Cheyenne WY; Department of Anatomy at UCSF, San Francisco CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.