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Alternative namesSarcoidosis - nervous system
DefinitionNeurosarcoidosis is a complication of sarcoidosis involving inflammation and abnormal deposits in the tissues of the nervous system (which includes the brain, the spinal cord, and other nerves).
Causes, incidence, and risk factors
Sarcoidosis is a chronic disorder that affects many body systems. The majority of cases involve the lungs, but the condition can affect any area of the body. Sarcoidosis is characterized by an abnormal immune system response that produces deposits of white blood cells and abnormal tissue cells in the affected organ. A small percentage of cases will involve some portion of the nervous system (neurosarcoidosis).
The condition can also affect a part of the brain called the hypothalamus, which is involved in regulating many body functions such as temperature, sleep, and stress responses. Muscle weakness or sensory losses can occur with peripheral nerve involvement. Other areas of the brain or the spinal cord may also be involved. Neurosarcoidosis can cause permanent disability and, in some cases, death.
SymptomsInvolvement of the pituitary gland can cause:
Signs and tests
An examination may indicate single nerve dysfunction ( mononeuropathy ) or dysfunction of multiple peripheral nerves ( sensorimotor polyneuropathy ). Signs may mimic diabetes insipidus , hypopituitarism , optic neuritis , meningitis , space-occupying lesions such as tumors, or other neurologic disorders.
A history of sarcoidosis followed by neurologic symptoms is highly suggestive of neurosarcoidosis. Blood tests are nonspecific. A lumbar puncture may show signs of inflammation. Elevated levels of angiotensin converting enzyme may be found in the blood or cerebrospinal fluid (CSF).
TreatmentThere is no known cure for the disorder. Treatment is indicated if symptoms are severe or progressive. The goal of treatment is to reduce symptoms.
Corticosteroids such as prednisone are prescribed to reduce inflammation. They are often prescribed for months to years, or until symptoms subside. Other medications, particularly those that suppress the immune system, may also be recommended.
Symptoms should be treated. For example, a numb body part should be protected from harm. Weak areas may require physical therapy or appliances to aid mobility and ability to function. Psychiatric disorders or dementia may require safety interventions, assistance with care, and other treatments such as medication for depression. Pituitary disorders may respond to conventional treatments such as hormone replacement.
The probable outcome varies. Some cases resolve on their own in 4 to 6 months. Other cases continue intermittently for the rest of the person's life.
A small percentage of people with sarcoidosis of any type will die as a direct result of the disorder, most commonly when brainstem or heart involvement causes a heart attack.
Calling your health care providerCall your health care provider if you have sarcoidosis and any neurologic symptoms occur.
Go to the emergency room or call the local emergency number (such as 911) if there is a sudden loss of sensation , movement, or function.
PreventionTreatment of known sarcoidosis may reduce the chance that neurologic symptoms will occur by shutting off the immune system before the abnormal response can harm nerves.
Update Date: 7/28/2002Elaine T. Kiriakopoulos, M.D., MSc, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard University, Boston, MA. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT