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Neurosarcoidosis
Definition
Neurosarcoidosis is a complication of sarcoidosis in which inflammation occurs in the brain, spinal cord, and other areas of the nervous system.
Alternative Names
Sarcoidosis - nervous system
Causes
Sarcoidosis is a long-term (chronic) disorder that affects many parts of the body, mostly the lungs. In a small number of patients, the disease involves some part of the nervous system. This is called neurosarcoidosis.
Neurosarcoidosis may affect any part of the nervous system. Sudden, facial weakness (facial palsy)is the most common neurological symptom and involves the nerves to the muscles of the face (cranial nerve VII). Any nerve in the skull can be affected, including those in the eye and those that control taste, smell, or hearing.
The condition can also affect the parts of the brain 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, including the pituitary gland at the base of the brain, or the spinal cord may also be involved.
Symptoms
Involvement of the pituitary gland can cause:
- Changes in menstrual periods
- Excessive tiredness (fatigue)
- Excessive thirst
- High urine output
The symptoms vary. Any part of the nervous system can be affected. Involvement of the brain or cranial nerves can cause:
- Confusion, disorientation
- Decreased hearing
- Dementia
- Delirium
- Dizziness or vertigo (abnormal sensation of movement)
- Double vision or other vision problems
- Facial palsy (weakness, drooping)
- Headache
- Loss of sense of smell
- Loss of sense of taste, abnormal tastes
- Psychiatric disturbances
- Seizures
- Speech impairment
Involvement of one or more peripheral nerves can lead to:
- Abnormal sensations in any body part
- Loss of movement of any body part
- Loss of sensation in any body part
- Weakness of any body part
Exams and Tests
An exam may show problems with one or more nerves.
A history of sarcoidosis followed nerve-related symptoms highly suggests neurosarcoidosis. However, symptoms of the condition can mimic other medical disorders, including diabetes insipidus, hypopituitarism, optic neuritis, meningitis, and certain tumors.
Blood tests are not very helpful in diagnosing the condition. A lumbar puncture may show signs of inflammation. Increased levels of angiotensin-converting enzyme may be found in the blood or cerebrospinal fluid (CSF). However, this is not a reliable diagnostic test.
MRI of the brain may be helpful. A chest x-ray often reveals signs of the sarcoidosis of the lungs. Nerve biopsy of affected nerve tissue confirms the disorder.
Treatment
There is no known cure for the sarcoidosis. 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 until symptom get better or go away. You may need to take the medicines for months, even years.
Other medications may include hormone replacement and medicines that suppress the immune system.
If you have numbness, weakness, vision or hearing problems, or other problems due to damage of the nerves in the head, you may need physical therapy, braces, a cane, or walker.
Psychiatric disorders or dementia may require medication for depression, safety interventions, and assistance with care.
Outlook (Prognosis)
Some cases go away on their own in 4-6 months. Other cases continue off and on for the rest of the person's life. Neurosarcoidosis can cause permanent disability and, in some cases, death.
Possible Complications
Complications vary depending on which part of the nervous system is involved and how you respond to treatment. Slowly worsening or permanent loss of neurological function is possible. In rare cases, the brainstem may be involved. This is life threatening.
When to Contact a Medical Professional
Call your health care provider if you have sarcoidosis and any neurological symptoms occur.
Go to the emergency room or call the local emergency number (such as 911) if you have a sudden loss of sensation, movement, or body function.
Prevention
Aggressive treatment of sarcoidosis turns off the body's faulty immune response before your nerves become damaged. This may reduce the chance that neurological symptoms will occur.
References
Aminoff MJ, Josephson SA. Neurological complications of systemic disease: adults. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradley’s Neurology in Clinical Practice. 6th ed. Philadelphia, Pa:Saunders Elsevier; 2012:chap 49A.
Iannuzzi M. Sarcoidosis. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 95.
Reviewed By: Luc Jasmin, MD, PhD, Department of Neurosurgery at Cedars-Sinai Medical Center, Los Angeles, and Department of Anatomy at UCSF, San Francisco, CA. Review provided by VeriMed Healthcare Network. David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.
