Sarcoidosis is a rare inflammatory disease characterized by the formation of immune cell clumps called granulomas. These granulomas can form in different parts of the body, and may interfere with the normal function of tissues and organs. When they form in and around the nervous system, the condition is called neurosarcoidosis.

What is neurosarcoidosis?

In about 5 to 15 percent of sarcoidosis patients, the granulomas develop in the nervous system — the brain, spinal cord, or peripheral nerves, those located outside the brain and spinal cord.

Most commonly, neurosarcoidosis affects the nerves in the head and face, and in regions of the brain called the hypothalamus and the pituitary gland. The hypothalamus is the main area of the brain that controls the production of hormones. The pituitary gland, located just underneath the hypothalamus, produces different hormones as directed by the hypothalamus.

Neurosarcoidosis can appear acutely, or as a slow, chronic illness. Because it develops in many different ways, diagnosis can be difficult.

Symptoms of neurosarcoidosis

Neurosarcoidosis symptoms vary depending on where the granulomas form. If the condition affects the pituitary gland, symptoms may include changes in menstrual periods and fatigue.

If the condition affects other parts of the brain or cranial nerves, symptoms may include:

  • Headache
  • Confusion, disorientation, and dementia
  • Hearing or vision loss
  • Bell’s palsy, which is characterized by weakness or drooping of the facial muscles
  • Seizures

If the condition affects peripheral nerves, it can cause muscle weakness, numbness or tingling, or the inability to move.

Diagnosis of neurosarcoidosis

Diagnosing neurosarcoidosis can be difficult if the nervous system is the only location of granuloma formation. However, this is quite rare, and in about 90 percent of sarcoidosis patients, the lungs are also affected.

To diagnose neurosarcoidosis, several tests are required. Magnetic resonance imaging (MRI) or computerized tomography (CT) scans are used to confirm the presence of inflammation in the spinal cord and brain. Laboratory tests of the cerebrospinal fluid, the fluid in the brain and spinal cord, should detect elevated protein levels and high numbers of immune cells.

Tissue biopsies are generally used to confirm sarcoidosis, but in neurosarcoidosis, biopsies of the brain or nerves are usually considered too invasive. Lung or muscle biopsies may be sufficient to confirm neurosarcoidosis; however, if all other tests are inconclusive, brain or nerve biopsies may be required.

Treatment of neurosarcoidosis

Treatment of neurosarcoidosis is generally focused on managing symptoms and suppressing the immune system. Patients may first be treated with corticosteroids such as prednisone, but not all patients respond to this treatment. Cytotoxic medications such as methotrexate or Imuran (azathioprine) may be prescribed in these cases.

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