What is neurosarcoidosis?
In patients with sarcoidosis there is a disorder of the immune system, causing a conglomeration of inflammatory cells. These are the so-called granulomas. These granulomas can occur in the entire human body, and can affect every organ system. They cause damage and scaring. The cause of the disease is currently unknown. We think that it is an exgagerated response to a so far unknown stimulus in patients who are vulnerable to develop the disease. Most frequently, these granulomas affect the lungs, but the skin and the eyes are also frequently affected. The nervous system is affected in 1 in 20 people with sarcoidosis - this is called neurosarcoidosis. The way the disease manifests and how serious the disease is varies between patients. If the granulomas affect the brain it can cause facial palsy, meningitis, or epilepsy. When the conglomerated inflammatory cells affect the spinal cord it can cause paraplegia. Furthermore it can affect the small nerve endings in the feet, which can cause a decreased senzation or pain.
How do I know that I have it?
The diagnosis neurosarcoidosis can be very hard to make. To be 100% sure of the diagnosis, a doctor needs to examine nerve- or brain tissue under a microscope for the prescense of these granulomas. The operations necessary to acquire this tissue are dangerous. Therefore, most of the time the diagnosis neurosarcoidosis is being made to establish the presence of granulomas in another part of the body, most frequetnly the lymph nodes or the lungs. The combination of neurological symptoms, abnormalities on scans or in cerebrospinal fluid, together with sarcoidosis in another part of the body, then leads to the diagnosis 'probable neurosarcoidosis'. In the whole diagnostic process, laboratory investigations are necessary, together with investigation of the cerebospinal fluid acquired by a lumbar puncture, nerve conduction studies and/or scans of the nervous sytem and other parts of the body. You may not have realised that u can find a example of a granuloma in the blue bar at the top of this webpage. This is how a granuloma looks like under a microscope. However, we have altered the color.
Can it be treated?
When the diagnosis neurosarcoidosis is probable, treatment may be necessary. The treatment of sarcoidosis consists out of agents that lower the immune system, like prednisone. This is a quick acting hormone that lowers the immune system, and can be administered in a high dose with 'pulse therapy' with an IV-drip or in tablets. Most frequently, a combination of both is necessary to quicly suppress the disease. When we do not succeed in controling the disease with treatment with prednisone or when we expect that a prolonged treatment with prednisone is necessary, a second agent can be added that lowers the immune system in a different way, for example methotrexate or azathioprine. These agents are also being used in other diseases of the immune system, like rheumatoid arthritis. In the case of persistent disease activity despite these agents, your doctors may choose to give a treatment with specific antibodies, like infliximab.
Neurosarcoidosis is a serious disease, after which one in three patients do not improve sufficiently despite adequate treatment. We are currently conducting multiple scientific research projects to improve the diagnostic process and treatment of patients with neurosarcoidosis. To do this, we are working closely together with the Erasmus MC in Rotterdam. You can find more information about our research projects when you click on the button 'Scientific research'.
We are a tertiary referral center for patients with sarcoidosis. Healthcare for patients with sarcoidosis can be complex. Our opinion is that because the disease is relatively rare, care for these patients with severe manifestations of the disease should be performed preferably in a few specialised hospitals. We want to improve Healthcare for these patients. Part of this is a specialist outpatient clinic for sarcoidosis patients with a multidisciplinary approach. Additionally we have started various scientific researchprojects, aimed at improving the diagnostic process and treatment of this disease. Below you can find more information concerning the pulmonary medicine department, who we are, what kind of research we are conducting and how you can refer patients to us.