How is neurosarcoidosis treated?

Updated: Nov 13, 2018
  • Author: Gabriel Bucurescu, MD, MS; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
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Answer

Neurosarcoidosis has no known cure. Spontaneous remission has been observed, but long-term therapy often is required. Treatment alleviates symptoms that are severe or progressive.

Immunosuppression is the principal method of controlling the disease, and corticosteroids are the cornerstone of therapy. In cases of exacerbation, intravenous pulsed methylprednisolone followed by oral taper may be necessary. Treatment is guided by the clinical response to corticosteroids. If the response is favorable (ie, steady amelioration of symptoms), then the dose may be tapered over several months. Follow-up by a neurologist every 3-6 months to monitor the progress of the disease is important.

Relapses may respond poorly, however, requiring long-term steroid therapy. The prognosis with peripheral neuropathy is more favorable than with central nervous system involvement.

A variety of immunosuppressant agents (eg, cyclosporine, methotrexate, cyclophosphamide) have been used in patients with refractory neurosarcoidosis, with varying results. Almost all of the studies completed to date have involved treatment of central nervous system sarcoidosis as opposed to peripheral neuropathy.

There have been anecdotal reports of improvement with intravenous immunoglobulin therapy in patients in whom conventional therapy has failed. [35] The response may be related to amelioration of vasculitic neuropathy.

In patients with brain involvement, low-dose radiation has produced clear symptomatic benefits in some patients. Since the adverse effects of low-dose cranial irradiation are minimal, using radiation therapy may be prudent for patients whose disease is refractory to steroids or who have had adverse responses to high-dose steroids. Removal of the space-occupying lesions in the brain has little or no benefit and should be attempted only in extreme cases. Hydrocephalus may require ventriculoperitoneal shunting.


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