Neurosarcoidosis

  : In Jan 1997 I was diagnosed with Bell's palsy. Now, Sept 1998, I have just been diagnosed with neurosarcoidosis. I have severe impairment of the 3rd, 5th, 7th, 9th, and 10th cranial nerves on the left side of my face including rather severe hemifacial spasm. Within the last 2 weeks I have experienced 5th and 7th cranial nerve impairment on the right side of my face.I am just beginning treatment. Please try to give me the expected long term prognosis following treatment with high dosage prednisone. Please also let me know of any acknowledged top experts in this field and any research or clinical trials that I might be eligible to participate in. Also, is surgical removal of granulatamous lesions of the brain stem ever an option? Thank you.
  ===========================================================================
  Dear Debra:
  To make a diagnosis of neurosarcoidosis with certainty, the patient's case needs to match one of the following two scenarios:
  1. The presence of typical neurological features of neurosarcoidosis on the background of definite systemic sarcoidosis (usually pulmonary - commonly diagnosed on chest xray, gallium scan, or bronchoscopy, less commonly skin, lymph node, or other, commonly diagnosed by biopsy), or
  2. The demonstration on biopsy of typical sarcoid pathology (non-caseating granuloma) within the nervous system, even in the absence of demonstrable systemic sarcoidosis.
  A definite diagnosis can be difficult. Conditions such as mycobacterial or fungal infection, and lymphoma can closely mimic neurosarcoidosis clinically and even pathologically.
  Neurosarcoidosis is typically a disease process that spreads along the meningeal spaces, and secondarily involves the brain by infiltration from the meninges, often along penetrating blood vessels. The cerebrospinal fluid is usually abnormal in neurosarcoidosis. Affection of multiple cranial nerves ("polyneuritis cranialis"), which is what you describe, is a rather typical neurosarcoid presentation, although it can potentially also occur in a wide range of other conditions.
  The prognosis of neurosarcoidosis veries considerably according to presentation. The most benign cases are those with only single episodes of facial weakness. The ones that do the worst are cases that have involvement of the brain substance (tumor-like presentation, seizures, hydrocephalus, involvement of the hypothalamus-pituitary system). The latter deteriorate within a few years unless aggressively treated. Cases with polyneuritis cranialis have an intermediate prognosis.
  Because of the rarity of neurosarcoidosis, and also its seriousness, no proper controlled therapeutic trials have been conducted yet. The mainstay of treatment is corticosteroids, often in rather high doses for prolonged periods for adequate control. Such doses often have unacceptable side effects. Less conventional therapies (methotrexate, azathioprine, cyclosporine, cyclophosphamide, radiation) have been successfully used (essentially anecdotally) for better efficacy, and also as a steroid-sparing measure, in the more aggessive presentations of neurosarcoidosis. Such unconventional therapies can also be very toxic and need to be attempted only after careful thought and consideration.
  Neuro-immunologists at the Cleveland Clinic (Drs. Richard Rudick, Jeffrey Cohen, and R. P. Kinkel) have extensive experience in the treatment of difficult cases of neurosarcoidosis. In case you wish to seek a second opinion, any of them would be most happy to see you. An appointment can be made by calling [phone], or [phone] locally.
N.B. Dear Debra: neurosurgery has little role to play in the treatment of neurosarcoidosis. Exceptions are shunting of hydrocephalus,and diagnostic biopsy.