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Neurosarcoidosis Indication for Radiotherapy Kevin Pandya, MD June 7, 2007
Introduction <ul><li>Neurosarcoidosis  develops in 5% of patients with sarcoidosis </li></ul><ul><li>Nervous system dysfun...
Clinical Features <ul><li>Cranial Neuropathy  50-75% </li></ul><ul><li>Parenchymal Dz 25-45% </li></ul><ul><li>Aseptic men...
Diagnostic Evaluation <ul><li>Search for extraneural sarcoidosis </li></ul><ul><li>Serum ACE levels – helpful if elevated ...
Treatment <ul><li>Corticosteroids </li></ul><ul><li>Alternative agents </li></ul><ul><li>Radiotherapy </li></ul><ul><li>Su...
Radiation Therapy <ul><li>Consider in patients who fail corticosteroids and at least 2 alternative agents </li></ul><ul><l...
Case Reports <ul><li>49 y/o male with pulmonary sarcoidosis (Bruns) </li></ul><ul><ul><li>Underwent RT with disease stabil...
Extrapolated Data <ul><li>Patient with laryngeal sarcoidosis refractory to medical treatment </li></ul><ul><ul><li>Underwe...
Radiation Therapy <ul><li>RT with 12-30 Gy utilized in most case reports; minimal side effects </li></ul><ul><li>Combining...
Conclusions <ul><li>NS can have variety of different clinical presentations – consider broad differential diagnosis </li><...
References <ul><li>Bruns, F, et al. Neurosarcoidosis: an unusual indication for radiotherapy.  The British Journal of Radi...
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Neurosarcoidosis Indication For Radiotherapy

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Neurosarcoidosis Indication For Radiotherapy

  1. 1. Neurosarcoidosis Indication for Radiotherapy Kevin Pandya, MD June 7, 2007
  2. 2. Introduction <ul><li>Neurosarcoidosis develops in 5% of patients with sarcoidosis </li></ul><ul><li>Nervous system dysfunction may be the presenting feature of sarcoidosis in 50% of patients with NS </li></ul><ul><li>Any portion of the nervous system can be affected by this disorder </li></ul><ul><li>1/3 of the patient have refractory illness that is associated with considerable morbidity and possible mortality </li></ul>
  3. 3. Clinical Features <ul><li>Cranial Neuropathy 50-75% </li></ul><ul><li>Parenchymal Dz 25-45% </li></ul><ul><li>Aseptic meningitis 10-20% </li></ul><ul><li>Hydrocephalus 10% </li></ul><ul><li>Peripheral Neuropathy 5-10% </li></ul><ul><li>Myopathy 10% </li></ul>
  4. 4. Diagnostic Evaluation <ul><li>Search for extraneural sarcoidosis </li></ul><ul><li>Serum ACE levels – helpful if elevated </li></ul><ul><li>Contrast enhanced MRI </li></ul><ul><li>CSF evaluation </li></ul><ul><li>Other neurologic testing </li></ul><ul><li>Biopsy </li></ul>
  5. 5. Treatment <ul><li>Corticosteroids </li></ul><ul><li>Alternative agents </li></ul><ul><li>Radiotherapy </li></ul><ul><li>Surgery </li></ul>
  6. 6. Radiation Therapy <ul><li>Consider in patients who fail corticosteroids and at least 2 alternative agents </li></ul><ul><li>Patients undergoing chronic medical therapy might not be able to tolerate it secondary to side effects </li></ul><ul><li>Refractory disease with rapid acute progression </li></ul><ul><li>Only isolated case reports showing success with RT </li></ul>
  7. 7. Case Reports <ul><li>49 y/o male with pulmonary sarcoidosis (Bruns) </li></ul><ul><ul><li>Underwent RT with disease stabilization and partial resolution of NS symptoms </li></ul></ul><ul><li>4 patients with refractory NS treated at U of Florida (Menninger) </li></ul><ul><ul><li>2 showed regression of disease; 1 showed stabalization; 1 showed progression </li></ul></ul>
  8. 8. Extrapolated Data <ul><li>Patient with laryngeal sarcoidosis refractory to medical treatment </li></ul><ul><ul><li>Underwent RT with complete remission at 16 month follow up </li></ul></ul><ul><li>Penile sarcoidosis refractory to medical and surgical management </li></ul><ul><ul><li>Underwent RT with complete remission </li></ul></ul><ul><li>Cutaneous manifestations of sarcoid which respond to steroids, but recur on discontinuation respond with better long term resolution s/p RT </li></ul><ul><li>RT works in treatment of other granulamatous disorders such as Histocytosis X </li></ul>
  9. 9. Radiation Therapy <ul><li>RT with 12-30 Gy utilized in most case reports; minimal side effects </li></ul><ul><li>Combining RT with other immunosuppressive/immunomodulatory regimens might be of benefit, but needs study </li></ul><ul><ul><li>RT is often effective in preventing the progression of local symptoms from neurosarcoidosis, but has limited application in reversing established neurologic deficits </li></ul></ul><ul><li>Two proposed mechanisms </li></ul><ul><ul><li>Cytotoxic effect of radiation on granuloma </li></ul></ul><ul><ul><li>Radiation induced changes in tissue matrix that alter autocrine and paracrine signals </li></ul></ul>
  10. 10. Conclusions <ul><li>NS can have variety of different clinical presentations – consider broad differential diagnosis </li></ul><ul><li>Corticosteroids is the primary treatment </li></ul><ul><li>Radiotherapy should be considered in patients with refractory disease </li></ul>
  11. 11. References <ul><li>Bruns, F, et al. Neurosarcoidosis: an unusual indication for radiotherapy. The British Journal of Radiology 2004; 77: 777-79 </li></ul><ul><li>Kang S, et al. Radiation therapy for neurosarcoidosis: report of three cases from a single institution. Radiation Oncology Investigations 1999; 7: 309-12 </li></ul><ul><li>UpToDate.com </li></ul><ul><li>Agbogu BN, et al. Therapeutic Considerations in Patients with Refractory Neurosarcoidosis; Arch Neurology 1995; 52: 875-79 </li></ul><ul><li>Menninger MD, et al. Role of radiotherapy in treatment of neurosarcoidosis. Am J Clin Oncol 2003; 26: 115-8. </li></ul>

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