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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
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Neurosarcoidosis.

Kenkichi Nozaki1, Marc A Judson

  • 1Division of Neurology, Department of Neurosciences, Medical University of South Carolina, 96 Jonathan Lucas Street, Suite 301 CSB, Charleston, SC, 29425, USA, nozaki@musc.edu.

Current Treatment Options in Neurology
|May 25, 2013
PubMed
Summary
This summary is machine-generated.

Neurosarcoidosis affects the central and peripheral nervous systems, presenting diverse neurological symptoms. Diagnosis requires ruling out other causes and confirming sarcoidosis histologically, with MRI being highly sensitive.

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Area of Science:

  • Neurology
  • Immunology
  • Systemic Inflammatory Diseases

Background:

  • Neurosarcoidosis involves the central nervous system (CNS) or peripheral nervous system (PNS), causing varied symptoms like neuropathy and meningitis.
  • Neurologic issues in sarcoidosis patients may stem from causes other than neurosarcoidosis, necessitating thorough evaluation.
  • Histologic confirmation of sarcoidosis and assessment for extra-neural involvement are crucial for diagnosis.

Purpose of the Study:

  • To outline the diagnostic approaches for neurosarcoidosis.
  • To detail treatment strategies for neurosarcoidosis, including medical and surgical interventions.
  • To emphasize the importance of differentiating neurosarcoidosis from other neurological conditions in sarcoidosis patients.

Main Methods:

  • Diagnostic workup includes evaluating extra-neural sarcoidosis and seeking histologic confirmation.
  • Gadolinium-enhanced MRI of the brain and spinal cord is the most sensitive imaging modality.
  • Cerebrospinal fluid (CSF) analysis has limited diagnostic specificity.

Main Results:

  • Corticosteroids are the primary treatment, with high-dose intravenous methylprednisolone for severe or refractory cases.
  • Immunomodulating agents like methotrexate and tumor necrosis factor-alpha inhibitors (e.g., infliximab) are used for steroid-refractory disease.
  • Surgical intervention is reserved for life-threatening complications such as hydrocephalus or spinal cord compression.

Conclusions:

  • Accurate diagnosis of neurosarcoidosis requires a comprehensive approach, including imaging and biopsy when necessary.
  • Treatment involves corticosteroids, often supplemented by immunomodulating agents for refractory cases.
  • Management addresses associated conditions like epilepsy and neuroendocrine dysfunction, with surgery for severe complications.