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

David Lacomis1

  • 1Departments of Neurology and Pathology (Neuropathology), University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15213, USA.

Current Neuropharmacology
|March 2, 2012
PubMed
Summary
This summary is machine-generated.

Neurosarcoidosis, a rare but serious condition, affects the nervous system. Diagnosis requires specific criteria, and while corticosteroids help many, other treatments are available for resistant cases.

Keywords:
Neurosarcoidosiscranial neuropathymeningitismyopathy.neuroendocrine dysfunctionperipheral neuropathysarcoidosisseizures

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Area of Science:

  • Neurology
  • Immunology
  • Pathology

Background:

  • Neurosarcoidosis is an uncommon neurological manifestation of sarcoidosis.
  • It can affect various parts of the nervous system, including cranial nerves, meninges, brain parenchyma, spinal cord, peripheral nerves, and muscle.
  • Diagnosis can be challenging, especially in isolated cases.

Purpose of the Study:

  • To summarize the current understanding of neurosarcoidosis, including its presentation, diagnostic criteria, and treatment options.
  • To highlight the challenges in diagnosing neurosarcoidosis.
  • To provide an overview of therapeutic strategies for neurosarcoidosis.

Main Methods:

  • Review of existing literature on neurosarcoidosis.
  • Analysis of diagnostic criteria, including histology (noncaseating granuloma), laboratory tests, imaging, and clinical course.
  • Summary of current and investigational treatment approaches.

Main Results:

  • Cranial nerves are the most commonly affected neurological structures.
  • Diagnostic criteria typically involve histological confirmation, supportive tests, and a compatible clinical presentation.
  • Corticosteroids are the primary treatment, offering substantial benefit to about half of patients.
  • Second-line treatments for refractory cases include azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate, and cranial irradiation.
  • Combination therapies, such as infliximab and mycophenolate mofetil, are under investigation.

Conclusions:

  • Neurosarcoidosis requires a high index of suspicion for diagnosis, especially when presenting in isolation.
  • Corticosteroids are effective for many patients, but alternative and combination therapies are crucial for refractory or intolerant individuals.
  • Further well-designed studies are needed to refine treatment strategies for neurosarcoidosis.