Neurosarcoidosis. Clinical description of 7 cases with a proposal for a new diagnostic strategy.

  • Marangoni S
  • Argentiero V
  • Tavolato B
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Abstract

OBJECTIVE: Chronic involvement of the nervous system is relatively rare in sarcoidosis. We describe 7 cases that fulfil Zajicek's criteria for neurosarcoidosis (NS) and propose some modifications to such criteria.

MATERIALS AND METHODS: The patients were admitted for various neurological syndromes: 2 cases presented with chronic lymphocytic meningitis, 4 with spinal cord symptoms, one case was initially confused with multiple sclerosis. Serological tests, immunological screening, cerebrospinal fluid (CSF) analysis, bacteriological and viral testing were performed in all patients. Spinal and cerebral MRI, gallium scan, bronchoscopy with biopsy and bronchoalveolar-lavage fluid analysis, high-resolution computed tomography (HRCT) of the chest, biopsy of the lungs, skin, mediastinal lymph-node and meninges, were useful in diagnosing NS.

RESULTS AND DISCUSSION: Laboratory tests showed serum inflammatory abnormalities, but were negative for infectious diseases, while CSF showed inflammatory signs in all patients. MRI revealed meningeal enhancement or hypertrophic pachymeningeal lesions in 4 patients, white matter abnormalities and mass lesions in 2 patients, and a spinal mass lesion in 1 patient. Gallium scan, HRCT, bronchoscopy were positive in most cases. Patients were treated with steroid and immunosuppressive therapy, with improvement in six cases. One patient died from infectious complications.

CONCLUSION: A definite diagnosis of NS requires demonstration of non-caseating granulomas affecting nervous tissues. In most cases, histological evidence of systemic disease (probable NS) is sufficient in the presence of compatible alterations in the CNS. In our patients the bronchoalveolarlavage fluid analysis, gallium scan, and chest HRCT were important for diagnosis, while serum ACE was always normal and chest radiographs were not suggestive of sarcoidosis.

Author-supplied keywords

  • Adrenal Cortex Hormones
  • Adrenal Cortex Hormones: therapeutic use
  • Adult
  • Aged
  • Brain
  • Brain: pathology
  • Brain: radiography
  • Bronchoalveolar Lavage Fluid
  • Bronchoalveolar Lavage Fluid: chemistry
  • Bronchoalveolar Lavage Fluid: cytology
  • CD4-CD8 Ratio
  • Cerebrospinal Fluid
  • Cerebrospinal Fluid: cytology
  • Electrocardiography
  • Electrophysiology
  • Female
  • Humans
  • Immunosuppressive Agents
  • Immunosuppressive Agents: therapeutic use
  • Leukocyte Count
  • Lung
  • Lung: pathology
  • Lung: radiography
  • Lymph Nodes
  • Lymph Nodes: pathology
  • Lymph Nodes: radiography
  • Magnetic Resonance Imaging
  • Male
  • Meningitis
  • Meningitis: complications
  • Multiple Sclerosis
  • Multiple Sclerosis: complications
  • Nervous System Diseases
  • Nervous System Diseases: cerebrospinal fluid
  • Nervous System Diseases: diagnosis
  • Nervous System Diseases: pathology
  • Peptidyl-Dipeptidase A
  • Peptidyl-Dipeptidase A: cerebrospinal fluid
  • Sarcoidosis
  • Sarcoidosis, Pulmonary
  • Sarcoidosis, Pulmonary: diagnosis
  • Sarcoidosis, Pulmonary: radiography
  • Sarcoidosis: cerebrospinal fluid
  • Sarcoidosis: diagnosis
  • Sarcoidosis: pathology
  • Spinal Cord Diseases
  • Spinal Cord Diseases: complications
  • Syringomyelia
  • Syringomyelia: complications
  • Tomography, X-Ray Computed

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Authors

  • Sabrina Marangoni

  • Vincenza Argentiero

  • Bruno Tavolato

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