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Neurosarcoidosis masquerading as recurrent tumefactive demyelinating-appearing brain lesions: An 8-year diagnostic
Nirmalya Ray1, Russoti Das2, Mithun Sekhar3
1Department of Neuroradiology, Manipal Hospitals, Kolkata, India.
Background:
Neurosarcoidosis is a rare inflammatory condition that can occasionally present with recurrent tumefactive lesions resembling those of idiopathic demyelinating diseases. Diagnosis is frequently delayed because clinical and radiological features overlap with demyelinating disorders, and systemic signs may initially be absent.
Case Presentation:
We report a woman who first presented at 23 years of age with a tumefactive demyelinating-appearing brain lesion and, eight years later, experienced recurrence with new involvement of the brain and spinal cord. The second episode featured a new tumefactive demyelinating-appearing brain lesion, longitudinally extensive transverse myelitis, vertebral enhancement, and systemic manifestations. Imaging revealed mediastinal lymphadenopathy and multiple hypodense lesions in the liver and spleen, while histopathologic examination of a mediastinal lymph node demonstrated noncaseating granulomas, confirming sarcoidosis.
Conclusion:
To our knowledge, this is the first detailed report of neurosarcoidosis evolving from an initially isolated tumefactive demyelinating-appearing brain lesion to a later episode characterized by recurrent brain involvement with simultaneous spinal and systemic disease after a prolonged asymptomatic interval. Clinicians should consider neurosarcoidosis in the differential diagnosis of tumefactive demyelinating-appearing brain lesions, particularly when an initially isolated presentation is followed by subsequent brain, spinal, and systemic involvement, even in young patients without systemic features at onset.
Insights
Neurosarcoidosis can mimic demyelinating diseases with tumefactive brain lesions. This case highlights neurosarcoidosis evolving from isolated brain lesions to widespread neurological and systemic involvement, emphasizing diagnostic challenges.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Neurosarcoidosis presents as rare inflammatory condition.
- Tumefactive lesions mimic demyelinating diseases.
- Delayed diagnosis due to overlapping features and absent systemic signs.
Purpose of the Study:
- To report a unique case of neurosarcoidosis.
- To illustrate diagnostic challenges in tumefactive lesions.
- To highlight progression from isolated to systemic disease.
Main Methods:
- Case report of a 23-year-old woman.
- Follow-up over eight years.
- Diagnostic imaging (MRI, CT) and lymph node biopsy.
Main Results:
- Initial isolated tumefactive brain lesion.
- Recurrence with brain, spinal cord, and systemic involvement.
- Histopathology confirmed noncaseating granulomas.
Conclusions:
- First report of neurosarcoidosis evolving from isolated brain lesion to systemic disease.
- Neurosarcoidosis should be considered in tumefactive demyelinating lesions.
- Emphasizes importance of considering neurosarcoidosis even without initial systemic signs.
