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Cranio-spinal Rosai Dorfman disease: case series and literature review
- Source :
- British journal of neurosurgery. 33(2)
- Publication Year :
- 2017
-
Abstract
- Rosai-Dorfman disease (RDD) is a rare condition similar to lymphomas, presenting with cervical lymphadenopathy in young adults. Extra-nodal involvement is relatively common but involvement of the central nervous system (CNS) is rare. Cranial RDD presents with symptoms of raised intracranial pressure, focal or generalised seizures, while spinal RDD presents with pain, peripheral neurological deficits and radiculopathy. In contrast to other similar neoplastic or degenerative conditions affecting the CNS, RDD is a benign, non-infective, granulomatous disorder. Radiologically cranio-spinal RDD often mimics commoner dural-based lesions like meningioma, with only subtle radiological differentiating findings on Magnetic Resonance Imaging (MRI). The histopathology of RDD is diagnostic. Surgical excision is preferred modality of treatment. However, adjuvant therapies like steroids and radiation may help controlling residual or recurrent disease. There are multiple sporadic reports and short case publications in the literature, often focusing on a particular aspect of RDD. In this study, authors aim to present five cases of craniospinal RDD, and comprehensive review of literature and highlight neurological complications of systemic RDD.
- Subjects :
- Adult
Male
medicine.medical_specialty
Pain
Disease
Meningioma
Diagnosis, Differential
03 medical and health sciences
0302 clinical medicine
Cervical lymphadenopathy
medicine
Meningeal Neoplasms
Humans
Radiculopathy
Rosai–Dorfman disease
Brain Diseases
medicine.diagnostic_test
business.industry
Magnetic resonance imaging
General Medicine
Middle Aged
medicine.disease
Magnetic Resonance Imaging
Emperipolesis
Research Design
030220 oncology & carcinogenesis
Radiological weapon
Surgery
Histopathology
Female
Spinal Diseases
Neurology (clinical)
Radiology
Dura Mater
medicine.symptom
Histiocytosis, Sinus
Intracranial Hypertension
business
Tomography, X-Ray Computed
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 1360046X
- Volume :
- 33
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- British journal of neurosurgery
- Accession number :
- edsair.doi.dedup.....18156b9e4b092dca0a4ddbe41c4bfda3