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Background: Rosai-Dorfman disease (RDD) is an idiopathic nonneoplastic lymphadenopathy disorder which is characterized by lymph node enlargement, but it may also presents primarily involving a variety of extranodal sites, including central nerves system and craniospinal axis. This study reports five cases of craniospinal RDD, with review of epidemiology, clinical presentation, imaging, and histopathological features with current management strategies. Case Description: Five cases of RDD are diagnosed at Hamad General Hospital, Qatar, during 2013–2018. Two cases had dural-based cranial lesions with overlying cranial involvement while three cases were having extradural thoracic spine lesions. All cases underwent surgical intervention and confirmed by histopathology. Conclusion: Craniospinal RDD is a rare clinical presentation and poses significant diagnostic challenges preoperatively due to its similarity with other neoplastic or inflammatory diseases. Surgical option to remove compressive neural pathology provides a good clinical outcome with no recurrence in long-term follow-up.

Background: Intradural extra-arachnoidal disc herniations (IEDHs) are rare. Here, we reviewed the clinical features and magnetic resonance (MR) diagnostic features of IEDH. Case Description: A 58-year-old male presented with mid-thoracic back pain radiating to the left leg associated with ipsilateral leg weakness. The thoracic MR documented T8-T9 focal spinal canal stenosis and a ventral disc herniation. Thoracic spine computed tomography scan confirmed the diseased level and did not show any calcification. The patient underwent a right-sided transpedicular extracavitary approach for disc excision. At surgery, IEDH was identified with soft cartilaginous consistency and was morcellated to remove in piece meal while preserving the integrity of the arachnoid layer. Conclusion: IEDH poses a significant challenge for its diagnosis and management. A careful preoperative MR imaging review with a high index of clinical suspicion may ensure a good clinical outcome.

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