Cranio-spinal Rosai Dorfman disease: case series and literature review.

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.

Time to implement a national referral pathway for suspected cauda equina syndrome: review and outcome of 250 referrals.

INTRODUCTION: Cauda equina syndrome (CES) is a condition with significant implications and medico-legal profile. The literature still lacks large primary studies to provide strong evidence for a robust management pathway. Statements from Neurosurgical and Spinal societies support early diagnosis and imaging but this has not resulted in any noticeable shift in referral pattern. We strongly feel the need for a nationally agreed, evidence-based referral pathway in practice. We present our large series and in-depth analysis of the referral pathway to provide strong evidence for more robust referrals and management. METHODS: We reviewed 250 referrals of suspected CES (sCES) to the regional neurosurgical unit, evaluating the importance of clinical findings and the imaging pathway. RESULTS: After clinico-radiological evaluation only 32 (13%) had confirmed CES requiring urgent surgery. There was no significant difference in terms of clinical presentation between these true cases of CES (tCES) and false cases (fCES). Imaging was therefore the key rate-limiting step. MRI was the most common investigation used. 73 patients presented without imaging out of hours (OOH). In this group, investigation was delayed to the next day in 60/73 (82%) patients while only 13 (18%) patients underwent OOH MRI. Only 2 (3%) were able to have this at their local hospital. CONCLUSIONS:  As with previous studies we conclude that signs/symptoms are insufficient to identify tCES. Taking into consideration the improved outcome with early diagnosis, the importance of early scanning in diagnosing tCES, and the poor availability of OOH MRI scanning outside of neurosurgical units, we recommend a national policy of 24/7 MRI availability for cases of sCES at all hospitals with MRI scanners. This would remove the 87% of patients not requiring urgent surgery from an unnecessary and distracting referral process.

Intracranial Epidermoid Lesions: our Experience of 38 Cases.

BACKGROUND: The clinical outcome following surgical resection of intracranial epidermoid lesions is not well documented. We describe a case series evaluating the clinical presentation and outcome following surgery in patients diagnosed with these lesions. MATERIALS AND METHODS: A retrospective study conducted over a 4-year period. RESULTS: A total 38 patients who underwent surgery for intracranial epidermoid lesions were identified. Of these, 20 were supertentorial and 18 were infratentorial lesions. 33 patients presented with headache. 10 patients with supratentorial lesions presented with seizures. Patients with suprasellar epidermoid lesions presented with headache and visual problems. 14 of the 18 patients with cerebellopontine angle lesions presented with symptoms of trigeminal neuralgia. Post-operatively, 3 patients with cerebellopontine angle tumors developed facial weakness which was transient in 2 patients but permanent in 1 patient. Visual impairment in patients with suprasellar epidermoid lesions improved post-operatively. Hormonal replacement therapy was required in 2 patients. Recovery of 4 patients was complicated by Cerebrospinal fluid (CSF) leak while meningitis developed in 2 patients. One patient operated for cerebellopontine angle epidermoid died post operatively. CONCLUSIONS: Epidermoid lesions can develop anywhere in the cranial cavity. Like other space occupying lesions they often present with symptoms of raised ICP and with specific neurological deficits depending on location of the tumour. Surgical outcome is excellent with low complication rates.