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.

Rare case of primary spinal ependymomatosis occurring in a 26-year-old man: a case report.

INTRODUCTION: The authors report a rare case of primary spinal ependymomatosis in a young adult man. Multiple primary ependymomatous lesions were seen on magnetic resonance imaging and no anaplasia was identified on the surgical-pathological analysis. The aetio-pathological mechanism and surgical significance of this rare occurrence is discussed. CASE PRESENTATION: A 26-year-old man of Polish origin presented with a ten-day history of pain in the left leg and lower back. This was followed by difficulty in urinating and a decrease in sensation in both legs. Examination revealed pyramidal signs and mild weakness in both lower limbs. He had early sphincter involvement requiring catheterization. Magnetic resonance imaging of the brain was normal. However, that of the spinal cord revealed multiple intradural spinal lesions, both intra- and extramedullary, extending from the cervical cord down to the cauda equina roots. T12-L1 laminectomy was performed. Multiple intradural, extra- and intra-medullary tumors were seen. After the operation, the patient deteriorated with a sensory level at T4. Post-operative cranio-spinal radiotherapy was administered but there was no clinical improvement in the lower limbs. CONCLUSION: Primary spinal ependymomatosis is a rare phenomenon involving multiple spinal segments in the absence of a primary intracranial tumor. Radical excision is unrealistic in this condition. Biopsy followed by radiotherapy is the preferred method of treatment.

Central neurocytoma: a multi-disciplinary review.

Benign central neurocytoma (CN) is a rare neuronal tumour of the central nervous system recognised since the early eighties. More than 300 articles have been published in the literature, mostly comprising of case reports and short series from individual specialties. These tumours, though normally benign, are more often likely to recur after surgery than previously thought. A multi-modality team involvement, therefore, has become increasingly necessary for their optimum management. In this article, the authors from various neurosciences sub-specialties, with a specific interest and experience in managing CN, review the epidemiology, clinical features, pathological findings, radiological characteristics and surgical treatment, with an emphasis on the latest developments in their histology, molecular biology and adjuvant treatment modalities for recurrent or residual disease.

Facial herpes zoster infection precipitated by surgical manipulation of the trigeminal nerve during exploration of the posterior fossa: a case report.

INTRODUCTION: We present a case of herpes zoster infection (shingles) precipitated by surgical manipulation of the trigeminal nerve root during an attempted microvascular decompression procedure. The pathogenesis of this phenomenon, as well as the importance and role of prophylactic acyclovir in its management, are discussed. CASE PRESENTATION: A 54-year-old Caucasian man with a classical long-standing left-sided V2 and V3 division primary trigeminal neuralgia refractory to medical management, underwent posterior fossa exploration for microvascular decompression via a standard retromastoid craniectomy. The patient had immediate and complete relief from pain. Three days after the operation, he developed severely painful vesicles with V2 and V3 dermatomal distribution. Rather than the classical paroxysmal, lancinating type of trigeminal neuralgia, the pain experienced by the patient was of a constant burning nature. A clinical diagnosis of herpes zoster (shingles) was made after smear confirmation from microbiological testing. The patient was commenced on antiviral treatment with acyclovir. His vesicular rash and pain gradually subsided over the next two weeks. He remains asymptomatic one year later. CONCLUSIONS: Postoperative shingles precipitated by trigeminal nerve manipulation during surgery for trigeminal neuralgia can be a distressing and demoralizing experience for the patient. A careful preoperative history, early recognition, and prompt antiviral therapy is necessary.

Aneurysm at the fenestrated anterior cerebral artery: surgical anatomy and management.

The authors report a rare case of an aneurysm arising at fenestrated anterior cerebral artery. The embryogenesis of this anatomical variation is discussed along with the review of relevant literature. Management of such lesions depends upon the location and morphology of the aneurysms and intracranial vascular anatomy. The authors discuss various management options and highlight possible technical difficulties that can be encountered in the surgical management of this rare sub-group of aneurysms.

Primary malignant melanoma of the cerebellopontine angle: a diagnostic dilemma: case report.

OBJECTIVE: To present a rare case of a primary malignant melanoma of the central nervous system presenting as a cerebellopontine angle (CPA) tumor and to delineate aspects of the clinical presentation and magnetic resonance imaging scan characteristics that may suggest this unusual condition. CLINICAL PRESENTATION: The clinical presentation consisted of a short duration of right-sided sensorineural hearing loss, facial weakness, and ataxia in a previously healthy man. Brain magnetic resonance imaging scans showed a right-sided CPA tumor exhibiting shortening of T1 and T2 relaxation times, but overall neuroradiological features were not consistent with any commonly occurring CPA tumors. INTERVENTION: Gross total excision of the lesion was accomplished via a right suboccipital craniectomy. Histological examination revealed a malignant melanoma. A detailed search excluded extracranial primary melanoma. CONCLUSION: Correlation of clinical and imaging findings offer the most important clues in the diagnosis of such unusual primary malignant tumors of the CPA. A history of rapid onset of audiovestibular symptoms, presence of facial palsy, and shortening of T1 and T2 relaxation times on magnetic resonance imaging scans should arouse the clinician's suspicions.

Isolated abducent nerve palsy after microvascular decompression for trigeminal neuralgia: case report.

OBJECTIVE AND IMPORTANCE: Microvascular decompression (MVD) is a safe and effective treatment for trigeminal neuralgia. Isolated postoperative ipsilateral abducent nerve palsy is an extremely rare complication of this procedure and has not been reported before. The author wishes to report this rare complication, discuss its aetio-pathogenesis and the natural course. CLINICAL PRESENTATION: A 57-year-old man underwent MVD for right sided medically refractory trigeminal neuralgia based on the MRI finding of ectatic basilar artery compression at its root entry zone. The procedure was uneventful with complete relief of pain. However, he developed isolated ipsilateral sixth nerve palsy following surgery that lasted for six weeks. INTERVENTION: Anterior displacement of tortuous and dilated basilar artery to create space between the artery and the nerve during MVD operation probably resulted in inadvertent neuropraxia to the abducent nerve that was located immediately anterior to the artery but was not in the surgical field. Immediate sixth nerve palsy resulted post-operatively. A conservative route was adopted with spontaneous improvement of the extraocular eye movements after six weeks. CONCLUSION: Isolated abducent nerve damage is a rare complication of the MVD procedure. Surgical manipulation of the dilated and tortuous vessels located anterior to the trigeminal nerve may cause indirect stretching of the delicate abducent nerve with resultant neuropraxia. This complication is difficult to recognize and prevent intra-operatively as the nerve lies on the far side of the artery. Fortunately, spontaneous recovery of the neural function seems to be the most likely outcome.