Clival Defect with Primary CSF Rhinorrhea: A Very Rare Presentation with Challenging Management.

BACKGROUND: Primary spontaneous cerebrospinal fluid (CSF) rhinorrhea due to clival defect is an extremely rare presentation, and only 6 cases have been reported in the literature so far17. CASE DESCRIPTION: We present a unique case of a 64-year-old woman who presented with a 2-year history of ongoing CSF rhinorrhea. She had similar episode at the age of 40 years and suffered from meningitis. She underwent magnetic resonance imaging, computed tomography cisternogram, as well as computed tomography of the brain, which showed clival defect with CSF leak into the sphenoid sinus. She underwent transsphenoidal repair of the clival defect with concurrent lumbar drain insertion for CSF drainage. She returned a few months later with recurrent CSF rhinorrhea and meningitis. She was administered broad-spectrum antibiotics and underwent septal reconstruction of clival defect. CONCLUSIONS: Timely endoscopic transnasal, transsphenoidal repair of CSF leaks is the gold standard practice and could prevent devastating complications including repeated episodes of meningitis.

Spontaneous peritoneal catheter knot formation: A rare cause of ventriculoperitoneal shunt malfunction.

Ventriculoperitoneal (VP) shunting remains invaluable in the management of hydrocephalus. It is a common procedure that can be complicated by shunt malfunction due to infection, blockage and disconnection. Spontaneous peritoneal catheter knot formation causing CSF flow obstruction is a rare phenomenon. We present a case of a 12years old boy with spontaneous knot formation in the peritoneal catheter causing VP shunt obstruction and hydrocephalus.

Neurotropic cutaneous malignant melanoma with contiguous spread to spinal cord, an extremely rare presentation.

Neurotropic melanoma (NM) is a rare variant of cutaneous melanomas. Compared with conventional melanoma, NM is more locally aggressive with an increased tendency for local recurrence but less likely for nodal or distant metastases. The often amelanotic, benign appearance may lead to treatment issues such as late presentation, diagnostic delay, misdiagnosis, insufficient surgical margins, and recurrence with resulting poor outcome. To our knowledge, this is the first case report of NM with contiguous spread to the spinal cord. We present a case report of a 73-year-old male with gradual decline in mobility over the period of few months. He deteriorated very rapidly whilst inpatient with progressive myelopathy, loss of sphincter function and dysphonia with dysphagia due to involvement of lower cranial nerves. The neurotropic nature of the disease and prevalence in the head and neck region results in perineural and neural invasion with resulting neuropathies. Patient underwent posterior cervical decompression and resection of the higher cervical intramedullary spinal cord NM lesion. He recovered well with improvement of his limb weakness as well as bulbar function. Wide local excision (WLE) with adjuvant radiotherapy where indicated remains the current practice for treatment, with chemotherapy predominately being reserved as a salvage treatment for patients with disseminated disease.

Malignant paraganglioma, a rare presentation with foot drop: a case report.

Paragangliomas (or extra-adrenal paragangliomas) are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, small organs consisting mainly of neuroendocrine cells that are derived from the embryonic neural crest and have the ability to secrete catecholamines. This is in contrast to pheochromocytomas, where tumors occur in the adrenal gland. Malignant paraganglioma is a very rare presentation. We present a case report of a 56-year-old woman with acute foot drop with no previous significant background history. To the best of our knowledge, the current case represents the first case of malignant paraganglioma with acute foot drop presentation.

Successful treatment of negative pressure hydrocephalus using timely titrated external ventricular drainage: a case series.

OBJECTIVE: Negative-pressure hydrocephalus (NegPH) is a rare clinical entity characterised by enlarged ventricles and symptoms consistent with increased intracranial pressure (ICP) in the setting of negative ICP. Little has been published regarding appropriate treatment and outcomes of negative-pressure hydrocephalus patients, and no data have been published demonstrating successful therapy producing acceptable long-term outcomes. Here we present 8 cases successfully treated by titrated external ventricular drainage (TEVD), including drainage at negative (subatmospheric) pressure, and subsequent low-pressure ventriculoperitoneal shunting. METHODS: A retrospective audit of all cases of negative-pressure hydrocephalus occurring at a university teaching hospital between 2006 and 2012 was undertaken. The clinical features of these cases, results of radiological investigations, treatment, and outcome were drawn from the patients' records. RESULTS: Eight cases of NegPH were identified. All patients had at least one preceding intracranial procedure (mean number of procedures 3.0). All cases were treated using TEVD, titrated to produce between 5 and 15 mL per hour of CSF drainage, including drainage under subatmospheric pressure if this was required to maintain CSF flow. Mean delay from first negative ICP to TEVD was 1.8 days. All 8 patients demonstrated clinical improvement. TEVD resulted in improvement in Glasgow Coma Scale (mean increase 4.6, p=0.003), and increases in ICP (mean increase 8.5, p<0.001). Mean length of follow-up was 471.8 days. At follow-up, four patients had returned to pre-morbid functioning, three had a reduction in functioning attributable to their initial presentation (not NegPH), and one had died of unknown cause. Illustrative case descriptions are included. CONCLUSIONS: Negative-pressure hydrocephalus is a rare but underrecognised syndrome that can be successfully treated by timely external ventricular drainage titrated to maintain CSF flow, and subsequent low-pressure ventriculoperitoneal shunting.

Charcot-Marie-Tooth syndrome and neurofibromatosis type 1 with multiple neurofibromas of the entire spinal nerve roots.

The coexistence of polyneuropathy which has the definite clinical and electromyographical findings consistent with Charcot-Marie-Tooth (CMT) syndrome and neurofibromatosis type 1 (NF1) has infrequently been reported. We describe a patient with both CMT and NF1, who had multiple neurofibromas involving the entire spinal neural axis. In addition, he had multiple neurofibromas distributed within the ileopsoas and gluteus muscles and subcutaneous tissues. These lesions were detected readily by MRI and the patient underwent successful surgical resection of the largest tumours compressing bilateral C2 nerve roots. To our knowledge, this is the first reported case of CMT syndrome coexisting with NF1 in which multiple neurofibromas involved the entire spinal nerve roots. We discuss the diagnostic and therapeutic challenges, emphasising the role of MRI and electrophysiology in such cases and provide a literature review.