CNS demyelination associated with immune dysregulation and a novel variant.

Background: The cytotoxic T-lymphocyte antigen-4 (CTLA-4) pathway acts as a negative immune regulator of T-cell activation and promotes self-tolerance.

Case: We report the first case of biopsy-proven central nervous system inflammatory demyelination in the context of primary immunodeficiency and a novel variant.

Conclusion: This case has significant implications for the development of novel treatments for autoimmune conditions including multiple sclerosis and further emphasises the need for caution with clinical use of CTLA-4 immune checkpoint inhibitors in those with a history of inflammatory demyelination.

Internal Carotid Artery Test Balloon Occlusion Using Single Photon Emission Computed Tomography Scan in the Management of Complex Cerebral Aneurysms and Skull Base Tumors: A 20-Year Review.

Background: Test balloon occlusion (TBO) is important in the management of complex cerebrovascular and skull base lesions when permanent occlusion (PO) of a parent artery may be indicated. Several adjuncts may be used to increase the sensitivity of TBO to predict whether PO will be tolerated. This is an observational study to evaluate the utility of internal carotid artery (ICA) TBO using single photon emission computed tomography (SPECT) scan in the management of complex vascular pathology and skull base tumors.

Tumefactive demyelination presenting during bevacizumab treatment.

We report the emergence of tumefactive demyelination during treatment with intravitreal bevacizumab (Avastin). This is of particular significance given that bevacizumab is currently being assessed as a potential treatment option for neuromyelitis optica, another demyelinating condition.

The negative predictive value of CT angiography in the setting of perimesencephalic subarachnoid hemorrhage.

Background: Perimesencephalic subarachnoid hemorrhage (PMSAH) is only rarely associated with a ruptured cerebral aneurysm and CT angiography (CTA) has very good sensitivity and specificity for aneurysm detection. The necessity for invasive imaging with digital subtraction angiography (DSA) is therefore debatable. We chose to assess the negative predictive value (NPV) of CTA in a series of patients with PMSAH treated at our institution over a 9-year period.

Is long-term follow-up of adequately coil-occluded ruptured cerebral aneurysms always necessary? A single-center study of recurrences after endovascular treatment.

Purpose: Aneurysm recurrence following coil occlusion is well recognized. However, there is controversy as to how long these patients should be followed up after coiling to detect reopening. We aimed to identify the rate of late reopening and the risk factors for reopening in a large single-center cohort of ruptured aneurysms that appeared adequately occluded at 6 months.

Endovascular therapy in hyperacute ischaemic stroke: history and current status.

This is a literature review on to the use of endovascular therapy in hyperacute ischaemic stroke secondary to large vessel occlusion (LVO). The prognosis for LVO is generally poor and the efficacy of intravenous tissue plasminogen activator (IV TPA) in the treatment of this subtype of stroke is questionable. It is well documented that recanalisation is associated with improved outcomes but IV TPA has limited efficacy in LVO recanalisation and the complication rates are higher for IV TPA in this stroke subset.

Endovascular treatment of children with cerebral venous sinus thrombosis: a case series.

Background: Cerebral venous sinus thrombosis is a potentially serious condition affecting 0.56 to 0.67 per 100,000 children annually; adverse outcomes are common.

Thrombus length estimation in acute ischemic stroke: a potential role for delayed contrast enhanced CT.

Purpose: Thrombus length has been shown to be an important determinant of recanalization using intravenous thrombolysis in hyperacute ischemic stroke. Various studies have attempted to quantify thrombus based on non-contrast CT (NCCT) or CT angiography (CTA). However, thrombus may not be seen on NCCT, and CTA may fail to delineate the distal extent of the thrombus.

A rare cause of neural foraminal widening.

The differential diagnosis for lesions causing neural foraminal widening is vast. The majority are solitary benign peripheral nerve sheath tumours, such as neurofibromas or schwannomas. We present a case of a rare cause of neural foraminal expansion secondary to a posterior thoracic extradural angiolipoma.

Trigeminal neuralgia caused by microarteriovenous malformations of the trigeminal nerve root entry zone: symptomatic relief following complete excision of the lesion with nerve root preservation.

Object: Within a series of 341 consecutive patients who underwent posterior fossa surgery for trigeminal neuralgia (TN), in five the cause was found to be a microarteriovenous malformation (micro-AVM) located in the region of the trigeminal nerve root entry zone (REZ). The surgical management and clinical outcomes of these cases are presented.

Methods: Patients were identified from a prospectively collected database of all cases of TN treated at one institution between 1980 and 2000.