HTLV-1 encephalitis.

A 53-year-old woman developed subacute onset of upper limb weakness, sensory loss and cerebellar dysfunction. She was known to have human T-lymphotropic virus type 1 (HTLV-1)-associated myelopathy. MR scan of the brain showed extensive T2 hyperintensity within the deep and subcortical white matter, with punctate contrast enhancement. Cerebrospinal fluid (CSF) was lymphocytic with very high levels of HTLV-1 provirus in both CSF and peripheral blood lymphocytes. We diagnosed HTLV-1 encephalomyelitis and started high-dose methylprednisolone followed by a slow corticosteroid taper. She recovered well and regained functional independence in the upper limbs. Neurological manifestations of HTLV-1 infection extend beyond classical 'tropical spastic paraparesis' and are under-recognised. We review the literature on HTLV-1 encephalitis and discuss its diagnosis and management.

Progressive bulbar symptomatology due to vascular brainstem compression.

Symptomatic brainstem compression from vertebral artery dolichoectasia is rare. There are no recognised diagnostic or treatment criteria to guide management of this disease. We report a case of medullary compression and cerebral ischaemia from an enlarged and tortuous vertebral artery. Our patient developed progressive dysphonia and dysphagia. Cerebral MRI revealed compression of the medulla oblongata by a right ectatic vertebral artery and a right occipital lobe infarct. Other causes of bulbar dysfunction were ruled out. He was treated with anticoagulation and underwent percutaneous endoscopic gastrostomy. We review selected literature on the presentation, diagnosis and management of this rare neurologic condition.

Lesson of the month: Oxycodone-induced leukoencephalopathy: a rare diagnosis.

Oxycodone-induced leukoencephalopathy is a rare diagnosis that should be considered in unconscious patients with appropriate history. We describe a case of a 57-year-old unconscious woman who required intubation and did not respond to naloxone infusion. The unconsciousness was initially thought to be due to hypoxic brain injury. However, a further review of brain imaging showed characteristic features of oxycodone-induced leukoencephalopathy. We describe the pathological and radiological features of this condition, and provide a concise review of the limited literature on this condition. Accurate diagnosis of this condition will be valuable to clinicians and patients in terms of their medium-term and long-term prognosis, and potential for rehabilitation.

Mural destabilization after aneurysm treatment with a flow-diverting device: a report of two cases.

BACKGROUND: Flow-diverting stents have demonstrated great promise for the treatment of cerebral aneurysms; however, clinical experience with the devices remains very preliminary. We present two cases of spontaneous delayed complications-one fatal aneurysm rupture and one symptomatic increase in aneurysm volume-following the treatment of intradural aneurysms with the Pipeline Embolization Device (PED). PRESENTATION/INTERVENTION: Two patients with unruptured, intradural aneurysms of the carotid artery underwent uneventful treatment with the PED (eV3, Irvine, California, USA). One patient, with a giant aneurysm of the carotid terminus, experienced worsening headache 5 days after the procedure and ultimately collapsed and became unresponsive. CT of the head demonstrated acute subarachnoid and intraventricular hemorrhage. The patient died the following day. A second patient with a large left posterior communicating artery aneurysm presented with progressive memory loss 3 months after PED reconstruction of the carotid artery. Although serial CT angiograms showed progressive thrombosis of the aneurysm to near-complete occlusion, MR of the brain demonstrated marked interval growth of the collective aneurysm-intra-aneurysmal thrombus mass with extensive edema throughout the adjacent left temporal lobe. CONCLUSIONS: Flow-diverting devices have demonstrated tremendous promise for the treatment of complex, unruptured cerebral aneurysms. However, experience with this novel approach to aneurysm treatment is preliminary and the consequences of its application within the cerebrovasculature remain incompletely defined. Mural destabilization resulting in delayed, spontaneous, aneurysm growth and/or rupture may occur in the days to weeks following the application of flow-diverting devices to treat previously unruptured intracranial aneurysms. A better understanding of the incidence and etiology of these complications is essential for this technology to be optimally applied.

Outcome study of the pipeline embolization device for treatment of intracranial aneurysms at a single UK institution.

BACKGROUND: The introduction of flow-diverting stents in the last decade provides an alternative endovascular treatment choice in selected intracranial aneurysms. This retrospective analysis of a UK centre's experience provides insight into clinical and radiographic outcomes. METHODS: Electronic patient records, diagnostic and procedural images and written procedural records for patients treated with the PED between August 2009 and April 2014 were reviewed. Follow-up TOF MRA was performed after treatment. Clinical and radiographic outcomes were analyzed and compared with other PED studies. RESULTS: Twenty-nine patients with 30 attempted PED treatments were reviewed representing 3.5% of the treated aneurysm patient cohort. 63.6% (21/33) of the aneurysms were wide-necked (>4 mm), 60.6% (20/33) were large or giant (≥10 mm). The mean aneurysm sac diameter was 12.0 mm; the mean neck width was 4.5 mm. Mortality and morbidity rates were 3.3% and 10.0%, respectively. The total adequate occlusion rate was 78.1% (25/32) at 18 months. The neck width of aneurysms with residual sac filling and complete occlusion differed significantly (p = 0.04). CONCLUSIONS: Highly selected aneurysms treated with a PED in a UK centre have similar occlusion and complication rates when compared to non-UK studies. Again, it appeared that delayed aneurysm rupture remained a risk for PED treatment in large or giant aneurysms. Follow-up with TOF MRA gave similar occlusion results compared to those obtained with DSA in other studies. The influence of neck size on occlusion rate should be examined in future PED studies.

Rare case of bilateral traumatic internal carotid artery dissection.

A 55-year-old man was working in a trench when the wall collapsed in on him, pinning him to the wall. On arrival in the emergency department the patient began reporting of right-sided headache. Neurological examination revealed left-sided reduced sensation with weakness. Whole-body CT scan showed right-sided flail chest and bilateral haemothorax as well as loss of flow and thinning of the distal right internal carotid artery (ICA) and loss of grey white matter differentiation in keeping with traumatic ICA dissection with a right middle cerebral artery (MCA) infarct. He was started on aspirin 300 mg once daily. 3 days postadmission the patient experienced worsening of vision and expressive dysphasia. CT angiogram showed bilateral ICA dissections extending from C2 to the skull base. The patient was managed conservatively in the stroke unit for infarction and was discharged home for follow-up in stroke clinic.

"Brain on fire": a new imaging sign.

Primary central nervous system (CNS) marginal zone B cell lymphoma is a rare condition. It has an indolent disease course and usually presents as a dural-based lesion. We present a patient with non-dural-based, primary CNS marginal zone B cell lymphoma with an unusual imaging appearance, not previously described to our knowledge.

Prevalence of recurrence and retreatment of ruptured intracranial aneurysms treated with endovascular coil occlusion.

OBJECT: Endovascular coiling is a common treatment for ruptured intracranial aneurysms. However, concerns have been raised over the durability of this treatment. The aim of this study was to establish the rate of recurrence and retreatment of coiled aneurysms treated in our unit. METHODS: We performed a retrospective analysis of 264 surviving patients with ruptured aneurysms treated by endovascular coiling between November 2003 and April 2007. Data was collected on patient age, location of aneurysm, angiogram results and any subsequent retreatment. RESULTS: Follow-up angiography performed at 6 months was available in 239 cases (91%) and revealed 158 (66%) aneurysms completely occluded, 51 (21%) had neck recurrence and 31 (13%) had significant recurrence. Thirty (12.6%) aneurysms required retreatment over a mean follow-up period of 46 (range 24-66) months. Younger age predisposed to a higher risk of recurrence and retreatment. Aneurysms of the anterior communicating and anterior cerebral arteries were less likely to recur or require retreatment (relative risk 0.42 and 0.29, respectively); aneurysms of the posterior communicating arteries were more likely to recur (relative risk 2.22). Aneurysms of the basilar and carotid arteries were more likely to undergo retreatment (relative risk 2.84 and 2.46, respectively). CONCLUSION: Long-term follow-up is required for ruptured aneurysms treated by coiling. Certain subgroups may require closer follow-up due to the increased risk of recurrence or retreatment, such as younger patients and those with aneurysms of the posterior communicating, basilar or carotid arteries.

Mural destabilization after aneurysm treatment with a flow-diverting device: a report of two cases.

BACKGROUND: Flow-diverting stents have demonstrated great promise for the treatment of cerebral aneurysms; however, clinical experience with the devices remains very preliminary. We present two cases of spontaneous delayed complications-one fatal aneurysm rupture and one symptomatic increase in aneurysm volume-following the treatment of intradural aneurysms with the Pipeline Embolization Device (PED). PRESENTATION/INTERVENTION: Two patients with unruptured, intradural aneurysms of the carotid artery underwent uneventful treatment with the PED (eV3, Irvine, California, USA). One patient, with a giant aneurysm of the carotid terminus, experienced worsening headache 5 days after the procedure and ultimately collapsed and became unresponsive. CT of the head demonstrated acute subarachnoid and intraventricular hemorrhage. The patient died the following day. A second patient with a large left posterior communicating artery aneurysm presented with progressive memory loss 3 months after PED reconstruction of the carotid artery. Although serial CT angiograms showed progressive thrombosis of the aneurysm to near-complete occlusion, MR of the brain demonstrated marked interval growth of the collective aneurysm-intra-aneurysmal thrombus mass with extensive edema throughout the adjacent left temporal lobe. CONCLUSIONS: Flow-diverting devices have demonstrated tremendous promise for the treatment of complex, unruptured cerebral aneurysms. However, experience with this novel approach to aneurysm treatment is preliminary and the consequences of its application within the cerebrovasculature remain incompletely defined. Mural destabilization resulting in delayed, spontaneous, aneurysm growth and/or rupture may occur in the days to weeks following the application of flow-diverting devices to treat previously unruptured intracranial aneurysms. A better understanding of the incidence and etiology of these complications is essential for this technology to be optimally applied.

Paroxysmal episodes, "re-build up" phenomenon and moyamoya disease.

Moyamoya disease is an idiopathic cerebral vasculopathy, which may be progressive or non-progressive. Non-idiopathic forms with an associated disease are called moyamoya-like syndrome. The electroencephalographic finding characteristically seen after hyperventilation in about 50% of children with cerebrovascular disease includes gradual frequency decrease and activation of amplitude of slow waves which appear after the disappearance or attenuation of ordinary build up. This is termed the "re-build up" phenomenon, which is rarely seen and therefore may be under-recognized. We present video telemetry during a transient ischaemic event of a child subsequently diagnosed with moyamoya-like syndrome. We highlight the potential for misdiagnosis of organic non-epileptic events. Hyperventilation during EEG should be avoided in children with known moyamoya disease.

Vascular neurosurgery following the International Subarachnoid Aneurysm Trial: modern practice reflected by subspecialization.

OBJECT: In this paper the authors' goal was to report on and examine (in the context of a large hospital with good endovascular intervention provisions) the activities of a neurosurgeon with a dedicated vascular interest in the era after the International Subarachnoid Aneurysm Trial in the United Kingdom. They also aimed to establish therapeutic trends and outcomes. METHODS: The authors reviewed the multidisciplinary team activity of 1 neurosurgeon and 2 interventional radiologists during a period of 22 months (2005-2007). They reviewed 281 aneurysm interventions; the majority was used to treat subarachnoid hemorrhage. Data analysis showed a strong preference for endovascular treatment for acute rupture (86.6 vs 13.4%), with a progressively greater role for open microsurgery in the more elective context (57% endovascular vs 43% surgical). They also reviewed 66 interventions for arteriovenous malformations, of which only 6 were surgical. These data are compared against a sample year from 2001 to 2002 (pre-International Subarachnoid Aneurysm Trial), showing comparable rates of surgically treated aneurysms versus endovascularly treated aneurysms, but an increase overall in the number of patients requiring open surgery. RESULTS: The authors found that excellent outcomes for microsurgical clipping compared with endovascular therapy can be achieved within the current climate. These and previously published data strongly support a continuing role for vascular neurosurgery as a subspecialist interest in combination with a dedicated endovascular service and a multidisciplinary team. CONCLUSIONS: Despite a trend to prefer coiling for ruptured aneurysms, the authors have shown that there is still a vital role for open surgery in the management of the ruptured and unruptured aneurysm. They consider the remaining role for surgery for arteriovenous malformations within the modern era of endovascular therapy.

Fatal granulomatous amoebic meningoencephalitis due to Balamuthia mandrillaris.

Amoebic infections of the central nervous system (CNS) are very rare and usually fatal. A 33-year-old Bolivian male injured his elbow 18 months ago in an accident and, months later, developed multiple skin lesions. He was admitted with confusion, and brain images showed large multifocal lesions with mass effect. Review of the skin biopsy revealed the presence of amoebic trophozoites within a granulomatous inflammation. Despite treatment, he continued to deteriorate and died 17 days after admission. The brain was swollen with prominent uncal herniation, and the leptomeninges showed patchy exudate. Coronal sections revealed widespread hemorrhagic and necrotic lesions. Histology confirmed granulomatous amoebic meningoencephalitis (GAM) with the presence of amoebic trophozoites and occasional cysts. Post-mortem culture confirmed Balamuthia mandrillaris. GAM due to B. mandrillaris may occur in any age group, may or may not be associated with immunosuppression, and is present worldwide. It usually has a subacute and fatal course from hematogenous dissemination of chronic skin or lung lesions.

The application of near-infrared oximetry to cerebral monitoring during aneurysm embolization: a comparison with intraprocedural angiography.

Near-infrared spectroscopy (NIRS) has been used to monitor regional cerebral oxygen saturation (rSO2) in patients at risk of cerebral desaturation during surgical and neurointerventional procedures. However, the quantitative capabilities of the method have been questioned, as has its validation compared with jugular bulb oximetry. Here, we compare NIRS data acquired during coil embolization procedures with incidence of vasospasm as detected from angiography. Thirty-two subarachnoid hemorrhage patients underwent embolization. Bilateral SomaSensor strips (Invos 4100, Somanetics) were affixed to the forehead at constant anatomic positions, avoiding frontal sinuses and scalp hair. Mean arterial pressure, SaO2, end-tidal pCO2, temperature and Hb were held within a narrow range during the procedure. Ipsilateral angiography was performed every 10 to 15 minutes. An independent neuroradiologist classified any vasospasm in the parent vessel as mild (25% baseline), moderate (50%), severe (75%), or total (100%). Of all, 15/32 (46.9%) patients developed spasm; in 2 it was severe or total. There was no significant association between World Federation of Neurological Surgeons grade and baseline rSO2 signal (either ipsilateral or contralateral to the side of the aneurysm) (P=0.598). There was no significant association between side of aneurysm and baseline rSO2 signal (P=0.243). However, episodes of angiographic spasm were strongly associated with reduction in trend ipsilateral NIRS signal (P<0.001); furthermore, the degree of spasm (especially more than 75% vessel diameter reduction) was associated with a greater reduction in same-side NIRS signal (P<0.001) (2-level random effects regression model, Stata 8.2, Stata Corp, TX). NIRS may have a useful role to play in the detection of cerebral desaturation secondary to vasospasm during neuroendovascular procedures.

Extracranial displacement of a ventricular catheter following trauma.

We report a case of complete extracranial displacement of the proximal part of a ventriculo-peritoneal shunt following trauma. The possible mechanism of the delayed displacement of the shunt is discussed. The importance of early recognition of this unusual cause of shunt dysfunction is considered.