Shelley Renowden

The relation of infantile spasms, tubers, and intelligence in tuberous sclerosis complex

Background: The aetiology of the learning difficulty in tuberous sclerosis is debated. It may be related to the amount of tubers in the brain or caused by the infantile spasms that occur in early life. Aims: To examine the relative contributions to final intelligence (IQ) made by both cerebral tubers and infantile spasms. Methods: As part of an epidemiological study of tuberous sclerosis in the south of England, patients were recruited who were able to undergo magnetic resonance imaging (MRI) without the need for an anaesthetic. Epilepsy history was determined by interview and review of clinical records. IQ was assessed using either Wechsler intelligence scales or Raven’s matrices. Results: A total of 41 patients consented to have an MRI scan. IQ scores were normally distributed about a mean of 91. Twenty six patients had a positive history of epilepsy, and 11 had suffered from infantile spasms. There was a significant relation between the number of tubers and IQ. Infantile spasm status partly confounded the relation between tubers and IQ, but did not render the relation statistically insignificant. The relation between infantile spasms and learning difficulty remained strong even when controlling for the number of tubers.

Cerebral venous sinus thrombosis.

A comprehensive synopsis on cerebral venous thrombosis is presented. It emphasizes the various aetiologies, the wide clinical spectrum and the unpredictable outcome. Imaging techniques and pitfalls are reported and the therapeutic options are discussed.

Treatment of refractory neurosarcoidosis with Infliximab

Background Neurological involvement in sarcoidosis is serious and often aggressive. Many patients respond to steroids but some show a progressive course despite treatment with steroids and even more potent immunosuppressive drugs. Objective The aim of this study was to describe our experience in the treatment of refractory neurosarcoidosis with Infliximab—its effect on the course of the disease and side effects. Methods A series of four patients are reported with neurosarcoidosis refractory to treatment with steroids combined with various immunosuppressive drugs in whom Infliximab was used. Results A good response, with improvement or stabilisation of the neurological condition, was seen in all cases, without significant side effects. Infliximab is a chimeric monoclonal antibody that neutralises the biological activity of tumour necrosis factor α, a cytokine thought to play an important role in the pathophysiology of sarcoidosis. Conclusion Our experience using Infliximab adds to the growing evidence that it may fulfil a useful role in cases of refractory neurosarcoidosis.

Neuroradiological aspects of subdural haemorrhages

Aims: To review the neuroimaging of a series of infants and young children admitted to hospital with subdural haemorrhage (SDH). Methods: Neuroradiological investigations of 74 children under 2 years of age, from South Wales and southwest England, in whom an SDH or subdural effusion had been diagnosed between 1992 and 2001, were reviewed. Two paediatric neuroradiologists blinded to the original radiological report reviewed all the relevant images. Results: Neuroradiological review of images identified radiological features which were highly suggestive of non-accidental head injury (NAHI). Interhemispheric haemorrhages and SDHs in multiple sites or of different densities were almost exclusively seen in NAHI. MRI was more sensitive in identifying SDHs of different signal characteristics, posterior and middle cranial fossa bleeds, and parenchymal changes in the brain. CT scans, if performed with suboptimal protocols, were likely to miss small subdural bleeds. Conclusions: Guidelines for neuroimaging in suspected NAHI are recommended. A radiologist with experience in NAHI should report or review these scans. The initial investigation should be CT, but MRI will also be necessary in most cases. Head CT should be an integral part of the skeletal survey in all infants less than 6 months of age referred for child protection investigation, and in children less than 2 years where child abuse is suspected and there are neurological signs, retinal haemorrhages, or fractures.

Subependymal nodules, giant cell astrocytomas and the tuberous sclerosis complex: a population-based study

Objectives: (1) In a population-based study of tuberous sclerosis (TSC), to identify the number of patients presenting with symptomatic giant cell astrocytomas (GCAs); (2) within a subset of this population, to identify the number who would be diagnosed with GCAs on predetermined radiological criteria. Methods: Patients with TSC in Wessex (a geographical region of England) were identified, and their medical history determined. A subset were invited to have a cranial MRI if they did not have a history of a symptomatic GCA and if they were likely to tolerate cranial imaging without a general anaesthetic. Scans were performed according to a standard protocol on a single scanner and were reported blindly by a neuroradiologist. Results: 179 people were identified with TSC. Ten of these had a history of treatment for a symptomatic GCA. Forty-one of the remainder had a cranial MRI. Thirty-nine of these had subependymal nodules, of whom 24 (59%) had at least one (maximum 11) that showed enhancement with gadolinium. In seven (17%), the lesion was >1 cm, and all of these lesions showed gadolinium enhancement. Conclusions: In this study, the proportion of patients with TSC who had a history of symptomatic GCA was 5.6%. In the subset without such a history, who underwent imaging, the number diagnosed as having a GCA on radiological criteria was much higher (59% gadolinium enhancement and 17% >1 cm in size). Screening for GCAs (performing scans on asymptomatic patients with TSC) would therefore identify large numbers of patients who had not presented with symptoms. This finding leads us to recommend that screening should not be undertaken.

Detachable coil embolisation of ruptured intracranial aneurysms: A single center study, a decade experience

OBJECTIVE The introduction of detachable coils revolutionised the management of patients with intracranial aneurysms and is now considered a first-line treatment in our institution. The purpose of this study was to review 10 years of experience with this method. METHODS A retrospective review of prospectively collected data on 711 patients undergoing endovascular treatment of ruptured intracranial aneurysm between 1996 and 2005 with regard to technical feasibility, procedural complications, rebleeding, anatomical outcome, need for retreatment and overall clinical outcome. RESULTS Endovascular treatment failed in 25 aneurysms from a total of 717 (4%). Aneurysm rupture complicated 37 procedures (4.7%) leaving 10 patients permanently disabled or dead (1.3%). Thromboembolic events complicated 35 procedures (4.5%) leaving 8 patients permanently disabled or dead (1%). One other patient died because of fatal parent vessel rupture. Further 6 procedures were complicated by arterial dissection and 18 by coil loop protrusion, however all of these patients achieved independent recovery. Overall morbidity-mortality was 2.9%. Further subarachnoid hemorrhage occurred in 16 patients (2.3%), 12 of which died. Altogether, 121 aneurysms from 511 (24%) were recanalized on follow up angiography, 52 required retreatment (7.1%). At 6 months follow up, 580 patients (82%) were independent, while 130 patients (18%) were disabled or dead. CONCLUSION Detachable coil embolisation of intracranial aneurysms is a very feasible treatment method associated with a small risk of permanent morbidity-mortality. Risk of further bleeding is small, but related with devastating outcome. Approximately 25% of aneurysms will recanalize and 7% will require retreatment. Despite these shortcomings, vast majority of patients will achieve independent recovery.

Memory loss resulting from fornix and septal damage: impaired supra-span recall but preserved recognition over a 24-hour delay.

Despite increasing evidence that the fornix is important for memory, uncertainty remains about the exact nature of subsequent impairments arising from damage to this tract. This uncertainty is often created by pathology in additional brain structures. The present study involved a young man, DN, who had almost complete bilateral loss of the rostral columns of the fornix and much of the surrounding septum in the left hemisphere following the surgical removal of a cavernous angioma. Quantitative MRI analyses of structure size, normalized to intracranial volume, showed no difference in any of the additional brain regions measured, apart from those areas removed to expose the tumor. DN showed a marked, stable anterograde memory impairment that was still present 4 years postsurgery. In contrast, DN performed within normal levels on most tests of recognition memory. This sparing was most striking when given a 24-hr delay between study and test of the Warrington Recognition Memory Test. This recall/recognition dissociation provides further evidence for neuroanatomical divisions within recognition memory processes.

Some patients with multiple sclerosis have neurovascular compression causing their trigeminal neuralgia and can be treated effectively with MVD: Report of five cases

The role of trigeminal ganglion percutaneous injection and radio-frequency lesioning procedures for the treatment of trigeminal neuralgia (TGN) in multiple sclerosis (MS) is well established. There is general acceptance that microvascular decompression (MVD) cannot be an appropriate treatment due to the view that the underlying aetiology is a demyelinating plaque affecting the root entry zone of the trigeminal pathway. Recently, MR-imaging has been used in the preoperative investigation of this group of patients demonstrating that neurovascular compression can occasionally be the responsible mechanism and that MVD can be the treatment of choice. We present five cases with MS and TGN. All the patients had failed to respond to medical treatment or percutaneous procedures. Magnetic resonance imaging demonstrated evidence of neurovascular compression in four cases. All the patients underwent MVD. Postoperatively four of the five patients made an uncomplicated recovery, were pain-free and fully satisfied with the result (mean follow-up 38.75 months; range 8 – 59 months). One patient developed recurrent pain 1 week following surgery and went on to have a total sensory rhizotomy. TGN in MS can be caused by neurovascular compression, which may be identified on MR-imaging. MVD has offered satisfactory short-term outcome for at least 2 years and does not inflict sensory loss. Longer follow-up will determine whether the outcome in MS patients will be as successful as in the TGN patients who do not suffer from MS.

Endovascular therapy for acute basilar artery occlusion: a review of the literature

Basilar artery occlusion is an infrequent form of acute stroke; clinical outcomes are heterogeneous, but the condition can be fatal. There is a lack of randomized controlled trial data in this field. Case series suggest that patients who are recanalized have much better outcomes than those who are not, and it is generally accepted that intra-arterial techniques achieve high rates of recanalization. Controversially, several studies, including a meta-analysis and registry-based investigation, that have compared intravenous thrombolysis (IVT) and intra-arterial treatment suggest similar outcomes. However, there are many potential sources of bias in each of these studies, precluding a firm conclusion. Indeed, there are many confounding factors that can influence the outcome including severity of presentation, site of occlusion, clot load, degree of collateral flow, timing of therapy, agent used for recanalization and dose of thrombolytic agent. Additionally, pretreatment infarct core imaging using diffusion-weighted imaging and the posterior circulation Acute Stroke Prognosis Early CT Score (pc-ASPECTS) scoring systems have been shown to predict outcome and therefore may be useful in selecting patients for aggressive therapy. Protocols combining intravenous agents such as glycoprotein IIb/IIIa receptor antagonists or thombolytics agents with intra-arterial techniques (‘bridging’ therapy) have shown encouraging improvements in neurological outcome and survival. Furthermore, initial case series describing the use of mechanical clot extraction devices or aspiration catheters suggest high rates of recanalization. What would be useful is a randomized trial comparing IVT, endovascular approaches and a combined IVT/endovascular approach. However, the small numbers of patients and multiple confounding factors are barriers to the development of such a trial.

Endovascular Treatment of 300 Consecutive Middle Cerebral Artery Aneurysms: Clinical and Radiologic Outcomes

Controversy about the best treatment method for MCA aneurysms persists and these authors report the results of endovascular treatment of these lesions in a large series in which 295 such aneurysms were treated during a 17-year period and the results retrospectively analyzed. Complete occlusion was achieved in 94% and treatment failed in 4.3%. Procedure-related morbidity and mortality occurred in nearly 8%. Death occurred in 13.6% of patients and 4.3% needed to be re-treated. Thus, endovascular treatment of MCA aneurysms is acceptable as a primary treatment. BACKGROUND AND PURPOSE: There is controversy as to the best mode of treating MCA aneurysms. We report the results of a large endovascular series of patients treated at our center. MATERIALS AND METHODS: This study was a retrospective analysis of a prospectively acquired data base. All patients with saccular MCA aneurysms treated between November 1996 and June 2012 were included. World Federation of Neurosurgical Societies grade, aneurysm site, size, and aneurysm neck size were recorded, along with clinical outcome assessed with the Glasgow Outcome Scale and radiographic occlusion assessed with the Raymond classification at 6 months and 2.5 years. RESULTS: A total of 295 patients with 300 MCA aneurysms were treated including 244 ruptured aneurysms (80.7%). The technical failure rate was 4.3% (13 patients). Complete occlusion or neck remnant was achieved in 264 (91.4%). Complications included rupture in 15 patients (5%), thromboembolism in 17 patients (5.7%), and early rebleeding in 3 patients (1%). Overall permanent procedural-related morbidity and mortality were seen in 12 patients (7.8%). Of the ruptured aneurysms, 189 (79.4%) had a favorable clinical outcome (Glasgow Outcome Scale score, 4–5). A total of 33 patients (13.6%) died. On initial angiographic follow-up, aneurysm remnant was seen in 18 aneurysms (8.1%). A total of 13 patients (4.3%) were re-treated. CONCLUSIONS: Our experience demonstrates that endovascular treatment of MCA aneurysms has an acceptable safety profile with low rates of technical failure and re-treatment. Therefore, coiling is acceptable as the primary treatment of MCA aneurysms.