Craniosynostosis.

Craniosynostosis is a complex condition, characterized by the premature fusion of one of more of the cranial sutures. They can be seen individually or as part of multisystem syndromes. This review uses computed tomography (CT) with three-dimensional reconstructions to help describe some of the types and classifications of craniosynostosis, as well as describing some of the associations and the management of craniosynostosis.

Case report of glomus jugulare tumour associated with a posterior fossa cyst.

A subgroup of complex glomus jugulare tumours exists. This includes: multiple, giant or neuropeptide-secreting lesions; those associated with other lesions, such as dural arterio-venous malformation or an adrenal tumour; and tumours in which there has been previous treatment with adverse outcome. To our knowledge, we present the first case of a glomus jugulare tumour associated with a posterior fossa cyst. This entity should be included in the subgroup of complex glomus jugulare tumours.

Intramedullary tumours in patients with neurofibromatosis type 2: MRI features associated with a favourable prognosis.

AIM: To assess the magnetic resonance imaging (MRI) features and natural history of intramedullary tumours in patients with neurofibromatosis type 2 (NF2). MATERIALS AND METHODS: Eleven NF2 patients with intramedullary spinal cord tumours were identified from the database of the multidisciplinary NF2 clinic. All the imaging studies of these patients were individually reviewed by two neuroradiologists to evaluate the size, number, location, imaging characteristics, and interval growth of the intramedullary tumours. RESULTS: Two of the 11 patients had lesions that required surgery. Both these lesions were in the cervical region, and extended over three and five segments respectively. Nine patients with a mean imaging follow-up period of 77 months had lesions that remained stable, apart from the development of small peritumoral cysts in three. The lesions were well circumscribed, often multiple, usually less than 1cm in diameter, and were most frequently found in the cervical cord. CONCLUSION: The majority of intramedullary tumours in NF2 patients are very slow growing and share certain MRI features that differ from those of progressive or symptomatic lesions.

Marrow proliferation as a cause of hearing loss in beta-thalassaemia major.

OBJECTIVE: The aim of this report was to highlight the fact that hearing loss in thalassaemia patients can be related to marrow expansion affecting the ossicles, resulting in a conductive loss. CASE REPORT: A six-year-old boy with transfusion-dependent beta-thalassaemia developed a unilateral hearing loss shortly after commencing desferrioxamine therapy. Ototoxicity was assumed, but the deficit was later found to be of a conductive nature, due to marrow proliferation within the ossicular chain as a consequence of the disease process--a phenomenon previously unreported in the literature. CONCLUSION: It is important to elucidate the precise nature of new onset hearing loss in patients receiving iron chelation therapy, in order to avoid unnecessary cessation of much needed medication, on the assumption of ototoxicity.

Imaging manifestations of neck masses in the immunocompromised host.

Human immunodeficiency virus (HIV) infection, haematological malignancy, and immunosuppression for transplantation and autoimmune disorders have led to a large increase in immunocompromised patients. Neck masses are relatively common in this patient group and include both opportunistic and severe manifestations of common infections, benign hyperplasia, and primary or secondary malignancies. Although biopsy may be necessary for definitive diagnosis, features on cross-sectional imaging may suggest a specific diagnosis or limit the differential diagnosis and facilitate optimal patient management. This article will review critical aspects of neck anatomy, illustrate the spectrum of imaging features, and discuss the interpretative pearls and pitfalls when evaluating neck masses in immunocompromised patients.

Immature teratoma arising from a benign cortical abnormality.

CASE REPORT: The authors report the case of an immature teratoma of the left parieto-occipital region in a 13-year-old girl. The patient had a computed tomographic (CT) scan of the brain aged 10 months old, following a minor head injury. This demonstrated an abnormality in the same region, which had been reported as 'a cortical malformation'. DIAGNOSIS: We propose that the lesion on the original imaging is a mature teratoma or other silent dystopic germ cell element that subsequently transformed into the immature teratoma. DISCUSSION: The potential triggers for such a transformation and the management of patients with similar incidental radiological findings are discussed.

Population-based study of event-rate, incidence, case fatality, and mortality for all acute vascular events in all arterial territories (Oxford Vascular Study).

BACKGROUND: Acute coronary, cerebrovascular, and peripheral vascular events have common underlying arterial pathology, risk factors, and preventive treatments, but they are rarely studied concurrently. In the Oxford Vascular Study, we determined the comparative epidemiology of different acute vascular syndromes, their current burdens, and the potential effect of the ageing population on future rates. METHODS: We prospectively assessed all individuals presenting with an acute vascular event of any type in any arterial territory irrespective of age in a population of 91 106 in Oxfordshire, UK, in 2002-05. FINDINGS: 2024 acute vascular events occurred in 1657 individuals: 918 (45%) cerebrovascular (618 stroke, 300 transient ischaemic attacks [TIA]); 856 (42%) coronary vascular (159 ST-elevation myocardial infarction, 316 non-ST-elevation myocardial infarction, 218 unstable angina, 163 sudden cardiac death); 188 (9%) peripheral vascular (43 aortic, 53 embolic visceral or limb ischaemia, 92 critical limb ischaemia); and 62 unclassifiable deaths. Relative incidence of cerebrovascular events compared with coronary events was 1.19 (95% CI 1.06-1.33) overall; 1.40 (1.23-1.59) for non-fatal events; and 1.21 (1.04-1.41) if TIA and unstable angina were further excluded. Event and incidence rates rose steeply with age in all arterial territories, with 735 (80%) cerebrovascular, 623 (73%) coronary, and 147 (78%) peripheral vascular events in 12 886 (14%) individuals aged 65 years or older; and 503 (54%), 402 (47%), and 105 (56%), respectively, in the 5919 (6%) aged 75 years or older. Although case-fatality rates increased with age, 736 (47%) of 1561 non-fatal events occurred at age 75 years or older. INTERPRETATION: The high rates of acute vascular events outside the coronary arterial territory and the steep rise in event rates with age in all territories have implications for prevention strategies, clinical trial design, and the targeting of funds for service provision and research.

Change in stroke incidence, mortality, case-fatality, severity, and risk factors in Oxfordshire, UK from 1981 to 2004 (Oxford Vascular Study).

BACKGROUND: The incidence of stroke is predicted to rise because of the rapidly ageing population. However, over the past two decades, findings of randomised trials have identified several interventions that are effective in prevention of stroke. Reliable data on time-trends in stroke incidence, major risk factors, and use of preventive treatments in an ageing population are required to ascertain whether implementation of preventive strategies can offset the predicted rise in stroke incidence. We aimed to obtain these data. METHODS: We ascertained changes in incidence of transient ischaemic attack and stroke, risk factors, and premorbid use of preventive treatments from 1981-84 (Oxford Community Stroke Project; OCSP) to 2002-04 (Oxford Vascular Study; OXVASC). FINDINGS: Of 476 patients with transient ischaemic attacks or strokes in OXVASC, 262 strokes and 93 transient ischaemic attacks were incident events. Despite more complete case-ascertainment than in OCSP, age-adjusted and sex-adjusted incidence of first-ever stroke fell by 29% (relative incidence 0.71, 95% CI 0.61-0.83, p=0.0002). Incidence declined by more than 50% for primary intracerebral haemorrhage (0.47, 0.27-0.83, p=0.01) but was unchanged for subarachnoid haemorrhage (0.83, 0.44-1.57, p=0.57). Thus, although 28% more incident strokes (366 vs 286) were expected in OXVASC due to demographic change alone (33% increase in those aged 75 or older), the observed number fell (262 vs 286). Major reductions were recorded in mortality rates for incident stroke (0.63, 0.44-0.90, p=0.02) and in incidence of disabling or fatal stroke (0.60, 0.50-0.73, p<0.0001), but no change was seen in case-fatality due to incident stroke (17.2% vs 17.8%; age and sex adjusted relative risk 0.85, 95% CI 0.57-1.28, p=0.45). Comparison of premorbid risk factors revealed substantial reductions in the proportion of smokers, mean total cholesterol, and mean systolic and diastolic blood pressures and major increases in premorbid treatment with antiplatelet, lipid-lowering, and blood pressure lowering drugs (all p<0.0001). INTERPRETATION: The age-specific incidence of major stroke in Oxfordshire has fallen by 40% over the past 20 years in association with increased use of preventive treatments and major reductions in premorbid risk factors.

Brain haemorrhage in five infants with coagulopathy.

Most intracranial haemorrhages in infants after the neonatal period are secondary to non-accidental injury. Occasionally brain haemorrhages in non-mobile infants are due to an inherited coagulopathy. This may often be diagnosed with a coagulation screen on admission. Little is known about the neurological outcome of infants in the latter group. Five infants are described who presented with acute spontaneous brain haemorrhage secondary to an inherited coagulopathy (n = 3) and vitamin K deficiency in alpha(1) antitrypsin deficiency (n = 1) and Alagille's syndrome (n = 1). Despite the critical clinical presentation and the severe imaging findings, these five infants made a good neurological recovery. Infants presenting with spontaneous ICH due to a significant (inherited) coagulopathy are usually easy to differentiate from non-accidental shaking injury; their bleeding pattern within the brain seems different from non-accidental shaking injury and neurodevelopment outcome appears better.

Differential diagnosis of intracranial lesions with high signal on T1 or low signal on T2-weighted MRI.

Magnetic resonance imaging (MRI) is the preferred technique for examining intracranial pathology in the non-acute setting in most cases. The aim of this review is to demonstrate the imaging appearances on unenhanced spin-echo MRI of lesions that show characteristic, unusual signal changes, which occur due to the presence of fat, cholesterol, paramagnetic substances, rapidly flowing fluids, air, cortical bone or calcification. The nature of the MRI signal, together with the anatomical location of the lesion should allow the provision of a narrow differential diagnosis, before the use of intravenous contrast medium or additional imaging sequences.

How high do the subclavian arteries ascend into the neck? A population study using magnetic resonance imaging.

BACKGROUND: The relationship between the larynx and the subclavian arteries was studied in a series of magnetic resonance images (MRIs) from 50 patients without neck pathology. METHODS: The vertical distances of the excursion of the subclavian arteries into the neck was measured, as was the distance from the cricoid cartilage to the highest point of this excursion. Statistical analysis allows the probability of any given cricoid-subclavian distance occurring in the population to be estimated. RESULTS: The mean (SD) excursion of the right subclavian artery above the clavicle was 10.4 (11.4) mm. The mean (SD) distance from the cricoid cartilage to the right subclavian artery was 30.6 (14.3) mm, and the data showed a high degree of variance. There was a linear relationship between neck length and cricoid-subclavian distance (r=0.58), which explained some of the variance in the latter, but there was also wide individual variance, which was independent of this regression. CONCLUSIONS: When performing a percutaneous tracheostomy, a 'safe' distance between the incision site and subclavian artery cannot be assumed or reliably predicted from the neck length.

Transverse sinus thrombosis and IVIg treatment: a case report and discussion of risk-benefit assessment for immunoglobulin treatment.

A 54 year old woman presented with symptoms resulting from a thrombosis of the lateral transverse and sagittal sinuses the day after an infusion of intravenous immunoglobulin (IVIg) replacement treatment. She had previously suffered a milder episode after IVIg. Following recurrent bacterial chest infections and sinusitis for more than 40 years, a diagnosis of IgG1 deficiency had been made two years earlier, after exclusion of other causes. She made a good recovery from the thrombosis but high platelet counts were investigated and primary thrombocythaemia was diagnosed. Investigation of humoral immunity revealed protective amounts of IgG antibodies to pathogens, and because the previous IgG1 deficiency had resolved IVIg infusions were not restarted. She made a good response to treatment with hydroxyurea, with improvement of the headaches and lowering of the platelet counts. Prophylactic antibiotics reduced the number of bacterial chest infections and nasal corticosteroids improved the chronic sinusitis. This case is presented to highlight the need to look for other contributing factors for severe recurrent headaches after IVIg treatment, and to consider the risk of thrombosis even when replacement doses of IVIg are used. It is also important to emphasise the need to ensure that an isolated IgG subclass deficiency is not transient; that failure to produce specific IgG antibodies to immunisation and/or exposure antigens is confirmed, thus meeting the criteria for the diagnosis of primary antibody deficiency. A thorough risk-benefit assessment is essential before blood product treatment is started.

Infantile herpes simplex encephalitis: diagnostic features and differentiation from non-accidental injury.

OBJECTIVES: Neonatal herpes simplex virus (HSV) encephalitis is rare, but associated with considerable morbidity and mortality. After a baby, subsequently proven to have HSE, had initially been diagnosed as non-accidental injury (NAI), we reviewed the clinical features and radiology of infants with HSE recently diagnosed by our laboratory. METHODS: Screening of cerebrospinal fluid (CSF) samples sent to Oxford for HSV polymerase chain reaction (PCR) analysis, from wide range of British hospitals, identified HSV infected infants. After a diagnosis was made, the case notes and neuroradiology (where available) were reviewed and a limited follow-up was undertaken. RESULTS: Thirteen infants had HSV encephalitis (HSE), which in four followed a relapsing course. On subsequent assessment six infants had neurological sequelae, six appeared to be normal, and one was lost to follow-up. Neither a history of primary HSV infection in pregnancy, nor skin lesions in the baby, were helpful diagnostically. Magnetic resonance imaging indicated haemorrhage in the cortex, but no subdural haematomata, a hallmark of NAI, in 5/6 infants. CONCLUSIONS: The early clinical features of HSE and NAI may be indistinguishable. As early diagnosis is important, infants with an unidentified encephalopathic illness should be examined by neuroradiology and their CSF tested for HSV DNA. Together these examinations can confirm and differentiate between those two conditions. Relapsing HSE may mimic recurrent encephalopathy caused by multiple NAIs.

Posterior spinal ligament rupture associated with laryngeal mask insertion in a patient with undisclosed unstable cervical spine.

A case of posterior spinal ligament rupture associated with a general anaesthetic for a laparoscopic cholecystectomy is reported. The role of the general anaesthetic in this case is discussed and a review of the literature is presented.

A unique presentation of retroclival chordoma.

Chordomas are rare tumours which arise from remnants of the primitive notochord. They occur primarily in the sacrum, clivus and cervical regions. We report a case of retroclival chordoma which presented as an extradural haemorrhage following minor trauma. The underlying tumour was not apparent on imaging performed immediately following the event, and chordoma presenting in this manner has not previously been described in the literature. The tumour became apparent on subsequent imaging, and progressed despite surgical debulking and radiotherapy.

Lateralized cognitive deficits in children following cerebellar lesions.

The aim of this preliminary study was to examine the developing cognitive profiles of children with cerebellar tumours in a consecutive series of clinical patients. MRI and longitudinal intellectual profiles were obtained on seven children (two females, five males; mean age 3 years at diagnosis; mean age 7 years at first assessment). Tumours in three of the children were astrocytomas; of the remaining tumours, two were medulloblastomas, one low-grade glioma, and one ependymoma. In right-handed children, we observed an association between greater damage to right cerebellar structures and a plateauing in verbal and/or literacy skills. In contrast, greater damage to left cerebellar structures was associated with delayed or impaired non-verbal/spatial skills. Long-term cognitive development of the children studied tentatively supports a role for the cerebellum in learning/development. These findings suggest that lateralized cerebellar damage may selectively impair the development of cognitive functions subserved by the contralateral cerebral hemisphere and, in addition, that all children with cerebellar lesions in early childhood should routinely undergo long-term monitoring of their intellectual development.

Case control study of cerebrovascular damage defined by magnetic resonance imaging in patients with OSA and normal matched control subjects.

STUDY OBJECTIVES: To assess whether MRI detectable evidence of silent cerebrovascular disease is more prevalent in patients with obstructive sleep apnea (OSA) when compared to carefully matched control subjects. DESIGN AND SETTING: Case-control study of patients with OSA attending a specialist sleep clinic and matched control subjects drawn from the normal community. PARTICIPANTS: Forty-five sleep clinic patients with moderate to severe OSA and excessive daytime sleepiness, matched to 45 control subjects without excessive sleepiness or evidence of OSA on a sleep study. Matched variables included age, body mass index (BMI), alcohol and cigarette consumption, treated hypertension, and ischaemic heart disease. INTERVENTIONS: N/A. MEASUREMENTS AND RESULTS: All subjects underwent 24-hour ambulatory blood pressure recordings (before treatment in OSA patients) and sagittal T1, axial T2, and coronal dual echo cerebral MRI imaging to detect clinically silent abnormalities related to hypertensive cerebrovascular disease; areas of high signal foci in deep white matter (DWM), lacunae, and periventricular hyperintensity. Lacunae/high signal foci in DWM and/or periventricular hyperintensity were present in 15 (33%) OSA subjects and 16 (35%) controls, despite significant increases in mean daytime diastolic blood pressure (4.6mmHg, p<0.05), and both nighttime diastolic (7.2mmHg, p<0.001) and systolic blood pressures (9.2mmHg, p<0.05) in OSA subjects. These data exclude more than a 17% excess prevalence of MRI detected minor cerebrovascular disease in the OSA patients, with 95% confidence. CONCLUSIONS: Sub-clinical cerebrovascular disease is prevalent in both clinic patients with OSA and their matched control subjects. Despite the increased arterial blood pressures, there is, however, no apparent excess of MRI-evident subclinical cerebrovascular disease in patients with OSA compared to appropriately matched control subjects.

The natural history of transdural herniation of the spinal cord: case report.

We report a patient with a Brown-Séquard syndrome who developed ventral transdural spinal cord herniation, showing the imaging findings changing over time from two thoracic disc protrusions with a normal spinal cord to a cord trapped within a bony defect in a thoracic vertebra.