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.

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.

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.

Is the course of brain development in schizophrenia delayed? Evidence from onsets in adolescence.

A degree of ventricular enlargement, together with a reduction of total cortical mass and loss of asymmetry is reported in schizophrenia, but the meaning is obscure. These changes may reflect an anomaly of brain development. Brain structure was assessed on a 1.5-Tesla MRI scan in a series of 29 adolescents at the time of a first episode of schizophrenia and compared with 15 adolescents with other serious psychiatric disturbance (mostly psychotic) and 20 normal adolescent controls. The age at scan ranged between 13 and 20 years. In the adolescents with a diagnosis of schizophrenia, total brain volume increased with age in a way that differed significantly (p=0.007) from that seen in patients with other psychiatric disturbance and normal controls. Thus, brain growth, as assessed by this index, had reached a plateau in the control group by the age of 13 years, but this was not true of patients with schizophrenia. The measure that most clearly distinguished the groups (p<0.001 after co-varying for height and sex) was the volume of the left lateral ventricle the ventricle was significantly larger in patients with schizophrenic illness, and ventricular size increased with age to a greater extent in the patient group, although not significantly so, than in normal controls. Thus, aspects of brain growth are delayed in patients with early onset schizophrenia, and the greatest severity of illness is reflected in a component of growth that is lateralized to the dominant hemisphere. Individuals who develop serious psychiatric illness, including schizophrenia, represent a fraction of the population in whom a component of the relative development of the cerebral hemispheres occurs late.

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.

Comparison of the inhibitory action on Saccharomyces cerevisiae of weak-acid preservatives, uncouplers, and medium-chain fatty acids.

This study was initiated to establish whether inhibition of growth of yeasts by medium-chain fatty acids resembled that caused by weak-acid preservatives or uncouplers. Unlike sorbic acid and 2,4-dinitrophenol, decanoic acid caused rapid cell death at its inhibitory concentration. This suggested a mode of action by medium-chain fatty acids, distinct from both weak-acid preservatives and uncouplers. Sorbic acid and 2,4-dinitrophenol both increased lag and doubling times, reduced cell yields and inhibitory concentrations of both were highly pH sensitive. The possibility is discussed as to whether weak-acid preservatives and uncouplers share common modes of inhibition.

Selective amygdalohippocampectomy for hippocampal sclerosis: postoperative MR appearance.

PURPOSE: To analyze the anatomic consequences of selective amygdalohippocampectomy (AH) in patients with hippocampal sclerosis and to correlate the clinical outcome with the MR appearance. METHODS: Seventeen patients were examined with clinical and neuropsychologic examination and cranial MR after AH (7 transcortical AH, 10 trans-Sylvian AH). The clinical and neuropsychologic outcomes after AH were compared with those of anterior lobectomy (ATL). RESULTS: There was no significant difference in seizure cure between transcortical or trans-Sylvian AH and ATL. However, patients with left AH fared significantly better in terms of verbal IQ and nonverbal memory when compared with those with left ATL. Verbal memory and cognition were not significantly different in the two AH groups. Variable amounts of hippocampal and amygdala remnants were found in both AH groups and did not correlate with seizure cure. White matter change consistent with gliosis probably secondary to wallerian degeneration was demonstrated in the anterior temporal lobe to a mean distance of 4.5 cm after transcortical AH and to a lesser degree as a consequence of trans-Sylvian AH. Nine patients (53%) (4 transcortical All, 5 trans-Sylvian AH) demonstrated wallerian degeneration in the optic radiations after surgery. All had incomplete contralateral quadrantanopia. CONCLUSIONS: There is more secondary damage to the temporal lobe after AH than was previously recognized. The extent of hippocampal and amygdala resection in AH do not seem to be directly related to seizure cure. Visual field defects are common in AH because of the anterior but variable course of the optic radiations

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.

Brain imaging in neonatal hypoglycaemia.

Magnetic resonance imaging studies in two cases of neonatal hypoglycaemia showed cortical and white matter cerebral damage that was most obvious in the occipital lobes. Both cases showed oedema in the parieto-occipital cortex and underlying white matter in the acute phase, with profound atrophy of these regions in the chronic phase. These findings support those of pathological studies which suggest that hypoglycaemia induces cerebral damage by a mechanism separate from the effects of cerebral hypoxia-ischaemia caused by secondary seizures.

CT-dacryocystography for nasolacrimal duct obstruction following paranasal sinus surgery.

Nasolacrimal duct obstruction and consequent epiphora is a complication that may develop in some patients following paranasal sinus surgery. We describe the technique of CT-dacryocystography which entails contrast injection of the lacrimal system and simultaneous computed tomography (CT) scanning of the facial structures. This technique provides concurrent information regarding obstruction of the nasolacrimal duct and the presence of disease recurrence or persistence in the adjacent paranasal sinuses. The role of CT-dacryocystography prior to endoscopic dacryocystorhinostomy is discussed briefly.

Imlink and continuing medical education: the use of an image transfer system to broadcast teaching cases nationally.

Imlink, a personal computer based image storage and transfer system, has been used regularly in Oxford since 1989, primarily to transmit computed tomography (CT) brain scans to the regional neurosurgery centre for management advice. Recently this system has been used to send a weekly educational programme (CT and magnetic resonance imaging neuroradiology cases) to district general hospitals.

Birth asphyxia.

The term Birth asphyxia covers a number of clinical and physiological definitions. Birth asphyxia is a relatively common clinical event. In the majority of cases the outcome in terms of brain damage and future development of the child is excellent. However, a small number of children go on to develop patterns of brain damage which are then associated with disability. The article seeks to provide a basic understanding of the various mechanisms involved in producing injury.

Pitch and timing abilities in adult left-hemisphere-dysphasic and right-hemisphere-damaged subjects.

The production and perception of pitch and rhythm were tested in patients with acquired unilateral left-hemisphere (LH) lesions (and subsequent motor dysphasia, n = 13), patients with unilateral right-hemisphere (RH) lesions (n = 14), and normal age-matched controls. While the LH dysphasic subjects were not generally impaired on the production or perception of pitch, they were grossly impaired on the production and perception of rhythm. The RH subjects, in contrast, were impaired on measures of pitch perception and production, including the discrimination and production of single notes and of melodies. It is concluded that the two hemispheres differ in their specialization for the perception and production of pitch and rhythm.

Can C.T. detect labyrinthine fistulae pre-operatively?

The aim of the study was to undertake a controlled prospective evaluation of high resolution computerized tomography in the pre-operative diagnosis of labyrinthine fistulae. Fifty consecutive patients with chronic suppurative otitis media were scanned prior to surgery, using a Siemens DRI scanner. The radiological predictions were then compared with the surgical findings. Five patients had labyrinthine erosions and these were predicted in 4 out of the 5 (80%). The one erosion that escaped radiological detection was less than 2 mm in length. The study shows that high resolution computerized tomography is a highly accurate method for demonstrating pre-operatively all but the smallest fistulae.

Heterotopic brain tissue: a rare cause of adult recurrent meningitis.

Heterotopic brain tissue is a rare cause of symptoms in neonates in whom it may present with respiratory obstruction. In infants it may produce unilateral nasal obstruction due to a nasal 'polyp' or cerebrospinal fluid rhinorrhoea with an associated risk of meningitis. It is exceedingly rare in adults but may nevertheless be a cause of significant morbidity as this case demonstrates.

MRI as a single screening procedure for acoustic neuroma: a cost effective protocol.

Magnetic resonance imaging (MRI) is accepted as the 'gold standard' in diagnosing acoustic neuromas. Limited availability and perceived high costs have prevented clinicians from using it as a first-line investigation. A prospective study was set up in a specially designated screening session to audit the cost effectiveness and accuracy of audiovestibular investigations compared to MRI. Ninety-nine patients with asymmetrical audiovestibular symptoms or signs were investigated. Of these 54 evoked response audiometry tests, and 39 calorics were either not performed or were inconclusive. One patient refused to enter the MRI machine. All others received an unequivocal report after MRI and four tumours (three intracanalicular) were detected. The total cost of the audiovestibular protocol was 12,545 pounds compared to 12,900 pounds for the MRI protocol, which is a diagnostic and well-tolerated procedure. This study shows that MRI can be cost effective, as well as accurate, when used as a single screening procedure for acoustic neuromas.

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.