Petros Zampakis

Gorlin-Goltz syndrome: incidental finding on routine ct scan following car accident.

IntroductionGorlin-Goltz syndrome is a rare hereditary disease. Pathogenesis of the syndrome is attributed to abnormalities in the long arm of chromosome 9 (q22.3-q31) and loss or mutations of human patched gene (PTCH1 gene). Multiple basal cell carcinomas (BCCs), odontogenic keratocysts, skeletal abnormalities, hyperkeratosis of palms and soles, intracranial ectopic calcifications of the falx cerebri and facial dysmorphism are considered the main clinical features. Diagnosis is based upon established major and minor clinical and radiological criteria and ideally confirmed by DNA analysis. Because of the different systems affected, a multidisciplinary approach team of various experts is required for a successful management.Case presentationWe report the case of a 19 year-old female who was involved in a car accident and found to present imaging findings of Gorlin-Goltz syndrome during a routine whole body computed tomography (CT) scan in order to exclude traumatic injuries.ConclusionRadiologic findings of the syndrome are easily identifiable on CT scans and may prompt to early verification of the disease, which is very important for regular follow-up and better survival rates from the co-existent diseases.

Thalamic atrophy predicts cognitive impairment in relapsing remitting multiple sclerosis. Effect on instrumental activities of daily living and employment status

INTRODUCTION Cognitive impairment is an important predictor of quality of life at all stages of MS. Magnetic Resonance Imaging (MRI) markers have been used to associate tissue damage with cognitive dysfunction. OBJECTIVE The aim of the study was to designate the MRI marker that predicts cognitive decline and explore its effect on every day activities and employment status. METHODS 50 RRMS patients and 31 healthy participants underwent neuropsychological assessment using the Trail Making Test (TMT) parts A and B, semantic and phonological verbal fluency task and a computerized cognitive screening battery (Central Nervous System Vital Signs). Everyday activities were evaluated with the instrumental activities of daily living (IADL) scale and employment status. Brain MRI was performed in all participants. We measured total lesion volume, third ventricle width, corpus callosum and thalamic atrophy. RESULTS The frequency of cognitive dysfunction for our RRMS patients was 38%. RRMS patients differed significantly from controls on the TMTA, TMTB, phonological verbal fluency task, memory, psychomotor speed, reaction time and cognitive flexibility. Neuropsychological measures had a strong correlation with all MRI atrophy measures and a weak or moderate correlation with lesion volume. Psychomotor speed was the most sensitive marker for IADL, while memory and TMTB for employment status. Thalamic area was the most sensitive MRI marker for memory, psychomotor speed and TMTB.. CONCLUSION Thalamic atrophy predicts the clinically meaningful cognitive decline in our RRMS patients.

Acute post-stroke adiponectin in relation to stroke severity, progression and 6 month functional outcome

Abstract Background: Circulating adiponectin (ADPN) has been inversely associated with the risk of coronary artery disease and ischemic stroke (IS), due to its anti-inflammatory and anti-atherosclerotic properties. Recent experimental studies have suggested that ADPN may as well exert cerebroprotective properties in brain ischemia, therefore modifying disease outcome. We investigated whether acute post-stroke ADPN in humans might be associated with disease severity, progression and outcome. Methods: Serum ADPN was measured in 82 consecutive acute IS patients. Severity at presentation and stroke progression within the first week were evaluated according to internationally agreed definitions. Disability and functional outcomes were assessed on months 1, 3 and 6 using the modified Rankin scale (mRS) and Barthel index (BI). Additional data included information on infarct size, mortality, recurrent IS and mental state. Results: Higher ADPN was indicative of greater disability (mRS) on month 1 (OR=1·141, 95% CI=1·012–1·286, p=0·031), but this result was not replicated using the BI. ADPN was not found to be associated with either stroke severity, clinical progression, infarct size, recurrent IS, mortality, mental state, disability or functional outcome during the 6 month follow-up. Conclusions: Despite previous experimental evidence, serum ADPN measured shortly after an acute IS in humans does not seem to reliably predict disease severity, progression or outcome. The concept that circulating ADPN may beneficially modify long-term outcome of an acute IS may not be the case for human stroke.

Endovascular Treatment of AVMs in Glasgow

The Institute of Neurological Sciences (INS) at the Southern General Hospital in Glasgow is the largest Neuroscience Centre in Scotland, and among the largest in the UK. The other 3 Scottish centres are Aberdeen, Dundee and Edinburgh. Scotland itself has a population of approximately 5.2 million people and the INS in Glasgow provides neurointervention cover to almost 3 million of these. Referrals also come from the rest of Britain and overseas (North Africa, Middle-East and Asia). Glasgow has been nominated as one of two centres in the UK for management of neonatal high-flow fistulae, and has the largest experience in the UK for management of cervico-facial vascular malformations. There are 3 consultant interventional neuroradiologists (in addition to 6 purely diagnostic neuroradiologists) and two fellows (at least one of whom is training in neurointervention). Overseas trainees are sometimes accommodated, most recently from Greece and Thailand. Patients requiring interventional neuroradiology are usually admitted into the neurosurgery unit, although care is shared with the neuroradiologists. An AVM clinic is run jointly with neurosurgical colleagues, to which most (but not all) AVM patients are referred, and close links are maintained with the National Stereotactic Radiosurgery Centre in Sheffield (which has overwhelmingly, the largest experience in the UK for the radiosurgical treatment of AVMs). The Scottish Intracranial Vascular Malformations Study (SIVMS) is a collaborative epidemiological study between the four Scottish centres1,2,5.

Mycotic aneurysm of the internal carotid artery presenting with multiple cerebral septic emboli.

Mycotic aneurysms of the extracranial carotid artery are uncommon and always warrant surgical treatment to prevent eventual rupture and death. Septic embolization to the brain is an even rarer complication of these aneurysms. We present a case of a 79-year-old male with an extracranial internal carotid artery mycotic aneurysm during Staphylococcus aureus bacteremia. He presented with hemiparesis owing to brain embolism from multiple septic emboli originating from the aneurysm. Multidetector computed tomographic angiography contributed to the diagnosis. Resection of the aneurysm and restoration of arterial supply to the brain by vein graft interpositioning was the therapeutic procedure along with long-term antibiotic treatment. A high index of suspicion is required for the diagnosis of a mycotic carotid aneurysm and aggressive treatment is always needed to prevent lethal complications.

Increased Prevalence and Severity of Coronary Artery Calcification in Patients with Chronic Kidney Disease Stage III and IV.

Background: Cardiovascular disease (CVD) is the main cause of mortality in patients with chronic kidney disease (CKD). The pathophysiology of coronary artery disease in CKD is multifactorial including, in addition to traditional risk factors (hypertension, hyperlipidemia, diabetes mellitus), parameters related to uremia. Methods: The study consisted of measuring coronary artery calcification (CAC) score in patients with CKD stage III and IV without history of CVD and in a group of controls with normal renal function matched for age, gender and risk factors using multi-detector computed tomography. Results: The study included 49 patients and 49 controls. CAC was present in 79.6% in the CKD group versus 59.2% in the control group (p = 0.028). The median CAC score value in CKD patients was 139 (interquartile range (IQR): 23–321) versus 61 (IQR: 6–205) in controls (p = 0.007). CAC was associated with traditional risk factors such as older age, hypertension and baseline cardiovascular risk score, while CKD patients with severe calcification had marginally lower estimated glomerular filtration rate and increased levels of parathormone. Conclusions: CAC is more frequent and severe in patients with CKD stage III and IV compared to matched controls with normal renal function, even though kidney disease-related parameters are not directly correlated with intensity of calcification.

Corpus callosum atrophy as a marker of clinically meaningful cognitive decline in secondary progressive multiple sclerosis. Impact on employment status

Cognitive impairment in Multiple Sclerosis (MS) is more frequent and pronounced in secondary progressive MS (SPMS). Cognitive decline is an important predictor of employment status in patients with MS. Magnetic Resonance Imaging (MRI) markers have been used to associate tissue damage with cognitive dysfunction. The aim of the study was to designate the MRI marker that predicts cognitive decline in SPMS and explore its effect on employment status. 30 SPMS patients and 30 healthy participants underwent neuropsychological assessment using the Trail Making Test (TMT) parts A and B, semantic and phonological verbal fluency task and a computerized cognitive screening battery (Central Nervous System Vital Signs). Employment status was obtained as a quality of life measure. Brain MRI was performed in all participants. We measured total lesion volume, third ventricle width, thalamic and corpus callosum atrophy. The frequency of cognitive decline for our SPMS patients was 80%. SPMS patients differed significantly from controls in all neuropsychological measures. Corpus callosum area was correlated with cognitive flexibility, processing speed, composite memory, executive functions, psychomotor speed, reaction time and phonological verbal fluency task. Processing speed and composite memory were the most sensitive markers for predicting employment status. Corpus callosum area was the most sensitive MRI marker for memory and processing speed. Corpus callosum atrophy predicts a clinically meaningful cognitive decline, affecting employment status in our SPMS patients.

MRA artefacts with Nexus coils

Sir, We read with interest the article ‘MR angiographic evaluation is limited in intracranial aneurysms embolized with Nexus coils’ by Kang HS et al. [1]. We are pleased to see that the authors have been able to substantiate our own observation of MR susceptibility artefacts associated with all Dendron, MTI and ev3 coils up to the Nexus generation. We presented a study of magnetic resonance angiography (MRA) artefacts following endovascular coiling at the World Federation of Interventional and Therapeutic Neuroradiology meeting in Venice in 2005 [2] and at the British Society of Neuroradiologists meeting in Edinburgh in 2005 showing that artefact associated with platinum:iridium coils (including Nexus) from ev3 was substantially greater than that seen with platinum:tungsten alloy coils at 1.5T and 3.0T. Our in vitro experiments at 1.5T and 3.0T with ev3 single coils (with and without the nitinol core wire) and coiled aneurysm models further agree with the authors that the susceptibility artefact is caused by the iridium component. We consider that all coiled aneurysms should have a digital subtraction angiogram (DSA) as well as an MRA for comparison at some stage, especially in centres where new devices are being assessed. As the authors correctly point out, even coils usually associated with the lowest susceptibility artefact (Micrus in our experience) can sometimes produce significant artefact. In centres that perform routine DSA follow-up after coiling, the MRA artefact is not a significant issue. Our standard imaging protocol following endovascular coiling includes a digital subtraction angiogram in addition to an MRA at 6 months post coiling. If there is good correlation between the two studies and no significant artefact, which is uncommon with Nexus coils, further follow-up is performed with MRA alone. Otherwise DSA is continued. We believe that Nexus coils offer many advantages including greater thrombogenicity, bioactivity, compaction resistance, softness and coil shape. They continue to be the most widely used coils in our department with 172 aneurysms in 162 patients treated purely with Nexus coils. We do not favour mixing coils from different manufacturers. Following our identification of the increased magnetic susceptibilty artefact associated with platinum:iridium coils, ev3 has produced a new coil series, Axium coils which use platinum:tungsten alloy. Our initial experience with these coils (submitted for publication) suggests that they produce no more artefact than other coil types. Axium coils are currently only available as bare coils, with further filament types due for launch in the near future. Until the filamentcoated Axium coils become available, we continue to use Nexus coils.

External Beam Irradiation and Restenosis Following Femoral Stenting: Long-Term Results of a Prospective Randomized Study

PurposeTo assess the long-term outcome of external beam irradiation (EBI) for the prevention of restenosis due to neointimal hyperplasia, following percutaneous transluminal angioplasty (PTA) and stenting of the superficial femoral artery.MethodsSixty consecutive patients with peripheral arterial disease, who were treated with “bail-out” stent implantation in the superficial femoral artery due to suboptimal PTA, were included in this study. Patients were randomly allocated into two groups, receiving either external beam irradiation (6 MV photons, total dose 24 Gy in a hypofractionated schedule) plus antiplatelet therapy (EBI group) or antiplatelet therapy alone (control group).ResultsNo procedure-related complications occurred, and all patients of the EBI group received the full dose of 24 Gy. During the long-term follow-up, an overall statistically significant difference was demonstrated in favor of the EBI group patients, regarding both the in-stent (log-rank test, p = 0.0072) and the in-segment binary restenosis (log-rank test, p = 0.0103). The primary patency rates were also significantly better in the EBI group at specific time-points, such as in the first (74.2% vs 46.5%, p = 0.019), second (62.5% vs 33.8%, p = 0.020), and third (54.6% vs 29.0%, p = 0.039) year, respectively. Moreover, the overall clinically driven reintervention rate was significantly lower among patients of the irradiated group (log-rank test, p = 0.038).ConclusionOur long-term follow-up analysis revealed that EBI following femoral artery PTA and stenting significantly reduces restenosis and reintervention rates, while improving primary patency.

Age and education adjusted normative data and discriminative validity for Rey’s Auditory Verbal Learning Test in the elderly Greek population

ABSTRACT Rey’s Auditory Verbal Learning Test (RAVLT) is a widely used neuropsychological test to assess episodic memory. In the present study we sought to establish normative and discriminative validity data for the RAVLT in the elderly population using previously adapted learning lists for the Greek adult population. We administered the test to 258 cognitively healthy elderly participants, aged 60–89 years, and two patient groups (192 with amnestic mild cognitive impairment, aMCI, and 65 with Alzheimer’s disease, AD). From the statistical analyses, we found that age and education contributed significantly to most trials of the RAVLT, whereas the influence of gender was not significant. Younger elderly participants with higher education outperformed the older elderly with lower education levels. Moreover, both clinical groups performed significantly worse on most RAVLT trials and composite measures than matched cognitively healthy controls. Furthermore, the AD group performed more poorly than the aMCI group on most RAVLT variables. Receiver operating characteristic (ROC) analysis was used to examine the utility of the RAVLT trials to discriminate cognitively healthy controls from aMCI and AD patients. Area under the curve (AUC), an index of effect size, showed that most of the RAVLT measures (individual and composite) included in this study adequately differentiated between the performance of healthy elders and aMCI/AD patients. We also provide cutoff scores in discriminating cognitively healthy controls from aMCI and AD patients, based on the sensitivity and specificity of the prescribed scores. Moreover, we present age- and education-specific normative data for individual and composite scores for the Greek adapted RAVLT in elderly subjects aged between 60 and 89 years for use in clinical and research settings.