Mutated PET117 causes complex IV deficiency and is associated with neurodevelopmental regression and medulla oblongata lesions.

The genetic basis of the many progressive, multi systemic, mitochondrial diseases that cause a lack of cellular ATP production is heterogeneous, with defects found both in the mitochondrial genome as well as in the nuclear genome. Many different mutations have been found in the genes encoding subunits of the enzyme complexes of the oxidative phosphorylation system. In addition, mutations in genes encoding proteins involved in the assembly of these complexes are known to cause mitochondrial disorders. Here we describe two sisters with a mitochondrial disease characterized by lesions in the medulla oblongata, as demonstrated by brain magnetic resonance imaging, and an isolated complex IV deficiency and reduced levels of individual complex IV subunits. Whole exome sequencing revealed a homozygous nonsense mutation resulting in a premature stop codon in the gene encoding Pet117, a small protein that has previously been predicted to be a complex IV assembly factor. PET117 has not been identified as a mitochondrial disease gene before. Lentiviral complementation of patient fibroblasts with wild-type PET117 restored the complex IV deficiency, proving that the gene defect is responsible for the complex IV deficiency in the patients, and indicating a pivotal role of this protein in the proper functioning of complex IV. Although previous studies had suggested a possible role of this protein in the insertion of copper into complex IV, studies in patient fibroblasts could not confirm this. This case presentation thus implicates mutations in PET117 as a novel cause of mitochondrial disease.

Evaluation of genetic risk loci for intracranial aneurysms in sporadic arteriovenous malformations of the brain.

BACKGROUND: In genome-wide association studies (GWAS) five putative risk loci are associated with intracranial aneurysm. As brain arteriovenous malformations (AVM) and intracranial aneurysms are both intracranial vascular diseases and AVMs often have associated aneurysms, we investigated whether these loci are also associated with sporadic brain AVM. METHODS: We included 506 patients (168 Dutch, 338 American) and 1548 controls, all Caucasians. Controls had been recruited as part of previous GWAS. Dutch patients were genotyped by KASPar assay and US patients by Affymetrix SNP 6.0 array. Associations in each cohort were tested by univariable logistic regression modelling, with subgroup analysis in 205 American cases with aneurysm data. Meta-analysis was performed by a Mantel-Haenszel fixed-effect method. RESULTS: In the Dutch cohort none of the single nucleotide polymorphisms (SNPs) were associated with AVMs. In the American cohort, genotyped SNPs near SOX-17 (OR 0.74; 95% CI 0.56-0.98), RBBP8 (OR 0.76; 95% CI 0.62-0.94) and an imputed SNP near CDKN2B-AS1 (OR 0.79; 95% CI 0.64-0.98) were significantly associated with AVM. The association with SNPs near SOX-17 and CDKN2B-AS1 but not RBBP8 were strongest in patients with AVM with associated aneurysms. In the meta-analysis we found no significant associations between allele frequencies and AVM occurrence, but rs9298506, near SOX-17 approached statistical significance (OR 0.77; 95% CI 0.57-1.03, p=0.08). CONCLUSIONS: Our meta-analysis of two Caucasian cohorts did not show an association between five aneurysm-associated loci and sporadic brain AVM. Possible involvement of SOX-17 and RBBP8, genes involved in cell cycle progression, deserves further investigation.

Lymph node detection by MRI before and after a systematic pelvic lymphadenectomy.

OBJECTIVE: Pelvic lymphadenectomy is considered the gold standard to diagnose and possibly treat lymphatic metastases in gynaecological cancer patients. The aim of this study is to evaluate whether all presurgical MRI detected lymph nodes were removed during the systematic pelvic lymph node dissection (PLND) in cervical cancer patients. METHODS: 21 consecutive cervical cancer patients who were scheduled to undergo a PLND were evaluated by a MRI scan prior to surgery and 6 weeks afterwards. All patients had tumour metastasis negative lymph nodes at histopathological examination. RESULTS: On average, 10 pelvic lymph nodes (range 5-17) per patient were detected by presurgical MRI. Postsurgical MRI scans showed that on average 1 (range 0-3) pelvic node per patient was not removed by surgery. In total, 14% of the presurgical MR detected nodes were not removed by surgery (31/225). Approximately half of all lymph nodes that remained after surgery were located in the obturator region. In spite of the remaining nodes, surgery and histopathological examination did detect more nodes than MRI: on average 21 lymph nodes per patient (range 9-59) were removed. Another 2 lymph nodes (range 0-6 per patient) were judged to be newly developed after surgery. CONCLUSION: Although surgery was able to remove many more lymph nodes than those detected by presurgical MRI, 14% of presurgical MRI detected lymph nodes were not removed by PLND. The value of MRI prior to surgery for the detection of pathological lymph nodes is a subject of further research.

Recovery of knee range of motion after total knee arthroplasty in the first postoperative weeks: poor recovery can be detected early.

PURPOSE: The aim of this study was to analyze in detail how knee flexion and extension progress in the first 8 weeks after primary total knee arthroplasty (TKA). The secondary goal was to compare knee range of motion (ROM) recovery patterns between patients with normal and delayed ROM recovery 8 weeks after TKA. METHODS: This prospective clinical trial included all patients who underwent a primary unilateral TKA between February and December 2016 with weekly ROM data documented by the treating outpatient physical therapists (n = 137). Goniometry was used to measure knee ROM preoperatively, postoperatively on day 1 and weekly until follow-up at the orthopedic clinic 8 weeks after surgery. ROM recovery patterns were compared between patients with sufficient (≥ 90°) or insufficient (< 90°) knee flexion 8 weeks after TKA. RESULTS: Knee flexion recovered from a median of 80° in the first postoperative week to 110° 8 weeks after surgery and knee extension from a mean of - 10.7° to - 3.2°. Recovery was nonlinear, with greatest improvements in the first 4 weeks for knee flexion. In contrast to patients with sufficient knee flexion 8 weeks postoperatively, the insufficient group (n = 8, 5.8%) had poor knee flexion on the first postoperative day and from week 4 to week 8 almost no improvement or even worsening of knee flexion. CONCLUSIONS: Both knee flexion and extension recover in a nonlinear manner after TKA surgery. Poor postoperative knee function can be detected early, using ROM data from the first postoperative day up to the fourth week.

New detected aneurysms on follow-up screening in patients with previously clipped intracranial aneurysms: comparison with DSA or CTA at the time of SAH.

BACKGROUND AND PURPOSE: Patients with a history of aneurysmal subarachnoid hemorrhage may have aneurysms on screening several years after the hemorrhage. For determining the benefits of follow-up screening, it is important to know whether these aneurysms have developed after the hemorrhage or are visible in retrospect, and if so, whether the size has increased. METHODS: Aneurysms were categorized into de novo aneurysms and aneurysms visible in retrospect (already present) with increased or stable size. We studied aneurysm characteristics for these 3 categories: the relation between aneurysm development or enlargement and duration of follow up and the relation between enlargement and initial size of the aneurysm. RESULTS: In 87 of 495 patients (17.6%), aneurysms were detected; for 51 of these patients with 62 aneurysms, the original catheter or computed tomographic angiogram was available for comparison. Of the 62 aneurysms, 19 were de novo and 43 were visible in retrospect, 10 with increased size and 33 with stable size. De novo aneurysms were mainly < or =5 mm (95%) and located at the middle cerebral artery (63%). For aneurysms visible in retrospect, the most frequent location was the posterior communicating artery (21%). There was no relation between the development of de novo aneurysms or enlargement and the duration of follow-up or between enlargement and the initial size of the aneurysm. CONCLUSIONS: Of aneurysms detected at screening, one third were de novo and two thirds were missed at the time of the initial hemorrhage. One quarter of initially small aneurysms had enlarged during follow-up.

Outcome after intracranial haemorrhage from dural arteriovenous fistulae; a systematic review and case-series.

Dural arteriovenous fistulae (DAVFs) are a rare cause of intracranial haemorrhage. We aimed to investigate outcome of patients with intracranial haemorrhage from a DAVF. We performed a systematic literature search for studies reporting outcome after intracranial haemorrhage caused by a DAVF. We used predefined selection criteria and assessed the quality of the studies. In addition, we studied outcome in all patients with DAVF who had presented with intracranial haemorrhage at two university centers in the Netherlands, between January 2007 and April 2012. We calculated case fatality and proportions of patients with poor outcome (defined as modified Rankin Scale ≥ 3 or Glasgow Outcome Scale ≤ 3) during follow-up. We investigated mean age, sex, mid-year of study and percentage of patients with parenchymal haemorrhage as determinants of case fatality and poor outcome. The literature search yielded 16 studies, all but two retrospective and all hospital-based. Combined with our cohort of 29 patients the total number of patients with DAVF-related intracranial haemorrhage was 326 (58% intracerebral haemorrhage). At a median follow-up of 12 months case fatality was 4.7% (95% CI 2.5-7.5; 17 cohorts) and the proportion of patients with poor outcome 8.3% (95% CI 3.1-15.7; nine cohorts). We found no effect of mean age, sex, mid-year of the cohorts and percentage of patients with parenchymal haemorrhage on either outcome. Hospital based case-series suggest a relatively low risk of death and poor outcome in patients with intracranial haemorrhage due to rupture of a DAVF. These risks may be underestimated because of bias.

Acute liver necrosis induced by iodine-131-MIBG in the treatment of metastatic carcinoid tumors.

Iodine-131-metaiodobenzylguanidine (MIBG) is used in the treatment of carcinoid tumors. Temporary palliation with complete subjective symptomatic response has been reported in these patients. This treatment is usually well tolerated and side-effects are generally limited to nausea, mild hepatic toxicity with spontaneous recovery and temporary myelosuppression. Our case report shows that repeated treatment with [131I]MIBG in a patient with extensive carcinoid liver metastasis may cause severe hepatic toxicity leading to death. Factors such as concomitant use of 5-fluorouracil and the progressive nature of the disease may have contributed to this event.

Curved reconstructions versus three-dimensional surface rendering in the demonstration of cortical lesions in patients with extratemporal epilepsy.

RATIONALE AND OBJECTIVES: To compare the visibility and localization of extratemporal cortical lesions in extratemporal epilepsy by using curved reconstruction (CR) and three-dimensional surface rendering (3D SR) of 3D-acquired MR images and to study the degree of confidence with which localizations are made, particularly at the gyral level. METHODS: Twenty patients with extratemporal epilepsy, based on seizure symptomatology and/or scalp electroencephalographic registrations, with an extratemporal structural lesion on conventional MR imaging, were selected for this study by a neuroradiologist with extensive experience in the assessment of epilepsy patients. Transverse T2 spin-echo, coronal fluid-attenuated inversion recovery, and transverse 3D-acquired/two-dimensionally reconstructed T1 MR images were used for the selection. A second neuroradiologist (observer 1) and a radiology resident (observer 2) assessed CR and 3D SR in random order. Both observers were masked to all patient data. The subjective visibility of lesions and gyral location were scored. The interobserver agreements for lesion visibility and localization and for degree of confidence were compared for CR and 3D SR. RESULTS: For both observers, the lesion was visible in 55% of 3D SRs and 95% of CRs. The proportion with "very clearly visible" lesions on 3D SR was 19% (4/20) according to observer 1 and 30% (6/20) according to observer 2. For CR, this proportion was substantially higher: 55% for both observers. This difference was significant for observer 1 but not for observer 2. The interobserver agreement was high for both methods. Agreement on gyral localization was 28% for CR and 40% for 3D SR. The percentage of similar confidence scores for the same gyral localization and for gyral localization with a maximum difference of one gyrus between the observers did not differ significantly for CR or 3D SR. The observers were more often confident in agreed cases in CR and moderately confident in 3D SR. CONCLUSIONS: These results suggest that CRs of the brain surface are superior to 3D SR for the visualization of extratemporal cortical lesions in patients with drug-resistant extratemporal epilepsy. If lesions are seen, no significant difference was found between the two techniques for localization; however, the degree of confidence appears higher for CR at the gyral level.

Fast fluid-attenuated inversion recovery (FLAIR) compared with T2-weighted spin-echo in the magnetic resonance diagnosis of mesial temporal sclerosis.

RATIONALE AND OBJECTIVES: The authors compare coronal fast fluid-attenuated inversion recovery (FLAIR) with coronal T2-weighted spin-echo (SE) magnetic resonance (MR) techniques in the diagnosis of mesial temporal sclerosis (MTS). METHODS: In this prospective study, the authors assessed MR scans of 30 patients with drug-resistant temporal lobe epilepsy (based on clinical symptomatology and electroencephalographic registrations) with MR features suggestive of MTS. MR scans of age-, sex-, and scanner-matched patients, referred for MR assessment of white matter disease, without a history of epilepsy and with no visible abnormalities on MR, were used as controls. In 16 patients the MR diagnosis was confirmed by histologic abnormalities consistent with MTS. Coronal T2 SE and FLAIR images of patients and controls were presented to two experienced radiologists in random order for independent blinded review. Hippocampal and associated extrahippocampal temporal lobe abnormalities were used for the diagnosis of MTS. RESULTS: The sensitivity of observer A was 97% for the T2 SE sequence and 100% for the FLAIR; the specificity of observer A for both techniques was 100%. The sensitivity of observer B was 53% for T2 SE and 83% for FLAIR; the specificity for observer B was 93% for the T2 SE and 100% for FLAIR. CONCLUSION: Coronal FLAIR images provide a similar or increased yield in the detection of MTS compared with T2-weighted SE images.

Relevance of temporal lobe white matter changes in hippocampal sclerosis. Magnetic resonance imaging and histology.

RATIONALE AND OBJECTIVES: To evaluate the diagnostic relevance of ipsilateral atrophy of the collateral white matter in the parahippocampal gyrus (ACWMp) and temporal lobe gray/white matter demarcation loss (GWDL) on magnetic resonance imaging in patients with histologically confirmed hippocampal sclerosis. In the second part of this investigation, histologic specimens were analyzed to find an explanation for GWDL. METHODS: Retrospective visual assessment of hippocampal signal intensity and size and of ACWMp and GWDL was performed using 4- to 5-mm coronal T2-weighted spin-echo magnetic resonance images of 80 patients with histologically proven hippocampal sclerosis and of 30 age-matched controls without epilepsy. Frequency of occurrence and likelihood ratios of ACWMp and GWDL were calculated and their contribution to the diagnosis of hippocampal sclerosis was assessed, particularly in patients with no or restricted hippocampal abnormalities (either high signal or smaller size) on magnetic resonance imaging. The second part of the study involved the morphologic histologic assessment of neocortical temporal lobe specimens of all patients. Myelin density was evaluated in specimens of a subgroup of six patients with hippocampal sclerosis and GWDL on MRI and six patients with hippocampal sclerosis without GWDL. RESULTS: ACWMp was found in 68% and GWDL in 65% of patients with hippocampal sclerosis on magnetic resonance imaging. Both features had an infinite positive likelihood ratio. Sixty-two patients (77.5%) had concomitant hippocampal signal increase and smaller size. Eighteen patients (22.5%) had no or restricted hippocampal abnormalities on magnetic resonance imaging. When using ACWMp and GWDL as additional diagnostic parameters, 13 of these 18 patients were more unambiguously diagnosed as having hippocampal sclerosis. No significant morphologic differences were found between GWDL-positive and GWDL-negative specimens. A significantly lower average myelin stain was found in the white matter of the GWDL-positive group compared to the GWDL-negative group. CONCLUSIONS: ACWMp and GWDL can improve the visual diagnosis of hippocampal sclerosis, particularly in patients with no or restricted hippocampal abnormalities. These results suggest that loss of myelin may be the underlying cause of GWDL in association with hippocampal sclerosis.

Assessment of the preferred plane and sequence in the depiction of mesial temporal sclerosis using magnetic resonance imaging.

RATIONALE AND OBJECTIVES: Definition of optimal magnetic resonance (MR) scanning plane and conventional MR sequence for the detection of mesial temporal sclerosis (MTS). METHODS: Coronal and axial T2-weighted images and axial T2-weighted images parallel to the long axis of the hippocampus (APLAH) and coronal inversion recovery (IR) images were obtained in patients with medically intractable temporal lobe epilepsy in their phase 1 preoperative evaluation. Thirty-three consecutive MR scans were reviewed by a panel of three radiologists. Twenty-three patients had MR abnormalities consistent with MTS, and ten scans were normal. To assess the best single scanning technique, another group of three radiologists, who were masked to all patient data, individually assessed the different planes and sequences of the 33 studies presented separately in a random fashion. For each plane and sequence, the likelihood (L) ratio for the correct diagnosis was determined separately. RESULTS: For all planes considered separately, a likelihood ratio of 4.4 was optimal for the coronal T2-weighted images. The likelihood ratio of APLAH T2 was 2.2; of axial T2, 3.9; of coronal IR, indefinite because of 100% specificity. CONCLUSIONS: For the assessment of MTS, coronal T2-weighted images were considered the best single scanning technique.

Immuno-detection of the virulence determinant OmpX at the cell surface of Enterobacter cloacae.

A model for the topology of the Enterobacter cloacae outer membrane protein OmpX has been proposed, based on the primary sequence and on analogy to homologous proteins. According to this model the membrane embedded part of the protein consists of eight antiparallel beta-strands. Four random coil loops are located at the bacterial surface and three beta-turns at the periplasmic side of the membrane. Antibodies were raised against synthetic peptides representing five OmpX domains, four of which are putative peripheral and one located in the membrane. The accessibilities of OmpX to these antibodies were tested in intact cells by immuno-gold electron microscopy. This study showed that OmpX is indeed an outer membrane protein, the N-proximal loop of which forms an IgG-accessible epitope at the cell surface.

Effects of mutations and deletions on expression of the Enterobacter cloacae ompX gene.

The Enterobacter cloacae outer membrane protein OmpX is involved in resistance to beta-lactams, and possesses virulence characteristics. To gain more insight into the genetic elements that are important for OmpX expression, several mutations were introduced at, and immediately upstream of, the N-terminus of the OmpX coding sequence. These mutations enabled us to delete the 5' untranslated region and the signal peptide coding sequence. The former led to decreased ompX expression, indicating an unexpected and hitherto unexplained role for this region. Deletion of the signal peptide coding sequence blocked transport across the cytoplasmic membrane, indicating that translocation of OmpX across the cytoplasmic membrane is mediated by the general secretory pathway.

Glycosylation of the Enterobacter cloacae outer membrane protein OmpX in eukaryotic cells.

The topological model of the Enterobacter cloacae outer membrane protein OmpX showed three putative glycosylation sites. When OmpX was expressed in bacteria that were cultured under aerated conditions, no glycosylation was observed. The coupling of carbohydrate chains to the ompX gene product was also investigated in the eukaryotic baculovirus expression system. For this purpose, a recombinant ompX gene-containing baculovirus was made. Infection of insect cells with this recombinant virus resulted in the production of sufficient amounts of OmpX to study glycosylation. In this system, all potential N-glycosylation sites of OmpX were utilized. Furthermore, it became clear that glycosylated OmpX was retained in the insect cells and was not secreted in the medium. Given the fact that OmpX plays a role in the invasion of E. cloacae in rabbit enterocytes, glycosylation of this protein occurring only under specific conditions may be involved in this process.

Structural involvement of the glutamatergic presynaptic boutons in a transgenic mouse model expressing early onset amyloid pathology.

While the cholinergic depletion in Alzheimer's disease (AD) has been known for some time, a definitive involvement of other neurotransmitter systems has been somewhat more elusive. Our study demonstrates a clear involvement of both glutamatergic and, to a lesser extent, GABAergic neurons in an early onset transgenic mouse model of AD-like amyloid pathology. Immunohistochemical staining and subsequent quantification has revealed a statistically significant increased density of glutamatergic and GABAergic presynaptic boutons in both the plaque free and plaque adjacent cortical neuropile areas of transgenic mice as compared to non-transgenic controls. Furthermore, amyloid plaque size was shown to have a statistically significant effect on the relative area occupied by dystrophic glutamatergic neurites in the peri-plaque neuropile. These findings support our hypothesis that the amyloid pathology progresses in a time and neurotransmitter specific manner, first in the cholinergic system which appears to be most vulnerable, followed by the glutamatergic presynaptic boutons and finally the somewhat more resilient GABAergic terminals.

Temporal lobe epilepsy: the various MR appearances of histologically proven mesial temporal sclerosis.

PURPOSE: To determine the frequency of appearance of various MR signs in mesial temporal sclerosis, to determine the optimal scanning planes for their visualization, and to propose a histologic explanation for the diminished demarcation between gray and white matter in the temporal lobe, a frequent MR finding in patients with mesial temporal sclerosis. METHODS: MR scans of 14 surgically treated patients with epilepsy and histologically proven mesial temporal sclerosis were assessed for the presence of six features: feature 1, high signal intensity in the hippocampus; 2, reduced hippocampal size; 3, ipsilateral atrophy of the hippocampal collateral white matter; 4, enlarged temporal horn; 5, reduced gray-white matter demarcation in the temporal lobe; and 6, decreased temporal lobe size. RESULTS: Feature 1 was present in 14 patients and was best appreciated on the T2-weighted images in planes parallel to the long axes of the hippocampi. Feature 2, present in 12 patients, and feature 6, present in 9 patients, were optimally seen in the coronal planes and on the inversion-recovery sequences in particular. Feature 3, present in 12 patients, was optimally seen on the coronal T2-weighted images. Feature 4, seen in 11 patients, was equally well seen in all planes (transverse, coronal, and parallel to the long axes of the hippocampi). Feature 5, seen in 10 patients, was best appreciated on the T2-weighted images in the planes of the long axes of the hippocampi. Histologic investigation of the temporal lobe white matter in the 10 patients with feature 5 demonstrated on the MR scan showed abnormalities in 7 cases. Oligodendroglia cell clusters were found in 6, with concomitant corpora amylacea in 1 case and perivascular macrophages with pigment a sole finding in another case. CONCLUSION: Of the six features found in cases of mesial temporal sclerosis on MR, increased hippocampal signal intensity is the most consistent. A decreased gray-white matter demarcation in the temporal lobe parenchyma is also a frequent feature of this disease. A combination of multiple scanning planes results in an optimal demonstration of lesions.

Production of cellulolytic and xylanolytic enzymes during growth of anaerobic fungi from ruminant and nonruminant herbivores on different substrates.

Three anaerobic fungi, two Neocallimastix strains isolated from a ruminant (sheep) and one Piromyces strain isolated from a nonruminant (black rhinoceros), were tested for their ability to ferment a range of substrates. Bagasse, filter paper cellulose, fructose, and wheat straw were good inducers of celluloytic and xylanolytic enzymes. These enzymes were produced constitutively by all three strains, although enzyme activities were generally lower, especially for both Neocallimastix strains, after growth on glucose and other soluble sugars. The isoenzyme patterns of extracellular enzyme preparations of Neocallimastix strains were influenced by the growth substrate.

[Persistent renal bleeding treated with selective vascular embolisation with preservation of renal function]. / Persisterend renaal bloedverlies behandeld door selectieve vasculaire embolisatie, met behoud van nierfunctie.

In three patients with persistent blood loss from bleeding or abnormal renal vessels, kidney function was preserved by treatment with selective embolisation. The first patient, a 42-year-old woman, suffered from persistent haematuria after undergoing percutaneous nephrolithotripsy on the left side. Because conservative methods had failed and renal artery bleeding as a result of the lithotripsy was suspected, angiography with selective coil embolisation of a segmental branch of the lower pole artery of the kidney was performed. The second patient, a 40-year-old man with severe haemophilia A had been suffering from recurring macroscopic haematuria for a few months. CT showed an arteriovenous malformation in the right kidney. Angiography in combination with embolisation with two detachable balloons resulted in occlusion of the malformation. The third patient, a 23-year-old woman with tuberous sclerosis, presented with left abdominal pain, haematuria and decreasing haemoglobin concentrations. CT revealed a left renal angiomyolipoma, 10 cm in size, with a large internal haematoma. Three pathological branches of the upper pole renal artery were successfully occluded with Gianturco coils. At follow-up after 2, 2.5 and 2.5 years respectively, no recurrence of bleeding had occurred. Selective embolisation should be attempted as means of treatment for persistent renal bleeding if conservative treatment fails. Selective embolisation is minimally invasive and has the important advantage of preserving renal function.

Improved arterial visualization in cerebral CT perfusion-derived arteriograms compared with standard CT angiography: a visual assessment study.

BACKGROUND AND PURPOSE: Invasive cerebral DSA has largely been replaced by CTA, which is noninvasive but has a compromised arterial view due to superimposed bone and veins. The purpose of this study was to evaluate whether arterial visualization in CTPa is superior to standard CTA, which would eliminate the need for an additional CTA scan to assess arterial diseases and therefore reduce radiation dose. MATERIALS AND METHODS: In this study, we included 24 patients with subarachnoid hemorrhage for whom CTA and CTP were available. Arterial quality and presence of superimposed veins and bone in CTPa were compared with CTA and scored by 2 radiologists by using a VAS (0%-100%). Average VAS scores were determined and VAS scores per patient were converted to a 10-point NRS. Arterial visualization was considered to be improved when the highest rate (NRS 10, VAS > 90%) was scored for arterial quality, and the lowest rate (NRS 1, VAS < 10%), for the presence of superimposed veins and bone. A sign test with continuity correction was used to test whether the number of cases with these rates was significant. RESULTS: Average VAS scores in the proximal area were 94% (arterial quality), 4% (presence of bone), and 7% (presence of veins). In this area, the sign test showed that a significant number of cases scored NRS 10 for arterial quality (P < .02) and NRS 1 for the presence of superimposed veins and bone (P < .01). CONCLUSIONS: Cerebral CTPa shows improved arterial visualization in the proximal area compared with CTA, with similar arterial quality but no superimposed bone and veins.