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Familial hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome resulting from defective cytotoxicity. A previously healthy 3-month-old female presented with fever, irritability, abdominal distention, and tachypnea. She ultimately met all eight HLH-2004 diagnostic criteria, accompanied by elevated CXCL9. Initial empiric anti-inflammatory treatment included anakinra and IVIg, which stabilized ferritin and cytopenias. She had molecular and genetic confirmation of perforin deficiency and was started on dexamethasone and etoposide per HLH-94. She clinically improved, though CXCL9 and sIL-2Ra remained elevated. She was readmitted at week 8 for relapsed HLH without clear trigger and HLH-94 induction therapy was reinitiated. Her systemic HLH symptoms failed to respond and she soon developed symptomatic CNS HLH. She was incidentally found to have multifocal lung and kidney nodules, which were sterile and consisted largely of histiocytes and activated, oligoclonal CD8 T cells. The patient had a laboratory response to salvage therapy with alemtuzumab and emapalumab, but progressive neurologic decline led to withdrawal of care. This report highlights HLH foci manifest as pulmonary/renal nodules, demonstrates the utility of monitoring an array of HLH biomarkers, and suggests possible benefit of earlier salvage therapy.

Please see the Editorial Comment by Ryne Didier discussing this article. Background: MRI is the gold standard for neonatal brain imaging but is expensive, time-consuming, potentially limited by ava...

Purpose To determine the performance of Shannon entropy (SE) as a diagnostic tool in patients with mild traumatic brain injury (mTBI) with posttraumatic migraines (PTMs) and those without PTMs on the basis of analysis of fractional anisotropy (FA) maps. Materials and Methods The institutional review board approved this retrospective study, with waiver of informed consent. FA maps were obtained and neurocognitive testing was performed in 74 patients with mTBI (57 with PTM, 17 without PTM). FA maps were obtained in 22 healthy control subjects and in 20 control patients with migraine headaches. Mean FA and SE were extracted from total brain FA histograms and were compared between patients with mTBI and control subjects and between patients with and those without PTM. Mean FA and SE were correlated with clinical variables and were used to determine the areas under the receiver operating characteristic curve (AUCs) and likelihood ratios for mTBI and development of PTM. Results Patients with mTBI had significantly lower SE (P < .001) and trended toward lower mean FA (P = .07) compared with control subjects. SE inversely correlated with time to recovery (TTR) (r = -0.272, P = .02). Patients with mTBI with PTM had significantly lower SE (P < .001) but not mean FA (P = .15) than did other patients with mTBI. SE provided better discrimination between patients with mTBI and control subjects than mean FA (AUC = 0.92; P = .01), as well as better discrimination between patients with mTBI with PTM and those without PTM (AUC = 0.85; P < .001). SE of less than 0.751 resulted in a 16.1 increased likelihood of having experienced mTBI and a 3.2 increased likelihood of developing PTM. Conclusion SE more accurately reveals mTBI than mean FA, more accurately reveals those patients with mTBI who develop PTM, and inversely correlates with TTR. (©) RSNA, 2016.

To determine if a central axonal injury underlies neuropsychiatric symptoms after mild traumatic brain injury (mTBI) by using tract-based spatial statistics analysis of diffusion-tensor images.The institutional review board approved this study, with waiver of informed consent. Diffusion-tensor imaging and serial neurocognitive testing with the Immediate Post-Concussion Assessment and Cognitive Testing evaluation were performed in 45 patients with mTBI (38 with irritability, 32 with depression, and 18 with anxiety). Control subjects consisted of 29 patients with mTBI without neuropsychiatric symptoms. Fractional anisotropy and diffusivity maps were analyzed by using tract-based spatial statistics with a multivariate general linear model. Diffusion-tensor imaging findings were correlated with symptom severity, neurocognitive test scores, and time to recovery with the Pearson correlation coefficient.Compared with control subjects, patients with mTBI and depression had decreased fractional anisotropy in the superior longitudinal fasciculus (P = .006), white matter around the nucleus accumbens (P = .03), and anterior limb of the internal capsule (P = .02). Patients with anxiety had diminished fractional anisotropy in the vermis (P = .04). No regions of significantly decreased fractional anisotropy were seen in patients with irritability relative to control subjects. Injury in the region of the nucleus accumbens inversely correlated with recovery time in patients with depression (r = -0.480, P = .005).Unique white matter injury patterns were seen for two major posttraumatic neuropsychiatric symptoms. Injury to the cerebellar vermis in patients with mTBI and anxiety may indicate underlying dysfunction in primitive fear conditioning circuits in the cerebellum. Involvement of the nucleus accumbens in depression after mTBI may suggest an underlying dysfunctional reward circuit that affects the prognosis in these patients.

The purpose of this study was to investigate the effects of body mass index (BMI), thickness of the abdominal subcutaneous adipose tissue (ASAT), thickness of the mesenteric adipose tissue (MAT), weight and height on spinopelvic parameters.A total of 400 patients presented to the radiology department for whole abdominal computed tomography were included in the study. Patients' weight and height were measured to calculate BMI. Thickness of ASAT and MAT, lumbosacral angle (LSA), sacral slope (SS), pelvic tilt (PT), and pelvic incidence (PI) were measured from the images obtained. We analyzed the effects of BMI, ASAT, and MAT on spinopelvic parameters. In addition, patients included in the study were screened for isthmic spondylolisthesis and transitional vertebrae. Statistical analysis was carried out using SPSS 15.0 package software. p0.05 Values were considered statistically significant.BMI and thickness of ASAT and MAT increased with aging both in male and female individuals. There was a positive correlation between PI and weight, thickness of ASAT and thickness of MAT, while a negative correlation was found between PI and height (p0.01). SS angle was positively correlated with weight (p0.01). A weak positive correlation was found between PT angle and thickness of MAT and height (p0.05). The rate of transitional vertebrae and isthmic spondylolisthesis was 2 and 4.6%, consecutively. PI and SS values were significantly higher in the subjects having spondylolisthesis than normal population (p0.01).It should be kept in mind that obesity might increase the risk for development of spondylolisthesis by causing increase in PI angle.

The purpose of this article is to quantitatively assess the rate of resolution of clot burden detected on pulmonary CT angiography (CTA) in patients with acute pulmonary embolism (PE).We evaluated 111 consecutive patients (55 men and 56 women) in a retrospective cohort who were diagnosed with PE by pulmonary CTA and had at least one follow-up pulmonary CTA within 1 year. Two radiologists in consensus measured the volume of each clot using a semiautomated quantification program. Semiquantitative measures of clot burden were also computed. The resolution rates of the total clot volume, as well as clot volumes of the central (main and lobar) and peripheral vessels (segmental and subsegmental), were analyzed.The mean (± SD) clot volume per study was 3403.3 ± 6505.6 mm(3) at baseline and 531.6 ± 2383.5 mm(3) at the follow-up pulmonary CTA. Overall, 85 patients (77% ) showed complete resolution at the follow-up pulmonary CTA. Complete resolution was seen in 17 of 30 patients (56.7%) at a follow-up interval of 1-14 days, in 24 of 31 patients (77.4%) at 29-90 days, and in 32 of 34 patients (94.1%) after 90 days. The total clot volume measurements summed for all patients decreased by 78% (central clot, 69.4%; peripheral clot, 86.0%) at 14 days, by 96.6% (central clot, 93.4%; peripheral clot, 100%) at 90 days, and by 97.7% (central clot, 95.9%; peripheral clot, 100%) after 90 days.Clot burden resolved completely in 77% of patients during the follow-up period. Our analysis showed that clots resolved faster in the peripheral arteries than in the central pulmonary arteries.

A potential hazard in midline posterior fossa craniectomy may be the injury of vertebral artery. That's why vertebral artery evaluation prior to surgery may prevent dangerous complications. Advancements in multidetector computed tomography (MDCT) have provided detailed demonstration of the vertebral artery at the craniocervical junction and its relationships with atlas and axis. We aimed to define the normal anatomic relationship of the V2 and V3 part of the vertebral artery on MDCT. In total, 33 patients underwent MDCT angiography scan with suspected cranial aneurysm. V2 and V3 segments of vertebral artery were evaluated. Eight measurements (B, C, D, E, G, H, X, and Y line) were taken from MDCT images. For B and C, a line initially passing through the body of axis and spinous process and determining the midline was formed. Then, the vertical distance of vertebral artery from the level of transverse foramen of axis and loop to this midline was measured. For D and G, the vertical distance of vertebral artery to the midline from the upper and lower margin levels of transverse foramen of atlas was measured after a line establishing the midline passing through the anterior and posterior tubercles of atlas was drawn first. For E, transverse diameter of vertebral artery was measured at the loop level of V2 segment. For H, the vertical distance at the point where vertebral artery entered dura in the line passing from the midline of foramen magnum at anterior–posterior plane was measured. For X and Y, two different points of horizontal part of the vertebral artery were determined. One of these two points was the lateral one which was the origin of the horizontal part in the transverse foramen, the other was the intersection point on atlas. Average distances for both sides from transverse foramen of the axis, the loop of axoatlantal part and the lower border of the atlas of the vertebral artery to the midline were 20.97 mm on the right, 22.29 mm on the left; 27.19 mm on the right, 28.34 mm on the left; and 25.75 mm on the right and 27.21 mm on the left, respectively. Average distances for both sides from the upper border of the atlas, and at its penetration through dura were 27.40 mm on the right, 28.94 mm on the left; and 10.90 mm on the right and 10.93 mm on the left, respectively. Distances between spinous process and intersection of vertebral artery with horizontal part were 35.79 mm on the right and 36.63 mm on the left laterally, and 22.27 mm on the right and 22.62 mm on the left medially. MDCT angiography is a powerful test to demonstrate the vasculature of the head and neck. Bony structures and adjacent vessel morphology can be evaluated by this technique. The evaluation of craniocervical region prior to surgery with MDCT may be helpful to avoid intraoperative vascular injuries.