Gupta, Vivek

IgG4-related disease (IgG4RD) with intracranial involvement is rare. We report a 56-year-old male who had an excellent response to rituximab and dexamethasone after going undiagnosed for 5 years. After 3 years of rituximab maintenance, he has no evidence of disease on brain MRI.

AbstractALK‐positive ALCL can lead to rapid irreversible destruction of the optic pathway. Early treatment should be instituted in patients with CNS involvement or those at increased CNS risk.

OBJECTIVEThe role of CT perfusion (CTP) in the management of patients with acute ischemic stroke (AIS) remains a matter of debate. The primary aim of this study was to evaluate the correlation between the areas of infarction and penumbra on CTP scans and functional outcome in patients with AIS.METHODSThis was a retrospective review of 100 consecutively treated patients with acute anterior circulation ischemic stroke who underwent CT angiography (CTA) and CTP at admission between February 2011 and October 2014. On CTP, the volume of ischemic core and penumbra was measured using the Alberta Stroke Program Early CT Score (ASPECTS). CTA findings were also noted, including the site of occlusion and regional leptomeningeal collateral (rLMC) score. Functional outcome was defined by modified Rankin Scale (mRS) score obtained at discharge. Associations of CTP and CTA parameters with mRS scores at discharge were assessed using multivariable proportional odds logistic regression models.RESULTSThe median age was 67 years (range 19–95 years), and the median NIH Stroke Scale score was 16 (range 2–35). In a multivariable analysis adjusting for potential confounding variables, having an infarct on CTP scans in the following regions was associated with a worse mRS score at discharge: insula ribbon (p = 0.043), perisylvian fissure (p < 0.001), motor strip (p = 0.007), M2 (p < 0.001), and M5 (p = 0.023). A worse mRS score at discharge was more common in patients with a greater volume of infarct core (p = 0.024) and less common in patients with a greater rLMC score (p = 0.004).CONCLUSIONSThe results of this study provide evidence that several CTP parameters are independent predictors of functional outcome in patients with AIS and have potential to identify those patients most likely to benefit from reperfusion therapy in the treatment of AIS.

Introduction: White matter hyperintensities (WMH) is the most prominent neuroimaging feature of small vessel disease (SVD). SVD is thought to be a result of arteriolosclerosis, chronic hypoperfusion of white matter, selective oligodendrocyte death and degeneration of myelinated fibers. The risk of ischemic stroke is significantly increased with the presence of WMH. The presence of intracranial collaterals is a strong predictor of salvageable neurological function in acute stroke; however, the factors affecting the ability to recruit collaterals are poorly understood. We wanted to study if the presence and severity of small vessel white matter disease had an adverse effect on cerebral collateral flow in acute embolic large vessel stroke. Methods: We identified a consecutive series of adult subjects with acute embolic large vessel strokes confirmed on CT angiography (CTA) from a 10-year institutional database. Patients without a brain MRI within six months of CTA were excluded. Collateral scores of the affected hemisphere were calculated on CTA using the modified Tan scale (0-4) by two independent trained reviewers and confirmed by a neuroradiologist. The WMH was quantified using the Fazekas scale (0-3) incorporating both deep and periventricular components. Areas of restricted diffusion on brain MRI in the peri-stroke period were eliminated from the assessment of Fazekas score. Spearman’s correlation tests were performed for the association between collateral scores and WMH. Results: A total of 234 patients (mean age 70±14 years, 47% [110/234] women) satisfied the inclusion criteria. The prevalence of periventricular and deep WMH was 83% and 87% respectively. Aging had a significant impact on periventricular WMH (r=0.48, p <0.001) and deep WMH (r=0.47, p <0.001). However, collateral scores exhibited no statistically significant relationship with the presence or severity of periventricular scores (r=0.08, p=0.17) or deep WMH scores (r=0.01, p=0.77). Conclusions: Our results indicate that the presence of SVD does not affect cerebral collateral flow. The vascular niche and risk factors of SVD appear to be distinct from that of intracranial vessels responsible for collateral reperfusion of ischemic territory in acute large vessel embolic stroke.

Introduction: Poor leptomeningeal collateral flow is related to worse neurological outcome in acute ischemic stroke. The increased release of stress hormones during the first hours of acute stroke leads to inhibition of insulin secretion and increase insulin resistance, thus inducing stress hyperglycemia, which is often evident on blood glucose levels at admission. While admission hyperglycemia, independent of diabetes mellitus (DM) is associated with poor clinical outcome in ischemic stroke, studies on its effect on collateral circulation after acute cerebral embolism are largely inconclusive. Methods: We identified a consecutive series of patients aged 18 years and older from 2008 to 2018 with acute embolic large vessel stroke confirmed on CT angiography (CTA) using an institutional database. Individual CTAs were over-read, and collateral scores of the affected hemisphere were calculated using a modification of the Tan scale (0-4), by two trained readers. We reviewed the electronic medical records for presenting NIHSS, admission glucose and HbA1C levels, and prior history of DM. Spearman’s ρ, odds ratios (OR), and their 95% confidence intervals (CI) were analyzed. All tests were considered significant when P<0.05. Results: Of the 405 patients identified through the database (mean age 70 years [range, 55-85], 45% [183/405] women), 35% (142/405) had a prior history of DM. Mean admission glucose was 134±51 mg/dL, and median NIHSS at presentation was 17. Blood glucose level at admission was negatively correlated with collateral scores (ρ= -0.23, P=0.008). Specifically, glucose levels above 120 mg/dL were significantly associated (OR 1.68 CI 1.13-2.5) with poor collateral scores (≤1). However, collateral scores exhibited no statistically significant relationship with HbA1c (ρ=-0.08, P=0.15) or prior history of DM (P=0.73). Conclusions: Patients with higher admission glucose levels have poor collateral scores, while HbA1c and prior DM do not have a similar effect on collateral flow. Our results suggest that poor collateral flow may explain adverse outcomes associated with hyperglycemia in acute ischemic stroke. Determining if there is a benefit of aggressive glycemic control within the first few hours of embolic stroke warrants further study.

Introduction: Good collateral flow is associated with improved outcomes in acute embolic stroke. There is compelling data linking smoking to all types of stroke, with current smokers at a higher risk than former or never smokers. Smoking has, however, been shown to promote collaterals in coronary vascular bed in humans. It is unknown if there is a similar effect in cerebral collateral flow. We aimed to investigate if cigarette smoking was positively associated with collateral blood flow in a homogenous cohort of patients with acute large vessel occlusive embolic stroke. Methods: We identified all patients aged ≥18 with acute ischemic stroke showing embolic occlusion of ICA terminus/M1 MCA on admission CT angiography (CTA), between 2008 to 2018, through an institutional database. Patients with chronic occlusions and stenotic lesions were excluded. Individual CTAs were independently reviewed by two trained investigators to calculate collateral scores of the affected hemisphere using the modified Tan scale (0-4) as a reference. Details of cigarette smoking history were obtained from the electronic medical records. Patients were grouped into smokers (former and current smokers) and never smokers. Odds ratios (OR) and their 95% confidence intervals (CI) were estimated, and all tests were considered significant when P<0.05. Results: Of the 612 patients identified through the database, 429/612 patients (mean age 71±16 years, 45% [194/429] women) satisfied the inclusion criteria. 64.8% (278/429) had a positive history of smoking with an average of 47±12 pack-years. There was a significant association between good collateral scores (≥2) with smoking history (OR 1.74 CI 1.17-2.61, P=0.006). This trend remained significant even after comparing raw collateral scores (0-4) ( P=0.006, Spearman’s ρ=0.22). However, number of pack-years and collateral scores exhibited no statistically significant relationship (P=0.6). Conclusions: Our study shows that patients who have ever smoked have higher intracranial collateral vessel scores in the event of an acute embolic stroke. Increased impedance and reduced cerebral blood flow resulting from cigarette smoking may stimulate collateral recruitment in cerebral pial circulation, similar to the effect in coronary bed.

OBJECTIVE Although it is still early in its application, laser interstitial thermal therapy (LiTT) has increasingly been employed as a surgical option for patients with mesial temporal lobe epilepsy. This study aimed to describe mesial temporal lobe ablation volumes and seizure outcomes following LiTT across the Mayo Clinic’s 3 epilepsy surgery centers. METHODS This was a multi-site, single-institution, retrospective review of seizure outcomes and ablation volumes following LiTT for medically intractable mesial temporal lobe epilepsy between October 2011 and October 2015. Pre-ablation and post-ablation follow-up volumes of the hippocampus were measured using FreeSurfer, and the volume of ablated tissue was also measured on intraoperative MRI using a supervised spline-based edge detection algorithm. To determine seizure outcomes, results were compared between those patients who were seizure free and those who continued to experience seizures. RESULTS There were 23 patients who underwent mesial temporal LiTT within the study period. Fifteen patients (65%) had left-sided procedures. The median follow-up was 34 months (range 12–70 months). The mean ablation volume was 6888 mm 3 . Median hippocampal ablation was 65%, with a median amygdala ablation of 43%. At last follow-up, 11 (48%) of these patients were seizure free. There was no correlation between ablation volume and seizure freedom (p = 0.69). There was also no correlation between percent ablation of the amygdala (p = 0.28) or hippocampus (p = 0.82) and seizure outcomes. Twelve patients underwent formal testing with computational visual fields. Visual field changes were seen in 67% of patients who underwent testing. Comparing the 5 patients with clinically noticeable visual field deficits to the rest of the cohort showed no significant difference in ablation volume between those patients with visual field deficits and those without (p = 0.94). There were 11 patients with follow-up neuropsychological testing. Within this group, verbal learning retention was 76% in the patients with left-sided procedures and 89% in those with right-sided procedures. CONCLUSIONS In this study, there was no significant correlation between the ablation volume after LiTT and seizure outcomes. Visual field deficits were common in formally tested patients, much as in patients treated with open temporal lobectomy. Further studies are required to determine the role of amygdalohippocampal ablation.

Intraosseous hemangiomas arising from the nasal turbinate are exceedingly rare, with few reported cases in the literature. We describe a 61-year-old man found to have a nasal cavity mass on sinus computed tomograph (CT) and magnetic resonance imaging (MRI). Although an atypical site of occurrence, distinctive internal honeycomb bony trabeculations demonstrated on CT allowed the correct diagnosis of an intraosseous hemangioma to be prospectively proposed by the interpreting radiologist which had direct clinical and surgical impacts.

We report the case of a 66-year-old woman with tumor-induced osteomalacia (TIO) caused by fibroblast growth factor 23 (FGF-23) secreting mesenchymal tumor localized in a lumbar vertebra and review other cases localized to the axial skeleton. She presented with nontraumatic low back pain and spontaneous bilateral femur fractures. Laboratory testing was remarkable for low serum phosphorus, phosphaturia, and significantly elevated serum FGF-23 level. Magnetic resonance imaging (MRI) of the lumbar spine showed a focal lesion in the L-4 vertebra which was hypermetabolic on positron emission tomography (PET) scan. A computed tomography (CT) guided needle biopsy showed a low grade spindle cell neoplasm with positive FGF-23 mRNA expression by reverse transcriptase polymerase chain reaction (RT-PCR), confirming the diagnosis of a phosphaturic mesenchymal tumor mixed connective tissue variant (PMTMCT). The patient elected to have surgery involving anterior resection of L-4 vertebra with subsequent normalization of serum phosphorus. Including the present case, we identified 12 cases of neoplasms localized to spine causing TIO. To our knowledge, this paper represents the first documented case of lumbar vertebra PMT causing TIO. TIO is a rare metabolic bone disorder that carries a favorable prognosis. When a lesion is identifiable, surgical intervention is typically curative.

The case of a patient with intractable temporal lobe seizures and inadvertent unilateral intraventricular depth electrode placement is presented. The resting electroencephalograph (EEG) showed marked amplitude differences between the intraventricular electrode on the left and the parenchymal electrode on the right. All recorded seizures originated on the left side and in spite of its intraventricular location, frequency power spectra during the early ictal phase showed a marked increase in power for all frequency bands in the left depth electrode, exceeding that on the right. Analysis with Brain Electrical Source Analysis (BESA) software demonstrated marked ictal baseline shifts which were initially limited to the left side but changed to the right during clinical secondary generalization. In the immediate postictal state, all, except for infraslow, frequencies were markedly reduced in power. We conclude that intraventricular depth electrode contacts placed adjacent to the hippocampal structure can record interictal and ictal activity for all frequency bands, albeit at reduced amplitudes. Furthermore, infraslow activity can provide supplementary information about the epileptogenic zone.