It has been hypothesized that many of the cognitive impairments commonly seen after right brain damage (RBD) can be characterized as a failure to build or update mental models. We (Danckert et al. in Neglect as a disorder of representational updating. NOVA Open Access, New York, 2012a; Cereb Cortex 22:2745-2760, 2012b) were the first to directly assess the association between RBD and updating and found that RBD patients were unable to exploit a strongly biased play strategy in their opponent in the children's game rock, paper, scissors. Given that this game required many other cognitive capacities (i.e., working memory, sustained attention, reward processing), RBD patients could have failed this task for various reasons other than a failure to update. To assess the generality of updating deficits after RBD, we had RBD, left brain-damaged (LBD) patients and healthy controls (HCs) describe line drawings that evolved gradually from one figure (e.g., rabbit) to another (e.g., duck) in addition to the RPS updating task. RBD patients took significantly longer to alter their perceptual report from the initial object to the final object than did LBD patients and HCs. Although both patient groups performed poorly on the RPS task, only the RBD patients showed a significant correlation between the two, very different, updating tasks. We suggest these data indicate a general deficiency in the ability to update mental representations following RBD.

Background: Children with Down syndrome frequently develop infantile spasms (IS); however, variable seizure outcome has been reported and not much is known about the clinical-electrophysiological factors affecting these outcomes. Therefore, we evaluated the clinical, neuroimaging and electrophysiological data in our DS with IS patient cohort, with a relatively long follow-up duration, to address some of these issues.  Methods: Disease characteristics, diagnostic tests and treatment outcomes from 15 children (F: M=7:8) with IS and DS (follow-up duration: 10-197 months) were analyzed. Results: The median age at onset of spasms was 7 (2-16) months. EEG showed hypsarrhythmia in 10 infants, and patterns of diffusely disorganised background with multifocal independent spike-wave activity in the remaining five. Brain MRI (n=8) revealed no abnormality in 3, microcephaly in 2, delayed myelination in one, and minimal volume changes in 2. PET scans (n=5) showed diffuse glucose hypometabolism in 3, focal hypo- or hypermetabolism in 1 and 2 patients, respectively, and increased or decreased basal-ganglia metabolism in one each. Spasms disappeared in 8 patients (53.3%; six off medication), with girls (5/7; 71%) responding slightly better than boys (3/8; 37.5%). Seven of the 8 responders had shown hypsarrhythmia. Cessation of spasms was achieved by ACTH alone (n=1) or in combination with vigabatrin or zonisamide (n=1, each), and monotherapy with zonisamide (n=2), topiramate (n=1), prednisone (n=1), and ketogenic diet (n=1). Two patients showed evolution into complex partial seizure or Lennox-Gastaut syndrome. One child had dystonic cerebral palsy associated with perinatal asphyxia. Conclusions: Spasms developed relatively later in patients with IS and DS, compared to cryptogenic IS patients, and showed modest response to treatment. Girls appear to have a better treatment response, with presence of hypsarrhythmia may signal a better treatment outcome.

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Introduction Intravenous tenecteplase (TNK) is currently being used as a thrombolytic agent in acute ischemic stroke (AIS) and has has been shown to be non‐inferior to intravenous alteplase according to recent studies. Intracranial hemorrhage (ICH) as a complication of alteplase is approximately at 6%. The aim of our study was to determine the rate of significant ICH in patients receiving TNK indicated for AIS in a real world setting. Methods A network‐wide (3 CSCs, 6 PSCs), multicenter retrospective chart review of patients receiving TNK from February 2020 to January 2022 was performed using the Get With The Guidelines database. TNK bolus dose of 0.25mg/kg was used according to a network‐wide policy. ICH was categorized using ECASS‐3 criteria. Fisher exact test statistic was used to determine if a significant association existed between the presence of ICH and baseline ASPECTS score, endovascular treatment (EVT), and IV eptifibatide use. A benchmark less than 2% PH‐2 incidence was set based on historical alteplase related PH‐2 rates within our network. Social science statistics software was used for data analysis. Results Out of 180 patients who received TNK, 25 subjects (13.89%) developed hemorrhagic transformation. Mean age was 71.88 (95% CI 65.54, 78.22). Forty‐eight percent of subjects were female. Median ASPECTS score was 8 (95% CI 7.54, 8.78). Median 90 day mRS was 3 (95% CI 2.1, 3.9). Hemorrhagic transformation was classified as HI‐1 in 5% (n = 9), HI‐2 in 1.7% (n = 3), PH‐1 in 3.8% (n = 7), and PH‐2 in 3.3% (n = 6) subjects. No significant difference between subjects with other subtypes versus PH‐2 was identified when adjusting for ASPECTS score > = 7 versus < 7 (Fisher value = 1), EVT versus no EVT (Fisher value = 0.65), or use of IV eptifibatide (Fisher value = 0.06). Conclusions Tenecteplase is associated with higher rates of PH‐2 intracranial hemorrhage when compared with our benchmark rates of alteplase‐related PH‐2. This study is significantly limited by small sample size, retrospective nature, and uncontrolled variables. Larger, prospective studies are needed to validate our results.