Neurocognitive speed associates with frontotemporal lobar degeneration TDP-43 subtypes

Frontotemporal lobar degeneration (FTLD) is pathologically heterogeneous with the TAR DNA binding protein 43 kDa (TDP-43) proteinopathy the most common substrate. Previous work has identified atrophy patterns across TDP-43 subtypes with Type A showing greater frontotemporal and parietal atrophy, Type C predominantly anterior temporal, and Type B predominantly posterior frontal. Despite neuroanatomical correlates of involvement, neuropsychological findings have been inconsistent. The current study utilized broader neurocognitive domains based on aggregated neuropsychological measures to distinguish between subtypes. We hypothesized that patterns of neurocognitive domain impairments would predict FTLD–TDP subtype. Fifty-one patients, aged 38–87, were identified post mortem with pathologically confirmed FTLD with TDP-43. Participants were classified into subtypes A, B, or C. Patients had completed neuropsychological assessments as part of their clinical evaluation. Six cognitive domains were created: Language; Cognitive Speed; Memory; Learning; Visuoperception; and Fluency. Binary logistic regression was conducted. All but three patients could be classified as FTLD–TDP Types A, B, or C: 26 as Type A; nine as Type B; and 13 as Type C. Cognitive Speed scores were associated with Types A and C (p < 0.001 and p = 0.003, respectively). Impaired performances on the Trail Making Test differentiated Types A and C. Worse Boston Naming Test and Logical Memory (Immediate) (p < 0.05) scores also increased the likelihood of Type C phenotype. Findings suggest Cognitive Speed associates with TDP-43 subtypes. Type C also demonstrated language-specific involvement. Differences between TDP-43 subtypes further supports the notion of differences in pathophysiology or topography across these types.     

What have we learned about ictal epileptic headache? A review of well-documented cases

PurposeThe case report published in this issue by Wang et al. offers us an opportunity to review previously published “ictal epileptic headache” cases and draw attention to the criteria that have recently been published for this condition, taking into consideration not only the clinical-EEG and physiopathogenetic investigations required to diagnose this condition, but also the therapeutic aspects of the issue.MethodsTo this aim we reviewed all well-documented cases that have been reported in the literature.ResultsThe relationship between headache and seizures is somewhat complicated. Although the nature of this association is not yet fully clear, several plausible explanations have been proposed. Further experimental and clinical investigations are, however, warranted to gain a better understanding of this relationship. Epilepsy and idiopathic headache/migraine share several pathophysiological mechanisms; a better understanding of these mechanisms will allow us to more accurately to assess the “real burden” and prevalence of the “ictal epileptic headache” phenomenon and its therapeutic implications.ConclusionsThe development of animal models and molecular studies and, above all, multicenter clinical studies conducted according to the proposed IEH criteria represent the starting point for a definitive international consensus on this intriguing topic. In addition, to improve the recognition of ictal epileptic headache, we should encourage the use of EEG recording in the emergency setting.     

Impact of aging on cardiac sympathetic innervation measured by <Superscript>123</Superscript>I-mIBG imaging in patients with systolic heart failure

Purpose

Sympathetic nervous system (SNS) hyperactivity is a salient characteristic of chronic heart failure (HF) and contributes to the progression of the disease. Iodine-123 meta-iodobenzylguanidine (¹²³I-mIBG) imaging has been successfully used to assess cardiac SNS activity in HF patients and to predict prognosis. Importantly, SNS hyperactivity characterizes also physiological ageing, and there is conflicting evidence on cardiac ¹²³I-mIBG uptake in healthy elderly subjects compared to adults. However, little data are available on the impact of ageing on cardiac sympathetic nerve activity assessed by ¹²³I-mIBG scintigraphy, in patients with HF.

Methods and results

We studied 180 HF patients (age?=?66.1?±?10.5 years [yrs]), left ventricular ejection fraction (LVEF?=?30.6?±?6.3 %) undergoing cardiac ¹²³I-mIBG imaging. Early and late heart to mediastinum (H/M) ratios and washout rate were calculated in all patients. Demographic, clinical, and echocardiographic data were also collected. Our study population consisted of 53 patients aged >75 years (age?=?77.7?±?4.0 year), 67 patients aged 62–72 years (age?=?67.9?±?3.2 years) and 60 patients aged ≤61 year (age?=?53.9?±?5.6 years). In elderly patients, both early and late H/M ratios were significantly lower compared to younger patients (p?<?0.05). By multivariate analysis, H/M ratios (both early and late) and washout rate were significantly correlated with LVEF and age.

Conclusions

Our data indicate that, in a population of HF patients, there is an independent age-related effect on cardiac SNS innervation assessed by ¹²³I-mIBG imaging. This finding suggests that cardiac ¹²³I-mIBG uptake in patients with HF might be affected by patient age.

     

Imaging of pediatric thyroid tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper

Pediatric thyroid cancer is rare in children; however, incidence is increasing. Papillary thyroid cancer and follicular thyroid cancer are the most common subtypes, comprising about 90% and 10% of cases, respectively. This paper provides consensus imaging recommendations for evaluation of pediatric patients with thyroid cancer at diagnosis and during follow-up.     

Imaging of pediatric adrenal tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper

Adrenal tumors other than neuroblastoma are uncommon in children. The most frequently encountered are adrenocortical carcinoma and pheochromocytoma. This paper offers consensus recommendations for imaging of pediatric patients with a known or suspected primary adrenal malignancy other than neuroblastoma at diagnosis and during follow-up.     

Impact of diagnostic and end-of-induction Curie scores with tandem high-dose chemotherapy and autologous transplants for metastatic high-risk neuroblastoma: A report from the Children's Oncology Group

Background

Diagnostic mIBG (meta-iodobenzylguanidine) scans are an integral component of response assessment in children with high-risk neuroblastoma. The role of end-of-induction (EOI) Curie scores (CS) was previously described in patients undergoing a single course of high-dose chemotherapy (HDC) and autologous hematopoietic cell transplant (AHCT) as consolidation therapy.

Objective

We now examine the prognostic significance of CS in patients randomized to tandem HDC and AHCT on the Children's Oncology Group (COG) trial ANBL0532.

Study design

A retrospective analysis of mIBG scans obtained from patients enrolled in COG ANBL0532 was performed. Evaluable patients had mIBG-avid, International Neuroblastoma Staging System (INSS) stage 4 disease, did not progress during induction therapy, consented to consolidation randomization, and received either single or tandem HDC (n = 80). Optimal CS cut points maximized the outcome difference (≤CS vs. >CS cut-off) according to the Youden index.

Results

For recipients of tandem HDC, the optimal cut point at diagnosis was CS = 12, with superior event-free survival (EFS) from study enrollment for patients with CS ≤ 12 (3-year EFS 74.2% ± 7.9%) versus CS > 12 (59.2% ± 7.1%) (p = .002). At EOI, the optimal cut point was CS = 0, with superior EOI EFS for patients with CS = 0 (72.9% ± 6.4%) versus CS > 0 (46.5% ± 9.1%) (p = .002).

Conclusion

In the setting of tandem transplantation for children with high-risk neuroblastoma, CS at diagnosis and EOI may identify a more favorable patient group. Patients treated with tandem HDC who exhibited a CS ≤ 12 at diagnosis or CS = 0 at EOI had superior EFS compared to those with CS above these cut points.     

Genomic Surveillance of Circulating SARS-CoV-2 in South East Italy: A One-Year Retrospective Genetic Study

In order to provide insights into the evolutionary and epidemiological viral dynamics during the current COVID-19 pandemic in South Eastern Italy, a total of 298 genomes of SARS-CoV-2 strains collected in the Apulia and Basilicata regions, between March 2020 and January 2021, were sequenced. The genomic analysis performed on the draft genomes allowed us to assign the genetic clades and lineages of belonging to each sample and provide an overview of the main circulating viral variants. Our data showed the spread in Apulia and Basilicata of SARS-CoV-2 variants which have emerged during the second wave of infections and are being currently monitored worldwide for their increased transmission rate and their possible impact on vaccines and therapies. These results emphasize the importance of genome sequencing for the epidemiological surveillance of the new SARS-CoV-2 variants’ spread.