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Background and Purpose: Primary lateral sclerosis (PLS) is a progressive upper motor neuron (UMN) disorder. It is debated whether PLS is part of the amyotrophic lateral sclerosis (ALS) spectrum, or a syndrome encompassing different neurodegenerative diseases. Recently, new diagnostic criteria for PLS have been proposed. We describe four patients of two pedigrees, meeting definite PLS criteria and harboring two different mutations in presenilin 1 (PSEN1). Methods: Patients underwent neurological and neuropsychological examination, MRI, 18F-fluorodeoxyglucose positron emission tomography (FDG-PET), amyloid-related biomarkers, and next-generation sequencing (NGS) testing. Results: Four patients, aged 25–45 years old, presented with a progressive UMN syndrome meeting clinical criteria of definite PLS. Cognitive symptoms and signs were mild or absent during the first year of the disease but appeared or progressed later in the disease course. Brain MRI showed microbleeds in two siblings, but iron-related hypointensities in the motor cortex were absent. Brain FDG-PET showed variable areas of hypometabolism, including the motor cortex and frontotemporal lobes. Amyloid deposition was confirmed with either cerebrospinal fluid (CSF) or imaging biomarkers. Two heterozygous likely pathogenic mutations in PSEN1 (p.Pro88Leu and p.Leu166Pro) were found in the NGS testing. Conclusion: Clinically defined PLS is a syndrome encompassing different neurodegenerative diseases. The NGS testing should be part of the diagnostic workup in patients with PLS, at least in those with red flags, such as early-onset, cognitive impairment, and/or family history of neurodegenerative diseases. [ABSTRACT FROM AUTHOR]

18F-FBB PET is a neuroimaging modality that is been increasingly used to assess brain amyloid deposits in potential patients with Alzheimer's disease (AD). In this work, we analyze the usefulness of these data to distinguish between AD and non-AD patients. A dataset with 18F-FBB PET brain images from 94 subjects diagnosed with AD and other disorders was evaluated by means of multiple analyses based on t-test, ANOVA, Fisher Discriminant Analysis and Support Vector Machine (SVM) classification. In addition, we propose to calculate amyloid standardized uptake values (SUVs) using only gray-matter voxels, which can be estimated using Computed Tomography (CT) images. This approach allows assessing potential brain amyloid deposits along with the gray matter loss and takes advantage of the structural information provided by most of the scanners used for PET examination, which allow simultaneous PET and CT data acquisition. The results obtained in this work suggest that SUVs calculated according to the proposed method allow AD and non-AD subjects to be more accurately differentiated than using SUVs calculated with standard approaches. [ABSTRACT FROM AUTHOR]

We present a case of a 60-year-old man with a history of severe hypoproteinemia and constitutional syndrome, suspected to have protein-losing enteropathy (PLE). Bone scintigraphy (Tc-MDP) performed to rule out the presence of bone metastases incidentally showed abnormal uptake in abdominal soft tissue. The patient unexpectedly died of heart failure, and autopsy revealed microscopic alterations consistent with PLE exclusively in the right colon, corresponding to the area of abnormal uptake. Few similar cases have been published, but none of them reported correlative pathological findings affecting the area of abnormal tracer uptake. In this case of PLE, Tc-MDP scintigraphy was a useful imaging method for localizing the site of protein loss, showing a focal area of alteration in the right colon. This finding could also have been of great help in case that surgery had been finally performed to control the protein loss. [ABSTRACT FROM AUTHOR]

Backgound: In the workup of follicular lymphoma (FL), bone marrow biopsy (BMB) assessment is a key component of FLIPI and FLIPI2, the most widely used outcome scores. During the previous decade, several studies explored the role of FDG-PET/CT for detecting nodal and extranodal disease, with only one large study comparing both techniques., Methods: The aim of our study was to evaluate the diagnostic accuracy and the prognostic impact of both procedures in a retrospective cohort of 299 FL patients with both tests performed at diagnosis. In order to avoid a collinearity bias, FLIPI2 was deconstructed in its founding parameters, and the bone marrow involvement (BMI) parameter separately included as: a positive BMB, a positive PET/CT, the combined "PET/CT and BMB positive" or "PET/CT or BMB positive". These variables were also confronted independently with the POD24 in 233 patients treated with intensive regimens., Results: In the total cohort, bone marrow was involved in 124 and 60 patients by BMB and PET/CT, respectively. In terms of overall survival, age > 60 y.o. and the combined "PET/CT or BMB positive" achieved statistical independence as a prognostic factor. In patients treated with an intensive regimen, only the combined "PET/CT or BMB positive" added prognostic value for a shorter overall survival, when confronted with the POD24., Conclusion: Our results show that in FL both BMB and PET/CT should be considered at diagnosis, as their combined assessment provides independent prognostic value in the context of the most widely use clinical scores., (© 2022 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Objective: To validate the sentinel lymph node selective biopsy (SLNSB) in the staging of Prostate Cancer with Briganti Index > 5 by comparison with extended lymphadenectomy (ePLND) in a prospective longitudinal study., Methods: SLNSB has been performed in 84 patients, the first 70 by injection of nanocoloids marked with Tc99m and preoperative SPECT-CT, and in the last 14 with mixed radiotracer (99mTc + ICG). After laparoscop ic removal of sentinel nodes all patients underwent an ePLND., Results: SPECT-CT showed radiotracer deposits outside the territory of the ePLND in 76% of patients and laparoscopic gamma probe in 57%. The median number of sentinel nodes removed was 5.2 with a total average number of lymph nodes removed of 22. In all cases with metastatic nodes (28% in the series) there was at least one positive sentinel node but metastatic sentinel nodes outside of the territory of the ePLND were found in 6/24 patients (25%). The sensitivity, specificity, PPV and NPV of 99mTc were 100%, 96.07%, 90.47% and 100%, respectively. In 5 out of 14 patients with mixed radiotracer, lymph node involvement was detected. In all of them there was at least one sentinel node affected with 99mTc, and only 3 showed fluorescence with 100% sensitivity and 100% NPV for 99mTc and 60% sensitivity and 77.77% NPV for ICG., Conclusion: The SLNSB with 99mTc has a high sensitivity and a VPN of 100%, increasing the identification of lymphatic metastases outside the territory of the ePLND. Fluorescence can facilitate the visualization of the sentinel nodes when they have been previously located by the SPECT-CT, although the sensitivity and the NPV of the ICG are lower than that of the 99mTc.

18 F-FBB PET is a neuroimaging modality that is been increasingly used to assess brain amyloid deposits in potential patients with Alzheimer's disease (AD). In this work, we analyze the usefulness of these data to distinguish between AD and non-AD patients. A dataset with 18 F-FBB PET brain images from 94 subjects diagnosed with AD and other disorders was evaluated by means of multiple analyses based on t -test, ANOVA, Fisher Discriminant Analysis and Support Vector Machine (SVM) classification. In addition, we propose to calculate amyloid standardized uptake values (SUVs) using only gray-matter voxels, which can be estimated using Computed Tomography (CT) images. This approach allows assessing potential brain amyloid deposits along with the gray matter loss and takes advantage of the structural information provided by most of the scanners used for PET examination, which allow simultaneous PET and CT data acquisition. The results obtained in this work suggest that SUVs calculated according to the proposed method allow AD and non-AD subjects to be more accurately differentiated than using SUVs calculated with standard approaches.

Unlabelled: Our objective was to determine the stability of stabilized (99m)Tc-hexamethylpropylene amine oxime ((99m)Tc-d,l-HMPAO) dispensed by vial and syringe, with the storage time and labeling activity varied., Methods: (99m)Tc-d,l-HMPAO was labeled according to the manufacturer's instructions, but with modification of the (99m)TcO(4)Na activity. Two groups were prepared: 1,110 MBq (30 mCi) and 2,600-3,700 MBq (70.3-100 mCi). Five minutes after labeling, the radiochemical purity (RCP) of the vial content was determined. Afterward, the same activity was distributed into two 2-mL syringes and into the manufacturer's vial. In one of the syringes, the radiopharmaceutical stayed in contact with the needle for 4 h. At 2 and 4 h after labeling, the RCP of the vial and syringe content was checked and compared., Results: The mean RCP of stabilized (99m)Tc-d,l-HMPAO labeled with 1,110 MBq (30 mCi) and stored in a vial decreased from 93.1% at 5 min to 92.1% at 2 h and to 91.1% at 4 h. With storage in a syringe, the RCP decreased from 89.8% at 2 h to 88.7% at 4 h. This diminution increased for labeling with higher activities (2,600-3,700 MBq [70.3-100 mCi]), ranging from 91.4% at 5 min, 89.0% at 2 h, and 85.3% at 4 h in a vial and from 85.9% at 2 h to 80.2% in a syringe. (99m)TcO(2) and secondary (99m)Tc-HMPAO were the main impurities at t = 0. (99m)TcO(4)(-) was an impurity that increased with time in both vials and syringes but significantly so in syringes. All these impurities were higher with labeling activities in the range of 2,600-3,700 MBq (70.3-100 mCi). Contact of the needle with (99m)Tc-d,l-HMPAO sharply decreased the RCP to 57.1% at 4 h., Conclusion: The RCP of stabilized (99m)Tc-d,l-HMPAO decreases significantly in both vials and syringes with high labeling activities. The product is less stable when stored in a syringe than in a vial. The fraction of dose in contact with the needle affects the RCP results.