Corticodependent and recurrent inflammatory pseudo tumor. Analysis of cases and review.

Inflammatory pseudo tumor (IP) is an infrequent process with benign evolution in most cases whose etiology and pathogenesis are unknown. It usually affects young men and children, in whom the macroscopic lesion can mimic a malignant process, which is ruled out after biopsy. Therefore, the diagnosis of certainty is histological and treatment consists of corticosteroids, leaving resection for cases in which biopsy is not possible or in which it produces local complications. We present a case of an inflammatory pseudo tumor with special corticodependence that began as a long-term periodic fever and splenic focal lesion that required splenectomy for its diagnosis and that, after decreasing the corticosteroid regimen, presented recurrences at the cerebellar and systemic level requiring the association of various immunosuppressants and rituximab to achieve remission. As a result of this case, we have performed an analysis of all the pseudo tumors diagnosed in adults in the hospitals of the province of Malaga, and it has been compared with that described in the bibliography.

Cenobamate and Clobazam Combination as Personalized Medicine in Autoimmune-Associated Epilepsy With Anti-Gad65 Antibodies.

BACKGROUND AND OBJECTIVES: Autoimmune-associated epilepsy (AAE) with antiglutamic acid decarboxylase 65 (GAD65) antibodies is considered a T-cell-mediated encephalitis that evolves to drug-resistant epilepsy. We do not have an effective therapeutic strategy for these patients. Because the GAD enzyme is primarily responsible for the conversion of glutamate to GABA, the mechanism of epileptogenesis in this condition predicts decreased levels of GABA content in synaptic vesicles. Cenobamate (CNB) acts as a positive allosteric modulator at synaptic and extra synaptic GABAA receptors, producing increased inhibitory neurotransmission in the brain. This mechanism could be especially beneficial in AAE with anti-GAD65 antibodies because it would be able to correct the imbalance due to the GABAergic stimulation deficit in postsynaptic neurons. METHODS: We recruit a retrospective multicentric consecutive case series of AAE with anti-GAD65 antibodies from 5 epilepsy units in Spain who have received treatment with CNB. RESULTS: A total of 8 patients were recruited. This cohort of highly refractory patients have failed a mean of 9.50 (SD = 3.20) ASM without control of seizures for sustained periods of time. The average number of seizures per month during the previous 3 months before CNB treatment was 19.63 (SD = 17.03). After the introduction of CNB improvement was achieved in all our patients, with a median reduction in the number of seizures of 92.22% (interquartile range [IQR]: 57.25-98.75). The mean follow-up was 156.75 days (SD = 68.23). In patients with concomitant treatment with clobazam (CLB), the median percentage of seizure reduction was higher than those not taking CLB: 94.72% (IQR: 87.25-100) vs 41.50% (p = 0.044) and also higher than the control group of patients with refractory epilepsy not related to anti-GAD65 treated with the same combination: 94.72% (IQR: 87.25-100) vs 45.00% (IQR: 25.00-87.00) (p = 0.019). DISCUSSION: Treatment with the combination CNB + CLB could be a type of personalized medicine in patients with AAE with anti-GAD65. Our preliminary data will need to be endorsed with new prospective and controlled studies.

Cutaneous and Vascular Deposits of 18F-NaF by PET/CT in the Follow-Up of Patients with Pseudoxanthoma Elasticum.

Active microcalcification of elastic fibers is a hallmark of pseudoxanthoma elasticum and it can be measured with the assessment of deposition of 18F-NaF using a PET/CT scan at the skin and vascular levels. It is not known whether this deposition changes over time in absence of specific therapy. We repeated in two years a PET/CT scan using 18F-NaF as a radiopharmaceutical in patients with the disease and compared the deposition at skin and vessel. Furthermore, calcium score values at the vessel wall were also assessed. Main results indicate in the vessel walls that calcification progressed in each patient; by contrast, the active microcalcification, measured and target-to-background ratio showed reduced active deposition. By contrast, at skin levels (neck and axillae) the uptake of the pharmaceutical remains unchanged. In conclusion, because calcification in the arterial wall is not specific for pseudoxanthoma elasticum condition, the measurement of the deposition of 18F-NaF in the neck might be potentially used as a surrogate marker in future trials for the disease.

Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum.

Pseudoxanthoma elasticum (PXE) is a genetic disease characterized by the calcification of elastin fibers. Our aim was to quantify vascular calcification in the arteries and the deposition of 18F-sodium-fluoride (18F-NaF) in the skin and vessel walls with positron emission tomography/computed tomography. This was an observational study including 18 patients with PXE. Vascular calcification was measured in Agatston units, and deposition in the skin and vessel walls was shown using target-to-background ratio (TBR). Severity of the disease was scored by Phenodex. We found higher vascular calcification in the popliteal, femoral, and aortic arch vessels compared to other vascular regions; however, the uptake of radiotracer was the highest in the aorta and femoral arteries. In the skin, the highest uptake was observed in the neck and the axillae. There was no significant association between 18F-NaF deposition in the arteries or skin and the global Phenodex score. In contrast, the Phenodex score was significantly associated in univariate analyses with the averaged vascular calcium score (p < 0.01). In the neck, patients with higher skin Phenodex scores exhibited higher radiotracer uptake. As a conclusion, because vascular calcification is physiological, our data suggested that the detection of cutaneous (neck) 18F-NaF deposits might serve to monitor the calcification process in the short-term for patients with PXE.

QModeling: a Multiplatform, Easy-to-Use and Open-Source Toolbox for PET Kinetic Analysis.

Kinetic modeling is at the basis of most quantification methods for dynamic PET data. Specific software is required for it, and a free and easy-to-use kinetic analysis toolbox can facilitate routine work for clinical research. The relevance of kinetic modeling for neuroimaging encourages its incorporation into image processing pipelines like those of SPM, also providing preprocessing flexibility to match the needs of users. The aim of this work was to develop such a toolbox: QModeling. It implements four widely-used reference-region models: Simplified Reference Tissue Model (SRTM), Simplified Reference Tissue Model 2 (SRTM2), Patlak Reference and Logan Reference. A preliminary validation was also performed: The obtained parameters were compared with the gold standard provided by PMOD, the most commonly-used software in this field. Execution speed was also compared, for time-activity curve (TAC) estimation, model fitting and image generation. QModeling has a simple interface, which guides the user through the analysis: Loading data, obtaining TACs, preprocessing the model for pre-evaluation, generating parametric images and visualizing them. Relative differences between QModeling and PMOD in the parameter values are almost always below 10-8. The SRTM2 algorithm yields relative differences from 10-3 to 10-5 when [Formula: see text] is not fixed, since different, validated methods are used to fit this parameter. The new toolbox works efficiently, with execution times of the same order as those of PMOD. Therefore, QModeling allows applying reference-region models with reliable results in efficient computation times. It is free, flexible, multiplatform, easy-to-use and open-source, and it can be easily expanded with new models.

Mesotelioma pleural maligno en adulto joven sin exposición conocida al asbesto / Malignant pleural mesothelioma in a young adult with no known exposure to asbestos

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Mild Developmental Foreign Accent Syndrome and Psychiatric Comorbidity: Altered White Matter Integrity in Speech and Emotion Regulation Networks.

Foreign accent syndrome (FAS) is a speech disorder that is defined by the emergence of a peculiar manner of articulation and intonation which is perceived as foreign. In most cases of acquired FAS (AFAS) the new accent is secondary to small focal lesions involving components of the bilaterally distributed neural network for speech production. In the past few years FAS has also been described in different psychiatric conditions (conversion disorder, bipolar disorder, and schizophrenia) as well as in developmental disorders (specific language impairment, apraxia of speech). In the present study, two adult males, one with atypical phonetic production and the other one with cluttering, reported having developmental FAS (DFAS) since their adolescence. Perceptual analysis by naïve judges could not confirm the presence of foreign accent, possibly due to the mildness of the speech disorder. However, detailed linguistic analysis provided evidence of prosodic and segmental errors previously reported in AFAS cases. Cognitive testing showed reduced communication in activities of daily living and mild deficits related to psychiatric disorders. Psychiatric evaluation revealed long-lasting internalizing disorders (neuroticism, anxiety, obsessive-compulsive disorder, social phobia, depression, alexithymia, hopelessness, and apathy) in both subjects. Diffusion tensor imaging (DTI) data from each subject with DFAS were compared with data from a group of 21 age- and gender-matched healthy control subjects. Diffusion parameters (MD, AD, and RD) in predefined regions of interest showed changes of white matter microstructure in regions previously related with AFAS and psychiatric disorders. In conclusion, the present findings militate against the possibility that these two subjects have FAS of psychogenic origin. Rather, our findings provide evidence that mild DFAS occurring in the context of subtle, yet persistent, developmental speech disorders may be associated with structural brain anomalies. We suggest that the simultaneous involvement of speech and emotion regulation networks might result from disrupted neural organization during development, or compensatory or maladaptive plasticity. Future studies are required to examine whether the interplay between biological trait-like diathesis (shyness, neuroticism) and the stressful experience of living with mild DFAS lead to the development of internalizing psychiatric disorders.

Structural and functional brain changes in middle-aged type 2 diabetic patients: a cross-sectional study.

BACKGROUND: Type 2 diabetes mellitus (T2DM) is an emerging risk factor for cognitive impairment. Whether this impairment is a direct effect of this metabolic disorder on brain function, a consequence of vascular disease, or both, remains unknown. Structural and functional neuroimaging studies in patients with T2DM could help to elucidate this question. OBJECTIVE: We designed a cross-sectional study comparing 25 T2DM patients with 25 age- and gender-matched healthy control participants. Clinical information, APOE genotype, lipid and glucose analysis, structural cerebral magnetic resonance imaging including voxel-based morphometry, and F-18 fluorodeoxyglucose positron emission tomography were obtained in all subjects. METHODS: Gray matter densities and metabolic differences between groups were analyzed using statistical parametric mapping. In addition to comparing the neuroimaging profiles of both groups, we correlated neuroimaging findings with HbA1c levels, duration of T2DM, and insulin resistance measurement (HOMA-IR) in the diabetic patients group. RESULTS: Patients with T2DM presented reduced gray matter densities and reduced cerebral glucose metabolism in several fronto-temporal brain regions after controlling for various vascular risk factors. Furthermore, within the T2DM group, longer disease duration, and higher HbA1c levels and HOMA-IR were associated with lower gray matter density and reduced cerebral glucose metabolism in fronto-temporal regions. CONCLUSION: In agreement with previous reports, our findings indicate that T2DM leads to structural and metabolic abnormalities in fronto-temporal areas. Furthermore, they suggest that these abnormalities are not entirely explained by the role of T2DM as a cardiovascular risk factor.

Clinical usefulness of coregistered 11C-methionine positron emission tomography/3-T magnetic resonance imaging at the follow-up of acromegaly.

OBJECTIVE: This study sought to characterize the utility of coregistered 11C-methionine positron emission tomography (MET-PET) with 3-T magnetic resonance imaging (3T MRI) in the diagnosis and follow-up of pituitary adenomas in patients with acromegaly and to compare MET-PET and 18F-fluorodeoxyglucose emission tomography (FDG-PET) for the evaluation of active or recurrent disease. METHODS: This is a prospective observational study. It included a total of 17 patients, 6 patients with a new diagnosis of acromegaly and 11 patients who had previously undergone resection of a confirmed growth hormone-secreting adenoma. The study protocol consisted of preoperative and postoperative evaluation with 3T MRI, and both MET-PET and FDG-PET. Coregistration of 3T MRI/MET-PET was accomplished. RESULTS: In all patients who underwent preoperative imaging, MET-PET demonstrated increased uptake coincident with location of the pituitary lesion on 3T MRI. In the postoperative group, the coregistered 3T MRI/MET-PET demonstrated evidence of residual tumor in all patients with active disease. MET-PET sensitivity was 86% and specificity was 86% for the diagnosis of recurrence. CONCLUSIONS: MET-PET is a sensitive technique for diagnosing persistent acromegaly, and its coregistration with 3T MRI has demonstrated a better definition of the interface, extension, and location of the lesion in the management of active postoperative acromegaly.