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INTRODUCTION: Sleep and rest-activity rhythm alterations are common in neurodegenerative diseases. However, their characterization in patients with behavioral variant frontotemporal dementia (bvFTD) has proven elusive. We investigated rest-activity rhythm alterations, sleep disturbances, and their neural correlates in bvFTD. METHODS: Twenty-seven bvFTD patients and 25 healthy controls completed sleep questionnaires and underwent 7 days of actigraphy while concurrently maintaining a sleep diary. Cortical complexity and thickness were calculated from T1-weighted magnetic resonance (MR) images. RESULTS: Compared to controls, bvFTD patients showed longer time in bed (95% confidence interval [CI]: 79.31, 321.83) and total sleep time (95% CI: 24.38, 321.88), lower sleep efficiency (95% CI: -12.58, -95.54), and rest-activity rhythm alterations in the morning and early afternoon. Increased sleep duration was associated with reduced cortical thickness in frontal regions. DISCUSSION: Patients with bvFTD showed longer sleep duration, lower sleep quality, and rest-activity rhythm alterations. Actigraphy could serve as a cost-effective and accessible tool for ecologically monitoring changes in sleep duration in bvFTD patients. HIGHLIGHTS: We assessed sleep and circadian rhythms in behavioral variant frontotemporal dementia (bvFTD) using actigraphy. Patients with bvFTD show increased sleep duration and reduced sleep quality. Patients with bvFTD show rest-activity alterations in the morning and early afternoon. Sleep duration is associated with reduced cortical thickness in frontal regions. These alterations may represent an early sign of neurodegeneration.
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Demência Frontotemporal , Humanos , Demência Frontotemporal/diagnóstico por imagem , Sono , Ritmo Circadiano , Imageamento por Ressonância Magnética/métodos , DescansoRESUMO
OBJECTIVE: Pectus excavatum (PE) repair is burdened by severe postoperative pain. This retrospective study aimed to determine whether the analgesic effect of ultrasound-guided erector spinae plane block (ESPB) plus standard intravenous analgesia (SIVA) might be superior to SIVA alone in pain control after PE surgical repair via Ravitch or Nuss technique. DESIGN: A retrospective cohort study. SETTING: At a university hospital. PARTICIPANTS: All participants were scheduled for surgical repair of PE. INTERVENTIONS: From January 2017 to December 2019, all patients who received ESPB plus SIVA or SIVA alone were investigated retrospectively. A 2:1 propensity-score matching analysis considering preoperative variables was used to compare analgesia efficacy in 2 groups. All patients received a 24-hour continuous infusion of tramadol, 0.1 mg/kg/h, and ketorolac, 0.05 mg/kg/h, via elastomeric pump, and morphine, 2 mg, intravenously as a rescue drug. The ESPB group received preoperative bilateral ESPB block. Postoperative pain, reported using a numerical rating scale at 1, 12, 24, and 48 hours after surgery; the number of required rescue doses; total postoperative morphine milligram equivalents consumption; and the incidence of postoperative nausea and vomit were analyzed. MEASUREMENT AND MAIN RESULTS: A total of 105 patients were identified for analysis. Propensity-score matching resulted in 38 patients in the SIVA group and 19 patients in the ESPB group. Postoperative pain, the number of rescue doses, and postoperative nausea and vomit incidences were lower in the ESPB group (p < 0.005). CONCLUSIONS: Erector spinae plane block may be an effective option for pain management after surgical repair of PE as part of a multimodal approach. This study showed good perioperative analgesia, opioid sparing, and reduced opioid-related adverse effects.
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Tórax em Funil , Bloqueio Nervoso , Humanos , Estudos Retrospectivos , Tórax em Funil/cirurgia , Bloqueio Nervoso/métodos , Analgésicos Opioides , Náusea e Vômito Pós-Operatórios/cirurgia , Dor Pós-Operatória/diagnóstico , Dor Pós-Operatória/etiologia , Dor Pós-Operatória/prevenção & controle , Ultrassonografia de Intervenção , Derivados da Morfina/uso terapêuticoRESUMO
Mutations in the PSEN1 gene are the most common cause of autosomal dominant Alzheimer's disease, and are characterized by a high phenotype variability. This study describes a five-generation family, with a prevalent late-onset of the disease and a high frequency of depression, in which a new missense mutation (c.789T > G, p.Cys263Trp) in exon 8 of the PSEN1 gene was found. Only the proband presented an early onset at the age of 45 with attention deficit, followed by spatial disorientation, psychiatric symptoms and parkinsonian signs. The other two cases had a late onset of the disease and a typical presentation with memory loss. Both were characterized by a high level of anxiety and depression. The disease course was different with signs of Lewy body dementia for the proband's mother, and pyramidal involvement and a shorter disease duration for the proband's maternal aunt. The other eight cases with late-onset dementia and three cases with a long history of depression have been reported in the family pedigree, underlying the high phenotype variability of PSEN1 mutations.
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Doença de Alzheimer/genética , Família , Mutação de Sentido Incorreto , Linhagem , Presenilina-1/genética , Idade de Início , Substituição de Aminoácidos , Feminino , Humanos , Itália , Masculino , Pessoa de Meia-IdadeRESUMO
PURPOSE: Acromegaly is usually due to growth hormone (GH)-secreting pituitary adenomas, but it may be exceptionally caused by GH-secreting ectopic pituitary adenomas (EPA). EPA are defined as extra-sellar pituitary tumours, extra- or intra-cranially sited, entirely separated from the pituitary stalk and gland. The aim of the study is to address the challenges in the management of clival GHEPA. METHODS: We reported a case of a 53-year-old acromegalic patient with a primary clival GHEPA and reviewed systematically the relevant English literature between 1975 and 2019, in keeping with the PRISMA guidelines. RESULTS: Four cases of primary clival GHEPA have been described in literature apart from ours. All patients presented with acromegalic features, elevated circulating GH and/or insulin-like growth factor-1 levels. Hyperprolactinemia and empty sella were described in two cases, respectively. These tumours show the typical imaging characteristics of pituitary adenomas, but their neuroradiological diagnosis may be challenging due to their sizes and the difficulty in defining the absence of connections with the pituitary fossa. CONCLUSION: Although primary clival GHEPA are exceedingly rare, even if likely under-reported in literature, they should be considered in the differential diagnosis of clival tumours because of their specific management. Surgery represents the first-line treatment option, while medical and radiation therapies can be adopted as neo-adjuvant, adjuvant or primary treatments according to tumour and patient characteristics.
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Acromegalia/diagnóstico , Adenoma/diagnóstico , Doenças Ósseas , Coristoma , Adenoma Hipofisário Secretor de Hormônio do Crescimento/diagnóstico , Hipófise , Neoplasias da Base do Crânio/diagnóstico , Acromegalia/metabolismo , Acromegalia/cirurgia , Adenoma/metabolismo , Adenoma/cirurgia , Fossa Craniana Posterior , Síndrome da Sela Vazia , Teste de Tolerância a Glucose , Adenoma Hipofisário Secretor de Hormônio do Crescimento/metabolismo , Adenoma Hipofisário Secretor de Hormônio do Crescimento/cirurgia , Hormônio do Crescimento Humano/metabolismo , Humanos , Fator de Crescimento Insulin-Like I/metabolismo , Masculino , Pessoa de Meia-Idade , Neoplasias da Base do Crânio/metabolismo , Neoplasias da Base do Crânio/cirurgiaRESUMO
OBJECTIVE: Several nerve block procedures are available for post-thoracotomy pain management. DESIGN: In this randomized trial, the authors aimed to determine whether the analgesic effect of preoperative ultrasound-guided erector spinae plane block (ESPB) might be superior to that of intraoperative intercostal nerve block (ICNB) in pain control in patients undergoing minithoracotomy. SETTING: University hospital. PARTICIPANTS: Sixty consecutive adult patients scheduled to undergo minithoracotomy for lung resection were enrolled. INTERVENTIONS: Patients were allocated randomly in a 1:1 ratio to receive either single-shot ESPB or ICNB. MEASUREMENTS AND MAIN RESULTS: The primary outcome was the intensity of postoperative pain at rest, assessed with the numeric rating scale (NRS). The secondary outcomes were (1) dynamic NRS values (during cough); (2) perioperative analgesic requirements; (3) patient satisfaction, on the basis of a verbal scale (Likert scale); and (4) respiratory muscle strength, considering the maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) variation from baseline. The ESPB group showed lower postoperative static and dynamic NRS values than the ICNB group (p < 0.05). Total remifentanil consumption and requirements for additional analgesics were lower in the ESPB group (p < 0.05). Patient satisfaction was higher in the ESPB group (p < 0.001). A significant overall time effect was found in MIP and MEP variation (p < 0.001); ESPB values were higher at all points, reaching a statistically significant level at the first and sixth hours for MIP, and at the first, 12th, 24th, and 48th hours for MEP (p < 0.05). CONCLUSIONS: ESPB was demonstrated to provide superior analgesia, lower perioperative analgesic requirements, better patient satisfaction, and less respiratory muscle strength impairment than ICNB in patients undergoing minithoracotomy.
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Dor Aguda , Bloqueio Nervoso , Adulto , Humanos , Nervos Intercostais , Dor Pós-Operatória/diagnóstico , Dor Pós-Operatória/prevenção & controle , Toracotomia/efeitos adversos , Ultrassonografia de IntervençãoRESUMO
The aim of this study is to evaluate the efficacy of the pericapsular nerve group block and local infiltration analgesia (LIA) combination as the only anesthesia technique for the total hip arthroplasty (THA). We considered the anesthetic plan, postoperative analgesia, hospital length of stay, functional recovery, bleeding, complications and the adverse events. We reported 10 ASA I-II patients admitted for elective primary THA, receiving LIA during (5) and at the end of surgery (5). For the PENG block we used a single injection of 40 ml levobupivacaine 0.25% and dexamethasone 4 mg. For LIA, a mixture of 0.25% levobupivacaine, ketorolac, epinephrine, and morphine was injected into periarticular tissues. The pain intensity was evaluated with a numeric rating scale. All patients were fully satisfied and improvement in pain relief, symptoms, and functional activity was remarkable. Intraoperative blood losses ranged 100-600 ml. No intraoperative complications or signs of toxicity occurred. The median duration of surgery was 59.5 ± 4.5 min and the hospital stay ranged between 2 and 3 days. PENG block and LIA could be hypothesized as an effective and safety anesthesia technique for the THA surgery, facilitating hip functional recovery and limit intraoperative blood losses and adverse events.
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Analgesia , Artroplastia de Quadril , Anestesia Local , Anestésicos Locais , Humanos , Cetorolaco , Manejo da Dor , Dor Pós-Operatória/tratamento farmacológicoRESUMO
BACKGROUND: Postoperative secondary hyperalgesia arises from central sensitization due to pain pathways facilitation and/or acute opioid exposure. The latter is also known as opioid-induced hyperalgesia (OIH). Remifentanil, a potent µ-opioid agonist, reportedly induces postoperative hyperalgesia and increases postoperative pain scores and opioid consumption. The pathophysiology underlying secondary hyperalgesia involves N-methyl-D-aspartate (NMDA)-mediated pain pathways. In this study, we investigated whether perioperatively infusing low-dose buprenorphine, an opioid with anti-NMDA activity, in patients receiving remifentanil infusion prevents postoperative secondary hyperalgesia. METHODS: Sixty-four patients, undergoing remifentanil infusion during general anaesthesia and major lung surgery, were randomly assigned to receive either buprenorphine i.v. infusion (25 µg h-1 for 24 h) or morphine (equianalgesic dose) perioperatively. The presence and extent of punctuate hyperalgesia were assessed one day postoperatively. Secondary outcome variables included postoperative pain scores, opioid consumption and postoperative neuropathic pain assessed one and three months postoperatively. RESULTS: A distinct area of hyperalgesia or allodynia around the surgical incision was found in more patients in the control group than in the treated group. Mean time from extubation to first morphine rescue dose was twice as long in the buprenorphine-treated group than in the morphine-treated group: 18 vs 9 min (P=0.002). At 30 min postoperatively, patients receiving morphine had a higher hazard ratio for the first analgesic rescue dose than those treated with buprenorphine (P=0.009). At three months, no differences between groups were noted. CONCLUSIONS: Low-dose buprenorphine infusion prevents the development of secondary hyperalgesia around the surgical incision but shows no long-term efficacy at three months follow-up.
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Analgésicos Opioides/uso terapêutico , Buprenorfina/uso terapêutico , Hiperalgesia/tratamento farmacológico , Pulmão/cirurgia , Dor Pós-Operatória/tratamento farmacológico , Remifentanil/efeitos adversos , Idoso , Analgésicos Opioides/administração & dosagem , Buprenorfina/administração & dosagem , Método Duplo-Cego , Feminino , Humanos , Hiperalgesia/induzido quimicamente , Infusões Intravenosas , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Remifentanil/administração & dosagem , Remifentanil/uso terapêuticoRESUMO
OBJECTIVE: Blood volume reserve for venous return and the effects of cardiopulmonary bypass (CPB) on microvascular bed partitioning and blood flow were examined in patients with valvular diseases. DESIGN: Prospective, consecutive, case-control study. SETTING: Single university hospital. PARTICIPANTS: The study comprised 20 adult cardiac surgery patients and 20 healthy volunteers. INTERVENTIONS: Cardiovascular and microvascular variables were collected soon after the induction of anesthesia, after commencement of CPB, 20 minutes after separation from CPB, and in the intensive care unit. MEASUREMENTS AND MAIN RESULTS: The unstressed and stressed volumes (Vu, Vs) and pressures therein (Pit, Ps) were measured in the brachioradial muscle with near-infrared spectroscopy, applying incremental venous occlusions. At the first time point, Vs and Pit showed lower and higher values, respectively, than those of control patients, but Vs increased with Vu during the study, whereas Pit remained unchanged. Fluid balance correlated with Pit (r = 0.83, p<0.001) and hemoglobin (r = 0.78, p = 0.004). A nonlinear regression was found between fluid balance and ΔVu (r = 0.90, p<0.001) [y = 1.85+37.43(-0.01×x)]. The Vu/Pit and Vs/Ps ratios were lower than those of the control patients. Blood flow correlated to Vs/Ps (r = 0.75, p<0.001). The time constant was lower than reference (p = 0.005) and increased 10 times after CPB. CONCLUSIONS: Cardiac surgery patients have a limited blood volume reserve for venous return due to a reduced microvascular bed capacitance. This study demonstrated that during CPB a positive fluid balance induced an extravascular pressure increase and further reduced blood volume reserve.
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Volume Sanguíneo/fisiologia , Ponte Cardiopulmonar/métodos , Doenças das Valvas Cardíacas/cirurgia , Idoso , Idoso de 80 Anos ou mais , Determinação do Volume Sanguíneo/métodos , Temperatura Corporal/fisiologia , Estudos de Casos e Controles , Complacência (Medida de Distensibilidade)/fisiologia , Feminino , Doenças das Valvas Cardíacas/fisiopatologia , Humanos , Masculino , Microcirculação/fisiologia , Pessoa de Meia-Idade , Monitorização Intraoperatória/métodos , Músculo Esquelético/irrigação sanguínea , Estudos Prospectivos , Fluxo Sanguíneo Regional/fisiologia , Espectroscopia de Luz Próxima ao Infravermelho/métodos , Resistência Vascular/fisiologiaRESUMO
OBJECTIVE: Quantitative NIRS measurements for MBV partitioning inside microvessels are of current physiologic and clinical interest. In this study, in healthy subjects, we sought new bedside NIRS variables for noninvasively measuring Vu and Pi changes. METHODS: Fifteen healthy subjects underwent graded venous congestion for MBV measurements with NIRS and the reference technique strain-gauge plethysmography. From ΔMBV we calculated vascular compliance, blood flow, and new NIRS variables including V(u) and P(it) and P(crit). RESULTS: Extrapolating MBV changes to 0 yielded Pit 4.19 ± 0.5 mmHg corresponding to a Vu of 2.53 ± 0.43 mL/100 mL T. The slope for MBV began steeper at values below 18 mmHg (P(crit)). Microvascular compliance measured with NIRS or with strain gauge gave matching results. The change in MBV depended on the oxyhemoglobin increase. No correlation was found between Vu and microvascular compliance or the overall ΔMBV. Cumulative pressure steps showed higher linearity in ΔMBV than that induced by discontinuous steps. CONCLUSIONS: The new NIRS variables we report could be a practical bench-to-bedside tool to assess venous driving pressure for systemic perfusion and measure changes in Vu within the microvascular bed.
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Determinação da Pressão Arterial/métodos , Determinação do Volume Sanguíneo/métodos , Músculo Esquelético/irrigação sanguínea , Sistemas Automatizados de Assistência Junto ao Leito , Espectroscopia de Luz Próxima ao Infravermelho/métodos , Adulto , Pressão Sanguínea , Volume Sanguíneo , Complacência (Medida de Distensibilidade) , Feminino , Hemoglobinometria/métodos , Hemoglobinas/análise , Humanos , Masculino , Microcirculação , Mioglobina/análise , Consumo de Oxigênio , Oxiemoglobinas/análise , Pletismografia/métodos , Valores de Referência , Fluxo Sanguíneo Regional , Resistência Vascular , Adulto JovemRESUMO
Multi Organ Dysfunction Syndrome (MODS) represents a continuum of physiologic derangements and is the major cause of death in the Intensive Care Unit (ICU). Scoring systems for organ failure have become an integral part of critical care practice and play an important role in ICU-based research by tracking disease progression and facilitating patient stratification based on evaluation of illness severity during ICU stay. In this study a Dynamic Bayesian Network (DBN) was applied to model SOFA severity score changes in 79 adult critically ill patients consecutively admitted to the general ICU of the Sant'Andrea University hospital (Rome, Italy) from September 2010 to March 2011, with the aim to identify the most probable sequences of organs failures in the first week after the ICU admission. Approximately 56% of patients were admitted into the ICU with lung failure and about 27% of patients with heart failure. Results suggest that, given the first organ failure at the ICU admission, a sequence of organ failures can be predicted with a certain degree of probability. Sequences involving heart, lung, hematologic system and liver turned out to be the more likely to occur, with slightly different probabilities depending on the day of the week they occur. DBNs could be successfully applied for modeling temporal systems in critical care domain. Capability to predict sequences of likely organ failures makes DBNs a promising prognostic tool, intended to help physicians in undertaking therapeutic decisions in a patient-tailored approach.
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Cuidados Críticos/métodos , Insuficiência de Múltiplos Órgãos/fisiopatologia , Idoso , Algoritmos , Teorema de Bayes , Estado Terminal , Sistemas de Apoio a Decisões Clínicas , Feminino , Mortalidade Hospitalar , Humanos , Unidades de Terapia Intensiva , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Insuficiência de Múltiplos Órgãos/terapia , Probabilidade , Prognóstico , Software , Fatores de TempoRESUMO
Introduction: Primary Progressive Aphasia (PPA) is a neurodegenerative disease characterized by linguistic impairment. The two main clinical subtypes are semantic (svPPA) and non-fluent/agrammatic (nfvPPA) variants. Diagnosing and classifying PPA patients represents a complex challenge that requires the integration of multimodal information, including clinical, biological, and radiological features. Structural neuroimaging can play a crucial role in aiding the differential diagnosis of PPA and constructing diagnostic support systems. Methods: In this study, we conducted a white matter texture analysis on T1-weighted images, including 56 patients with PPA (31 svPPA and 25 nfvPPA), and 53 age- and sex-matched controls. We trained a tree-based algorithm over combined clinical/radiomics measures and used Shapley Additive Explanations (SHAP) model to extract the greater impactful measures in distinguishing svPPA and nfvPPA patients from controls and each other. Results: Radiomics-integrated classification models demonstrated an accuracy of 95% in distinguishing svPPA patients from controls and of 93.7% in distinguishing svPPA from nfvPPA. An accuracy of 93.7% was observed in differentiating nfvPPA patients from controls. Moreover, Shapley values showed the strong involvement of the white matter near left entorhinal cortex in patients classification models. Discussion: Our study provides new evidence for the usefulness of radiomics features in classifying patients with svPPA and nfvPPA, demonstrating the effectiveness of an explainable machine learning approach in extracting the most impactful features for assessing PPA.
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BACKGROUND: Cognitive changes are common in corticobasal syndrome (CBS) and significantly impact quality of life and caregiver burden. However, relatively few studies have investigated the neural substrates of cognitive changes in CBS, and reliable predictors of cognitive impairment are currently lacking. The nucleus basalis of Meynert (NbM), which serves as the primary source of cortical cholinergic innervation, has been functionally associated with cognition. This study aimed to explore whether patients with CBS exhibit reduced NbM volumes compared with healthy control participants and whether NbM degeneration can serve as a predictor of cognitive impairment in patients with CBS. METHODS: In this study, we investigated in vivo volumetric changes of the NbM in 38 patients with CBS and 84 healthy control participants. Next, we assessed whether gray matter degeneration of the NbM evaluated at baseline could predict cognitive impairment during a 12-month follow-up period in patients with CBS. All volumetric analyses were performed using 3T T1-weighted images obtained from the 4-Repeat Tauopathy Neuroimaging Initiative. RESULTS: Patients with CBS displayed significantly lower NbM volumes than control participants (p < .001). Structural damage of the NbM also predicted the development of cognitive impairment in patients with CBS as assessed by longitudinal measurements of the Clinical Dementia Rating Sum of Boxes (p < .001) and Mini-Mental State Examination (p = .035). CONCLUSIONS: Our findings suggest that NbM atrophy may represent a promising noninvasive in vivo marker of cognitive decline in CBS and provide new insights into the neural mechanisms that underlie cognitive impairment in CBS.
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Núcleo Basal de Meynert , Disfunção Cognitiva , Imageamento por Ressonância Magnética , Humanos , Masculino , Feminino , Disfunção Cognitiva/diagnóstico por imagem , Disfunção Cognitiva/fisiopatologia , Disfunção Cognitiva/etiologia , Disfunção Cognitiva/patologia , Idoso , Núcleo Basal de Meynert/patologia , Núcleo Basal de Meynert/diagnóstico por imagem , Pessoa de Meia-Idade , Substância Cinzenta/diagnóstico por imagem , Substância Cinzenta/patologia , Degeneração Corticobasal/diagnóstico por imagem , Degeneração Corticobasal/patologia , Degeneração Corticobasal/complicações , Atrofia/patologiaRESUMO
Purpose To develop a fast and fully automated deep learning (DL)-based method for the MRI planimetric segmentation and measurement of the brainstem and ventricular structures most affected in patients with progressive supranuclear palsy (PSP). Materials and Methods In this retrospective study, T1-weighted MR images in healthy controls (n = 84) were used to train DL models for segmenting the midbrain, pons, middle cerebellar peduncle (MCP), superior cerebellar peduncle (SCP), third ventricle, and frontal horns (FHs). Internal, external, and clinical test datasets (n = 305) were used to assess segmentation model reliability. DL masks from test datasets were used to automatically extract midbrain and pons areas and the width of MCP, SCP, third ventricle, and FHs. Automated measurements were compared with those manually performed by an expert radiologist. Finally, these measures were combined to calculate the midbrain to pons area ratio, MR parkinsonism index (MRPI), and MRPI 2.0, which were used to differentiate patients with PSP (n = 71) from those with Parkinson disease (PD) (n = 129). Results Dice coefficients above 0.85 were found for all brain regions when comparing manual and DL-based segmentations. A strong correlation was observed between automated and manual measurements (Spearman ρ > 0.80, P < .001). DL-based measurements showed excellent performance in differentiating patients with PSP from those with PD, with an area under the receiver operating characteristic curve above 0.92. Conclusion The automated approach successfully segmented and measured the brainstem and ventricular structures. DL-based models may represent a useful approach to support the diagnosis of PSP and potentially other conditions associated with brainstem and ventricular alterations. Keywords: MR Imaging, Brain/Brain Stem, Segmentation, Quantification, Diagnosis, Convolutional Neural Network Supplemental material is available for this article. © RSNA, 2024 See also the commentary by Mohajer in this issue.
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Tronco Encefálico , Aprendizado Profundo , Imageamento por Ressonância Magnética , Paralisia Supranuclear Progressiva , Humanos , Paralisia Supranuclear Progressiva/diagnóstico por imagem , Paralisia Supranuclear Progressiva/patologia , Imageamento por Ressonância Magnética/métodos , Feminino , Estudos Retrospectivos , Tronco Encefálico/diagnóstico por imagem , Tronco Encefálico/patologia , Masculino , Idoso , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Ventrículos Cerebrais/diagnóstico por imagem , Ventrículos Cerebrais/patologia , Interpretação de Imagem Assistida por Computador/métodosRESUMO
Background: Behavioral variant frontotemporal dementia (bvFTD) typically involves subtle changes in personality that can delay a timely diagnosis. Objective: Here, we report the case of a patient diagnosed of GRN-positive bvFTD at the age of 52 presenting with a 7-year history of narcissistic personality disorder, accordingly to DSM-5 criteria. Methods: The patient was referred to neurological and neuropsychological examination. She underwent 3 Tesla magnetic resonance imaging (MRI) and genetic studies. Results: The neuropsychological examination revealed profound deficits in all cognitive domains and 3T brain MRI showed marked fronto-temporal atrophy. A mutation in the GRN gene further confirmed the diagnosis. Conclusions: The present case documents an unusual onset of bvFTD and highlights the problematic nature of the differential diagnosis between prodromal psychiatric features of the disease and primary psychiatric disorders. Early recognition and diagnosis of bvFTD can lead to appropriate management and support for patients and their families. This case highlights the importance of considering neurodegenerative diseases, such as bvFTD, in the differential diagnosis of psychiatric disorders, especially when exacerbations of behavioral traits manifest in adults.
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Demência Frontotemporal , Feminino , Humanos , Demência Frontotemporal/diagnóstico por imagem , Demência Frontotemporal/genética , Transtorno da Personalidade Narcisística , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Imageamento por Ressonância Magnética , Testes Neuropsicológicos , ProgranulinasRESUMO
The presence of parkinsonism features in primary progressive aphasia (PPA) is a subject of ongoing research. These features are usually more pronounced in the advanced stages of the disease, particularly in the non-fluent/agrammatic subtype, and are exceptionally rare in the logopenic variant (lvPPA). Here we report a case of a 63-year-old man presenting as language impairment, predominantly naming and word-finding difficulties, emerged alongside a left-sided internal tremor. Neurological examination revealed bilateral, left-side predominant rigidity, bradykinesia, and resting tremor. Notably, anosmia and constipation were present. Language assessments showed preserved single-word comprehension, object knowledge, and a minimal apraxia of speech, as well as sentence repetition issues. Neuroimaging and biomarker analysis supported a diagnosis of primary progressive logopenic aphasia with amyloid pathology co-existing with prominent and early parkinsonism. This case underlines the intricate relationship between language disorders, parkinsonism, and amyloid pathology in lvPPA.
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BACKGROUND: The clinical diagnosis of behavioral variant frontotemporal dementia (bvFTD) in patients with a history of primary psychiatric disorder (PPD) is challenging. PPD shows the typical cognitive impairments observed in patients with bvFTD. Therefore, the correct identification of bvFTD onset in patients with a lifetime history of PPD is pivotal for an optimal management. METHODS: Twenty-nine patients with PPD were included in this study. After clinical and neuropsychological evaluations, 16 patients with PPD were clinically classified as bvFTD (PPD-bvFTD+), while in 13 cases clinical symptoms were associated with the typical course of the psychiatric disorder itself (PPD-bvFTD-). Voxel- and surface-based investigations were used to characterize gray matter changes. Volumetric and cortical thickness measures were used to predict the clinical diagnosis at a single-subject level using a support vector machine (SVM) classification framework. Finally, we compared classification performances of magnetic resonance imaging (MRI) data with automatic visual rating scale of frontal and temporal atrophy. RESULTS: PPD-bvFTD+ showed a gray matter decrease in thalamus, hippocampus, temporal pole, lingual, occipital, and superior frontal gyri compared to PPD-bvFTD- (p < .05, family-wise error-corrected). SVM classifier showed a discrimination accuracy of 86.2% in differentiating PPD patients with bvFTD from those without bvFTD. CONCLUSIONS: Our study highlights the utility of machine learning applied to structural MRI data to support the clinician in the diagnosis of bvFTD in patients with a history of PPD. Gray matter atrophy in temporal, frontal, and occipital brain regions may represent a useful hallmark for a correct identification of dementia in PPD at a single-subject level.
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Demência Frontotemporal , Humanos , Demência Frontotemporal/diagnóstico , Imageamento por Ressonância Magnética/métodos , Encéfalo/patologia , Substância Cinzenta/diagnóstico por imagem , Substância Cinzenta/patologia , Testes Neuropsicológicos , Atrofia/patologiaRESUMO
Loss of empathy is an early and central symptom of frontotemporal lobar degeneration spectrum diseases. We aimed to investigate the topographical distribution of morphometric brain changes associated with empathy in Progressive Supranuclear Palsy (PSP) and Corticobasal Syndrome (CBS) patients. Twenty-seven participants with CBS and 31 with PSP were evaluated using Interpersonal Reactivity Index scales in correlation with gray matter atrophy using a voxel-based morphometry approach. Lower levels of empathy were associated with an increased atrophy in fronto-temporal cortical structures. At subcortical level, empathy scores were positively correlated with gray matter volume in the amygdala, hippocampus and the cerebellum. These findings allow to extend the traditional cortico-centric view of cognitive empathy to the cerebellar regions in patients with neurodegenerative disorders and suggest that the cerebellum may play a more prominent role in social cognition than previously appreciated.
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BACKGROUND AND OBJECTIVES: Depressive symptoms are frequently reported in patients affected by frontotemporal dementia (FTD). At structural MRI, cortical features of depressed FTD patients have been poorly described. Our objective was to investigate correlations between cortical measures and depression severity in FTD patients. METHODS: Data were obtained from the Frontotemporal Lobar Degeneration Neuroimaging Initiative (FTLDNI) database. We included 98 controls and 92 FTD patients, n = 38 behavioral variant FTD (bvFTD), n = 26 non-fluent variant Primary Progressive Aphasia (nfvPPA), and n = 28 semantic variant Primary Progressive Aphasia (svPPA). Patients underwent clinical and cognitive evaluations, as well as a 3D T1-weighted MRI on a 3 Tesla scanner (Siemens, Trio Tim system). Depression was evaluated by means of Geriatric Depression Scale (GDS). Surface-based analysis was performed on T1-weighted images to evaluate cortical thickness, a measure of gray matter integrity, and local gyrification index (lGI), a quantitative metric of cortical folding. RESULTS: Patients affected by svPPA were more depressed than controls at NPI and depression severity at GDS was higher in svPPA and bvFTD. Severity of depression correlated with a decrease in lGI in left precentral and superior frontal gyrus, supramarginal and postcentral gyrus and right precentral, supramarginal, superior parietal and superior frontal gyri. Furthermore, depression severity correlated positively with cortical thickness in the left medial orbitofrontal cortex. DISCUSSION: We found that lGI was associated with depressive symptoms over brain regions involved in the pathophysiology of major depressive disorder. This finding provides novel insights into the mechanisms underlying psychiatric symptoms in FTD.
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Afasia Primária Progressiva , Transtorno Depressivo Maior , Demência Frontotemporal , Doença de Pick , Humanos , Idoso , Demência Frontotemporal/diagnóstico por imagem , Demência Frontotemporal/psicologia , Depressão/diagnóstico por imagem , Encéfalo , Afasia Primária Progressiva/diagnóstico por imagemRESUMO
Introduction: Primary Progressive Aphasia (PPA) is a neurological disease characterized by linguistic deficits. Semantic (svPPA) and non-fluent/agrammatic (nfvPPA) variants are the two main clinical subtypes. We applied a novel analytical framework, based on radiomic analysis, to investigate White Matter (WM) asymmetry and to examine whether asymmetry is associated with verbal fluency performance. Methods: Analyses were performed on T1-weighted images including 56 patients with PPA (31 svPPA and 25 nfvPPA) and 53 age- and sex-matched controls. Asymmetry Index (AI) was computed for 86 radiomics features in 34 white matter regions. The relationships between AI, verbal fluency performance (semantic and phonemic) and Boston Naming Test score (BNT) were explored through Spearman correlation analysis. Results: Relative to controls, WM asymmetry in svPPA patients involved regions adjacent to middle temporal cortex as part of the inferior longitudinal (ILF), fronto-occipital (IFOF) and superior longitudinal fasciculi. Conversely, nfvPPA patients showed an asymmetry of WM in lateral occipital regions (ILF/IFOF). A higher lateralization involving IFOF, cingulum and forceps minor was found in nfvPPA compared to svPPA patients. In nfvPPA patients, semantic fluency was positively correlated to asymmetry in ILF/IFOF tracts. Performances at BNT were associated with AI values of the middle temporal (ILF/SLF) and parahippocampal (ILF/IFOF) gyri in svPPA patients. Discussion: Radiomics features depicted distinct pathways of asymmetry in svPPA and nfvPPA involving damage of principal fiber tracts associated with speech and language. Assessing asymmetry of radiomics in PPA allows achieving a deeper insight into the neuroanatomical damage and may represent a candidate severity marker for language impairments in PPA patients.
RESUMO
INTRODUCTION: We report the experiences of 25 Italian centers, analyzing intra- and periprocedural complications of endovascular treatment of intracranial aneurysms using Silk (Balt Extrusion, Montmorency, France) and pipeline embolization devices (EV3 Inc, Irvine California). METHODS: Two hundred seventy-three patients with 295 cerebral aneurysms, enrolled in 25 centers in Italy and treated with the new flow-diverter devices, were evaluated; 142 patients were treated with Silk and 130 with pipeline (in one case, both devices were used). In 14 (5.2 %) cases devices were used with coils. Aneurysm size was >15 mm in 46.9 %, 5-15 mm in 42.2 %, and <5 mm in 10.8 %. Aneurysm locations were supraclinoid internal carotid artery (ICA) in 163 cases (55.2 %), cavernous ICA in 76 (25.7 %), middle cerebral artery in 11 (3.7 %), PCoA in 6 (2 %), and ACoA in 2 (0.7 %); the vertebrobasilar system accounted for 32 cases (10.8 %) and PCA in 5 (1.7 %). RESULTS: Technical adverse events occurred with 59 patients (21.6 %); 5 patients died after ischemic events, 10 to hemorrhagic complications, and 1 from external ventricular drain positioning. At 1 month, morbidity and mortality rates were 3.7 % and 5.9 %, respectively CONCLUSION: Our retrospective study confirms that morbidity and mortality rates in treatment with FDD of unruptured wide-neck or untreatable cerebral aneurysms do not differ from those reported in the largest series.