Person: Benito León, Julián
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First Name
Julián
Last Name
Benito León
Affiliation
Universidad Complutense de Madrid
Faculty / Institute
Medicina
Department
Medicina
Area
Medicina
Identifiers
9 results
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Item Acute onset movement disorders in diabetes mellitus: A clinical series of 59 patients(European Journal of Neurology, 2022) Dubey, Souvik; Chatterjee, Subhankar; Ghosh, Ritwik; Louis, Elan D.; Hazra, Avijit; Sengupta, Samya; Das, Shambaditya; Banerjee, Abhirup; Pandit, Alak; Ray, Biman Kanti; Benito León, JuliánBackground and purpose: No previous study has assessed the frequency and clinical– radiological characteristics of patients with diabetes mellitus (DM) and acute onset non-choreic and nonballistic movements. We conducted a prospective study to investigate the spectrum of acute onset movement disorders in DM.Methods: We recruited all the patients with acute onset movement disorders and hyper-glycemia who attended the wards of three hospitals in West Bengal, India from August 2014 to July 2021.Results: Among the 59 patients (mean age = 55.4± 14.3 years, 52.5% men) who were included, 41 (69.5%) had choreic or ballistic movements, and 18 (30.5%) had nonchoreic and nonballistic movements. Ballism was the most common movement disorder (n= 18, 30.5%), followed by pure chorea (n= 15, 25.4%), choreoathetosis (n= 8, 13.6%), tremor (n= 5, 8.5%), hemifacial spasm (n= 3, 5.1%), parkinsonism (n= 3, 5.1%), myoclonus (n= 3, 5.1%), dystonia (n= 2, 3.4%), and restless leg syndrome (n= 2, 3.4%). The mean duration of DM was 9.8 ± 11.4 years (89.8% of the patients had type 2 DM). Nonketotic hypergly-cemia was frequently (76.3%) detected. The majority (55.9%) had no magnetic resonance imaging (MRI) changes; the remaining showed striatal hyperintensity. Eight patients with MRI changes exhibited discordance with sidedness of movements. Most of the patients (76.3%) recovered completely.Conclusions: This is the largest clinical series depicting the clinical–radiological spectrum of acute onset movement disorders in DM. Of note was that almost one third of patients had nonchoreic and nonballistic movements. Our findings highlight the importance of a capillary blood glucose measurement in patients with acute or subacute onset movement disorders, irrespective of their past glycemic status.Item Abnormal functional connectivity in radiologically isolated syndrome: A resting-state fMRI study(Multiple Sclerosis Journal, 2023) Benito León, Julián; Pino, Ana Belén del; Aladro, Yolanda; Cuevas, Constanza; Domingo-Santos, Ángela; Galán Sánchez-Seco, Victoria; Labiano-Fontcuberta, Andrés; Gómez-López, Ana; Salgado-Cámara, Paula; Costa-Frossard, Lucienne; Monreal, Enrique; Sainz de la Maza, Susana; Matías-Guiu, Jordi A; Matías-Guiu Guía, Jorge; Delgado Álvarez, Alfonso; Montero-Escribano, Paloma; Martínez-Ginés, María Luisa; Higueras Hernández, Yolanda; Ayuso-Peralta, Lucía; Malpica, Norberto; Melero Carrasco, HelenaBackground: Radiologically isolated syndrome (RIS) patients might have psychiatric and cognitive deficits, which suggests an involvement of major resting-state functional networks. Notwithstanding, very little is known about the neural networks involved in RIS. Objective: To examine functional connectivity differences between RIS and healthy controls using resting-state functional magnetic resonance imaging (fMRI). Methods: Resting-state fMRI data in 25 RIS patients and 28 healthy controls were analyzed using an independent component analysis; in addition, seed-based correlation analysis was used to obtain more information about specific differences in the functional connectivity of resting-state networks. Participants also underwent neuropsychological testing. Results: RIS patients did not differ from the healthy controls regarding age, sex, and years of education. However, in memory (verbal and visuospatial) and executive functions, RIS patients’ cognitive performance was significantly worse than the healthy controls. In addition, fluid intelligence was also affected. Twelve out of 25 (48%) RIS patients failed at least one cognitive test, and six (24.0%) had cognitive impairment. Compared to healthy controls, RIS patients showed higher functional connectivity between the default mode network and the right middle and superior frontal gyri and between the central executive network and the right thalamus ( pFDR < 0.05; corrected). In addition, the seed-based correlation analysis revealed that RIS patients presented higher functional connectivity between the posterior cingulate cortex, an important hub in neural networks, and the right precuneus. Conclusion: RIS patients had abnormal brain connectivity in major resting-state neural networks and worse performance in neurocognitive tests. This entity should be considered not an “incidental finding” but an exclusively non-motor (neurocognitive) variant of multiple sclerosis.Item Abnormal functional connectivity in radiologically isolated syndrome: A resting-state fMRI study(Multiple Sclerosis Journal, 2023) del Pino, Ana Belén; Aladro, Yolanda; Cuevas, Constanza; Domingo-Santos, Ángela; Galán Sánchez-Seco, Victoria; Labiano-Fontcuberta, Andrés; Gómez-López, Ana; Salgado-Cámara, Paula; Costa-Frossard. Lucienne; Monreal. Enrique; Sainz de la Maza, Susana; Montero-Escribano, Paloma; Martínez-Ginés, María Luisa; Higueras, Yolanda; Ayuso-Peralta, Lucía; Malpica, Norberto; Melero Carrasco, Helena; Benito León, Julián; Higueras Hernández, Yolanda; Matías-Guiu Guía, JorgeRadiologically isolated syndrome (RIS) patients might have psychiatric and cognitive deficits, which suggests an involvement of major resting-state functional networks. Notwithstanding, very little is known about the neural networks involved in RIS. Objective: To examine functional connectivity differences between RIS and healthy controls using resting-state functional magnetic resonance imaging (fMRI). Methods: Resting-state fMRI data in 25 RIS patients and 28 healthy controls were analyzed using an independent component analysis; in addition, seed-based correlation analysis was used to obtain more information about specific differences in the functional connectivity of resting-state networks. Participants also underwent neuropsychological testing. Results: RIS patients did not differ from the healthy controls regarding age, sex, and years of education. However, in memory (verbal and visuospatial) and executive functions, RIS patients’ cognitive performance was significantly worse than the healthy controls. In addition, fluid intelligence was also affected. Twelve out of 25 (48%) RIS patients failed at least one cognitive test, and six (24.0%) had cognitive impairment. Compared to healthy controls, RIS patients showed higher functional connectivity between the default mode network and the right middle and superior frontal gyri and between the central executive network and the right thalamus (pFDR < 0.05; corrected). In addition, the seed-based correlation analysis revealed that RIS patients presented higher functional connectivity between the posterior cingulate cortex, an important hub in neural networks, and the right precuneus. Conclusion: RIS patients had abnormal brain connectivity in major resting-state neural networks and worse performance in neurocognitive tests. This entity should be considered not an “incidental finding” but an exclusively non-motor (neurocognitive) variant of multiple sclerosis.Item Graph theory analysis of resting‐state functional magnetic resonance imaging in essential tremor(Human Brain Mapping, 2019) Benito León, Julián; Sanz‐Morales, Emilio; Melero Carrasco, Helena; Louis, Elan D.; Romero, Juan P.; Rocon, Eduardo; Malpica, NorbertoEssential tremor (ET) is a neurological disease with both motor and nonmotor manifestations; however, little is known about its underlying brain basis. Furthermore, the overall organization of the brain network in ET remains largely unexplored. We investigated the topological properties of brain functional network, derived from resting‐state functional magnetic resonance imaging (MRI) data, in 23 ET patients versus 23 healthy controls. Graph theory analysis was used to assess the functional network organization. At the global level, the functional network of ET patients was characterized by lower small‐worldness values than healthy controls—less clustered functionality of the brain. At the regional level, compared with the healthy controls, ET patients showed significantly higher values of global efficiency, cost and degree, and a shorter average path length in the left inferior frontal gyrus (pars opercularis), right inferior temporal gyrus (posterior division and temporo‐occipital part), right inferior lateral occipital cortex, left paracingulate, bilateral precuneus bilaterally, left lingual gyrus, right hippocampus, left amygdala, nucleus accumbens bilaterally, and left middle temporal gyrus (posterior part). In addition, ET patients showed significant higher local efficiency and clustering coefficient values in frontal medial cortex bilaterally, subcallosal cortex, posterior cingulate cortex, parahippocampal gyri bilaterally (posterior division), right lingual gyrus, right cerebellar flocculus, right postcentral gyrus, right inferior semilunar lobule of cerebellum and culmen of vermis. Finally, the right intracalcarine cortex and the left orbitofrontal cortex showed a shorter average path length in ET patients, while the left frontal operculum and the right planum polare showed a higher betweenness centrality in ET patients. In conclusion, the efficiency of the overall brain functional network in ET is disrupted. Further, our results support the concept that ET is a disorder that disrupts widespread brain regions, including those outside of the brain regions responsible for tremor.Item Headache as the presenting manifestation of Gorlin‐Goltz syndrome with diastematomyelia: A case report(Neurology and Clinical Neuroscience, 2023) Ghosh, Ritwik; León Ruiz, Moisés; Purkait, Siktha; Roy, Dipayan; Ghosh, Tapas; Benito León, JuliánGorlin-Goltz syndrome (GGS) is an autosomal dominant multisystemic disease with high penetrance. Headache heralding GGS has been previously reported but without discussing potential sources. We report a patient with headache and a novel association (diastematomyelia), which helped with the diagnosis. A 46-year-old woman presented with persistent holocranial headache. On examination, countless hyperpigmented basal cell nevi over the face, pits over the palmar/plantar surface, and palmar and plantar keratosis were observed. A magnetic resonance imaging (MRI) of the spinal cord revealed diastematomyelia. Diagnosis of GGS was finally made. Headache and diastematomyelia should be included in the clinical picture of GGS.Item P300 Wave Alterations and Cognitive Impairment in Cerebellum Lesions(The Cerebellum, 2023) Nanda, Sourav; Lapeña Motilva, José; Kumar Misra, Amar; Guha, Gautam; Ghosh, Sinjan; Manna, Akash; Roy, Soumit; Benito León, JuliánAbstractPatients with cognitive deficits have a prolonged latency and reduced amplitude of the P300 wave. However, no study has correlated P300 wave alterations with the cognitive performance of patients with cerebellar lesions. We aimed to determine if the cognitive status of these patients was associated with P300 wave alterations. We recruited 30 patients with cerebellar lesions from the wards of the N.R.S. Medical College, Kolkata, in West Bengal (India). The Kolkata Cognitive Screening Battery tasks and the Frontal Assessment Battery (FAB) were used to assess the cognitive status and the International Cooperative Ataxia Rating Scale (ICARS) for cerebellar signs. We compared the results with the normative data of the Indian population. Patients had P300 wave alterations with a significant increase in latency and a non-significant trend in amplitude. In a multivariate model, P300 wave latency was positively associated with the ICARS kinetic subscale (p = 0.005) and age (p = 0.009), regardless of sex and years of education. In the model that included cognitive variables, P300 wave latency was negatively associated with performance in phonemic fluency (p = 0.035) and construction (p = 0.009). Furthermore, P300 wave amplitude was positively associated with the FAB total score (p < 0.001). In closing, patients with cerebellar lesions had an increase in latency and a decrease in the amplitude of the P300 wave. These P300 wave alterations were also associated with worse cognitive performance and some of the subscales of the ICARS, reinforcing that the cerebellum has motor, cognitive, and affective functions.Item Candidate Genes for Eyelid Myoclonia with Absences, Review of the Literature(International Journal of Molecular Sciences, 2021) Mayo, Sonia; Gómez Manjón, Irene; Fernández Martínez, Fco. Javier; Camacho Salas, Ana; Martínez, Francisco; Benito León, JuliánEyelid myoclonia with absences (EMA), also known as Jeavons syndrome (JS) is a childhood onset epileptic syndrome with manifestations involving a clinical triad of absence seizures with eyelid myoclonia (EM), photosensitivity (PS), and seizures or electroencephalogram (EEG) paroxysms induced by eye closure. Although a genetic contribution to this syndrome is likely and some genetic alterations have been defined in several cases, the genes responsible for have not been identified. In this review, patients diagnosed with EMA (or EMA-like phenotype) with a genetic diagnosis are summarized. Based on this, four genes could be associated to this syndrome (SYNGAP1, KIA02022/NEXMIF, RORB, and CHD2). Moreover, although there is not enough evidence yet to consider them as candidate for EMA, three more genes present also different alterations in some patients with clinical diagnosis of the disease (SLC2A1, NAA10, and KCNB1). Therefore, a possible relationship of these genes with the disease is discussed in this review.Item Norovirus-associated neurological manifestations: summarizing the evidence(Journal of NeuroVirology, 2023) Deb, Shramana; Mondal, Ritwick; Lahiri, Durjoy; Shome, Gourav; Guha Roy, Aakash; Sarkar, Vramanti; Sarkar, Shramana; Benito León, JuliánNorovirus, a positive-stranded RNA virus, is one of the leading causes of acute gastroenteritis among all age groups worldwide. The neurological manifestations of norovirus are underrecognized, but several wide-spectrum neurological manifestations have been reported among infected individuals in the last few years. Our objective was to summarize the features of norovirus-associated neurological disorders based on the available literature. We used the existing PRISMA consensus statement. Data were collected from PubMed, EMBASE, Web of Science, and Scopus databases up to Jan 30, 2023, using pre‐specified searching strategies. Twenty-one articles were selected for the qualitative synthesis. Among these, seven hundred and seventy-four patients with norovirus-associated neurological manifestations were reported. Most cases were seizure episodes, infection-induced encephalopathy, and immune-driven disorders. However, only a few studies have addressed the pathogenesis of norovirus-related neurological complications. The pathogenesis of these manifestations may be mediated by either neurotropism or aberrant immune-mediated injury, or both, depending on the affected system. Our review could help clinicians to recognize these neurological manifestations better and earlier while deepening the understanding of the pathogenesis of this viral infection.- Project number: 204
Item Curso de formación de profesorado en estructura departamental(2019) Collado Yurrita, Luis Rodolfo; Ciudad Cabañas, María José; Hernández Gallego, Jesús; Gomez-Lus Centelles, Maria Luisa; Tejedor Jorge, Alberto; Madrigal Martinez-Pereda, Cristina María; Alou Cervera, Luis; Sevillano Fernández, David; Benito León, Julián; Cuenca Caraballo, Maigualida; García Chacón, Marta; Oconnor de la Oliva, Anna; Marín Cuenda, María José; Roiz Sastrón, María del Carmen; San Mauro Martín, Ismael; Marín Viecho, Julia; Muñoz Lucas, María Angeles; Nieto Barbero, Maria Asunción; Callol Sánchez, Luis Miguel; Sanz Esporrín, Javier; García Torrent, María Jesús; Cuadrado Cenzual, María ÁngelesEl Proyecto propuesto es un Programa Formacion del Profesorado en Dirección y Gestión de Departamentos Universitarios dirigido al PDI, con apoyo del PAS, con la finalidad de obtener un cierto grado de profesionalización en Dirección Departamental.