Julián Benito-León

• M.D., Ph.D.
• University Hospital "12 de Octubre"

Toxic Oil Syndrome (TOS) was a major food-borne epidemic in Spain in May 1981, caused by the ingestion of rapeseed oil adulterated with aniline. While somatic sequelae have been widely documented, little is known about the long-term cognitive consequences more than four decades after exposure. This case-control study compared 50 individuals with cl...

Background Orientia tsutsugamushi, the causative agent of scrub typhus, is increasingly recognised for its neurological complications. Among these, movement and gait disorders are poorly understood. We systematically examined their clinical spectrum and explored underlying mechanisms through in‐silico protein–protein interaction modelling. Methods...

A hallmark of type 2 diabetes mellitus is the presence of abundant amyloid deposits composed of islet amyloid polypeptide (also known as amylin) in the pancreatic islets of Langerhans. Given the conspicuous prevalence of these deposits in diabetic patients, it was long assumed that human islet amyloid polypeptide fibrillization plays a crucial path...

A unique case study on PPAOS with advanced neuroradiological investigation, employing DTI and fMRI.

Background: We observed a COVID-19 survivor with a ventriculoperitoneal shunt who developed increased intracranial pressure during hemodialysis. We hypothesized that post-SARS-CoV-2 infection, patients may have altered cerebral perfusion pressure regulation in response to intracranial pressure changes. Methods: From April to July 2021, we recruited...

Introduction The complex process of amyloid-β (Aβ) transportation across the blood–brain and blood–cerebrospinal fluid barriers is crucial for preventing Aβ accumulation, which linked to dementia and neurodegeneration. This review explores therapeutic plasma exchange with albumin replacement in Alzheimer's disease, based on the dynamics of amyloid-...

Background Diabetic striatopathy is a rare complication of diabetes mellitus characterised by movement disorders secondary to hyperglycemia. While most commonly associated with chorea and ballism, it may also present with other hyperkinetic manifestations, including, albeit rarely, isolated dystonia and myoclonus. We report a case of a patient with...

Background Monkeypox virus (MPXV) has emerged as a significant global health concern with outbreaks worldwide. While MPXV is primarily known for its dermatological and systemic manifestations, it can also cause central nervous system (CNS) complications. This systematic review describes the demographic, clinical, diagnostic, and therapeutic charact...

Background: The association between essential tremor (ET) and mortality risk remains uncertain. This study investigated the impact of episodic memory performance, measured through a word recall task, on mortality risk in ET within the Neurological Disorders in Central Spain (NEDICES) cohort, a population-based study of older adults. Methods: Partic...

Background: Parkinson’s disease (PD) is one of the most prevalent neurodegenerative disorders among older adults, yet its long-term impact on mortality within population-based cohorts remains insufficiently characterized. This study leverages data from the Neurological Disorders in Central Spain (NEDICES) cohort to provide a comprehensive 23-year m...

Background: Diabetic striatopathy (DS) typically presents with hemichoreoballism and contralateral striatal lesions on neuroimaging. However, cases of unilateral movement disorders with predominant ipsilateral striatal lesions are rare. Case presentation: We present a case of DS in a 62-year-old woman from rural India with poorly controlled diabet...

Hepatitis E virus (HEV) triggers a lot of extrahepatic manifestations, especially neurological disturbances. The most common are neuralgic amyotrophy (ParsonageTurner syndrome), Guillain-Barré syndrome (GBS), myelitis, and encephalitis. Their exact pathophysiology remains obscure. The most common subtypes of GBS are acute inflammatory demyelinatin...

Background: Cardiac light-chain amyloidosis represents a critical component of this multi-systemic disease, significantly impacting prognosis. The extent of cardiac free light-chain deposition is the primary determinant of survival. Case Presentation We report the case of a 67-year-old male with a 10-year history of diabetes mellitus and arterial...

The relationship between essential tremor (ET) and mortality risk remains uncertain. This study investigated the impact of episodic memory performance, measured through a word recall task, on mortality risk in ET within the NEDICES cohort, a population-based study of older adults. Participants were followed until death or December 31, 2017, and div...

Many clinical, neuroimaging, neuropathological, epidemiological, and genetic data suggest a relationship between essential tremor (ET) and Parkinson’s disease (PD). Several hypothesis-based gene association studies attempted to find a genetic association between these diseases. Recent case–control association studies in Chinese and Spanish populati...

Parkinson's disease (PD) is a prevalent neurodegenerative disorder in older adults, yet its long-term mortality impact remains inadequately defined. This study builds on prior findings from the Neurological Disorders in Central Spain (NEDICES) cohort, extending mortality analysis to a 23-year follow-up within a Spanish population-based sample. This...

Crossed Wernicke's Aphasia (CWA) following a stroke is a rare clinical phenomenon, especially in the context of seizures. This case report documents a unique presentation of CWA accompanied by focal clonic cluster seizures involving the left arm and face with secondary generalization in a Bengali-speaking patient following a right middle-cerebral-a...

Objective Obesity-associated insulin resistance (IR) is responsible for considerable morbidity and mortality globally. Despite vast genomic data, many areas, from pathogenesis to management, still have significant knowledge gaps. We aimed to characterize visceral adipose tissue (VAT) in obesity and IR through a multi-omics approach. Methods We proc...

Alzheimer’s disease (AD), the most prevalent form of dementia, is expected to rise dramatically in incidence due to the global population aging. Traditional diagnostic approaches, such as cerebrospinal fluid analysis and positron emission tomography, are expensive and invasive, limiting their routine clinical use. Recent advances in blood-based bio...

Neurometabolic homeostasis in the brain relies on the dynamic transport of glucose and other substrates across brain barriers, primarily the blood–brain barrier and the blood-cerebrospinal fluid barrier. In type 2 diabetes mellitus (T2DM), chronic hyperglycemia leads to disruptions in this balance, causing neurovascular and cognitive dysfunction. T...

Alzheimer's disease (AD) is the most prevalent form of dementia. With the global aging population, the incidence of AD and related dementias is projected to rise dramatically, posing significant public health challenges. Traditionally, the diagnosis of AD has relied on the detection of amyloid-beta (Aβ) plaques and neurofibrillary tangles of hyperp...

Background The Monkeypox virus, a zoonotic pathogen of the Orthopoxvirus genus, has shown a marked global spread, resulting in cases of neuroinflammatory disorders. This systematic review aims to summarize the central nervous system manifestations linked to monkeypox virus infection. Methods We conducted a systematic review according to PRISMA gui...

Introduction Rapid correction of hyponatremia can result in osmotic demyelination syndrome (ODS). Sheehan’s syndrome, a rare pituitary disorder caused by severe postpartum hemorrhage, is a potential cause of chronic hyponatremia. This case report describes a rare progression of extrapontine myelinolysis to central pontine myelinolysis, ultimately l...

Background and objectives: The 2023 criteria for myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) perform well in adults but have not been assessed in children. Methods: This prospective observational nationwide study includes children and adults with demyelinating syndromes or encephalitis, whose serum or CSF was found MO...

The protein-coding regions of approximately 30Mb of the human exome, targeting around 99% of the regions in the Consensus Coding Sequence (CCDS) and RefSeq databases, as well as the complete mitochondrial genome, were sequenced using Illumina next-generation sequencing (NGS) systems. Sequencing was conducted at an average depth of 80-100X, with mor...

Introduction: Hyponatremia, when rapidly corrected, can lead to osmotic demyelination syndrome (ODS). Sheehan's syndrome, a rare pituitary disorder caused by severe postpartum hemorrhage, can result in chronic hyponatremia. This case report presents a unique instance of extrapontine myelinolysis progressing to central pontine myelinolysis, culminat...

Background: Scrub typhus, an acute febrile infectious disease highly prevalent in the Asia Pacific region (tsutsugamushi triangle), is a mite-born rickettsial zoonosis caused by Orientia tsutsugamushi. This intracellular Gram-negative organism targets endothelial cells, causing vasculitis and leading to multisystem involvement. Meningoencephalitis...

Introduction: The complex process of amyloid-β(Aβ) transportation across the blood—brain and blood—cerebrospinal fluid barriers is crucial for preventing A‌‌β accumulation, which linked to dementia and neurodegeneration. This review explores therapeutic plasma exchange with albumin replacement in Alzheimer’s disease, based on the dynamics of amyloi...

Background: Brugada syndrome is generally considered a cardiac channelopathy disorder characterized by syncope or sudden cardiac death. The sodium voltage-gated channel alpha subunit 5 (SCN5A) gene is the most commonly mutated gene associated with Brugada syndrome. Recent discoveries of new variants of this gene, along with current guidance of fami...

The relationship between spirituality and religiosity and their impact on mental health is intricate and underexplored. This exploratory review aims to elucidate the distinct effects of these constructs, highlighting their contributions to psychological well-being and clinical practices. By dissecting the impacts of spirituality and religiosity on...

Alzheimer’s disease (AD), the most common cause of dementia, is a complex and multifactorial condition without cure at present. The latest treatments, based on anti-amyloid monoclonal antibodies, have only a modest effect in reducing the progression of cognitive decline in AD, whereas the possibility of preventing AD has become a crucial area of re...

Background and Objectives: Despite its extensive utilization, research on Chat Generative Pre-trained Transformer (ChatGPT)'s potential negative impact on specific cognitive processes is scarce. This article explores the widespread use of ChatGPT in educational, corporate, and various other sectors, focusing on its interaction with distinct co...

Abstract: Introduction: Various types of lymphoma can involve the lumbosacral plexus, mainly diffuse large B-cell lymphoma, in which cancer-related persistent fatigue can occur and also fatigability (a new concept that assess fatigue concerning specific activities). We report a rare case of acute right L2-S1 lumbosacral plexopathy secondary to dif...

ABSTRACT: Introduction: The scarcity of epidemiological data on acute febrile illnesses from South Asia impairs evidence-based clinical decision-making. Our study aimed to explore the etiological spectrum of short-duration fever in patients admitted to a tertiary care hospital in West Bengal, India. Methods: We conducted a cross- sectional study f...

Introduction Seizure disorders have often been found to be associated with corpus callosum injuries, but in most cases, they remain undiagnosed. Understanding the clinical, electrographic, and neuroradiological alternations can be crucial in delineating this entity. Objective This systematic review aims to analyze the effects of corpus callosum in...

The complex link between cognitive impairment and neurological disorders underscores the intricacies of neurological sciences [...]

Myotonic dystrophy type 1 (DM1), recognized as the most prevalent form of muscular dystrophy in adults, is an autosomal dominant disorder that impacts multiple systems, including skeletal, cardiac, and smooth muscles, as well as neurological components (including the central nervous system), the endocrine system, and others. Notably, the heart emer...

Cerebral venous sinus thrombosis (CVST) is one of the most recognized causes of stroke among young adults. Clinical manifestation at onset is highly variable, including headache, behavioral abnormalities, seizures, altered consciousness, and motor paresis. In India, common venomous snakes include the common krait (Bungarus caeruleus), the Indian...

Background and aims: Acute onset de novo abnormal movement disorder is increasingly recognized, yet under-reported complication of diabetes. Hyperglycemia can cause a range of different movement disorders, hemichorea-hemiballism being the commonest. This article delves into the current knowledge about this condition, its diverse presentations, ongo...

A 40-year-old male farmer from rural West Bengal, India, presented with fever, headaches, seizure, eschar, and palsy of left cranial nerves VII, VIII, IX, and X as a rare clinical manifestation of scrub typhus. Positive IgM enzyme-linked immunosorbent assay tests in serum and cerebrospinal fluid confirmed the diagnosis, with brain magnetic resonanc...

Background A wide variety of associated movement disorders has been described in multiple sclerosis. Phenomenology Shown A 57-year-old woman with primary progressive multiple sclerosis developed spinal segmental myoclonus associated with focal myelitis. Educational Value Movement disorders in multiple sclerosis are phenomenologically diverse and...

Background: A protective effect of education on cognitive decline after stroke has been claimed, but evidence from prospective population-based cohorts is very limited. The differential role of literacy and education on dementia after stroke remains unexplored. Objective: This research addresses the role of education and literacy in dementia incide...

Cockayne syndrome (CS) is a rare autosomal recessive disease caused by biallelic mutations in ERCC6 (excision repair 6, chromatin remodeling factor) (CS type B [CSB]) or ERCC8 (excision repair 8, chromatin remodeling factor) (CS type A [CSA]) genes showing significant genotypic and phenotypic heterogeneity. It manifests as a progressive multisy...

BACKGROUND: Scrub typhus, a mite-borne zoonosis caused by Orientia tsutsugamushi, is prevalent in the Asia Pacific region and presents with various neurological manifestations, including meningoencephalitis, polyneuropathy, and, rarely, cerebral venous sinus thrombosis (CVST). CASE REPORT: We report a unique case of CVST following scrub typhus meni...

Introduction Fatigue is one of the most disabling symptoms of multiple sclerosis (MS), and effective treatments are lacking. Amantadine is one of the most used treatments, although its efficacy is under debate. Transcranial magnetic stimulation (TMS) is a promising intervention that has shown positive effects in some preliminary investigations. We...

This video abstract delves into the expanded definition of diabetic striatopathy, linked initially to hyperglycemia-induced choreoballism and striatal hyperintensity on magnetic resonance imaging, but now recognized to encompass a broader range of acute onset, non-choreoballistic movement disorders in diabetes mellitus, including tremors, hemifacia...

Background: Six previous observational studies have found a higher risk for stroke among traumatic head injury survivors. However, these studies have used records linkage systems, which could lead to underestimation of the stroke numbers. This study aims to re-examine the relationship between head trauma and the risk of ischemic stroke/ transient i...

BACKGROUND: Hypercalcemia-induced posterior reversible encephalopathy syndrome (PRES) is a rare entity primarily associated with iatrogenic vitamin D/calcium overdose, malignancy, or, infrequently, primary hyperparathyroidism. CASE REPORT: We present a novel case of an adult male from rural India who experienced recurrent acute pancreatitis caused...

Orthostatic tremor is a rare and enigmatic movement disorder characterized by rapid tremor of both legs and the trunk while standing, which disappears while the patient is either lying down or walking. It may be primary with or without postural arm tremor or associated with other neurological features, mainly parkinsonism (“orthostatic tremor plus”...

Bálint syndrome (BS) is a rare and disabling higher-level visual cognitive impairment consisting of a triad of simultanagnosia (inability to see more than a small number of items simultaneously), optic ataxia (impaired visual guidance of movement of the limbs and body), and oculomotor apraxia (inability to volitionally direct gaze despite the r...

Background Obstructive sleep apnea is a highly prevalent disorder, characterized by recurrent events of upper airway obstruction during sleep and associated with recurrent cycles of desaturation and re-oxygenation, sympathetic hyperactivity, and intra-thoracic pressure fluctuations, resulting in fragmentation of sleep and subsequent daytime fatigue...

Background 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 control...

BACKGROUND Students, teachers, corporate houses, and people from various streams have started using Chat Generative Pre-trained Transformer (ChatGPT) for already integrated information and writing. Discussions have already gained attention on ChatGPT, and some researchers also wanted to categorize this invention as a revolution. OBJECTIVE The auth...

Beriberi, a treatable thiamine (vitamin B1) deficiency disease, may present as dry beriberi (commonly manifesting as a symmetric sensorimotor polyneuropathy that primarily affects the distal limbs) and wet beriberi with heart failure, with or without polyneuropathy. We report the case of a lactating mother who developed polyneuropathy associated wi...

Background and objectives: The cerebello-thalamo-cortical circuit plays a critical role in essential tremor (ET). However, abnormalities have been reported in multiple brain regions outside this circuit, leading to inconsistent characterization of ET pathophysiology. Here, we test whether these mixed findings in ET localize to a common functional...