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Neurological features - Top 30 Publications

Clinical analysis of 59 children with hand foot and mouth diseases due to enterovirus EV71 and concomitant viral encephalitis.

To analyzed the clinical features of children with HFMD and viral encephalitis and to summarize some treatments.

Clinical features and diagnosis of diabetic polyneuropathy.

Diabetic polyneuropathy (DPN) is a serious complication of diabetes that leads to early disability in patients if late diagnosed. There has been a lot of research into pathophysiological mechanisms of nerve fiber damage and risk factors for neurological complications of diabetes. Early diagnosis of the latter is quite a challenge and, thus, the problem of finding more reliable diagnostic modalities is rather pressing. In this review, an interdisciplinary approach to the said problem, high-end diagnostic methods, and clinical features of DPN are described.

Superficial siderosis of central nervous system with unknown cause: report of 2 cases and review of the literature.

To report 2 cases of superficial siderosis of central nervous system (SS-CNS) and a review of the literature.

Encephalitis and myelitis in tropical countries: Report from the Task Force on Tropical Diseases by the World Federation of Societies of Intensive and Critical Care Medicine.

Tropical diseases are those that occur primarily or solely in the tropics, and as such include infectious diseases that are particularly prevalent in hot, humid conditions. The incidence of encephalitis in tropical countries is reported to be as high as 6.34/100,000/year. The term encephalitis implies inflammation of the brain and includes the presence of encephalopathy with two and more of the following features: fever, seizures and/or focal neurological findings; a cerebrospinal fluid pleocytosis; electroencephalographic findings or abnormal neuroimaging suggestive of encephalitis. Transverse myelitis (TM) is an inflammation of the spinal cord which has a wide variety of clinical presentations depending on the degree (severity of myelin and neuronal injury) and site of spinal cord involvement. In the present article we discuss the various forms of tropical, viral encephalitides and myelitis and the diagnosis and management.

CT and MRI Findings in Cerebral Aspergilloma.

Purpose Invasive aspergillosis usually affects immunocompromised patients. It carries a high risk of morbidity and mortality and usually has a nonspecific clinical presentation. Early diagnosis is essential in order to start effective treatment and improve clinical outcome. Materials and Methods In a retrospective search of the PACS databases from two medical centers, we identified 9 patients with histologically proven cerebral aspergilloma. We systematically analyzed CT and MRI imaging findings to identify typical imaging appearances of cerebral aspergilloma. Results CT did not show a typical appearance of the aspergillomas. In 100 % (9/9) there was a rim-attenuated diffusion restriction on MRI imaging. Multiple hypointense layers in the aspergillus wall, especially on the internal side, were detected in 100 % on T2-weighted imaging (9/9). Aspergillomas were T1-hypointense in 66 % of cases (6/9) and partly T1-hyperintense in 33 % (3/9). In 78 % (7/9) of cases, a rim-attenuated diffusion restriction was detected after contrast agent application. Conclusion Nine cases were identified. Whereas CT features were less typical, we observed the following imaging features on MRI: A strong, rim-attenuated diffusion restriction (9/9); onion layer-like hypointense zones, in particular in the innermost part of the abscess wall on T2-weighted images (9/9). Enhancement of the lesion border was present in the majority of the cases (7/9). Key points  · There are typical MRI imaging features of aspergillomas.. · However, these findings could be affected by the immune status of the patient.. · Swift identification of aspergilloma imaging patterns is essential to allow for adequate therapeutic decision making.. Citation Format · Gärtner F, Forstenpointner J, Ertl-Wagner B et al. CT and MRI Findings in Cerebral Aspergilloma. Fortschr Röntgenstr 2017; DOI: 10.1055/s-0043-120766.

Clinical presentation of anti-N-methyl-d-aspartate receptor and anti-voltage-gated potassium channel complex antibodies in children: A series of 24 cases.

The symptomatology and paraclinical findings of antibody-mediated encephalitis, a relatively novel disorder, are still being characterized in adults and children. A high index of suspicion is needed in order to identify these cases among children presenting with various neurological symptoms. The aim of this study is to examine the clinical, demographic and laboratory findings and outcome of children with anti-NMDAR and anti-VGKC encephalitis for any typical or distinctive features.

Broad spectrum of neuropsychiatric phenotypes associated with white matter disease in PTEN hamartoma tumor syndrome.

White matter lesions have been described in patients with PTEN hamartoma tumor syndrome (PHTS). How these lesions correlate with the neurocognitive features associated with PTEN mutations, such as autism spectrum disorder (ASD) or developmental delay, has not been well established. We report nine patients with PTEN mutations and white matter changes on brain magnetic resonance imaging (MRI), eight of whom were referred for reasons other than developmental delay or ASD. Their clinical presentations ranged from asymptomatic macrocephaly with normal development/intellect, to obsessive compulsive disorder, and debilitating neurological disease. To our knowledge, this report constitutes the first detailed description of PTEN-related white matter changes in adult patients and in children with normal development and intelligence. We present a detailed assessment of the neuropsychological phenotype of our patients and discuss the relationship between the wide array of neuropsychiatric features and observed white matter findings in the context of these individuals.

Cerebral Palsy: An Overview.

Cerebral palsy (CP) is a neurodevelopmental disorder characterized by abnormalities of muscle tone, movement and motor skills, and is attributed to injury to the developing brain. The clinical features of this entity evolve over time and the specific CP syndrome may be recognizable only after 3-5 y of age; although suggestive signs and symptoms may be present at an earlier age. The management involves neurological rehabilitation (addressing muscle tonal abnormalities, and devising physical and occupational therapies) and diagnosis and management of co-morbidities (including epilepsy, impairment of cognition, vision, hearing, and disturbances of growth and gastrointestinal function). The management, therefore, is multidisciplinary involving the treating physician working with a team of rehabilitation-, orthopedic-, psychologic-, and social care- providers.

Late-onset hemophagocytic lymphohistiocytosis with neurological presentation.

Missense mutations in genes involved in familial hemophagocytic lymphohistiocytosis can delay the onset of this life-threatening disease. In children and adults, early recognition of aspecific features as neurological symptoms is crucial as urgent treatment is required.

Parkinson's disease: experimental models and reality.

Parkinson's disease (PD) is a chronic, progressive movement disorder of adults and the second most common neurodegenerative disease after Alzheimer's disease. Neuropathologic diagnosis of PD requires moderate-to-marked neuronal loss in the ventrolateral substantia nigra pars compacta and α-synuclein (αS) Lewy body pathology. Nigrostriatal dopaminergic neurodegeneration correlates with the Parkinsonian motor features, but involvement of other peripheral and central nervous system regions leads to a wide range of non-motor features. Nigrostriatal dopaminergic neurodegeneration is shared with other parkinsonian disorders, including some genetic forms of parkinsonism, but many of these disorders do not have Lewy bodies. An ideal animal model for PD, therefore, should exhibit age-dependent and progressive dopaminergic neurodegeneration, motor dysfunction, and abnormal αS pathology. Rodent models of PD using genetic or toxin based strategies have been widely used in the past several decades to investigate the pathogenesis and therapeutics of PD, but few recapitulate all the major clinical and pathologic features of PD. It is likely that new strategies or better understanding of fundamental disease processes may facilitate development of better animal models. In this review, we highlight progress in generating rodent models of PD based on impairments of four major cellular functions: mitochondrial oxidative phosphorylation, autophagy-lysosomal metabolism, ubiquitin-proteasome protein degradation, and endoplasmic reticulum stress/unfolded protein response. We attempt to evaluate how impairment of these major cellular systems contribute to PD and how they can be exploited in rodent models. In addition, we review recent cell biological studies suggesting a link between αS aggregation and impairment of nuclear membrane integrity, as observed during cellular models of apoptosis. We also briefly discuss the role of incompetent phagocytic clearance and how this may be a factor to consider in developing new rodent models of PD.

NMR Metabolomics for Stem Cell type discrimination.

Cell metabolism is a key determinant factor for the pluripotency and fate commitment of Stem Cells (SCs) during development, ageing, pathological onset and progression. We derived and cultured selected subpopulations of rodent fetal, postnatal, adult Neural SCs (NSCs) and postnatal glial progenitors, Olfactory Ensheathing Cells (OECs), respectively from the subventricular zone (SVZ) and the olfactory bulb (OB). Cell lysates were analyzed by proton Nuclear Magnetic Resonance ((1)H-NMR) spectroscopy leading to metabolites identification and quantitation. Subsequent multivariate analysis of NMR data by Principal Component Analysis (PCA), and Partial Least Square Discriminant Analysis (PLS-DA) allowed data reduction and cluster analysis. This strategy ensures the definition of specific features in the metabolic content of phenotypically similar SCs sharing a common developmental origin. The metabolic fingerprints for selective metabolites or for the whole spectra demonstrated enhanced peculiarities among cell types. The key result of our work is a neat divergence between OECs and the remaining NSC cells. We also show that statistically significant differences for selective metabolites characterizes NSCs of different ages. Finally, the retrived metabolome in cell cultures correlates to the physiological SC features, thus allowing an integrated bioengineering approach for biologic fingerprints able to dissect the (neural) SC molecular specificities.

Exertional Chest Pain and Dyspnea From Hyperviscosity Syndrome Related to Marginal Zone Lymphoma.

Hyperviscosity syndrome is usually seen in Waldenstrom macroglobulinemia with the serum viscosity > 4 cP. It commonly manifests as skin or mucosal bleeding, visual abnormalities and neurological symptoms. We describe a case of a 65-year-old man, who presented with chronic exertional chest pain and dyspnea without other manifestation and had hyperviscosity syndrome related to marginal zone lymphoma (MZL) even with a marginally elevated plasma viscosity. The symptoms resolved after initiation of chemotherapy. This case illustrates that MZL can cause hyperviscosity syndrome, which can manifest with cardiopulmonary symptoms without other clinical features even at a marginally elevated plasma viscosity. Atypical cases of hyperviscosity syndrome can, thus, present diagnostic challenges and require a high index of suspicion.

Resting-state fMRI in sleeping infants more closely resembles adult sleep than adult wakefulness.

Resting state functional magnetic resonance imaging (rs-fMRI) in infants enables important studies of functional brain organization early in human development. However, rs-fMRI in infants has universally been obtained during sleep to reduce participant motion artifact, raising the question of whether differences in functional organization between awake adults and sleeping infants that are commonly attributed to development may instead derive, at least in part, from sleep. This question is especially important as rs-fMRI differences in adult wake vs. sleep are well documented. To investigate this question, we compared functional connectivity and BOLD signal propagation patterns in 6, 12, and 24 month old sleeping infants with patterns in adult wakefulness and non-REM sleep. We find that important functional connectivity features seen during infant sleep closely resemble those seen during adult sleep, including reduced default mode network functional connectivity. However, we also find differences between infant and adult sleep, especially in thalamic BOLD signal propagation patterns. These findings highlight the importance of considering sleep state when drawing developmental inferences in infant rs-fMRI.

An extensive chronic aortic dissection presenting with acute embolic stroke.

Herein, we present a rare case of extensive chronic aortic dissection with extension to bilateral subclavian arteries, bilateral common carotid arteries, right internal carotid artery, bilateral proximal external iliac arteries and simultaneous presentation of acute embolic stroke and seizure. The rarity of this case presentation and the presence of neurological features necessitated a high index of clinical suspicion to reach the definitive diagnosis. This study also demonstrates a unique situation requiring correlation between chronic aortic dissection and multi-organ system dysfunction from chronic ischemia.

Phantom Sensations, Supernumerary Phantom Limbs and Apotemnophilia: Three Body Representation Disorders.

Body representation disorders continue to be mysterious and involve the anatomical substrate that underlies the mental representation of the body. These disorders sit on the boundaries of neurological and psychiatric diseases. We present the main characteristics of 3 examples of body representation disorders: phantom sensations, supernumerary phantom limb, and apotemnophilia. The dysfunction of anatomical circuits that regulate body representation can sometimes have paradoxical features. In the case of phantom sensations, the patient feels the painful subjective sensation of the existence of the lost part of the body after amputation, surgery or trauma. In case of apotemnophilia, now named body integrity identity disorder, the subject wishes for the disappearance of the existing and normal limb, which can occasionally lead to self-amputation. More rarely, a brain-damaged patient with 4 existing limbs can report the existence of a supernumerary phantom limb.

Central pain processing in "drug-naïve" pain-free patients with Parkinson's disease.

Despite its clinical relevance, the pathophysiology of pain in Parkinson's disease (PD) is still largely unknown, and both central and peripheral mechanisms have been invoked.

Posterior circulation involvement in pediatric and adult patients with moyamoya disease: a single center experience in 574 patients.

The importance of the posterior cerebral artery (PCA) involvement in moyamoya disease has been highlighted in recent years. However, few studies compared the impact of PCA lesions in moyamoya disease between pediatric and adult patients. We conducted this study to summarize the clinical features of moyamoya patients with PCA lesions and describe the difference between pediatric and adult patients. We reviewed the records of 696 consecutive moyamoya vasculopathy patients from 2009 to 2015. The Suzuki and the Miyamoto stages were used to evaluate the steno-occlusive lesions of the anterior and posterior arteries. Clinical and radiographic features were compared between those with and without PCA involvement, also between pediatric and adult patients. A total of 574 angiograms (140 pediatrics and 434 adults) were reviewed. The prevalence of PCA steno-occlusion did not differ significantly between pediatric patients and adult patients (35.0% vs. 30.4%, P = 0.347). Pediatric patients had more advanced PCA stages compared to adult patients (P = 0.045). There was a significant correlation of the PCA angiographic stages with the ipsilateral internal carotid artery (ICA) stages, both in pediatrics and in adults (both P < 0.001). The frequency of ipsilateral cerebral infarction positively correlated with the advancement of PCA stages in adult patients (P < 0.001), but not significant in pediatric patients (P = 0.106). Pediatric patients tend to have more advanced PCA lesions than adult patients. The degree of PCA steno-occlusion positively correlates with the ipsilateral ICA stage, both in pediatrics and in adults.

Clinical features, surgical management, and prognostic factors of secretory meningiomas: a single-center case series of 149 patients.

Secretory meningioma (SM) is a rare histological subtype of the meningioma family. Few reports investigating SM have been published due to its extremely low incidence; thus, the current understanding of this disease is poor. We analyzed the incidence and clinical, radiological, pathological, and prognostic features of SM. Approximately 12,380 intracranial meningiomas were surgically resected at Beijing Tiantan Hospital between April 2008 and January 2017. All pathologically confirmed SM cases were identified. SMs accounted for approximately 1.2% of the intracranial meningiomas (149 of 12,380). The patients with SM had a mean age of 51.0 years and were predominantly female (112 female and 37 male). Radiologically, peritumoral brain edema was observed in 49 (32.9%) patients. Gross total resection was achieved in 115 (77.2%) cases. At the 35-months median follow-up (range 4-109 months), six patients had tumor recurrence, and one patient died from the tumor recurrence. The 5-year progression-free survival rates were 95.9%, and the 5-year overall survival rate was 99.3%. A skull base location and a tumor size ≥ 3.5 cm were significantly associated with poor short-term outcomes, and a skull base location was significantly associated with an increased risk of poor long-term outcomes (P < 0.05). A skull base location (OR 3.797; 95% CI 1.071-13.468; P = 0.039) and tumor size ≥ 3.5 cm (OR 2.616; 95% CI 1.107-6.181; P = 0.028) were independent risk factors for non-gross total resection. A son-skull base location (OR 0.070; 95% CI 0.028-0.177; P = 0.001) was the only independent risk factor that correlated with more severe peritumoral brain edema. SM is a rare subtype of meningiomas with a female predominance and low recurrence. Our results highlight the risk factors for short- and long-term outcomes, which can be useful for selecting treatments and predicting prognosis. Microsurgical treatment of a skull base SM remains a formidable challenge due to a large tumor size and critical neurovascular structure encasement.

Characteristics of two distinct clinical phenotypes of functional (psychogenic) dystonia: follow-up study.

The fixed dystonia phenotype was originally established as a prototype of functional dystonia. Nevertheless, in recent reports different functional dystonia phenotypes have been recognized with dystonic movement comprising phasic instead of tonic contraction.

Nonalcoholic fatty liver disease in spinal and bulbar muscular atrophy.

To determine the prevalence and features of fatty liver disease in spinal and bulbar muscular atrophy (SBMA).

Pathophysiology and Risk of Atrial Fibrillation Detected after Ischemic Stroke (PARADISE): A Translational, Integrated, and Transdisciplinary Approach.

It has been hypothesized that ischemic stroke can cause atrial fibrillation. By elucidating the mechanisms of neurogenically mediated paroxysmal atrial fibrillation, novel therapeutic strategies could be developed to prevent atrial fibrillation occurrence and perpetuation after stroke. This could result in fewer recurrent strokes and deaths, a reduction or delay in dementia onset, and in the lessening of the functional, structural, and metabolic consequences of atrial fibrillation on the heart.

Visual snow syndrome: what we know so far.

We provide an overview of the neurological condition known as visual snow syndrome. Patients affected by this chronic disorder suffer with a pan-field visual disturbance described as tiny flickering dots, which resemble the static noise of an untuned television.

A Case of C5 Vertebral Chordoma in a 73-Year-Old Patient with More Than 8 Years of Follow-Up after Total Piecemeal Spondylectomy.

Chordoma arising from the cervical spine is rare and the traditional long-term prognosis is typically poor. Total en bloc spondylectomy with a wide margin is generally accepted to be the most appropriate management for thoracic and lumbar malignant tumors. However, this method is still challenging for the cervical spine because of the proximity of the tumor to the vertebral arteries and neural elements. Here, we report a 73-year-old man with a C5 vertebral chordoma treated with total piecemeal spondylectomy. Histological examination revealed pathognomonic physaliphorous cells with mucus-filled cytoplasm in the tumor, and the ratio of Ki-67-positive cells within the tumor was high (19.0%), showing active proliferation rate. Local recurrences were found at 9 months, 4 years and 2 months, and 6 years after the initial surgery. All the recurrences were encapsulated and isolated and treated with an additional en bloc resection successfully at each stage. Eight years after the initial total piecemeal spondylectomy, the patient maintained his intact neurological status without local recurrence or metastasis. The prognosis of cervical chordoma depends on the patient's age, surgical procedures, and histological features. In this report, we present that piecemeal spondylectomy is an alternative management for aged patients with cervical chordoma, even for those with high MIB-1 index.

Stem Cell Tracking Technologies for Neurological Regenerative Medicine Purposes.

The growing field of stem cell therapy is moving toward clinical trials in a variety of applications, particularly for neurological diseases. However, this translation of cell therapies into humans has prompted a need to create innovative and breakthrough methods for stem cell tracing, to explore the migration routes and its reciprocity with microenvironment targets in the body, to monitor and track the outcome after stem cell transplantation therapy, and to track the distribution and cell viability of transplanted cells noninvasively and longitudinally. Recently, a larger number of cell tracking methods in vivo were developed and applied in animals and humans, including magnetic resonance imaging, nuclear medicine imaging, and optical imaging. This review has been intended to summarize the current use of those imaging tools in tracking stem cells, detailing their main features and drawbacks, including image resolution, tissue penetrating depth, and biosafety aspects. Finally, we address that multimodality imaging method will be a more potential tracking tool in the future clinical application.

Genetic and immune features of resectable malignant brainstem gliomas.

We surveyed common genetic mutations (IDH1, H3F3A, PPM1D, and TP53) and immune features (PD-L1 expression and CD8(+) T cell tumor infiltration) in a series of 62 malignant brainstem gliomas that were resected via microsurgery. IDH1 mutations were mutually exclusive with H3F3A mutations. IDH1 mutations appeared only in adults and occurred more frequently in tumors larger than 10cm(3) (8/29 vs 1/32, Fisher's exact test, p=0.010). H3F3A mutations occurred more frequently in children and adolescent patients (19/24 vs 18/38, chi-square test, p=0.013), low preoperative Karnofsky Performance Scale (KPS) patients (10/11 vs 20/43, chi-square test, p=0.021), and higher grade brainstem gliomas (8/21 in grade II vs 16/24 in grade III vs 13/17 in grade IV; chi-square test, p=0.038). PPM1D mutations clustered in H3F3A-mutated tumors (12/37), whereas were rare in H3F3A wildtype tumors (1/25). MGMT promoter methylations clustered in IDH1-mutated tumors (4/9), but were rare in H3F3A-mutated tumors (1/37). PD-L1 staining was detected in 59.7% of brainstem glioma specimens (37/62). High intra-tumoral CD8(+) T cell density was less frequent in the H3F3A-mutated than H3F3A-wild-type tumors (4/37 vs. 11/25, p=0.005). Patients with H3F3A-mutated tumors (13.8 months overall survival) had much worse prognoses than those with IDH1-mutated (54.9 months, p=0.001) or H3F3A-IDH1 co-wildtype tumors (38.4 months, p=0.001). H3F3A mutations independently increased the relative risk of death as much as 4.19-fold according to a multivariate Cox regression model. Taken together, resectable malignant brainstem gliomas can be classified into three subtypes: H3F3A-mutated, IDH1 mutated and H3F3A-IDH1 co-wildtype tumors, which have distinct clinical characteristics, prognoses, genetic and immune features.

Immediate Label-Free Ex Vivo Evaluation of Human Brain Tumor Biopsies With Confocal Reflectance Microscopy.

Confocal microscopy utilizing fluorescent dyes is widely gaining use in the clinical setting as a diagnostic tool. Reflectance confocal microscopy is a method of visualizing tissue specimens without fluorescent dyes while relying on the natural refractile properties of cellular and subcellular structures. We prospectively evaluated 76 CNS lesions with confocal reflectance microscopy (CRM) to determine cellularity, architecture, and morphological characteristics. A neuropathologist found that all cases showed similar histopathological features when compared to matched hematoxylin and eosin-stained sections. RNA isolated from 7 tissues following CRM imaging retained high RNA integrity, suggesting that CRM does not alter tissue properties for molecular studies. A neuropathologist and surgical pathologist masked to the imaging results independently evaluated a subset of CRM images. In these evaluations, 100% of images reviewed by the neuropathologist and 95.7% of images reviewed by the surgical pathologist were correctly diagnosed as lesional or nonlesional. Furthermore, 97.9% and 91.5% of cases were correctly diagnosed as tumor or not tumor by the neuropathologist and surgical pathologist, respectively, while 95.8% and 85.1% were identified with the correct diagnosis. Our data indicate that CRM is a useful tool for rapidly screening patient biopsies for diagnostic adequacy, molecular studies, and biobanking.

Artificial intelligence in neurodegenerative disease research: use of IBM Watson to identify additional RNA-binding proteins altered in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease with no effective treatments. Numerous RNA-binding proteins (RBPs) have been shown to be altered in ALS, with mutations in 11 RBPs causing familial forms of the disease, and 6 more RBPs showing abnormal expression/distribution in ALS albeit without any known mutations. RBP dysregulation is widely accepted as a contributing factor in ALS pathobiology. There are at least 1542 RBPs in the human genome; therefore, other unidentified RBPs may also be linked to the pathogenesis of ALS. We used IBM Watson(®) to sieve through all RBPs in the genome and identify new RBPs linked to ALS (ALS-RBPs). IBM Watson extracted features from published literature to create semantic similarities and identify new connections between entities of interest. IBM Watson analyzed all published abstracts of previously known ALS-RBPs, and applied that text-based knowledge to all RBPs in the genome, ranking them by semantic similarity to the known set. We then validated the Watson top-ten-ranked RBPs at the protein and RNA levels in tissues from ALS and non-neurological disease controls, as well as in patient-derived induced pluripotent stem cells. 5 RBPs previously unlinked to ALS, hnRNPU, Syncrip, RBMS3, Caprin-1 and NUPL2, showed significant alterations in ALS compared to controls. Overall, we successfully used IBM Watson to help identify additional RBPs altered in ALS, highlighting the use of artificial intelligence tools to accelerate scientific discovery in ALS and possibly other complex neurological disorders.

Clinical characteristics of lumbosacral spinal dural arteriovenous fistula (DAVF)-comparison to thoracic DAVF.

Spinal dural arteriovenous fistula (DAVF) occurs at any spinal level but the clinical characteristics of lumbosacral DAVF have not been well documented. Purpose of this study was to evaluate clinical characteristics of lumbosacral DAVF and compare these features to those in thoracic DAVF.

Patients' and caregivers' contributions for differentiating epileptic from psychogenic nonepileptic seizures. Value and limitations of self-reporting questionnaires: A pilot study.

Questionnaires investigating semiology and comorbidities of psychogenic non-epileptic seizures (PNES) have been used mainly to help physicians expedite referrals to epilepsy centres for confirmation of diagnosis rather than as alternative diagnostic tool when video-EEG monitoring (VEM), the current gold standard, is not available or is inconclusive.

Molecular function of α7 nicotinic receptors as drug targets.

Nicotinic acetylcholine receptors (nAChR) are pentameric ligand-gated ion channels involved in many physiological and pathological processes. In vertebrates, there are seventeen different nAChR subunits that combine to yield a variety of receptors with different pharmacology, function, and localization. The homomeric α7 receptor is one of the most abundant nAChRs in the nervous system and it is also present in non-neuronal cells. It plays important roles in cognition, memory, pain, neuroprotection, and inflammation. Its diverse physiological actions and associated disorders have made of α7 an attractive novel target for drug modulation. Potentiation of the α7 receptor has emerged as a novel therapeutic strategy for several neurological diseases, such as Alzheimer's and Parkinson's diseases, and inflammatory disorders. In contrast, increased α7 activity has been associated to cancer cell proliferation. The presence of different drug target sites offers a great potential for α7 modulation in different pathological contexts. In particular, compounds that target allosteric sites offer significant advantages over orthosteric agonists due to higher selectivity and a broader spectrum of degrees and mechanisms of modulation. Heterologous expression of α7, together with chaperone proteins, combined with patch clamp recordings have provided important advances in our knowledge of the molecular basis of α7 responses and their potential modulation for pathological processes. This review gives a synthetic view of α7 and its molecular function, focusing on how its unique activation and desensitization features can be modified by pharmacological agents. This fundamental information offers insights into therapeutic strategies. This article is protected by copyright. All rights reserved.