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Sample records for neurological disorders charcot

  1. A neurological bias in the history of hysteria: from the womb to the nervous system and Charcot

    Directory of Open Access Journals (Sweden)

    Marleide da Mota Gomes

    2014-12-01

    Full Text Available Hysteria conceptions, from ancient Egypt until the 19th century Parisian hospital based studies, are presented from gynaecological and demonological theories to neurological ones. The hysteria protean behavioral disorders based on nervous origin was proposed at the beginning, mainly in Great Britain, by the “enlightenment nerve doctors”. The following personages are highlighted: Galen, William, Sydenham, Cullen, Briquet, and Charcot with his School. Charcot who had hysteria and hypnotism probably as his most important long term work, developed his conceptions, initially, based on the same methodology he applied to studies of other neurological disorder. Some of his associates followed him in his hysteria theories, mainly Paul Richer and Gilles de La Tourette who produced, with the master's support, expressive books on Salpêtrière School view on hysteria.

  2. A neurological bias in the history of hysteria: from the womb to the nervous system and Charcot.

    Science.gov (United States)

    Mota Gomes, Marleide da; Engelhardt, Eliasz

    2014-12-01

    Hysteria conceptions, from ancient Egypt until the 19th century Parisian hospital based studies, are presented from gynaecological and demonological theories to neurological ones. The hysteria protean behavioral disorders based on nervous origin was proposed at the beginning, mainly in Great Britain, by the "enlightenment nerve doctors". The following personages are highlighted: Galen, William, Sydenham, Cullen, Briquet, and Charcot with his School. Charcot who had hysteria and hypnotism probably as his most important long term work, developed his conceptions, initially, based on the same methodology he applied to studies of other neurological disorder. Some of his associates followed him in his hysteria theories, mainly Paul Richer and Gilles de La Tourette who produced, with the master's support, expressive books on Salpêtrière School view on hysteria.

  3. Functional neurological disorders: imaging.

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    Voon, V

    2014-10-01

    Functional neurological disorders, also known as conversion disorder, are unexplained neurological symptoms. These symptoms are common and can be associated with significant consequences. This review covers the neuroimaging literature focusing on functional motor symptoms including motor functioning and upstream influences including self-monitoring and internal representations, voluntariness and arousal and trauma. Copyright © 2014. Published by Elsevier SAS.

  4. Genetics of neurological disorders.

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    Faghihi, Mohammad Ali; Mottagui-Tabar, Salim; Wahlestedt, Claes

    2004-05-01

    Neurological diseases are defined as an inappropriate function of the peripheral or central nervous system due to impaired electrical impulses throughout the brain and/or nervous system that may present with heterogeneous symptoms according to the parts of the system involved in these pathologic processes. Growing evidence on genetic components of neurological disease have been collected during recent years. Genetic studies have opened the way for understanding the underlying pathology of many neurological disorders. The outcome of current intense research into the genetics of neurological disorders will hopefully be the introduction of new diagnostic tools and the discovery of potential targets for new and more effective medications and preventive measures.

  5. Cardiomyopathy in neurological disorders.

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    Finsterer, Josef; Stöllberger, Claudia; Wahbi, Karim

    2013-01-01

    According to the American Heart Association, cardiomyopathies are classified as primary (solely or predominantly confined to heart muscle), secondary (those showing pathological myocardial involvement as part of a neuromuscular disorder) and those in which cardiomyopathy is the first/predominant manifestation of a neuromuscular disorder. Cardiomyopathies may be further classified as hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, or unclassified cardiomyopathy (noncompaction, Takotsubo-cardiomyopathy). This review focuses on secondary cardiomyopathies and those in which cardiomyopathy is the predominant manifestation of a myopathy. Any of them may cause neurological disease, and any of them may be a manifestation of a neurological disorder. Neurological disease most frequently caused by cardiomyopathies is ischemic stroke, followed by transitory ischemic attack, syncope, or vertigo. Neurological disease, which most frequently manifests with cardiomyopathies are the neuromuscular disorders. Most commonly associated with cardiomyopathies are muscular dystrophies, myofibrillar myopathies, congenital myopathies and metabolic myopathies. Management of neurological disease caused by cardiomyopathies is not at variance from the same neurological disorders due to other causes. Management of secondary cardiomyopathies is not different from that of cardiomyopathies due to other causes either. Patients with neuromuscular disorders require early cardiologic investigations and close follow-ups, patients with cardiomyopathies require neurological investigation and avoidance of muscle toxic medication if a neuromuscular disorder is diagnosed. Which patients with cardiomyopathy profit most from primary stroke prevention is unsolved and requires further investigations. Copyright © 2013 Elsevier Inc. All rights reserved.

  6. [Neurological sleep disorders].

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    Khatami, Ramin

    2014-11-01

    Neurological sleep disorders are common in the general population and may have a strong impact on quality of life. General practitioners play a key role in recognizing and managing sleep disorders in the general population. They should therefore be familiar with the most important neurological sleep disorders. This review provides a comprehensive overview of the most prevalent and important neurological sleep disorders, including Restless legs syndrome (with and without periodic limb movements in sleep), narcolepsy, NREM- and REM-sleep parasomnias and the complex relationship between sleep and epilepsies. Although narcolepsy is considered as a rare disease, recent discoveries in narcolepsy research provided insight in the function of brain circuitries involved in sleep wake regulation. REM sleep behavioral parasomnia (RBD) is increasingly recognized to represent an early manifestation of neurodegenerative disorders, in particular evolving synucleinopathies. Early diagnosis may thus open new perspectives for developing novel treatment options by targeting neuroprotective substances.

  7. Jean-Martin Charcot and art: relationship of the "founder of neurology" with various aspects of art.

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    Bogousslavsky, Julien; Boller, François

    2013-01-01

    Jean-Martin Charcot (1825-1893), the "father of neurology" in France and much beyond, was also the man who established academic psychiatry in Paris, differentiating it from clinical alienism. In his teaching, he used artistic representations from previous centuries to illustrate the historical developments of hysteria, mainly with the help of his pupil Paul Richer. Charcot liked to draw portraits (in particular, sketches of colleagues during boring faculty meetings and students' examinations), caricatures of himself and others, church sculptures, landscapes, soldiers, etc. He also used this skill in his clinical and scientific work; he drew histological or anatomic specimens, as well as patients' features and demeanor. His most daring artistic experiments were drawing under the influence of hashish. Charcot's tastes in art were conservative; he displayed no affinity for the avant-gardes of his time, including impressionism, or for contemporary musicians, such as César Franck or Hector Berlioz. Léon Daudet, son of Charcot's former friend and famous writer Alphonse Daudet, described Charcot's home as a pseudo-gothic kitsch accumulation of heteroclite pieces of furniture and materials. However, as Henry Meige wrote a few years after his mentor's death, Charcot the artist remains "inseparable from Charcot the physician." © 2013 Elsevier B.V. All rights reserved.

  8. Wikipedia and neurological disorders.

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    Brigo, Francesco; Igwe, Stanley C; Nardone, Raffaele; Lochner, Piergiorgio; Tezzon, Frediano; Otte, Willem M

    2015-07-01

    Our aim was to evaluate Wikipedia page visits in relation to the most common neurological disorders by determining which factors are related to peaks in Wikipedia searches for these conditions. Millions of people worldwide use the internet daily as a source of health information. Wikipedia is a popular free online encyclopedia used by patients and physicians to search for health-related information. The following Wikipedia articles were considered: Alzheimer's disease; Amyotrophic lateral sclerosis; Dementia; Epilepsy; Epileptic seizure; Migraine; Multiple sclerosis; Parkinson's disease; Stroke; Traumatic brain injury. We analyzed information regarding the total article views for 90 days and the rank of these articles among all those available in Wikipedia. We determined the highest search volume peaks to identify possible relation with online news headlines. No relation between incidence or prevalence of neurological disorders and the search volume for the related articles was found. Seven out of 10 neurological conditions showed relations in search volume peaks and news headlines. Six out of these seven peaks were related to news about famous people suffering from neurological disorders, especially those from showbusiness. Identification of discrepancies between disease burden and health seeking behavior on Wikipedia is useful in the planning of public health campaigns. Celebrities who publicly announce their neurological diagnosis might effectively promote awareness programs, increase public knowledge and reduce stigma related to diagnoses of neurological disorders. Copyright © 2015 Elsevier Ltd. All rights reserved.

  9. Jean-Martin Charcot's Role in the 19th Century Study of Music Aphasia

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    Johnson, Julene K.; Lorch, Marjorie; Nicolas, Serge; Graziano, Amy

    2013-01-01

    Jean-Martin Charcot (1825-93) was a well-known French neurologist. Although he is widely recognized for his discovery of several neurological disorders and his research into aphasia, Charcot's ideas about how the brain processes music are less well known. Charcot discussed the music abilities of several patients in the context of his "Friday…

  10. Wikipedia and neurological disorders

    NARCIS (Netherlands)

    Brigo, Francesco; Igwe, Stanley C.; Nardone, Raffaele; Lochner, Piergiorgio; Tezzon, Frediano; Otte, WM

    2015-01-01

    Our aim was to evaluate Wikipedia page visits in relation to the most common neurological disorders by determining which factors are related to peaks in Wikipedia searches for these conditions. Millions of people worldwide use the internet daily as a source of health information. Wikipedia is a

  11. Vaccination and neurological disorders

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    Anastasia Gkampeta

    2015-12-01

    Full Text Available Active immunization of children has been proven very effective in elimination of life threatening complications of many infectious diseases in developed countries. However, as vaccination-preventable infectious diseases and their complications have become rare, the interest focuses on immunization-related adverse reactions. Unfortunately, fear of vaccination-related adverse effects can led to decreased vaccination coverage and subsequent epidemics of infectious diseases. This review includes reports about possible side effects following vaccinations in children with neurological disorders and also published recommendations about vaccinating children with neurological disorders. From all international published data anyone can conclude that vaccines are safer than ever before, but the challenge remains to convey this message to society.

  12. Sleep disorders in Charcot-Marie-Tooth disease type 1.

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    Boentert, Matthias; Knop, Katharina; Schuhmacher, Christine; Gess, Burkhard; Okegwo, Angelika; Young, Peter

    2014-03-01

    Obstructive sleep apnoea (OSA) and restless legs syndrome (RLS) have been reported in Charcot-Marie-Tooth disease (CMT) type 1A and axonal subtypes of CMT, respectively. The aim of this case-control study was to investigate both prevalence and severity of OSA, RLS and periodic limb movements in sleep (PLMS) in adult patients with genetically proven CMT1. 61 patients with CMT1 and 61 insomnic control subjects were matched for age, sex, and Body Mass Index. Neurological disability in patients with CMT was assessed using the Functional Disability Scale (FDS). RLS diagnosis was based on a screening questionnaire and structured clinical interviews. All participants underwent overnight polysomnography. OSA was present in 37.7% of patients with CMT1 and 4.9% of controls (psleep quality. In addition to known risk factors, CMT may predispose to OSA. RLS is highly prevalent not only in axonal subtypes of CMT but also in primarily demyelinating subforms of CMT. PLMS are common in CMT1, but do not significantly impair sleep quality.

  13. Neurologic disorder and criminal responsibility.

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    Yaffe, Gideon

    2013-01-01

    Sufferers from neurologic and psychiatric disorders are not uncommonly defendants in criminal trials. This chapter surveys a variety of different ways in which neurologic disorder bears on criminal responsibility. It discusses the way in which a neurologic disorder might bear on the questions of whether or not the defendant acted voluntarily; whether or not he or she was in the mental state that is required for guilt for the crime; and whether or not he or she is deserving of an insanity defense. The discussion demonstrates that a just determination of whether a sufferer from a neurologic disorder is diminished in his or her criminal responsibility for harmful conduct requires equal appreciation of the nature of the relevant disorder and its impact on behavior, on the one hand, and of the legal import of facts about the psychologic mechanisms through which behavior is generated, on the other. © 2013 Elsevier B.V. All rights reserved.

  14. Sleep disorders in neurological practice

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    Mikhail Guryevich Poluektov

    2012-01-01

    Full Text Available Sleep disorders are closely associated with both nervous system diseases and mental disorders; however, such patients prefer to seek just neurological advice. Insomnia is the most common complaint in routine clinical practice. It is characterized by different impairments in sleep and daytime awakening. Obstructive sleep apnea syndrome is less common, but more clinically important because of its negative impact on the cardiovascular and nervous systems. The common neurological disorders are restless legs syndrome and REM sleep behavior disorder, as well as narcolepsy, the major manifestations of which are impaired nocturnal sleep and daytime awakening.

  15. Acupuncture application for neurological disorders.

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    Lee, Hyangsook; Park, Hi-Joon; Park, Jongbae; Kim, Mi-Ja; Hong, Meesuk; Yang, Jongsoo; Choi, Sunmi; Lee, Hyejung

    2007-01-01

    Acupuncture has been widely used for a range of neurological disorders. Despite its popularity, the evidence to support the use of acupuncture is contradictory. This review was designed to summarize and to evaluate the available evidence of acupuncture for neurological disorders. Most of the reviewed studies suffer from lack of methodological rigor. Owing to paucity and poor quality of the primary studies, no firm conclusion could be drawn on the use of acupuncture for epilepsy, Alzheimer's disease, Parkinson's disease, ataxic disorders, multiple sclerosis, amyotrophic lateral sclerosis and spinal cord injury. For stroke rehabilitation, the evidence from recent high-quality trials and previous systematic reviews is not convincing. More rigorous trials are warranted to establish acupuncture's role in neurological disorders.

  16. Functional Disorders in Neurology : Case Studies

    NARCIS (Netherlands)

    Stone, Jon; Hoeritzauer, Ingrid; Gelauff, Jeannette; Lehn, Alex; Gardiner, Paula; van Gils, Anne; Carson, Alan

    Functional, often called psychogenic, disorders are common in neurological practice. We illustrate clinical issues and highlight some recent research findings using six case studies of functional neurological disorders. We discuss dizziness as a functional disorder, describing the relatively new

  17. Charcot in contemporary literature.

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    Goetz, Christopher G

    2006-03-01

    Charcot and his medical observations remain an enduring topic of scientific study in neurology, but he is also the topic of modern literary works. This essay examines the depiction of Jean-Martin Charcot (1825-1893) as a character in late-twentieth-century literature as an index of the contemporary nonmedical literary public's interest in neurology and Charcot. It focuses on three contemporary works that involve Charcot as a central figure with comparison between primary source documents and the rendered context, character development, and plot lines of these literary works. The two French novels [Slumbers of Indiscretion and Dr. Charcot of the Salpêtrière] and one American play [Augustine (Big Hysteria)] approach Charcot and neurology with differing levels of historical accuracy. All create a figure of authority, each with a different coloration of the balance between power and its abuse. Two focus almost exclusively on his work with hysteria and inaccurately amplify Charcot's concern with symbolic sexual conflict as the origin of hysteria and fictionalize more extensive interactions with Freud than historical documents support. The three works demonstrate that Charcot retains an enduring fascination with an enigmatic personality, a controversial career, and a pivotal role in the development of studies involving the brain and behavior. Neurologists should not look to these works as replacements for more seriously composed historical studies, but as enrichments anchored in the imaginative possibilities of Charcot and his fin de siècle era.

  18. Proprioceptive reflexes and neurological disorders

    NARCIS (Netherlands)

    Schouten, A.C.

    2004-01-01

    Proprioceptive reflexes play an important role during the control of movement and posture. Disturbed modulation of proprioceptive reflexes is often suggested as the cause for the motoric features present in neurological disorders. In this thesis methods are developed and evaluated to quantify

  19. Poor Beard!! Charcot's internationalization of neurasthenia, the "American disease".

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    Goetz, C G

    2001-08-14

    To analyze the role of the seminal 19th-century neurologist, Jean-Martin Charcot, in the internationalization of neurasthenia, previously known as "the American disease." The New York neurologist, George Beard, first described neurasthenia in 1869 and considered it a disorder related to the particular stress of modern civilization, most typified in the United States. Charcot's personal files on neurasthenia from the Bibliothèque Charcot, Salpêtrière Hospital, Paris, were studied and his teaching lessons and lectures were examined. Charcot presented numerous cases of neurasthenia, always crediting Beard with the original name and description. Calling Beard's 1880 work a "remarkable monograph," Charcot emphasized that patients with neurasthenia also comprised the bulk of his own private practice. Focusing on the signs of sexual impotency, fatigue, and a tight band of pressure around the head, Charcot categorically distinguished patients with neurasthenica from patients with hysteria. Like Beard, Charcot concluded that the origin of neurasthenia was psychological stress and felt the European society also fostered the environment to precipitate the disease. Charcot adamantly opposed extrapolations that called for early childhood educational reforms to reduce current classroom stress. Charcot sympathized more with the treating physician than the patient, calling neurasthenics insufferable (insupportables). On the front sheet of his neurasthenia file, he wrote in large script, "Poor Beard!!" By emphasizing the prevalence of neurasthenia and extending Beard's observations, Charcot internationally legitimized the new diagnosis. Adding neurasthenia to the other neurologic descriptions from the United States by Hammond, Mitchell, and Dana, Charcot helped to foster the recognition of the American Neurologic School.

  20. Aphasia, Just a Neurological Disorder?

    Directory of Open Access Journals (Sweden)

    Mehmet Ozdemir

    2016-02-01

    Full Text Available Hashimoto%u2019s encephalopathy (HE is a rare disorder associated with autoimmune thyroiditis. Etiology of HE is not completely understood. High levels of serum antithyroid antibodies are seen in HE. Presentation with otoimmune thyroiditis, cognitive impairment, psychiatric and neurologic symptoms and absence of bacterial or viral enfections are characteristics of HE. HE is a steroid responsive encephalopathy. 60 years old male patient admitted to hospital with forget fulness continuing for 9 months and speech loss starting 2 days ago. Strong positivity of antithyroid antibodies increases the odds for HE. Thyroid function tests showed severe hypothyroidism. Electroencephalography and magnetic resonance imaging results were compatible with HE. HE is diagnosed with differantial diagnosis and exclusion of other reasons. This uncommon disorder is not recognised enough. High titres of serum antithyroid antiboides are always needed for diagnosis. Correct diagnosis requires awareness of wide range of cognitive and clinical presentations of HE.

  1. Neurological disorders in hypertensive patients

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    N. V. Vakhnina

    2015-01-01

    Full Text Available Hypertension is one of the most common vascular diseases. The brain as target organs in hypertension is damaged more often and earlier. Neurological complications due to hypertension are frequently hyperdiagnosed in Russian neurological practice. Thus, headache, dizziness, impaired recall of recent events, nocturnal sleep disorders, and many other complaints in a hypertensive patient are usually regarded as a manifestation of dyscirculatory encephalopathy. At the same time headaches (tension headache and migraine in hypertensive patients are predominantly primary; headache associated with dramatic marked elevations in blood pressure is encountered in only a small number of patients. The role of cerebrovascular diseases in the development of dizziness in hypertensive patients is also overestimated. The vast majority of cases, patients with this complaint are in fact identified to have benign paroxysmal postural vertigo, Mеniеre’s disease, vestibular neuronitis, or vestibular migraine. Psychogenic disorders or multisensory insufficiency are generally responsible for non-systemic vertigo in hypertensive patients. Chronic cerebral circulatory insufficiency may cause non-systemic vertigo as a subjective equivalent of postural instability.Cognitive impairments (CIs are the most common and earliest manifestation of cerebrovascular lesion in hypertension. In most cases, CIs in hypertension were vascular and associated with cerebrovascular lesion due to lacunar infarcts and leukoaraiosis. However, mixed CIs frequently occur when hypertensive patients are also found to have signs of a degenerative disease, most commonly in Alzheimer’s disease.

  2. Charcot and Brazil Charcot e o Brasil

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    Hélio A. Ghizoni Teive

    2001-06-01

    Full Text Available OBJECTIVE: To describe the relationship between Professor Charcot and Brazil. BACKGROUND: During the XIX century, French Neurology and its most prominent figure, Professor Charcot, dominated the area of nervous system diseases in the world. METHOD: We have reviewed some of the main publications about Charcot's life, the biography of Dom Pedro II, Emperor of Brazil and the development of Neurology in Brazil. RESULTS: Among the most important patients in Charcot's practice was the Emperor of Brazil. Dom Pedro II became a close friend of Charcot and he was a distinguished guest at Charcot's house, particularly at Tuesday soirées on boulevard St. Germain. In 1887, during the visit of Dom Pedro II to France, Charcot evaluated him and made the diagnosis of surmenage. In 1889, Dom Pedro II was deposed and went to Paris, where he lived until his death in 1891. Charcot signed the death certificate and gave the diagnosis of pneumonitis. Charcot had a passionate affection for animals, a feeling shared by Dom Pedro II. Dom Pedro II was affiliated to the French Society for the Protection of Animals. It is conceivable that Charcot's little monkey, from South America, was given to him by Dom Pedro II. The Brazilian Neurological School was founded by Professor A . Austregésilo in 1911, in Rio de Janeiro. At the time, of Charcot's death in 1893, his influence was still very important in the whole world. He and his pupils played a major role in the development of Brazilian Neurology. CONCLUSION: Professor Charcot had a close relationship with the Emperor of Brazil, Dom Pedro II. He was his private physician and they were close friends. The neurological school, created by professor Charcot, contributed significantly, albeit in an indirect way, to the development of Brazilian Neurology, starting in 1911, in Rio de Janeiro, by Professor A . Austregésilo.OBJETIVO: Descrever a relação entre o Professor Charcot e o Brasil. FUNDAMENTO: Durante o século XIX, a

  3. Sleep Disorders in Childhood Neurological Diseases

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    Abdullah Tolaymat

    2017-09-01

    Full Text Available Sleep problems are frequently addressed as a primary or secondary concern during the visit to the pediatric neurology clinic. Sleep disorders can mimic other neurologic diseases (e.g., epilepsy and movement disorders, and this adds challenges to the diagnostic process. Sleep disorders can significantly affect the quality of life and functionality of children in general and those with comorbid neurological diseases in particular. Understanding the pathophysiology of sleep disorders, recognizing the implications of sleep disorder in children with neurologic diseases and behavioral difficulties, and early intervention continue to evolve resulting in better neurocognitive outcomes.

  4. Endocannabinoid System in Neurological Disorders.

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    Ranieri, Roberta; Laezza, Chiara; Bifulco, Maurizio; Marasco, Daniela; Malfitano, Anna M

    2016-01-01

    Several studies support the evidence that the endocannabinoid system and cannabimimetic drugs might have therapeutic potential in numerous pathologies. These pathologies range from neurological disorders, atherosclerosis, stroke, cancer to obesity/metabolic syndrome and others. In this paper we review the endocannabinoid system signaling and its alteration in neurodegenerative disorders like multiple sclerosis, Alzheimer's disease, Parkinson's disease and Huntington's disease and discuss the main findings about the use of cannabinoids in the therapy of these pathologies. Despite different etiologies, neurodegenerative disorders exhibit similar mechanisms like neuro-inflammation, excitotoxicity, deregulation of intercellular communication, mitochondrial dysfunction and disruption of brain tissue homeostasis. Current treatments ameliorate the symptoms but are not curative. Interfering with the endocannabinoid signaling might be a valid therapeutic option in neuro-degeneration. To this aim, pharmacological intervention to modulate the endocannabinoid system and the use of natural and synthetic cannabimimetic drugs have been assessed. CB1 and CB2 receptor signaling contributes to the control of Ca2+ homeostasis, trophic support, mitochondrial activity, and inflammatory conditions. Several studies and patents suggest that the endocannabinoid system has neuro-protective properties and might be a target in neurodegenerative diseases.

  5. Charcot's irony and sarcasm.

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    Teive, Hélio A G; Germiniani, Francisco M B; Munhoz, Renato P

    2017-06-01

    Jean-Martin Charcot is considered the father of modern neurology and was the first neurologist to hold a professorship of international stature devoted to the study of the diseases of the nervous system. His biographers paint an image of an austere presence, reserved manner, shyness, economy of gestures and an impenetrable, impassive face. However, a wry and sarcastic side of Charcot can be demonstrated in several situations, and these examples help to clarify the intricacies of his personality and work style.

  6. [Gait disorders due to neurological conditions

    NARCIS (Netherlands)

    Warrenburg, B.P.C. van de; Snijders, A.H.; Munneke, M.; Bloem, B.R.

    2007-01-01

    Gait disorders are seen frequently and often have a neurological cause. The clinical management of patients presenting with a gait disorder is often complicated due to the large number of diseases that can cause a gait disorder and to the difficulties in interpreting a specific gait disorder

  7. Axon guidance proteins in neurological disorders

    NARCIS (Netherlands)

    Van Battum, Eljo Y.; Brignani, Sara; Pasterkamp, R. Jeroen|info:eu-repo/dai/nl/197768814

    2015-01-01

    Many neurological disorders are characterised by structural changes in neuronal connections, ranging from presymptomatic synaptic changes to the loss or rewiring of entire axon bundles. The molecular mechanisms that underlie this perturbed connectivity are poorly understood, but recent studies

  8. Neurological disorders in children with autism

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    N. N. Zavadenko

    2015-01-01

    Full Text Available During a clinical examination of children with autistic spectrum disorders, attention should be drawn to both their major clinical manifestations and neurological comorbidities. The paper considers the mechanisms of autism-induced neurological disorders, the spectrum of which may include manifestations, such as retarded and disharmonic early psychomotor development; the specific features of sensory perception/processing; rigidity and monotony of motor and psychic reactions; motor disinhibition and hyperexcitability; motor stereotypies; uncoordinated movements; developmental coordination disorders (dyspraxia; impaired expressive motor skills; speech and articulation disorders; tics; epilepsy. It describes the specific features of neurological symptoms in Asperger’s syndrome, particularly in semantic-pragmatic language disorders, higher incidence rates of hyperlexia, motor and vocal tics. The incidence rate of epilepsy in autistic spectrum disorders is emphasized to be greater than the average population one. At the same time, the risk of epilepsy is higher in mentally retarded patients with autism. Identification of neurological disorders is of great importance in determining the tactics of complex care for patients with autistic spectrum disorders

  9. Neurological Disorders in Adult Celiac Disease

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    Hugh J Freeman

    2008-01-01

    Full Text Available Celiac disease may initially present as a neurological disorder. Alternatively, celiac disease may be complicated by neurological changes. With impaired nutrient absorption, different deficiency syndromes may occur and these may be manifested clinically with neurological changes. However, in patients with deficiency syndromes, extensive involvement of the small intestine with celiac disease is often evident. There are a number of reports of celiac disease associated with neuropathy, ataxia, dementia and seizure disorder. In these reports, there is no clear relationship with nutrient deficiency and a precise mechanism for the neurological changes has not been defined. A small number of patients have been reported to have responded to vitamin E administration, but most do not. In some, gluten antibodies have also been described, especially in those with ataxia, but a consistent response to a gluten-free diet has not been defined. Screening for celiac disease should be considered in patients with unexplained neurological disorders, including ataxia and dementia. Further studies are needed, however, to determine if a gluten-free diet will lead to improvement in the associated neurological disorder.

  10. [Voice disorders caused by neurological diseases].

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    Gamboa, J; Jiménez-Jiménez, F J; Mate, M A; Cobeta, I

    To review voice disorders in neurological diseases, with special emphasis to acoustic analysis. In the first part of this article we describe data regarding neural control of voice, physiology of phonation, and examination of the patient with voice disturbances, including the use of voice laboratory, acoustic analysis fundamentals, phonetometric measures and aerodynamic measures. In the second part, we review the voice disturbances associated to neurological diseases, emphasizing into movement disorders (specially Parkinson s disease, essential tremor, and spasmodic dysphonia). A number of neurological diseases causing alterations of corticospinal pathway, cerebellum, basal ganglia and upper and/or lower motoneurons can induce voice disturbances. Voice examination using ear, nose & throat examination, endoscopy and videorecording of laryngeal movements, acoustic analysis, elecroglottography, laryngeal electromyography, and aerodynamic measures, could be useful in the clinical examination of some neurological diseases.

  11. Advances in genetic diagnosis of neurological disorders.

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    Toft, M

    2014-01-01

    Neurogenetics has developed enormously in recent years, and the genetic basis of human disorders is being unravelled rapidly. Many neurological disorders are Mendelian disorders, caused by mutations in genes involved in normal function of the brain, spinal cord, peripheral nerves or muscles. Due to high costs and time-consuming procedures, genetic tests have normally been performed late in the diagnostic process, when clinical examination and other tests have indicated a specific gene as the likely disease cause. Many neurological phenotypes are genetically very heterogeneous, and testing of all possible disease genes has been impossible. As a result, many patients with genetic neurological disorders have remained without a specific diagnosis, even when the disease is caused by mutations in known disease genes. Recent technological advances, in particular next-generation DNA sequencing techniques, have resulted in rapid identification of genes involved in Mendelian disorders and provided new possibilities for diagnostic genetic testing. The development of methods for coupling targeted capture and massively parallel DNA sequencing has made it possible to examine a large number of genes in a single reaction. Diagnostic genetic testing can today be performed by the use of gene panels and exome sequencing. This allows a more precise diagnosis of many neurological disorders, and genetic testing should now be considered earlier in the diagnostic procedure. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  12. Pilot Data Bank Networks for Neurological Disorders

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    Kunitz, Selma C.; Havekost, Charles L.; Gross, Cynthia R.

    1979-01-01

    National pilot data bank networks for stroke and traumatic coma have recently been initiated at multiple centers by the National Institute of Neurological and Communicative Disorders and Stroke. The characteristics of these pilot data bank projects include: 1) the overall development and statement of research issues by a multidisciplinary team; 2) dual emphasis on patient management and clinical research; 3) the definition and use of a uniform clinical vocabulary; 4) the use of a clinically-oriented data base management system; and 5) the use of intelligent terminals for data entry, retrieval, and patient management. This paper will describe the data bank approach used by the neurological disorders programs.

  13. Sexual function in women with neurological disorders

    OpenAIRE

    Hulter, Birgitta

    1999-01-01

    The purpose of this investigation was to study sexual function in women with neurological disorders at fairly distinct and separate locations. The dissertation comprises descriptive, retrospective, quantitative studies on sexual functioning in women with hypothalamo-pituitary disorders (HPD) (n:48), multiple sclerosis (MS)(n:47), and insulin-dependent diabetes mellitus (IDDM) (n:42). The results werecompared with those in an age-matched control group (C) (n:42), and as reported by representat...

  14. Proton MRS in neurological disorders

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    Bonavita, S.; Di Salle, F.; Tedeschi, G

    1999-05-01

    Proton magnetic resonance spectroscopy ({sup 1}H MRS) permits the acquisition of the signal arising from several brain metabolites. At long echo-time (TE) {sup 1}H MRS can detect N-acetyl-aspartate containing compounds, choline containing compounds, creatine+phosphocreatine and lactate. At short TE, lipids, tryglicerides, alanine, glutamate, glutamine, GABA, scyllo-inositol, glucose, myo-inositol, carnosine and histydine are visible. {sup 1}H MRS can be performed with single-voxel, multivoxel, single slice and multislice techniques. With single voxel {sup 1}H MRS it is possible to measure metabolites relaxation time, which allows the measurement of metabolite concentrations. This technique can be useful in the study of focal lesions in the central nervous system (CNS) such as epilepsy (pre-surgical identification of epileptic focus), brain tumors (evaluation of recurrence and radiation necrosis), stroke, multiple sclerosis, etc. Single slice and multislice {sup 1}H MRS imaging ({sup 1}H MRSI) can be performed only at long TE and permits the mapping of the brain metabolites distribution which makes them particularly useful in studying diffuse diseases and heterogeneous lesions of the CNS. {sup 1}H MRS can also be useful in the evaluation of 'ischemic penumbra' of stroke; developmental (myelin and neuronal dysgenesis); head trauma (evaluation of cerebral damage not visible with MRI); degenerative disorders (identification of microscopic pathology not visible with MRI); and metabolic diseases (metabolic disturbances with specific metabolic patterns)

  15. Sleep disorders in children with neurologic diseases.

    Science.gov (United States)

    Zucconi, M; Bruni, O

    2001-12-01

    Pediatric neurologic diseases are often associated with different kinds of sleep disruption (mainly insomnia, less frequently hypersomnia or parasomnias). Due to the key-role of sleep for development, the effort to ameliorate sleep patterns in these children could have important prognostic benefits. Study of sleep architecture and organization in neurologic disorders could lead to a better comprehension of the pathogenesis and a better treatment of the disorders. This article focuses on the following specific neurologic diseases: nocturnal frontal lobe epilepsy and abnormal motor behaviors of epileptic origin, evaluating differential diagnosis with parasomnias; achondroplasia, confirming the crucial role of craniofacial deformity in determining sleep-disordered breathing; neuromuscular diseases, mainly Duchenne's muscular dystrophy and myotonic dystrophy; cerebral palsy, evaluating either the features of sleep architecture and the importance of the respiratory problems associated; headaches, confirming the strict relationships with sleep in terms of neurochemical and neurobehavioral substrates; and finally a review on the effectiveness of melatonin for sleep problems in children with neurologic syndromes and mental retardation, blindness, and epilepsy.

  16. Acupuncture for Small Animal Neurologic Disorders.

    Science.gov (United States)

    Roynard, Patrick; Frank, Lauren; Xie, Huisheng; Fowler, Margaret

    2018-01-01

    Modern research on traditional Chinese veterinary medicine (TCVM), including herbal medicine and acupuncture, has made evident the role of the nervous system as a cornerstone in many of the mechanisms of action of TCVM. Laboratory models and clinical research available are supportive for the use of TCVM in the management of neurologic conditions in small animals, specifically in cases of intervertebral disk disease, other myelopathies, and painful conditions. This article is meant to help guide the use of TCVM for neurologic disorders in small animals, based on available information and recommendations from experienced TCVM practitioners. Copyright © 2017 Elsevier Inc. All rights reserved.

  17. Stem Cell Therapy in Pediatric Neurological Disorders

    Directory of Open Access Journals (Sweden)

    Farnaz Torabian

    2015-06-01

    Full Text Available Pediatric neurological disorders including muscular dystrophy, cerebral palsy, and spinal cord injury are defined as a heterogenous group of diseases, of which some are known to be genetic. The two significant features represented for stem cells, leading to distinguish them from other cell types are addressed as below: they can renew themselves besides the ability to differentiate into cells with special function as their potency. Researches about the role of stem cells in repair of damaged tissues in different organs like myocardium, lung, wound healing, and others are developing. In addition, the use of stem cells in the treatment and improving symptoms of neurological diseases such as autism are known. Many epigenetic and immunological studies on effects of stem cells have been performed. The action of stem cells in tissue repair is a need for further studies. The role of these cells in the secretion of hormones and growth factors in the niche, induction of cell division and differentiation in local cells and differentiation of stem cells in damaged tissue is the samples of effects of tissue repair by stem cells.Cognitive disorders, epilepsy, speech and language disorders, primary sensory dysfunction, and behavioral challenges are symptoms of non-neuromotor dysfunction in half of pediatrics with CP. Occupational therapy, oral medications, and orthopedic surgery for supportive and rehabilitative approaches are part of Conventional remedy for cerebral palsy. This paper summarizes the clinical world wide experience about stem cell based therapeutic procedures for pediatric neurological disorders.

  18. Stem cell therapy in pediatric neurological disorders

    Directory of Open Access Journals (Sweden)

    Farnaz Torabian

    2015-06-01

    Full Text Available Pediatric neurological disorders including muscular dystrophy, cerebral palsy, and spinal cord injury are defined as a heterogenous group of diseases, of which some are known to be genetic. The two significant features represented for stem cells, leading to distinguish them from other cell types are addressed as below: they can renew themselves besides the ability to differentiate into cells with special function as their potency. Researches about the role of stem cells in repair of damaged tissues in different organs like myocardium, lung, wound healing, and others are developing. In addition, the use of stem cells in the treatment and improving symptoms of neurological diseases such as autism are known. Many epigenetic and immunological studies on effects of stem cells have been performed. The action of stem cells in tissue repair is a need for further studies. The role of these cells in the secretion of hormones and growth factors in the niche, induction of cell division and differentiation in local cells and differentiation of stem cells in damaged tissue is the samples of effects of tissue repair by stem cells.Cognitive disorders, epilepsy, speech and language disorders, primary sensory dysfunction, and behavioral challenges are symptoms of non-neuromotor dysfunction in half of pediatrics with CP. Occupational therapy, oral medications, and orthopedic surgery for supportive and rehabilitative approaches are part of Conventional remedy for cerebral palsy. This paper summarizes the clinical world wide experience about stem cell based therapeutic procedures for pediatric neurological disorders.

  19. Nanotechnology based diagnostics for neurological disorders

    Energy Technology Data Exchange (ETDEWEB)

    Kurek, Nicholas S.; Chandra, Sathees B., E-mail: schandra@roosevelt.edu [Department of Biological, Chemical and Physical Sciences, Roosevelt University, Chicago, IL (United States)

    2012-07-01

    Nanotechnology involves probing and manipulating matter at the molecular level. Nanotechnology based molecular diagnostics have the potential to alleviate the suffering caused by many diseases, including neurological disorders, due to the unique properties of nanomaterials. Most neurological illnesses are multifactorial conditions and many of these are also classified as neurobehavioral disorders. Alzheimer's disease, Parkinson's disease, Huntington disease, cerebral ischemia, epilepsy, schizophrenia and autism spectrum disorders like Rett syndrome are some examples of neurological disorders that could be better treated, diagnosed, prevented and possibly cured using nanotechnology. In order to improve the quality of life for disease afflicted people, a wide range of nanomaterials that include gold and silica nanoparticles, quantum dots and DNA along with countless other forms of nanotechnology have been investigated regarding their usefulness in advancing molecular diagnostics. Other small scaled materials like viruses and proteins also have potential for use as molecular diagnostic tools. Information obtained from nanotechnology based diagnostics can be stored and manipulated using bioinformatics software. More advanced nanotechnology based diagnostic procedures for the acquisition of even greater proteomic and genomic knowledge can then be developed along with better ways to fight various diseases. Nanotechnology also has numerous applications besides those related to biotechnology and medicine. In this article, we will discuss and analyze many novel nanotechnology based diagnostic techniques at our disposal today. (author)

  20. Oral Health, Temporomandibular Disorder, and Masticatory Performance in Patients with Charcot-Marie-Tooth Type 2

    Science.gov (United States)

    Rezende, Rejane L. S.; Bonjardim, Leonardo R.; Neves, Eduardo L. A.; Santos, Lidiane C. L.; Nunes, Paula S.; Garcez, Catarina A.; Souza, Cynthia C.; Araújo, Adriano A. S.

    2013-01-01

    Background. The aim of this study was to evaluate the oral health status of temporomandibular disorders (TMD) and bruxism, as well as to measure masticatory performance of subjects with Charcot-Marie-Tooth type 2 (CMT2). Methods and Results. The average number of decayed, missing, and filled teeth (DMFT) for both groups, control (CG) and CMT2, was considered low (CG = 2.46; CMT2 = 1.85, P = 0.227). The OHIP-14 score was considered low (CG = 2.86, CMT2 = 5.83, P = 0.899). The prevalence of self-reported TMD was 33.3% and 38.9% (P = 0.718) in CG and CMT2 respectively and for self-reported bruxism was 4.8% (CG) and 22.2% (CMT2), without significant difference between groups (P = 0.162). The most common clinical sign of TMD was masseter (CG = 38.1%; CMT2 = 66.7%) and temporalis (CG = 19.0%; GCMT2 = 33.3%) muscle pain. The geometric mean diameter (GMD) was not significantly different between groups (CG = 4369; CMT2 = 4627, P = 0.157). Conclusion. We conclude that the CMT2 disease did not negatively have influence either on oral health status in the presence and severity of TMD and bruxism or on masticatory performance. PMID:24391462

  1. Oral Health, Temporomandibular Disorder, and Masticatory Performance in Patients with Charcot-Marie-Tooth Type 2

    Directory of Open Access Journals (Sweden)

    Rejane L. S. Rezende

    2013-01-01

    Full Text Available Background. The aim of this study was to evaluate the oral health status of temporomandibular disorders (TMD and bruxism, as well as to measure masticatory performance of subjects with Charcot-Marie-Tooth type 2 (CMT2. Methods and Results. The average number of decayed, missing, and filled teeth (DMFT for both groups, control (CG and CMT2, was considered low (CG = 2.46; CMT2 = 1.85, P=0.227. The OHIP-14 score was considered low (CG = 2.86, CMT2 = 5.83, P=0.899. The prevalence of self-reported TMD was 33.3% and 38.9% (P=0.718 in CG and CMT2 respectively and for self-reported bruxism was 4.8% (CG and 22.2% (CMT2, without significant difference between groups (P=0.162. The most common clinical sign of TMD was masseter (CG = 38.1%; CMT2 = 66.7% and temporalis (CG = 19.0%; GCMT2 = 33.3% muscle pain. The geometric mean diameter (GMD was not significantly different between groups (CG = 4369; CMT2 = 4627, P=0.157. Conclusion. We conclude that the CMT2 disease did not negatively have influence either on oral health status in the presence and severity of TMD and bruxism or on masticatory performance.

  2. Oral health, temporomandibular disorder, and masticatory performance in patients with Charcot-Marie-Tooth type 2.

    Science.gov (United States)

    Rezende, Rejane L S; Bonjardim, Leonardo R; Neves, Eduardo L A; Santos, Lidiane C L; Nunes, Paula S; Garcez, Catarina A; Souza, Cynthia C; Araújo, Adriano A S

    2013-01-01

    The aim of this study was to evaluate the oral health status of temporomandibular disorders (TMD) and bruxism, as well as to measure masticatory performance of subjects with Charcot-Marie-Tooth type 2 (CMT2). The average number of decayed, missing, and filled teeth (DMFT) for both groups, control (CG) and CMT2, was considered low (CG = 2.46; CMT2 = 1.85, P = 0.227). The OHIP-14 score was considered low (CG = 2.86, CMT2 = 5.83, P = 0.899). The prevalence of self-reported TMD was 33.3% and 38.9% (P = 0.718) in CG and CMT2 respectively and for self-reported bruxism was 4.8% (CG) and 22.2% (CMT2), without significant difference between groups (P = 0.162). The most common clinical sign of TMD was masseter (CG = 38.1%; CMT2 = 66.7%) and temporalis (CG = 19.0%; GCMT2 = 33.3%) muscle pain. The geometric mean diameter (GMD) was not significantly different between groups (CG = 4369; CMT2 = 4627, P = 0.157). We conclude that the CMT2 disease did not negatively have influence either on oral health status in the presence and severity of TMD and bruxism or on masticatory performance.

  3. Charcot-like joints in calcium pyrophosphate dihydrate deposition disease

    Energy Technology Data Exchange (ETDEWEB)

    Helms, C.A.; Chapman, G.S.; Wild, J.H.

    1981-10-01

    Two cases of Charcot-like joints in patients with pseudogout who were otherwise neurologically intact are presented. The arthropathy of pseudogout should include Charcot-like joints and it is emphasized that an apparent Charcot joint should raise the question of pseudogout.

  4. Neuromuscular Hip Dysplasia in Charcot-Marie-Tooth Disease Type 1A

    Science.gov (United States)

    Bamford, Nigel S.; White, Klane K.; Robinett, Stephanie A.; Otto, Randolph K.; Gospe, Sidney M., Jr.

    2009-01-01

    Charcot-Marie-Tooth disease (CMT) is one of the most common inherited neurological disorders, affecting 36 in 100,000 people. CMT type 1A (hereditary motor and sensory neuropathy) is the most frequent form of this disease, affecting 60 to 80% of the CMT population, but its diagnosis may be delayed because of inconsistent clinical signs and…

  5. Charcot arthropathy of the wrist associated with cervical spondylotic myelopathy.

    Science.gov (United States)

    Jackson, Keith; Ramadorai, Uma; Abell, Brian; Devine, John

    2012-12-01

    Background Charcot arthropathy is a cascade of destructive changes that can effect joints of both the axial and appendicular skeleton. The pathogenesis of this condition centers around the accumulation of minor traumatic events after the loss of normal joint sensation. The most frequently cited cause of Charcot arthropathy of the upper extremity is syringomyelia, and magnetic resonance imaging of the cervical spine should be obtained at presentation. Case Report A 72-year-old woman presented with a painless right wrist deformity. Radiographs demonstrated advanced destructive changes of the radiocarpal joint. Magnetic resonance imaging of the cervical spine revealed multilevel cervical spondylotic stenosis with cord deformation, but no evidence of syringomyelia. Neurological examination confirmed the presence of myelopathy. Literature Review The most frequently cited cause of Charcot arthropathy of the upper extremity is syringomyelia, although pathologies such as diabetes mellitus, tabes dorsalis, leprosy, and other disorders affecting the nervous system have been reported to lead to this condition. Neuropathic arthropathy involving the wrist is a rare phenomenon with fewer than 20 published reports in modern literature. Clinical Relevance Charcot arthropathy of the wrist is a rare but potentially disabling condition. The diagnosis of spondylotic myelopathy should be considered when evaluating a patient with this presentation. Evaluation consisting of a detailed neurological examination and advanced imaging of the cervical spine is warranted to identify the etiology.

  6. An overview of curcumin in neurological disorders.

    Science.gov (United States)

    Kulkarni, S K; Dhir, A

    2010-03-01

    Curcumin, the principal curcuminoid found in spice turmeric, has recently been studied for its active role in the treatment of various central nervous system disorders. Curcumin demonstrates neuroprotective action in Alzheimer's disease, tardive dyskinesia, major depression, epilepsy, and other related neurodegenerative and neuropsychiatric disorders. The mechanism of its neuroprotective action is not completely understood. However, it has been hypothesized to act majorly through its anti-inflammatory and antioxidant properties. Also, it is a potent inhibitor of reactive astrocyte expression and thus prevents cell death. Curcumin also modulates various neurotransmitter levels in the brain. The present review attempts to discuss some of the potential protective role of curcumin in animal models of major depression, tardive dyskinesia and diabetic neuropathy. These studies call for well planned clinical studies on curcumin for its potential use in neurological disorders.

  7. Wilson's disease and other neurological copper disorders.

    Science.gov (United States)

    Bandmann, Oliver; Weiss, Karl Heinz; Kaler, Stephen G

    2015-01-01

    The copper metabolism disorder Wilson's disease was first defined in 1912. Wilson's disease can present with hepatic and neurological deficits, including dystonia and parkinsonism. Early-onset presentations in infancy and late-onset manifestations in adults older than 70 years of age are now well recognised. Direct genetic testing for ATP7B mutations are increasingly available to confirm the clinical diagnosis of Wilson's disease, and results from biochemical and genetic prevalence studies suggest that Wilson's disease might be much more common than previously estimated. Early diagnosis of Wilson's disease is crucial to ensure that patients can be started on adequate treatment, but uncertainty remains about the best possible choice of medication. Furthermore, Wilson's disease needs to be differentiated from other conditions that also present clinically with hepatolenticular degeneration or share biochemical abnormalities with Wilson's disease, such as reduced serum ceruloplasmin concentrations. Disordered copper metabolism is also associated with other neurological conditions, including a subtype of axonal neuropathy due to ATP7A mutations and the late-onset neurodegenerative disorders Alzheimer's disease and Parkinson's disease. Copyright © 2015 Elsevier Ltd. All rights reserved.

  8. Clinical management of acute diabetic Charcot foot in Denmark

    DEFF Research Database (Denmark)

    Jansen, Rasmus Bo; Svendsen, Ole Lander; Kirketerp-Møller, Klaus

    2016-01-01

    INTRODUCTION: Charcot foot is a severe complication to diabetes mellitus and treatment involves several different clinical specialities. Our objective was to describe the current awareness, knowledge and treatment practices of Charcot foot among doctors who handle diabetic foot disorders. METHODS...

  9. Pattern Of Neurological Disorders In Child Neurology Clinic Of The ...

    African Journals Online (AJOL)

    Other disorders seen include acute flaccid paralysis, autism, developmental delay, headache, mental retardation, HIV encephalopathy, cranial neuropathy, Erb Duchene paralysis, deafness, cerebella ataxia, conversion disorder, polyneuritis, syncope, Tic disorder and tuberous sclerosis. Concerted effort at the training of ...

  10. [Neurological soft signs in pervasive developmental disorders].

    Science.gov (United States)

    Halayem, S; Bouden, A; Halayem, M B; Tabbane, K; Amado, I; Krebs, M O

    2010-09-01

    Many studies have focused on specific motor signs in autism and Asperger's syndrome, but few has been published on the complete range of neurological soft signs (NSS) in children with pervasive developmental disorder (PDD). Scarce are the studies evaluating NSS in children suffering from PDD not otherwise specified (PDDNOS). This study compared performance of 11 autistic children (AD) and 10 children with PDDNOS, with controls matched on age, sex and cognitive performance on Krebs et al.'s NSS scale. Because of the duration of the assessments and specific difficulties encountered in managing some items, an adaptation of the scale had to be made during a pilot study with the agreement of the author. To be eligible, patients had to meet the following inclusion criteria: an age range of 6-16 years, a diagnosis of autistic disorder or PDDNOS based on the DSM IV criteria (American Psychiatric Association 1994). The autism diagnostic interview-revised (ADI-R) was used in order to confirm the diagnosis and to evaluate the association of the symptoms to the severity of the NSS. The childhood autism rating scale (CARS) was completed for the patients in order to evaluate symptoms at the time of the NSS examination. Cognitive ability was assessed with Raven's progressive matrices. Were excluded patients with: history of cerebral palsy, congenital anomaly of the central nervous system, epilepsy, known genetic syndrome, tuberous sclerosis, neurofibromatosis, antecedent of severe head trauma, Asperger's syndrome, obvious physical deformities or sensory deficits that would interfere with neurological assessment, deep mental retardation and recent or chronic substance use or abuse. Healthy controls shared the same exclusion criteria, with no personal history of neurological, psychiatric disorder or substance abuse, no family history of psychiatric disorder and normal or retardation in schooling. All study procedures were approved by the local Ethics Committee (Comité d

  11. [Neurological disorders in the narrative works of Benito Pérez Galdós].

    Science.gov (United States)

    Alvaro, L C; Martín del Burgo, A

    2007-06-01

    Benito Pérez Galdós was a realistic writer. As such, he was devoted to displaying reality in its full complexity. He shared the traits of experimental medicine of Claude Bernard (Introduction à l'étude de la médecine expérimentale). The writer was a close friend of famous contemporary doctors (Gregorio Marañón, Manuel Tolosa Latour), had textbooks with neurological content, and was familiar with Charcot. With this background, we have foreseen neurological descriptions in his works. To search for them, we have reviewed three paramount novels: Fortunata y Jacinta, La de Bringas and Tormento. We found: a) headache, usually migraine with and without aura; common precipitants and inheritance are present. The novelist himself suffered from severe migraine; b) movement disorders: Parkinson syndrome and hemifacial spasm; c) convulsive and non-convulsive epilepsy in a girl as well as syncope and psychogenic loss of consciousness; d) congenital syphilis, based on a typical physical appearance in a character with madness (paranoid schizophrenia); the reason was probably that syphilis was regarded as the most common cause of "dementia praecox"; e) alcoholism: acute intoxication, deprivation, behavior disorders, hepatic encephalopathy and a likely pellagra; f) sleep disorders: parasomnias (somnambulism, somniloquy) and sleep paralysis, and g) stroke and also inverted metamorphopsia of psychogenic origin. Ailments and disease pervaded life in the 19th century. Neurological disorders were highly prevalent and are fully integrated into Galdos realistic works. Many of them fulfill criteria of disorders contemplated according to degeneration theories. Nevertheless, humanitarian features raise the characters above their tragic destiny. Biographical factors, particularly his many love affairs and his political implication as a liberal, could have contributed to the plethora of precise descriptions.

  12. Wilson's disease and other neurological copper disorders.

    Science.gov (United States)

    Bandmann, Oliver; Weiss, Karl Heinz; Kaler, Stephen G.

    2015-01-01

    Summary The classic copper metabolism disorder, Wilson disease (WD), was first defined in 1912. Both early onset presentations in infancy and late onset manifestations in adults > 70 years are now well recognized. Modern biochemical and genetic prevalence studies suggest that WD may be considerably more common than previously appreciated. Early diagnosis of WD is crucial to ensure that patients can be started on adequate treatment but uncertainty remains about the best possible choice of medication. Direct genetic testing for ATP7B mutations is increasingly available to confirm the clinical diagnosis of WD. WD needs to be differentiated from other conditions that present clinically with hepatolenticular degeneration or share biochemical abnormalities with WD, such as reduced serum cerulo plasmin levels. Disordered copper metabolism is also implied in an increasing number of other neurological conditions, including a subtype of axonal neuropathy due to ATP7A mutations, and the common late-onset neurodegenerative disorders Alzheimer’s disease and Parkinson’s disease. PMID:25496901

  13. Isoprenoid Pathway And Neurological And Psychiatric Disorders

    Directory of Open Access Journals (Sweden)

    Ravikumar A

    1999-01-01

    Full Text Available The coexistence of neuronal degeneration, psychiatric manifestation, immune activation and malignant transformation has been documented in literature, suggesting a central dysfunction in the pathophysiology of these disorders. The isoprenoid pathway may be candidate in this respect, in view of the changes in the concentration of some products of this pathway in many of these disorders, however, no detailed study has been carried out in this respect. In view of this, a study was undertaken on the isoprenoid pathway in some of these disorders - primary generalized epilepsy, Parkinson’s disease (PD, schizophrenia, manic depressive psychosis (MDP, CNS glioma, multiple sclerosis, subacute sclerosing panencephalitis (SSPEand a familial group with familial coexistence of schizophrenia, PD, primary generalized epilepsy, malignant neoplasia, rheumatoid arthritis and syndrome-X over three generations. The following parameters were studied in the patients of these disorders as compared to age and sex matched control subjects - ubiquinone dolichol, digoxin, activity of HMG CoA reductase in the plasma and erthyorcyte membrane Na -K--ATpase. Increase in the activity of HMG CoA reductase and in the concentration of plasma digoxin and dolichol was observed in most of these cases. On the other hand, there was decrease in the concentration of plasma ubiquinone. Decrease in the activity of erythrocyte membrane Na-K- ATpase activity for which digoxin is an inhibitor was also observed in all the cases studied. These results indicate an upregulation of the isoprenoid pathway in the neurological and psychiatric disorders studied. The implications of this change is discussed in details.

  14. Bridging Neuroanatomy, Neuroradiology and Neurology: Three-Dimensional Interactive Atlas of Neurological Disorders

    OpenAIRE

    Nowinski, W. L.; Chua, B.C.

    2013-01-01

    Understanding brain pathology along with the underlying neuroanatomy and the resulting neurological deficits is of vital importance in medical education and clinical practice. To facilitate and expedite this understanding, we created a three-dimensional (3D) interactive atlas of neurological disorders providing the correspondence between a brain lesion and the resulting disorder(s). The atlas contains a 3D highly parcellated atlas of normal neuroanatomy along with a brain pathology database. ...

  15. Staging neurological disorders: expressions of cognitive and motor disorder.

    Science.gov (United States)

    Archer, Trevor; Kostrzewa, Richard M

    2010-08-01

    In neurologic disorders, there are progressive losses in regional brain structural integrity, circuitry, and neuronal process that threaten individuals' ability to express functional capacity at several levels of severity. The classification of (a) patients on the basis of diagnosis, risk prognosis, and intervention outcome forms the basis of clinical staging and (b) laboratory animals on the basis of animal model of brain disorder, extent of insult and dysfunctional expression, provides the components for the clinical staging and preclinical staging, respectively, of the disease state with certain associated epidemiological, biological, and genetic characteristics. The investigation of epigenetics and biomarkers is intrinsic to any analysis of the progressive nature of the neurogenerative disorders, in the present account disorders relating to Alzheimer's disease, Parkinson's disease, depression, and diabetes.

  16. Charcot spinal arthropathy in a diabetic patient.

    Science.gov (United States)

    van Eeckhoudt, S; Minet, M; Lecouvet, F; Galant, C; Banse, X; Lambert, M; Lefèbvre, C

    2014-08-01

    We report a case of Charcot spinal arthropathy in a diabetic patient and emphasize the clinical reasoning leading to the diagnosis, discuss the differential diagnosis, and insist on the crucial role of the radiologist and pathologist which allows the distinction between Charcot spinal arthropathy and infectious or tumoural disorders of the spine.

  17. Clinical perspectives on medical marijuana (cannabis) for neurologic disorders

    OpenAIRE

    Fife, Terry D.; Moawad, Heidi; Moschonas, Constantine; Shepard, Katie; Hammond, Nancy

    2015-01-01

    The American Academy of Neurology published an evidence-based systematic review of randomized controlled trials using marijuana (Cannabis sativa) or cannabinoids in neurologic disorders. Several cannabinoids showed effectiveness or probable effectiveness for spasticity, central pain, and painful spasms in multiple sclerosis. The review justifies insurance coverage for dronabinol and nabilone for these indications. Many insurance companies already cover these medications for other indications....

  18. Autism spectrum symptoms in children with neurological disorders

    Directory of Open Access Journals (Sweden)

    Ryland Hilde K

    2012-11-01

    Full Text Available Abstract Background The aims of the present study were to assess symptoms associated with an autism spectrum disorder (ASD in children with neurological disorders as reported by parents and teachers on the Autism Spectrum Screening Questionnaire (ASSQ, as well as the level of agreement between informants for each child. Methods The ASSQ was completed by parents and teachers of the 5781 children (11–13 years who participated in the second wave of the Bergen Child Study (BCS, an on-going longitudinal population-based study. Out of these children, 496 were reported to have a chronic illness, including 99 whom had a neurological disorder. The neurological disorder group included children both with and without intellectual disabilities. Results Children with neurological disorders obtained significantly higher parent and teacher reported ASSQ scores than did non-chronically ill children and those with other chronic illnesses (p Conclusions The ASSQ identifies a high rate of ASD symptoms in children with neurological disorders, and a large number of children screened in the positive range for ASD. Although a firm conclusion awaits further clinical studies, the present results suggest that health care professionals should be aware of potential ASD related problems in children with neurological disorders, and should consider inclusion of the ASSQ or similar screening instruments as part of their routine assessment of this group of children.

  19. [Charcot's arthropathy

    DEFF Research Database (Denmark)

    Christensen, T.M.; Yderstraede, K.; Ejskjaer, N.

    2008-01-01

    Charcot's arthropathy is a rare complication to diabetes with peripheral neuropathy. The diagnosis is based on a red, oedematous foot with 2 degrees C difference in skin temperature between the affected foot compared to the unaffected foot. The condition is characterised by fractures, dislocation...... of joints and deformity. The treatment is off-loading. When the off-loading treatment is completed, the patient should gradually adjust to wearing bespoken shoes. Lifelong control of the feet and shoes is necessary Udgivelsesdato: 2008/8/11...

  20. Aquatic rehabilitation for the treatment of neurological disorders.

    Science.gov (United States)

    Morris, D M

    1994-01-01

    Patients with neurological disorders present therapists with complex challenges for treatment, including weakness, hypertonicity, voluntary movement deficit, limited range of motion, sensory loss, incoordination, and postural instability. The presence of one or more of these impairments negatively influences these patients by contributing to problems in walking, transferring, and reaching. Aquatic rehabilitation offers a unique, versatile approach to the treatment of these disabilities. This article examines the problems encountered by patients with neurological disorders, general principles guiding neurotreatment, and aquatic neurorehabilitation approaches.

  1. Hypnosis as a model of functional neurologic disorders.

    Science.gov (United States)

    Deeley, Q

    2016-01-01

    In the 19th century it was recognized that neurologic symptoms could be caused by "morbid ideation" as well as organic lesions. The subsequent observation that hysteric (now called "functional") symptoms could be produced and removed by hypnotic suggestion led Charcot to hypothesize that suggestion mediated the effects of ideas on hysteric symptoms through as yet unknown effects on brain activity. The advent of neuroimaging 100 years later revealed strikingly similar neural correlates in experiments matching functional symptoms with clinical analogs created by suggestion. Integrative models of suggested and functional symptoms regard these alterations in brain function as the endpoint of a broader set of changes in information processing due to suggestion. These accounts consider that suggestions alter experience by mobilizing representations from memory systems, and altering causal attributions, during preconscious processing which alters the content of what is provided to our highly edited subjective version of the world. Hypnosis as a model for functional symptoms draws attention to how radical alterations in experience and behavior can conform to the content of mental representations through effects on cognition and brain function. Experimental study of functional symptoms and their suggested counterparts in hypnosis reveals the distinct and shared processes through which this can occur. © 2016 Elsevier B.V. All rights reserved.

  2. Charcot-Marie-Tooth Disease

    Science.gov (United States)

    Charcot-Marie-Tooth disease (CMT) is a group of genetic nerve disorders. It is named after the three doctors who first identified it. ... a nerve biopsy. There is no cure. The disease can be so mild you don't realize ...

  3. Progress in gene therapy for neurological disorders.

    Science.gov (United States)

    Simonato, Michele; Bennett, Jean; Boulis, Nicholas M; Castro, Maria G; Fink, David J; Goins, William F; Gray, Steven J; Lowenstein, Pedro R; Vandenberghe, Luk H; Wilson, Thomas J; Wolfe, John H; Glorioso, Joseph C

    2013-05-01

    Diseases of the nervous system have devastating effects and are widely distributed among the population, being especially prevalent in the elderly. These diseases are often caused by inherited genetic mutations that result in abnormal nervous system development, neurodegeneration, or impaired neuronal function. Other causes of neurological diseases include genetic and epigenetic changes induced by environmental insults, injury, disease-related events or inflammatory processes. Standard medical and surgical practice has not proved effective in curing or treating these diseases, and appropriate pharmaceuticals do not exist or are insufficient to slow disease progression. Gene therapy is emerging as a powerful approach with potential to treat and even cure some of the most common diseases of the nervous system. Gene therapy for neurological diseases has been made possible through progress in understanding the underlying disease mechanisms, particularly those involving sensory neurons, and also by improvement of gene vector design, therapeutic gene selection, and methods of delivery. Progress in the field has renewed our optimism for gene therapy as a treatment modality that can be used by neurologists, ophthalmologists and neurosurgeons. In this Review, we describe the promising gene therapy strategies that have the potential to treat patients with neurological diseases and discuss prospects for future development of gene therapy.

  4. Neurological soft signs discriminating mood disorders from first episode schizophrenia

    NARCIS (Netherlands)

    Boks, MPM; Liddle, PF; Burgerhof, JGM; Knegtering, R; Bosch, RJ

    Objective: To investigate the specificity of neurological soft signs (NSS) for first episode schizophrenia compared with mood disorders. Method: We assessed NSS in a sample of 60 healthy controls, 191 first episode psychosis patients and 81 mood disorder patients. We used a principle component

  5. NEUROLOGICAL DISORDERS IN PATIENTS WITH HYPERTENSION AND THEIR CORRECTION

    Directory of Open Access Journals (Sweden)

    N. V. Vakhnina

    2016-01-01

    Full Text Available Neurological disorders in hypertensive patients can be caused by both brain injury and concomitant diseases. The elucidation of the causes of neurological disorders and their effective treatment contribute to hypertensive patients’ better adherence to long-term antihypertensive therapy, which normalizes blood pressure (BP and reduces the risk of cerebral complications Objective: to study of the causes of neurological disorders in hypertensive patients and the efficiency of their correction using a new dispersible vinpocetine formulation (Cavinton® Comforte in combined therapy.Patients and methods. A total of 80 patients (men (20% and women (80%; mean age 63±12.3  years with neurological complaints in the presence of hypertension were examined. All the patients were diagnosed with dyscirculatory encephalopathy or chronic brain ischemia, whether they had vascular cognitive impairment. The examination of patients revealed that the neurological complaints were mainly due to concomitant diseases, such as migraine (12%, tension-type headache (66%, and the latter concurrent with migraine (4%.Results and  discussion. The  effective treatment of concomitant diseases in  combination with antihypertensive therapy contributed to normalization of BP and regression of complaints. The most pronounced effect was noted in 40 patients whose combination therapy included Vinpocetine (Cavinton® Comforte 10 mg thrice daily.Conclusion. The therapy resulted in the less severity of both the symptoms of cerebrovascular disease (vascular cognitive impairment and comorbid neurological disorders (headache, dizziness, etc..

  6. [Can music therapy for patients with neurological disorders?].

    Science.gov (United States)

    Myskja, Audun

    2004-12-16

    Recent developments in brain research and in the field of music therapy have led to the development of music-based methods specifically aimed at relieving symptoms of Parkinson's disease and other neurologic disorders. Rhythmic auditory stimulation uses external rhythmic auditory cues from song, music or metronome to aid patients improving their walking functioning and has been shown to be effective both within sessions and as a result of training over time. Melodic intonation therapy and related vocal techniques can improve expressive dysphasia and aid rehabilitation of neurologic disorders, particularly Parkinson's disease, stroke and developmental disorders.

  7. Hypnosis as therapy for functional neurologic disorders.

    Science.gov (United States)

    Deeley, Q

    2016-01-01

    Suggestion in hypnosis has been applied to the treatment of functional neurologic symptoms since the earliest descriptions of hypnosis in the 19th century. Suggestion in this sense refers to an intentional communication of beliefs or ideas, whether verbally or nonverbally, to produce subjectively convincing changes in experience and behavior. The recognition of suggestion as a psychologic process with therapeutic applications was closely linked to the derivation of hypnosis from earlier healing practices. Animal magnetism, the immediate precursor of hypnosis, arrived at a psychologic concept of suggestion along with other ideas and practices which were then incorporated into hypnosis. Before then, other forms of magnetism and ritual healing practices such as exorcism involved unintentionally suggestive verbal and nonverbal stimuli. We consider the derivation of hypnosis from these practices not only to illustrate the range of suggestive processes, but also the consistency with which suggestion has been applied to the production and removal of dissociative and functional neurologic symptoms over many centuries. Nineteenth-century practitioners treated functional symptoms with induction of hypnosis per se; imperative suggestions, or commands for specific effects; "medical clairvoyance" in hypnotic trance, in which patients diagnosed their own condition and predicted the time and manner of their recovery; and suggestion without prior hypnosis, known as "fascination" or "psychotherapeutics." Modern treatments largely involve different types of imperative suggestion with or without hypnosis. However, the therapeutic application of suggestion in hypnosis to functional and other symptoms waned in the first half of the 20th century under the separate pressures of behaviorism and psychoanalysis. In recent decades suggestion in hypnosis has been more widely applied to treating functional neurologic symptoms. Suggestion is typically applied within the context of other

  8. Uroflowmetry in neurologically normal children with voiding disorders

    DEFF Research Database (Denmark)

    Jensen, K M; Nielsen, K.K.; Kristensen, E S

    1985-01-01

    of neurological deficits underwent a complete diagnostic program including intravenous urography, voiding cystography and cystoscopy as well as spontaneous uroflowmetry, cystometry-emg and pressure-flow-emg study. The incidence of dyssynergia was 22%. However, neither the flow curve pattern nor single flow...... variables were able to identify children with dyssynergia. Consequently uroflowmetry seems inefficient in the screening for dyssynergia in neurological normal children with voiding disorders in the absence of anatomical bladder outlet obstruction....

  9. Nanotechnology Based Treatments for Neurological Disorders from Genetics Perspective

    OpenAIRE

    Nicholas S. Kurek; Sathees B. Chandra

    2013-01-01

    Nanotechology involves the application, analysis and manipulation of nanomaterials. These materials have unique and medically useful properties due to their nanoscale parameters. Nanotechnology based treatments and diagnostics might eventually bring great relief to people suffering from neurological disorders including autism spectrum disorders, Alzheimer’s disease and Parkinson’s disorders. A large variety of nonmaterials such as viruses, carbon nanotubes, gold and silica nanoparti...

  10. An Overview of Curcumin in Neurological Disorders

    OpenAIRE

    Kulkarni S; Dhir A

    2010-01-01

    Curcumin, the principal curcuminoid found in spice turmeric, has recently been studied for its active role in the treatment of various central nervous system disorders. Curcumin demonstrates neuroprotective action in Alzheimer′s disease, tardive dyskinesia, major depression, epilepsy, and other related neurodegenerative and neuropsychiatric disorders. The mechanism of its neuroprotective action is not completely understood. However, it has been hypothesized to act majorly through its a...

  11. Zolpidem for the Treatment of Neurologic Disorders: A Systematic Review.

    Science.gov (United States)

    Bomalaski, Martin N; Claflin, Edward S; Townsend, Whitney; Peterson, Mark D

    2017-09-01

    Given its selective action on the ω1 subtype of the γ-aminobutyric acid A receptor, zolpidem tartrate presents a potential treatment mechanism for other neurologic disorders. To synthesize studies that used zolpidem to treat neurologic disorders. Eligibility criteria included any published English-language article that examined the use of zolpidem for noninsomnia neurologic disorders in humans for all dates up to March 20, 2015. Searched databases included PubMed, Scopus, Web of Science Core Collection, the Cochrane Library, EMBASE, CENTRAL, and clinicaltrials.gov. Publication bias was mitigated by searching clinicaltrials.gov for unpublished studies. Two rounds of screening were performed based on title and then abstract, and coding was performed by 2 coders. All methods followed the PRISMA Reporting Guidelines for systematic reviews of the literature. The initial search produced 2314 articles after removing duplicates. After exclusion based on a review of abstracts, 67 articles remained for full manuscript review. Thirty-one studies treated movement disorders, 22 treated disorders of consciousness, and 14 treated other neurologic conditions, including stroke, traumatic brain injury, encephalopathy, and dementia. Study designs included case reports (n = 28), case series (n = 8), single-patient interventional (n = 13), pretest and posttest (n = 9), randomized clinical trials (n = 9), and crossover studies (n = 5). Only 11 studies had more than 10 participants. Effects of zolpidem were wide ranging (eg, improvement on the JFK Coma Recovery Scale-Revised, the Unified Parkinson Disease Rating Scale, and the Burke-Fahn-Marsden Dystonia Rating Scale) and generally lasted 1 to 4 hours before the participant returned to baseline. Sedation was the most common adverse effect. Zolpidem has been observed to transiently treat a large variety of neurologic disorders, most often related to movement disorders and disorders of consciousness. Much of what

  12. An overview of the Charcot foot pathophysiology

    Directory of Open Access Journals (Sweden)

    Gökhan Kaynak

    2013-08-01

    Full Text Available Charcot arthropathy of the foot is a rare but devastating complication of diabetes that remains to be a challenging issue for the foot and ankle surgeons. Charcot foot fails to be an obvious diagnostic option that comes to mind, even in a pathognomonic clinical appearance. The rarity of the disorder, more common pathologies that mimic the condition, and the self-limiting prognosis deviate the clinician from the right diagnosis. The clinical challenges in the diagnosis of Charcot foot require in-depth investigations of its enigmatic nature to establish useful guidelines. Yet, this goal seems to be beyond reach, without a holistic view of the immense literature concerning the pathophysiology of the disorder. The primary objective of this article is to put together and review the recent advancements about the etiology and intrinsic mechanisms of diabetic Charcot foot.

  13. An overview of the Charcot foot pathophysiology

    Science.gov (United States)

    Kaynak, Gökhan; Birsel, Olgar; Güven, Mehmet Fatih; Öğüt, Tahir

    2013-01-01

    Charcot arthropathy of the foot is a rare but devastating complication of diabetes that remains to be a challenging issue for the foot and ankle surgeons. Charcot foot fails to be an obvious diagnostic option that comes to mind, even in a pathognomonic clinical appearance. The rarity of the disorder, more common pathologies that mimic the condition, and the self-limiting prognosis deviate the clinician from the right diagnosis. The clinical challenges in the diagnosis of Charcot foot require in-depth investigations of its enigmatic nature to establish useful guidelines. Yet, this goal seems to be beyond reach, without a holistic view of the immense literature concerning the pathophysiology of the disorder. The primary objective of this article is to put together and review the recent advancements about the etiology and intrinsic mechanisms of diabetic Charcot foot. PMID:23919113

  14. Herbal Medicines In The Treatment of Psychiatric and Neurological Disorders

    Directory of Open Access Journals (Sweden)

    Shahin Akhondzadeh

    2006-04-01

    Full Text Available Objective: This review will indicate the quality of the evidence supporting the clinical effects of a number of commonly used types of herbal medicines for psychiatric and neurological disorders. Method: We conducted a review of literature to understand the biochemical and evidential bases for the use of herbs in psychiatric and neurological disorders as follow: 1 Alzheimer’s disease, 2 Depression, 3 Anxiety, 4 Insomnia, 5 Substance use disorders, 6 Attention deficit/hyperactivity disorder (ADHD, 7 Migraine. Results: Evidences support use of Ginkgo biloba, Huperzine A, Galantamine, Melissa officinalis,and Salvia officinalis for Alzheimer’s disease; St. John’s wort, Lavender, and Saffron for depression; Passionflower, and Kava, for anxiety disorders; Valerian, and English Lavender for sleep disorders; Hypericum for substance related disorders; Ginkgo biloba, and Passionflower for ADHD; and feverfew, and Butterbur root for migraine. The highest level of confidence derives from well-designed, randomized, double blind controlled studies. Conclusion: Herbs may have beneficial effects in variety of psychiatric and neurological disorder; however we must consider their potential side effects and drug-drug interactions.

  15. Hypocretin/orexin disturbances in neurological disorders.

    NARCIS (Netherlands)

    Fronczek, R.; Baumann, C.R.; Lammers, G.J.; Bassetti, C.L.; Overeem, S.

    2009-01-01

    The hypothalamic hypocretin (orexin) system plays a crucial role in the regulation of sleep and wakefulness. The strongest evidence for this is the fact that the primary sleep disorder narcolepsy is caused by disrupted hypocretin signaling in humans as well as various animal models. There is a

  16. Therapeutic potential of fluoxetine in neurological disorders

    NARCIS (Netherlands)

    Mostert, Jop P.; Koch, Marcus W.; Heerings, Marco; Heersema, Dorothea J.; De Keyser, Jacques

    2008-01-01

    The selective serotonin reuptake inhibitor (SSRI) fluoxetine, which is registered for a variety of psychiatric disorders, has been found to stimulate the cAMP-responsive element binding protein (CREB), increase the production of brain-derived neurotrophic factor (BNDF) and the neurotrophic peptide

  17. Multiple roles of metalloproteinases in neurological disorders.

    Science.gov (United States)

    Yang, Yi; Hill, Jeff W; Rosenberg, Gary A

    2011-01-01

    Once thought to mainly act in brain to remodel the extracellular matrix, the family of metalloproteinases is important in many normal and pathological processes in the nervous system. Matrix metalloproteinases (MMPs) and A disintegrin and metalloproteinases (ADAMs) are the two major families of metalloproteinases in the brain. MMPs are comprised of several related enzymes that act on extracellular molecules. Normally, they are important in angiogenesis and neurogenesis in development. In neuroinflammatory illnesses, they disrupt the basal lamina and tight junction proteins to open the blood-brain barrier (BBB). ADAMs are important in neuroinflammation through activation of tumor necrosis factor-α (TNF-α) and their action as secretases that modulate the action of receptors on the cell surface. Four tissue inhibitors of metalloproteinases (TIMPs) are the main inhibitors of the MMPs and ADAMs. Recently, MMPs were found to affect DNA repair processes by an unexpected intranuclear action. MMPs and ADAMs have been implicated in the pathophysiology of neurodegenerative diseases such as Alzheimer's disease and vascular cognitive impairment. Growing literature on the functions of MMPs and ADAMs in the central nervous system is opening up new and exciting areas of research that may lead to novel approaches to treatment of neurological diseases. Copyright © 2011 Elsevier Inc. All rights reserved.

  18. Urea cycle disorders: brain MRI and neurological outcome

    Energy Technology Data Exchange (ETDEWEB)

    Bireley, William R. [University of Colorado, Department of Radiology, Aurora, CO (United States); Van Hove, Johan L.K. [University of Colorado, Department of Genetics and Inherited Metabolic Diseases, Aurora, CO (United States); Gallagher, Renata C. [Children' s Hospital Colorado, Department of Genetics and Inherited Metabolic Diseases, Aurora, CO (United States); Fenton, Laura Z. [Children' s Hospital Colorado, Department of Pediatric Radiology, Aurora, CO (United States)

    2012-04-15

    Urea cycle disorders encompass several enzyme deficiencies that can result in cerebral damage, with a wide clinical spectrum from asymptomatic to severe. The goal of this study was to correlate brain MRI abnormalities in urea cycle disorders with clinical neurological sequelae to evaluate whether MRI abnormalities can assist in guiding difficult treatment decisions. We performed a retrospective chart review of patients with urea cycle disorders and symptomatic hyperammonemia. Brain MRI images were reviewed for abnormalities that correlated with severity of clinical neurological sequelae. Our case series comprises six urea cycle disorder patients, five with ornithine transcarbamylase deficiency and one with citrullinemia type 1. The observed trend in distribution of brain MRI abnormalities as the severity of neurological sequelae increased was the peri-insular region first, extending into the frontal, parietal, temporal and, finally, the occipital lobes. There was thalamic restricted diffusion in three children with prolonged hyperammonemia. Prior to death, this site is typically reported to be spared in urea cycle disorders. The pattern and extent of brain MRI abnormalities correlate with clinical neurological outcome in our case series. This suggests that brain MRI abnormalities may assist in determining prognosis and helping clinicians with subsequent treatment decisions. (orig.)

  19. [Charcot in Rayer's wake].

    Science.gov (United States)

    Théodoridès, J

    1994-01-01

    Charcot occupies an important place among Rayer's disciples although they did not publish any work together. They met for the first time in 1848 and later at the Société de Biologie. From 1853 to 1855 Charcot was Piorry's chef de Clinique" near Rayer's department at the Charité hospital. In 1860 Charcot was nominated "professeur agrégé with Rayer's backing. Several publications of Charcot with other disciples of Rayer (Ch. Robin, C. Davaine) are recalled as well as his paper, signed alone in 1858, on chronical arterial ischemia (Syndrome of Bouley and Charcot). An unpublished letter from Rayer recommending Charcot to the librarian of the Institut de France (1859) as well as a partial and undated one from Charcot back from England to Rayer are published here.

  20. Managing patients with neurologic disorders who participate in sports activities.

    Science.gov (United States)

    Crutchfield, Kevin E

    2014-12-01

    Patients with neurologic conditions have been discouraged from participating in organized sports because of theoretical detrimental effects of these activities to their underlying conditions. The purpose of this article is to review known risks associated with three specific clinical conditions most commonly encountered in a sports neurology clinic (epilepsy, migraines, and multiple sclerosis and to add to the neurologist's toolkit suggested interventions regarding management of athletes with these disorders. Increased participation in sports and athletics has positive benefits for patients with neurologic conditions and can be safely integrated into the lives of these patients with proper supervision from their treating neurologists. Patients with neurologic conditions can and should be encouraged to participate in organized sports as a method of maintaining their overall fitness, improving their overall level of function, and reaping the physical and psychological benefits that athletic competition has to offer.

  1. Neurological disorders in children and adolescents.

    Science.gov (United States)

    Banerjee, Tapas K; Hazra, Avijit; Biswas, Atanu; Ray, Jayanta; Roy, Trishit; Raut, Deepak K; Chaudhuri, Arijit; Das, Shyamal K

    2009-02-01

    To ascertain the prevalence of active epilepsy, febrile seizures (FS), cerebral palsy (CP) and tic disorders (TD) in aged 19 years or less. This was a cross-sectional observational study conducted as a two-stage door-to-door survey of a stratified randomly selected population in 2003-04. Trained field workers screened the population followed by case examination by the field neurologist. A total of 16979 (male 8898, female 8081) subjects aged Indian studies. Birth anoxia is a common cause of CP and educational underachievement is frequent.

  2. Telerehabilitation, Virtual Therapists, and Acquired Neurologic Speech and Language Disorders

    OpenAIRE

    Cherney, Leora R.; van Vuuren, Sarel

    2012-01-01

    Telerehabilitation (telererehab) offers cost effective services that potentially can improve access to care for those with acquired neurologic communication disorders. However, regulatory issues including licensure, reimbursement, and threats to privacy and confidentiality hinder the routine implementation of telerehab services into the clinical setting. Despite these barriers, rapid technological advances and a growing body of research regarding the use of telerehab applications support its ...

  3. Secondary Abnormalities of Neurotransmitters in Infants with Neurological Disorders

    Science.gov (United States)

    Garcia-Cazorla, A.; Serrano, M.; Perez-Duenas, B.; Gonzalez, V.; Ormazabal, A.; Pineda, M.; Fernandez-Alvarez, E.; Campistol, J. M. D.; Artuch, R. M. D.

    2007-01-01

    Neurotransmitters are essential in young children for differentiation and neuronal growth of the developing nervous system. We aimed to identify possible factors related to secondary neurotransmitter abnormalities in pediatric patients with neurological disorders. We analyzed cerebrospinal fluid (CSF) and biogenic amine metabolites in 56 infants…

  4. Neurological disorders in rural Africa - a systematic approach ...

    African Journals Online (AJOL)

    The aims of our study were to assess the hospital-based prevalence of neurological disorders in a rural African setting and to describe the pattern of disease by using a systematic approach. Methods The study was conducted at the Haydom Lutheran Hospital in northern Tanzania, Manyara region. Over a period of eight ...

  5. Minor neurological dysfunction in children with autism spectrum disorder

    NARCIS (Netherlands)

    De Jong, Marianne; Punt, Marja; De Groot, Erik; Minderaa, Ruud B.; Hadders-Algra, Mijna

    Aim The aim of this study was to improve the understanding of brain function in children with autism spectrum disorder (ASD) in relation to minor neurological dysfunctions (MNDs). Method We studied MNDs in 122 children (93 males, 29 females; mean age 8y 1mo, SD 2y 6mo) who, among a total cohort of

  6. Disruptive technology disorder: A past, present, and future neurologic syndrome.

    Science.gov (United States)

    Weaver, Donald F

    2017-07-25

    Based upon an analysis of 6 major historical technological advances over the last 150 years, a new syndrome, disruptive technology disorder (DTD), is introduced. DTD describes the human health ailments that accompany the implementation of disruptive technologies. Elevator sickness, railway spine, and bicycle face are representative examples. Though the underlying causative disruptive technologies may differ, many neurologic symptoms (headache, dizziness, weakness) are common to multiple DTDs. Born of technology-driven societal change, DTDs manifest as a complex interplay between biological and psychological symptoms. © 2017 American Academy of Neurology.

  7. [Brain-machine interface (BMI) - application to neurological disorders].

    Science.gov (United States)

    Yoshimine, Toshiki; Yanagisawa, Takufumi; Hirata, Masayuki

    2013-01-01

    Brain-machine interface (BMI) is a new technology to receive input from the brain which is translated to operate a computer or other external device in real time. After significant progress during the recent 10 years, this technology is now very close to the clinical use to restore neural functions of patients with severe neurologic impairment. This technology is also a strong tool to investigate the mode of neuro-signal processing in the brain and to understand the mechanism of neural dysfunction which leads to the development of novel neurotechnology for the treatment of various sorts of neurological disorders.

  8. Neurologic disorders associated with weight lifting and bodybuilding.

    Science.gov (United States)

    Busche, Kevin

    2009-02-01

    Weight lifting and other forms of strength training are becoming more common because of an increased awareness of the need to maintain individual physical fitness. Emergency room data indicate that injuries caused by weight training have become more universal over time, likely because of increased participation rates. Neurologic injuries can result from weight lifting and related practices. Although predominantly peripheral nervous system injuries have been described, central nervous system disease may also occur. This article illustrates the types of neurologic disorders associated with weight lifting.

  9. Charcot-Marie-Tooth disease.

    LENUS (Irish Health Repository)

    Reilly, Mary M

    2011-03-01

    Charcot-Marie-Tooth (CMT) disease is the commonest inherited neuromuscular disorder affecting at least 1 in 2,500. Over the last two decades, there have been rapid advances in understanding the molecular basis for many forms of CMT with more than 30 causative genes now described. This has made obtaining an accurate genetic diagnosis possible but at times challenging for clinicians. This review aims to provide a simple, pragmatic approach to diagnosing CMT from a clinician\\'s perspective.

  10. Clinical perspectives on medical marijuana (cannabis) for neurologic disorders.

    Science.gov (United States)

    Fife, Terry D; Moawad, Heidi; Moschonas, Constantine; Shepard, Katie; Hammond, Nancy

    2015-08-01

    The American Academy of Neurology published an evidence-based systematic review of randomized controlled trials using marijuana (Cannabis sativa) or cannabinoids in neurologic disorders. Several cannabinoids showed effectiveness or probable effectiveness for spasticity, central pain, and painful spasms in multiple sclerosis. The review justifies insurance coverage for dronabinol and nabilone for these indications. Many insurance companies already cover these medications for other indications. It is unlikely that the review will alter coverage for herbal marijuana. Currently, no payers cover the costs of herbal medical marijuana because it is illegal under federal law and in most states. Cannabinoid preparations currently available by prescription may have a role in other neurologic conditions, but quality scientific evidence is lacking at this time.

  11. Spasmodic dysphonia: description of the disease and associated neurologic disorders

    Directory of Open Access Journals (Sweden)

    Coelho, Marina Serrato

    2010-06-01

    Full Text Available Introduction: Spasmodic dysphonia (SD is a problem that affects speech and vocalization, one of the most devastating disorders of oral communication. It is characterized by vocal quality tensaestrangulada, harshly and / or interspersed with abrupt vocal attack and a great tension in the vocal tract. The etiology of spasmodic dysphonia is unclear. Some authors point to psychogenic causes, neurological or even unknown. Objective: To assess the prevalence of muscular dystonias and other neurological symptoms in patients with ED. Method: A retrospective study of 10 cases with diagnosis of ED for symptoms and neurological disorders associated. Results: There was a significant predominance of the disease in females (9:1. The average age of onset of symptoms was 32 years, ranging between 14 and 60 years. The mean disease duration was 10 years. Among the patients, 87.5% had a diagnosis of disorders of movement made by a neurologist, including orofacial dystonias (50%, essential tremor (50% and spastic paraparesis (12%. Conclusion: The presence of movement disorders followed almost all cases of spasmodic dysphonia. More studies are needed to clarify the pathophysiological basis of disease.

  12. Neurological Disorders in Medical Use of Cannabis: An Update.

    Science.gov (United States)

    Solimini, Renata; Rotolo, Maria Concetta; Pichini, Simona; Pacifici, Roberta

    2017-01-01

    Medical cannabis is increasingly used as a treatment or adjunct treatment with different levels of efficacy in several neurological disorders or related symptoms (such as multiple sclerosis, autism, Parkinson and Alzheimer disease, Tourette's syndrome, Huntington's disease, neuropathic pain, epilepsy, headache), as well as in other medical conditions (e.g. nausea and vomiting, glaucoma, appetite stimulation, cancer, inflammatory conditions, asthma). Nevertheless, a number of neurological adverse effects from use of medical cannabis on the short- and on the longterm have been reported, in addition to other adverse health events. It has been noticed that the use of medical cannabis can lead to a paradoxical effects depending on the amount of delta-9-tetrahydrocannabinol (THC) -like cannabinoids the preparation contain. Accordingly, some neurological disorders or symptoms (e.g. multiple sclerosis, seizures, epilepsy, headache) may be caused or exacerbated by the same treatment supposed to cure them. The current review presents an update of the neurological adverse effects resulting from the use of cannabis for medical purposes, highlighting the need to weigh the benefits and risks, when using cannabinoidbased treatments. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  13. Retromer in Alzheimer disease, Parkinson disease and other neurological disorders.

    Science.gov (United States)

    Small, Scott A; Petsko, Gregory A

    2015-03-01

    Retromer is a protein assembly that has a central role in endosomal trafficking, and retromer dysfunction has been linked to a growing number of neurological disorders. First linked to Alzheimer disease, retromer dysfunction causes a range of pathophysiological consequences that have been shown to contribute to the core pathological features of the disease. Genetic studies have established that retromer dysfunction is also pathogenically linked to Parkinson disease, although the biological mechanisms that mediate this link are only now being elucidated. Most recently, studies have shown that retromer is a tractable target in drug discovery for these and other disorders of the nervous system.

  14. Factors Related to Social Support in Neurological and Mental Disorders.

    Directory of Open Access Journals (Sweden)

    Kaloyan Kamenov

    Full Text Available Despite the huge body of research on social support, literature has been primarily focused on its beneficial role for both physical and mental health. It is still unclear why people with mental and neurological disorders experience low levels of social support. The main objective of this study was to explore what are the strongest factors related to social support and how do they interact with each other in neuropsychiatric disorders. The study used cross-sectional data from 722 persons suffering from dementia, depression, epilepsy, migraine, multiple sclerosis, Parkinson's disease, schizophrenia, stroke, and substance use disorders. Multiple linear regressions showed that disability was the strongest factor for social support. Extraversion and agreeableness were significant personality variables, but when the interaction terms between personality traits and disability were included, disability remained the only significant variable. Moreover, level of disability mediated the relationship between personality (extraversion and agreeableness and level of social support. Moderation analysis revealed that people that had mental disorders experienced lower levels of support when being highly disabled compared to people with neurological disorders. Unlike previous literature, focused on increasing social support as the origin of improving disability, this study suggested that interventions improving day-to-day functioning or maladaptive personality styles might also have an effect on the way people perceive social support. Future longitudinal research, however, is warranted to explore causality.

  15. Factors Related to Social Support in Neurological and Mental Disorders

    Science.gov (United States)

    Kamenov, Kaloyan; Cabello, Maria; Caballero, Francisco Félix; Cieza, Alarcos; Sabariego, Carla; Raggi, Alberto; Anczewska, Marta; Pitkänen, Tuuli; Ayuso-Mateos, Jose Luis

    2016-01-01

    Despite the huge body of research on social support, literature has been primarily focused on its beneficial role for both physical and mental health. It is still unclear why people with mental and neurological disorders experience low levels of social support. The main objective of this study was to explore what are the strongest factors related to social support and how do they interact with each other in neuropsychiatric disorders. The study used cross-sectional data from 722 persons suffering from dementia, depression, epilepsy, migraine, multiple sclerosis, Parkinson's disease, schizophrenia, stroke, and substance use disorders. Multiple linear regressions showed that disability was the strongest factor for social support. Extraversion and agreeableness were significant personality variables, but when the interaction terms between personality traits and disability were included, disability remained the only significant variable. Moreover, level of disability mediated the relationship between personality (extraversion and agreeableness) and level of social support. Moderation analysis revealed that people that had mental disorders experienced lower levels of support when being highly disabled compared to people with neurological disorders. Unlike previous literature, focused on increasing social support as the origin of improving disability, this study suggested that interventions improving day-to-day functioning or maladaptive personality styles might also have an effect on the way people perceive social support. Future longitudinal research, however, is warranted to explore causality. PMID:26900847

  16. Neurologic complications of disorders of the adrenal glands.

    Science.gov (United States)

    Bertorini, Tulio E; Perez, Angel

    2014-01-01

    Disorders of the adrenal glands frequently have secondary neurological manifestations, while some diseases that involve the central nervous system are accompanied by adrenal gland dysfunction. Excessive corticosteroid secretions in primary or secondary Cushing's syndrome causes muscle weakness and behavioral disturbances, such as emotional lability and sometimes depression, while adrenal insufficiency may cause fatigue, weakness, and depression. Adrenoleukodystrophy and adrenoneuromyelopathy are X-linked recessive disorders of the metabolism of very long chain fatty acids that manifest with white matter abnormalities of the brain, myelopathy and/or neuropathy, as well as adrenal insufficiency. Other disorders of the adrenal glands include hyperaldosteroidism, which may cause weakness from hypokalemia. Dysfunction of the adrenal medulla causes excessive or deficient secretion of catecholamines, primarily causing cardiovascular symptoms. This chapter reviews the clinical manifestations and diagnostic aspects and treatment of the various disorders of the adrenal glands. Some of the congenital adrenal diseases are also discussed. © 2014 Elsevier B.V. All rights reserved.

  17. Nanotechnology Based Treatments for Neurological Disorders from Genetics Perspective

    Directory of Open Access Journals (Sweden)

    Nicholas S. Kurek

    2013-02-01

    Full Text Available Nanotechology involves the application, analysis and manipulation of nanomaterials. These materials have unique and medically useful properties due to their nanoscale parameters. Nanotechnology based treatments and diagnostics might eventually bring great relief to people suffering from neurological disorders including autism spectrum disorders, Alzheimer’s disease and Parkinson’s disorders. A large variety of nonmaterials such as viruses, carbon nanotubes, gold and silica nanoparticles, nanoshells, quantum dots, genetic material and proteins as well as hordes of other forms of nanotechnology have been researched in order to determine their potential in enhancing disease treatments and diagnostics. Nanotechnology has shown countless applications and might eventually be used in every biotech/health industry. Nevertheless, many nanomaterials may pose some safety risks and whether their benefits overweigh the risk is still being debated. Once the proper ethical and safety protocols are established and enough research is completed, nanotechnology is expected to benefit the mankind enormously. In this article, we will discuss and analyze many ways in which, nanotechnology based treatments and diagnostics will be used to help people with neurological disorders through the methods that we currently have at our disposal. [Archives Medical Review Journal 2013; 22(1.000: 12-32

  18. Neurobehavioral, neurologic, and neuroimaging characteristics of fetal alcohol spectrum disorders.

    Science.gov (United States)

    Glass, Leila; Ware, Ashley L; Mattson, Sarah N

    2014-01-01

    Alcohol consumption during pregnancy can have deleterious consequences for the fetus, including changes in central nervous system development leading to permanent neurologic alterations and cognitive and behavioral deficits. Individuals affected by prenatal alcohol exposure, including those with and without fetal alcohol syndrome, are identified under the umbrella of fetal alcohol spectrum disorders (FASD). While studies of humans and animal models confirm that even low to moderate levels of exposure can have detrimental effects, critical doses of such exposure have yet to be specified and the most clinically significant and consistent consequences occur following heavy exposure. These consequences are pervasive, devastating, and can result in long-term dysfunction. This chapter summarizes the neurobehavioral, neurologic, and neuroimaging characteristics of FASD, focusing primarily on clinical research of individuals with histories of heavy prenatal alcohol exposure, although studies of lower levels of exposure, particularly prospective, longitudinal studies, will be discussed where relevant. © 2014 Elsevier B.V. All rights reserved.

  19. Telerehabilitation, virtual therapists, and acquired neurologic speech and language disorders.

    Science.gov (United States)

    Cherney, Leora R; van Vuuren, Sarel

    2012-08-01

    Telerehabilitation (telerehab) offers cost-effective services that potentially can improve access to care for those with acquired neurologic communication disorders. However, regulatory issues including licensure, reimbursement, and threats to privacy and confidentiality hinder the routine implementation of telerehab services into the clinical setting. Despite these barriers, rapid technological advances and a growing body of research regarding the use of telerehab applications support its use. This article reviews the evidence related to acquired neurologic speech and language disorders in adults, focusing on studies that have been published since 2000. Research studies have used telerehab systems to assess and treat disorders including dysarthria, apraxia of speech, aphasia, and mild Alzheimer disease. They show that telerehab is a valid and reliable vehicle for delivering speech and language services. The studies represent a progression of technological advances in computing, Internet, and mobile technologies. They range on a continuum from working synchronously (in real-time) with a speech-language pathologist to working asynchronously (offline) with a stand-in virtual therapist. One such system that uses a virtual therapist for the treatment of aphasia, the Web-ORLA™ (Rehabilitation Institute of Chicago, Chicago, IL) system, is described in detail. Future directions for the advancement of telerehab for clinical practice are discussed. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  20. Induced pluripotent stem cells for modeling neurological disorders

    Science.gov (United States)

    Russo, Fabiele B; Cugola, Fernanda R; Fernandes, Isabella R; Pignatari, Graciela C; Beltrão-Braga, Patricia C B

    2015-01-01

    Several diseases have been successfully modeled since the development of induced pluripotent stem cell (iPSC) technology in 2006. Since then, methods for increased reprogramming efficiency and cell culture maintenance have been optimized and many protocols for differentiating stem cell lines have been successfully developed, allowing the generation of several cellular subtypes in vitro. Gene editing technologies have also greatly advanced lately, enhancing disease-specific phenotypes by creating isogenic cell lines, allowing mutations to be corrected in affected samples or inserted in control lines. Neurological disorders have benefited the most from iPSC-disease modeling for its capability for generating disease-relevant cell types in vitro from the central nervous system, such as neurons and glial cells, otherwise only available from post-mortem samples. Patient-specific iPSC-derived neural cells can recapitulate the phenotypes of these diseases and therefore, considerably enrich our understanding of pathogenesis, disease mechanism and facilitate the development of drug screening platforms for novel therapeutic targets. Here, we review the accomplishments and the current progress in human neurological disorders by using iPSC modeling for Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, spinal muscular atrophy, amyotrophic lateral sclerosis, duchenne muscular dystrophy, schizophrenia and autism spectrum disorders, which include Timothy syndrome, Fragile X syndrome, Angelman syndrome, Prader-Willi syndrome, Phelan-McDermid, Rett syndrome as well as Nonsyndromic Autism. PMID:26722648

  1. Clinical management of acute diabetic Charcot foot in Denmark

    DEFF Research Database (Denmark)

    Jansen, Rasmus Bo; Svendsen, Ole Lander; Kirketerp-Møller, Klaus

    2016-01-01

    INTRODUCTION: Charcot foot is a severe complication to diabetes mellitus and treatment involves several different clinical specialities. Our objective was to describe the current awareness, knowledge and treatment practices of Charcot foot among doctors who handle diabetic foot disorders. METHODS......: This study is based on a questionnaire survey sent out to healthcare professionals, primarily doctors, working with diabetic foot ulcers and Charcot feet in the public sector of the Danish healthcare system. RESULTS: The survey obtained a 52% response rate. A temperature difference of > 2 °C between the two...... and treatment practices of acute diabetic Charcot foot at diabetes foot clinics in Denmark. The responders seem to follow the international recommendations and guidelines on management of the acute diabetic Charcot foot, despite a lack of Danish guidelines. FUNDING: none. TRIAL REGISTRATION: not relevant....

  2. An Analysis of the Symptomatic Domains Most Relevant to Charcot Marie Tooth Neuropathy (CMT) Patients

    Science.gov (United States)

    2017-06-09

    Charcot Marie Tooth Disease (CMT); Hereditary Sensory and Motor Neuropathy; Nerve Compression Syndromes; Tooth Diseases; Congenital Abnormalities; Genetic Diseases, Inborn; Heredodegenerative Disorders, Nervous System

  3. Pathology of neurologic disorders of raccoons (Procyon lotor).

    Science.gov (United States)

    Hamir, Amir N

    2011-09-01

    The raccoon (Procyon lotor) is almost ubiquitous in North America. In recent times, it was introduced in many parts of the world where it has now become largely feral. Since the outbreak of raccoon rabies epizootic in eastern United States and Canada, most diagnostic laboratories have had increased numbers of raccoon carcasses or raccoon brain submissions for diagnosis of rabies. However, since a number of other diseases that affect the central nervous system and have similar clinical signs as rabies have been documented in this species, the current review attempts to bring together the published information on neurologic disorders of raccoons.

  4. Neurological Disorders in a Murine Model of Chronic Renal Failure

    Directory of Open Access Journals (Sweden)

    Jean-Marc Chillon

    2014-01-01

    Full Text Available Cardiovascular disease is highly prevalent in patients with chronic renal failure (CRF. However, data on the impact of CRF on the cerebral circulatory system are scarce—despite the fact that stroke is the third most common cause of cardiovascular death in people with CRF. In the present study, we examined the impact of CRF on behavior (anxiety, recognition and ischemic stroke severity in a well-defined murine model of CRF. We did not observe any significant increases between CRF mice and non-CRF mice in terms of anxiety. In contrast, CRF mice showed lower levels of anxiety in some tests. Recognition was not impaired (vs. controls after 6 weeks of CRF but was impaired after 10 weeks of CRF. Chronic renal failure enhances the severity of ischemic stroke, as evaluated by the infarct volume size in CRF mice after 34 weeks of CRF. Furthermore, neurological test results in non-CRF mice tended to improve in the days following ischemic stroke, whereas the results in CRF mice tended to worsen. In conclusion, we showed that a murine model of CRF is suitable for evaluating uremic toxicity and the associated neurological disorders. Our data confirm the role of uremic toxicity in the genesis of neurological abnormalities (other than anxiety.

  5. Cocaine addiction as a neurological disorder: implications for treatment.

    Science.gov (United States)

    Majewska, M D

    1996-01-01

    Clinical and preclinical studies provide convincing evidence for persistent neurological/psychiatric impairments and possible neuronal degeneration associated with chronic cocaine/stimulant abuse. These impairments include multifocal and global cerebral ischemia, cerebral hemorrhages, infarctions, optic neuropathy, cerebral atrophy, cognitive impairments, and mood and movement disorders. These findings may encourage the placement of stimulant addiction into the category of organic brain disorders. Functional and microanatomical anomalies in the frontal and temporal cortex as well as other brain regions may be responsible for certain aspects of phenomenology and neuropsychopathology that are characteristic of stimulant polydrug addictions. These may include broad spectrum of deficits in cognition, motivation, and insight; behavioral disinhibition; attention deficits; emotional instability; impulsiveness; aggressiveness; depression; anhedonia; and persistent movement disorders. Although it is still debated whether the hypofrontality and other brain anomalies observed in stimulant abusers are a consequence or an antecedent of drug abuse, this debate seems purely academic and irrelevant with respect to the importance of compensating for these deficits in the development of treatment strategies. The neuropsychiatric impairments accompanying stimulant abuse may contribute to the very high rate of relapse in addicts that can take place after long periods (years) of abstinence. It is possible that the neurological deficits present in stimulant addicts, whether they are primary or secondary to stimulant abuse, are responsible for perpetual drug abuse which may be a form of self-medication (Weiss et al. 1991, 1992). In this context, addiction to stimulants, once fully developed, may represent a true biological dependency on drugs that temporarily compensate for existing neurological deficits. The concept of self-medication by drug addicts is supported by major theories of

  6. Herb-Drug Interactions in Neurological Disorders: A Critical Appraisal.

    Science.gov (United States)

    Wilson, Vinu; Maulik, Subir Kumar

    2017-10-31

    Herbal drugs are being used worldwide in a variety of disorders with the misguided belief that they are absolutely safe compared to drugs of modern medicine. This trend is also evident in debilitating neurological and psychiatric disorders such as cerebrovascular accident, Alzheimer's disease, Parkinson's disease and schizophrenia. However, unlike drugs of modern medicine, herbal drugs are complex products containing multiple pharmacologically active constituents. The nature and relative amounts of these constituents vary due to diverse factors such as but not limited to source of the plant(s), local environmental conditions, parts of the plant used, storage, method of extract preparation, accidental contamination or intentional adulteration. Further, they are handled by the human body like modern drugs and subjected to the processes of absorption, distribution, metabolism and excretion. In each of these processes, they can potentially interact with modern drugs due to sharing of similar transport proteins, metabolizing cytochrome P450 (CYP450) enzymes and uptake / efflux pumps. Moreover, herbal drugs can also inhibit or induce CYP450 enzymes or inactivate transporters leading to Herb-Drug interactions (HDIs). Though reported scarcely, many pharmacodynamic HDIs are also being unraveled. In this review, we have analyzed the clinically reported as well as potential HDIs between 10 common herbal drugs and modern medicines used in neurological and psychiatric disorders with their proven or postulated underlying mechanism(s). Physicians and patients should exercise caution when using herbal drugs and modern medicines concomitantly so that the recognized serious HDIs can be avoided. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  7. Genetic testing and reproductive choice in neurological disorders.

    Science.gov (United States)

    Lee, Omay; Porteous, Mary

    2017-08-01

    Genetic testing is increasingly important for investigating suspected inherited neurological conditions. A genetic diagnosis can have a huge impact on patients and also their families. It is important for neurologists to appreciate the presymptomatic and prenatal testing options available to patients and their at-risk relatives once a genetic disorder is diagnosed. Asymptomatic family members can experience considerable psychological distress from the knowledge that they might have inherited a neurodegenerative condition. They may also be concerned about the risk of their children inheriting the condition. Information on reproductive options can provide hope and reassurance. This paper reviews the principles of genetic testing in neurological practice, and how they can be applied in prenatal and preimplantation genetic diagnosis. We explain the basis for direct and exclusion testing, use case examples to illustrate the process by which families are counselled and discuss the ethical implications of reproductive technologies. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  8. [Depressive disorder in cardiovascular, neurological and oncologic diseases].

    Science.gov (United States)

    Cesková, E

    2005-01-01

    The discovery of antidepressants meant undoubtedly a revolution in psychiatry. The development of antidepressants has changed the image of psychiatry, brought a progress in the treatment and became a stimulus for investigations of mental illnesses ethiopathogenesis. Nowadays it is becoming evident, that many biologic, psychologic and with high probability also social aspects are common for the depression and for somatic disorders. The more prominent is the association of depression with cardiovascular disease. Neurological disease, mainly the epilepsy, Parkinson disease an stroke represent further common sphere. Historically, association between cancer and depression was identified first. The article presents epidemiological data and analyses possible common mechanisms of somatic disease and depression. In the last part the actual data about the treatment of depression in individual somatic diseases are described.

  9. The therapeutic value of yoga in neurological disorders

    Directory of Open Access Journals (Sweden)

    Shri K Mishra

    2012-01-01

    Full Text Available Background: The ancient mind and body healing methods of yoga recently sparked fervor in the scientific community as an alternative and complementary means of therapy. Since the World Health Organization officially began promoting yoga in developing countries in 1978, yoga has been cited for its therapeutic potential and has been widely recognized in Western culture. However, as an increasing number of people practice yoga for remedial purposes, researchers raise two important questions: 1 Is yoga a valid complementary management and rehabilitation treatment modality? 2 What conditions show promise of treatment with this intervention?. Objective: This review article uses comprehensive scientific, evidence-based studies to analyze the efficacy of various basic and applied aspects of yoga in disease prevention and health promotion. It specifically intends to expose the effects of yoga in neurological disorders, particularly epilepsy, stroke, multiple sclerosis, Alzheimer′s disease, peripheral nervous system disease, and fibromyalgia. Materials and Methods: Information was gathered from various resources including PubMed, Ovid, MD-Consult, USC, and U.C.L.A. libraries. Studies were selected and reviewed on the basis of sample size, control, randomization, double-blinding, and statistical analysis of results. Results: The pratice of yoga and meditation demonstrates statistically encouraging physiological and psychological improvements in the aforementioned neurological disorders. However, there were certain flaws and inadequacies in the study designs employed to evaluate the same. A critical analysis of these studies is presented. Conclusions: With the aim to focus attention on this widespread yet largely unexamined treatment modality, this paper seeks to provide direction and support for further research necessary to validate yoga as an integrative, alternative, and complementary therapy.

  10. Evidence-based guideline update: Plasmapheresis in neurologic disorders

    Science.gov (United States)

    Cortese, I.; Chaudhry, V.; So, Y.T.; Cantor, F.; Cornblath, D.R.; Rae-Grant, A.

    2011-01-01

    Objective: To reassess the role of plasmapheresis in the treatment of neurologic disorders. Methods: We evaluated the available evidence based on a structured literature review for relevant articles from 1995 through September 2009. In addition, due to revision of the definitions of classification of evidence since the publication of the previous American Academy of Neurology assessment in 1996, the evidence cited in that manuscript was reviewed and reclassified. Results and Recommendations: Plasmapheresis is established as effective and should be offered in severe acute inflammatory demyelinating polyneuropathy (AIDP)/Guillain-Barré syndrome (GBS) and in the short-term management of chronic inflammatory demyelinating polyneuropathy (Class I studies, Level A). Plasmapheresis is established as ineffective and should not be offered for chronic or secondary progressive multiple sclerosis (MS) (Class I studies, Level A). Plasmapheresis is probably effective and should be considered for mild AIDP/GBS, as second-line treatment of steroid-resistant exacerbations in relapsing forms of MS, and for neuropathy associated with immunoglobulin A or immunoglobulin G gammopathy, based on at least one Class I or 2 Class II studies (Level B). Plasmapheresis is probably not effective and should not be considered for neuropathy associated with immunoglobulin M gammopathy, based on one Class I study (Level B). Plasmapheresis is possibly effective and may be considered for acute fulminant demyelinating CNS disease (Level C). There is insufficient evidence to support or refute the use of plasmapheresis for myasthenia gravis, pediatric autoimmune neuropsychiatric disorders associated with streptococcus infection, and Sydenham chorea (Class III evidence, Level U). PMID:21242498

  11. Cerebral circulation, neurological and neuropsychological disorders in idiopathic arterial hypotension

    Directory of Open Access Journals (Sweden)

    Andrei Viktorovich Fonyakin

    2011-01-01

    Full Text Available Objective: to evaluate the cerebral circulation in idiopathic arterial hypotension (IAH in relation to neurological and neuropsychological disorders. Patients and methods. Sixty-five patients (mean age 40.2 [8, 14] years with prolonged IAH were examined. Neuropsychological examination was made using the procedure adapted by A.R. Luria; different psychic functions (memory, speech, gnosis, praxis, thinking, attention, counting, writing, and reading were studied. Cerebral hemodynamics was investigated using duplex scanning of the brachiocephalic arteries (BCA, middle cerebral arteries (MCA, internal jugular (IJV and vertebral veins (VV. The patients were assigned to 2 groups: 1 19 (29% patients with somatoform disorders, 2 46 (71% patients with signs of the initial manifestations of chronic cerebrovascular insufficiency. Group 2 patients were older and had a longer history of IAH. Results. In all the patients, cerebral blood supply in the carotid system showed moderately lower arterial blood inflow with a compensatory vascular resistance decrease and balanced venous outflow reduction with increased vascular resistance. Group 2 patients had a substantial (to the lower normal range blood flow decline in the vertebral artery along with increased peripheral resistance in the VV. The degree of neuropsychological derangement was inversely proportional to blood flow velocity in BCA and MCA and to blood outflow in IJV and VV.

  12. Time perception distortion in neuropsychiatric and neurological disorders.

    Science.gov (United States)

    Teixeira, Silmar; Machado, Sergio; Paes, Flavia; Velasques, Bruna; Silva, Julio Guilherme; Sanfim, Antonio L; Minc, Daniel; Anghinah, Renato; Menegaldo, Luciano L; Salama, Mohamed; Cagy, Mauricio; Nardi, Antonio E; Pöppel, Ernst; Bao, Yan; Szelag, Elzbieta; Ribeiro, Pedro; Arias-Carrión, Oscar

    2013-08-01

    There is no sense organ specifically dedicated to time perception, as there is for other senses such as hearing and vision. However, this subjective sense of time is fundamental to our conception of reality and it creates the temporal course of events in our lives. Here, we explored neurobiological relations from the clinical perspective, examining timing ability in patients with different neurological and psychiatric conditions (e.g. Parkinson's disease, depression, bipolar disorder, anxiety disorders and schizophrenia). The neural bases of present distortions in time perception and temporal information processing still remain poorly understood. We reviewed: a) how the brain is capable of encoding time in different environments and multiple tasks, b) different models of interval timing, c) brain structures and neurotransmitters associated with time perception, d) the relationship between memory and time perception, e) neural mechanisms underlying different theories in neural and mental processes, and f) the relationship between different mental diseases and time perception. Bibliographic research was conducted based on publications over the past thirteen years written in English in the databases Scielo, Pubmed/MEDLINE, ISI Web of Knowledge. The time perceptions research are executed to evaluate time perception in mental diseases and can provide evidence for future clinical applications.

  13. Construction of standardized Arabic questionnaires for screening neurological disorders (dementia, stroke, epilepsy, movement disorders, muscle and neuromuscular junction disorders

    Directory of Open Access Journals (Sweden)

    El Tallawy HN

    2016-08-01

    Full Text Available Hamdy N El Tallawy,1 Wafaa MA Farghaly,1 Tarek A Rageh,1 Ahmed O Saleh,2 Taha AH Mestekawy,3 Manal MM Darwish,4 Mohamed A Abd El Hamed,1 Anwar M Ali,1 Doaa M Mahmoud1 1Department of Neurology and Psychiatry, Faculty of Medicine, 2Psychology Department, Faculty of Education, 3Psychology Department, Faculty of Arts, 4Public Health Department, Faculty of Medicine, Assiut University, Assiut, Egypt Abstract: A screening questionnaire is an important tool for early diagnosis of neurological disorders, and for epidemiological research. This screening instrument must be both feasible and valid. It must be accepted by the community and must be sensitive enough. So, the aim of this study was to prepare different Arabic screening questionnaires for screening different neurological disorders. This study was carried out in three stages. During the first stage, construction of separate questionnaires designed for screening the five major neurological disorders: cerebrovascular stroke, dementias, epilepsy, movement disorders, and muscle and neuromuscular disorders were done. Validation of the screening questionnaires was carried out in the second stage. Finally, questionnaire preparation was done in the third stage. Questions with the accepted sensitivity and specificity in each questionnaire formed the refined separate questionnaires. Keywords: screening questionnaires, neurological disorders

  14. Influenza vaccination in children with neurologic or neurodevelopmental disorders.

    Science.gov (United States)

    Smith, Michael; Peacock, Georgina; Uyeki, Timothy M; Moore, Cynthia

    2015-05-11

    Children with neurologic or neurodevelopmental disorders (NNDDs) are at increased risk of complications from influenza. Although the Advisory Committee on Immunization Practices (ACIP) has recognized NNDDs as high-risk conditions for influenza complications since 2005, little is known about influenza vaccination practices in this population. CDC collaborated with Family Voices, a national advocacy group for children with special healthcare needs, to recruit parents of children with chronic medical conditions. Parents were surveyed about their knowledge, attitudes, and practices surrounding influenza vaccination. The primary outcome of interest was parental report of vaccination, or intent to vaccinate, at the time of survey participation. CDC also collaborated with the American Academy of Pediatrics to recruit primary care and specialty physicians who provide care for high-risk children, specifically those with neurologic conditions. The primary outcome was physician recognition of ACIP high-risk influenza conditions. 2138 surveys were completed by parents of children with high-risk conditions, including 1143 with at least one NNDD. Overall, 50% of children with an NNDD were vaccinated, or their parents planned to have them vaccinated against influenza. Among all 2138 children, in multivariable analysis, the presence of a respiratory condition and prior seasonal influenza vaccination was significantly associated with receipt or planned current season influenza vaccination, but the presence of an NNDD was not. 412 pediatricians completed the provider survey. Cerebral palsy was recognized as a high-risk influenza condition by 74% of physician respondents, but epilepsy (51%) and intellectual disability (46%) were less commonly identified. Our estimates of influenza vaccination in children with NNDDs are comparable to published reports of vaccination in healthy children, which continue to be suboptimal. Education of parents of children with NNDDs and healthcare

  15. Upper limb impairments associated with spasticity in neurological disorders

    Directory of Open Access Journals (Sweden)

    Mirbagheri Mehdi M

    2007-11-01

    Full Text Available Abstract Background While upper-extremity movement in individuals with neurological disorders such as stroke and spinal cord injury (SCI has been studied for many years, the effects of spasticity on arm movement have been poorly quantified. The present study is designed to characterize the nature of impaired arm movements associated with spasticity in these two clinical populations. By comparing impaired voluntary movements between these two groups, we will gain a greater understanding of the effects of the type of spasticity on these movements and, potentially a better understanding of the underlying impairment mechanisms. Methods We characterized the kinematics and kinetics of rapid arm movement in SCI and neurologically intact subjects and in both the paretic and non-paretic limbs in stroke subjects. The kinematics of rapid elbow extension over the entire range of motion were quantified by measuring movement trajectory and its derivatives; i.e. movement velocity and acceleration. The kinetics were quantified by measuring maximum isometric voluntary contractions of elbow flexors and extensors. The movement smoothness was estimated using two different computational techniques. Results Most kinematic and kinetic and movement smoothness parameters changed significantly in paretic as compared to normal arms in stroke subjects (p Conclusion The findings suggest that although the cause and location of injury are different in spastic stroke and SCI subjects, the impairments in arm voluntary movement were similar in the two spastic groups. Our results also suggest that the non-paretic arm in stroke subjects was not distinguishable from the normal, and might therefore be used as an appropriate control for studying movement of the paretic arm.

  16. Limiting exercise inhibits neuronal recovery from neurological disorders

    Directory of Open Access Journals (Sweden)

    Stefan S Anthony

    2017-01-01

    Full Text Available Patients who are bedridden often suffer from muscular atrophy due to reduced daily activities and can become depressed. However, patients who undergo physical therapy sometimes demonstrate positive benefits including a reduction of stressful and depressed behavior. Regenerative medicine has seen improvements in two stem cell-based therapies for central nervous system disorders. One therapy is through the transfer of exogenous stem cells. The other therapy is a more natural method and focuses on the increasing endogenous neurogenesis and restoring the neurological impairments. This study overviews how immobilization-induced disuse atrophy affects neurogenesis in rats, specifically hypothesizing that immobilization diminishes circulating trophic factor levels, like vascular endothelial growth factors or brain-derived neurotrophic factor, which in turn limits neurogenesis. This hypothesis requires the classification of the stem cell microenvironment by probing growth factors in addition to other stress-related proteins that correlate with exercise-induced neurogenesis. There is research examining the effects of increased exercise on neurogenesis while limiting exercise, which better demonstrates the pathological states of immobile stroke patients, remains relatively unexplored. To examine the effects of immobilization on neurogenesis quantitative measurements of movements, 5-bromo-2deoxyuridine labeling of proliferative cells, biochemical assays of serum, cerebrospinal fluid and neurological levels of trophic factors, growth factors, and stress-related proteins will indicate levels of neurogenesis. In further research, studies are needed to show how in vivo stimulation, or lack thereof, affects stem cell microenvironments to advance treatment procedures for strengthening neurogenesis in bedridden patients. This paper is a review article. Referred literature in this paper has been listed in the references section. The datasets supporting the

  17. 76 FR 2129 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Science.gov (United States)

    2011-01-12

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of a meeting of the National Advisory Neurological Disorders and Stroke... who plan to attend and need special assistance, such as sign language interpretation or other...

  18. Biological and perceived stress in motor functional neurological disorders.

    Science.gov (United States)

    Apazoglou, Kalliopi; Mazzola, Viridiana; Wegrzyk, Jennifer; Polara, Giulia Frasca; Aybek, Selma

    2017-11-01

    Current models explaining motor functional neurological disorders (FND) integrate both the neurobiological mechanisms underlying symptoms production and the role of psychosocial stressors. Imaging studies have suggested abnormal motor control linked to impaired emotional and stress regulation. However, little is known on the biological stress regulation in FND. Our aim was to study the biological and perceived response to stress in patients with motor FND. Sixteen patients with motor FND (DSM-5 criteria) and fifteen healthy controls underwent the Trier Social Stress Test. Hypothalamo-pituitary-adrenal axis (HPA) response was evaluated with salivary cortisol and autonomous sympathetic response with salivary alpha-amylase. Area under the curve was computed to reflect background levels (AUCg) and change over time (AUCi). Life adversities and perceived subjective stress on a visual analog scale (VAS) were correlated with biological responses. FND patients had significantly higher background levels (AUCg) of both stress markers (cortisol and amylase) than controls. The biological response (AUCi) to stress did not differ between groups for both markers but the subjective response showed an interaction effect with patients reporting higher levels of stress than controls. After stress, controls showed a strong correlation between subjective and objective sympathetic values (amylase) but not patients. The number and subjective impact of adverse life events correlated with cortisol AUCg in patients only. This study confirms a baseline HPA-axis and sympathetic hyperarousal state in motor FND related to life adversities. During a social stress, dissociation between perceived stress and biological markers was observed in patients only, reflecting a dysregulation of interoception capacity, which might represent an endophenotype of this disorder. Copyright © 2017 Elsevier Ltd. All rights reserved.

  19. Perspectives for computational modeling of cell replacement for neurological disorders

    Energy Technology Data Exchange (ETDEWEB)

    Aimone, James B. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Weick, Jason P. [Univ. of New Mexico, Albuquerque, NM (United States)

    2013-01-01

    In mathematical modeling of anatomically-constrained neural networks we provide significant insights regarding the response of networks to neurological disorders or injury. Furthermore, a logical extension of these models is to incorporate treatment regimens to investigate network responses to intervention. The addition of nascent neurons from stem cell precursors into damaged or diseased tissue has been used as a successful therapeutic tool in recent decades. Interestingly, models have been developed to examine the incorporation of new neurons into intact adult structures, particularly the dentate granule neurons of the hippocampus. These studies suggest that the unique properties of maturing neurons, can impact circuit behavior in unanticipated ways. In this perspective, we review the current status of models used to examine damaged CNS structures with particular focus on cortical damage due to stroke. Secondly, we suggest that computational modeling of cell replacement therapies can be made feasible by implementing approaches taken by current models of adult neurogenesis. The development of these models is critical for generating hypotheses regarding transplant therapies and improving outcomes by tailoring transplants to desired effects.

  20. Perspectives for computational modeling of cell replacement for neurological disorders

    Energy Technology Data Exchange (ETDEWEB)

    Aimone, James B.; Weick, Jason P.

    2013-01-01

    Mathematical modeling of anatomically-constrained neural networks has provided significant insights regarding the response of networks to neurological disorders or injury. A logical extension of these models is to incorporate treatment regimens to investigate network responses to intervention. The addition of nascent neurons from stem cell precursors into damaged or diseased tissue has been used as a successful therapeutic tool in recent decades. Interestingly, models have been developed to examine the incorporation of new neurons into intact adult structures, particularly the dentate granule neurons of the hippocampus. These studies suggest that the unique properties of maturing neurons, can impact circuit behavior in unanticipated ways. In this perspective, we review the current status of models used to examine damaged CNS structures with particular focus on cortical damage due to stroke. Secondly, we suggest that computational modeling of cell replacement therapies can be made feasible by implementing approaches taken by current models of adult neurogenesis. The development of these models is critical for generating hypotheses regarding transplant therapies and improving outcomes by tailoring transplants to desired effects.

  1. Impaired movement timing in neurological disorders: rehabilitation and treatment strategies.

    Science.gov (United States)

    Hove, Michael J; Keller, Peter E

    2015-03-01

    Timing abnormalities have been reported in many neurological disorders, including Parkinson's disease (PD). In PD, motor-timing impairments are especially debilitating in gait. Despite impaired audiomotor synchronization, PD patients' gait improves when they walk with an auditory metronome or with music. Building on that research, we make recommendations for optimizing sensory cues to improve the efficacy of rhythmic cuing in gait rehabilitation. Adaptive rhythmic metronomes (that synchronize with the patient's walking) might be especially effective. In a recent study we showed that adaptive metronomes synchronized consistently with PD patients' footsteps without requiring attention; this improved stability and reinstated healthy gait dynamics. Other strategies could help optimize sensory cues for gait rehabilitation. Groove music strongly engages the motor system and induces movement; bass-frequency tones are associated with movement and provide strong timing cues. Thus, groove and bass-frequency pulses could deliver potent rhythmic cues. These strategies capitalize on the close neural connections between auditory and motor networks; and auditory cues are typically preferred. However, moving visual cues greatly improve visuomotor synchronization and could warrant examination in gait rehabilitation. Together, a treatment approach that employs groove, auditory, bass-frequency, and adaptive (GABA) cues could help optimize rhythmic sensory cues for treating motor and timing deficits. © 2014 New York Academy of Sciences.

  2. SIRP/CD47 signaling in neurological disorders.

    Science.gov (United States)

    Zhang, Haiyue; Li, Fengwu; Yang, Yuanyuan; Chen, Jun; Hu, Xiaoming

    2015-10-14

    Microglia play important roles in the process of neuronal injury and recovery. Numeous surface receptors have been described to regulate microglial activation. These receptors tightly mediate normal microglial functions including cell mobility, phagocytosis, and production of inflammatory mediators or trophic factors. In recent years, significant progresses have been achieved for understanding the signaling mechanisms underlying these receptors. Their specific roles in neurological diseases have been documented. This review will focus on the signal regulatory protein (SIRP) and its ligand CD47, two surface receptors expressed on microglia and other cells in the central nervous system (CNS) such as neurons. We will discuss the involvement of SIRP/CD47 signaling in microglial activation and in the interplay between microglia and other CNS cells. Current studies reveal the importance of CD47 and SIRPα in the process of neuroinflammation in the CNS disorders. The dual and contradictory role of CD47 suggests that targeting the SIRPα/CD47 signaling may achieve different effects depending on disease stage. This article is part of a Special Issue entitled SI: Cell Interactions In Stroke. Copyright © 2015 Elsevier B.V. All rights reserved.

  3. Cannabinoid-based medicines for neurological disorders--clinical evidence.

    Science.gov (United States)

    Wright, Stephen

    2007-08-01

    Whereas the cannabis plant has a long history of medicinal use, it is only in recent years that a sufficient understanding of the pharmacology of the main plant constituents has allowed for a better understanding of the most rational therapeutic targets. The distribution of cannabinoid receptors, both within the nervous system and without, and the development of pharmacological tools to investigate their function has lead to a substantial increase in efforts to develop cannabinoids as therapeutic agents. Concomitant with these efforts, the understanding of the pharmacology of plant cannabinoids at receptor and other systems distinct from the cannabinoid receptors suggests that the therapeutic applications of plant-derived cannabinoids (and presumably their synthetic derivatives also) may be diverse. This review aims to discuss the clinical evidence investigating the use of medicines derived, directly or indirectly, from plant cannabinoids with special reference to neurological disorders. Published studies suggest that the oral administration of cannabinoids may not be the preferred route of administration and that plant extracts show greater evidence of efficacy than synthetic compounds. One of these, Sativex (GW Pharmaceuticals), was approved as a prescription medicine in Canada in 2005 and is currently under regulatory review in the EU.

  4. Prion proteins: physiological functions and role in neurological disorders.

    Science.gov (United States)

    Hu, Wei; Kieseier, Bernd; Frohman, Elliot; Eagar, Todd N; Rosenberg, Roger N; Hartung, Hans-Peter; Stüve, Olaf

    2008-01-15

    Stanley Prusiner was the first to promote the concept of misfolded proteins as a cause for neurological disease. It has since been shown by him and other investigators that the scrapie isoform of prion protein (PrP(Sc)) functions as an infectious agent in numerous human and non-human disorders of the central nervous system (CNS). Interestingly, other organ systems appear to be less affected, and do not appear to lead to major co-morbidities. The physiological function of the endogenous cellular form of the prion protein (PrP(C)) is much less clear. It is intriguing that PrP(c) is expressed on most tissues in mammals, suggesting not only biological functions outside the CNS, but also a role other than the propagation of its misfolded isotype. In this review, we summarize accumulating in vitro and in vivo evidence regarding the physiological functions of PrP(C) in the nervous system, as well as in lymphoid organs.

  5. Hysteria to conversion disorders: Babinski's contributions

    Directory of Open Access Journals (Sweden)

    Marleide da Mota Gomes

    2014-04-01

    Full Text Available The main objective of this paper is to present the importance of hysteria on Babinski's oeuvre, and the conceptions of pithiatism from Babinski until the one of conversion disorder. Babinski gave a mental basis for hysteria in the place of Charcot's encephalopatic one, and several important semiotic tools to differentiate organic from hysterical manifestations based on studies from 1893-1917/8. His teachings were spread worldwide, and in Brazil they were also appreciated in the work on hysteria by Antonio Austregesilo, the first Brazilian neurology chairman. The neurobiological basis of hysteria conceived by Charcot is nowadays reappraised, and Babinski's neurosemiological contribution is everlasting. The patients believed to be hysterical, and the two outstanding neurologists, Charcot and Babinski, gave support for the development of the modern neurology.

  6. Cognitive-analytical therapy for a patient with functional neurological symptom disorder-conversion disorder (psychogenic myopia: A case study

    Directory of Open Access Journals (Sweden)

    Hamid Nasiri

    2015-01-01

    Full Text Available Functional neurological symptom disorder commonly presents with symptoms and defects of sensory and motor functions. Therefore, it is often mistaken for a medical condition. It is well known that functional neurological symptom disorder more often caused by psychological factors. There are three main approaches namely analytical, cognitive and biological to manage conversion disorder. Any of such approaches can be applied through short-term treatment programs. In this case, study a 12-year-old boy with the diagnosed functional neurological symptom disorder (psychogenic myopia was put under a cognitive-analytical treatment. The outcome of this treatment modality was proved successful.

  7. Arthroplasty of a Charcot knee

    Science.gov (United States)

    Babazadeh, Sina; Stoney, James D.; Lim, Keith; Choong, Peter F.M.

    2010-01-01

    The Charcot knee - or neuropathic arthropathy - presents a considerable challenge to the orthopaedic surgeon. Caused by a combination of sensory, motor and autonomic neuropathy, it was originally described as an arthritic sequelae of neurosyphilis. In today's western orthopaedics it is more often caused by diabetes. A Charcot knee is often symptomatically painful and unstable. Traditional management has usually been conservative or arthrodesis, with limited success. Arthroplasty of a Charcot joint has commonly been avoided at all costs. However, in the right patient, using the right technique, arthroplasty can significantly improve the symptoms of a Charcot joint. This article explores the evidence surrounding the role of arthroplasty in the management of a Charcot knee. Arthroplasty is compared to other forms of treatment and specific patient demographics and surgical techniques are explored in an attempt to define the role of arthroplasty in the management of a Charcot knee. PMID:21808708

  8. Arthroplasty of a Charcot knee

    Directory of Open Access Journals (Sweden)

    Sina Babazadeh

    2010-08-01

    Full Text Available The Charcot knee - or neuropathic arthropathy - presents a considerable challenge to the orthopaedic surgeon. Caused by a combination of sensory, motor and autonomic neuropathy, it was originally described as an arthritic sequelae of neurosyphilis. In today’s western orthopaedics it is more often caused by diabetes. A Charcot knee is often symptomatically painful and unstable. Traditional management has usually been conservative or arthrodesis, with limited success. Arthroplasty of a Charcot joint has commonly been avoided at all costs. However, in the right patient, using the right technique, arthroplasty can significantly improve the symptoms of a Charcot joint. This article explores the evidence surrounding the role of arthroplasty in the management of a Charcot knee. Arthroplasty is compared to other forms of treatment and specific patient demographics and surgical techniques are explored in an attempt to define the role of arthroplasty in the management of a Charcot knee.

  9. Global, regional, and national burden of neurological disorders during 1990-2015

    DEFF Research Database (Denmark)

    2017-01-01

    BACKGROUND: Comparable data on the global and country-specific burden of neurological disorders and their trends are crucial for health-care planning and resource allocation. The Global Burden of Diseases, Injuries, and Risk Factors (GBD) Study provides such information but does not routinely......-specific prevalence, mortality, disability-adjusted life-years (DALYs), years of life lost (YLLs), and years lived with disability (YLDs) for various neurological disorders that in the GBD classification have been previously spread across multiple disease groupings. The more inclusive grouping of neurological...... aggregate results that are of interest to clinicians specialising in neurological conditions. In this systematic analysis, we quantified the global disease burden due to neurological disorders in 2015 and its relationship with country development level. METHODS: We estimated global and country...

  10. Neurological associations in auditory neuropathy spectrum disorder: Results from a tertiary hospital in South India

    Directory of Open Access Journals (Sweden)

    Anjali Lepcha

    2015-01-01

    Full Text Available Aims: To find out the prevalence and types of neurological abnormalities associated in auditory neuropathy spectrum disorder in a large tertiary referral center. Settings and Design: A prospective clinical study was conducted on all patients diagnosed with auditory neuropathy spectrum disorder in the ear, nose, and throat (ENT and neurology departments during a 17-month period. Patients with neurological abnormalities on history and examination were further assessed by a neurologist to determine the type of disorder present. Results: The frequency of auditory neuropathy spectrum disorder was 1.12%. Sixty percent were found to have neurological involvement. This included cerebral palsy in children, peripheral neuropathy (PN, spinocerebellar ataxia, hereditary motor-sensory neuropathy, spastic paresis, and ponto-bulbar palsy. Neurological lesions did not present simultaneously with hearing loss in most patients. Sixty-six percent of patients with auditory neuropathy spectrum disorder were born of consanguineous marriages. Conclusions: There is a high prevalence of neurological lesions in auditory neuropathy spectrum disorder which has to be kept in mind while evaluating such patients. Follow-up and counselling regarding the appearance of neuropathies is therefore important in such patients. A hereditary etiology is indicated in a majority of cases of auditory neuropathy spectrum disorder.

  11. Freud with Charcot: Freud's discovery and the question of diagnosis.

    Science.gov (United States)

    Lepoutre, Thomas; Villa, François

    2015-04-01

    Although Charcot's seminal role in influencing Freud is widely stated, although Freud's trip to Paris to study with Charcot is well recognized as pivotal in his shift from neurological to psychopathological work, a key fact of the Freudian heuristic remains largely underestimated: namely, that Freud's psychopathological breakthrough, which gave birth to psychoanalysis, cannot be separated from his 'diagnostic preoccupation', which is a crucial and at times the first organizing principle of his earliest writings. The purpose of this article is therefore to reopen the question of diagnosis by following its development along the path leading from Charcot to Freud. The authors demonstrate that Freud's careful attention to diagnostic distinctions follows strictly in the direction of Charcot's 'nosological method'. More importantly, the article intends to identify the precise way in which his ideas operate in Freud's own work, in order to understand how Freud reinvests them to forge his own nosological system. If the authors trace the destiny of Charcot's lessons as they reach Freud's hands, it is the importance granted to mixed neuroses in Freud's psychopathology that allows them to pinpoint the role played by the diagnostic process in the rationality of psychoanalysis. Copyright © 2014 Institute of Psychoanalysis.

  12. 77 FR 19024 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Science.gov (United States)

    2012-03-29

    ... Neurological Disorders and Stroke Special Emphasis Panel; Diversity Research Education in Neuroscience. Date...: National Institutes of Health, Neuroscience Center, 6001 Executive Boulevard, Rockville, MD 20852... Review Branch, Division of Extramural Research, NINDS/ NIH/DHHS/Neuroscience Center, 6001 Executive Blvd...

  13. 78 FR 39299 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Science.gov (United States)

    2013-07-01

    ... Institute of Neurological Disorders and Stroke Special, Emphasis Panel, Collaborative Research on Chronic Traumatic Encephalopathy and Delayed Effects of Traumatic Brain Injury. Date: July 19, 2013. Time: 1:30 p.m...

  14. Psychiatry and the Necker Cube. Neurological and Psychological Conceptions of Psychiatric Disorder

    Directory of Open Access Journals (Sweden)

    D. Rogers

    1988-01-01

    Full Text Available Neurological and psychological conceptions of psychiatric disorder are in conflict at the present time. This conflict is considered in the context of the history of psychiatry and the philosophy of science. Its practical consequences are considered for the motor disorder of schizophrenia, the cognitive impairment in psychiatric illnesses, the use of the terms organic and functional and the association of neurological disorder with psychotic and neurotic disorders. The conflict is also examined in individual cases and the implications for treatment assessed.

  15. Distinct neurological disorders with ATP1A3 mutations

    NARCIS (Netherlands)

    Heinzen, E.L.; Arzimanoglou, A.; Brashear, A.; Clapcote, S.J.; Gurrieri, F.; Goldstein, D.B; Johannesson, S.H.; Mikati, M.A.; Neville, B.; Nicole, S.; Ozelius, L.J.; Poulsen, H.; Schyns, T.; Sweadner, K.J.; Maagdenberg, A. van den; Vilsen, B.; Koenderink, J.B.

    2014-01-01

    Genetic research has shown that mutations that modify the protein-coding sequence of ATP1A3, the gene encoding the alpha3 subunit of Na(+)/K(+)-ATPase, cause both rapid-onset dystonia parkinsonism and alternating hemiplegia of childhood. These discoveries link two clinically distinct neurological

  16. The Clinical Spectrum Of Paediatric Neurological Disorders In ...

    African Journals Online (AJOL)

    The predominant neurologic morbidities included: cerebral palsy (42.4%), epilepsy (27.8%), febrile seizure (6.5%), mental retardation(6.2%), microcephaly (5.6%), behavioral problems (5.6%), poliomyelitis (4.5%), hydrocephalus (4.2%), visual impairment (2.8%), down syndrome (1.7%), and attention deficit hyperactivity ...

  17. An Analysis of Disorders seen at the Paediatric Neurology Clinic ...

    African Journals Online (AJOL)

    METHODS: All children presenting at the paediatric neurology clinic of the University College Hospital, Ibadan, Nigeria over a period of 20 months were prospectively studied. Diagnoses were made from detailed history, thorough physical examination, with particular emphasis on the central nervous system and appropriate ...

  18. Study of the usefulness of magnetic resonance imaging on neurological disorders

    Energy Technology Data Exchange (ETDEWEB)

    Hokezu, Youichi (Kagoshima Univ. (Japan). Faculty of Medicine)

    1992-05-01

    One hundred and ten patients with several neurological disorders including cerebrovascular diseases (CVD), degenerative diseases, demyelinating diseases, infections of the nervous systems, neurometabolic disorders, myopathies, diseases of the spine or spinal cord and myelopathy were studied by employing magnetic resonance imaging (MRI). Superconductive magnetic systems of 0.5 T, 1.0 T or 1.5 T were used for this study. MRI could show the lesions clearly in many neurological disorders such as CVD, demyelinating diseases, infections of the nervous systems, neurometabolic disorders, myopathies and myeloythy. However, MRI could not necessarily show the lesions cleary in neurodegenerative disease and bone or calcified lesions such as ossification of posterior longitudinal ligament. MRI is better than CT in spatial and tissue resolution. MRI study is expected to be the more beneficial procedure in neurological disorders, if a much shorter scanning time can be achieved. (author).

  19. Neurological disorders in Iraqi refugees in Jordan: data from the United Nations Refugee Assistance Information System.

    Science.gov (United States)

    Mateen, Farrah J; Carone, Marco; Nyce, Sayre; Ghosn, Jad; Mutuerandu, Timothy; Al-Saedy, Huda; Lowenstein, Daniel H; Burnham, Gilbert

    2012-04-01

    The United Nations High Commissioner for Refugees (UNHCR) recognizes 43.7 million forcibly displaced persons and asylum seekers due to conflict and persecution worldwide. Neurological disorders have rarely been described in displaced persons but likely pose a significant burden of disease. We describe the disease spectrum and health service utilization of Iraqi refugees and asylum seekers with neurological disorders using an information system developed by the UNHCR. Neurological disorders were actively monitored among the 7,642 UNHCR-registered Iraqi refugees and asylum seekers who received health and humanitarian assistance using a pilot, centralized, database called the Refugee Assistance Information System (RAIS) in the Kingdom of Jordan in 2010. There were 122 neurological diagnoses reported in 1,328 refugees (mean age 41 years, 49% female, 10% disabled, 43% with pending resettlement applications) in 2,659 health visits, accounting for 17% of all refugees who sought health assistance in RAIS. Referral to a neurologist occurred in 178 cases (13.4%). The most frequent ICD-10 neurological diagnoses were dorsalgia (back pain) (29.7% of individuals with neurological disorders), headache (13.1%), and epilepsy (12.6%). Approximately 1 in 20 Iraqi refugees with a neurological diagnosis self-reported a history of torture, which was higher than Iraqi refugees without a history of torture [66/1,328 versus 196/6,314, odds ratio (OR) = 1.63, 95% confidence interval (CI) 1.21-2.18]. Neurological disease affects a high proportion of Iraqi refugees, including victims of torture and the disabled. Refugees require dedicated care for treatment of neurological disease with a focus on pain disorders and epilepsy.

  20. Male sexual dysfunction and infertility associated with neurological disorders

    DEFF Research Database (Denmark)

    Fode, Mikkel; Krogh-Jespersen, Sheila; Brackett, Nancy L

    2012-01-01

    always require assisted reproductive techniques including intrauterine insemination or in vitro fertilization with or without intracytoplasmic sperm injection. The method of choice depends largely on the number of motile sperm in the ejaculate.Asian Journal of Andrology advance online publication, 5...... is managed by medications to reverse the condition in mild cases and in bladder harvest of semen after ejaculation in more severe cases. Anejaculation might also be managed by medication in mild cases while assisted ejaculatory techniques including penile vibratory stimulation and electroejaculation are used......Normal sexual and reproductive functions depend largely on neurological mechanisms. Neurological defects in men can cause infertility through erectile dysfunction, ejaculatory dysfunction and semen abnormalities. Among the major conditions contributing to these symptoms are pelvic...

  1. Distinct neurological disorders with ATP1A3 mutations

    Science.gov (United States)

    Heinzen, Erin L.; Arzimanoglou, Alexis; Brashear, Allison; Clapcote, Steven J.; Gurrieri, Fiorella; Goldstein, David B.; Jóhannesson, Sigurður H.; Mikati, Mohamad A.; Neville, Brian; Nicole, Sophie; Ozelius, Laurie J.; Poulsen, Hanne; Schyns, Tsveta; Sweadner, Kathleen J.; van den Maagdenberg, Arn; Vilsen, Bente

    2014-01-01

    Genetic research has shown that mutations that modify the protein-coding sequence of ATP1A3, the gene encoding the α3 subunit of Na+/K+-ATPase, cause both rapid-onset dystonia parkinsonism and alternating hemiplegia of childhood. These discoveries link two clinically distinct neurological diseases to the same gene, however, ATP1A3 mutations are, with one exception, disease-specific. Although the exact mechanism of how these mutations lead to disease is still unknown, much knowledge has been gained about functional consequences of ATP1A3 mutations using a range of in vitro and animal model systems, and the role of Na+/K+-ATPases in the brain. Researchers and clinicians are attempting to further characterise neurological manifestations associated with mutations in ATP1A3, and to build on the existing molecular knowledge to understand how specific mutations can lead to different diseases. PMID:24739246

  2. Increased pediatric functional neurological symptom disorders after the Boston marathon bombings: a case series.

    Science.gov (United States)

    Guerriero, Réjean M; Pier, Danielle B; de Gusmão, Claudio M; Bernson-Leung, Miya E; Maski, Kiran P; Urion, David K; Waugh, Jeff L

    2014-11-01

    Functional neurological symptom disorders are frequently the basis for acute neurological consultation. In children, they are often precipitated by high-frequency everyday stressors. The extent to which a severe traumatic experience may also precipitate functional neurological abnormalities is unknown. For the 2-week period after the Boston Marathon bombings, we prospectively collected data on patients whose presentation suggested a functional neurological symptom disorder. We assessed clinical and demographic variables, duration of symptoms, extent of educational impact, and degree of connection to the Marathon bombing. We contacted all patients at 6 months after presentation to determine the outcome and accuracy of the diagnosis. In a parallel study, we reported a baseline of 2.6 functional neurological presentations per week in our emergency room. In the week after the Marathon bombings, this frequency tripled. Ninety-one percent of presentations were delayed by 1 week, with onset around the first school day after a city-wide lockdown. Seventy-three percent had a history of a prior psychiatric diagnosis. At the 6 months follow-up, no functional neurological symptom disorder diagnoses were overturned and no new organic diagnosis was made. Pediatric functional neurological symptom disorder may be precipitated by both casual and high-intensity stressors. The 3.4-fold increase in incidence after the Boston Marathon bombings and city-wide lockdown demonstrates the marked effect that a community-wide tragedy can have on the mental health of children. Care providers must be aware of functional neurological symptom disorders after stressful community events in vulnerable patient populations, particularly those with prior psychiatric diagnoses. Copyright © 2014 Elsevier Inc. All rights reserved.

  3. Household food insecurity and symptoms of neurologic disorder in Ethiopia: An observational analysis

    Directory of Open Access Journals (Sweden)

    Tessema Fasil

    2010-12-01

    Full Text Available Abstract Background Food insecurity (FI has been shown to be associated with poor health both in developing and developed countries. Little is known about the relation between FI and neurological disorder. We assessed the relation between FI and risk for neurologic symptoms in southwest Ethiopia. Methods Data about food security, gender, age, household assets, and self-reported neurologic symptoms were collected from a representative, community-based sample of adults (N = 900 in Jimma Zone, Ethiopia. We calculated univariate statistics and used bivariate chi-square tests and multivariate logistic regression models to assess the relation between FI and risk of neurologic symptoms including seizures, extremity weakness, extremity numbness, tremors/ataxia, aphasia, carpal tunnel syndrome, vision dysfunction, and spinal pain. Results In separate multivariate models by outcome and gender, adjusting for age and household socioeconomic status, severe FI was associated with higher odds of seizures, movement abnormalities, carpal tunnel, vision dysfunction, spinal pain, and comorbid disorders among women. Severe FI was associated with higher odds of seizures, extremity numbness, movement abnormalities, difficulty speaking, carpal tunnel, vision dysfunction, and comorbid disorders among men. Conclusion We found that FI was associated with symptoms of neurologic disorder. Given the cross-sectional nature of our study, the directionality of these associations is unclear. Future research should assess causal mechanisms relating FI to neurologic symptoms in sub-Saharan Africa.

  4. Den diabetiske Charcots fod

    DEFF Research Database (Denmark)

    Christensen, Tomas Møller; Yderstraede, Knud; Ejskjaer, Niels

    2008-01-01

    Charcot's arthropathy is a rare complication to diabetes with peripheral neuropathy. The diagnosis is based on a red, oedematous foot with 2 degrees C difference in skin temperature between the affected foot compared to the unaffected foot. The condition is characterised by fractures, dislocation...... of joints and deformity. The treatment is off-loading. When the off-loading treatment is completed, the patient should gradually adjust to wearing bespoken shoes. Lifelong control of the feet and shoes is necessary. Udgivelsesdato: 2008-Aug-11...

  5. Den diabetiske Charcots fod

    DEFF Research Database (Denmark)

    Christensen, T.M.; Yderstraede, K.; Ejskjaer, N.

    2008-01-01

    Charcot's arthropathy is a rare complication to diabetes with peripheral neuropathy. The diagnosis is based on a red, oedematous foot with 2 degrees C difference in skin temperature between the affected foot compared to the unaffected foot. The condition is characterised by fractures, dislocation...... of joints and deformity. The treatment is off-loading. When the off-loading treatment is completed, the patient should gradually adjust to wearing bespoken shoes. Lifelong control of the feet and shoes is necessary Udgivelsesdato: 2008/8/11...

  6. The "urban myth" of the association between neurological disorders and vaccinations.

    Science.gov (United States)

    Gasparini, R; Panatto, D; Lai, P L; Amicizia, D

    2015-06-10

    In modern society, a potentially serious adverse event attributed to a vaccination is likely to be snapped up by the media, particularly newspapers and television, as it appeals to the emotions of the public. The widespread news of the alleged adverse events of vaccination has helped to create the "urban myth" that vaccines cause serious neurological disorders and has boosted antivaccination associations. This speculation is linked to the fact that the true causes of many neurological diseases are largely unknown. The relationship between vaccinations and the onset of serious neuropsychiatric diseases is certainly one of coincidence rather than causality. This claim results from controlled studies that have excluded the association between vaccines and severe neurological diseases, therefore it can be said, with little risk of error, that the association between modern vaccinations and serious neurological disorders is a true "urban myth". © Copyright by Pacini Editore SpA, Pisa, Italy.

  7. The Spanish Burden of Disease 2010: Neurological, mental and substance use disorders.

    Science.gov (United States)

    Lara, Elvira; Garin, Noé; Ferrari, Alize J; Tyrovolas, Stefanos; Olaya, Beatriz; Sànchez-Riera, Lidia; Whiteford, Harvey A; Haro, Josep Maria

    2015-01-01

    We used data from the Global Burden of Disease, Injuries, and Risk Factors Study 2010 to report on the burden of neuropsychiatric disorders in Spain. The summary measure of burden used in the study was the disability-adjusted life-year (DALY), which sums of the years of life lost due to premature mortality (YLLs) and the years lived with disability (YLDs). DALYs were adjusted for comorbidity and estimated with 95% uncertainty intervals. The burden of neuropsychiatric disorders accounted for 18.4% of total all-cause DALYs generated in Spain for 2010. Within this group, the top five leading causes of DALYs were: depressive disorders, Alzheimer's disease, migraine, substance-use disorders, and anxiety disorder, which accounted for 70.9% of all DALYs due to neuropsychiatric disorders. Neurological disorders represented 5.03% of total all cause YLLs, whereas mental and substance-use disorders accounted for 0.8%. Mental and substance-use disorders accounted for 22.4% of total YLDs, with depression being the most disabling disorder. Neurological disorders represented 8.3% of total YLDs. Neuropsychiatric disorders were one of the leading causes of disability in 2010. This finding contributes to our understanding of the burden of neuropsychiatric disorders in the Spanish population and highlights the importance of prioritising neuropsychiatric disorders in the Spanish public health system. Copyright © 2014 SEP y SEPB. Published by Elsevier España. All rights reserved.

  8. Current perspectives on deep brain stimulation for severe neurological and psychiatric disorders

    Directory of Open Access Journals (Sweden)

    Kocabicak E

    2015-04-01

    Full Text Available Ersoy Kocabicak,1–3 Yasin Temel,1,2 Anke Höllig,4 Björn Falkenburger,5 Sonny KH Tan2,4 1Department of Neurosurgery, Maastricht University Medical Centre, 2Department of Neuroscience, Maastricht University, Maastricht, the Netherlands; 3Department of Neurosurgery, Ondokuz Mayis University, Samsun, Turkey; 4Department of Neurosurgery, 5Department of Neurology, RWTH Aachen University, Aachen, Germany Abstract: Deep brain stimulation (DBS has become a well-accepted therapy to treat movement disorders, including Parkinson’s disease, essential tremor, and dystonia. Long-term follow-up studies have demonstrated sustained improvement in motor symptoms and quality of life. DBS offers the opportunity to selectively modulate the targeted brain regions and related networks. Moreover, stimulation can be adjusted according to individual patients’ demands, and stimulation is reversible. This has led to the introduction of DBS as a treatment for further neurological and psychiatric disorders and many clinical studies investigating the efficacy of stimulating various brain regions in order to alleviate severe neurological or psychiatric disorders including epilepsy, major depression, and obsessive–compulsive disorder. In this review, we provide an overview of accepted and experimental indications for DBS therapy and the corresponding anatomical targets. Keywords: deep brain stimulation, movement disorders, neurological disorders, psychiatric disorders, Parkinson’s disease

  9. Male sexual dysfunction and infertility associated with neurological disorders

    Science.gov (United States)

    Fode, Mikkel; Krogh-Jespersen, Sheila; Brackett, Nancy L; Ohl, Dana A; Lynne, Charles M; Sønksen, Jens

    2012-01-01

    Normal sexual and reproductive functions depend largely on neurological mechanisms. Neurological defects in men can cause infertility through erectile dysfunction, ejaculatory dysfunction and semen abnormalities. Among the major conditions contributing to these symptoms are pelvic and retroperitoneal surgery, diabetes, congenital spinal abnormalities, multiple sclerosis and spinal cord injury. Erectile dysfunction can be managed by an increasingly invasive range of treatments including medications, injection therapy and the surgical insertion of a penile implant. Retrograde ejaculation is managed by medications to reverse the condition in mild cases and in bladder harvest of semen after ejaculation in more severe cases. Anejaculation might also be managed by medication in mild cases while assisted ejaculatory techniques including penile vibratory stimulation and electroejaculation are used in more severe cases. If these measures fail, surgical sperm retrieval can be attempted. Ejaculation with penile vibratory stimulation can be done by some spinal cord injured men and their partners at home, followed by in-home insemination if circumstances and sperm quality are adequate. The other options always require assisted reproductive techniques including intrauterine insemination or in vitro fertilization with or without intracytoplasmic sperm injection. The method of choice depends largely on the number of motile sperm in the ejaculate. PMID:22138899

  10. Salvage arthrodesis for charcot arthropathy.

    Science.gov (United States)

    Panagakos, Panagiotis; Ullom, Nathan; Boc, Steven F

    2012-01-01

    The principles of fusion of a Charcot joint arise from the assertion that successful fusion requires removal of all cartilage, debris, and sclerotic bone. The authors believe that reconstruction can prevent amputation in patients who have unbraceable or unstable deformities, or recurrent ulcerations. The goal with any Charcot reconstruction procedure is to achieve a plantigrade foot free of ulceration, and to prevent any future collapse, deformity, or ulcerations. The authors strongly believe arthrodesis of unstable joints of the Charcot neuropathic foot can lead to limb salvage and better quality of life. Copyright © 2012 Elsevier Inc. All rights reserved.

  11. Neurological disorders and dependence of nursing care in patients with brain tumor

    OpenAIRE

    Guzzo Souza, Renata; K. Olm Cunha, Isabel Cristina; Franchini Reichert, Magaly Cecília; Diccini,Solange

    2008-01-01

    The aim of this study was to identify neurological disorders in 32 patients with diagnosis of brain tumor, and to evaluate the dependency of nursing care before and after the surgery. This study was carried out at the Neurosurgery Unit of São Paulo Hospital, Brazil, from June to December, 2004. One instrument was used to collect physical and neurological data, and another to evaluate the dependence degree. An increase of the dependence degree was observed after surgical intervention, which th...

  12. Gait variability in people with neurological disorders: A systematic review and meta-analysis.

    Science.gov (United States)

    Moon, Yaejin; Sung, JongHun; An, Ruopeng; Hernandez, Manuel E; Sosnoff, Jacob J

    2016-06-01

    There has been growing evidence showing gait variability provides unique information about gait characteristics in neurological disorders. This study systemically reviewed and quantitatively synthesized (via meta-analysis) existing evidence on gait variability in various neurological diseases, including Alzheimer's disease (AD), amyotrophic lateral sclerosis (ALS), cerebellar ataxia (CA), Huntington's disease (HD), multiple sclerosis (MS), and Parkinson's disease (PD). Keyword search were conducted in PubMed, Web of science, Cumulative Index to Nursing and Allied Health Literature, and Cochrane Library. Meta-analysis was performed to estimate the pooled effect size for gait variability for each neurological group. Meta-regression was performed to compare gait variability across multiple groups with neurological diseases. Gait variability of 777 patients with AD, ALS, CA, HD, MS, or PD participating in 25 studies was included in meta-analysis. All pathological groups had increased amount of gait variability and loss of fractal structure of gait dynamics compared to healthy controls, and gait variability differentiated distinctive neurological conditions. The HD groups had the highest alterations in gait variability among all pathological groups, whereas the PD, AD and MS groups had the lowest. Interventions that aim to improve gait function in patients with neurological disorders should consider the heterogeneous relationship between gait variability and neurological conditions. Copyright © 2016 Elsevier B.V. All rights reserved.

  13. An Analysis of Disorders seen at the Paediatric Neurology Clinic ...

    African Journals Online (AJOL)

    daddy

    45.3%), cerebral palsy (36.0%), neuro-muscular disorders (4.5%) and mental retardation. (4.5%). ... Correspondence: Dr I. A. Lagunju, Department of Paediatrics, University College Hospital, Ibadan. ... considerable burden on the family, society.

  14. Neurological Soft Signs In Psychoses A Comparison Between Schizophrenia & Other Psychotic Disorders

    Directory of Open Access Journals (Sweden)

    Shahsavand. E. Noroozian. M

    2002-07-01

    Full Text Available Schizophrenia is one of the most important and disabling mental disorders in the world. Males and females are equally affected. Diagnosis is a very difficult problem in this disorder. Because the diagnostic systems such as ICD-10 and DSM-IV are mainly subjective, they are not valid and reliable. Essentially, in the future, we will need to more objective criteria in psychiatry especially in diagnosis of schizophrenia. Neurological soft signs are an example of these objective criteria. In this study we evaluated the prevalence of neurological soft signs in schizophrenic patients and compared it with the prevalence of these signs in other psychotic patients (except mood disorders with psychotic features and normal subjects."nMethods: We compared the neurological soft signs (sensory motor integration, motor. Coordination, consequent complex motor acts, primary reflexes, and eye movements in 30 schizophrenic patients, 30 other psychotic patients (other than mood disorders with psychotic features and 30 normal subjects. Diagnosis of schizophrenia and also other psychoses were based on DSM-IN criteria. Normal subjects have been selected form the staff of Roozbeh hospital randomly."nResults: The difference between the means of motor coordination subscale of neurological soft signs in schizophrenia and other psychotic disorders (other than mood disorders with psychotic features were significant (P value < 0.04. There were no significant differences between the means of other subscales of neurological soft signs in two groups of patients."nConclusion: There are some disturbances of motor coordination subscale of neurological soft signs in patients with schizophrenia. It seems that, these disturbances are evidence of involvements of basal ganglia, motor cerebral cortex, and cerebellum. So it may be suggested that motor coordination as a marker can be used in differentiation between the schizophrenia and other psychotic disorders.

  15. Occurrence of communication and swallowing problems in neurological disorders: analysis of forty patients.

    Science.gov (United States)

    Jani, Mansi Pankaj; Gore, Geeta Bharat

    2014-01-01

    Communication and swallowing problems are common as a result of neurological conditions like stroke, traumatic brain injury, neoplasms of the nervous systems, viral encephalitis, diseases affecting neuromuscular junction and neuro degenerative conditions. The most frequently encountered problems are dysarthria, aphasia, dysphagia and apraxia of speech. Although these disorders are mentioned in literature, very few studies describing the occurrence in different neurological conditions are available in Indian context. Hence, a need was felt to carry out such a study. A heterogenous group of forty patients with neurological conditions were assessed for presence of speech, language and swallowing problems. A percent analysis was carried out to determine the occurrence of aphasia, dysarthria and dysphagia in general, in specific diseases and also to describe type of aphasia and dysarthria according to the characteristics presented. It was seen that the most frequently occurring disorder was dysarthria (60%), followed by dysphagia (55%) and aphasia (18%). It was also noted that dysarthria and dysphagia co-existed in around 45% patients with neurological diseases. It can be concluded that speech, language and swallowing problems are frequent in individuals with neurological conditions. Speech language pathologist plays an important role as a member of the rehabilitation team in a neurological setup with respect to identifying these problems and initiating intervention at the earliest. Hence, it is necessary for speech language pathologist to be well versed with the features each disorder may present with in terms of communication and swallowing.

  16. A Comprehensive Atlas of E3 Ubiquitin Ligase Mutations in Neurological Disorders

    Directory of Open Access Journals (Sweden)

    Arlene J. George

    2018-02-01

    Full Text Available Protein ubiquitination is a posttranslational modification that plays an integral part in mediating diverse cellular functions. The process of protein ubiquitination requires an enzymatic cascade that consists of a ubiquitin activating enzyme (E1, ubiquitin conjugating enzyme (E2 and an E3 ubiquitin ligase (E3. There are an estimated 600–700 E3 ligase genes representing ~5% of the human genome. Not surprisingly, mutations in E3 ligase genes have been observed in multiple neurological conditions. We constructed a comprehensive atlas of disrupted E3 ligase genes in common (CND and rare neurological diseases (RND. Of the predicted and known human E3 ligase genes, we found ~13% were mutated in a neurological disorder with 83 total genes representing 70 different types of neurological diseases. Of the E3 ligase genes identified, 51 were associated with an RND. Here, we provide an updated list of neurological disorders associated with E3 ligase gene disruption. We further highlight research in these neurological disorders and discuss the advanced technologies used to support these findings.

  17. Charcot foot syndrome.

    Science.gov (United States)

    Jeffcoate, W J

    2015-06-01

    Charcot foot syndrome is an uncommon complication of diabetes but is potentially devastating in its consequences. Outcome is made worse by widespread professional ignorance leading to delayed diagnosis, but it is also hampered by lack of understanding of its causes and lack of treatments with proven effectiveness, other than offloading. There remains a desperate need for studies into its causes as well as comparative audit and trials designed to determine the best treatment for this difficult condition. Such work can probably only be effectively carried out through the establishment of multicentre networks. Nevertheless, improved understanding in recent years of the likely role of inflammatory pathways has raised awareness of the multiple ways in which the effects of neuropathy may be manifest in the development of the Charcot foot. This awareness is also leading to the realization that similar processes may conceivably contribute to the refractoriness of other foot diseases in diabetes, including both chronic unhealing ulcers and osteomyelitis. © 2015 The Author. Diabetic Medicine © 2015 Diabetes UK.

  18. Manipulations of MeCP2 in glutamatergic neurons highlight their contributions to Rett and other neurological disorders

    Science.gov (United States)

    Many postnatal onset neurological disorders such as autism spectrum disorders (ASDs) and intellectual disability are thought to arise largely from disruption of excitatory/inhibitory homeostasis. Although mouse models of Rett syndrome (RTT), a postnatal neurological disorder caused by loss-of-functi...

  19. Advantages of Structure-Based Drug Design Approaches in Neurological Disorders.

    Science.gov (United States)

    Aarthy, Murali; Panwar, Umesh; Selvaraj, Chandrabose; Singh, Sanjeev Kumar

    2017-11-14

    The purpose of the review is to portray the theoretical concept on neurological disorders from research data. The freak changes in chemical response of nerve impulse causes neurological disorders. The research evidence of the effort done in the older history suggests that the biological drug targets and their effective feature with responsive drugs could be valuable in promoting the future development of health statistics structure for improved treatment for curing the nervous disorders. In this review, we summarized the most iterative theoretical concept of structure based drug design approaches in various neurological disorders to unfathomable understanding of reported information for future drug design and development. On the premise of reported information we analyzed the model of theoretical drug designing process for understanding the mechanism and pathology of the neurological diseases which covers the development of potentially effective inhibitors against the biological drug targets. Finally, it also suggests the management and implementation of the current treatment in improving the human health system behaviors. With the survey of reported information we concluded the development strategies of diagnosis and treatment against neurological diseases which leads to supportive progress in the drug discovery. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  20. Review on Graph Clustering and Subgraph Similarity Based Analysis of Neurological Disorders.

    Science.gov (United States)

    Thomas, Jaya; Seo, Dongmin; Sael, Lee

    2016-06-01

    How can complex relationships among molecular or clinico-pathological entities of neurological disorders be represented and analyzed? Graphs seem to be the current answer to the question no matter the type of information: molecular data, brain images or neural signals. We review a wide spectrum of graph representation and graph analysis methods and their application in the study of both the genomic level and the phenotypic level of the neurological disorder. We find numerous research works that create, process and analyze graphs formed from one or a few data types to gain an understanding of specific aspects of the neurological disorders. Furthermore, with the increasing number of data of various types becoming available for neurological disorders, we find that integrative analysis approaches that combine several types of data are being recognized as a way to gain a global understanding of the diseases. Although there are still not many integrative analyses of graphs due to the complexity in analysis, multi-layer graph analysis is a promising framework that can incorporate various data types. We describe and discuss the benefits of the multi-layer graph framework for studies of neurological disease.

  1. Fibrinolytic activity in cerebrospinal fluid of dogs with different neurological disorders.

    Science.gov (United States)

    de la Fuente, C; Monreal, L; Cerón, J; Pastor, J; Viu, J; Añor, S

    2012-01-01

    Fibrinolytic activity in cerebrospinal fluid (CSF) is activated in humans by different pathologic processes. To investigate fibrinolytic activity in the CSF of dogs with neurological disorders by measuring CSF D-dimer concentrations. One hundred and sixty-nine dogs with neurological disorders, 7 dogs with systemic inflammatory diseases without central nervous system involvement (SID), and 7 healthy Beagles were included in the study. Dogs with neurological disorders included 11 with steroid-responsive meningitis-arteritis (SRMA), 37 with other inflammatory neurological diseases (INF), 38 with neoplasia affecting the central nervous system (NEO), 28 with spinal compressive disorders (SCC), 15 with idiopathic epilepsy (IE), and 40 with noninflammatory neurological disorders (NON-INF). Prospective observational study. D-dimers and C-reactive protein (CRP) were simultaneously measured in paired CSF and blood samples. D-dimers and CRP were detected in 79/183 (43%) and in 182/183 (99.5%) CSF samples, respectively. All dogs with IE, SID, and controls had undetectable concentrations of D-dimers in the CSF. CSF D-dimer concentrations were significantly (P dogs with SRMA than in dogs with other diseases and controls. CSF CRP concentration in dogs with SRMA was significantly (P dogs of other groups and controls, except for the SID group. No correlation was found between blood and CSF D-dimer concentrations. Intrathecal fibrinolytic activity seems to be activated in some canine neurological disorders, and it is high in severe meningeal inflammatory diseases. CSF D-dimer concentrations may be considered a diagnostic marker for SRMA. Copyright © 2012 by the American College of Veterinary Internal Medicine.

  2. [Application of whole exome sequencing in the diagnosis of hereditary neurological diseases].

    Science.gov (United States)

    Ilinsky, V V; Korneeva, V A; Shatalov, P A

    2015-01-01

    Whole Exome Sequencing (WES) is a promising method in human genetics. Because the majority of pathogenic mutations that lead to the development of diseases are localized in exons and splice sites, WES could become a major tool for the diagnosis of diseases with a complex hereditary nature. This tool appears to be particularly useful for hereditary neurological diseases, such as autism spectrum disorders, Charcot-Marie-Tooth disease and others. In our review, we discuss the clinical application of WES, with special emphasis on the diagnosis of hereditary neurological diseases.

  3. Stress, childhood trauma, and cognitive functions in functional neurologic disorders

    NARCIS (Netherlands)

    Roelofs, K.; Pasman, J.A.

    2017-01-01

    Conversion disorder (CD) has traditionally been ascribed to psychologic factors such as trauma, stress, or emotional conflict. Although reference to the psychologic origin of CD has been removed from the criteria list in DSM-5, many theories still incorporate CD as originating from adverse events.

  4. Potential of robots as next-generation technology for clinical assessment of neurological disorders and upper-limb therapy

    National Research Council Canada - National Science Library

    Scott, Stephen H; Dukelow, Sean P

    2011-01-01

    .... Our general hypothesis is that these same attributes make robotic technologies ideal for clinically assessing sensory, motor, and cognitive impairments in stroke and other neurological disorders...

  5. Optimization of nutritional correction treatment for neurological disorders in children

    Directory of Open Access Journals (Sweden)

    L. A. Tekebaeva

    2016-01-01

    Full Text Available The paper gives the results of a study project at the Infant Neurology Department, which has shown the urgency of nutritional rehabilitation in children aged 3 months to 5 years with infantile cerebral paralysis. Thirty patients were followed up to study the causes of malnutrition, to assess and correct their nutritional status, and to show the efficiency of the measures implemented. A diet corrected by a nutritionist was combined with nonsurgical interventions, such as positioning, a decision on the transition to nasogastric tube feeding, and maternal work. The complementary foods were FrutoNyanya products as the latter are low-immunogenic, cause no allergic reactions, and may be used both in the feeding of high-risk group children and as ingredients of a therapeutic diet for patients with different diseases. This resulted in 305-g weight gain within 7-10 day of hospital stay in those whose underweight averaged 28%. The emotional status of the patients and their caregivers was improved by 2-3 scores on 5-point rating scale. There were improvements in their emotional tone (in 75%, chewing (in 28%, and swallowing (in 35% and reductions in reflux episodes (in 19% and stress in the caregivers (in 86%.

  6. Clinical Application of Adsorption Therapy for Neurological Disorders

    OpenAIRE

    松尾, 秀徳; Hidenori, Matsuo; 国立療養所川棚病院神経内科; Department of Neurology, Kawatana National Hospital

    2000-01-01

    Immunoadsorption plasmapheresis (IAPP) has been widely applied for the treatment of neuroimmunological disorders, including myasthenia gravis (MG). Guillain-Barre syndrome (GBS), chronic iuflammatory demyelinating polyneuropathy (CIDP). and multiple sclerosis (MS). In antibody-mediated autoimmune disease. such as MG, IAPP seems more rational and efficiently removes causative autoantibodies without need for replacement fluid. On the other hand, in T cell mediated neuroimmunological disease, th...

  7. [Psychosocial adjustment difficulties among hospitalized adolescents with neurological and physical disorders].

    Science.gov (United States)

    Gostautas, Antanas; Pakrosnis, Rytis; Krikscionaityte, Simona; Endziniene, Milda; Sergutina, Irina; Glamba, Vitalijus

    2006-01-01

    Psychosocial adjustment of adolescents with health disorders has been studied extensively. However, it is unclear whether different health disorders have specific impact on adjustment, or disorder in general relates to the development of adjustment difficulties as a factor limiting adolescent's physical and social activity. This study was aimed to identify peculiarities of psychosocial adjustment difficulties among hospitalized adolescents with neurological and physical disorders and secondary school students. The sample consisted of 738 adolescents (aged 12-18 years), of which 100 had neurological and 94 - physical disorders, and 544 secondary school students. Psychosocial adjustment difficulties were evaluated using Standardized Interview for the Evaluation of Adolescents' Problems. Results show that emotional-mood difficulties and low self-esteem are more prevalent and severe in the clinical groups comparing with the healthy controls, while the prevalence and the severity of conflicts are higher among students. Comparison of both clinical groups shows that neurological disorders are related to higher prevalence of difficulties in emotional and cognitive functioning and higher severity of suicidal tendencies, while physical disorders relate to higher prevalence of behavior difficulties.

  8. Charcot foot and ankle with osteomyelitis

    Science.gov (United States)

    Donegan, Ryan; Sumpio, Bauer; Blume, Peter A.

    2013-01-01

    This paper presents a review of the current literature discussing topics of Charcot osteoarthropathy, osteomyelitis, diagnosing osteomyelitis, antibiotic management of osteomyelitis, and treatment strategies for management of Charcot osteoarthropathy with concurrent osteomyelitis. PMID:24098835

  9. Current perspectives on deep brain stimulation for severe neurological and psychiatric disorders

    Science.gov (United States)

    Kocabicak, Ersoy; Temel, Yasin; Höllig, Anke; Falkenburger, Björn; Tan, Sonny KH

    2015-01-01

    Deep brain stimulation (DBS) has become a well-accepted therapy to treat movement disorders, including Parkinson’s disease, essential tremor, and dystonia. Long-term follow-up studies have demonstrated sustained improvement in motor symptoms and quality of life. DBS offers the opportunity to selectively modulate the targeted brain regions and related networks. Moreover, stimulation can be adjusted according to individual patients’ demands, and stimulation is reversible. This has led to the introduction of DBS as a treatment for further neurological and psychiatric disorders and many clinical studies investigating the efficacy of stimulating various brain regions in order to alleviate severe neurological or psychiatric disorders including epilepsy, major depression, and obsessive–compulsive disorder. In this review, we provide an overview of accepted and experimental indications for DBS therapy and the corresponding anatomical targets. PMID:25914538

  10. The Significance of the Default Mode Network (DMN) in Neurological and Neuropsychiatric Disorders: A Review.

    Science.gov (United States)

    Mohan, Akansha; Roberto, Aaron J; Mohan, Abhishek; Lorenzo, Aileen; Jones, Kathryn; Carney, Martin J; Liogier-Weyback, Luis; Hwang, Soonjo; Lapidus, Kyle A B

    2016-03-01

    The relationship of cortical structure and specific neuronal circuitry to global brain function, particularly its perturbations related to the development and progression of neuropathology, is an area of great interest in neurobehavioral science. Disruption of these neural networks can be associated with a wide range of neurological and neuropsychiatric disorders. Herein we review activity of the Default Mode Network (DMN) in neurological and neuropsychiatric disorders, including Alzheimer's disease, Parkinson's disease, Epilepsy (Temporal Lobe Epilepsy - TLE), attention deficit hyperactivity disorder (ADHD), and mood disorders. We discuss the implications of DMN disruptions and their relationship to the neurocognitive model of each disease entity, the utility of DMN assessment in clinical evaluation, and the changes of the DMN following treatment.

  11. Detection and analysis of Borna disease virus in Chinese patients with neurological disorders.

    Science.gov (United States)

    Li, Q; Wang, Z; Zhu, D; Xu, M; Chen, X; Peng, D; Iwata, Y; Xie, P

    2009-03-01

    Borna disease virus (BDV) is a neurotropic RNA virus that is known to cause neurological disturbances in various animal species, potentially even humans. However, the association between BDV infection and human neurological disorders remains unclear. Between August 2005 and March 2006, 65 patients with neurological disorders were enrolled into our study. The presence of BDV p24 RNA from peripheral blood mononuclear cells (PBMCs) was investigated by using nested reverse transcriptase PCR (RT-PCR) assay. Borna disease virus p24 RNA was detected from PBMCs in six patients with viral encephalitis by using nested RT-PCR assay. However, BDV p24 RNA was not detected in patients with multiple sclerosis or peripheral nerve diseases. There might be possible associations between BDV infection and human viral encephalitis.

  12. Quantitative Evaluation System of Soft Neurological Signs for Children with Attention Deficit Hyperactivity Disorder

    Directory of Open Access Journals (Sweden)

    Miki Kaneko

    2016-01-01

    Full Text Available Attention deficit hyperactivity disorder (ADHD is a neurodevelopmental disorder characterized by symptoms of inattention, hyperactivity, and impulsivity. Soft neurological signs (SNS are minor neurological abnormalities in motor performance, and are used as one evaluation method for neurodevelopmental delays in children with ADHD. Our aim is to establish a quantitative evaluation system for children with ADHD. We focused on the arm movement called pronation and supination, which is one such soft neurological sign. Thirty three children with ADHD aged 7–11 years (27 males, six females and twenty five adults participants aged 21–29 years old (19 males, six females participated in our experiments. Our results suggested that the pronation and supination function in children with ADHD has a tendency to lag behind that of typically developing children by several years. From these results, our system has a possibility to objectively evaluate the neurodevelopmental delay of children with ADHD.

  13. The core competencies for mental, neurological, and substance use disorder care in sub-Saharan Africa.

    OpenAIRE

    Collins, PY; Musisi, S; Frehywot, S; Patel, V

    2015-01-01

    The 2010 Global Burden of Disease Study points to a changing landscape in which non-communicable diseases, such as mental, neurological, and substance use (MNS) disorders, account for an increasing proportion of premature mortality and disability globally. Despite evidence of the need for care, a remarkable deficit of providers for MNS disorder service delivery persists in sub-Saharan Africa. This critical workforce can be developed from a range of non-specialist and specialist health workers...

  14. 78 FR 70310 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Science.gov (United States)

    2013-11-25

    ... Stroke Special Emphasis Panel; Neuroscience Research Education (R25). Date: December 16, 2013. Time: 2:00... Health, Neuroscience Center, 6001 Executive Boulevard, Rockville, MD 20852 (Telephone Conference Call..., Clinical Research Related to Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences...

  15. 76 FR 10381 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Science.gov (United States)

    2011-02-24

    ... cycle (Catalogue of Federal Domestic Assistance Program Nos. 93.853, Clinical Research Related to Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences, National Institutes of Health... Emphasis Panel; Medicinal Chemistry. Date: February 28, 2011. Time: 1 p.m. to 5 p.m. Agenda: To review and...

  16. 75 FR 64315 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Science.gov (United States)

    2010-10-19

    .... [email protected] . (Catalogue of Federal Domestic Assistance Program Nos. 93.853, Clinical Research Related to Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences, National... Emphasis Panel, Medicinal Chemistry. Date: November 9-10, 2010. Time: 8 a.m. to 5 p.m. Agenda: To review...

  17. Population-based studies on risk of fracture in patients with neurological disorders

    NARCIS (Netherlands)

    Pouwels, S.|info:eu-repo/dai/nl/34158990X

    2014-01-01

    Introduction Patients with neurological disorders may be at an increased risk of fracture via multiple causal pathways, including increases in the risk of falls, changes in bone mineral density and quality of bone microarchitecture. Risk of fracture may be increased by the disease itself, by

  18. Maple Syrup Urine Disease (MSUD detected in neurologic disorders Iraqi children

    Directory of Open Access Journals (Sweden)

    Adel A. Kareem

    2016-09-01

    Conclusion In the absence of newborn screening, MSUD is not uncommon in neurologically disorder patients where MSUD was still diagnosed clinically, but delayed. The importance of clinical awareness and accurate biochemical analysis were the key tools for diagnosis and the necessity for a comprehensive national newborn screening program.

  19. 75 FR 57971 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Science.gov (United States)

    2010-09-23

    ..., Review Group, Neurological Sciences and Disorders A. Date: November 3, 2010. Time: 8 a.m. to 6 p.m. Agenda: To review and evaluate grant applications. Place: Embassy Suites Hotel, 4300 Military Road... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND...

  20. Current Issues in the Neurology and Genetics of Learning-Related Traits and Disorders: Introduction to the Special Issue.

    Science.gov (United States)

    Gilger, Jeffrey W.

    2001-01-01

    This introductory article briefly describes each of the following eight articles in this special issue on the neurology and genetics of learning related disorders. It notes the greater appreciation of learning disability as a set of complex disorders with broad and intricate neurological bases and of the large individual differences in how these…

  1. Stress, childhood trauma, and cognitive functions in functional neurologic disorders.

    Science.gov (United States)

    Roelofs, K; Pasman, J

    2016-01-01

    Conversion disorder (CD) has traditionally been ascribed to psychologic factors such as trauma, stress, or emotional conflict. Although reference to the psychologic origin of CD has been removed from the criteria list in DSM-5, many theories still incorporate CD as originating from adverse events. This chapter provides a critical review of the literature on stressful life events in CD and discusses current cognitive and neurobiologic models linking psychologic stressors with conversion symptomatology. In addition, we propose a neurobiologic stress model integrating those cognitive models with neuroendocrine stress research and propose that stress and stress-induced changes in hypothalamus-pituitary-adrenal (HPA) axis function may result in cognitive alterations, that in turn contribute to experiencing conversion symptoms. Experimental studies indeed suggest that basal as well as stress-induced changes in HPA axis responding lead to alterations in attentional processing in CD. Although those changes are stronger in traumatized patients, similar patterns have been observed in patients who do not report a history of traumatic events. We conclude that, whereas adverse events may play an important role in many cases of CD, a substantial proportion of patients do not report a history of traumatization or recent stressful events. Studies integrating effects of stress on cognitive functioning in CD are scarce. We propose that, instead of focusing research on defining etiologic events in terms of symptom-eliciting events, future research should work towards an integrated mechanistic account, assessing alterations in cognitive and biologic stress systems in an integrated manner in patients with CD. Such an account may not only serve early symptom detection, it might also provide a starting point for better-targeted interventions. © 2016 Elsevier B.V. All rights reserved.

  2. Neuroactive compounds obtained from arthropod venoms as new therapeutic platforms for the treatment of neurological disorders.

    Science.gov (United States)

    Monge-Fuentes, Victoria; Gomes, Flávia Maria Medeiros; Campos, Gabriel Avohay Alves; Silva, Juliana de Castro; Biolchi, Andréia Mayer; Dos Anjos, Lilian Carneiro; Gonçalves, Jacqueline Coimbra; Lopes, Kamila Soares; Mortari, Márcia Renata

    2015-01-01

    The impact of neurological disorders in society is growing with alarming estimations for an incidence increase in the next decades. These disorders are generally chronic and can affect individuals early during productive life, imposing real limitations on the performance of their social roles. Patients can have their independence, autonomy, freedom, self-image, and self-confidence affected. In spite of their availability, drugs for the treatment of these disorders are commonly associated with side effects, which can vary in frequency and severity. Currently, no effective cure is known. Nowadays, the biopharmaceutical research community widely recognizes arthropod venoms as a rich source of bioactive compounds, providing a plethora of possibilities for the discovery of new neuroactive compounds, opening up novel and attractive opportunities in this field. Several identified molecules with a neuropharmacological profile can act in the central nervous system on different neuronal targets, rendering them useful tools for the study of neurological disorders. In this context, this review aims to describe the current main compounds extracted from arthropod venoms for the treatment of five major existing neurological disorders: stroke, Alzheimer's disease, epilepsy, Parkinson's disease, and pathological anxiety.

  3. Diagnosis and Treatment of Neurological and Ischemic Disorders Employing Carbon Nanotube Technology

    Directory of Open Access Journals (Sweden)

    Patrick P. Komane

    2016-01-01

    Full Text Available Extensive research on carbon nanotubes has been conducted due to their excellent physicochemical properties. Based on their outstanding physicochemical properties, carbon nanotubes have the potential to be employed as theranostic tools for neurological pathologies such as Alzheimer’s disease and Parkinson’s disease including ischemic stroke diagnosis and treatment. Stroke is currently regarded as the third root cause of death and the leading source of immobility around the globe. The development and improvement of efficient and effective procedures for central nervous system disease diagnosis and treatment is necessitated. The main aim of this review is to discuss the application of nanotechnology, specifically carbon nanotubes, to the diagnosis and treatment of neurological disorders with an emphasis on ischemic stroke. Areas covered include the conventional current diagnosis and treatment of neurological disorders, as well as a critical review of the application of carbon nanotubes in the diagnosis and treatment of ischemic stroke, covering areas such as functionalization of carbon nanotubes and carbon nanotube-based biosensors. A broad perspective on carbon nanotube stimuli-responsiveness, carbon nanotube toxicity, and commercially available carbon nanotubes is provided. Potential future studies employing carbon nanotubes have been discussed, evaluating their extent of advancement in the diagnosis and treatment of neurological and ischemic disorders.

  4. Medical management of Charcot arthropathy.

    Science.gov (United States)

    Petrova, N L; Edmonds, M E

    2013-03-01

    Charcot arthropathy is a major complication of diabetes and it poses management challenges to health care professionals. Early diagnosis and timely intervention are essential for improved outlook of these patients. Casting therapy has been accepted as the mainstay treatment of the acute Charcot foot, although there are still controversies regarding its duration, the choice of removable and non-removable device and weight-bearing casts vs. non-weight-bearing casts. Two groups of antiresorptive therapies have been evaluated in the treatment of the acute Charcot foot, bisphosphonates (intravenous and oral) and calcitonin. These therapies have clearly shown a reduction of bone turnover, although, they have not shown a significant effect on temperature reduction. Current evidence to support their use is weak. An anabolic agent to speed up clinical resolution and fracture healing may be helpful and a clinical trial to evaluate the possible benefit of 1-84 recombinant human parathyroid hormone on fracture healing in the acute Charcot foot is in progress. This paper summarises the current approach to medical management of acute Charcot arthropathy with specific emphasis on casting and pharmacological therapy. Emerging new studies of the pathogenesis of this condition are also discussed. © 2012 Blackwell Publishing Ltd.

  5. [Neurological disorders after cardiac surgery: Diagnosis of cerebral tumors in the postoperative period].

    Science.gov (United States)

    López Álvarez, A; Rodríguez Fernández, P; Román Fernández, A; Filgueira Dávila, E; Gálvez Gómez, D; González Monzón, V

    2014-11-01

    The incidence of neurologic disorders in the postoperative period of cardiac surgery is high and usually due to a combination of pre- and intraoperative factors. We present 2 patients with brain tumors diagnosed in the immediate postoperative period after sudden onset of neurologic dysfunction. Image studies yielded clinically useful information in these 2 cases. Copyright © 2013 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

  6. The ketogenic diet as a treatment paradigm for diverse neurological disorders.

    Science.gov (United States)

    Stafstrom, Carl E; Rho, Jong M

    2012-01-01

    Dietary and metabolic therapies have been attempted in a wide variety of neurological diseases, including epilepsy, headache, neurotrauma, Alzheimer disease, Parkinson disease, sleep disorders, brain cancer, autism, pain, and multiple sclerosis. The impetus for using various diets to treat - or at least ameliorate symptoms of - these disorders stems from both a lack of effectiveness of pharmacological therapies, and also the intrinsic appeal of implementing a more "natural" treatment. The enormous spectrum of pathophysiological mechanisms underlying the aforementioned diseases would suggest a degree of complexity that cannot be impacted universally by any single dietary treatment. Yet, it is conceivable that alterations in certain dietary constituents could affect the course and impact the outcome of these brain disorders. Further, it is possible that a final common neurometabolic pathway might be influenced by a variety of dietary interventions. The most notable example of a dietary treatment with proven efficacy against a neurological condition is the high-fat, low-carbohydrate ketogenic diet (KD) used in patients with medically intractable epilepsy. While the mechanisms through which the KD works remain unclear, there is now compelling evidence that its efficacy is likely related to the normalization of aberrant energy metabolism. The concept that many neurological conditions are linked pathophysiologically to energy dysregulation could well provide a common research and experimental therapeutics platform, from which the course of several neurological diseases could be favorably influenced by dietary means. Here we provide an overview of studies using the KD in a wide panoply of neurologic disorders in which neuroprotection is an essential component.

  7. Oscillatory Activities in Neurological Disorders of Elderly: Biomarkers to Target for Neuromodulation

    Directory of Open Access Journals (Sweden)

    Giovanni Assenza

    2017-06-01

    Full Text Available Non-invasive brain stimulation (NIBS has been under investigation as adjunct treatment of various neurological disorders with variable success. One challenge is the limited knowledge on what would be effective neuronal targets for an intervention, combined with limited knowledge on the neuronal mechanisms of NIBS. Motivated on the one hand by recent evidence that oscillatory activities in neural systems play a role in orchestrating brain functions and dysfunctions, in particular those of neurological disorders specific of elderly patients, and on the other hand that NIBS techniques may be used to interact with these brain oscillations in a controlled way, we here explore the potential of modulating brain oscillations as an effective strategy for clinical NIBS interventions. We first review the evidence for abnormal oscillatory profiles to be associated with a range of neurological disorders of elderly (e.g., Parkinson’s disease (PD, Alzheimer’s disease (AD, stroke, epilepsy, and for these signals of abnormal network activity to normalize with treatment, and/or to be predictive of disease progression or recovery. We then ask the question to what extent existing NIBS protocols have been tailored to interact with these oscillations and possibly associated dysfunctions. Our review shows that, despite evidence for both reliable neurophysiological markers of specific oscillatory dis-functionalities in neurological disorders and NIBS protocols potentially able to interact with them, there are few applications of NIBS aiming to explore clinical outcomes of this interaction. Our review article aims to point out oscillatory markers of neurological, which are also suitable targets for modification by NIBS, in order to facilitate in future studies the matching of technical application to clinical targets.

  8. THE KETOGENIC DIET AS A TREATMENT PARADIGM FOR DIVERSE NEUROLOGICAL DISORDERS

    Directory of Open Access Journals (Sweden)

    Jong Min Rho

    2012-04-01

    Full Text Available Dietary and metabolic therapies have been attempted in a wide variety of neurological diseases, including epilepsy, headache, neurotrauma, Alzheimer disease, Parkinson disease, sleep disorders, brain cancer, autism, pain, and multiple sclerosis. The impetus for using various diets to treat – or at least ameliorate symptoms of – these disorders stems from both a lack of effectiveness of pharmacological therapies, and also the intrinsic appeal of implementing a more natural treatment. The enormous spectrum of pathophysiological mechanisms underlying the aforementioned diseases would suggest a degree of complexity that cannot be impacted universally by any single dietary treatment. Yet, it is conceivable that alterations in certain dietary constituents could affect the course and impact the outcome of these brain disorders. Further, it is possible that a final common neurometabolic pathway might be influenced by a variety of dietary interventions. The most notable example of a dietary treatment with proven efficacy against a neurological condition is the high-fat, low-carbohydrate ketogenic diet (KD used in patients with medically intractable epilepsy. While the mechanisms through which the KD works remain unclear, there is now compelling evidence that its efficacy is likely related to the normalization of aberrant energy metabolism. The concept that many neurological conditions are linked pathophysiologically to energy dysregulation could well provide a common research and experimental therapeutics platform, from which the course of several neurological diseases could be favorably influenced by dietary means. Here we provide an overview of studies using the KD in a wide panoply of neurologic disorders in which neuroprotection is an essential component.

  9. Prevalence of mental, neurological, and substance use disorders in China and India: a systematic analysis.

    Science.gov (United States)

    Baxter, Amanda J; Charlson, Fiona J; Cheng, Hui G; Shidhaye, Rahul; Ferrari, Alize J; Whiteford, Harvey A

    2016-09-01

    Population-representative prevalence data for mental, neurological, and substance use disorders are essential for evidence-based decision making. As a background to the China-India Mental Health Alliance Series, we aim to examine the availability of data and report prevalence for the most common mental, neurological, and substance use disorders in China and India from the Global Burden of Disease study 2013 (GBD 2013). In this systematic analysis, data sources were identified from GBD 2013 for the prevalence of mental, neurological, and substance use disorders in China and India published up to Dec 31, 2013. We calculated the proportion of the population represented by the data with the adjusted population coverage (APC) method adjusting for age, sex, and population size. We developed prevalence models with DisMod-MR 2.0, a Bayesian meta-regression instrument used to pool population-representative epidemiological data as part of GBD 2013. We report estimates and 95% uncertainly intervals (95% UI) for 15 mental, neurological, and substance use disorders for China and India in 1990 and 2013, and benchmark these against those for other BRICS countries (Brazil, Russia, and South Africa) in 2013. Few population-representative data were found for the disorders, with an average coverage of 15% of the population of the Chinese mainland and 1% of the population of India. For men in both China and India, major depressive disorder, anxiety disorders, and alcohol dependence were the most common mental, neurological, and substance use disorders. Prevalence of major depressive disorder was 2·2% (95% UI 1·5-2·8) in Chinese men and 3·5% (2·4-4·6) in Indian men; prevalence of anxiety disorders was 2·0% (1·1-3·2) and 1·9% (1·2-2·3), respectively. For women, anxiety disorders, major depressive disorder, and dysthymia were the most common. Prevalence of major depressive disorder was 3·3% (2·3-4·1) in Chinese women and 4·7% (95% UI 3·3-6·2) in Indian women; prevalence

  10. Robotic gait rehabilitation and substitution devices in neurological disorders: where are we now?

    Science.gov (United States)

    Calabrò, Rocco Salvatore; Cacciola, Alberto; Bertè, Francesco; Manuli, Alfredo; Leo, Antonino; Bramanti, Alessia; Naro, Antonino; Milardi, Demetrio; Bramanti, Placido

    2016-04-01

    Gait abnormalities following neurological disorders are often disabling, negatively affecting patients' quality of life. Therefore, regaining of walking is considered one of the primary objectives of the rehabilitation process. To overcome problems related to conventional physical therapy, in the last years there has been an intense technological development of robotic devices, and robotic rehabilitation has proved to play a major role in improving one's ability to walk. The robotic rehabilitation systems can be classified into stationary and overground walking systems, and several studies have demonstrated their usefulness in patients after severe acquired brain injury, spinal cord injury and other neurological diseases, including Parkinson's disease, multiple sclerosis and cerebral palsy. In this review, we want to highlight which are the most widely used devices today for gait neurological rehabilitation, focusing on their functioning, effectiveness and challenges. Novel and promising rehabilitation tools, including the use of virtual reality, are also discussed.

  11. Anti-ganglioside antibodies in patients with Guillain Barré syndrome and other neurological disorders

    Directory of Open Access Journals (Sweden)

    C Vaishnavi

    2013-01-01

    Full Text Available A study was performed on 59 Guillain-Barré syndrome (GBS cases, 58 neurological controls (NC and 60 non-neurological controls (NNC to investigate the association of anti-ganglioside antibodies in GBS and other neurological disorders. Campylobacter jejuni was isolated from 5.7% of GBS patients. Anti-ganglioside immunoglobulin G was present in 82% and immunoglobulin M in 46% in acute inflammatory demyelinating polyneuropathy patients, 70% and 44% respectively in acute motor axonal neuropathy subgroup and 38% each in acute motor sensory axonal neuropathy subgroup. Though high intensity of anti-gangliosides was present in the GBS patients, the NC patients also had adequate anti-gangliosides compared with the NNC group.

  12. The Charcot Foot in Diabetes

    Science.gov (United States)

    Frykberg, Robert G.; Armstrong, David G.; Boulton, Andrew J.M.; Edmonds, Michael; Van, Georges Ha; Hartemann, Agnes; Game, Frances; Jeffcoate, William; Jirkovska, Alexandra; Jude, Edward; Morbach, Stephan; Morrison, William B.; Pinzur, Michael; Pitocco, Dario; Sanders, Lee; Wukich, Dane K.; Uccioli, Luigi

    2011-01-01

    The diabetic Charcot foot syndrome is a serious and potentially limb-threatening lower-extremity complication of diabetes. First described in 1883, this enigmatic condition continues to challenge even the most experienced practitioners. Now considered an inflammatory syndrome, the diabetic Charcot foot is characterized by varying degrees of bone and joint disorganization secondary to underlying neuropathy, trauma, and perturbations of bone metabolism. An international task force of experts was convened by the American Diabetes Association and the American Podiatric Medical Association in January 2011 to summarize available evidence on the pathophysiology, natural history, presentations, and treatment recommendations for this entity. PMID:21868781

  13. The Brazilian Neurology centenary (1912-2012 and the common origin of the fields of Neurology and Psychiatry

    Directory of Open Access Journals (Sweden)

    Marleide da Mota Gomes

    2013-01-01

    Full Text Available It is reported the Brazilian Neurology birth (1912, that has as the hallmark its first Neurology Cathedra of Rio de Janeiro, and the links between Neurology and Psychiatry, besides the main medical protagonists at that time in Rio de Janeiro: João Carlos Teixeira Brandão (1854-1921, first professor of the cathedra of Clinical Psychiatry and Nervous Diseases (1883-1921; Juliano Moreira (1873-1933, the founder of the Brazilian scientific Psychiatry and director of the Hospício Nacional de Alienados (National Hospice for the Insane (1903-1930; Antônio Austregésilo Rodrigues de Lima (1876-1960, first professor of the cathedra of Neurology, considered the father of the Brazilian Neurology. Aloysio de Castro (1881-1959 was a great Brazilian neurosemiologist at that time. Austregésilo practiced both disciplines, Neurology and Psychiatry, and like Jean-Martin-Charcot, he was very interested in a typically psychiatric disorder, the hysteria. It is also considered in this paper the first Brazilian authors of Neurology and/or Psychiatric texts and the places where Neurology was initially developed by the main founders: Hospício Nacional de Alienados, Santa Casa de Misericórdia do Rio de Janeiro and Policlínica Geral do Rio de Janeiro.

  14. Novel OPA1 missense mutation in a family with optic atrophy and severe widespread neurological disorder.

    Science.gov (United States)

    Liskova, Petra; Ulmanova, Olga; Tesina, Petr; Melsova, Hana; Diblik, Pavel; Hansikova, Hana; Tesarova, Marketa; Votruba, Marcela

    2013-05-01

    To identify the underlying molecular genetic cause in a Czech family with optic atrophy, deafness, ptosis, ophthalmoplegia, polyneuropathy and ataxia transmitted as an autosomal dominant trait. Ophthalmological and neurological examination followed by molecular genetic analyses. Seven family members were clinically affected. There was a variable but progressive visual, hearing and neurological disability across the family as a whole. The majority of subjects presented with impairment of visual function and a variable degree of ptosis and/or ophthalmoplegia from the first to the third decade of life. Deafness, neuropathy and ataxia appeared later, in the third and fourth decade. Migraine, tachycardia, intention tremor, nystagmus and cervical dystonia were observed in isolated individuals. A significant overall feature was the high level of neurological disability leading to 3 of 4 members being unable to walk or stand unaided before the age of 60 years. A novel missense mutation c.1345A>C (p.Thr449Pro) in OPA1 segregating with the disease phenotype over three generations was detected. In silico analysis supported pathogenicity of the identified sequence variant. Our work expands the spectrum of mutation in OPA1, which may lead to severe multisystem neurological disorder. The molecular genetic cause of dominant optic atrophy in the Czech population is reported for the first time. We propose that regular cardiac follow-up in patients diagnosed with dominant optic atrophy and widespread neurological disease should be considered. © 2013 The Authors. Acta Ophthalmologica © 2013 Acta Ophthalmologica Scandinavica Foundation.

  15. Quantitative Evaluation of the Use of Actigraphy for Neurological and Psychiatric Disorders

    Directory of Open Access Journals (Sweden)

    Weidong Pan

    2014-01-01

    Full Text Available Quantitative and objective evaluation of disease severity and/or drug effect is necessary in clinical practice. Wearable accelerometers such as an actigraph enable long-term recording of a patient’s movement during activities and they can be used for quantitative assessment of symptoms due to various diseases. We reviewed some applications of actigraphy with analytical methods that are sufficiently sensitive and reliable to determine the severity of diseases and disorders such as motor and nonmotor disorders like Parkinson’s disease, sleep disorders, depression, behavioral and psychological symptoms of dementia (BPSD for vascular dementia (VD, seasonal affective disorder (SAD, and stroke, as well as the effects of drugs used to treat them. We believe it is possible to develop analytical methods to assess more neurological or psychopathic disorders using actigraphy records.

  16. CHARCOT-MARIE-TOOTH DISEASE

    Directory of Open Access Journals (Sweden)

    Lea Leonardis

    2003-09-01

    Full Text Available Background. Charcot-Marie-Tooth (CMT disease is a common inherited disorder of the peripheral nervous system. In our paper, different types of CMT are described with their typical clinical pictures, electrophysiological signs and molecular genetic studies. CMT is classified as demyelinative and axonal type and distal motor neuronopathy.Conclusions. CMT can be of autosomal dominant, recessive and X-linked inheritance. The most frequent form of CMT is the result of the dominantly inherited duplication of chromosome 17p11.2 and is marked as CMT1A. The same group involves also rare patients with point mutation in the peripheral myelin protein-22 gene. CMT1B is associated with point mutations in protein zero gene. CMT1C is linked to chromosome 16p13.1–12.3. Patients with point mutations in early growth response 2 gene (EGR2 are included in group CMT1D. The disease can be also inhereted X-linked (CMTX with the mutations in connexin-32 gene. In autosomal recessive inherited demyelinating polyneuropathies (CMT4, mutations are found in the myotubularin-related protein-2 (CMT4B, N-myc downstream-regulated gene 1 (CMT4D, EGR2 (CMT4E, and in the periaksin (CMT4F genes. In axonal inherited neuropathy, mutations are found in KIF1beta (CMT2A and in light neurofilament (CMT2E genes, other forms map to different chromosomal loci (CMT2B, CMT2D, CMT2F. Some suggestions for the diagnostic procedures of patients with CMT are given.

  17. Jean-Martin Charcot and his vibratory chair for Parkinson disease.

    Science.gov (United States)

    Goetz, Christopher G

    2009-08-11

    Vibration therapy is currently used in diverse medical specialties ranging from orthopedics to urology to sports medicine. The celebrated 19th-century neurologist, J.-M. Charcot, used vibratory therapy to treat Parkinson disease (PD). This study analyzed printed writings by Charcot and other writers on vibratory therapy and accessed unpublished notes from the Salpêtrière Hospital, Paris. Charcot lectured on several occasions on vibratory therapy and its neurologic applications. He developed a vibration chair for patients with PD after he observed that patients were more comfortable and slept better after a train or carriage ride. He replicated this experience by having patients undergo daily 30-minute sessions in the automated vibratory chair (fauteuil trépidant). His junior colleague, Gilles de la Tourette, extended these observations and developed a helmet that vibrated the head on the premise that the brain responded directly to the pulsations. Although after Charcot's death vibratory therapy was not widely pursued, vibratory appliances are reemerging in 21st century medicine and can be retested using adaptations of Charcot's neurologic protocols.

  18. Charcot, la salpêtrière, and hysteria as represented in European literature.

    Science.gov (United States)

    Koehler, Peter J

    2013-01-01

    In this chapter, I describe the influence of Jean-Martin Charcot (1825-1893), his neurological school at the Salpêtrière (Paris), and his teaching of hysteria on European literature. Many references to Charcot and descriptions of hysterical attacks are found not only in French naturalistic literature but also subsequently in naturalistic novels from other European countries (the Netherlands, Russia, Scandinavian countries, Spain, Italy, and Germany) and furthermore in novels written in new literary movements that followed naturalism. At first, objective descriptions were presented, but in the periods that followed, in particular during the past decades, criticism, rather than objective descriptions, became the motivation for continuing to use Charcot and his teaching of hysteria as inspiration for novels and plays, although Charcot as an admired founder of neurology did not quite disappear, even in recent novels. It is quite impressive to observe how Charcot and his demonstrations of hysterical attacks still resound throughout European literature, even after more than a century. © 2013 Elsevier B.V. All rights reserved.

  19. Architects of the genome: CHD dysfunction in cancer, developmental disorders and neurological syndromes.

    Science.gov (United States)

    Li, Wangzhi; Mills, Alea A

    2014-01-01

    Chromatin is vital to normal cells, and its deregulation contributes to a spectrum of human ailments. An emerging concept is that aberrant chromatin regulation culminates in gene expression programs that set the stage for the seemingly diverse pathologies of cancer, developmental disorders and neurological syndromes. However, the mechanisms responsible for such common etiology have been elusive. Recent evidence has implicated lesions affecting chromatin-remodeling proteins in cancer, developmental disorders and neurological syndromes, suggesting a common source for these different pathologies. Here, we focus on the chromodomain helicase DNA binding chromatin-remodeling family and the recent evidence for its deregulation in diverse pathological conditions, providing a new perspective on the underlying mechanisms and their implications for these prevalent human diseases.

  20. Marine natural product drug discovery: Leads for treatment of inflammation, cancer, infections, and neurological disorders.

    Science.gov (United States)

    Villa, Francisco A; Gerwick, Lena

    2010-06-01

    Natural products, secondary metabolites, isolated from plants, animals and microbes are important sources for bioactive molecules that in many cases have been developed into treatments for diseases. This review will focus on describing the potential for finding new treatments from marine natural products for inflammation, cancer, infections, and neurological disorders. Historically terrestrial natural products have been studied to a greater extent and such classic drugs as aspirin, vincristine and many of the antibiotics are derived from terrestrial natural products. The need for new therapeutics in the four areas mentioned is dire. Within the last 30 years marine natural products, with their unique structures and high level of halogenation, have shown many promising activities against the inflammatory response, cancer, infections and neurological disorders. The review will outline examples of such compounds and activities.

  1. Severe neurological disorders and refractory aspergillosis in an adolescent treated by vincristine and voriconazole.

    Science.gov (United States)

    Bennis, Y; Bodeau, S; Lutun, A; Gourmel, A; Solas, C; Quaranta, S; Guillaume, N; Chouaki, T; Lemaire-Hurtel, A-S; Masmoudi, K

    2017-08-10

    Voriconazole and vincristine are major therapeutics in paediatric haematology. However, the risk-benefit ratio of the treatment of invasive aspergillosis with voriconazole in patients receiving vincristine-based chemotherapy remains unclear. We report severe peripheral and central neurological disorders in a 14-year-old girl with T-cell acute lymphoblastic leukaemia and pulmonary aspergillosis. The case describes a strong exacerbation by voriconazole of the vincristine-induced neuropathic pains. It shows the high variability of the trough serum concentration of voriconazole leading to antifungal treatment failure and suggests that its own central neurotoxicity could also be potentiated by vincristine. Given the risk of either insufficient antifungal efficacy or excessive neurological disorders, this case warns on a probable unfavourable risk-benefit profile of voriconazole during vincristine-based chemotherapy in adolescents. © 2017 John Wiley & Sons Ltd.

  2. 5-HT2A Agonists: A Novel Therapy for Functional Neurological Disorders?

    Science.gov (United States)

    Bryson, Alexander; Carter, Olivia; Norman, Trevor; Kanaan, Richard

    2017-05-01

    Functional neurological disorders are frequently encountered in clinical practice. They have a poor prognosis and treatment options are limited. Their etiology is unknown, but leading theories propose a disturbance of somatic self-representation: the mind perceives dysfunction of a body region despite intact motor and sensory pathways. Central to this model is the concept of an abnormal top-down cognitive influence upon sensorimotor function. There is growing interest in the use of 5-HT2A agonists in the management of neuropsychiatric conditions. Recent studies have shown that these agents induce changes in neural activity that disrupt hierarchical brain dynamics and modulate networks subserving self-related processing. Converging evidence suggests they may hold unique therapeutic potential in functional neurological disorders. This is of importance given the considerable personal and societal burden of this condition and we argue a clinical trial to test this hypothesis is warranted. © The Author 2017. Published by Oxford University Press on behalf of CINP.

  3. Comparative Study of Neurological Soft Signs in Patients with Schizophrenia or Obsessive-compulsive Disorder, and Healthy Controls.

    Science.gov (United States)

    Tripathi, R; Soni, A; Tyagi, A; Mehta, S; Gupta, S

    2015-06-01

    The primary objective of this study was to examine neurological soft signs in patients with obsessive-compulsive disorder compared with patients with schizophrenia and a control group in the Indian setting. The secondary objective was to find any correlation between age at onset and neurological soft signs scores, as well as that between severity of obsessive-compulsive disorder symptoms (total Yale-Brown Obsessive Compulsive Scale score) and neurological soft signs scores. This was a cross-sectional hospital-based study of 135 individuals (45 patients with schizophrenia, 45 patients with obsessive-compulsive disorder who were attending the psychiatric outpatient department of Sawai Man Singh Medical College, Jaipur, India, and 45 matched healthy controls) from 20 June 2013 to 22 December 2014. After applying strict inclusion and exclusion criteria, the participants completed the study instruments (Cambridge Neurological Inventory [Part 2] and Yale-Brown Obsessive Compulsive Scale). Their socio-demographic data were also recorded. The neurological soft signs total score and domain scores (motor coordination, sensory integration, and disinhibition) were significantly higher in patients with schizophrenia (p disorder group or the control group. The obsessive-compulsive disorder group did not significantly differ from the control group in terms of neurological soft signs scores. No correlation was found between neurological soft signs scores and age at onset as well as that between neurological soft signs scores and total Yale-Brown Obsessive Compulsive Scale score. Neurological soft signs assessed by the Cambridge Neurological Inventory and Yale-Brown Obsessive Compulsive Scale, which discriminate patients with schizophrenia from controls, appear to be relatively specific to schizophrenia. Further studies are required to explore neurological soft signs in patients with obsessive-compulsive disorder.

  4. EFhd2, a Protein Linked to Alzheimer's Disease and Other Neurological Disorders.

    Science.gov (United States)

    Vega, Irving E

    2016-01-01

    EFhd2 is a conserved calcium binding protein linked to different neurological disorders and types of cancer. Although, EFhd2 is more abundant in neurons, it is also found in other cell types. The physiological function of this novel protein is still unclear, but it has been shown in vitro to play a role in calcium signaling, apoptosis, actin cytoskeleton, and regulation of synapse formation. Recently, EFhd2 was shown to promote cell motility by modulating the activity of Rac1, Cdc42, and RhoA. Although, EFhd2's role in promoting cell invasion and metastasis is of great interest in cancer biology, this review focusses on the evidence that links EFhd2 to Alzheimer's disease (AD) and other neurological disorders. Altered expression of EFhd2 has been documented in AD, Parkinson's disease, Huntington's disease, Amyotrophic Lateral Sclerosis, and schizophrenia, indicating that Efhd2 gene expression is regulated in response to neuropathological processes. However, the specific role that EFhd2 plays in the pathophysiology of neurological disorders is still poorly understood. Recent studies demonstrated that EFhd2 has structural characteristics similar to amyloid proteins found in neurological disorders. Moreover, EFhd2 co-aggregates and interacts with known neuropathological proteins, such as tau, C9orf72, and Lrrk2. These results suggest that EFhd2 may play an important role in the pathophysiology of neurodegenerative diseases. Therefore, the understanding of EFhd2's role in health and disease could lead to decipher molecular mechanisms that become activated in response to neuronal stress and degeneration.

  5. Charcot-Marie-Tooth Disease in a Child with Acute Lymphoblastic ...

    African Journals Online (AJOL)

    Alasia Datonye

    Patients with Charcot-Marie-Tooth disease may show severe toxicities with vincristine. It is therefore recommended that an extensive neurologic examination should be conducted on any paediatric patient with a diagnosis of malignancy to ... the Paediatric oncology unit of the University of Port. Harcourt, teaching hospital ...

  6. Genetics Home Reference: Charcot-Marie-Tooth disease

    Science.gov (United States)

    ... autosomal recessive Genetic Testing Registry: Charcot-Marie-Tooth disease, demyelinating, type 1b Genetic Testing Registry: Charcot-Marie-Tooth disease, demyelinating, type 1d Genetic Testing Registry: Charcot-Marie-Tooth ...

  7. Guidelines for uniform reporting of body fluid biomarker studies in neurologic disorders

    DEFF Research Database (Denmark)

    Gnanapavan, Sharmilee; Hegen, Harald; Khalil, Michael

    2014-01-01

    OBJECTIVE: The aim of these guidelines is to make the process of reporting body fluid biomarker studies in neurologic disorders more uniform and transparent, in line with existing standards for reporting research in other biomedical areas. Although biomarkers have been around for decades, there a......OBJECTIVE: The aim of these guidelines is to make the process of reporting body fluid biomarker studies in neurologic disorders more uniform and transparent, in line with existing standards for reporting research in other biomedical areas. Although biomarkers have been around for decades......, there are concerns over the high attrition rate of promising candidate biomarkers at later phases of development. METHODS: BioMS-eu consortium, a collaborative network working toward improving the quality of biomarker research in neurologic disorders, discussed the merits of standardizing the reporting of body fluid...... biomarker research. A checklist of items integrating the results of other published guidances, literature, conferences, regulatory opinion, and personal expertise was created to ultimately form a structured summary guidance incorporating the key features. RESULTS: The summary guidance is comprised of a 10...

  8. Semantic Pattern Analysis for Verbal Fluency Based Assessment of Neurological Disorders

    Energy Technology Data Exchange (ETDEWEB)

    Sukumar, Sreenivas R [ORNL; Ainsworth, Keela C [ORNL; Brown, Tyler C [ORNL

    2014-01-01

    In this paper, we present preliminary results of semantic pattern analysis of verbal fluency tests used for assessing cognitive psychological and neuropsychological disorders. We posit that recent advances in semantic reasoning and artificial intelligence can be combined to create a standardized computer-aided diagnosis tool to automatically evaluate and interpret verbal fluency tests. Towards that goal, we derive novel semantic similarity (phonetic, phonemic and conceptual) metrics and present the predictive capability of these metrics on a de-identified dataset of participants with and without neurological disorders.

  9. The core competencies for mental, neurological, and substance use disorder care in sub-Saharan Africa

    Directory of Open Access Journals (Sweden)

    Pamela Y. Collins

    2015-03-01

    Full Text Available The 2010 Global Burden of Disease Study points to a changing landscape in which non-communicable diseases, such as mental, neurological, and substance use (MNS disorders, account for an increasing proportion of premature mortality and disability globally. Despite evidence of the need for care, a remarkable deficit of providers for MNS disorder service delivery persists in sub-Saharan Africa. This critical workforce can be developed from a range of non-specialist and specialist health workers who have access to evidence-based interventions, whose roles, and the associated tasks, are articulated and clearly delineated, and who are equipped to master and maintain the competencies associated with providing MNS disorder care. In 2012, the Neuroscience Forum of the Institute of Medicine convened a meeting of key stakeholders in Kampala, Uganda, to discuss a set of candidate core competencies for the delivery of mental health and neurological care, focusing specifically on depression, psychosis, epilepsy, and alcohol use disorders. This article discusses the candidate core competencies for non-specialist health workers and the complexities of implementing core competencies in low- and middle-income country settings. Sub-Saharan Africa, however, has the potential to implement novel training initiatives through university networks and through structured processes that engage ministries of health. Finally, we outline challenges associated with implementing competencies in order to sustain a workforce capable of delivering quality services for people with MNS disorders.

  10. The core competencies for mental, neurological, and substance use disorder care in sub-Saharan Africa.

    Science.gov (United States)

    Collins, Pamela Y; Musisi, Seggane; Frehywot, Seble; Patel, Vikram

    2015-01-01

    The 2010 Global Burden of Disease Study points to a changing landscape in which non-communicable diseases, such as mental, neurological, and substance use (MNS) disorders, account for an increasing proportion of premature mortality and disability globally. Despite evidence of the need for care, a remarkable deficit of providers for MNS disorder service delivery persists in sub-Saharan Africa. This critical workforce can be developed from a range of non-specialist and specialist health workers who have access to evidence-based interventions, whose roles, and the associated tasks, are articulated and clearly delineated, and who are equipped to master and maintain the competencies associated with providing MNS disorder care. In 2012, the Neuroscience Forum of the Institute of Medicine convened a meeting of key stakeholders in Kampala, Uganda, to discuss a set of candidate core competencies for the delivery of mental health and neurological care, focusing specifically on depression, psychosis, epilepsy, and alcohol use disorders. This article discusses the candidate core competencies for non-specialist health workers and the complexities of implementing core competencies in low- and middle-income country settings. Sub-Saharan Africa, however, has the potential to implement novel training initiatives through university networks and through structured processes that engage ministries of health. Finally, we outline challenges associated with implementing competencies in order to sustain a workforce capable of delivering quality services for people with MNS disorders.

  11. Chronic hepatitis C virus infection and neurological and psychiatric disorders: An overview

    Science.gov (United States)

    Adinolfi, Luigi Elio; Nevola, Riccardo; Lus, Giacomo; Restivo, Luciano; Guerrera, Barbara; Romano, Ciro; Zampino, Rosa; Rinaldi, Luca; Sellitto, Ausilia; Giordano, Mauro; Marrone, Aldo

    2015-01-01

    Hepatitis C virus (HCV) infection is considered a systemic disease because of involvement of other organs and tissues concomitantly with liver disease. Among the extrahepatic manifestations, neuropsychiatric disorders have been reported in up to 50% of chronic HCV infected patients. Both the central and peripheral nervous system may be involved with a wide variety of clinical manifestations. Main HCV-associated neurological conditions include cerebrovascular events, encephalopathy, myelitis, encephalomyelitis, and cognitive impairment, whereas “brain fog”, depression, anxiety, and fatigue are at the top of the list of psychiatric disorders. Moreover, HCV infection is known to cause both motor and sensory peripheral neuropathy in the context of mixed cryoglobulinemia, and has also been recently recognized as an independent risk factor for stroke. These extrahepatic manifestations are independent of severity of the underlying chronic liver disease and hepatic encephalopathy. The brain is a suitable site for HCV replication, where the virus may directly exert neurotoxicity; other mechanisms proposed to explain the pathogenesis of neuropsychiatric disorders in chronic HCV infection include derangement of metabolic pathways of infected cells, alterations in neurotransmitter circuits, autoimmune disorders, and cerebral or systemic inflammation. A pathogenic role for HCV is also suggested by improvement of neurological and psychiatric symptoms in patients achieving a sustained virologic response following interferon treatment; however, further ad hoc trials are needed to fully assess the impact of HCV infection and specific antiviral treatments on associated neuropsychiatric disorders. PMID:25741133

  12. Surgical management of Charcot neuroarthropathy.

    Science.gov (United States)

    Idusuyi, Osaretin B

    2015-02-01

    Charcot neuroarthropathy is one of the more devastating complications of diabetes. It is a progressive, destructive condition that is characterized by acute fracture, dislocation, and joint destruction in weight-bearing neuropathic foot. In its acute phase, it is often misdiagnosed and can lead to several deformities such as ulcerations and amputation. Early diagnosis and management is, therefore, imperative to avoid rapid progression. Review current literature on the diagnosis and management of diabetic patients with Charcot neuroarthropathy. Narrative review. Particular attention is directed to the role of surgical management in achieving long term osseous stability and alignment so that appropriate footwear and bracing are possible. Charcot neuroarthropathy is a serious and potentially limb-threatening lower extremity late complication of diabetes. Correct timing, adequate fixation and a long post-operative weightbearing period are key to optimizing reconstructive surgery. Primary care providers who treat diabetic patients should be cognizant of the possible complication among patients with diabetic neuropathy of which includes Charcot neuroarthropathy. In this paper, discussion is provided on a rational approach to functional limb salvage with various surgical techniques when non-operative management fails. © The International Society for Prosthetics and Orthotics 2014.

  13. Aerobic exercise to improve cognitive function in adults with neurological disorders: a systematic review.

    Science.gov (United States)

    McDonnell, Michelle N; Smith, Ashleigh E; Mackintosh, Shylie F

    2011-07-01

    To evaluate whether aerobic exercise improves cognition in adults diagnosed with neurologic disorders. The Cochrane Central Register of Controlled Clinical Trials, MEDLINE, CINAHL, PubMed, EMBASE, PEDro, AMED, SPORTDiscus, PsycINFO, ERIC, and Google Scholar, with the last search performed in December 2010. We included controlled clinical trials and randomized controlled trials with adults diagnosed with a neurologic disorder. Studies were included if they compared a control group with a group involved in an aerobic exercise program to improve cardiorespiratory fitness and if they measured cognition as an outcome. Two reviewers independently extracted data and methodologic quality of the included trials. From the 67 trials reviewed, a total of 7 trials, involving 249 participants, were included. Two trials compared the effectiveness of yoga and aerobic exercise in adults with multiple sclerosis. Two trials evaluated the effect of exercise on patients with dementia, and 2 trials evaluated the effectiveness of exercise to improve cognition after traumatic brain injury. One trial studied the effect of a cycling program in people with chronic stroke. Lack of commonality between measures of cognition limited meta-analyses. Results from individual studies show that aerobic exercise improved cognition in people with dementia, improved attention and cognitive flexibility in patients with traumatic brain injury, improved choice reaction time in people with multiple sclerosis, and enhanced motor learning in people with chronic stroke. There is limited evidence to support the use of aerobic exercise to improve cognition in adults with neurologic disorders. Of the 67 studies retrieved, less than half included cognition as an outcome, and few studies continued the aerobic exercise program long enough to be considered effective. Further studies investigating the effect of aerobic exercise interventions on cognition in people with neurologic conditions are required. Copyright

  14. The Ability of the Eating Assessment Tool-10 to Detect Aspiration in Patients With Neurological Disorders

    Science.gov (United States)

    Arslan, Selen Serel; Demir, Numan; Kılınç, Hasan E; Karaduman, Aynur A

    2017-01-01

    Background/Aims Dysphagia is common in patients with neurological disorders. There is a need to identify patients at risk early by a useful clinical tool to prevent its serious complications. The study aims to determine the ability of the Turkish version of Eating Assessment Tool-10 (T-EAT-10) to detect aspiration in patients with neurological disorders. Methods Two hundred fifty-nine patients with neurological disorders who had complaints about swallowing difficulty and referred for a swallowing evaluation were included. Oropharyngeal dysphagia was evaluated with the T-EAT-10 and videofluoroscopic swallowing study in the same day. The penetration-aspiration scale (PAS) was used to document the penetration and aspiration severity. Results The mean age of the patients was 59.72 ± 17.24 years (minimum [min] = 18, maximum [max] = 96), of which 57.1% were male. The mean T-EAT-10 of patients who had aspiration (PAS > 5) was 25.91 ± 10.31 (min = 1, max = 40) and the mean T-EAT-10 of patients who did not have aspiration (PAS < 6) was 15.70 ± 10.54 (min = 0, max = 40) (P < 0.001). Patients with a T-EAT-10 score higher than 15 were 2.4 times more likely to aspirate. A linear correlation was found between T-EAT-10 and PAS scores of the patients (r = 0.416, P < 0.001). The sensitivity of a T-EAT-10 higher than 15 in detecting aspiration was 81.0% and the specificity was 58.0%. A T-EAT-10 score of higher than 15 has a positive predictive value of 72.0% and a negative predictive value of 69.0%. Conclusion The T-EAT-10 can be used to detect unsafe airway protection in neurology clinics to identify and refer dysphagic patients for further evaluation. PMID:28545185

  15. Computational profiling of the gut-brain axis: microflora dysbiosis insights to neurological disorders.

    Science.gov (United States)

    Dovrolis, Nikolas; Kolios, George; Spyrou, George M; Maroulakou, Ioanna

    2017-11-27

    Almost 2500 years after Hippocrates' observations on health and its direct association to the gastrointestinal tract, a paradigm shift has recently occurred, making the gut and its symbionts (bacteria, fungi, archaea and viruses) a point of convergence for studies. It is nowadays well established that the gut microflora's compositional diversity regulates via its genes (the microbiome) the host's health and provides preliminary insights into disease progression and regulation. The microbiome's involvement is evident in immunological and physiological studies that link changes in its biodiversity to its contributions to the host's phenotype but also in neurological investigations, substantiating the aptly named gut-brain axis. The definitive mechanisms of this last bidirectional interaction will be our main focus because it presents researchers with a new conundrum. In this review, we prospect current literature for computational analysis methodologies that accommodate the need for better understanding of the microbiome-gut-brain interactions and neurological disorder onset and progression, through cross-disciplinary systems biology applications. We will present bioinformatics tools used in exploring these synergies that help build and interpret microbial 16S ribosomal RNA data sets, produced by shotgun and high-throughput sequencing of healthy and neurological disorder samples stored in biological databases. These approaches provide alternative means for researchers to form hypotheses to their inquests faster, cheaper and swith precision. The goal of these studies relies on the integration of combined metagenomics and metabolomics assessments. An accurate characterization of the microbiome and its functionality can support new diagnostic, prognostic and therapeutic strategies for neurological disorders, customized for each individual host. © The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  16. [Charcot and Babinski: beyond a simple teacher-student relationship].

    Science.gov (United States)

    Massie, Rami

    2004-08-01

    Jean-Martin Charcot (1825-1893) is now considered to be the father of clinical neurology in France. He trained a generation of eminent neurologists, among them Joseph Babinski, with whom he had a special relationship. Babinski was undoubtedly Charcot's favorite pupil and they enjoyed an excellent collaboration at la Salpétrière. Even though both men felt tremendous respect for each other, it is sad that this relationship may, in one instance, have been detrimental to Babinski. This is probably the reason why Bouchard denied him full professorship, a decision with eventual consequences for both men. In spite of this, the neurologist of Polish origin held his master in tremendous admiration, even as he pursued Charcot's research on hysteria after his death. Even though Babinski eventually contradicted his master on many fundamental issues, it did not affect his devotion to him. The relationship between the two men can be considered as more than a simple relationship between a teacher and his pupil and may be compared to a father-son relationship, which is a reminder of the original model of Hippocratic teaching.

  17. Zinc in Gut-Brain Interaction in Autism and Neurological Disorders

    Directory of Open Access Journals (Sweden)

    Guillermo Vela

    2015-01-01

    Full Text Available A growing amount of research indicates that abnormalities in the gastrointestinal (GI system during development might be a common factor in multiple neurological disorders and might be responsible for some of the shared comorbidities seen among these diseases. For example, many patients with Autism Spectrum Disorder (ASD have symptoms associated with GI disorders. Maternal zinc status may be an important factor given the multifaceted effect of zinc on gut development and morphology in the offspring. Zinc status influences and is influenced by multiple factors and an interdependence of prenatal and early life stress, immune system abnormalities, impaired GI functions, and zinc deficiency can be hypothesized. In line with this, systemic inflammatory events and prenatal stress have been reported to increase the risk for ASD. Thus, here, we will review the current literature on the role of zinc in gut formation, a possible link between gut and brain development in ASD and other neurological disorders with shared comorbidities, and tie in possible effects on the immune system. Based on these data, we present a novel model outlining how alterations in the maternal zinc status might pathologically impact the offspring leading to impairments in brain functions later in life.

  18. The prospect of molecular therapy for Angelman syndrome and other monogenic neurologic disorders

    Science.gov (United States)

    2014-01-01

    Background Angelman syndrome is a monogenic neurologic disorder that affects 1 in 15,000 children, and is characterized by ataxia, intellectual disability, speech impairment, sleep disorders, and seizures. The disorder is caused by loss of central nervous system expression of UBE3A, a gene encoding a ubiquitin ligase. Current treatments focus on the management of symptoms, as there have not been therapies to treat the underlying molecular cause of the disease. However, this outlook is evolving with advances in molecular therapies, including artificial transcription factors a class of engineered DNA-binding proteins that have the potential to target a specific site in the genome. Results Here we review the recent progress and prospect of targeted gene expression therapies. Three main issues that must be addressed to advance toward human clinical trials are specificity, toxicity, and delivery. Conclusions Artificial transcription factors have the potential to address these concerns on a level that meets and in some cases exceeds current small molecule therapies. We examine the possibilities of such approaches in the context of Angelman syndrome, as a template for other single-gene, neurologic disorders. PMID:24946931

  19. A decade from discovery to therapy: Lingo-1, the dark horse in neurological and psychiatric disorders.

    Science.gov (United States)

    Andrews, Jessica L; Fernandez-Enright, Francesca

    2015-09-01

    Leucine-rich repeat and immunoglobulin domain-containing protein (Lingo-1) is a potent negative regulator of neuron and oligodendrocyte survival, neurite extension, axon regeneration, oligodendrocyte differentiation, axonal myelination and functional recovery; all processes highly implicated in numerous brain-related functions. Although playing a major role in developmental brain functions, the potential application of Lingo-1 as a therapeutic target for the treatment of neurological disorders has so far been under-estimated. A number of preclinical studies have shown that various methods of antagonizing Lingo-1 results in neuronal and oligodendroglial survival, axonal growth and remyelination; however to date literature has only detailed applications of Lingo-1 targeted therapeutics with a focus primarily on myelination disorders such as multiple sclerosis and spinal cord injury; omitting important information regarding Lingo-1 signaling co-factors. Here, we provide for the first time a complete and thorough review of the implications of Lingo-1 signaling in a wide range of neurological and psychiatric disorders, and critically examine its potential as a novel therapeutic target for these disorders. Copyright © 2015 Elsevier Ltd. All rights reserved.

  20. Coraco- or Costoclavicular Paraosteoarthropathies in Patients with Severe Central Neurological Disorders

    Energy Technology Data Exchange (ETDEWEB)

    Lacout, A.; Mompoint, D.; Perrier, Y.; Vallee, C.A.; Carlier, R.Y. (Service de Radiologie, Hopital Raymond Poincare, Garches (France))

    2008-03-15

    Background: Paraosteoarthropathy (POA) is a frequent disabling orthopedic complication after severe central neurological impairment. The hip is the most frequently affected joint (32.1%) followed by the elbow and the shoulder (25%). Purpose: To evaluate coraco- and costoclavicular paraosteoarthropathy in patients with severe central neurological disorders. Material and Methods: We report a series of five consecutive patients with severe central neurological disorders who developed a POA of the clavicular region (coracoclavicular or costoclavicular POA). Every patient underwent a clinical, radiological, and computed tomographic (CT) examination of the shoulder region. Results: Four patients had a history of traumatic brain injury (TBI), and one an acute disseminated encephalomyelitis (ADEM). They developed POA of the clavicular region, although not around the glenohumeral joint. The patients complained of shoulder pain and of moderate limitation of movements. Radiological and CT examinations showed the presence of a bony formation in the coracoclavicular space in four cases and extending from the clavicle to the first rib around the costoclavicular joint in one case. Conclusion: In patients with severe brain lesions suffering from shoulder pain and moderate limitation of joint movements, POAs of the clavicular region are rare but should be considered

  1. Improving access to essential medicines for mental, neurological, and substance use disorders in Sub-Saharan Africa: workshop summary

    National Research Council Canada - National Science Library

    Pankevich, Diana E; Norris, Sheena M. Posey; Wizemann, Theresa M; Altevogt, Bruce M

    2014-01-01

    "In 2011 the Grand Challenges in Global Mental Health initiative identified priorities that have the potential to make a significant impact on the lives of people with mental, neurological, and substance use disorders...

  2. CT and MR findings of neurological disorders associated with pregnancy and childbirth

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jee Young; Ahn, Kook Jin; Kim, Young Joo; Kim, Bum Soo; Hahn, Seong Tae [College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of)

    2008-08-15

    The onset of pregnancy may predispose women to a variety of neurological diseases due to changes in their hemodynamics, hormonal effects, and complications associated with childbirth. The spectrum of neurological disorders associated with pregnancy and childbirth include hypertensive intracerebral hemorrhaging, posterior reversible encephalopathy syndrome (PRES) (secondary to eclampsia), Wernicke encephalopathy, cerebral venous sinus thrombosis, Sheehan's syndrome, hypoxic ischemic encephalopathy (secondary to pulmonary amniotic fluid embolism), multifocal infarctions, and extra-potine myelinolysis. The recognition of the various imaging findings of these diseases, along with the clinical presentations should aid in their early diagnosis and prompt treatment. The purpose of this pictorial assay is to describe the characteristic CT and MR findings of these diseases with a literature review to explain the mechanisms and clinical symptoms.

  3. Psychiatric and neurological disorders in late adolescence and risk of convictions for violent crime in men.

    Science.gov (United States)

    Moberg, Tomas; Stenbacka, Marlene; Tengström, Anders; Jönsson, Erik G; Nordström, Peter; Jokinen, Jussi

    2015-11-23

    The relationship between mental illness and violent crime is complex because of the involvement of many other confounding risk factors. In the present study, we analysed psychiatric and neurological disorders in relation to the risk of convictions for violent crime, taking into account early behavioural and socio-economic risk factors. The study population consisted of 49,398 Swedish men, who were thoroughly assessed at conscription for compulsory military service during the years 1969-1970 and followed in national crime registers up to 2006. Five diagnostic groups were analysed: anxiety-depression/neuroses, personality disorders, substance-related disorders, mental retardation and neurological conditions. In addition, eight confounders measured at conscription and based on the literature on violence risk assessment, were added to the analyses. The relative risks of convictions for violent crime during 35 years after conscription were examined in relation to psychiatric diagnoses and other risk factors at conscription, as measured by odds ratios (ORs) and confidence intervals (CIs) from bivariate and multivariate logistic regression analyses. In the bivariate analyses there was a significant association between receiving a psychiatric diagnosis at conscription and a future conviction for violent crime (OR = 3.83, 95 % CI = 3.47-4.22), whereas no significant association between neurological conditions and future violent crime (OR = 1.03, 95 % CI = 0.48-2.21) was found. In the fully adjusted multivariate logistic regression model, mental retardation had the strongest association with future violent crime (OR = 3.60, 95 % CI = 2.73-4.75), followed by substance-related disorders (OR = 2.81, 95 % CI = 2.18-3.62), personality disorders (OR = 2.66, 95 % CI = 2.21-3.19) and anxiety-depression (OR = 1.29, 95 % CI = 1.07-1.55). Among the other risk factors, early behavioural problem had the strongest association with

  4. Prevalence and risk factors for neurological disorders in children aged 6 months to 2 years in northern India.

    Science.gov (United States)

    Kumar, Rashmi; Bhave, Anupama; Bhargava, Roli; Agarwal, Girdhar G

    2013-04-01

    To study prevalence and risk factors for neurological disorders--epilepsy, global developmental delay, and motor, vision, and hearing defects--in children aged 6 months to 2 years in northern India. A two-stage community survey for neurological disorders was conducted in rural and urban areas of Lucknow. After initial screening with a new instrument, the Lucknow Neurodevelopment Screen, screen positives and a random proportion of screen negatives were validated using predefined criteria. Prevalence was calculated by weighted estimates. Demographic, socio-economic, and medical risk factors were compared between validated children who were positive and negative for neurological disorders by univariate and logistic regression analysis. Of 4801 children screened (mean age [SD] 15.32mo [5.96]; 2542 males, 2259 females), 196 were positive; 190 screen positives and 269 screen negatives were validated. Prevalence of neurological disorders was 27.92 per 1000 (weighted 95% confidence interval 12.24-43.60). Significant risk factors (p≤0.01) for neurological disorders were higher age in months (p=0.010), lower mean number of appliances in the household (p=0.001), consanguineous marriage of parents (p=0.010), family history of neurological disorder (p=0.001), and infants born exceptionally small (parental description; p=0.009). On logistic regression, the final model included age (p=0.0193), number of appliances (p=0.0161), delayed cry at birth (p=0.0270), postneonatal meningoencephalitis (p=0.0549), and consanguinity (p=0.0801). Perinatal factors, lower socio-economic status, and consanguinity emerged as predictors of neurological disorders. These factors are largely modifiable. © The Authors. Developmental Medicine & Child Neurology © 2013 Mac Keith Press.

  5. Imaging of Charcot foot; Bildgebung des Charcot-Fusses

    Energy Technology Data Exchange (ETDEWEB)

    Erlemann, Rainer; Schmitz, Annette [Helios Klinikum Duisburg, Helios St. Johannes Klinik, Duisburg (Germany). Inst. fuer Radiologie

    2014-03-15

    The onset of a Charcot foot ist a feared complication of a long lasting diabetes mellitus. A peripheral neuropathy and continuous weight bearing of the foot subsequent to repeated traumas depict the conditions. There exist three types of a Charcot foot, an atrophic, a hypertophic and a mixed type. In early stages a differentiation from osteoarthritis is difficult. Subluxation or luxation within the Lisfranc's joint is typical. The joints of the foot could rapidly and extensively be destroyed or may present the morphology of a 'superosteoarthritis'. Often, soft tissue infections or osteomyelitis evolve from ulcers of the skin as entry points. Diagnosis of osteomyelitis necessitate MR imaging as plain radiography offers only low sensitivity for detection of an osteomyelitis. The existence of periosteal reactions is not a proof for osteomyelitis. Bone marrow edema and soft tissue edema also appear in a non infected Charcot foot. The range of soft tissue infections goes from cellulitis over phlegmon to abscesses. The ghost sign is the most suitable diagnostic criterion for osteomyelitis. In addition, the penumbra sign or the existence of a sinus tract between a skin ulcer and the affected bone may be helpful. (orig.)

  6. Using deep learning to investigate the neuroimaging correlates of psychiatric and neurological disorders: Methods and applications.

    Science.gov (United States)

    Vieira, Sandra; Pinaya, Walter H L; Mechelli, Andrea

    2017-03-01

    Deep learning (DL) is a family of machine learning methods that has gained considerable attention in the scientific community, breaking benchmark records in areas such as speech and visual recognition. DL differs from conventional machine learning methods by virtue of its ability to learn the optimal representation from the raw data through consecutive nonlinear transformations, achieving increasingly higher levels of abstraction and complexity. Given its ability to detect abstract and complex patterns, DL has been applied in neuroimaging studies of psychiatric and neurological disorders, which are characterised by subtle and diffuse alterations. Here we introduce the underlying concepts of DL and review studies that have used this approach to classify brain-based disorders. The results of these studies indicate that DL could be a powerful tool in the current search for biomarkers of psychiatric and neurologic disease. We conclude our review by discussing the main promises and challenges of using DL to elucidate brain-based disorders, as well as possible directions for future research. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

  7. Hippotherapy acute impact on heart rate variability non-linear dynamics in neurological disorders.

    Science.gov (United States)

    Cabiddu, Ramona; Borghi-Silva, Audrey; Trimer, Renata; Trimer, Vitor; Ricci, Paula Angélica; Italiano Monteiro, Clara; Camargo Magalhães Maniglia, Marcela; Silva Pereira, Ana Maria; Rodrigues das Chagas, Gustavo; Carvalho, Eliane Maria

    2016-05-15

    Neurological disorders are associated with autonomic dysfunction. Hippotherapy (HT) is a therapy treatment strategy that utilizes a horse in an interdisciplinary approach for the physical and mental rehabilitation of people with physical, mental and/or psychological disabilities. However, no studies have been carried out which evaluated the effects of HT on the autonomic control in these patients. Therefore, the objective of the present study was to investigate the effects of a single HT session on cardiovascular autonomic control by time domain and non-linear analysis of heart rate variability (HRV). The HRV signal was recorded continuously in twelve children affected by neurological disorders during a HT session, consisting in a 10-minute sitting position rest (P1), a 15-minute preparatory phase sitting on the horse (P2), a 15-minute HT session (P3) and a final 10-minute sitting position recovery (P4). Time domain and non-linear HRV indices, including Sample Entropy (SampEn), Lempel-Ziv Complexity (LZC) and Detrended Fluctuation Analysis (DFA), were calculated for each treatment phase. We observed that SampEn increased during P3 (SampEn=0.56±0.10) with respect to P1 (SampEn=0.40±0.14, p<0.05), while DFA decreased during P3 (DFA=1.10±0.10) with respect to P1 (DFA=1.26±0.14, p<0.05). A significant SDRR increase (p<0.05) was observed during the recovery period P4 (SDRR=50±30ms) with respect to the HT session period P3 (SDRR=30±10ms). Our results suggest that HT might benefit children with disabilities attributable to neurological disorders by eliciting an acute autonomic response during the therapy and during the recovery period. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Intestinal helminthiasis in children with chronic neurological disorders in Benin City, Nigeria: intensity and behavioral risk factors.

    Science.gov (United States)

    Nwaneri, Damia Uchechukwu; Ibadin, Michael Okoeguale; Ofovwe, Gabriel Egberue; Sadoh, Ayebo Evawere

    2013-05-01

    Behavioral aberrations such as nail biting, finger sucking, and pica have been postulated as risk factors that enhance helminths ova transmission. These aberrations may present commonly in children with chronic neurological disorders and predispose them to heavy intensity of intestinal helminthiasis. This comparative cross-sectional study was to determine the prevalence, intensity, and behavioral risk factors for intestinal helminthiasis in children with chronic neurological disorders and apparently healthy controls. Fresh stool samples from 155 children (2-17 years) with chronic neurological disorders seen at the child neurology clinic and 155 age and sex matched controls from nursery and primary schools in Benin City were analyzed using the Kato-Katz technique for detection of ova of helminths from November 2008 to April 2009. The prevalence of intestinal helminthiasis (31.0%) was significantly higher in children with chronic neurological disorders compared with the controls (19.4%) (P=0.03). The intensity of infections in both groups was light ranging 24-144 eggs per gram. Ascaris lumbricoides, Trichuris trichiura and hookworm were the intestinal helminths isolated in both groups. Behavioral aberrations were significantly more represented in the subjects than in the controls (Pworming exercise were practices significantly associated with decreased prevalence of intestinal helminthiasis in the subjects and controls. Behavioral modification in children with chronic neurological disorders should be an integral part of the control program for intestinal helminthiasis.

  9. Systematic review: Efficacy and safety of medical marijuana in selected neurologic disorders

    Science.gov (United States)

    Koppel, Barbara S.; Brust, John C.M.; Fife, Terry; Bronstein, Jeff; Youssof, Sarah; Gronseth, Gary; Gloss, David

    2014-01-01

    Objective: To determine the efficacy of medical marijuana in several neurologic conditions. Methods: We performed a systematic review of medical marijuana (1948–November 2013) to address treatment of symptoms of multiple sclerosis (MS), epilepsy, and movement disorders. We graded the studies according to the American Academy of Neurology classification scheme for therapeutic articles. Results: Thirty-four studies met inclusion criteria; 8 were rated as Class I. Conclusions: The following were studied in patients with MS: (1) Spasticity: oral cannabis extract (OCE) is effective, and nabiximols and tetrahydrocannabinol (THC) are probably effective, for reducing patient-centered measures; it is possible both OCE and THC are effective for reducing both patient-centered and objective measures at 1 year. (2) Central pain or painful spasms (including spasticity-related pain, excluding neuropathic pain): OCE is effective; THC and nabiximols are probably effective. (3) Urinary dysfunction: nabiximols is probably effective for reducing bladder voids/day; THC and OCE are probably ineffective for reducing bladder complaints. (4) Tremor: THC and OCE are probably ineffective; nabiximols is possibly ineffective. (5) Other neurologic conditions: OCE is probably ineffective for treating levodopa-induced dyskinesias in patients with Parkinson disease. Oral cannabinoids are of unknown efficacy in non–chorea-related symptoms of Huntington disease, Tourette syndrome, cervical dystonia, and epilepsy. The risks and benefits of medical marijuana should be weighed carefully. Risk of serious adverse psychopathologic effects was nearly 1%. Comparative effectiveness of medical marijuana vs other therapies is unknown for these indications. PMID:24778283

  10. Neurological Soft Signs in Obsessive Compulsive Disorder: Standardised Assessment and Comparison with Schizophrenia

    Directory of Open Access Journals (Sweden)

    D. Bolton

    1999-01-01

    Full Text Available While several studies have detected raised levels of neurological soft signs in patients with obsessive compulsive disorder (OCD, the specificity of these abnormalities remains uncertain. This study used a new standardised measure, the Cambridge Neurological Inventory (CNI, to assess soft signs in 51 subjects with OCD. Comparison was made with data on patients with schizophrenia and a non-clinical control group from a previously reported study. Individuals with OCD showed raised levels of soft signs compared with non-clinical controls in many categories of the CNI: Motor Coordination, Sensory Integration, Primitive Reflexes, Extrapyramidal Signs, and Failure of Suppression. Compared with patients with schizophrenia, the OCD group had lower levels of neurological signs in some CNI categories: Hard Signs, Motor Co-ordination, Tardive Dyskinesia, Catatonic Signs, and Extrapyramidal Signs. However, levels of soft signs in the OCD group did not significantly differ from those in the schizophrenia group in other CNI categories: Sensory Integration, Primitive Reflexes and Failure of Suppression. The significance of these patterns of findings is discussed.

  11. Charcot-Marine-Tooth CMT-2 Polyneuropathy Syndrome A Case Study.

    Directory of Open Access Journals (Sweden)

    Arbind Kumar Choudhary

    2015-06-01

    Full Text Available Abstract Charcot-Marie-Tooth disease CMT refers to the inherited peripheral neuropathies affect approximately one in 2500 people they are among the most common inherited neurological disorders. The majority of CMT patients have autosomal dominant inheritance although X-linked dominant and autosomal recessive forms also exist. The majority of cases are demyelinating although up to one third appear to be primary axonal or neuronal disorders. A patient of 9-year-old girl visited our hospital because of began to suffer from an insidious onset of progressive distal weakness and numbness and muscle twitching in both in her upper and lower limbs. Nerve conduction studies showed sensory nerve conduction SNCV of bilateral median and ulnar nerve was reduced in upper limb and bilateral sural nerve was reduced in lower limb While in case of motor nerve conduction MNCV bilateral median and ulnar nerve was reduced in upper limb and common peroneal nerve CPN as well as posterior tibial nerve was decreased leg. F response latencies were markedly prolonged in patient. Family history along with electrophysiological studied showed It was typical case of autosomal dominant CMT 2 axonal neuropathy. CMT is currently an untreatable disorder and at the moment the treatment of CMT is only supportive as there are no drugs available that would halt the disease symptoms. The care of a CMT patient is challenging for the health care team.

  12. Pattern of traditional medicine use by adult Saudi patients with neurological disorders.

    Science.gov (United States)

    Mohammad, Yousef; Al-Ahmari, Ahmed; Al-Dashash, Fahad; Al-Hussain, Fawaz; Al-Masnour, Firas; Masoud, Abdullah; Jradi, Hoda

    2015-04-01

    Traditional medicine (TM) has been established as a two-edged sword. On one edge numerous forms of TM have been proven safe and effective, while on the other edge various modes of TM have been shown to be futile and potentially dangerous. Resorting to TM, especially for chronic diseases, is common world-wide and includes Saudi Arabia. Most neurological diseases are chronic. No data is available on the utilization of TM among patients with neurological disorders. We conducted this study to assess for the prevalence, pattern, perception and triggers for TM use by the adult Saudi patients with neurological disorders. A survey written in Arabic and comprised of 15 questions was used to collect data on the practice of TM among the neurology patients of King Saud University Ambulatory Clinic. The questions in the survey pertain mainly to the frequency of TM practice, its form and the patient's opinion of this practice. The data was collected through a face to face interview by three medical students who were instructed on the survey questions prior to the launch of the study. 292 patients completed the survey (35.9% males and 64.0% females). 67% (n = 196) of the sample used TM. Cupping or what is commonly known as "hojamah" was the most prevalent method (45.4%) followed by herbs, skin cauterization and the Reciting of the Holy Quran (42.3%, 33.7% and 20.4% respectively). The prevalence of TM use did not differ across gender (chi-sq = 2.02; p-value = 0.15), level of education (chi-sq = 4.02; p-value = 0.40), health status (chi-sq = 2.29; p-value = 0.68), age groups (chi-sq = 5.12; p-value = 0.16), or perception toward TM (chi-sq = 2.67; p-value = 0.26) in this population. The practice of TM is common among the neurology patients of Saudi Arabia. Cupping, herbs, and skin cauterization, which can be harmful when wrongly employed, are frequently utilized in this patient population. Measures and policies to endorse the appropriate use of TM by Saudi society must be

  13. Conversion Disorder, Functional Neurological Symptom Disorder, and Chronic Pain: Comorbidity, Assessment, and Treatment.

    Science.gov (United States)

    Tsui, Patricia; Deptula, Andrew; Yuan, Derek Y

    2017-06-01

    This paper examines the overlap of conversion disorder with chronic pain conditions, describes ways to assess for conversion disorder, and provides an overview of evidence-based treatments for conversion disorder and chronic pain, with a focus on conversion symptoms. Conversion disorder is a significant problem that warrants further study, given that there are not many well-established guidelines. Accurate and timely assessment should help move treatment in a more fruitful direction and avoid unnecessary medical interventions. Advances in neuroimaging may also help further our understanding of conversion disorder. Creating a supportive environment and a collaborative treatment relationship and improving understanding of conversion symptoms appear to help individuals diagnosed with conversion disorder engage in appropriate treatments. Novel uses of earlier treatments, such as hypnosis and psychodynamic approaches, could potentially be beneficial and require a more vigorous and systematic study. There are treatments that produce significant improvements in functioning and reduction of physical symptoms from conversion disorder even for very severe cases. Hypnotherapy, cognitive behavioral therapy, and inpatient multidisciplinary treatment with intensive physiotherapy for severe cases have the most evidence to support reduction of symptoms. Components of treatment for conversion disorder overlap with treatments for chronic pain and can be used together to produce therapeutic effects for both conditions. Treatment needs to be tailored for each individual's specific symptoms.

  14. Frontiers in therapeutic development of allopregnanolone for Alzheimer's disease and other neurological disorders

    Directory of Open Access Journals (Sweden)

    Ronald W. Irwin

    2014-07-01

    Full Text Available Allopregnanolone (Allo, a neurosteroid, has emerged as a promising promoter of endogenous regeneration in brain. In a mouse model of Alzheimer’s disease, Allo induced neurogenesis, oligodendrogenesis, white matter generation and cholesterol homeostasis while simultaneously reducing β-amyloid and neuroinflammatory burden. Allo activates signaling pathways and gene expression required for regeneration of neural stem cells and their differentiation into neurons. In parallel, Allo activates systems to sustain cholesterol homeostasis and reduce β-amyloid generation. To advance Allo into studies for chronic human neurological conditions, we examined translational and clinical parameters: dose, regimen, route, formulation, outcome measures, and safety regulations. A treatment regimen of once per week at sub-sedative doses of Allo was optimal for regeneration and reduction in Alzheimer’s pathology. This regimen had a high safety profile following chronic exposure in aged normal and Alzheimer’s mice. Formulation of Allo for multiple routes of administration has been developed for both preclinical and clinical testing. Preclinical evidence for therapeutic efficacy of Allo spans multiple neurological diseases including Alzheimer’s, Parkinson’s, multiple sclerosis, Niemann-Pick, diabetic neuropathy, status epilepticus, and traumatic brain injury. To successfully translate Allo as a therapeutic for multiple neurological disorders, it will be necessary to tailor dose and regimen to the targeted therapeutic mechanisms and disease etiology. Treatment paradigms conducted in accelerated disease models in young animals have a low probability of successful translation to chronic diseases in adult and aged humans. Gender, genetic risks, stage and burden of disease are critical determinants of efficacy. This review focuses on recent advances in development of Allo for Alzheimer’s disease that have the potential to accelerate therapeutic translation for

  15. The Neurological Sequelae of Neonatal Hyperbilirubinemia: Definitions, Diagnosis and Treatment of the Kernicterus Spectrum Disorders (KSDs).

    Science.gov (United States)

    Le Pichon, Jean-Baptiste; Riordan, Sean M; Watchko, Jon; Shapiro, Steven M

    2017-01-01

    Despite its lengthy history, the study of jaundice, hyperbilirubinemia and kernicterus suffers from a lack of clarity and consistency in the key terms used to describe both the clinical and pathophysiological nature of these conditions. For example, the term Bilirubin-induced Neurological Dysfunction (BIND) has been used to refer to all neurological sequelae caused by exposure to high levels of bilirubin, to only mild neurological sequelae, or to scoring systems that quantitate the progressive stages of Acute Bilirubin Encephalopathy (ABE). We seek to clarify and simplify terminology by introducing, defining, and proposing new terms and diagnostic criteria for kernicterus. We propose a systematic nomenclature based on pathophysiological and clinical criteria, presenting a logical argument for each term. Acknowledging observations that kernicterus is symptomatically broad and diverse, we propose the use of the overarching term Kernicterus Spectrum Disorders (KSDs) to encompass all the neurological sequelae of bilirubin neurotoxicity including Acute Bilirubin Neurotoxicity (ABE). We further suggest subclassification of KSDs based on the principal disabling features of kernicterus (motor, auditory). Finally, we suggest the term subtle KSD to designate a child with a history of significant bilirubin neurotoxicity with mild or subtle developmental delays. We conclude with a brief description of the limited treatments currently available for KSD, thereby underscoring the importance of further research. We believe that adopting a systematic nomenclature for the spectrum of clinical consequences of hyperbilirubinemia will help unify the field and promote more effective research in both prevention and treatment of KSDs. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  16. [Conversion disorder: from DSM IV to DSM 5 or from a psychiatric to a neurological diagnosis].

    Science.gov (United States)

    Vermeulen, M; Willems, M H A

    2015-01-01

    According to one of the diagnostic criteria of the dsm iv for conversion disorder there has to be a temporal relationship between psychological factors and the onset, or the worsening, of the symptoms. This criterion has been omitted in the dsm-5. Another criterion, namely that the symptoms are not produced intentionally, has also been abandoned. A new recommendation is that therapists should look for neurological symptoms that support the diagnosis. To investigate whether studies support the changes in the criteria. We searched literature using PubMed. When the symptoms first appear, trauma or stress in 37% of patients is of a physical rather than a psychological nature. Different forms of stress were found in equal proportions (20%) in patients with or without conversion disorder. There are no specific stressors, except possibly in patients with dysphonia. The percentages of childhood abuse vary widely, namely from 0 to 85%. The characteristic phenomenon of 'la belle indifference' occurs in only 3% of patients with conversion disorder versus only 2% of controls. Most of the 'positive' clinical tests for partial paralysis and sensory and gait disorders are highly specific. There are no reliable tests for distinguishing conversion disorder from simulation. The changes of the criteria are supported by recent studies.

  17. Copper mediated neurological disorder: visions into amyotrophic lateral sclerosis, Alzheimer and Menkes disease.

    Science.gov (United States)

    Ahuja, Anami; Dev, Kapil; Tanwar, Ranjeet S; Selwal, Krishan K; Tyagi, Pankaj K

    2015-01-01

    Copper (Cu) is a vital redox dynamic metal that is possibly poisonous in superfluous. Metals can traditionally or intricately cause propagation in reactive oxygen species (ROS) accretion in cells and this may effect in programmed cell death. Accumulation of Cu causes necrosis that looks to be facilitated by DNA damage, followed by activation of P53. Cu dyshomeostasis has also been concerned in neurodegenerative disorders such as Alzheimer, Amyotrophic lateral sclerosis (ALS) or Menkes disease and is directly related to neurodegenerative syndrome that usually produces senile dementia. These mortal syndromes are closely related with an immense damage of neurons and synaptic failure in the brain. This review focuses on copper mediated neurological disorders with insights into amyotrophic lateral sclerosis, Alzheimer and Menkes disease. Copyright © 2014 Elsevier GmbH. All rights reserved.

  18. Ketogenic diet and childhood neurological disorders other than epilepsy: an overview.

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    Verrotti, Alberto; Iapadre, Giulia; Pisano, Simone; Coppola, Giangennaro

    2017-05-01

    In the last years, ketogenic diet (KD) has been experimentally utilized in various childhood neurologic disorders such as mitochondriopathies, alternating hemiplegia of childhood (AHC), brain tumors, migraine, and autism spectrum disorder (ASD). The aim of this review is to analyze how KD can target these different medical conditions, highlighting possible mechanisms involved. Areas covered: We have conducted an analysis on literature concerning KD use in mitochondriopathies, AHC, brain tumors, migraine, and ASD. Expert commentary: The role of KD in reducing seizure activity in some mitochondriopathies and its efficacy in pyruvate dehydrogenase deficiency is known. Recently, few cases suggest the potentiality of KD in decreasing paroxysmal activity in children affected by AHC. A few data support its potential use as co-adjuvant and alternative therapeutic option for brain cancer, while any beneficial effect of KD on migraine remains unclear. KD could improve cognitive and social skills in a subset of children with ASD.

  19. Door-to-door survey of major neurological disorders (project in Al Quseir City, Red Sea Governorate, Egypt

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    El Tallawy HN

    2013-05-01

    Full Text Available Hamdy NA El Tallawy,1 Wafaa MA Farghaly,1 Tarek A Rageh,1 Ghaydaa A Shehata,1 Reda Badry,1 Nabil A Metwally,2 Esam A El Moselhy,2 Mahmoud Hassan,2 Mohamed A Sayed,3 Ahmed A Waris,1 Yaser Hamed,2 Islam Shaaban,2 Mohamed A Hamed,1 Mahmoud Raafat Kandil11Department of Neurology, Faculty of Medicine, Assiut University, Assiut, Egypt; 2Department of Neurology and Public Health, Faculty of Medicine, Al-Azhar University (Assiut branch, Assiut, Egypt; 3Department of Neurology, Faculty of Medicine, Sohag University, Sohag, EgyptAbstract: A door-to-door survey, including every household, was conducted for all inhabitants of Al Quseir City (33,283, Red Sea Governorate, Egypt by three specialists of neurology as well as nine senior staff members of neurology and 15 female social workers to assess the epidemiology of major neurological disorders. Over six phases, from July 1, 2009 to January 31, 2012, screening of all eligible people in the population was carried out, by which case ascertainment of all major neurological disorders included in the study was done according to the accepted definitions and diagnostic criteria of the World Health Organization. The order of frequency of prevalence of the studied neurological disorders was dementia (3.83% for those aged > 60 years, migraine (2.8% for those aged > 8 years, stroke (6.2/1000 for those aged > 20 years, epilepsy (5.5/1000, Parkinson’s disease (452.1/100,000 for those aged > 40 years, cerebral palsy (3.6/1000 among children 37 years, chorea (21.03/100,000, athetosis (15/100,000, and multiple sclerosis (13.74/100,000. The incidence rates of stroke, epilepsy, and Bell’s palsy were 181/100,000, 48/100,000, and 98.9/100,000 per year, respectively.Keywords: prevalence, incidence, neurological disorders

  20. Charcot-Leyden crystals in pleural fluids.

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    Naylor, B; Novak, P M

    1985-01-01

    Charcot-Leyden crystals have rarely been reported in serous fluids. We present eight examples of Charcot-Leyden crystals, all found in eosinophilic pleural effusions. The crystals were found in toluidine blue-stained wet films of pleural fluid after either the fluid or the wet film had stood for at least 24 hours at 4 C.

  1. Prevalence nutritional disorders among patients hospitalised for stroke and discopathy in the neurology department

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    Regina Sierżantowicz

    2015-10-01

    Full Text Available Introduction: Nutritional disorders pose a huge health problem worldwide. In Poland, symptoms of malnutrition are found on admission to hospital in approximately 30% of patients. Among neurological disorders that predispose to malnutrition, brain injuries are the most frequent. The disease leads to difficulties with self-care, disorientation, reduced intellectual capacity, and dysphagia. Acute spinal pain syndromes affect weight loss because of persistent severe pain, and frequent dizziness and headaches accompanying cervical discopathy. Aim of the research: To assess the degree of malnutrition in patients with stroke and discopathy hospitalised in the neurology ward. Material and methods : The study group consisted of 141 patients, including 90 with stroke and 51 with discopathy, hospitalised in the neurology ward. Research material was collected based on medical records and a proprietary questionnaire. Body mass index (BMI was calculated and assessed for each patient on admission and after hospitalisation. Results and conclusions: The study sample consisted of a similar group of women (49% and men (51% aged from 30 to over 70 years. Ischaemic stroke was diagnosed more often in women (66.2%, whereas discopathy was more common in men (43.4%. The differences in BMI present on admission and after hospitalisation in men and women indicated a falling tendency. A slightly greater drop in BMI was found in women after hospital stay (from 24.1 to 23.3 kg/m 2 . The lowest BMI on admission was observed in students and pensioners. Long-term hospitalisation significantly affected weight reduction – the longer the patients were hospitalised, the lower their BMI was. Preliminary assessment of the nutrition status on admission to a hospital ward and customising individual diets may help reduce the effects of malnutrition.

  2. Neonatal neurological disorders involving the brainstem: neurosonographic approaches through the squamous suture and the foramen magnum

    Energy Technology Data Exchange (ETDEWEB)

    Tu, Yi-Fang [National Cheng Kung University Hospital, Department of Emergency Medicine, Tainan (Taiwan); Chen, Cheng-Yu [National Defense Medical Center, Department of Radiology, Taipei (Taiwan); Lin, Yuh-Jey [National Cheng Kung University Hospital, Department of Pediatrics, Tainan (Taiwan); Chang, Ying-Chao [Kaohsiung Chang Gung Children Hospital, Department of Pediatrics, Kaohsiung (Taiwan); Huang, Chao-Ching [National Cheng Kung University Hospital, Department of Pediatrics, Tainan (Taiwan); National Cheng Kung University Hospital, Department of Institute of Molecular Medicine, Tainan (Taiwan)

    2005-09-01

    Brainstem damage which often indicates a critical condition is usually underestimated by trans-anterior-fontanel neurosonography (NS) owing to the far-field limitations. Instead, NS alternately scanning through the squamous suture of the temporal bones and the foramen magnum could provide a better visualization of the brainstem structures. The NS characteristics of brainstem lesions caused by various neonatal neurological disorders, such as hypoxic-ischemic encephalopathy (HIE), metabolic encephalopathy, birth trauma and bacterial meningoencephalitis, can be depicted at the acute stage. An echogenic change in the midbrain was found in patients with HIE or metabolic encephalopathy. In addition to the echogenic change, bilateral transtentorial temporal lobe herniation distorting the contour of the midbrain was observed in a patient with group B streptococcus meningoencephalitis, whereas echogenic changes at the level of the pons and/or the medulla oblongata, mainly localized in the dorsal part, could be observed in newborns with severe HIE, maple syrup urine disease or birth trauma. In this pictorial assay, we demonstrate the feasibility of NS imaging in evaluating the entire brainstem structure of critically ill neonates in the near field and illustrate the characteristic features of brainstem involvement in various neonatal neurological disorders along with computed tomography or magnetic resonance imaging correlation. (orig.)

  3. Lost in Translation: Defects in Transfer RNA Modifications and Neurological Disorders

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    Andrea Bednářová

    2017-05-01

    Full Text Available Transfer RNAs (tRNAs are key molecules participating in protein synthesis. To augment their functionality they undergo extensive post-transcriptional modifications and, as such, are subject to regulation at multiple levels including transcription, transcript processing, localization and ribonucleoside base modification. Post-transcriptional enzyme-catalyzed modification of tRNA occurs at a number of base and sugar positions and influences specific anticodon–codon interactions and regulates translation, its efficiency and fidelity. This phenomenon of nucleoside modification is most remarkable and results in a rich structural diversity of tRNA of which over 100 modified nucleosides have been characterized. Most often these hypermodified nucleosides are found in the wobble position of tRNAs, where they play a direct role in codon recognition as well as in maintaining translational efficiency and fidelity, etc. Several recent studies have pointed to a link between defects in tRNA modifications and human diseases including neurological disorders. Therefore, defects in tRNA modifications in humans need intensive characterization at the enzymatic and mechanistic level in order to pave the way to understand how lack of such modifications are associated with neurological disorders with the ultimate goal of gaining insights into therapeutic interventions.

  4. Cohort Study on Respiratory and Neurological Disorders among Workers in a Bone Glue Factory in Egypt

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    MA Al-Batanony

    2012-03-01

    Full Text Available Background: Glues are strong, liquid adhesive derived from animal tissues. It has been shown that glue sniffing is associated with demyelinating polyneuropathy. The low molecular weight agents which cause occupational lung disease have generally included the isocyanates exposure to which could result in asthma among workers. Toluene is also used widely in glue and adhesive industry and households where toluene exposure and abuse can occur. Objectives: To study some respiratory and neurological disorders that may arise in workers in a bone glue factory in Queisna industrial zone, Menoufyia governorate, Egypt. Methods: In a historical cohort study, the exposed participants (n=50 were recruited from workers in a bone glue factory in Queisna industrial zone, Menoufyia governorate. The unexposed group was selected from workers' relatives who had never worked in glue industry. All participants completed a pre-designed questionnaire on personal and occupational histories. Pulmonary function tests as well as electromyography (EMG were performed for all participants. Urinary hippuric acid was also measure in all participants. Results: The prevalence of cough, asthmatic attacks and paresthesia were significantly higher among exposed than unexposed participants. Abnormal spirometric measurements (particularly towards obstruction, abnormal EMG and positive urinary hippuric acid were significantly more prevalent among exposed than unexposed group. Conclusion: Spirometry and EMG should be included in the periodic medical examination for exposed workers for early detection of respiratory and neurological disorders. Urinary hippuric acid could be a useful indicator of the nerve conduction abnormalities and should be measured periodically for these workers.

  5. Video Analysis of Human Gait and Posture to Determine Neurological Disorders

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    Ivan Lee

    2008-08-01

    Full Text Available This paper investigates the application of digital image processing techniques to the detection of neurological disorder. Visual information extracted from the postures and movements of a human gait cycle can be used by an experienced neurologist to determine the mental health of the person. However, the current visual assessment of diagnosing neurological disorder is based very much on subjective observation, and hence the accuracy of diagnosis heavily relies on experience. Other diagnostic techniques employed involve the use of imaging systems which can only be operated under highly constructed environment. A prototype has been developed in this work that is able to capture the subject's gait on video in a relatively simple setup, and from which to process the selected frames of the gait in a computer. Based on the static visual features such as swing distances and joint angles of human limbs, the system identifies patients with Parkinsonism from the test subjects. To our knowledge, it is the first time swing distances are utilized and identified as an effective means for characterizing human gait. The experimental results have shown a promising potential in medical application to assist the clinicians in diagnosing Parkinsonism.

  6. Devices for Ambulatory Monitoring of Sleep-Associated Disorders in Children with Neurological Diseases

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    Adriana Ulate-Campos

    2017-12-01

    Full Text Available Good sleep quality is essential for a child’s wellbeing. Early sleep problems have been linked to the later development of emotional and behavioral disorders and can negatively impact the quality of life of the child and his or her family. Sleep-associated conditions are frequent in the pediatric population, and even more so in children with neurological problems. Monitoring devices can help to better characterize sleep efficiency and sleep quality. They can also be helpful to better characterize paroxysmal nocturnal events and differentiate between nocturnal seizures, parasomnias, and obstructive sleep apnea, each of which has a different management. Overnight ambulatory detection devices allow for a tolerable, low cost, objective assessment of sleep quality in the patient’s natural environment. They can also be used as a notification system to allow for rapid recognition and prompt intervention of events like seizures. Optimal monitoring devices will be patient- and diagnosis-specific, but may include a combination of modalities such as ambulatory electroencephalograms, actigraphy, and pulse oximetry. We will summarize the current literature on ambulatory sleep devices for detecting sleep disorders in children with neurological diseases.

  7. On the early history of male hysteria and psychic trauma. Charcot's influence on Freudian thought.

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    Libbrecht, K; Quackelbeen, J

    1995-10-01

    This paper discusses the influence of Jean-Martin Charcot's views on Sigmund Freud's early theory of hysteria and the notion of psychical trauma. We consider the early history of both psychical trauma and male hysteria, for in Charcot's view traumatic hysteria and male hysteria are identical. Freud's two 1886 lectures on male hysteria, delivered after his return from Paris, are crucial to the subject because they present Freud's first impressions of Charcot and his teaching. Some of the ideas presented in the two lectures foreshadow Freud's later generalization of the etiological role of trauma and his theory of the role of psychical trauma in the genesis of hysteria; that is, each hysterical symptom is due to a psychical trauma reviving an earlier traumatic event--the so-called principle of deferred action (Nachträglichkeit). Several arguments substantiate the thesis that Freud's notion of psychical (sexual) trauma was developed in reference to Charcot's notion of traumatic hysteria, and that the early psychoanalytic theory of psychical trauma is clearly indebted to Freud's encounter with Charcot's male traumatic hysterical patients. The discussed Freudian development points out the major role of (physical) traumata in eliciting psychopathological pictures and in this way is of definite historical relevance for the present-day discussion on the traumatic nature of the so-called multiple personality syndrome and other dissociative disorders, and post-traumatic stress disorders.

  8. Assessment of speech in neurological disorders: development of a Swahili screening test.

    Science.gov (United States)

    Miller, Nick; Mshana, Gerry; Msuya, Oliva; Dotchin, Catherine; Walker, Richard; Aris, Eric

    2012-12-01

    Assessments for acquired motor-speech disorders that look at movements of the articulators would appear at first glance to be universal. This may be true for the most basic non-speech aspects of movement. We argue that assessments for speech motor control must be attuned to language-specific variables to be fully valid. We describe the rationale for, and development of a motor-speech-disorder screening test for Swahili speakers which includes impairment measures as well as measures of intelligibility and speech-voice naturalness. We further describe its initial validation in terms of content validity, feasibility of administration and scoring without requirements for lengthy training and technical expertise and application to groups of people with and without Parkinson's disease in Tanzania. Results indicate that the protocol is ready to use in so far as it is acceptable to users (clinicians, patients), is feasible to use, shows good interrater reliability, and is capable of differentiating performance in healthy speakers and those whose speech is disordered. We highlight needs for further development, including issues around training, development of local norms for healthy speakers and for speakers with a variety of neurological disturbances, and extension of the tool to cover culturally valid assessment of impact of communication disorders.

  9. Clinical Utility and Lifespan Profiling of Neurological Soft Signs in Schizophrenia Spectrum Disorders

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    Chan, Raymond C. K.; Xie, Weizhen; Geng, Fu-lei; Wang, Ya; Lui, Simon S. Y.; Wang, Chuan-yue; Yu, Xin; Cheung, Eric F. C.; Rosenthal, Robert

    2016-01-01

    Neurological soft signs (NSSs) bear the promise for early detection of schizophrenia spectrum disorders. Nonetheless, the sensitivity and specificity of NSSs in the psychosis continuum remains a topic of controversy. It is also unknown how NSSs reveal neurodevelopmental abnormality in schizophrenia. We investigated the effect sizes of NSSs in differentiating individuals with schizophrenia spectrum disorders from individuals with other psychiatric conditions and from covariate-matched healthy subjects. We also investigated the partitioned age-related variations of NSSs in both schizophrenia and healthy individuals. NSSs were assessed by the abridged version of the Cambridge Neurological Inventory (CNI) in 3105 participants, consisting of healthy individuals (n =1577), unaffected first-degree relatives of schizophrenia patients (n = 155), individuals with schizotypal personality disorder (n = 256), schizophrenia patients (n = 738), and other psychiatric patients (n = 379). Exact matching and propensity score matching procedures were performed to control for covariates. Multiple regression was used to partition age-related variations. Individuals along the schizophrenia continuum showed elevated levels of NSSs, with moderate effect sizes, in contrast to other psychiatric patients who had minimal NSSs, as well as matched healthy controls. Furthermore, the age-and-NSS relationship in schizophrenia patients was represented by a flat but overall elevated pattern, in contrast to a U-shaped pattern in healthy individuals. In sum, NSSs capture a moderate portion of psychosis proneness with reasonable specificity. Lifespan profiling reveals an abnormal developmental trajectory of NSSs in schizophrenia patients, which supports the endophenotype hypothesis of NSSs by associating it with the neurodevelopmental model of schizophrenia. PMID:26712863

  10. Disrupted avoidance learning in functional neurological disorder: Implications for harm avoidance theories.

    Science.gov (United States)

    Morris, Laurel S; To, Benjaman; Baek, Kwangyeol; Chang-Webb, Yee-Chien; Mitchell, Simon; Strelchuk, Daniela; Mikheenko, Yevheniia; Phillips, Wendy; Zandi, Michael; Jenaway, Allison; Walsh, Cathy; Voon, Valerie

    2017-01-01

    Functional neurological disorder (FND) is an elusive disorder characterized by unexplained neurological symptoms alongside aberrant cognitive processing and negative affect, often associated with amygdala reactivity. We examined the effect of negative conditioning on cognitive function and amygdala reactivity in 25 FND patients and 20 healthy volunteers (HV). Participants were first conditioned to stimuli paired with negative affective or neutral (CS +/CS -) information. During functional MRI, subjects then performed an instrumental associative learning task to avoid monetary losses in the context of the previously conditioned stimuli. We expected that FND patients would be better at learning to avoid losses when faced with negatively conditioned stimuli (increased harm avoidance). Multi-echo resting state fMRI was also collected from the same subjects and a robust denoising method was employed, important for removing motion and physiological artifacts. FND subjects were more sensitive to the negative CS + compared to HV, demonstrated by a reinforcement learning model. Contrary to expectation, FND patients were generally more impaired at learning to avoid losses under both contexts (CS +/CS -), persisting to choose the option that resulted in a negative outcome demonstrated by both behavioural and computational analyses. FND patients showed enhanced amygdala but reduced dorsolateral prefrontal cortex responses when they received negative feedback. Patients also had increased resting state functional connectivity between these two regions. FND patients had impaired instrumental avoidance learning, findings that parallel previous observations of impaired action-outcome binding. FND patients further show enhanced behavioural and neural sensitivity to negative information. However, this did not translate to improved avoidance learning. Put together, our findings do not support the theory of harm avoidance in FND. We highlight a potential mechanism by which

  11. Mammalian Target of Rapamycin: Hitting the Bull's-Eye for Neurological Disorders

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    Zhao Zhong Chong

    2010-01-01

    Full Text Available The mammalian target of rapamycin (mTOR and its associated cell signaling pathways have garnered significant attention for their roles in cell biology and oncology. Interestingly,the explosion of information in this field has linked mTOR to neurological diseases with promising initial studies. mTOR, a 289 kDa serine/threonine protein kinase, plays an important role in cell growth and proliferation and is activated through phosphorylation in response to growth factors, mitogens and hormones. Growth factors, amino acids, cellular nutrients and oxygen deficiency can downregulate mTOR activity. The function of mTOR signaling is mediated primarily through two mTOR complexes: mTORC1 and mTORC2. mTORC1 initiates cap-dependent protein translation, a rate-limiting step of protein synthesis, through the phosphorylation of the targets eukaryotic initiation factor 4E-binding protein 1 (4EBP1 and p70 ribosomal S6 kinase (p70S6K. In contrast, mTORC2 regulates development of the cytoskeleton and also controls cell survival. Although closely tied to tumorigenesis, mTOR and the downstream signaling pathways are significantly involved in the central nervous system (CNS with synaptic plasticity, memory retention, neuroendocrine regulation associated with food intake and puberty and modulation of neuronal repair following injury. The signaling pathways of mTOR also are believed to be a significant component in a number of neurological diseases, such as Alzheimer disease, Parkinson disease and Huntington disease, tuberous sclerosis, neurofibromatosis, fragile X syndrome, epilepsy, traumatic brain injury and ischemic stroke. Here we describe the role of mTOR in the CNS and illustrate the potential for new strategies directed against neurological disorders.

  12. The use of ketogenic diet in special situations: expanding use in intractable epilepsy and other neurologic disorders

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    Munhyang Lee

    2012-09-01

    Full Text Available The ketogenic diet has been widely used and proved to be effective for intractable epilepsy. Although the mechanisms underlying its antiepileptic effects remain to be proven, there are increasing experimental evidences for its neuroprotective effects along with many researches about expanding use of the diet in other neurologic disorders. The first success was reported in glucose transporter type 1 deficiency syndrome, in which the diet served as an alternative metabolic source. Many neurologic disorders share some of the common pathologic mechanisms such as mitochondrial dysfunction, altered neurotransmitter function and synaptic transmission, or abnormal regulation of reactive oxygen species, and the role of the ketogenic diet has been postulated in these mechanisms. In this article, we introduce an overview about the expanding use and emerging trials of the ketogenic diet in various neurologic disorders excluding intractable epilepsy and provide explanations of the mechanisms in that usage.

  13. The use of ketogenic diet in special situations: expanding use in intractable epilepsy and other neurologic disorders

    Science.gov (United States)

    2012-01-01

    The ketogenic diet has been widely used and proved to be effective for intractable epilepsy. Although the mechanisms underlying its anti-epileptic effects remain to be proven, there are increasing experimental evidences for its neuroprotective effects along with many researches about expanding use of the diet in other neurologic disorders. The first success was reported in glucose transporter type 1 deficiency syndrome, in which the diet served as an alternative metabolic source. Many neurologic disorders share some of the common pathologic mechanisms such as mitochondrial dysfunction, altered neurotransmitter function and synaptic transmission, or abnormal regulation of reactive oxygen species, and the role of the ketogenic diet has been postulated in these mechanisms. In this article, we introduce an overview about the expanding use and emerging trials of the ketogenic diet in various neurologic disorders excluding intractable epilepsy and provide explanations of the mechanisms in that usage. PMID:23049588

  14. Effectiveness of Music Therapy as an aid to Neurorestoration of children with severe neurological disorders

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    Maria L Bringas

    2015-11-01

    Full Text Available This study was a two-armed parallel group design aimed at testing real world effectiveness of a music therapy (MT intervention for children with severe neurological disorders. The control group received only the standard neurorestoration program and the experimental group received an additional MT Auditory Attention plus Communication (ACC protocol just before the usual occupational and speech therapy. Multivariate Item Response Theory (MIRT identified a neuropsychological status-latent variable manifested in all children and which exhibited highly significant changes only in the experimental group. Changes in brain plasticity also occurred in the experimental group, as evidenced using a Mismatch Event Related paradigm which revealed significant post intervention positive responses in the latency range between 308 and 400 ms in frontal regions. LORETA EEG source analysis identified prefrontal and midcingulate regions as differentially activated by the MT in the experimental group. Taken together, our results showing improved attention and communication as well as changes in brain plasticity in children with severe neurological impairments, highlight/comfort the importance of MT for the rehabilitation of patients across a wide range of dysfunctions.

  15. Childhood developmental disorders: an academic and clinical convergence point for psychiatry, neurology, psychology and pediatrics.

    Science.gov (United States)

    Reiss, Allan L

    2009-01-01

    Significant advances in understanding brain development and behavior have not been accompanied by revisions of traditional academic structure. Disciplinary isolation and a lack of meaningful interdisciplinary opportunities are persistent barriers in academic medicine. To enhance clinical practice, research, and training for the next generation, academic centers will need to take bold steps that challenge traditional departmental boundaries. Such change is not only desirable but, in fact, necessary to bring about a truly innovative and more effective approach to treating disorders of the developing brain. I focus on developmental disorders as a convergence point for transcending traditional academic boundaries. First, the current taxonomy of developmental disorders is described with emphasis on how current diagnostic systems inadvertently hinder research progress. Second, I describe the clinical features of autism, a phenomenologically defined condition, and Rett and fragile X syndromes, neurogenetic diseases that are risk factors for autism. Finally, I describe how the fields of psychiatry, psychology, neurology, and pediatrics now have an unprecedented opportunity to promote an interdisciplinary approach to training, research, and clinical practice and, thus, advance a deeper understanding of developmental disorders. Research focused on autism is increasingly demonstrating the heterogeneity of individuals diagnosed by DSM criteria. This heterogeneity hinders the ability of investigators to replicate research results as well as progress towards more effective, etiology-specific interventions. In contrast, fragile X and Rett syndromes are 'real' diseases for which advances in research are rapidly accelerating towards more disease-specific human treatment trials. A major paradigm shift is required to improve our ability to diagnose and treat individuals with developmental disorders. This paradigm shift must take place at all levels - training, research and clinical

  16. First report of persistent dengue-1-associated autoimmune neurological disturbance: neuromyelitis optica spectrum disorder.

    Science.gov (United States)

    Puccioni-Sohler, Marzia; Ornelas, Alice M M; de Souza, Andrea S; Cabral-Castro, Mauro Jorge; Ramos, Jessyca T M A; Rosadas, Carolina; Salgado, Maria Cecilia F; Castiglione, Alexandre A; Ferry, Fernando; Peralta, Jose Mauro; Voloch, Carolina Moreira; Tanuri, Amilcar; Tovar-Moll, Fernanda; Aguiar, Renato Santana

    2017-10-01

    Dengue virus (DENV) causes immune-mediated diseases. Neurological involvement represents a severe condition that is rarely observed in DENV-1 infection. Neuromyelitis optica (NMO)/NMO spectrum disorders (NMOSD) are idiopathic immune-mediated demyelinating syndromes of the central nervous system. We report a 17-year-old female with oligosymptomatic DENV-1 viremia, diagnosed as NMOSD. Magnetic resonance imaging showed spinal cord and brainstem lesions. Antibody for aquaporin 4 was negative. DENV-1 RNA infection was detected by serial RT-PCR and confirmed by phylogenetic analysis in serum. Although there are some reports of NMO post-dengue infection, there are not any published accounts of NMOSD with coexistent and persistent DENV-1 infection.

  17. Current Perspectives on the Beneficial Role of Ginkgo biloba in Neurological and Cerebrovascular Disorders

    Science.gov (United States)

    Nash, Kevin M.; Shah, Zahoor A.

    2015-01-01

    Ginkgo biloba extract is an alternative medicine available as a standardized formulation, EGb 761®, which consists of ginkgolides, bilobalide, and flavonoids. The individual constituents have varying therapeutic mechanisms that contribute to the pharmacological activity of the extract as a whole. Recent studies show anxiolytic properties of ginkgolide A, migraine with aura treatment by ginkgolide B, a reduction in ischemia-induced glutamate excitotoxicity by bilobalide, and an alternative antihypertensive property of quercetin, among others. These findings have been observed in EGb 761 as well and have led to clinical investigation into its use as a therapeutic for conditions such as cognition, dementia, cardiovascular, and cerebrovascular diseases. This review explores the therapeutic mechanisms of the individual EGb 761 constituents to explain the pharmacology as a whole and its clinical application to cardiovascular and neurological disorders, in particular ischemic stroke. PMID:26604665

  18. Molecular tracking of antigen-specific T cell clones in neurological immune-mediated disorders

    Science.gov (United States)

    Muraro, Paolo A.; Wandinger, Klaus-Peter; Bielekova, Bibiana; Gran, Bruno; Marques, Adriana; Utz, Ursula; McFarland, Henry F.; Jacobson, Steve; Martin, Roland

    2016-01-01

    Summary T cells recognizing self or microbial antigens may trigger or reactivate immune-mediated diseases. Monitoring the frequency of specific T cell clonotypes to assess a possible link with the course of disease has been a difficult task with currently available technology. Our goal was to track individual candidate pathogenic T cell clones, selected on the basis of previous extensive studies from patients with immune-mediated disorders of the CNS, including multiple sclerosis, HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/ TSP) and chronic Lyme neuroborreliosis. We developed and applied a highly specific and sensitive technique to track single CD4+ and CD8+ T cell clones through the detection and quantification of T cell receptor (TCR) α or β chain complementarity-determining region 3 transcripts by real-time reverse transcriptase (RT)-PCR. We examined the frequency of the candidate pathogenic T cell clones in the peripheral blood and CSF during the course of neurological disease. Using this approach, we detected variations of clonal frequencies that appeared to be related to clinical course, significant enrichment in the CSF, or both. By integrating clono-type tracking with direct visualization of antigen-specific staining, we showed that a single T cell clone contributed substantially to the overall recognition of the viral peptide/MHC complex in a patient with HAM/ TSP. T cell clonotype tracking is a powerful new technology enabling further elucidation of the dynamics of expansion of autoreactive or pathogen-specific T cells that mediate pathological or protective immune responses in neurological disorders. PMID:12477694

  19. Targeting Astrocytes for Treating Neurological Disorders: Carbon Monoxide and Noradrenaline-Induced Increase in Lactate.

    Science.gov (United States)

    Horvat, Anemari; Vardjan, Nina; Zorec, Robert

    2017-06-22

    There are at least three reasons why brain astrocytes represent a new target for treating neurological disorders. First, although the human neocortex represents over 80% of brain mass, neurons are outnumbered by non-neuronal cells, including astrocytes, a neuroglial cell type. Second, as in neurons, vesicle-based release of transmitters is present in astrocytes, however with much slower kinetics than in neurons. Third, astrocytes contain glycogen, which can be transformed to L-lactate in glycolysis. L-lactate is considered to be a fuel and a signalling molecule involved in cognition and neuroprotection. The mechanisms of neuroprotection are unclear but may be linked to carbon monoxide, a product of the heme oxygenase, an evolutionarily conserved cellular cytoprotectant. Increased levels of local carbon monoxide arising from heme oxygenase activity may increase L-lactate, but direct measurements of cytosolic L-lactate are lacking. A fluorescence resonance energy transfer-based nanosensor selective for L-lactate was used to monitor cytosolic levels of L-lactate while cultured astrocytes were exposed to carbon monoxide. The results revealed that in astrocytes exposed to carbon monoxide there is no significant increase in L-lactate, however, when noradrenaline, a potent glycogenolytic agent, is applied, cytosolic levels of L-lactate are increased, but strongly attenuated in astrocytes pretreated with carbon monoxide. These first measurements of carbon monoxide-modulated L-lactate levels in astrocytes provide evidence that the L-lactate and heme oxygenase neuroprotective systems may interact. In conclusion, not only the abundance of astrocytes but their signalling capacity using vesicles and metabolites, such as L-lactate, are valid targets for neurological disorders. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  20. Interleukin-6 analysis of 572 consecutive CSF samples from neurological disorders: A special focus on neuromyelitis optica.

    Science.gov (United States)

    Uzawa, Akiyuki; Mori, Masahiro; Masuda, Hiroki; Ohtani, Ryohei; Uchida, Tomohiko; Sawai, Setsu; Kuwabara, Satoshi

    2017-06-01

    Elevation of cerebrospinal fluid (CSF) interleukin (IL)-6 has been reported in various neurological disorders but has never been systematically analyzed. Our main objectives are to compare the CSF IL-6 levels among various neurological disorders and to evaluate the significance of CSF IL-6 measurements for the diagnosis of neuromyelitis optica (NMO). We retrospectively investigated the IL-6 levels of 572 consecutive CSF samples in patients with various neurological disorders. Additionally, the associations between clinical manifestations in NMO patients and CSF IL-6 levels were closely investigated. Among the neurological disorders, patients with NMO had the highest CSF IL-6 level. Receiver operating characteristic analysis found the optimal cutoff CSF IL-6 value for diagnosing NMO as 7.8pg/ml, and the sensitivity and specificity were 0.7317 and 0.7694, respectively. In NMO, CSF IL-6 levels were correlated with the length of the spinal cord lesion and anti-aquaporin-4 antibody-positivity and decreased after treatment. CSF IL-6 can be high in various inflammatory and non-inflammatory CNS disorders, but its upregulation appears to be the most remarkable in NMO. Copyright © 2017 Elsevier B.V. All rights reserved.

  1. Systematic review: efficacy and safety of medical marijuana in selected neurologic disorders: report of the Guideline Development Subcommittee of the American Academy of Neurology.

    Science.gov (United States)

    Koppel, Barbara S; Brust, John C M; Fife, Terry; Bronstein, Jeff; Youssof, Sarah; Gronseth, Gary; Gloss, David

    2014-04-29

    To determine the efficacy of medical marijuana in several neurologic conditions. We performed a systematic review of medical marijuana (1948-November 2013) to address treatment of symptoms of multiple sclerosis (MS), epilepsy, and movement disorders. We graded the studies according to the American Academy of Neurology classification scheme for therapeutic articles. Thirty-four studies met inclusion criteria; 8 were rated as Class I. The following were studied in patients with MS: (1) Spasticity: oral cannabis extract (OCE) is effective, and nabiximols and tetrahydrocannabinol (THC) are probably effective, for reducing patient-centered measures; it is possible both OCE and THC are effective for reducing both patient-centered and objective measures at 1 year. (2) Central pain or painful spasms (including spasticity-related pain, excluding neuropathic pain): OCE is effective; THC and nabiximols are probably effective. (3) Urinary dysfunction: nabiximols is probably effective for reducing bladder voids/day; THC and OCE are probably ineffective for reducing bladder complaints. (4) Tremor: THC and OCE are probably ineffective; nabiximols is possibly ineffective. (5) Other neurologic conditions: OCE is probably ineffective for treating levodopa-induced dyskinesias in patients with Parkinson disease. Oral cannabinoids are of unknown efficacy in non-chorea-related symptoms of Huntington disease, Tourette syndrome, cervical dystonia, and epilepsy. The risks and benefits of medical marijuana should be weighed carefully. Risk of serious adverse psychopathologic effects was nearly 1%. Comparative effectiveness of medical marijuana vs other therapies is unknown for these indications.

  2. Charcot-Marie-Tooth disease

    Science.gov (United States)

    Sivera, Rafael; Vílchez, Juan Jesús; Martínez-Rubio, Dolores; Chumillas, María José; Vázquez, Juan Francisco; Muelas, Nuria; Bataller, Luis; Millán, José María; Palau, Fancesc; Espinós, Carmen

    2013-01-01

    Objectives: To determine the genetic distribution and the phenotypic correlation of an extensive series of patients with Charcot-Marie-Tooth disease in a geographically well-defined Mediterranean area. Methods: A thorough genetic screening, including most of the known genes involved in this disease, was performed and analyzed in this longitudinal descriptive study. Clinical data were analyzed and compared among the genetic subgroups. Results: Molecular diagnosis was accomplished in 365 of 438 patients (83.3%), with a higher success rate in demyelinating forms of the disease. The CMT1A duplication (PMP22 gene) was the most frequent genetic diagnosis (50.4%), followed by mutations in the GJB1 gene (15.3%), and in the GDAP1 gene (11.5%). Mutations in 13 other genes were identified, but were much less frequent. Sixteen novel mutations were detected and characterized phenotypically. Conclusions: The relatively high frequency of GDAP1 mutations, coupled with the scarceness of MFN2 mutations (1.1%) and the high proportion of recessive inheritance (11.6%) in this series exemplify the particularity of the genetic distribution of Charcot-Marie-Tooth disease in this region. PMID:24078732

  3. Auditory processing in patients with Charcot-Marie-Tooth disease type 1A.

    NARCIS (Netherlands)

    Neijenhuis, C.A.M.; Beynon, A.J.; Snik, A.F.M.; Engelen, B.G.M. van; Broek, P. van den

    2003-01-01

    HYPOTHESIS: It is unclear whether Charcot-Marie-Tooth (CMT) disease, type 1A, causes auditory processing disorders. Therefore, auditory processing abilities were investigated in five CMT1A patients with normal hearing. BACKGROUND: Previous studies have failed to separate peripheral from central

  4. Plasma Signature of Neurological Disease in the Monogenetic Disorder Niemann-Pick Type C*

    Science.gov (United States)

    Alam, Md. Suhail; Getz, Michelle; Yi, Sue; Kurkewich, Jeffrey; Safeukui, Innocent; Haldar, Kasturi

    2014-01-01

    Early diagnosis of neurological disorders would greatly improve their management and treatment. A major hurdle is that inflammatory products of cerebral disease are not easily detected in blood. Inflammation in multiple organs and heterogeneity in disease present additional challenges in distinguishing the extent to which a blood-based marker reflects disease in brain or other afflicted organs. Murine models of the monogenetic disorder Niemann-Pick Type C present aggressive forms of cerebral and liver inflammatory disease. Microarray analyses previously revealed age-dependent changes in innate immunity transcripts in the mouse brain. We have now validated four putative secretory inflammatory markers that are also elevated in mouse liver. We include limited, first time analysis of human Niemann-Pick Type C liver and cerebellum. Furthermore, we utilized 2-hydroxypropyl-β-cyclodextrin (HPβCD, an emerging therapeutic) administered intraperitoneally in mice, which abrogates inflammatory pathology in the liver but has limited effect on the brain. By analyzing the corresponding effects on inflammatory plasma proteins, we identified cathepsin S as a lead indicator of liver disease. In contrast, lysozyme was a marker of both brain and liver disease. 2-Hydroxypropyl-β-cyclodextrin had no effect on transcripts of neuron-specific 24-hydroxylase, and its product 24(S)-hydroxycholesterol was not a useful indicator in mouse plasma. Our data suggest that dual analysis of levels of the inflammatory markers lysozyme and cathepsin S may enable detection of multiple distinct states of neurodegeneration in plasma. PMID:24488491

  5. [Meta-analytic assessment of parenteral cytoflavin effectiveness in different neurologic disorders].

    Science.gov (United States)

    Mazin, P V; Sheshunov, I V; Mazina, N K

    To carry out an integral quantitative assessment of the clinical efficacy of parenteral use of cytoflavin in treatment of patients with CNS disorders based on the systemic selection of published controlled clinical trials and their meta-analysis. Twenty-one high evidence-based studies on the efficacy of parenteral use of cytoflavin in different CNS disorders (4314 patients) have been analyzed. Comparisons with basic treatment groups were undertaken to clarify the drug clinical effects adjusted for heterogeneity and variability of response parameters. An analysis of formalized efficacy indicators (increase in the absolute and relative value, odds ratio (OR) etc.) has demonstrated the advantages of cytoflavin. Group combination has increased the statistical power of the meta-analysis. Two models with fixed (Mantel-Haenszel amendment) and random effects were used. All the estimation protocols in different clinical groups of patients with neurologic diseases provided similar results and confirmed the stability of calculated values irrespective of heterogeneity of data arrays. OR of positive outcomes in neuropathologies treated with cytoflavin was 3,02 with χ2 heterogeneity 427,3 and p = 0,000…, I2= 85,7% (82%; 88%).

  6. Stiff Person Syndrome: A Rare Neurological Disorder, Heterogeneous in Clinical Presentation and Not Easy to Treat

    Directory of Open Access Journals (Sweden)

    Susanne Buechner

    2015-01-01

    Full Text Available Background. Stiff person syndrome (SPS is a rare neurological disorder characterized by progressive rigidity of axial and limb muscles associated with painful spasms. SPS can be classified into classic SPS, paraneoplastic SPS, and SPS variants. Its underlying pathogenesis is probably autoimmune, as in most cases antibodies against glutamic acid decarboxylase (GAD are observed. Similarly, paraneoplastic SPS is usually linked to anti-amphiphysin antibodies. Treatment is based on drugs enhancing gamma-aminobutyric acid (GABA transmission and immunomodulatory agents. Case Series. Patient 1 is a 45-year-old male affected by the classic SPS, Patient 2 is a 73-year-old male affected by paraneoplastic SPS, and Patient 3 is a 68-year-old male affected by the stiff limb syndrome, a SPS variant where symptoms are confined to the limbs. Symptoms, diagnostic findings, and clinical course were extremely variable in the three patients, and treatment was often unsatisfactory and not well tolerated, thus reducing patient compliance. Clinical manifestations also included some unusual features such as recurrent vomiting and progressive dysarthria. Conclusions. SPS is a rare disorder that causes significant disability. Because of its extensive clinical variability, a multitask and personalized treatment is indicated. A clearer understanding of uncommon clinical features and better-tolerated therapeutic strategies are still needed.

  7. Next generation sequencing for molecular diagnosis of neurological disorders using ataxias as a model.

    Science.gov (United States)

    Németh, Andrea H; Kwasniewska, Alexandra C; Lise, Stefano; Parolin Schnekenberg, Ricardo; Becker, Esther B E; Bera, Katarzyna D; Shanks, Morag E; Gregory, Lorna; Buck, David; Zameel Cader, M; Talbot, Kevin; de Silva, Rajith; Fletcher, Nicholas; Hastings, Rob; Jayawant, Sandeep; Morrison, Patrick J; Worth, Paul; Taylor, Malcolm; Tolmie, John; O'Regan, Mary; Valentine, Ruth; Packham, Emily; Evans, Julie; Seller, Anneke; Ragoussis, Jiannis

    2013-10-01

    Many neurological conditions are caused by immensely heterogeneous gene mutations. The diagnostic process is often long and complex with most patients undergoing multiple invasive and costly investigations without ever reaching a conclusive molecular diagnosis. The advent of massively parallel, next-generation sequencing promises to revolutionize genetic testing and shorten the 'diagnostic odyssey' for many of these patients. We performed a pilot study using heterogeneous ataxias as a model neurogenetic disorder to assess the introduction of next-generation sequencing into clinical practice. We captured 58 known human ataxia genes followed by Illumina Next-Generation Sequencing in 50 highly heterogeneous patients with ataxia who had been extensively investigated and were refractory to diagnosis. All cases had been tested for spinocerebellar ataxia 1-3, 6, 7 and Friedrich's ataxia and had multiple other biochemical, genetic and invasive tests. In those cases where we identified the genetic mutation, we determined the time to diagnosis. Pathogenicity was assessed using a bioinformatics pipeline and novel variants were validated using functional experiments. The overall detection rate in our heterogeneous cohort was 18% and varied from 8.3% in those with an adult onset progressive disorder to 40% in those with a childhood or adolescent onset progressive disorder. The highest detection rate was in those with an adolescent onset and a family history (75%). The majority of cases with detectable mutations had a childhood onset but most are now adults, reflecting the long delay in diagnosis. The delays were primarily related to lack of easily available clinical testing, but other factors included the presence of atypical phenotypes and the use of indirect testing. In the cases where we made an eventual diagnosis, the delay was 3-35 years (mean 18.1 years). Alignment and coverage metrics indicated that the capture and sequencing was highly efficient and the consumable cost

  8. [Charcot-arthropathy in diabetes mellitus

    NARCIS (Netherlands)

    Schoonbeek, A.; Ottens, R.L.J.M.; Lutterman, J.A.

    2002-01-01

    Charcot's arthropathy is a relative uncommon complication of diabetic neuropathy. The aetiology remains poorly understood. According to the neurotraumatic theory, the foot, which has become insensitive through neuropathy, is subjected to extensive (micro)trauma through continuation of use.

  9. Differential diagnosis between dementia and psychiatric disorders: Diagnostic criteria and supplementary exams. Recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology.

    Science.gov (United States)

    Bottino, Cássio M C; de Pádua, Analuiza Camozzato; Smid, Jerusa; Areza-Fegyveres, Renata; Novaretti, Tânia; Bahia, Valeria S

    2011-01-01

    In 2005, the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology published recommendations for the diagnosis of Alzheimer's disease These recommendations were updated following a review of evidence retrieved from national and international studies held on PUBMED, SCIELO and LILACS medical databases. The main aims of this review article are as follows: 1) to present the evidence found on Brazilian (LILACS, SCIELO) and International (MEDLINE) databases from articles published up to May 2011, on the differential diagnosis of these psychiatric disorders and dementia, with special focus on Dementia due to Alzheimer's and vascular dementia, including a review of supplementary exams which may facilitate the diagnostic process; and2) to propose recommendations for use by clinicians and researchers involved in diagnosing patients with dementia. Differential diagnosis between dementia and other neuropsychiatric disorders should always include assessments for depression, delirium, and use of psychoactive substances, as well as investigate the use of benzodiazepines, anti-epileptics and pattern of alcohol consumption.

  10. Evidence-based guideline update: Plasmapheresis in neurologic disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology.

    Science.gov (United States)

    Cortese, I; Chaudhry, V; So, Y T; Cantor, F; Cornblath, D R; Rae-Grant, A

    2011-01-18

    To reassess the role of plasmapheresis in the treatment of neurologic disorders. We evaluated the available evidence based on a structured literature review for relevant articles from 1995 through September 2009. In addition, due to revision of the definitions of classification of evidence since the publication of the previous American Academy of Neurology assessment in 1996, the evidence cited in that manuscript was reviewed and reclassified. Plasmapheresis is established as effective and should be offered in severe acute inflammatory demyelinating polyneuropathy (AIDP)/Guillain-Barré syndrome (GBS) and in the short-term management of chronic inflammatory demyelinating polyneuropathy (Class I studies, Level A). Plasmapheresis is established as ineffective and should not be offered for chronic or secondary progressive multiple sclerosis (MS) (Class I studies, Level A). Plasmapheresis is probably effective and should be considered for mild AIDP/GBS, as second-line treatment of steroid-resistant exacerbations in relapsing forms of MS, and for neuropathy associated with immunoglobulin A or immunoglobulin G gammopathy, based on at least one Class I or 2 Class II studies (Level B). Plasmapheresis is probably not effective and should not be considered for neuropathy associated with immunoglobulin M gammopathy, based on one Class I study (Level B). Plasmapheresis is possibly effective and may be considered for acute fulminant demyelinating CNS disease (Level C). There is insufficient evidence to support or refute the use of plasmapheresis for myasthenia gravis, pediatric autoimmune neuropsychiatric disorders associated with streptococcus infection, and Sydenham chorea (Class III evidence, Level U).

  11. Differential diagnosis between dementia and psychiatric disorders: Diagnostic criteria and supplementary exams Recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology

    Directory of Open Access Journals (Sweden)

    Cássio M.C. Bottino

    Full Text Available Abstract In 2005, the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology published recommendations for the diagnosis of Alzheimer's disease These recommendations were updated following a review of evidence retrieved from national and international studies held on PUBMED, SCIELO and LILACS medical databases. The main aims of this review article are as follows: 1 to present the evidence found on Brazilian (LILACS, SCIELO and International (MEDLINE databases from articles published up to May 2011, on the differential diagnosis of these psychiatric disorders and dementia, with special focus on Dementia due to Alzheimer's and vascular dementia, including a review of supplementary exams which may facilitate the diagnostic process; and 2 to propose recommendations for use by clinicians and researchers involved in diagnosing patients with dementia. Differential diagnosis between dementia and other neuropsychiatric disorders should always include assessments for depression, delirium, and use of psychoactive substances, as well as investigate the use of benzodiazepines, anti-epileptics and pattern of alcohol consumption.

  12. Common data elements for clinical research in mitochondrial disease: a National Institute for Neurological Disorders and Stroke project

    NARCIS (Netherlands)

    Karaa, A.; Rahman, S.; Lombes, A.; Yu-Wai-Man, P.; Sheikh, M.K.; Alai-Hansen, S.; Cohen, B.H.; Dimmock, D.; Emrick, L.; Falk, M.J.; McCormack, S.; Mirsky, D.; Moore, T.; Parikh, S.; Shoffner, J.; Taivassalo, T.; Tarnopolsky, M.; Tein, I.; Odenkirchen, J.C.; Goldstein, A.; Koene, S.; Smeitink, J.A.M.; et al.,

    2017-01-01

    OBJECTIVES: The common data elements (CDE) project was developed by the National Institute of Neurological Disorders and Stroke (NINDS) to provide clinical researchers with tools to improve data quality and allow for harmonization of data collected in different research studies. CDEs have been

  13. An Overview of Multiple Sclerosis: Medical, Psychosocial, and Vocational Aspects of a Chronic and Unpredictable Neurological Disorder

    Science.gov (United States)

    Rumrill, Phillip D., Jr.; Roessler, Richard T.

    2015-01-01

    This article presents an overview of multiple sclerosis (MS), one of the most common neurological disorders in the western hemisphere. Medical and psychosocial aspects of the disease such as causes and risk factors, diagnosis, incidence and prevalence, symptoms, courses, and treatment are described. Existing research regarding the employment…

  14. Generation of induced pluripotent stem cell (iPSC) line from Charcot-Marie-Tooth disease patient with MPZ mutation (CMT1B).

    Science.gov (United States)

    Son, Daryeon; Kang, Phil Jun; Yun, Wonjin; You, Seungkwon

    2017-10-01

    Charcot-Marie-Tooth disease (CMT1B) is an inherited neurological disorder caused by mutation of the myelin protein zero (MPZ) gene. We generated an induced pluripotent stem cell (iPSC) line from an 81-year-old patient with CMT1B by electroporating of lymphoblastoid cell lines with episomal plasmids encoding OCT4, SOX2, KLF4, L-MYC, LIN28, and p53-targeting shRNA. The established iPSCs expressed various pluripotency markers, demonstrated the potential to differentiate into cells of the three germ layers in vitro, had a normal karyotype and retained the MPZ mutation. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

  15. Pierre Janet, Sigmund Freud and Charcot's psychological and psychiatric legacy.

    Science.gov (United States)

    Pérez-Rincón, Héctor

    2011-01-01

    A key moment in the history of psychiatry occurred during Charcot's time at La Salpêtriere. Though his studies on hysteria and hypnotism, the founder of neurology inspired the work of two of his alumni: a Viennese Nervenartz and a French philosopher interested in the dissociation of personality. Even though neither of them was originally an alienist, their respective work allowed the field of neurosis--then belonging to internal medicine--to pass to psychiatry. The parallel lives of these frères enemis, both of whom were treated differently by fame, developed inside a very complex cultural and scientific milieu. Therefore, it is necessary to consider them together with other physicians, some of whom are much less well-known nowadays, who one way or another carried Charcot's influence into psychiatry, psychology and psychotherapy. The fates of the Dioscuri have been reversed--the fame and success of Freudian psychoanalysis ran parallel to Janet's oblivion and his long 'purgatory', but now the 'renaissance' of his work coincides with the decline of psychoanalysis as a theoretical explanation for mental pathology. Copyright © 2011 S. Karger AG, Basel.

  16. Is shoulder arthroplasty an option for charcot arthropathy?

    National Research Council Canada - National Science Library

    Schoch, Bradley; Werthel, Jean-David; Sperling, John W; Cofield, Robert H; Sanchez-Sotelo, Joaquin

    2016-01-01

    Charcot arthropathy is a rare cause of debilitating joint destruction. Shoulder arthroplasty for Charcot arthropathy is challenging secondary to local bone and soft tissue loss, lack of protective sensation, and altered muscle control...

  17. Cingulo-insular structural alterations associated with psychogenic symptoms, childhood abuse and PTSD in functional neurological disorders.

    Science.gov (United States)

    Perez, David L; Matin, Nassim; Barsky, Arthur; Costumero-Ramos, Victor; Makaretz, Sara J; Young, Sigrid S; Sepulcre, Jorge; LaFrance, W Curt; Keshavan, Matcheri S; Dickerson, Bradford C

    2017-06-01

    Adverse early-life events are predisposing factors for functional neurological disorder (FND) and post-traumatic stress disorder (PTSD). Cingulo-insular regions are implicated in the biology of both conditions and are sites of stress-mediated neuroplasticity. We hypothesised that functional neurological symptoms and the magnitude of childhood abuse would be associated with overlapping anterior cingulate cortex (ACC) and insular volumetric reductions, and that FND and PTSD symptoms would map onto distinct cingulo-insular areas. This within-group voxel-based morphometry study probes volumetric associations with self-report measures of functional neurological symptoms, adverse life events and PTSD symptoms in 23 mixed-gender FND patients. Separate secondary analyses were also performed in the subset of 18 women with FND to account for gender-specific effects. Across the entire cohort, there were no statistically significant volumetric associations with self-report measures of functional neurological symptom severity or childhood abuse. In women with FND, however, parallel inverse associations were observed between left anterior insular volume and functional neurological symptoms as measured by the Patient Health Questionnaire-15 and the Screening for Somatoform Symptoms Conversion Disorder subscale. Similar inverse relationships were also appreciated between childhood abuse burden and left anterior insular volume. Across all subjects, PTSD symptom severity was inversely associated with dorsal ACC volume, and the magnitude of lifetime adverse events was inversely associated with left hippocampal volume. This study reveals distinct cingulo-insular alterations for FND and PTSD symptoms and may advance our understanding of FND. Potential biological convergence between stress-related neuroplasticity, functional neurological symptoms and reduced insular volume was identified. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017

  18. Decoding Crucial LncRNAs Implicated in Neurogenesis and Neurological Disorders.

    Science.gov (United States)

    Ayana, R; Singh, Shailja; Pati, Soumya

    2017-04-15

    Unraveling transcriptional heterogeneity and the labyrinthine nature of neurodevelopment can probe insights into neuropsychiatric disorders. It is noteworthy that adult neurogenesis is restricted to the subventricular and subgranular zones of the brain. Recent studies suggest long non-coding RNAs (lncRNAs) as an avant-garde class of regulators implicated in neurodevelopment. But, paucity exists in the knowledge regarding lncRNAs in neurogenesis and their associations with neurodevelopmental defects. To address this, we extensively reviewed the existing literature databases as well as performed relevant in-silico analysis. We utilized Allen Brain Atlas (ABA) differential search module and generated a catalogue of ∼30,000 transcripts specific to the neurogenic zones, including coding and non-coding transcripts. To explore the existing lncRNAs reported in neurogenesis, we performed extensive literature mining and identified 392 lncRNAs. These degenerate lncRNAs were mapped onto the ABA transcript list leading to detection of 20 lncRNAs specific to neurogenic zones (Dentate gyrus/Lateral ventricle), among which 10 showed associations to several neurodevelopmental disorders following in-silico mapping onto brain disease databases like Simons Foundation Autism Research Initiative, AutDB, and lncRNADisease. Notably, using ABA correlation module, we could establish lncRNA-to-mRNA coexpression networks for the above 10 candidate lncRNAs. Finally, pathway prediction revealed physical, biochemical, or regulatory interactions for nine lncRNAs. In addition, ABA differential search also revealed 54 novel significant lncRNAs from the null set (∼30,000). Conclusively, this review represents an updated catalogue of lncRNAs in neurogenesis and neurological diseases, and overviews the field of OMICs-based data analysis for understanding lncRNome-based regulation in neurodevelopment.

  19. Survey of Japanese pediatricians on vaccination of children with neurological disorders.

    Science.gov (United States)

    Tanabe, Takuya; Tagawa, Tetsuzo; Arai, Hiroshi; Imaishi, Hidenori; Uno, Risa; Tanaka, Junko; Nagai, Toshisaburou; Nishida, Masaru; Awaya, Yutaka; Maekawa, Kihei

    2011-10-01

    Primary care physicians in Japan are often unwilling to vaccinate children with neurological disorders. The aim of the present study was to determine the state of vaccination in children who are severely handicapped and/or have convulsive disorders, in order to increase the vaccination rate in this patient population. Six hundred and eighty pediatricians belonging to Osaka Shonika Ikai were asked to answer a questionnaire, and 359 doctors responded. Two hundred and thirty-four doctors consulted for febrile seizures (Fs), 190 for epilepsy and 145 for conditions affecting severely handicapped children, responded that they refused to vaccinate. The reasons for reluctance to vaccinate these children were short interval since the last seizure, including febrile (226 doctors) and epileptic (121 doctors) seizures. It was especially likely that a child with a past history of status epilepticus would be refused vaccination. Primary care doctors are very cautious about the indications for vaccination, especially the inoculation of live vaccines, because they often induce post-vaccination fever-associated convulsions. Intractable daily epileptic seizures was the most common reason for refusal to vaccinate severely handicapped children. Examples of inadequate decision-making as regards the indications for vaccination were: "need more than 6 months observation since last seizure whether Fs or epileptic", "need EEG examination for Fs", "contraindication because of low bodyweight and/or chronic wheezing in severely handicapped children". There is a need to provide correct information about the adverse effects of vaccination and for greater cooperation between primary care doctors and pediatric neurologists. © 2011 The Authors. Pediatrics International © 2011 Japan Pediatric Society.

  20. Neurologic abnormalities in mouse models of the lysosomal storage disorders mucolipidosis II and mucolipidosis III γ.

    Directory of Open Access Journals (Sweden)

    Rachel A Idol

    Full Text Available UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase is an α2β2γ2 hexameric enzyme that catalyzes the synthesis of the mannose 6-phosphate targeting signal on lysosomal hydrolases. Mutations in the α/β subunit precursor gene cause the severe lysosomal storage disorder mucolipidosis II (ML II or the more moderate mucolipidosis III alpha/beta (ML III α/β, while mutations in the γ subunit gene cause the mildest disorder, mucolipidosis III gamma (ML III γ. Here we report neurologic consequences of mouse models of ML II and ML III γ. The ML II mice have a total loss of acid hydrolase phosphorylation, which results in depletion of acid hydrolases in mesenchymal-derived cells. The ML III γ mice retain partial phosphorylation. However, in both cases, total brain extracts have normal or near normal activity of many acid hydrolases reflecting mannose 6-phosphate-independent lysosomal targeting pathways. While behavioral deficits occur in both models, the onset of these changes occurs sooner and the severity is greater in the ML II mice. The ML II mice undergo progressive neurodegeneration with neuronal loss, astrocytosis, microgliosis and Purkinje cell depletion which was evident at 4 months whereas ML III γ mice have only mild to moderate astrocytosis and microgliosis at 12 months. Both models accumulate the ganglioside GM2, but only ML II mice accumulate fucosylated glycans. We conclude that in spite of active mannose 6-phosphate-independent targeting pathways in the brain, there are cell types that require at least partial phosphorylation function to avoid lysosomal dysfunction and the associated neurodegeneration and behavioral impairments.

  1. Global gene expression profiling of healthy human brain and its application in studying neurological disorders.

    Science.gov (United States)

    Negi, Simarjeet K; Guda, Chittibabu

    2017-04-18

    Brain function is governed by precise regulation of gene expression across its anatomically distinct structures; however, the expression patterns of genes across hundreds of brain structures are not clearly understood. Here, we describe a gene expression model, which is representative of the healthy human brain transcriptome by using data from the Allen Brain Atlas. Our in-depth gene expression profiling revealed that 84% of genes are expressed in at least one of the 190 brain structures studied. Hierarchical clustering based on gene expression profiles delineated brain regions into structurally tiered spatial groups and we observed striking enrichment for region-specific processes. Further, weighted co-expression network analysis identified 19 robust modules of highly correlated genes enriched with functional associations for neurogenesis, dopamine signaling, immune regulation and behavior. Also, structural distribution maps of major neurotransmission systems in the brain were generated. Finally, we developed a supervised classification model, which achieved 84% and 81% accuracies for predicting autism- and Parkinson's-implicated genes, respectively, using our expression model as a baseline. This study represents the first use of global gene expression profiling from healthy human brain to develop a disease gene prediction model and this generic methodology can be applied to study any neurological disorder.

  2. Matrix metalloproteinase-3 in myasthenia gravis compared to other neurological disorders and healthy controls.

    Science.gov (United States)

    Luckman, Steven P; Gilhus, Nils Erik; Romi, Fredrik

    2011-01-01

    MMP-3 is capable of degrading a variety of proteins, including agrin, which plays a critical role in neuromuscular signaling by controlling acetylcholine receptor clustering. High MMP-3 levels in a proportion of myasthenia gravis (MG) patients have been reported. A pathogenic role of MMP-3 in other neurological disorders has been suggested but not proven. We have therefore examined the levels of MMP-3 in 124 MG patients and compared them to 59 multiple sclerosis (MS) patients, 74 epilepsy patients, 33 acute stroke patients, and 90 healthy controls. 15.3% of the patients in the MG group were MMP-3-positive (defined as higher than cutoff value 48 ng/mL) with very high mean MMP-3 concentration (79.9 ng/mL), whereas the proportion of MMP-3 positive patients in the MS (3.4%), epilepsy (6.7%), stroke (0%), and the control group (4.4%) was significantly lower. Mean MMP-3 concentration in the total MG group (25.5 ng/mL) was significantly higher than in the MS (16.6 ng/mL) and stroke (11.7 ng/mL) groups, but did not differ significantly from the epilepsy (19.4 ng/mL) and the control group (23.4 ng/mL). MMP-3 may have a specific pathogenic effect in MG in addition to being associated with autoimmune diseases in general.

  3. Matrix Metalloproteinase-3 in Myasthenia Gravis Compared to Other Neurological Disorders and Healthy Controls

    Directory of Open Access Journals (Sweden)

    Steven P. Luckman

    2011-01-01

    Full Text Available MMP-3 is capable of degrading a variety of proteins, including agrin, which plays a critical role in neuromuscular signaling by controlling acetylcholine receptor clustering. High MMP-3 levels in a proportion of myasthenia gravis (MG patients have been reported. A pathogenic role of MMP-3 in other neurological disorders has been suggested but not proven. We have therefore examined the levels of MMP-3 in 124 MG patients and compared them to 59 multiple sclerosis (MS patients, 74 epilepsy patients, 33 acute stroke patients, and 90 healthy controls. 15.3% of the patients in the MG group were MMP-3-positive (defined as higher than cutoff value 48 ng/mL with very high mean MMP-3 concentration (79.9 ng/mL, whereas the proportion of MMP-3 positive patients in the MS (3.4%, epilepsy (6.7%, stroke (0%, and the control group (4.4% was significantly lower. Mean MMP-3 concentration in the total MG group (25.5 ng/mL was significantly higher than in the MS (16.6 ng/mL and stroke (11.7 ng/mL groups, but did not differ significantly from the epilepsy (19.4 ng/mL and the control group (23.4 ng/mL. MMP-3 may have a specific pathogenic effect in MG in addition to being associated with autoimmune diseases in general.

  4. Detection of enteroviruses in cases of neurological disorders in the State of Pará, Brazil

    Directory of Open Access Journals (Sweden)

    GOMES Maria de Lourdes Contente

    2001-01-01

    Full Text Available Eighty-one cerebrospinal fluid (CSF samples mainly from cases of aseptic meningitis and motor deficiency syndrome were sent to the Virology Section of Evandro Chagas Institute, Belém Pará, in the period of January 1995 to January 1996 in order to isolate viruses. All samples were inoculated onto HEp-2 cell culture and newborn mice, with negative results. The probability of isolating viruses by these methods is reduced because of the low concentration of viral particles in these specimens. In order to obtain more information about the etiology of these cases, a group of 23 samples were selected to be tested by a more sensitive technique than the virus isolation - the reverse transcription polymerase chain reaction (RT-PCR. Specific primers directed to conserved regions in the enterovirus genome were used, considering that this group of viruses is frequently associated with these neurological disorder. The age of the patients ranged from 1 to 55 years and nearly all of them lived in Belém, State of Pará, North of Brazil. Of 15 samples analyzed by RT PCR nine (60% were positive; of these, 6 (66.6% had motor deficiency and 3 (33.3% developed aseptic meningitis. These results show that it is important to investigate enterovirus as cause of these syndromes.

  5. A novel predicted calcium-regulated kinase family implicated in neurological disorders.

    Directory of Open Access Journals (Sweden)

    Małgorzata Dudkiewicz

    Full Text Available The catalogues of protein kinases, the essential effectors of cellular signaling, have been charted in Metazoan genomes for a decade now. Yet, surprisingly, using bioinformatics tools, we predicted protein kinase structure for proteins coded by five related human genes and their Metazoan homologues, the FAM69 family. Analysis of three-dimensional structure models and conservation of the classic catalytic motifs of protein kinases present in four out of five human FAM69 proteins suggests they might have retained catalytic phosphotransferase activity. An EF-hand Ca(2+-binding domain in FAM69A and FAM69B proteins, inserted within the structure of the kinase domain, suggests they may function as Ca(2+-dependent kinases. The FAM69 genes, FAM69A, FAM69B, FAM69C, C3ORF58 (DIA1 and CXORF36 (DIA1R, are by large uncharacterised molecularly, yet linked to several neurological disorders in genetics studies. The C3ORF58 gene is found deleted in autism, and resides in the Golgi. Unusually high cysteine content and presence of signal peptides in some of the family members suggest that FAM69 proteins may be involved in phosphorylation of proteins in the secretory pathway and/or of extracellular proteins.

  6. Vocal acoustic analysis as a biometric indicator of information processing: implications for neurological and psychiatric disorders.

    Science.gov (United States)

    Cohen, Alex S; Dinzeo, Thomas J; Donovan, Neila J; Brown, Caitlin E; Morrison, Sean C

    2015-03-30

    Vocal expression reflects an integral component of communication that varies considerably within individuals across contexts and is disrupted in a range of neurological and psychiatric disorders. There is reason to suspect that variability in vocal expression reflects, in part, the availability of "on-line" resources (e.g., working memory, attention). Thus, understanding vocal expression is a potentially important biometric index of information processing, not only across but within individuals over time. A first step in this line of research involves establishing a link between vocal expression and information processing systems in healthy adults. The present study employed a dual attention experimental task where participants provided natural speech while simultaneously engaged in a baseline, medium or high nonverbal processing-load task. Objective, automated, and computerized analysis was employed to measure vocal expression in 226 adults. Increased processing load resulted in longer pauses, fewer utterances, greater silence overall and less variability in frequency and intensity levels. These results provide compelling evidence of a link between information processing resources and vocal expression, and provide important information for the development of an automated, inexpensive and uninvasive biometric measure of information processing. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  7. Diagnosis and Treatment of Neurological and Ischemic Disorders Employing Carbon Nanotube Technology

    National Research Council Canada - National Science Library

    Patrick P. Komane; Yahya E. Choonara; Lisa C. du Toit; Pradeep Kumar; Pierre P. D. Kondiah; Girish Modi; Viness Pillay

    2016-01-01

    .... Based on their outstanding physicochemical properties, carbon nanotubes have the potential to be employed as theranostic tools for neurological pathologies such as Alzheimer's disease and Parkinson's...

  8. Neuroprotective Effects of Exercise on Brain Edema and Neurological Movement Disorders Following the Cerebral Ischemia and Reperfusion in Rats.

    Science.gov (United States)

    Shamsaei, Nabi; Erfani, Soheila; Fereidoni, Masoud; Shahbazi, Ali

    2017-01-01

    Cerebral ischemia and reperfusion causes physiological and biochemical changes in the neuronal cells that will eventually lead to cell damage. Evidence indicates that exercise reduces the ischemia and reperfusion-induced brain damages in animal models of stroke. In the present study, the effect of exercise preconditioning on brain edema and neurological movement disorders following the cerebral ischemia and reperfusion in rats was investigated. Twenty-one adult male wistar rats (weighing 260-300 g) were randomly divided into three groups: sham operated, exercise plus ischemia, and ischemia group (7 rats per group). The rats in exercise group were trained to run on a treadmill 5 days a week for 4 weeks. Transient focal cerebral ischemia and reperfusion were induced by middle cerebral artery occlusion (MCAO) for 60 minutes, followed by reperfusion for 23 hours. After 24 hours ischemia, movement disorders were tested by a special neurological examination. Also, cerebral edema was assessed by determining the brain water content. The results showed that pre-ischemic exercise significantly reduced brain edema (Pexercise preconditioning decreased the neurological movement disorders caused by brain ischemia and reperfusion (Pexercise had neuroprotective effects against brain ischemia and reperfusion-induced edema and movement disorders. Thus, it could be considered as a useful strategy for prevention of ischemic injuries, especially in people at risk.

  9. The feasibility of home polysomnographic recordings prescribed for sleep-related neurological disorders: a prospective observational study.

    Science.gov (United States)

    Carpentier, N; Jonas, J; Schaff, J-L; Koessler, L; Maillard, L; Vespignani, H

    2014-09-01

    Home polysomnography is being increasingly developed for sleep studies, with various grades of quality. This study aimed to determine the feasibility of affordable, high quality home polysomnographic recordings prescribed for suspected sleep-related neurological disorders. We prospectively screened all patients referred to the specialist sleep disorders clinic in Nancy University Hospital between May 2011 and August 2011. Patients were eligible for inclusion if they required polysomnography for the diagnosis of a sleep-related neurological disorder. One-night, polysomnography was performed in each patient's home by a trained sleep technician. Financial cost was determined prior to inclusion. A recording was considered as satisfactory if all the following criteria were present: at least, one EEG channel with continuous signal allowing determination of sleep stages and wake during more than 66% of sleep time; at least, one usable respiratory channel (airflow or either band) during more than 66% of sleep time; and usable oximetry during more than 66% of sleep time. Forty-eight of the 139 screened patients were included. Among the 48 home polysomnography recordings, 35 (72.9%) were satisfactory. Thirteen (27.1%) tracings displayed an unsatisfactory loss of EEG data, including seven (14.6%) tracings with an unsatisfactory loss of respiratory data. Home polysomnography prescribed for suspected sleep-related neurological disorders is feasible, with affordable costs, whilst maintaining high quality recording. Further studies are needed to measure the real medico-economic impact of promoting outpatient domiciliary explorations for sleep-related neurological disorders. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  10. Phosphorylated neurofilament H (pNF-H) as a potential diagnostic marker for neurological disorders in horses.

    Science.gov (United States)

    Intan-Shameha, A R; Divers, Thomas J; Morrow, Jennifer K; Graves, Amy; Olsen, Emil; Johnson, Amy L; Mohammed, Hussni O

    2017-10-01

    The current study aimed at the investigating the potential use of phosphorylated neurofilament H (pNF-H) as a diagnostic biomarker for neurologic disorders in the horse. Paired serum and cerebrospinal fluid (CSF) samples (n=88) and serum only (n=30) were obtained from horses diagnosed with neurologic disorders and clinically healthy horses as control. The neurologic horses consisted of equine protozoal myeloencephalitis (EPM) (38 cases) and cervical vertebral malformation (CVM) (23 cases). Levels of pNF-H were determined using an ELISA. The correlation between CSF and serum concentrations of pNF-H was evaluated using Spearman's Rank test and the significance of the difference among the groups was assessed using a nonparametric test. Horses had higher pNF-H levels in the CSF than serum. Horses afflicted with EPM had significantly higher serum pNF-H levels in comparison to controls or CVM cases. The correlation between CSF and serum pNF-H levels was poor in both the whole study population and among subgroups of horses included in the study. There was significant association between the likelihood of EPM and the concentrations of pNF-H in either the serum or CSF. These data suggest that pNF-H could be detected in serum and CSF samples from neurologic and control horses. This study demonstrated that pNF-H levels in serum and CSF have the potential to provide objective information to help in the early diagnosis of horses afflicted with neurologic disorders. Published by Elsevier Ltd.

  11. Additional Virtual Reality Sitting Balance Training Using XBox Kinect™ in Patients with Neurological Disorders: A Pilot Study

    Directory of Open Access Journals (Sweden)

    Xina Henry Quadros

    2017-10-01

    Full Text Available Introduction: Sitting balance is a prerequisite to upper extremity function, standing and walking, which is affected in various neurological diseases. It is important to attain a good level of sitting balance before one can proceed to standing. In recent years, virtual reality game training has gained a widespread application. Aim: This pilot study aimed to examine the role of additional virtual reality sitting balance training using a commercial interactive virtual reality system- Xbox Kinect™ in patients with neurological disorders. Materials and Methods: Four patients with sitting balance impairments following neurological disorders received two weeks of virtual reality based therapy along with the conventional physiotherapy. Sitting balance was evaluated using FIST (Function In Sitting Test scores at baseline, one week and after two weeks of intervention. Results: All four patients showed clinically significant improvement in FIST score between the pre and post intervention. Percentage of improvement in FIST score was approximately 27% with a minimum change of 10 points in the FIST score Minimal Clinically Important Difference (MCID=6.5. Conclusion: Additional virtual reality training may improve sitting balance control in neurological patients with balance impairments. It can be used as an adjunct in routine neurorehabilitation.

  12. Charcot arthropathy presenting with primary bone resorption.

    Science.gov (United States)

    Jones, Christopher W; Agolley, David; Burns, Kharis; Gupta, Sanjeev; Horsley, Mark

    2012-09-01

    The clinical presentation of acute Charcot arthropathy in the diabetic population usually follows the Eichenholtz classification. We present three usual cases of Charcot arthropathy presenting with rapid primary bone resorption in the absence of subluxation, dislocation and/or fracture. A review of the literature was performed. To our knowledge Charcot arthropathy has not been previously described as primary bone resorption. Three cases encountered at our specialist multidisciplinary High Risk Foot Clinic (HRFC) presented with primary bony resorption without features of subluxation, dislocation and/or fracture. Aggressive primary bone resorption was initially thought due to infection; a diagnostic dilemma that delayed optimal treatment. Late bone resorption in typical Charcot is linked to unregulated proinflammatory cytokines (IL-1β, IL-6 and TNFα) that lead to increased osteoclastic activity. The pathophysiology of osteolysis in aggressive primary bony resorption may relate to a disturbance in the balance between RANK-L and OPG. Primary resorption of bone without subluxation, dislocation and/or fracture can represent an active Charcot process. Prudent use of serial radiography and early MRI to look for the widespread bone and soft tissue oedema is recommended. Copyright © 2012 Elsevier Ltd. All rights reserved.

  13. Bilateral Charcot arthropathy of shoulder secondary to syringomyelia: An unusual case report

    Directory of Open Access Journals (Sweden)

    Ashok Panagariya

    2012-01-01

    Full Text Available Neuropathic arthropathy of the shoulder is a relatively rare disorder characterized by destruction of joint secondary to loss of sensory innervation. Bilateral Charcot arthropathy is an even rarer disorder, with very few cases reported in the English literature. We herein present a case of bilateral shoulder arthropathy secondary to syringomyelia with classical clinical and radiological findings. Radiological finding on one side was of resorptive type and resorptive mixed with productive on the other side.

  14. Bilateral Charcot arthropathy of shoulder secondary to syringomyelia: An unusual case report.

    Science.gov (United States)

    Panagariya, Ashok; Sharma, Arun Kumar

    2012-07-01

    Neuropathic arthropathy of the shoulder is a relatively rare disorder characterized by destruction of joint secondary to loss of sensory innervation. Bilateral Charcot arthropathy is an even rarer disorder, with very few cases reported in the English literature. We herein present a case of bilateral shoulder arthropathy secondary to syringomyelia with classical clinical and radiological findings. Radiological finding on one side was of resorptive type and resorptive mixed with productive on the other side.

  15. Computerized Functional Reach Test to Measure Balance Stability in Elderly Patients With Neurological Disorders.

    Science.gov (United States)

    Scena, Silvio; Steindler, Roberto; Ceci, Moira; Zuccaro, Stefano Maria; Carmeli, Eli

    2016-10-01

    The ability to maintain static and dynamic balance is a prerequisite for safe walking and for obtaining functional mobility. For this reason, a reliable and valid means of screening for risk of falls is needed. The functional reach test (FRT) is used in many countries, yet it does not provide some kinematic parameters such as shoulder or pelvic girdles translation. The purpose was to analyze video records measuring of distance, velocity, time length, arm direction and girdles translation while doing FRT. A cross-sectional, descriptive study was conducted where the above variables were correlated to the mini-mental state examination (MMSE) for mental status and the Tinetti balance assessment test, which have been validated, in order to computerize the FRT (cFRT) for elderly patients with neurological disorders. Eighty patients were tested and 54 were eligible to serve as experimental group. The patients underwent the MMSE, the Tinetti test and the FRT. LAB view software was used to record the FRT performances and to process the videos. The control group consisted of 51 healthy subjects who had been previously tested. The experimental group was not able to perform the tests as well as the healthy control subjects. The video camera provided valuable kinematic results such as bending down while performing the forward reach test. Instead of manual measurement, we proposed to use a cheap with fair resolution web camera to accurately estimate the FRT. The kinematic parameters were correlated with Tinetti and MMSE scores. The performance values established in this study indicate that the cFRT is a reliable and valid assessment, which provides more accurate data than "manual" test about functional reach.

  16. Neuroprotective Effects of Exercise on Brain Edema and Neurological Movement Disorders Following the Cerebral Ischemia and Reperfusion in Rats

    OpenAIRE

    Shamsaei, Nabi; Erfani, Soheila; Fereidoni, Masoud; Shahbazi, Ali

    2017-01-01

    Introduction: Cerebral ischemia and reperfusion causes physiological and biochemical changes in the neuronal cells that will eventually lead to cell damage. Evidence indicates that exercise reduces the ischemia and reperfusion-induced brain damages in animal models of stroke. In the present study, the effect of exercise preconditioning on brain edema and neurological movement disorders following the cerebral ischemia and reperfusion in rats was investigated. Methods: Twenty-one adult male wis...

  17. An Evaluation of Educational Neurological Eye Movement Disorder Videos Posted on Internet Video Sharing Sites.

    Science.gov (United States)

    Hickman, Simon J

    2016-03-01

    Internet video sharing sites allow the free dissemination of educational material. This study investigated the quality and educational content of videos of eye movement disorders posted on such sites. Educational neurological eye movement videos were identified by entering the titles of the eye movement abnormality into the search boxes of the video sharing sites. Also, suggested links were followed from each video. The number of views, likes, and dislikes for each video were recorded. The videos were then rated for their picture and sound quality. Their educational value was assessed according to whether the video included a description of the eye movement abnormality, the anatomical location of the lesion (if appropriate), and the underlying diagnosis. Three hundred fifty-four of these videos were found on YouTube and Vimeo. There was a mean of 6,443 views per video (range, 1-195,957). One hundred nineteen (33.6%) had no form of commentary about the eye movement disorder shown apart from the title. Forty-seven (13.3%) contained errors in the title or in the text. Eighty (22.6%) had excellent educational value by describing the eye movement abnormality, the anatomical location of the lesion, and the underlying diagnosis. Of these, 30 also had good picture and sound quality. The videos with excellent educational value had a mean of 9.84 "likes" per video compared with 2.37 for those videos without a commentary (P educational value with good picture and sound quality had a mean of 10.23 "likes" per video (P = 0.004 vs videos with no commentary). There was no significant difference in the mean number of "dislikes" between those videos that had no commentary or which contained errors and those with excellent educational value. There are a large number of eye movement videos freely available on these sites; however, due to the lack of peer review, a significant number have poor educational value due to having no commentary or containing errors. The number of "likes

  18. Beyond neural cubism: promoting a multidimensional view of brain disorders by enhancing the integration of neurology and psychiatry in education.

    Science.gov (United States)

    Taylor, Joseph J; Williams, Nolan R; George, Mark S

    2015-05-01

    Cubism was an influential early-20th-century art movement characterized by angular, disjointed imagery. The two-dimensional appearance of Cubist figures and objects is created through juxtaposition of angles. The authors posit that the constrained perspectives found in Cubism may also be found in the clinical classification of brain disorders. Neurological disorders are often separated from psychiatric disorders as if they stemmed from different organ systems. Maintaining two isolated clinical disciplines fractionalizes the brain in the same way that Pablo Picasso fractionalized figures and objects in his Cubist art. This Neural Cubism perpetuates a clinical divide that does not reflect the scope and depth of neuroscience. All brain disorders are complex and multidimensional, with aberrant circuitry and resultant psychopharmacology manifesting as altered behavior, affect, mood, or cognition. Trainees should receive a multidimensional education based on modern neuroscience, not a partial education based on clinical precedent. The authors briefly outline the rationale for increasing the integration of neurology and psychiatry and discuss a nested model with which clinical neuroscientists (neurologists and psychiatrists) can approach and treat brain disorders.

  19. Beyond Neural Cubism: Promoting a Multidimensional View of Brain Disorders by Enhancing the Integration of Neurology and Psychiatry in Education

    Science.gov (United States)

    Taylor, Joseph J.; Williams, Nolan R.; George, Mark S.

    2014-01-01

    Cubism was an influential early 20th century art movement characterized by angular, disjointed imagery. The two-dimensional appearance of Cubist figures and objects is created through juxtaposition of angles. The authors posit that the constrained perspectives found in Cubism may also be found in the clinical classification of brain disorders. Neurological disorders are often separated from psychiatric disorders as if they stem from different organ systems. Maintaining two isolated clinical disciplines fractionalizes the brain in the same way that Pablo Picasso fractionalized figures and objects in his Cubist art. This Neural Cubism perpetuates a clinical divide that does not reflect the scope and depth of neuroscience. All brain disorders are complex and multidimensional, with aberrant circuitry and resultant psychopharmacology manifesting as altered behavior, affect, mood or cognition. Trainees should receive a multidimensional education based on modern neuroscience, not a partial education based on clinical precedent. The authors briefly outline the rationale for increasing the integration of neurology and psychiatry and discuss a nested model with which clinical neuroscientists (neurologists and psychiatrists) can approach and treat brain disorders. PMID:25340364

  20. Neurologic disorders, in-hospital deaths, and years of potential life lost in the USA, 1988-2011.

    Science.gov (United States)

    Rosenbaum, Benjamin P; Kelly, Michael L; Kshettry, Varun R; Weil, Robert J

    2014-11-01

    Premature mortality is a public health concern that can be quantified as years of potential life lost (YPLL). Studying premature mortality can help guide hospital initiatives and resource allocation. We investigated the categories of neurologic and neurosurgical conditions associated with in-hospital deaths that account for the highest YPLL and their trends over time. Using the Nationwide Inpatient Sample (NIS), we calculated YPLL for patients hospitalized in the USA from 1988 to 2011. Hospitalizations were categorized by related neurologic principal diagnoses. An estimated 2,355,673 in-hospital deaths accounted for an estimated 25,598,566 YPLL. The traumatic brain injury (TBI) category accounted for the highest annual mean YPLL at 361,748 (33.9% of total neurologic YPLL). Intracerebral hemorrhage, cerebral ischemia, subarachnoid hemorrhage, and anoxic brain damage completed the group of five diagnoses with the highest YPLL. TBI accounted for 12.1% of all inflation adjusted neurologic hospital charges and 22.4% of inflation adjusted charges among neurologic deaths. The in-hospital mortality rate has been stable or decreasing for all of these diagnoses except TBI, which rose from 5.1% in 1988 to 7.8% in 2011. Using YPLL, we provide a framework to compare the burden of premature in-hospital mortality on patients with neurologic disorders, which may prove useful for informing decisions related to allocation of health resources or research funding. Considering premature mortality alone, increased efforts should be focused on TBI, particularly in and related to the hospital setting. Copyright © 2014 Elsevier Ltd. All rights reserved.

  1. Surgical treatment of the Charcot foot.

    Science.gov (United States)

    Pinzur, Michael S

    2016-01-01

    With the increased number of diabetics worldwide and the increased incidence of morbid obesity in more prosperous cultures, there has become an increased awareness of Charcot arthropathy of the foot and ankle. Outcome studies would suggest that patients with deformity associated with Charcot Foot arthropathy have impaired health related quality of life. This awareness has led reconstructive-minded foot and ankle surgeons to develop surgical strategies to treat these acquired deformities. This article outlines the current clinical approach to this disabling medical condition. Copyright © 2016 John Wiley & Sons, Ltd.

  2. Jean-Baptiste Charcot and Brazil

    Directory of Open Access Journals (Sweden)

    Hélio Afonso Ghizoni Teive

    2014-06-01

    Full Text Available Jean-Baptiste Charcot, a neurologist from the famous Salpêtrière school and a renowned maritime explorer, visited Brazil twice. The first visit was in 1903, when the first French Antarctic expedition, traveling aboard the ship Français, made a very short stopover in Recife, in the state of Pernambuco. The second took place in 1908, during the famous voyage of the Pourquoi Pas? to the Antarctic, when Charcot and his crew stayed in the city of Rio de Janeiro for eight days.

  3. Diagnosis of Attention-Deficit/Hyperactivity Disorder and Its Behavioral, Neurological, and Genetic Roots

    Science.gov (United States)

    Mueller, Kathryn L.; Tomblin, J. Bruce

    2012-01-01

    Attention-deficit/hyperactivity disorder (ADHD) is a common developmental disorder often associated with other developmental disorders including speech, language, and reading disorders. Here, we review the principal features of ADHD and current diagnostic standards for the disorder. We outline the ADHD subtypes, which are based upon the dimensions…

  4. Efficacy of modafinil on fatigue and excessive daytime sleepiness associated with neurological disorders: a systematic review and meta-analysis.

    Directory of Open Access Journals (Sweden)

    Ping Sheng

    Full Text Available BACKGROUND: Modafinil is a novel wake-promoting agent approved by the FDA ameliorating excessive daytime sleepiness (EDS in three disorders: narcolepsy, shift work sleep disorder and obstructive sleep apnea. Existing trials of modafinil for fatigue and EDS associated with neurological disorders provided inconsistent results. This meta-analysis was aimed to assess drug safety and effects of modafinil on fatigue and EDS associated with neurological disorders. METHODS: A comprehensive literature review was conducted in order to identify published studies assessing the effects of modafinil on fatigue and EDS associated with neurological disorders. Primary outcomes included fatigue and EDS. Secondary outcomes included depression and adverse effects. FINDINGS: Ten randomized controlled trials were identified including 4 studies of Parkinson's disease (PD, 3 of multiple sclerosis (MS, 2 of traumatic brain injury (TBI and 1 of post-polio syndrome (PPS. A total of 535 patients were enrolled. Our results suggested a therapeutic effect of modafinil on fatigue in TBI (MD -0.82 95% CI -1.54 - -0.11 p=0.02, I(2=0%, while a beneficial effect of modafinil on fatigue was not confirmed in the pooled studies of PD or MS. Treatment results demonstrated a clear beneficial effect of modafinil on EDS in patients with PD (MD -2.45 95% CI -4.00 - -0.91 p=0.002 I(2=14%, but not with MS and TBI. No difference was seen between modafinil and placebo treatments in patients with PPS. Modafinil seemed to have no therapeutic effect on depression. Adverse events were similar between modafinil and placebo groups except that more patients were found with insomnia and nausea in modafinil group. CONCLUSIONS: Existing trials of modafinil for fatigue and EDS associated with PD, MS, TBI and PPS provided inconsistent results. The majority of the studies had small sample sizes. Modafinil is not yet sufficient to be recommended for these medical conditions until solid data are available.

  5. Efficacy of modafinil on fatigue and excessive daytime sleepiness associated with neurological disorders: a systematic review and meta-analysis.

    Science.gov (United States)

    Sheng, Ping; Hou, Lijun; Wang, Xiang; Wang, Xiaowen; Huang, Chengguang; Yu, Mingkun; Han, Xi; Dong, Yan

    2013-01-01

    Modafinil is a novel wake-promoting agent approved by the FDA ameliorating excessive daytime sleepiness (EDS) in three disorders: narcolepsy, shift work sleep disorder and obstructive sleep apnea. Existing trials of modafinil for fatigue and EDS associated with neurological disorders provided inconsistent results. This meta-analysis was aimed to assess drug safety and effects of modafinil on fatigue and EDS associated with neurological disorders. A comprehensive literature review was conducted in order to identify published studies assessing the effects of modafinil on fatigue and EDS associated with neurological disorders. Primary outcomes included fatigue and EDS. Secondary outcomes included depression and adverse effects. Ten randomized controlled trials were identified including 4 studies of Parkinson's disease (PD), 3 of multiple sclerosis (MS), 2 of traumatic brain injury (TBI) and 1 of post-polio syndrome (PPS). A total of 535 patients were enrolled. Our results suggested a therapeutic effect of modafinil on fatigue in TBI (MD -0.82 95% CI -1.54 - -0.11 p=0.02, I(2)=0%), while a beneficial effect of modafinil on fatigue was not confirmed in the pooled studies of PD or MS. Treatment results demonstrated a clear beneficial effect of modafinil on EDS in patients with PD (MD -2.45 95% CI -4.00 - -0.91 p=0.002 I(2)=14%), but not with MS and TBI. No difference was seen between modafinil and placebo treatments in patients with PPS. Modafinil seemed to have no therapeutic effect on depression. Adverse events were similar between modafinil and placebo groups except that more patients were found with insomnia and nausea in modafinil group. Existing trials of modafinil for fatigue and EDS associated with PD, MS, TBI and PPS provided inconsistent results. The majority of the studies had small sample sizes. Modafinil is not yet sufficient to be recommended for these medical conditions until solid data are available.

  6. Inertial Sensors to Assess Gait Quality in Patients with Neurological Disorders: A Systematic Review of Technical and Analytical Challenges

    Directory of Open Access Journals (Sweden)

    Aliénor Vienne

    2017-05-01

    Full Text Available Gait disorders are major causes of falls in patients with neurological diseases. Understanding these disorders allows prevention and better insights into underlying diseases. InertiaLocoGraphy (ILG –the quantification of gait by using inertial measurement units (IMUs –shows great potential to address this public health challenge, but protocols vary widely and normative values of gait parameters are still unavailable. This systematic review critically compares ILG protocols, questions features extracted from inertial signals and proposes a semeiological analysis of clinimetric characteristics for use in neurological clinical routine. For this systematic review, PubMed, Cochrane and EMBASE were searched for articles assessing gait quality by using IMUs that were published from January 1, 2014 to August 31, 2016. ILG was used to assess gait in a wide range of neurological disorders – including Parkinson disease, mild cognitive impairment, Alzheimer disease, cerebral palsy, and cerebellar atrophy – as well as in the faller or frail older population and in people presenting rheumatological pathologies. However, results have not yet been driving changes in clinical practice. One reason could be that studies mainly aimed at comparing pathological gait to healthy gait, but there is stronger need for semiological descriptions of gait perturbation, severity or prognostic assessment. Furthermore, protocols used to assess gait using IMUs are too many. Likely, outcomes are highly heterogeneous and difficult to compare across large panels of studies. Therefore, homogenization is needed to foster the use of ILG to assess gait quality in neurological routine practice. The pros and cons of each protocol are emphasized so that a compromise can be reached. As well, analysis of seven complementary clinical criteria (springiness, sturdiness, smoothness, steadiness, stability, symmetry, synchronization is advocated.

  7. Common gene-network signature of different neurological disorders and their potential implications to neuroAIDS.

    Directory of Open Access Journals (Sweden)

    Vidya Sagar

    Full Text Available The neurological complications of AIDS (neuroAIDS during the infection of human immunodeficiency virus (HIV are symptomized by non-specific, multifaceted neurological conditions and therefore, defining a specific diagnosis/treatment mechanism(s for this neuro-complexity at the molecular level remains elusive. Using an in silico based integrated gene network analysis we discovered that HIV infection shares convergent gene networks with each of twelve neurological disorders selected in this study. Importantly, a common gene network was identified among HIV infection, Alzheimer's disease, Parkinson's disease, multiple sclerosis, and age macular degeneration. An mRNA microarray analysis in HIV-infected monocytes showed significant changes in the expression of several genes of this in silico derived common pathway which suggests the possible physiological relevance of this gene-circuit in driving neuroAIDS condition. Further, this unique gene network was compared with another in silico derived novel, convergent gene network which is shared by seven major neurological disorders (Alzheimer's disease, Parkinson's disease, Multiple Sclerosis, Age Macular Degeneration, Amyotrophic Lateral Sclerosis, Vascular Dementia, and Restless Leg Syndrome. These networks differed in their gene circuits; however, in large, they involved innate immunity signaling pathways, which suggests commonalities in the immunological basis of different neuropathogenesis. The common gene circuits reported here can provide a prospective platform to understand how gene-circuits belonging to other neuro-disorders may be convoluted during real-time neuroAIDS condition and it may elucidate the underlying-and so far unknown-genetic overlap between HIV infection and neuroAIDS risk. Also, it may lead to a new paradigm in understanding disease progression, identifying biomarkers, and developing therapies.

  8. Inertial Sensors to Assess Gait Quality in Patients with Neurological Disorders: A Systematic Review of Technical and Analytical Challenges.

    Science.gov (United States)

    Vienne, Aliénor; Barrois, Rémi P; Buffat, Stéphane; Ricard, Damien; Vidal, Pierre-Paul

    2017-01-01

    Gait disorders are major causes of falls in patients with neurological diseases. Understanding these disorders allows prevention and better insights into underlying diseases. InertiaLocoGraphy (ILG) -the quantification of gait by using inertial measurement units (IMUs) -shows great potential to address this public health challenge, but protocols vary widely and normative values of gait parameters are still unavailable. This systematic review critically compares ILG protocols, questions features extracted from inertial signals and proposes a semeiological analysis of clinimetric characteristics for use in neurological clinical routine. For this systematic review, PubMed, Cochrane and EMBASE were searched for articles assessing gait quality by using IMUs that were published from January 1, 2014 to August 31, 2016. ILG was used to assess gait in a wide range of neurological disorders - including Parkinson disease, mild cognitive impairment, Alzheimer disease, cerebral palsy, and cerebellar atrophy - as well as in the faller or frail older population and in people presenting rheumatological pathologies. However, results have not yet been driving changes in clinical practice. One reason could be that studies mainly aimed at comparing pathological gait to healthy gait, but there is stronger need for semiological descriptions of gait perturbation, severity or prognostic assessment. Furthermore, protocols used to assess gait using IMUs are too many. Likely, outcomes are highly heterogeneous and difficult to compare across large panels of studies. Therefore, homogenization is needed to foster the use of ILG to assess gait quality in neurological routine practice. The pros and cons of each protocol are emphasized so that a compromise can be reached. As well, analysis of seven complementary clinical criteria (springiness, sturdiness, smoothness, steadiness, stability, symmetry, synchronization) is advocated.

  9. Diagnosis and Treatment of Neurological Disorders by Millimeter-Wave Stimulation

    Science.gov (United States)

    Siegel, Peter H.; Pikov, Victor

    2011-01-01

    Increasingly, millimeter waves are being employed for telecomm, radar, and imaging applications. To date in the U.S, however, very few investigations on the impact of this radiation on biological systems at the cellular level have been undertaken. In the beginning, to examine the impact of millimeter waves on cellular processes, researchers discovered that cell membrane depolarization may be triggered by low levels of integrated power at these high frequencies. Such a situation could be used to advantage in the direct stimulation of neuronal cells for applications in neuroprosthetics and diagnosing or treating neurological disorders. An experimental system was set up to directly monitor cell response on exposure to continuous-wave, fixed-frequency, millimeter-wave radiation at low and modest power levels (0.1 to 100 safe exposure standards) between 50 and 100 GHz. Two immortalized cell lines derived from lung and neuronal tissue were transfected with green fluorescent protein (GFP) that locates on the inside of the cell membrane lipid bi-layer. Oxonol dye was added to the cell medium. When membrane depolarization occurs, the oxonal bound to the outer wall of the lipid bi-layer can penetrate close to the inner wall where the GFP resides. Under fluorescent excitation (488 nm), the normally green GFP (520 nm) optical signal quenches and gives rise to a red output when the oxonol comes close enough to the GFP to excite a fluorescence resonance energy transfer (FRET) with an output at 620 nm. The presence of a strong FRET signature upon exposures of 30 seconds to 2 minutes at 5-10 milliwatts per square centimeter RF power at 50 GHz, followed by a return to the normal 520-nm GFP signal after a few minutes indicating repolarization of the membrane, indicates that low levels of RF energy may be able to trigger non-destructive membrane depolarization without direct cell contact. Such a mechanism could be used to stimulate neuronal cells in the cortex without the need for

  10. Anaesthesia and Charcot-Marie- Tooth Disease

    African Journals Online (AJOL)

    Adele

    family history of peripheral motor and sensory neuropathy that had been diagnosed as Charcot-Marie-Tooth (CMT) disease type 1. He had undergone genetic testing which showed a duplication of the peripheral myelin protein (PMP 22) gene consistent with CMT Type. 1A. The previous anaesthetic for right foot surgery was ...

  11. Global, regional, and national burden of neurological disorders during 1990-2015: a systematic analysis for the Global Burden of Disease Study 2015.

    Science.gov (United States)

    2017-11-01

    Comparable data on the global and country-specific burden of neurological disorders and their trends are crucial for health-care planning and resource allocation. The Global Burden of Diseases, Injuries, and Risk Factors (GBD) Study provides such information but does not routinely aggregate results that are of interest to clinicians specialising in neurological conditions. In this systematic analysis, we quantified the global disease burden due to neurological disorders in 2015 and its relationship with country development level. We estimated global and country-specific prevalence, mortality, disability-adjusted life-years (DALYs), years of life lost (YLLs), and years lived with disability (YLDs) for various neurological disorders that in the GBD classification have been previously spread across multiple disease groupings. The more inclusive grouping of neurological disorders included stroke, meningitis, encephalitis, tetanus, Alzheimer's disease and other dementias, Parkinson's disease, epilepsy, multiple sclerosis, motor neuron disease, migraine, tension-type headache, medication overuse headache, brain and nervous system cancers, and a residual category of other neurological disorders. We also analysed results based on the Socio-demographic Index (SDI), a compound measure of income per capita, education, and fertility, to identify patterns associated with development and how countries fare against expected outcomes relative to their level of development. Neurological disorders ranked as the leading cause group of DALYs in 2015 (250·7 [95% uncertainty interval (UI) 229·1 to 274·7] million, comprising 10·2% of global DALYs) and the second-leading cause group of deaths (9·4 [9·1 to 9·7] million], comprising 16·8% of global deaths). The most prevalent neurological disorders were tension-type headache (1505·9 [UI 1337·3 to 1681·6 million cases]), migraine (958·8 [872·1 to 1055·6] million), medication overuse headache (58·5 [50·8 to 67·4 million

  12. 76 FR 69747 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Science.gov (United States)

    2011-11-09

    ... and evaluate grant applications. Place: National Institutes of Health, Neuroscience Center, 6001.../NIH/DHHS/Neuroscience Center, 6001 Executive Blvd., Suite 3208, MSC 9529, Bethesda, MD 20892, (301... Special Emphasis Panel; NINDS Research Education Programs for Residents and Fellows in Neurology...

  13. 75 FR 3475 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Science.gov (United States)

    2010-01-21

    ... Emphasis Panel; Pediatric Neurology Training Review. Date: February 8-9, 2010. Time: 8 a.m. to 6 p.m. ] Agenda: To review and evaluate grant applications. Place: Embassy Suites Hotel, 1250 22nd Street... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND...

  14. Epigenetic regulation of memory by acetylation and methylation of chromatin: implications in neurological disorders, aging, and addiction.

    Science.gov (United States)

    Sen, Nilkantha

    2015-06-01

    Synaptic plasticity is one of the most fundamental properties of neurons that underlie the formation of the memory in brain. In recent years, epigenetic modification of both DNA and histones such as DNA methylation and histone acetylation and methylation emerges as a potential regulatory mechanism that governs the transcription of several genes responsible for memory formation and behavior. Furthermore, the recent identification of nitrosylation of proteins has shown to either activate or repress gene transcription by modulating histone methylation or acetylation status in mature neuron. Recent studies suggest that the use of major substrates of abuse, e.g., cocaine, induces alterations in molecular and cellular mechanisms of epigenetics that underlie long-term memories in the striatum and prefrontal cortex. Moreover, downregulation of genes due to alterations in epigenetics leads to cognitive deficiencies associated with neurological disorders such as Alzheimer's disease, Huntington's disease, psychiatric disorder such as Rett's syndrome and aging. In this review, I will discuss the evidence for several epigenetic mechanisms in the coordination of complex memory formation and storage. In addition, I will address the current literature highlighting the role of acetylation and methylation of chromatin in memory impairment associated with several neurological disorders, aging, and addiction.

  15. Charcot spinal arthropathy in patients with congenital insensitivity to pain: a report of two cases and review of the literature.

    Science.gov (United States)

    Staudt, Michael D; Bailey, Christopher S; Siddiqi, Fawaz

    2017-01-26

    Charcot spine, or neuropathic spinal arthropathy, involves the progressive destruction of the spinal joint due to the lack of normal protective sensations and proprioception. A rare cause of Charcot spine is congenital insensitivity to pain, which is an absent or abnormal response to painful stimuli. There are few case reports describing this condition, and long-term follow-up data are limited. The presentation and treatment of two patients with Charcot spine secondary to congenital insensitivity to pain are described. Both cases were characterized by lumbar involvement and were treated with circumferential decompression and an extended lumbo-pelvic fusion construct. The cases described here demonstrate stable neurological status at 1.5 and 5 years follow-up. Patient characteristics, pre- and post-operative imaging, operative approach, and outcomes are described. The literature regarding this rare condition is also reviewed, with an emphasis on operative management and outcomes. Surgical management is traditionally complicated by a high rate of hardware failure and adjacent segment degeneration. The current review highlights the importance of prompt and aggressive management following diagnosis of Charcot spine, as well as extended follow-up.

  16. Detection of meningococcal meningitis in cerebrospinal fluid of patients with neurological disorders in government hospitals of Karachi.

    Science.gov (United States)

    Taj, Aneela; Jamil, Nusrat

    2016-11-01

    To investigate the microbiological yield from the apparently transparent cerebrospinal fluid samples of in-patients with suspected neurological disorders. Samples of CSF were collected from Neurology and Neurosurgery Wards of the Jinnah Postgraduate Medical Centre and the Civil Hospital, Karachi, from December 2007 to March 2012, and comprised cerebrospinal fluid samples collected from neurologically compromised patients through lumbar puncture. The processing of the samples was done at the Department of Microbiology, University of Karachi. Moreover, 10ml of each sample was streaked separately on different culture media, i.e. Nutrient Agar, Blood Agar and Chocolate Agar, for the isolation of both aerobic and anaerobic bacteria. Of the 92 samples, bacterial meningitis was found in 21(22.8%), whereas 71(77.1%) samples did not yield any bacterial aetiology. Besides, 1(4.7%) sample revealed Gram-positive rods of L. monocytogenes while Gram-negative kidney-shaped N. meningitidis was found in 20(95.24%) samples. Present study highlighted that apparently clear CSF samples predominantly revealed meningococcal meningitis. It is important to note that extracellular bacterial growth in CSF is not always the primary goal of pathogenesis, therefore establishing a fact that turbidity of CSF is not the cardinal symptom for the diagnosis of bacterial meningitis.

  17. Bedside screening to detect oropharyngeal dysphagia in patients with neurological disorders: an updated systematic review.

    Science.gov (United States)

    Kertscher, Berit; Speyer, Renée; Palmieri, Maria; Plant, Chris

    2014-04-01

    Oropharyngeal dysphagia is a highly prevalent comorbidity in neurological patients and presents a serious health threat, which may le to outcomes of aspiration pneumonia ranging from hospitalization to death. Therefore, an early identification of risk followed by an accurate diagnosis of oropharyngeal dysphagia is fundamental. This systematic review provides an update of currently available bedside screenings to identify oropharyngeal dysphagia in neurological patients. An electronic search was carried out in the databases PubMed, Embase, CINAHL, and PsychInfo (formerly PsychLit), and all hits from 2008 up to December 2012 were included in the review. Only studies with sufficient methodological quality were considered, after which the psychometric characteristics of the screening tools were determined. Two relevant bedside screenings were identified, with a minimum sensitivity and specificity of ≥70 and ≥60 %, respectively.

  18. Mapping of brain function with positron emission tomography for pathophysiological analysis of neurological disorders

    Energy Technology Data Exchange (ETDEWEB)

    Nariai, Tadashi [Tokyo Medical and Dental Univ. (Japan). Graduate School

    2001-02-01

    The role of PET is discussed mainly through author's clinical experience in patients with brain lesions from the view of mapping of brain function. Procedure for PET concept in clinical practice is summarized. PET using tracers like [{sup 15}O]water and [{sup 18}F]fluorodeoxyglucose for mapping of the function has been used in combination with MRI, MEG (magnetoencephalography), SPECT and other imaging means for morphological identification. Actual those images before and after surgery are presented in cases of epilepsy, moyamoya disease, stegnosis of cervical artery, arteriovenous malformation and oligodendroglioma. Images of [{sup 11}C]flumazenil in epilepsies are also presented to show the neurological dysfunctions. PET evaluation of neurological functions is concluded to become more important in parallel with the advancement of therapeutics. (K.H.)

  19. Plasmapheresis in neurological disorders: six years experience from University Clinical center Tuzla

    OpenAIRE

    Osman Sinanović; Sanela Zukić; Adnan Burina; Nermina Pirić; Renata Hodžić; Mirza Atić; Mirna Alečković-Halilović; Enisa Mešić

    2017-01-01

    Background: Therapeutic plasma exchange (TPE) is an extracorporeal blood purification technique that is designed to remove substances with a large molecular weight. The TPE procedure includes removal of antibodies, alloantibodies, immune complexes, monoclonal protein, toxins or cytokines, and involves the replenishment of a specific plasma factor. The aim of the study was to describe the clinical response to TPE in various neurological patients, and to assess the clinical response to this the...

  20. Sexual functioning in women with mild and severe symptoms of Charcot-Marie-Tooth disease.

    Science.gov (United States)

    Gargiulo, Paola; Vinci, Paola; Navarro-Cremades, Felipe; Rellini, Alessandra H

    2013-07-01

    Charcot-Marie-Tooth (CMT) disease is one of the most frequently inherited neurological disorders, and while it is known that individuals suffering from this condition have low quality of life, little is known about their sexual function and satisfaction. To describe the functioning on different domains of sexuality in a relatively large sample of women with CMT, provide comparisons between mildly and severely affected patients and between women with the two different types of CMT (demyelinating vs. axonal), and assess the relationship between sexual function and age of onset. Fifty-seven women (age: 18-60 years) were approached in a CMT rehabilitation clinic by a psychologist and administered the Italian version of the McCoy Female Sexuality Questionnaire (MFSQ). Data from 40 patients who had had sexual intercourse in the previous 4 weeks were analyzed. The main outcome measures are the factors MFSQ-SEX and MFSQ-PARTNER, which describe sexual functioning and sexual satisfaction with a partner, respectively. Almost 30% of women did not engage in sexual intercourse with a partner. Overall sexual problems were more prominent in younger women and tended to be lower as age increased: this pattern was different from what was reported in previous studies in comparable samples of healthy Italian women. Severity of CMT was associated with better sexual functioning in the areas of desire, arousal, orgasm, and satisfaction, with women with more severe symptoms reporting greater functioning. Women with more severe CMT symptoms reported more pain during intercourse. Age of CMT onset and type of CMT (demyelinating vs. axonal) were not associated with differences in sexual functioning. Findings point to the importance of including assessment of sexual dysfunction in young women with mild CMT symptoms and the importance of providing sex therapy or counseling to these patients. © 2013 International Society for Sexual Medicine.

  1. Integral Characterization of Defective BDNF/TrkB Signalling in Neurological and Psychiatric Disorders Leads the Way to New Therapies

    Science.gov (United States)

    Tejeda, Gonzalo S.; Díaz-Guerra, Margarita

    2017-01-01

    Enhancement of brain-derived neurotrophic factor (BDNF) signalling has great potential in therapy for neurological and psychiatric disorders. This neurotrophin not only attenuates cell death but also promotes neuronal plasticity and function. However, an important challenge to this approach is the persistence of aberrant neurotrophic signalling due to a defective function of the BDNF high-affinity receptor, tropomyosin-related kinase B (TrkB), or downstream effectors. Such changes have been already described in several disorders, but their importance as pathological mechanisms has been frequently underestimated. This review highlights the relevance of an integrative characterization of aberrant BDNF/TrkB pathways for the rational design of therapies that by combining BDNF and TrkB targets could efficiently promote neurotrophic signalling. PMID:28134845

  2. Reducing the treatment gap for mental, neurological and substance use disorders in Africa: lessons from the Friendship Bench in Zimbabwe.

    Science.gov (United States)

    Chibanda, D

    2017-08-01

    Mental, neurological and substance use disorders (MNS) are a leading cause of disability in Africa. In response to the large treatment gap for MNS, a growing body of evidence-based treatments (EBTs) is emerging from Africa; however, there is a dearth of knowledge on how to scale up EBT. The Friendship Bench intervention is a brief psychological treatment delivered through the primary health care system in Zimbabwe by trained lay health workers. It has contributed significantly towards narrowing the treatment gap for common mental disorders in Zimbabwe where it has been scaled up to over 70 primary health care facilities. A three-pronged approach consisting of community engagement, use of EBTs and a government endorsed scale-up plan is described as part of the key strategy leading to the scale up of the Friendship Bench.

  3. Neurological soft signs, dissociation and alexithymia in patients with obsessive-compulsive disorder (OCD) and healthy subjects.

    Science.gov (United States)

    Tapancı, Zafer; Yıldırım, Abdullah; Boysan, Murat

    2017-11-21

    A body of evidence has supported that patients with obsessive-compulsive disorder (OCD) have increased rates of various neurological soft signs (NSS) compared to controls. Various lines of research has documented robust relationships between OCD and dissociative symptomatology. The study aimed to examine the associations between obsessive-compulsive symptoms, dissociative experiences alexithymia, and NSS. The study included thirty OCD patients and thirty healthy controls, matched for age, marital status, education, and income. The Neurological Evaluation Scale (NES), Padua Inventory-Revised (PI-R), Dissociative Experiences Scale (DES) and Toronto Alexithymia Scale (TAS-20) were administered. In comparison to healthy controls, patients with OCD had difficulty sequencing for complex motor acts and greater absorption/ imaginative involvement. Using latent class analysis, the study sample was classified into two homogenous subsets as mild NSS (n = 45) and severe NSS (n = 15). Majority of the participants who were grouped into severe NSS latent class were OCD patient (n = 14, 93.3%). Furthermore, those with severe NSS reported greater levels of alexithymia and more severe obsessive-compulsive symptoms, particularly precision. We concluded that relationships between OCD severity and NSS appear to be of crucial importance. Our data along with accumulated evidence suggest that OCD associated with pronounced NSS may represent a specific subtype of the disorder. Copyright © 2017 Elsevier B.V. All rights reserved.

  4. Occurrence of complications and survival rates in elderly with neurological disorders undergoing enteral nutrition therapy.

    Science.gov (United States)

    Martins, Aline Stangherlin; Rezende, Nilton Alves de; Torres, Henrique Oswaldo da Gama

    2012-01-01

    To evaluate the occurrence of complications, as well as the survival rates, in elderly people having neurological diseases and undergoing enteral nutrition therapy (ENT). Patients aged over 60 years, assisted by a home medical service from a healthcare plan in the city of Belo Horizonte, MG, Brazil, were thoroughly evaluated. The mentioned evaluation occurred at their homes after hospital discharge with enteral nutrition (EN) after a three-month period, a six-month period, and at the end of the study. A nutritional assessment was performed along with data collection performed on the patients' electronic medical records, and interviews performed with patients' family members and caregivers. Seventy-nine patients aged 82.9 ± 10.4 years old were evaluated; of these, 49.4% presented dementia, and 50.6% presented other neurological diagnoses. 100% of patients presented a high dependence level, assessed by the Katz index. The majority of patients (91.2%) presented some complications such as: pneumonia, catheter loss, diarrhea, constipation, vomiting, fluid leakage, periostotomy, tube obstruction, reflux, and myiasis. Pneumonia was the most frequent complication, occurring in 55.9% of cases. The mortality rates were 15.2% at a three-month period, 22.8% at a six-month period, and 43% at the end of study. The median survival after starting EN was 364 days. Differences among the mortality rate and neurological diagnosis, EN routes of access, and complications were not observed. The survival rate was lower in patients having inadequate nutritional status and albumin levels nutritional status according to the clinical assessment and albumin levels lower than 3.5 mg/dL significantly influenced the survival rates.

  5. The prefaces by Charcot: leitmotifs of an international career.

    Science.gov (United States)

    Goetz, Christopher G

    2003-04-22

    The objective of this article was to examine the prefaces that Jean-Martin Charcot wrote for other colleagues' books and to evaluate these short essays from three perspectives: scientific material discussed, medicopolitical strategies revealed, and larger thematic issues developed. Charcot (1825-1893) was the most celebrated clinical neurologist of his epoch. In his mid and late career, he wrote very few manuscripts, delegating such work to junior colleagues or to Bourneville, who compiled Charcot's Complete Works. One particular source of direct writing from Charcot's mature career is the group of prefaces he composed for selected monographs authored by other colleagues. The prefaces from the Gasser tabulation (Bibliothèque Charcot, Paris) of Charcot's works were examined and analyzed in the context of Charcot's career. Of the 21 prefaces, 10 concerned books by other colleagues with whom he had not worked, and 11 introduced books of his students or direct colleagues. The prefaces concerned primarily two topics of direct medical interest to Charcot: localization studies of cortical and spinal cord diseases, and the combined subject of hysteria and hypnotism. In placing the works in scientific context, Charcot systematically emphasized the contributions of French neurologists, largely focusing on the Salpêtrière school and its (his) discoveries. Charcot used these prefaces to summarize the major thematic elements of his career, drafting the essays in the context of such topics as medical specialization, the concept of experimental medicine, and the prioritization of human studies over experimental laboratory medicine. Although an exhaustive reading of Charcot's entire opus is needed to provide a comprehensive view of his ideas, the short and pithy prefaces, comprising fewer than 60 pages, coalesce and distill the primary themes of his international career.

  6. Charcot-Leyden crystals in pemphigus vegetans.

    Science.gov (United States)

    Kuo, T T; Wang, C N

    1986-06-01

    In the skin lesion of a patient with pemphigus vegetans, Charcot-Leyden crystals were observed in the eosinophilic abscess cavities of the epidermis. The finding is documented for the first time. In addition, some of the intraepidermal eosinophilic abscess cavities have been shown to be due to accumulation of eosinophils in dilated acrosyringia, but their formation from acantholysis and spongiosis can not be entirely excluded.

  7. Charcot arthropathy of the first metatarsophalangeal joint.

    Science.gov (United States)

    Wünschel, Markus; Wülker, Nikolaus; Gesicki, Marco

    2012-01-01

    Charcot arthropathy is an acute or subacute, often indolent, non-infectious or tumorous osteoarticular destruction of weightbearing skeletal structures in patients with reduced pain perception due to peripheral neuropathy. The authors present a rare case of progressive Charcot arthropathy of the first metatarsophalangeal joint with accompanying ulcer and foot deformity due to peripheral neuropathy. An arthrodesis of the first metatarsophalangeal joint with resection of the hypertrophic bone and osteophytes using a locking plate was performed. Also a condylectomy of the base of the proximal phalanx digitus II and III as well as a shortening osteotomy of the third metatarsal were conducted. The ulcer was debrided and primarily closed by suture. Mobilization was performed without weightbearing in a postoperative shoe for 6 weeks, the ulcer was completely healed and the arthrodesis had fused. Owing to the complexity of Charcot arthropathy careful preoperative evaluation, timing and dimension of surgery as well as treatment of associated comorbidities and sufficient postoperative care is important to reduce the complication rate and improve long-term results.

  8. Synaptic Interactome Mining Reveals p140Cap as a New Hub for PSD Proteins Involved in Psychiatric and Neurological Disorders

    Directory of Open Access Journals (Sweden)

    Annalisa Alfieri

    2017-06-01

    Full Text Available Altered synaptic function has been associated with neurological and psychiatric conditions including intellectual disability, schizophrenia and autism spectrum disorder (ASD. Amongst the recently discovered synaptic proteins is p140Cap, an adaptor that localizes at dendritic spines and regulates their maturation and physiology. We recently showed that p140Cap knockout mice have cognitive deficits, impaired long-term potentiation (LTP and long-term depression (LTD, and immature, filopodia-like dendritic spines. Only a few p140Cap interacting proteins have been identified in the brain and the molecular complexes and pathways underlying p140Cap synaptic function are largely unknown. Here, we isolated and characterized the p140Cap synaptic interactome by co-immunoprecipitation from crude mouse synaptosomes, followed by mass spectrometry-based proteomics. We identified 351 p140Cap interactors and found that they cluster to sub complexes mostly located in the postsynaptic density (PSD. p140Cap interactors converge on key synaptic processes, including transmission across chemical synapses, actin cytoskeleton remodeling and cell-cell junction organization. Gene co-expression data further support convergent functions: the p140Cap interactors are tightly co-expressed with each other and with p140Cap. Importantly, the p140Cap interactome and its co-expression network show strong enrichment in genes associated with schizophrenia, autism, bipolar disorder, intellectual disability and epilepsy, supporting synaptic dysfunction as a shared biological feature in brain diseases. Overall, our data provide novel insights into the molecular organization of the synapse and indicate that p140Cap acts as a hub for postsynaptic complexes relevant to psychiatric and neurological disorders.

  9. Assessment of speech in neurological disorders: development of a Swahili screening test

    National Research Council Canada - National Science Library

    Nick Miller; Gerry Mshana; Oliva Msuya; Catherine Dotchin; Richard Walker; Eric Aris

    2012-01-01

    .... We describe the rationale for, and development of a motor-speech-disorder screening test for Swahili speakers which includes impairment measures as well as measures of intelligibility and speech-voice naturalness...

  10. Charcot arthropathy risk elevation in the obese diabetic population.

    Science.gov (United States)

    Stuck, Rodney M; Sohn, Min-Woong; Budiman-Mak, Elly; Lee, Todd A; Weiss, Kevin B

    2008-11-01

    To examine the association of obesity, peripheral neuropathy, and other risk factors with the Charcot arthropathy incidence rate in a large diabetic population. The Department of Veterans Affairs inpatient and outpatient administrative datasets were used to identify persons with diabetes in 2003. Logistic regressions were used to model the likelihood of a person developing Charcot arthropathy as a function of individual characteristics, obesity, peripheral neuropathy, diabetic control, and comorbidities. Of Veterans Affairs users with diabetes, 652 (0.12%) were newly diagnosed with Charcot arthropathy in 2003. Compared with persons without obesity or peripheral neuropathy, those with obesity alone were approximately 59% more likely, those with neuropathy alone were 14 times more likely, and those with both obesity and neuropathy were 21 times more likely to develop Charcot arthropathy. Ages 55 to 64 years, diabetes duration 6 years or more, hemoglobin-A1c 7% or more, renal failure, arthritis, and deficiency anemia also were associated with an increased incidence of Charcot arthropathy. Obesity is significantly associated with an increased incidence of Charcot arthropathy independently of other risk factors. When obesity is combined with neuropathy, the Charcot arthropathy incidence rate increases multiplicatively. Prevention and detection of Charcot arthropathy should take the interaction between obesity and neuropathy into consideration.

  11. Significance of Charcot Leyden crystals in hepatic aspirates

    Directory of Open Access Journals (Sweden)

    Misra Vatsala

    2009-01-01

    Full Text Available Charcot Leyden crystals are hexagonal bipyramidal structures localised in the primary granules of the cytoplasm of eosinophils and basophils. Their presence, along with eosinophilic infiltrate, is an indirect evidence of parasitic infestation particularly with Toxocara, Capilliriasis, Ascariasis, or Fasciola. We report here two cases where Charcot Leyden crystals with eosinophilic infiltrate were found in the smears prepared from hepatic abscess.

  12. Significance of Charcot Leyden crystals in hepatic aspirates

    OpenAIRE

    Misra Vatsala; Debnath Sharmistha; Misra Sri; Singh Premala

    2009-01-01

    Charcot Leyden crystals are hexagonal bipyramidal structures localised in the primary granules of the cytoplasm of eosinophils and basophils. Their presence, along with eosinophilic infiltrate, is an indirect evidence of parasitic infestation particularly with Toxocara, Capilliriasis, Ascariasis, or Fasciola. We report here two cases where Charcot Leyden crystals with eosinophilic infiltrate were found in the smears prepared from hepatic abscess.

  13. Development of a Kinect-based exergaming system for motor rehabilitation in neurological disorders

    Science.gov (United States)

    Estepa, A.; Sponton Piriz, S.; Albornoz, E.; Martínez, C.

    2016-04-01

    The development of videogames for physical therapy, known as exergames, has gained much interest in the last years. In this work, a sytem for rehabilitation and clinical evaluation of neurological patients is presented. The Microsoft Kinect device is used to track the full body of patients, and three games were developed to exercise and assess different aspects of balance and gait rehabilitation. The system provides visual feedback by means of an avatar that follows the movements of the patients, and sound and visual stimuli for giving orders during the experience. Also, the system includes a database and management tools for further analysis and monitoring of therapies. The results obtained show, on the one side, a great reception and interest of patients to use the system. On the other side, the specialists considered very useful the data collected and the quantitative analysis provided by the system, which was then adopted for the clinical routine.

  14. Evolution of language assessment in patients with acquired neurological disorders in Brazil

    Directory of Open Access Journals (Sweden)

    Maria Alice de Mattos Pimenta Parente

    Full Text Available The objective of this paper was to describe the evolution of language assessments in patients with acquired neurological diseases over a period of around 45 years from 1970, when interdisciplinarity in Neuropsychology first began in Brazil, to the present day. The first twenty years of data was based on memories of Speech Pathology University Professors who were in charge of teaching aphasia. We then show the contributions of Linguistics, Cognitive Psychology, as well as Psycholinguistic and Psychometric criteria, to language evaluation. Finally, the current panorama of adaptations and creations of validated and standardized instruments is given, based on a search of the databases Pubmed, Scopus and Lilacs. Our closing remarks highlight the diversity in evaluation approaches and the recent tendency of language evaluations linked to new technologies such as brain imaging and computational analysis.

  15. Evolution of language assessment in patients with acquired neurological disorders in Brazil.

    Science.gov (United States)

    Parente, Maria Alice de Mattos Pimenta; Baradel, Roberta Roque; Fonseca, Rochele Paz; Pereira, Natalie; Carthery-Goulart, Maria Teresa

    2014-01-01

    The objective of this paper was to describe the evolution of language assessments in patients with acquired neurological diseases over a period of around 45 years from 1970, when interdisciplinarity in Neuropsychology first began in Brazil, to the present day. The first twenty years of data was based on memories of Speech Pathology University Professors who were in charge of teaching aphasia. We then show the contributions of Linguistics, Cognitive Psychology, as well as Psycholinguistic and Psychometric criteria, to language evaluation. Finally, the current panorama of adaptations and creations of validated and standardized instruments is given, based on a search of the databases Pubmed, Scopus and Lilacs. Our closing remarks highlight the diversity in evaluation approaches and the recent tendency of language evaluations linked to new technologies such as brain imaging and computational analysis.

  16. Neurological disorder associated with pestivirus infection in sheep in Rio Grande do Sul, Brazil

    Directory of Open Access Journals (Sweden)

    Pescador Caroline Argenta

    2004-01-01

    Full Text Available A two-month-old lamb showing signs of severe neurological disease characterized by muscular tremors, hypermetria, and motor incoordination was submitted to the Veterinary Pathology Laboratory - Universidade Federal do Rio Grande do Sul, Brazil. At necropsy, the major findings were a marked reduction of the size of the cerebellum and bilateral dilatation of the lateral ventricles. Microscopically, areas of cellular disorganization in the cerebellar cortex, reduction of the granular layer of cerebellum associated with decreased density of cells, and the presence of large cytoplasmic vacuoles in the molecular layer were observed. Neurons of the gray matter of the brain and macrophages of the mesenteric lymph nodes stained positively by the immunohistochemistry test using the monoclonal antibody 15C5 against Bovine Viral Diarrhea Virus. Taken together, those results are consistent with a pestivirus infection, either Border Disease Virus (BDV or BVDV.

  17. Guided self-help interventions for mental health disorders in children with neurological conditions: study protocol for a pilot randomised controlled trial.

    Science.gov (United States)

    Bennett, Sophie; Heyman, Isobel; Coughtrey, Anna; Simmonds, Jess; Varadkar, Sophia; Stephenson, Terence; DeJong, Margaret; Shafran, Roz

    2016-11-04

    Rates of mental health disorders are significantly greater in children with physical illnesses than in physically well children. Children with neurological conditions, such as epilepsy, are known to have particularly high rates of mental health disorders. Despite this, mental health problems in children with neurological conditions have remained under-recognised and under-treated in clinical settings. Evidence-based guided self-help interventions are efficacious in reducing symptoms of mental health disorders in children, but their efficacy in reducing symptoms of common mental health disorders in children with neurological conditions has not been investigated. We aim to pilot a guided self-help intervention for the treatment of mental health disorders in children with neurological conditions. A pilot randomised controlled trial with 18 patients with neurological conditions and mental health disorders will be conducted. Participants attending specialist neurology clinics at a National UK Children's Hospital will be randomised to receive guided self-help for common mental health disorders or to a 12-week waiting list control. Participants in the treatment group will receive 10 sessions of guided self-help delivered over the telephone. The waiting list control group will receive the intervention after a waiting period of 12 weeks. The primary outcome measure is reduction in symptoms of mental health disorders. Exclusion criteria are limited to those at significant risk of harm to self or others, the presence of primary mental health disorder other than anxiety, depression or disruptive behaviour (e.g. psychosis, eating disorder, obsessive-compulsive disorder) or intellectual disability at a level meaning potential participants would be unable to access the intervention. The study has ethical approval from the Camden and Islington NHS Research Ethics Committee, registration number 14.LO.1353. Results will be disseminated to patients, the wider public, clinicians and

  18. Function Over Form: Modeling Groups of Inherited Neurological Conditions in Zebrafish.

    Science.gov (United States)

    Kozol, Robert A; Abrams, Alexander J; James, David M; Buglo, Elena; Yan, Qing; Dallman, Julia E

    2016-01-01

    Zebrafish are a unique cell to behavior model for studying the basic biology of human inherited neurological conditions. Conserved vertebrate genetics and optical transparency provide in vivo access to the developing nervous system as well as high-throughput approaches for drug screens. Here we review zebrafish modeling for two broad groups of inherited conditions that each share genetic and molecular pathways and overlap phenotypically: neurodevelopmental disorders such as Autism Spectrum Disorders (ASD), Intellectual Disability (ID) and Schizophrenia (SCZ), and neurodegenerative diseases, such as Cerebellar Ataxia (CATX), Hereditary Spastic Paraplegia (HSP) and Charcot-Marie Tooth Disease (CMT). We also conduct a small meta-analysis of zebrafish orthologs of high confidence neurodevelopmental disorder and neurodegenerative disease genes by looking at duplication rates and relative protein sizes. In the past zebrafish genetic models of these neurodevelopmental disorders and neurodegenerative diseases have provided insight into cellular, circuit and behavioral level mechanisms contributing to these conditions. Moving forward, advances in genetic manipulation, live imaging of neuronal activity and automated high-throughput molecular screening promise to help delineate the mechanistic relationships between different types of neurological conditions and accelerate discovery of therapeutic strategies.

  19. Common data elements for clinical research in mitochondrial disease: a National Institute for Neurological Disorders and Stroke project.

    Science.gov (United States)

    Karaa, Amel; Rahman, Shamima; Lombès, Anne; Yu-Wai-Man, Patrick; Sheikh, Muniza K; Alai-Hansen, Sherita; Cohen, Bruce H; Dimmock, David; Emrick, Lisa; Falk, Marni J; McCormack, Shana; Mirsky, David; Moore, Tony; Parikh, Sumit; Shoffner, John; Taivassalo, Tanja; Tarnopolsky, Mark; Tein, Ingrid; Odenkirchen, Joanne C; Goldstein, Amy

    2017-05-01

    The common data elements (CDE) project was developed by the National Institute of Neurological Disorders and Stroke (NINDS) to provide clinical researchers with tools to improve data quality and allow for harmonization of data collected in different research studies. CDEs have been created for several neurological diseases; the aim of this project was to develop CDEs specifically curated for mitochondrial disease (Mito) to enhance clinical research. Nine working groups (WGs), composed of international mitochondrial disease experts, provided recommendations for Mito clinical research. They initially reviewed existing NINDS CDEs and instruments, and developed new data elements or instruments when needed. Recommendations were organized, internally reviewed by the Mito WGs, and posted online for external public comment for a period of eight weeks. The final version was again reviewed by all WGs and the NINDS CDE team prior to posting for public use. The NINDS Mito CDEs and supporting documents are publicly available on the NINDS CDE website ( https://commondataelements.ninds.nih.gov/ ), organized into domain categories such as Participant/Subject Characteristics, Assessments, and Examinations. We developed a comprehensive set of CDE recommendations, data definitions, case report forms (CRFs), and guidelines for use in Mito clinical research. The widespread use of CDEs is intended to enhance Mito clinical research endeavors, including natural history studies, clinical trial design, and data sharing. Ongoing international collaboration will facilitate regular review, updates and online publication of Mito CDEs, and support improved consistency of data collection and reporting.

  20. Diabetic db/db mice exhibit central nervous system and peripheral molecular alterations as seen in neurological disorders

    Science.gov (United States)

    Ernst, A; Sharma, A N; Elased, K M; Guest, P C; Rahmoune, H; Bahn, S

    2013-01-01

    The db/db mouse is a widely used preclinical model in diabetes research. Recent studies have shown that these mice also display aspects of psychosis and depression-like behaviors as seen in some psychiatric disorders. Here, we have performed multiplex immunoassay and liquid chromatography mass spectrometry profiling of the plasma and brain samples from db/db and control mice to identify altered pathways, which could be related to these behavioral abnormalities. This is the first study to carry out profiling of the brain proteome in this model. Plasma from the db/db mice had increased levels of leptin and insulin, decreased levels of peptide YY, glucagon and prolactin and alterations in inflammation-related proteins, compared with control mice. Frontal cortex tissue from the db/db mice showed changes in proteins involved in energy metabolism, cellular structure and neural functioning, and the hippocampus had changes in proteins involved in the same pathways, with additional effects on cellular signalling proteins. The overlap of these findings with effects seen in type 2 diabetes, schizophrenia, major depressive disorder and Alzheimer's disease might contribute to a common endophenotype seen in metabolic and neurological disorders. PMID:23715298

  1. Mixed methods inquiry into traditional healers’ treatment of mental, neurological and substance abuse disorders in rural South Africa

    Science.gov (United States)

    Audet, Carolyn M.; Ngobeni, Sizzy; Graves, Erin; Wagner, Ryan G.

    2017-01-01

    Background Traditional healers are acceptable and highly accessible health practitioners throughout sub-Saharan Africa. Patients in South Africa often seek concurrent traditional and allopathic treatment leading to medical pluralism. Methods & findings We studied the cause of five traditional illnesses known locally as “Mavabyi ya nhloko” (sickness of the head), by conducting 27 in-depth interviews and 133 surveys with a randomly selected sample of traditional healers living and working in rural, northeastern South Africa. These interviews were carried out to identify treatment practices of mental, neurological, and substance abuse (MNS) disorders. Participating healers were primarily female (77%), older in age (median: 58.0 years; interquartile range [IQR]: 50–67), had very little formal education (median: 3.7 years; IQR: 3.2–4.2), and had practiced traditional medicine for many years (median: 17 years; IQR: 9.5–30). Healers reported having the ability to successfully treat: seizure disorders (47%), patients who have lost touch with reality (47%), paralysis on one side of the body (59%), and substance abuse (21%). Female healers reported a lower odds of treating seizure disorders (Odds Ratio (OR):0.47), patients who had lost touch with reality (OR:0.26; p-valuedisorders, patients who have lost touch with reality, paralysis of one side of the body, and substance abuse. Healers charged a median of 500 South African Rand (~US$35) to treat substance abuse, 1000 Rand (~US$70) for seizure disorders or paralysis of one side of the body, and 1500 Rand (~US$105) for patients who have lost touch with reality. Conclusions While not all healers elect to treat MNS disorders, many continue to do so, delaying allopathic health services to acutely ill patients. PMID:29261705

  2. Surgical management of Charcot spinal arthropathy: a single-center retrospective series highlighting the evolution of management.

    Science.gov (United States)

    Jacobs, W Bradley; Bransford, Richard J; Bellabarba, Carlo; Chapman, Jens R

    2012-11-01

    Charcot spinal arthropathy (CSA) is an uncommon disorder that occurs in the setting of conditions with decreased protective sensation of the vertebral column, resulting in vertebral joint degeneration, pain, and deformity. Historically, CSA treatment has been fraught with high failure rates. Over time, the authors' institution has trended toward a CSA treatment paradigm of intralesional debridement, circumferential fusion, and four-rod lumbopelvic fixation. As such, the overall objectives of this study were to define the specific clinical characteristics of this rare condition and to determine whether the authors' treatment paradigm has decreased the incidence of revision due to hardware failure/presumed pseudarthrosis or the development of a new CSA over the course of the study and in comparison with historical controls. The authors performed a retrospective review of the clinical and radiographic records for all patients with CSA treated by the Spine Service at the University of Washington between 1997 and 2009. Twenty-three patients with CSA were identified. The mean age at presentation was 43.1 years, and the mean latency between spinal cord injury and CSA diagnosis was 19.6 years. The mean follow-up was 33.1 months. Pain and progressive deformity were the major presenting symptoms. Concomitant infection was identified in 17% of patients. Patients with CSA were noted to have long initial fusion constructs spanning an average of 8.4 vertebral levels. Charcot spinal arthropathy did not occur above the level of neurological injury. The vast majority of CSA cases occurred caudally along the spinal axis, with 65% occurring within 1 level of the caudal end of the index fusion construct and 35% occurring even farther distally. Revision due to hardware failure or the development of a new CSA level occurred in 35% of patients. Rates of treatment failure requiring revision significantly decreased over the course of the study, with revision occurring in 6 (66%) of 9

  3. The role of placebo in the diagnosis and treatment of functional neurologic disorders.

    Science.gov (United States)

    Rommelfanger, K S

    2016-01-01

    Placebo therapy can produce meaningful, clinical relief for a variety of conditions. While placebos are not without their ethically fraught history, they continue to be used, largely covertly, even today. Because the prognosis for psychogenic disorders is often poor and recovery may be highly dependent on the patient's belief in the diagnosis and treatment regimen, some physicians find placebo therapy for psychogenic disorders compelling, but also particularly contentious. Yet placebos also have a long tradition of being used for provocative diagnosis (wherein placebo is used to elicit and/or terminate the symptoms as a way of diagnosing symptoms as "psychogenic"). In this chapter we discuss cases describing placebo as therapy for psychogenic disorders and the challenges related to embedded Cartesian beliefs in Western medicine. The legitimate ethical reservations against placebo therapy, in general, have been related to assumptions about their "inertness" and a requirement for deception, both which are being refuted by emerging data. In this chapter, we also re-evaluate the concerns associated with placebo therapy for psychogenic disorders by asking, "Are we harming patients by withholding placebo treatment?" © 2016 Elsevier B.V. All rights reserved.

  4. A population genetic approach to mapping neurological disorder genes using deep resequencing.

    Directory of Open Access Journals (Sweden)

    Rachel A Myers

    2011-02-01

    Full Text Available Deep resequencing of functional regions in human genomes is key to identifying potentially causal rare variants for complex disorders. Here, we present the results from a large-sample resequencing (n  =  285 patients study of candidate genes coupled with population genetics and statistical methods to identify rare variants associated with Autism Spectrum Disorder and Schizophrenia. Three genes, MAP1A, GRIN2B, and CACNA1F, were consistently identified by different methods as having significant excess of rare missense mutations in either one or both disease cohorts. In a broader context, we also found that the overall site frequency spectrum of variation in these cases is best explained by population models of both selection and complex demography rather than neutral models or models accounting for complex demography alone. Mutations in the three disease-associated genes explained much of the difference in the overall site frequency spectrum among the cases versus controls. This study demonstrates that genes associated with complex disorders can be mapped using resequencing and analytical methods with sample sizes far smaller than those required by genome-wide association studies. Additionally, our findings support the hypothesis that rare mutations account for a proportion of the phenotypic variance of these complex disorders.

  5. Clinical Case of Newly Diagnosed Hypoglycemic Paroxysm Complicated by Severe Neurological Disorders

    Directory of Open Access Journals (Sweden)

    L.V. Shkala

    2013-02-01

    Full Text Available The paper presents a case of hypoglycemic paroxysm, manifested as epilepsy, in 53-year-old man, suffering from diabetes mellitus type 1, complicated by steatohepatosis following excretory and endocrine pancreatic insufficiency, disorders of renal excretory function, triggered by the lack of food after insulin administration.

  6. Hemolytic anemia following high dose intravenous immunoglobulin in patients with chronic neurological disorders

    DEFF Research Database (Denmark)

    Markvardsen, L H; Christiansen, Ingelise; Harbo, T

    2014-01-01

    High dose intravenous immunoglobulin (IVIG) is an established treatment for various neuromuscular disorders. Recently, cases of hemolytic anemia following IVIG have been observed. The objective of this study was to determine the extent of anemia and hemolysis after IVIG and its relationship...... to the AB0 blood type system....

  7. Validation of the Persian version of the dysphagia handicap index in patients with neurological disorders

    Science.gov (United States)

    Barzegar-Bafrooei, Ebrahim; Bakhtiary, Jalal; Khatoonabadi, Ahmad Reza; Fatehi, Farzad; Maroufizadeh, Saman; Fathali, Mojtaba

    2016-01-01

    Background: Dysphagia as a common condition affecting many aspects of the patient’s life. The Dysphagia Handicap Index (DHI) is a reliable self-reported questionnaire developed specifically to measure the impact of dysphagia on the patient’s quality of life. The aim of this study was to translate the questionnaire to Persian and to measure its validity and reliability in patients with neurogenic oropharyngeal dysphagia. Methods: A formal forward-backward translation of DHI was performed based on the guidelines for the cross-cultural adaptation of self-report measures. A total of 57 patients with neurogenic dysphagia who were referred to the neurology clinics of Tehran University of Medical Sciences, Iran, participated in this study. Internal consistency reliability of the DHI was examined using Cronbach’s alpha, and test-retest reliability of the scale was evaluated using intraclass correlation coefficient (ICC). Results: The internal consistency of the Persian DHI (P-DHI) was considered to be good; Cronbach’s alpha coefficient for the total P-DHI was 0.88. The test-retest reliability for the total and three subscales of the P-DHI ranged from 0.95 to 0.98 using ICC. Conclusion: The P-DHI demonstrated a good reliability, and it can be a valid instrument for evaluating the dysphagia effects on quality of life among Persian language population. PMID:27648173

  8. Venous hemodynamics in neurological disorders: an analytical review with hydrodynamic analysis

    Science.gov (United States)

    2013-01-01

    Venous abnormalities contribute to the pathophysiology of several neurological conditions. This paper reviews the literature regarding venous abnormalities in multiple sclerosis (MS), leukoaraiosis, and normal-pressure hydrocephalus (NPH). The review is supplemented with hydrodynamic analysis to assess the effects on cerebrospinal fluid (CSF) dynamics and cerebral blood flow (CBF) of venous hypertension in general, and chronic cerebrospinal venous insufficiency (CCSVI) in particular. CCSVI-like venous anomalies seem unlikely to account for reduced CBF in patients with MS, thus other mechanisms must be at work, which increase the hydraulic resistance of the cerebral vascular bed in MS. Similarly, hydrodynamic changes appear to be responsible for reduced CBF in leukoaraiosis. The hydrodynamic properties of the periventricular veins make these vessels particularly vulnerable to ischemia and plaque formation. Venous hypertension in the dural sinuses can alter intracranial compliance. Consequently, venous hypertension may change the CSF dynamics, affecting the intracranial windkessel mechanism. MS and NPH appear to share some similar characteristics, with both conditions exhibiting increased CSF pulsatility in the aqueduct of Sylvius. CCSVI appears to be a real phenomenon associated with MS, which causes venous hypertension in the dural sinuses. However, the role of CCSVI in the pathophysiology of MS remains unclear. PMID:23724917

  9. The assessment and treatment of prosodic disorders and neurological theories of prosody.

    Science.gov (United States)

    Diehl, Joshua J; Paul, Rhea

    2009-08-01

    In this article, we comment on specific aspects of Peppé (Peppé, 2009). In particular, we address the assessment and treatment of prosody in clinical settings and discuss current theory on neurological models of prosody. We argue that in order for prosodic assessment instruments and treatment programs to be clinical effective, we need assessment instruments that: (1) have a representative normative comparison sample and strong psychometric properties; (2) are based on empirical information regarding the typical sequence of prosodic acquisition and are sensitive to developmental change; (3) meaningfully subcategorize various aspects of prosody; (4) use tasks that have ecological validity; and (5) have clinical properties, such as length and ease of administration, that allow them to become part of standard language assessment batteries. In addition, we argue that current theories of prosody processing in the brain are moving toward network models that involve multiple brain areas and are crucially dependent on cortical communication. The implications of these observations for future research and clinical practice are outlined.

  10. Astrocyte differentiation of human pluripotent stem cells: new tools for neurological disorder research

    Directory of Open Access Journals (Sweden)

    Abinaya Chandrasekaran

    2016-09-01

    Full Text Available Astrocytes have a central role in brain development and function, and so have gained increasing attention over the past two decades. Consequently, our knowledge about their origin, differentiation and function has increased significantly, with new research showing that astrocytes cultured alone or co-cultured with neurons have the potential to improve our understanding of various central nervous system (CNS diseases, such as Amyotrophic lateral sclerosis, Alzheimer’s disease or Alexander disease. The generation of astrocytes derived from pluripotent stem cells (PSCs opens up a new area for studying neurologic diseases in vitro; these models could be exploited to identify and validate potential drugs by detecting adverse effects in the early stages of drug development. However, as it is now known that a range of astrocyte populations exist in the brain, it will be important in vitro to develop standardized protocols for the in vitro generation of astrocyte subsets with defined maturity status and phenotypic properties. This will then open new possibilities for co-cultures with neurons and the generation of neural organoids for research purposes. The aim of this review article is to compare and summarize the currently available protocols and their strategies to generate human astrocytes from PSCs. Furthermore, we discuss the potential role of human-induced PSCs derived astrocytes in disease modeling.

  11. Brain disease, connectivity, plasticity and cognitive therapy: A neurological view of mental disorders.

    Science.gov (United States)

    Lubrini, G; Martín-Montes, A; Díez-Ascaso, O; Díez-Tejedor, E

    2017-04-25

    Our conception of the mind-brain relationship has evolved from the traditional idea of dualism to current evidence that mental functions result from brain activity. This paradigm shift, combined with recent advances in neuroimaging, has led to a novel definition of brain functioning in terms of structural and functional connectivity. The purpose of this literature review is to describe the relationship between connectivity, brain lesions, cerebral plasticity, and functional recovery. Assuming that brain function results from the organisation of the entire brain in networks, brain dysfunction would be a consequence of altered brain network connectivity. According to this approach, cognitive and behavioural impairment following brain damage result from disrupted functional organisation of brain networks. However, the dynamic and versatile nature of these circuits makes recovering brain function possible. Cerebral plasticity allows for functional reorganisation leading to recovery, whether spontaneous or resulting from cognitive therapy, after brain disease. Current knowledge of brain connectivity and cerebral plasticity provides new insights into normal brain functioning, the mechanisms of brain damage, and functional recovery, which in turn serve as the foundations of cognitive therapy. Copyright © 2017 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  12. Convection-Enhanced Delivery for the Treatment of Pediatric Neurologic Disorders

    Science.gov (United States)

    Song, Debbie K.; Lonser, Russell R.

    2013-01-01

    Direct perfusion of specific regions of the central nervous system by convection-enhanced delivery is becoming more widely used for the delivery of compounds in the research and treatment of various neural disorders. In contrast to other currently available central nervous system delivery techniques, convection-enhanced delivery relies on bulk flow for distribution of solute. This allows for safe, targeted, reliable, and homogeneous delivery of small- and large-molecular-weight substances over clinically relevant volumes in a manner that bypasses the blood-central nervous system barrier. Recent studies have also shown that coinfused imaging surrogate tracers can be used to monitor and control the convective distribution of therapeutic agents in vivo. The unique features of convection-enhanced delivery, including the ability to monitor distribution in real-time, provide an opportunity to develop new research and treatment paradigms for pediatric patients with a variety of intrinsic central nervous system disorders. PMID:18952590

  13. Disrupted avoidance learning in functional neurological disorder: Implications for harm avoidance theories

    Directory of Open Access Journals (Sweden)

    Laurel S. Morris

    2017-01-01

    Conclusions: FND patients had impaired instrumental avoidance learning, findings that parallel previous observations of impaired action-outcome binding. FND patients further show enhanced behavioural and neural sensitivity to negative information. However, this did not translate to improved avoidance learning. Put together, our findings do not support the theory of harm avoidance in FND. We highlight a potential mechanism by which negative contexts interfere with adaptive behaviours in this under-explored disorder.

  14. Impulse control disorders in Parkinson's disease: crossroads between neurology, psychiatry and neuroscience.

    Science.gov (United States)

    Bugalho, Paulo; Oliveira-Maia, Albino J

    2013-01-01

    Non-motor symptoms contribute significantly to Parkinson's disease (PD) related disability. Impulse control disorders (ICDs) have been recently added to the behavioural spectrum of PD-related non-motor symptoms. Such behaviours are characterized by an inappropriate drive to conduct repetitive behaviours that are usually socially inadequate or result in harmful consequences. Parkinson disease impulse control disorders (PD-ICDs) have raised significant interest in the scientific and medical community, not only because of their incapacitating nature, but also because they may represent a valid model of ICDs beyond PD and a means to study the physiology of drive, impulse control and compulsive actions in the normal brain. In this review, we discuss some unresolved issues regarding PD-ICDs, including the association with psychiatric co-morbidities such as obsessive-compulsive disorder and with dopamine related side effects, such as hallucinations and dyskinesias; the relationship with executive cognitive dysfunction; and the neural underpinnings of ICDs in PD. We also discuss the contribution of neuroscience studies based on animal-models towards a mechanistic explanation of the development of PD-ICDs, specifically regarding corticostriatal control of goal directed and habitual actions.

  15. Detection and molecular analysis of Toxoplasma gondii and Neospora caninum from dogs with neurological disorders.

    Science.gov (United States)

    Langoni, Helio; Matteucci, Guilherme; Medici, Bruno; Camossi, Lucilene Granuzio; Richini-Pereira, Virgínia Bodelão; Silva, Rodrigo Costa da

    2012-06-01

    Toxoplasma gondii and Neospora caninum are related Apicomplexa parasites responsible for systemic diseases in many species of animals, including dogs. This study aimed to determine the occurrence of T. gondii and N. caninum infections in 50 dogs with neurological signs that were admitted to the Veterinary Hospital of Universidade Estadual Paulista, City of Botucatu, Brazil. All animals were screened for antibodies using an immunofluorescent antibody test for both parasites. Tissues of positive animals were bioassayed in mice (T. gondii) and gerbils (N. caninum), and DNA was analyzed using the polymerase chain reaction (PCR). Positive samples for T. gondii by PCR were typed using restriction fragment length polymorphism-PCR for 11 markers: SAG1, SAG2 (5'-3'-SAG2 and alt.SAG2), SAG3, Btub, GRA6, L358, c22-8, c29-6, PK1 and Apico, and CS3 marker for virulence analysis. Specific antibodies were detected in 11/50 (22%; 95% confidence interval (CI95%), 12.8-35.3%) animals for T. gondii and 7/50 (14%; CI95%, 7.02-26.3%) for N. caninum. In the bioassay and PCR, 7/11 (63.6%; CI95%, 34.9-84.8%) samples were positive for T. gondii and 3/7 (42.9%; CI95%I, 15.7-75.5%) samples were positive for N. caninum. Three different genotypes were identified, but only 1 was unique. These data confirm the presence of T. gondii and N. caninum in dogs from Brazil, indicating the importance of this host as a sentinel of T. gondii for human beings, and the genotypic variation of this parasite in Brazil.

  16. Inertial Sensor-Based Robust Gait Analysis in Non-Hospital Settings for Neurological Disorders

    Directory of Open Access Journals (Sweden)

    Can Tunca

    2017-04-01

    Full Text Available The gold standards for gait analysis are instrumented walkways and marker-based motion capture systems, which require costly infrastructure and are only available in hospitals and specialized gait clinics. Even though the completeness and the accuracy of these systems are unquestionable, a mobile and pervasive gait analysis alternative suitable for non-hospital settings is a clinical necessity. Using inertial sensors for gait analysis has been well explored in the literature with promising results. However, the majority of the existing work does not consider realistic conditions where data collection and sensor placement imperfections are imminent. Moreover, some of the underlying assumptions of the existing work are not compatible with pathological gait, decreasing the accuracy. To overcome these challenges, we propose a foot-mounted inertial sensor-based gait analysis system that extends the well-established zero-velocity update and Kalman filtering methodology. Our system copes with various cases of data collection difficulties and relaxes some of the assumptions invalid for pathological gait (e.g., the assumption of observing a heel strike during a gait cycle. The system is able to extract a rich set of standard gait metrics, including stride length, cadence, cycle time, stance time, swing time, stance ratio, speed, maximum/minimum clearance and turning rate. We validated the spatio-temporal accuracy of the proposed system by comparing the stride length and swing time output with an IR depth-camera-based reference system on a dataset comprised of 22 subjects. Furthermore, to highlight the clinical applicability of the system, we present a clinical discussion of the extracted metrics on a disjoint dataset of 17 subjects with various neurological conditions.

  17. Inertial Sensor-Based Robust Gait Analysis in Non-Hospital Settings for Neurological Disorders

    Science.gov (United States)

    Tunca, Can; Pehlivan, Nezihe; Ak, Nağme; Arnrich, Bert; Salur, Gülüstü; Ersoy, Cem

    2017-01-01

    The gold standards for gait analysis are instrumented walkways and marker-based motion capture systems, which require costly infrastructure and are only available in hospitals and specialized gait clinics. Even though the completeness and the accuracy of these systems are unquestionable, a mobile and pervasive gait analysis alternative suitable for non-hospital settings is a clinical necessity. Using inertial sensors for gait analysis has been well explored in the literature with promising results. However, the majority of the existing work does not consider realistic conditions where data collection and sensor placement imperfections are imminent. Moreover, some of the underlying assumptions of the existing work are not compatible with pathological gait, decreasing the accuracy. To overcome these challenges, we propose a foot-mounted inertial sensor-based gait analysis system that extends the well-established zero-velocity update and Kalman filtering methodology. Our system copes with various cases of data collection difficulties and relaxes some of the assumptions invalid for pathological gait (e.g., the assumption of observing a heel strike during a gait cycle). The system is able to extract a rich set of standard gait metrics, including stride length, cadence, cycle time, stance time, swing time, stance ratio, speed, maximum/minimum clearance and turning rate. We validated the spatio-temporal accuracy of the proposed system by comparing the stride length and swing time output with an IR depth-camera-based reference system on a dataset comprised of 22 subjects. Furthermore, to highlight the clinical applicability of the system, we present a clinical discussion of the extracted metrics on a disjoint dataset of 17 subjects with various neurological conditions. PMID:28398224

  18. Asynchronous telemedicine applications in rehabilitation of acquired speech-language disorders in neurologic patients

    Directory of Open Access Journals (Sweden)

    Beijer L

    2015-02-01

    Full Text Available Lilian Beijer,1 Toni Rietveld2 1Sint Maartenskliniek, 2Centre of Language Studies, Radboud University of Nijmegen, Nijmegen, The Netherlands Abstract: In this time of an aging population in the Western world and a concomitant need for cost reduction, there is an obvious need for innovative health care delivery. One of the consequences is that a growing number of telemedicine applications are emerging in different health care domains. Also in the area of speech-language (SL disorders, particularly neurogenic disorders, telemedicine is rapidly gaining interest. In this paper, we place applications for neurogenic SL disorders in a telemedicine taxonomy in order to establish common features along the dimensions of functionality, application, and technology, and their components. Thus, we aim at identifying common features in a wide variety of telemedicine applications and to establish common interests of stakeholders in health care for classified groups of telemedicine applications. This may facilitate decision-making with regard to expansion of innovative products, and give directions to measures needed for upscaling and structural embedding of feasible and effective SL telemedicine applications in health care. Common interests of stakeholders in health care, established using telemedicine taxonomy, is a key factor in decision-making with regard to which telemedicine applications should be given priority for genuine utilization. Priorities of health care institutions, patients, and reimbursement companies are also leading for researchers aiming at solid scientific evidence for the beneficial effects of target applications. That is, although research results tend to indicate the potential of telemedicine in the area of SL pathology, the alleged benefits of most applications have not been confirmed according to the accepted standards for clinical outcome testing as yet. Methodologic obstacles and the lack of adequate speech materials and suitable

  19. The Role of TREM2 in Alzheimer's Disease and Other Neurological Disorders.

    Science.gov (United States)

    Yaghmoor, Faris; Noorsaeed, Ahmed; Alsaggaf, Samar; Aljohani, Waleed; Scholtzova, Henrieta; Boutajangout, Allal; Wisniewski, Thomas

    2014-11-01

    Alzheimer's disease (AD) is the leading cause of dementia worldwide. Late-onset AD (LOAD), is the most common form of Alzheimer's disease, representing about >95% of cases and early-onset AD represents apolipoprotein (apo) E4 allele. Recently, rare variants of TREM2 have been reported as a significant risk factor for LOAD, comparable to inheritance of apoE4. In this review we will focus on the role(s) of TREM2 in AD as well as in other neurodegenerative disorders.

  20. Traumatic Brain Injury Induces Genome-Wide Transcriptomic, Methylomic, and Network Perturbations in Brain and Blood Predicting Neurological Disorders

    Directory of Open Access Journals (Sweden)

    Qingying Meng

    2017-02-01

    Full Text Available The complexity of the traumatic brain injury (TBI pathology, particularly concussive injury, is a serious obstacle for diagnosis, treatment, and long-term prognosis. Here we utilize modern systems biology in a rodent model of concussive injury to gain a thorough view of the impact of TBI on fundamental aspects of gene regulation, which have the potential to drive or alter the course of the TBI pathology. TBI perturbed epigenomic programming, transcriptional activities (expression level and alternative splicing, and the organization of genes in networks centered around genes such as Anax2, Ogn, and Fmod. Transcriptomic signatures in the hippocampus are involved in neuronal signaling, metabolism, inflammation, and blood function, and they overlap with those in leukocytes from peripheral blood. The homology between genomic signatures from blood and brain elicited by TBI provides proof of concept information for development of biomarkers of TBI based on composite genomic patterns. By intersecting with human genome-wide association studies, many TBI signature genes and network regulators identified in our rodent model were causally associated with brain disorders with relevant link to TBI. The overall results show that concussive brain injury reprograms genes which could lead to predisposition to neurological and psychiatric disorders, and that genomic information from peripheral leukocytes has the potential to predict TBI pathogenesis in the brain.

  1. Clinical relevance of cortical spreading depression in neurological disorders: migraine, malignant stroke, subarachnoid and intracranial hemorrhage, and traumatic brain injury.

    Science.gov (United States)

    Lauritzen, Martin; Dreier, Jens Peter; Fabricius, Martin; Hartings, Jed A; Graf, Rudolf; Strong, Anthony John

    2011-01-01

    Cortical spreading depression (CSD) and depolarization waves are associated with dramatic failure of brain ion homeostasis, efflux of excitatory amino acids from nerve cells, increased energy metabolism and changes in cerebral blood flow (CBF). There is strong clinical and experimental evidence to suggest that CSD is involved in the mechanism of migraine, stroke, subarachnoid hemorrhage and traumatic brain injury. The implications of these findings are widespread and suggest that intrinsic brain mechanisms have the potential to worsen the outcome of cerebrovascular episodes or brain trauma. The consequences of these intrinsic mechanisms are intimately linked to the composition of the brain extracellular microenvironment and to the level of brain perfusion and in consequence brain energy supply. This paper summarizes the evidence provided by novel invasive techniques, which implicates CSD as a pathophysiological mechanism for this group of acute neurological disorders. The findings have implications for monitoring and treatment of patients with acute brain disorders in the intensive care unit. Drawing on the large body of experimental findings from animal studies of CSD obtained during decades we suggest treatment strategies, which may be used to prevent or attenuate secondary neuronal damage in acutely injured human brain cortex caused by depolarization waves.

  2. Navicular subluxation as a radiographic finding in Charcot neuroarthropathy.

    Science.gov (United States)

    Estess, Allyson; Marquand, Nicole; Charlton, Timothy P; Thordarson, David B

    2013-11-01

    Treatment of patients with Charcot midfoot destruction is a difficult and increasingly common clinical problem. The pathoanatomical features of Charcot neuropathy have been evaluated in only a few studies. This study evaluated whether medial navicular subluxation (adduction of the navicular on the talus) is a radiographic finding present in patients with Charcot neuroarthropathy. A retrospective review of 143 consecutive patients diagnosed with Charcot arthropathy of the foot from January 2004 to May 2011 was performed. Patients were identified based on a clinical diagnosis code 713.5 during an outpatient clinic visit with 2 surgeons at a single institution. After exclusion criteria were applied, a series of radiographs of 50 feet in 40 patients were compared with radiographs from an age-matched control group. Radiographic data including talonavicular coverage angle and talonavicular uncoverage percentage were collected. The mean talonavicular coverage angle of the Charcot arthropathy group was 1.5 degrees and of the control group 12.1 degrees (P Charcot arthropathy group was 12.2% and for the control group 22.0% (P Charcot arthropathy than in a matched control group. It is theorized that this finding is attributable to an unopposed pull of the posterior tibial tendon on the destabilized navicular. Level IV, retrospective case series.

  3. Charcot Arthropathy Versus Osteomyelitis: Evaluation and Management.

    Science.gov (United States)

    Womack, John

    2017-04-01

    Charcot arthropathy of the foot and ankle is a severe complication of peripheral neuropathy and is most commonly seen in the developed world in association with diabetes mellitus. Correct diagnosis and differentiation from osteomyelitis of the foot and ankle are critical to guide treatment. It can exist concomitantly with osteomyelitis, typically in the setting of an advanced midfoot ulcer. Simple plain radiographs and contrasted MRI studies often yield inconclusive or confusing data. Correct use of imaging studies and a clinical algorithm can be effective tools to help make accurate and early diagnoses and guide clinical interventions for these conditions. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Republished: Addressing the burden of mental, neurological, and substance use disorders: key messages from Disease Control Priorities, 3rd edition

    Directory of Open Access Journals (Sweden)

    Vikram Patel

    2016-01-01

    Full Text Available The burden of mental, neurological, and substance use (MNS disorders increased by 41% between 1990 and 2010 and now accounts for one in every 10 lost years of health globally. This sobering statistic does not take into account the substantial excess mortality associated with these disorders or the social and economic consequences of MNS disorders on affected persons, their caregivers, and society. A wide variety of effective interventions, including drugs, psychological treatments, and social interventions, can prevent and treat MNS disorders. At the population-level platform of service delivery, best practices include legislative measures to restrict access to means of self-harm or suicide and to reduce the availability of and demand for alcohol. At the community-level platform, best practices include life-skills training in schools to build social and emotional competencies. At the health-care-level platform, we identify three delivery channels. Two of these delivery channels are especially relevant from a public health perspective: self-management (eg, web-based psychological therapy for depression and anxiety disorders and primary care and community outreach (eg, non-specialist health worker delivering psychological and pharmacological management of selected disorders. The third delivery channel, hospital care, which includes specialist services for MNS disorders and first-level hospitals providing other types of services (such as general medicine, HIV, or paediatric care, play an important part for a smaller proportion of cases with severe, refractory, or emergency presentations and for the integration of mental health care in other health-care channels, respectively. The costs of providing a significantly scaled up package of specified cost-effective interventions for prioritised MNS disorders in low-income and lower-middle-income countries is estimated at US$3-4 per head of population per year. Since a substantial proportion of MNS

  5. Addressing the burden of mental, neurological, and substance use disorders: key messages from Disease Control Priorities, 3rd edition.

    Science.gov (United States)

    Patel, Vikram; Chisholm, Dan; Parikh, Rachana; Charlson, Fiona J; Degenhardt, Louisa; Dua, Tarun; Ferrari, Alize J; Hyman, Steve; Laxminarayan, Ramanan; Levin, Carol; Lund, Crick; Medina Mora, María Elena; Petersen, Inge; Scott, James; Shidhaye, Rahul; Vijayakumar, Lakshmi; Thornicroft, Graham; Whiteford, Harvey

    2016-04-16

    The burden of mental, neurological, and substance use (MNS) disorders increased by 41% between 1990 and 2010 and now accounts for one in every 10 lost years of health globally. This sobering statistic does not take into account the substantial excess mortality associated with these disorders or the social and economic consequences of MNS disorders on affected persons, their caregivers, and society. A wide variety of effective interventions, including drugs, psychological treatments, and social interventions, can prevent and treat MNS disorders. At the population-level platform of service delivery, best practices include legislative measures to restrict access to means of self-harm or suicide and to reduce the availability of and demand for alcohol. At the community-level platform, best practices include life-skills training in schools to build social and emotional competencies. At the health-care-level platform, we identify three delivery channels. Two of these delivery channels are especially relevant from a public health perspective: self-management (eg, web-based psychological therapy for depression and anxiety disorders) and primary care and community outreach (eg, non-specialist health worker delivering psychological and pharmacological management of selected disorders). The third delivery channel, hospital care, which includes specialist services for MNS disorders and first-level hospitals providing other types of services (such as general medicine, HIV, or paediatric care), play an important part for a smaller proportion of cases with severe, refractory, or emergency presentations and for the integration of mental health care in other health-care channels, respectively. The costs of providing a significantly scaled up package of specified cost-effective interventions for prioritised MNS disorders in low-income and lower-middle-income countries is estimated at US$3-4 per head of population per year. Since a substantial proportion of MNS disorders run a

  6. Neurological Complications of AIDS

    Science.gov (United States)

    ... the National Library of Medicine’s MedlinePlus Living with HIV/AIDS × What research is being done? The National Institute of Neurological ... the National Library of Medicine’s MedlinePlus Living with HIV/AIDS See More About Research The National Institute of Neurological Disorders and Stroke ( ...

  7. Mixed methods inquiry into traditional healers' treatment of mental, neurological and substance abuse disorders in rural South Africa.

    Science.gov (United States)

    Audet, Carolyn M; Ngobeni, Sizzy; Graves, Erin; Wagner, Ryan G

    2017-01-01

    Traditional healers are acceptable and highly accessible health practitioners throughout sub-Saharan Africa. Patients in South Africa often seek concurrent traditional and allopathic treatment leading to medical pluralism. We studied the cause of five traditional illnesses known locally as "Mavabyi ya nhloko" (sickness of the head), by conducting 27 in-depth interviews and 133 surveys with a randomly selected sample of traditional healers living and working in rural, northeastern South Africa. These interviews were carried out to identify treatment practices of mental, neurological, and substance abuse (MNS) disorders. Participating healers were primarily female (77%), older in age (median: 58.0 years; interquartile range [IQR]: 50-67), had very little formal education (median: 3.7 years; IQR: 3.2-4.2), and had practiced traditional medicine for many years (median: 17 years; IQR: 9.5-30). Healers reported having the ability to successfully treat: seizure disorders (47%), patients who have lost touch with reality (47%), paralysis on one side of the body (59%), and substance abuse (21%). Female healers reported a lower odds of treating seizure disorders (Odds Ratio (OR):0.47), patients who had lost touch with reality (OR:0.26; p-valuesubstance abuse (OR:0.36) versus males. Each additional year of education received was found to be associated with lower odds, ranging from 0.13-0.27, of treating these symptoms. Each additional patient seen by healers in the past week was associated with roughly 1.10 higher odds of treating seizure disorders, patients who have lost touch with reality, paralysis of one side of the body, and substance abuse. Healers charged a median of 500 South African Rand (~US$35) to treat substance abuse, 1000 Rand (~US$70) for seizure disorders or paralysis of one side of the body, and 1500 Rand (~US$105) for patients who have lost touch with reality. While not all healers elect to treat MNS disorders, many continue to do so, delaying allopathic

  8. Mixed methods inquiry into traditional healers' treatment of mental, neurological and substance abuse disorders in rural South Africa.

    Directory of Open Access Journals (Sweden)

    Carolyn M Audet

    Full Text Available Traditional healers are acceptable and highly accessible health practitioners throughout sub-Saharan Africa. Patients in South Africa often seek concurrent traditional and allopathic treatment leading to medical pluralism.We studied the cause of five traditional illnesses known locally as "Mavabyi ya nhloko" (sickness of the head, by conducting 27 in-depth interviews and 133 surveys with a randomly selected sample of traditional healers living and working in rural, northeastern South Africa. These interviews were carried out to identify treatment practices of mental, neurological, and substance abuse (MNS disorders. Participating healers were primarily female (77%, older in age (median: 58.0 years; interquartile range [IQR]: 50-67, had very little formal education (median: 3.7 years; IQR: 3.2-4.2, and had practiced traditional medicine for many years (median: 17 years; IQR: 9.5-30. Healers reported having the ability to successfully treat: seizure disorders (47%, patients who have lost touch with reality (47%, paralysis on one side of the body (59%, and substance abuse (21%. Female healers reported a lower odds of treating seizure disorders (Odds Ratio (OR:0.47, patients who had lost touch with reality (OR:0.26; p-value<0.05, paralysis of one side of the body (OR:0.36, and substance abuse (OR:0.36 versus males. Each additional year of education received was found to be associated with lower odds, ranging from 0.13-0.27, of treating these symptoms. Each additional patient seen by healers in the past week was associated with roughly 1.10 higher odds of treating seizure disorders, patients who have lost touch with reality, paralysis of one side of the body, and substance abuse. Healers charged a median of 500 South African Rand (~US$35 to treat substance abuse, 1000 Rand (~US$70 for seizure disorders or paralysis of one side of the body, and 1500 Rand (~US$105 for patients who have lost touch with reality.While not all healers elect to treat MNS

  9. Quality of clinical trials for selected priority mental and neurological disorders in sub-Saharan Africa: a systematic review

    Directory of Open Access Journals (Sweden)

    Mulugeta A

    2016-12-01

    Full Text Available Anwar Mulugeta,1 Girmay Medhin,2 Getnet Yimer,1 Rahimush Jemal,3 Abebaw Fekadu,4,5 1Department of Pharmacology, School of Medicine, College of Health Sciences, Addis Ababa University, 2Aklilu Lemma Institute of Pathobiology, Addis Ababa University, 3Department of Pharmacy, Tikur Anbesa Hospital, 4Department of Psychiatry, School of Medicine, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia; 5Department of Psychological Medicine, Centre for Affective Disorders, Institute of Psychiatry, King’s College London, London, UK Background: There is a developing consensus on the effectiveness of various interventions for mental disorders in low- and middle-income countries, and it has been proposed that the main task is to scale up these interventions. In this context, we aimed to review the quality and extent of intervention trials for selected priority mental and neurological disorders in sub-Saharan Africa.Methods: Medline and African Journals Online databases were used for searching relevant articles. Both randomized and nonrandomized clinical trials for the treatment of schizophrenia, depression, maternal depression, bipolar disorder, and epilepsy/seizure disorders that involve pharmacotherapy, psychotherapy, and physical therapy were included. An extensive list of search terms that identified locations, disorders, interventions, and study types were employed. The qualities of the trials were appraised using the single-component quality assessment of the consolidated standards of reporting trials (CONSORT statement and the Jadad scale.Results: From 1,136 studies identified, only 34 trials that fulfilled inclusion criteria were used for quality analysis. Most studies were clinical trials of treatments for epilepsy and were conducted after 2006. In terms of region, the majority of studies were conducted in South Africa (22 of the 34 studies. Approximately half of the trials (53% were conducted in single center and the majority

  10. Marcel Proust's diseases and doctors: the neurological story of a life.

    Science.gov (United States)

    Bogousslavsky, Julien

    2007-01-01

    Marcel Proust (1871-1922), one of the greatest writers of all times, suffered from asthma beginning at age 9, in an era when the illness was considered a 'nervous' disorder belonging to what Beard, in 1870, called 'neurasthenia'. Proust's father, Adrien, was himself a professor of medicine (hygiene) who had met Charcot, and who contributed to neurology with studies on aphasia, stroke, hysteria, and neurasthenia - a condition about which he, along with Gilbert Ballet, published a book in 1897. Through his father, Proust met Edouard Brissaud, the co-founder of the Revue Neurologique in 1893, and, in 1896, the author of The Hygiene of the Asthmatics, with a foreword by Adrien Proust. Shortly after his mother's death in 1905, Proust contemplated admitting himself to a private hospital to reset his irregular sleep patterns and to improve his asthma. He hesitated in his choice of care between Jules Dejerine in Paris, Henry-Auguste Widmer at Valmont, and Paul Dubois in Bern. Finally, he decided to enter Paul Sollier's clinic, in Boulogne-sur-Seine, on the advice of Brissaud, and stayed there for 6 weeks in semi-isolation. Together with Babinski, Sollier was, at that time, considered the most gifted follower of Charcot. He was a forerunner of studies on emotional memory, which strongly influenced Proust. In Proust's opus magnum work In Search of Lost Time, 'involuntary memory' indeed forms the core mechanism of the entire novel, counterbalancing the decaying effects of time. A few years before his death from complicated bronchopneumonia at age 52, Proust became terrified of developing a stroke, like his mother and father, and he consulted with Joseph Babinski, who tried to reassure him. Proust's life followed an unusual neurological itinerary, which has been largely overlooked, but which is in fact critical for an understanding of his literary work.

  11. Mechanisms of action for the medium-chain triglyceride ketogenic diet in neurological and metabolic disorders.

    Science.gov (United States)

    Augustin, Katrin; Khabbush, Aziza; Williams, Sophie; Eaton, Simon; Orford, Michael; Cross, J Helen; Heales, Simon J R; Walker, Matthew C; Williams, Robin S B

    2018-01-01

    High-fat, low-carbohydrate diets, known as ketogenic diets, have been used as a non-pharmacological treatment for refractory epilepsy. A key mechanism of this treatment is thought to be the generation of ketones, which provide brain cells (neurons and astrocytes) with an energy source that is more efficient than glucose, resulting in beneficial downstream metabolic changes, such as increasing adenosine levels, which might have effects on seizure control. However, some studies have challenged the central role of ketones because medium-chain fatty acids, which are part of a commonly used variation of the diet (the medium-chain triglyceride ketogenic diet), have been shown to directly inhibit AMPA receptors (glutamate receptors), and to change cell energetics through mitochondrial biogenesis. Through these mechanisms, medium-chain fatty acids rather than ketones are likely to block seizure onset and raise seizure threshold. The mechanisms underlying the ketogenic diet might also have roles in other disorders, such as preventing neurodegeneration in Alzheimer's disease, the proliferation and spread of cancer, and insulin resistance in type 2 diabetes. Analysing medium-chain fatty acids in future ketogenic diet studies will provide further insights into their importance in modified forms of the diet. Moreover, the results of these studies could facilitate the development of new pharmacological and dietary therapies for epilepsy and other disorders. Copyright © 2018 Elsevier Ltd. All rights reserved.

  12. Magnesium/calcium related neurological disorders in the ALS focus of the Kii Peninsula

    Energy Technology Data Exchange (ETDEWEB)

    Yasui, Masayuki; Yoshida, Munehito; Tamaki, Tetsuya; Taniguchi, Yasunori; Minamide, Akihito; Ota, Kiichiro [Wakayama Medical Coll. (Japan); Sasajima, Kazuhisa

    1997-01-01

    Current epidemiological surveys in the Western Pacific area and Kii Peninsula have suggested that low calcium(Ca), magnesium(Mg) and high aluminum(Al) and manganese(Mn) in river, soil and drinking water may be implicated in the pathogenetic process of amyotrophic lateral sclerosis(ALS) and Parkinsonism-dementia(PD). The condition of unbalanced minerals was experimentally mimicked in this study using rats. Male Wistar rats, weighing 200 g, were maintained for 90 days on the following diets: (A) standard diet, (B) low Ca diet, (C) low Ca-Mg diet, (D) low Ca-Mg diet with high Al. In the groups maintained on unbalanced mineral diets, Ca and Mg contents of the bones were lower than standard diet. On the other hand, Ca content of CNS showed higher values in the unbalanced diet groups than those in the standard diet group. This was determined by neutron activation analysis(NAA) at KUR. Also, Ca content in soft tissues of rats given unbalanced mineral diets was higher than those on standard diet. Mg content of soft tissues and spinal cord of rats was markedly lower in the low Ca-Mg plus high Al diet group than the other three groups as determined by inductively coupled plasma emission spectrometry(ICP). Six Kii cases with amyotrophic lateral sclerosis(ALS) also showed higher Ca and lower Mg contents in the CNS tissues than those of neurologically normal controls. The calcification of the spinal ligaments(CSL) has been reported in only 120 cases in the world and 28 cases of CSL in the Kii Peninsula have been found in the same foci as ALS. We analyzed Mg content of 7 spinal bones and 10 ligaments of the CSL and Ca content of 5 spinal bones compared with controls. The CSL showed lower values of Mg contents in bones and ligaments compared to controls. The Ca content in bones of CSL was significantly lower than that of controls. This suggests that the environmental factor may contribute to the pathogenesis of CSL due to low Ca and Mg intake as well as for ALS. (J.P.N.)

  13. Combined detection of depression and anxiety in epilepsy patients using the Neurological Disorders Depression Inventory for Epilepsy and the World Health Organization well-being index

    DEFF Research Database (Denmark)

    Hansen, Christian Pilebæk; Amiri, Moshgan

    2015-01-01

    PURPOSE: To validate the Danish version of the Neurological Disorders Depression Inventory for Epilepsy (NDDI-E), and compare it with the World Health Organization index for psychological well-being (WHO-5) as screening tests for depression and anxiety in epilepsy patients. METHODS: Epilepsy...

  14. Neurologic and neuromuscular functional disorders of the pharynx and esophagus; Neurologisch bedingte und neuromuskulaere Funktionsstoerungen des Pharynx und Oesophagus

    Energy Technology Data Exchange (ETDEWEB)

    Wuttge-Hannig, A. [Gemeinschaftspraxis fuer Radiologie, Nuklearmedizin und Strahlentherapie, Muenchen (Germany); Hannig, C. [Klinikum rechts der Isar der Technischen Universitaet Muenchen, Institut fuer Roentgendiagnostik, Muenchen (Germany)

    2007-02-15

    Neurologic swallowing disorders are an increasing diagnostic problem in our overaged population. Undiagnosed chronic aspiration pneumonia is the cause of death in 20-40% of all inhabitants of nursing homes. In neurologic diseases of the pharynx, the physiologic interaction of pharyngeal contraction, closure of the pharynx, and esophageal motility are frequently disturbed. This may be due to cortical, bulbar, or cerebellar brain damage of ischemic or traumatic origin. Furthermore diseases or peripheral nerves, muscles, and synapses cause disturbances. The most life-threatening complication of these disturbances is tracheal aspiration, which requires an iso-osmolar contrast medium for imaging studies that cause no or minimal pulmonary problems. Utilizing fast dynamic documentation we can analyze the swallowing act in 35 images within the passage time of 0.7 s. This requires digital frame sequences from 15-50 images/s, which can be provided by DSI or videofluoroscopy. Neurologic and neuromuscular patterns are demonstrated with and without tracheal aspiration. The differentiation of aspiration in a so-called pre-, intra-, and postdeglutitive form is possible. We distinguish four grades of severity of aspiration, which is also of great clinical impact for the differential rehabilitation therapy. The efficiency of the rehabilitation protocol can be assessed by the dynamic swallowing studies. (orig.) [German] Neurologische Schluckstoerungen stellen mit zunehmender Ueberalterung der Bevoelkerung ein wachsendes diagnostisches Problem dar. 20-40% aller Alter- und Pflegeheiminsassen versterben an einer nicht erkannten aspirationsbedingten Pneumonie. Gerade bei den neurologischen Erkrankungen des Pharynx und der Speiseroehre ist die physiologische Interaktion zwischen Pharynxkontraktion, Larynxschluss und oesophagealer Motilitaet haeufig gestoert. Hierbei koennen sowohl kortikale, bulbaere sowie zerebellaere Hirnschaeden ischaemischer oder traumatischer Genese, Erkrankungen

  15. Insights into the Pathology of the α2-Na(+)/K(+)-ATPase in Neurological Disorders; Lessons from Animal Models.

    Science.gov (United States)

    Isaksen, Toke J; Lykke-Hartmann, Karin

    2016-01-01

    A functional Na(+)/K(+)-ATPase consists of a catalytic α subunit and a regulatory β subunit. Four α isoforms of the Na(+)/K(+)-ATPase are found in mammals, each with a unique expression pattern and catalytic activity. The α2 isoform, encoded by the ATP1A2 gene, is primarily found in the central nervous system (CNS) and in heart-, skeletal- and smooth muscle tissues. In the CNS, the α2 isoform is mainly expressed in glial cells. In particular, the α2 isoform is found in astrocytes, important for astrocytic K(+) clearance and, consequently, the indirect uptake of neurotransmitters. Both processes are essential for proper brain activity, and autosomal dominantly mutations in the ATP1A2 gene cause the neurological disorder Familial hemiplegic migraine type 2 (FHM2). FHM2 is a severe subtype of migraine with aura including temporary numbness or weakness, and affecting only one side of the body. FHM2 patients often suffer from neurological comorbidities such as seizures, sensory disturbances, cognitive impairment, and psychiatric manifestations. The functional consequences of FHM2 disease mutations leads to a partial or complete loss of function of pump activity; however, a clear phenotype-genotype correlation has yet to be elucidated. Gene-modified mouse models targeting the Atp1a2 gene have proved instrumental in the understanding of the pathology of FHM2. Several Atp1a2 knockout (KO) mice targeting different exons have been reported. Homozygous Atp1a2 KO mice die shortly after birth due to respiratory malfunction resulting from abnormal Cl(-) homeostasis in brainstem neurons. Heterozygous KO mice are viable, but display altered behavior and neurological deficits such as altered spatial learning, decreased motor activity and enhanced fear/anxiety compared to wild type mice. FHM2 knock-in (KI) mouse models carrying the human in vivo disease mutations W887R and G301R have also been reported. Both models display altered cortical spreading depression (CSD) and point

  16. INSIGHTS INTO THE PATHOLOGY OF THE α2-Na+/K+-ATPase IN NEUROLOGICAL DISORDERS; LESSONS FROM ANIMAL MODELS

    Directory of Open Access Journals (Sweden)

    Toke Jost Isaksen

    2016-05-01

    Full Text Available A functional Na+/K+-ATPase consists of a catalytic α subunit and a regulatory β subunit. Four α isoforms of the Na+/K+-ATPase are found in mammals, each with a unique expression pattern and catalytic activity. The α2 isoform, encoded by the ATP1A2 gene, is primarily found in the central nervous system (CNS and in heart-, skeletal- and smooth muscle tissues. In the CNS, the α2 isoform is mainly expressed in neuroglial cells. In particular, the α2 isoform is found in astrocytes, and is important for astrocytic K+ clearance and, consequently, the indirect uptake of neurotransmitters. Both processes are essential for proper brain activity, and autosomal dominantly mutations in the ATP1A2 gene cause the neurological disorder Familial hemiplegic migraine type 2 (FHM2. FHM2 is a severe subtype of migraine with aura that involving temporary numbness or weakness, and affecting only one side of the body. FHM2 patients often suffer from neurological comorbidities such as seizures, sensory disturbances, cognitive impairment and psychiatric manifestations. The functional consequences of FHM2 disease mutations leads to a partial or complete loss of function of pump activity; however a clear phenotype-genotype correlation has yet to be elucidated. Gene-modified mouse models targeting the Atp1a2 gene have proved instrumental in the understanding of the pathology of FHM2. Several Atp1a2 knockout (KO mice targeting different exons have been reported. Homozygous Atp1a2 KO mice die shortly after birth due to respiratory malfunction resulting from abnormal Cl- homeostasis in brainstem neurons. Heterozygous KO mice are viable, but display altered behavior and neurological deficits such as altered spatial learning, decreased motor activity and enhanced fear/anxiety compared to wild type mice. FHM2 knock-in (KI mouse models carrying the human in vivo disease mutations W887R and G301R have also been reported. Both models display altered cortical spreading

  17. Systemic and neurologic abnormalities distinguish the lysosomal disorders sialidosis and galactosialidosis in mice.

    Science.gov (United States)

    de Geest, Natalie; Bonten, Erik; Mann, Linda; de Sousa-Hitzler, Jean; Hahn, Christopher; d'Azzo, Alessandra

    2002-06-01

    Neuraminidase initiates the hydrolysis of sialo-glycoconjugates by removing their terminal sialic acid residues. In humans, primary or secondary deficiency of this enzyme leads to two clinically similar neurodegenerative lysosomal storage disorders: sialidosis and galactosialidosis (GS). Mice nullizygous at the Neu1 locus develop clinical abnormalities reminiscent of early-onset sialidosis in children, including severe nephropathy, progressive edema, splenomegaly, kyphosis and urinary excretion of sialylated oligosaccharides. Although the sialidosis mouse model shares clinical and histopathological features with GS mice and GS patients, we have identified phenotypic abnormalities that seem specific for sialidosis mice. These include progressive deformity of the spine, high incidence of premature death, age-related extramedullary hematopoiesis, and lack of early degeneration of cerebellar Purkinje cells. The differences and similarities identified in these sialidosis and GS mice may help to better understand the pathophysiology of these diseases in children and to identify more targeted therapies for each of these diseases.

  18. Maternal Factors that Induce Epigenetic Changes Contribute to Neurological Disorders in Offspring.

    Science.gov (United States)

    Banik, Avijit; Kandilya, Deepika; Ramya, Seshadri; Stünkel, Walter; Chong, Yap Seng; Dheen, S Thameem

    2017-05-24

    It is well established that the regulation of epigenetic factors, including chromatic reorganization, histone modifications, DNA methylation, and miRNA regulation, is critical for the normal development and functioning of the human brain. There are a number of maternal factors influencing epigenetic pathways such as lifestyle, including diet, alcohol consumption, and smoking, as well as age and infections (viral or bacterial). Genetic and metabolic alterations such as obesity, gestational diabetes mellitus (GDM), and thyroidism alter epigenetic mechanisms, thereby contributing to neurodevelopmental disorders (NDs) such as embryonic neural tube defects (NTDs), autism, Down's syndrome, Rett syndrome, and later onset of neuropsychological deficits. This review comprehensively describes the recent findings in the epigenetic landscape contributing to altered molecular profiles resulting in NDs. Furthermore, we will discuss potential avenues for future research to identify diagnostic markers and therapeutic epi-drugs to reverse these abnormalities in the brain as epigenetic marks are plastic and reversible in nature.

  19. Identification of Charcot-Leyden crystals by electron microscopy.

    Science.gov (United States)

    Carson, H J; Buschmann, R J; Weisz-Carrington, P; Choi, Y S

    1992-01-01

    Observations of the ultrastructure of Charcot-Leyden crystals are sporadic in the literature. These crystals appear occasionally in clinical materials, however, and may pose diagnostic dilemmas if not correctly identified. Two cases in which unusual crystallike structures were seen on electron micrographs of specimens were evaluated for diagnostic purposes. These structures were tentatively identified as Charcot-Leyden crystals and subsequently were confirmed as such by immunoperoxidase labeling. The cases are reported together with a review of the ultrastructure, histology, immunology, and natural history of Charcot-Leyden crystals.

  20. Neurological diseases and pain

    OpenAIRE

    Borsook, David

    2011-01-01

    Chronic pain is a frequent component of many neurological disorders, affecting 20–40% of patients for many primary neurological diseases. These diseases result from a wide range of pathophysiologies including traumatic injury to the central nervous system, neurodegeneration and neuroinflammation, and exploring the aetiology of pain in these disorders is an opportunity to achieve new insight into pain processing. Whether pain originates in the central or peripheral nervous system, it frequentl...

  1. The role of nanotechnology and nano and micro-electronics in monitoring and control of cardiovascular diseases and neurological disorders

    Science.gov (United States)

    Varadan, Vijay K.

    2007-04-01

    Nanotechnology has been broadly defined as the one for not only the creation of functional materials and devices as well as systems through control of matter at the scale of 1-100 nm, but also the exploitation of novel properties and phenomena at the same scale. Growing needs in the point-of-care (POC) that is an increasing market for improving patient's quality of life, are driving the development of nanotechnologies for diagnosis and treatment of various life threatening diseases. This paper addresses the recent development of nanodiagnostic sensors and nanotherapeutic devices with functionalized carbon nanotube and/or nanowire on a flexible organic thin film electronics to monitor and control of the three leading diseases namely 1) neurodegenerative diseases, 2) cardiovascular diseases, and 3) diabetes and metabolic diseases. The sensors developed include implantable and biocompatible devices, light weight wearable devices in wrist-watches, hats, shoes and clothes. The nanotherapeutics devices include nanobased drug delivery system. Many of these sensors are integrated with the wireless systems for the remote physiological monitoring. The author's research team has also developed a wireless neural probe using nanowires and nanotubes for monitoring and control of Parkinson's disease. Light weight and compact EEG, EOG and EMG monitoring system in a hat developed is capable of monitoring real time epileptic patients and patients with neurological and movement disorders using the Internet and cellular network. Physicians could be able to monitor these signals in realtime using portable computers or cell phones and will give early warning signal if these signals cross a pre-determined threshold level. In addition the potential impact of nanotechnology for applications in medicine is that, the devices can be designed to interact with cells and tissues at the molecular level, which allows high degree of functionality. Devices engineered at nanometer scale imply a

  2. Mannose-binding Lectin Mediated Complement Pathway in Autoimmune Neurological Disorders.

    Science.gov (United States)

    Farrokhi, Mehrdad; Dabirzadeh, Mehrnoosh; Dastravan, Nastaran; Etemadifar, Masoud; Ghadimi, Keyvan; Saadatpour, Zahra; Rezaei, Ali

    2016-06-01

    Multiple sclerosis (MS) is a complex, demyelinating disease of the central nervous system (CNS) with variable phenotypic presentations, while Guillain-Barre Syndrome (GBS) is the prototypic acute inflammatory disorder that affects the peripheral nervous system. Myasthenia gravis (MG) is a T cell dependent and antibody mediated autoimmune disease. Although it has been shown that complement plays a critical role in the pathogenesis of MS, GBS, and MG, the role of mannose-binding lectin (MBL) as a biomarker of immunopathogensis of these diseases and also its association with the severity of them have been poorly investigated. Therefore, in this study we aimed to measure plasma levels of MBL in patients with MS, GBS, and MG. In a case-control study, plasma was obtained from healthy controls (n=100) and also patients with MS (n=120), GBS (n=30), and MG (n=30). Plasma level measurement of MBL was performed using enzyme-linked immunosorbent assay (ELISA). The mean serum level of MBL was significantly different between groups of patients and healthy controls (p<0.001). We also found a positive correlation between plasma levels of MBL and severity scores of MS, MG, and GBS patients including: expanded disability status scale (EDSS) (r=+0.60 and p=<0.001), quantitative myasthenia gravis score (QMGS) (r=+0.56 and p=0.01), and GBS disability scale (GDS) (r=+0.37 and p=0.04). Taken together, our findings suggest that complement activation mediated by MBL contributes to the pathogenesis and also severity of MS, MG, and GBS. However, because the lectin pathway can be involved in several phases of the immune response, further evidence will be required to elucidate the underlying mechanism.

  3. Mutations in the MORC2 gene cause axonal Charcot-Marie-Tooth disease.

    Science.gov (United States)

    Sevilla, Teresa; Lupo, Vincenzo; Martínez-Rubio, Dolores; Sancho, Paula; Sivera, Rafael; Chumillas, María J; García-Romero, Mar; Pascual-Pascual, Samuel I; Muelas, Nuria; Dopazo, Joaquín; Vílchez, Juan J; Palau, Francesc; Espinós, Carmen

    2016-01-01

    Charcot-Marie-Tooth disease (CMT) is a complex disorder with wide genetic heterogeneity. Here we present a new axonal Charcot-Marie-Tooth disease form, associated with the gene microrchidia family CW-type zinc finger 2 (MORC2). Whole-exome sequencing in a family with autosomal dominant segregation identified the novel MORC2 p.R190W change in four patients. Further mutational screening in our axonal Charcot-Marie-Tooth disease clinical series detected two additional sporadic cases, one patient who also carried the same MORC2 p.R190W mutation and another patient that harboured a MORC2 p.S25L mutation. Genetic and in silico studies strongly supported the pathogenicity of these sequence variants. The phenotype was variable and included patients with congenital or infantile onset, as well as others whose symptoms started in the second decade. The patients with early onset developed a spinal muscular atrophy-like picture, whereas in the later onset cases, the initial symptoms were cramps, distal weakness and sensory impairment. Weakness and atrophy progressed in a random and asymmetric fashion and involved limb girdle muscles, leading to a severe incapacity in adulthood. Sensory loss was always prominent and proportional to disease severity. Electrophysiological studies were consistent with an asymmetric axonal motor and sensory neuropathy, while fasciculations and myokymia were recorded rather frequently by needle electromyography. Sural nerve biopsy revealed pronounced multifocal depletion of myelinated fibres with some regenerative clusters and occasional small onion bulbs. Morc2 is expressed in both axons and Schwann cells of mouse peripheral nerve. Different roles in biological processes have been described for MORC2. As the silencing of Charcot-Marie-Tooth disease genes have been associated with DNA damage response, it is tempting to speculate that a deregulation of this pathway may be linked to the axonal degeneration observed in MORC2 neuropathy, thus adding a

  4. Strokes with minor symptoms: an exploratory analysis of the National Institute of Neurological Disorders and Stroke recombinant tissue plasminogen activator trials.

    Science.gov (United States)

    Khatri, Pooja; Kleindorfer, Dawn O; Yeatts, Sharon D; Saver, Jeffrey L; Levine, Steven R; Lyden, Patrick D; Moomaw, Charles J; Palesch, Yuko Y; Jauch, Edward C; Broderick, Joseph P

    2010-11-01

    The pivotal National Institute of Neurological Disorders and Stroke recombinant tissue plasminogen activator trials excluded patients with ischemic stroke with specific minor presentations or rapidly improving symptoms. The recombinant tissue plasminogen activator product label notes that its use for minor neurological deficit or rapidly improving stroke symptoms has not been evaluated. As a result, patients with low National Institutes of Health Stroke Scale scores are not commonly treated in clinical practice. We sought to further characterize the patients with minor stroke who were included in the National Institute of Neurological Disorders and Stroke trials. Minor strokes were defined as National Institutes of Health Stroke Scale score ≤ 5 at baseline for this retrospective analysis, because this subgroup is most commonly excluded from treatment in clinical practice and trials. Clinical stroke syndromes were defined based on prespecified National Institutes of Health Stroke Scale item score clusters. Clinical outcomes were reviewed generally and within these cluster subgroups. Only 58 cases had National Institutes of Health Stroke Scale scores of 0 to 5 in the National Institute of Neurological Disorders and Stroke trials (42 recombinant tissue plasminogen activator and 16 placebo), and 2971 patients were excluded from the trials due to "rapidly improving" or "minor symptoms" as the primary reason. No patients were enrolled with isolated motor symptoms, isolated facial droop, isolated ataxia, dysarthria, isolated sensory symptoms, or with only symptoms/signs not captured by the National Institutes of Health Stroke Scale score (ie, National Institutes of Health Stroke Scale=0). There were ≤ 3 patients with each of the other isolated deficits enrolled in the trial. The National Institute of Neurological Disorders and Stroke trials excluded a substantial number of strokes with minor presentations, those that were included were small in number, and conclusions

  5. [Charcot arthropathy and diabetic foot].

    Science.gov (United States)

    López-Gavito, E; Parra-Téllez, P; Vázquez-Escamilla, J

    2016-01-01

    Diabetes mellitus is a major chronic degenerative disease, which currently is taking on alarming proportions in the population of our country. Neuropathic arthropathy is one of the most interesting degenerative joint disorders and increasingly common within the orthopedic pathology. It is defined as a progressive degenerative arthropathy, chronic and affecting one or more peripheral joints, and develops as a result of the lack of sensory perception normal in the innervation of joints. As a result the joints of the feet are subjected to trauma and repetitive injury causing a neurotraumatic effect with progressive damage to the joints of the hindfoot, midfoot and forefoot. Diagnosis includes a proper medical history, careful examination of the affected limb, conventional X-ray, scintigraphy, computed tomography and magnetic resonance imaging in some cases. Conservative treatment includes: drugs, rest of the affected limb, and the use of appliances like total-contact cast, orthotics or special shoes. Surgical treatment depends on the stage of the disease, and may require one or more surgical procedures, in order to achieve a full foot plantar support to prevent ulcers. One of the surgeries performed most often is the fusion of damaged joints. Surgery must be performed only in the coalescence phase of the disease, using internal, or external fixation or both.

  6. A preliminary investigation of sleep quality in functional neurological disorders: Poor sleep appears common, and is associated with functional impairment.

    Science.gov (United States)

    Graham, Christopher D; Kyle, Simon D

    2017-07-15

    Functional neurological disorders (FND) are disabling conditions for which there are few empirically-supported treatments. Disturbed sleep appears to be part of the FND context; however, the clinical importance of sleep disturbance (extent, characteristics and impact) remains largely unknown. We described sleep quality in two samples, and investigated the relationship between sleep and FND-related functional impairment. We included a sample recruited online via patient charities (N=205) and a consecutive clinical sample (N=20). Participants completed validated measures of sleep quality and sleep characteristics (e.g. total sleep time, sleep efficiency), mood, and FND-related functional impairment. Poor sleep was common in both samples (89% in the clinical range), which was characterised by low sleep efficiency (M=65.40%) and low total sleep time (M=6.05h). In regression analysis, sleep quality was negatively associated with FND-related functional impairment, accounting for 16% of the variance and remaining significant after the introduction of mood variables. These preliminary analyses suggest that subjective sleep disturbance (low efficiency, short sleep) is common in FND. Sleep quality was negatively associated with the functional impairment attributed to FND, independent of depression. Therefore, sleep disturbance may be a clinically important feature of FND. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. Common Data Elements for Spinal Cord Injury Clinical Research: A National Institute for Neurological Disorders and Stroke Project

    Science.gov (United States)

    Biering-Sørensen, Fin; Alai, Sherita; Anderson, Kim; Charlifue, Susan; Chen, Yuying; DeVivo, Michael; Flanders, Adam E.; Jones, Linda; Kleitman, Naomi; Lans, Aria; Noonan, Vanessa K.; Odenkirchen, Joanne; Steeves, John; Tansey, Keith; Widerström-Noga, Eva; Jakeman, Lyn B.

    2015-01-01

    Objective To develop a comprehensive set of common data elements (CDEs), data definitions, case report forms and guidelines for use in spinal cord injury (SCI) clinical research, as part of the CDE project at the National Institute of Neurological Disorders and Stroke (NINDS) of the USA National Institutes of Health. Setting International Working Groups Methods Nine working groups composed of international experts reviewed existing CDEs and instruments, created new elements when needed, and provided recommendations for SCI clinical research. The project was carried out in collaboration with and cross-referenced to development of the International Spinal Cord Society (ISCoS) International SCI Data Sets. The recommendations were compiled, subjected to internal review, and posted online for external public comment. The final version was reviewed by all working groups and the NINDS CDE team prior to release. Results The NINDS SCI CDEs and supporting documents are publically available on the NINDS CDE website and the ISCoS website. The CDEs span the continuum of SCI care and the full range of domains of the International Classification of Functioning, Disability and Health. Conclusions Widespread use of common data elements can facilitate SCI clinical research and trial design, data sharing, and retrospective analyses. Continued international collaboration will enable consistent data collection and reporting, and will help ensure that the data elements are updated, reviewed and broadcast as additional evidence is obtained. PMID:25665542

  8. Reliability and validity of the Chinese version of the Neurological Disorders Depression Inventory for Epilepsy (C-NDDI-E).

    Science.gov (United States)

    Guo, Yi; Chen, Zhe-Meng; Zhang, Yin-Xi; Ge, Ye-Bo; Shen, Chun-Hong; Ding, Yao; Wang, Shuang; Tang, Ye-Lei; Ding, Mei-Ping

    2015-04-01

    The aim of this study was to evaluate the clinical reliability and validity of the Chinese version of the Neurological Disorders Depression Inventory for Epilepsy (C-NDDI-E). A total of 248 Chinese patients with epilepsy underwent psychometric tests, including the Chinese version of the Mini International Neuropsychiatric Interview (C-MINI), the Chinese version of the Beck Depression Inventory - II (C-BDI-II), and the C-NDDI-E. None of the patients had difficulties understanding or completing the C-NDDI-E. Cronbach's α coefficient was 0.824. At a cutoff score of ≥14, the C-NDDI-E had a sensitivity of 0.854, a specificity of 0.899, a positive predictive value of 0.625, and a negative predictive value of 0.969. The scores for the C-NDDI-E were positively correlated with those for the C-BDI-II (Pdepression in Chinese patients with epilepsy. Copyright © 2015 Elsevier Inc. All rights reserved.

  9. Antidepressants in Parkinson's disease. Recommendations by the movement disorder study group of the Neurological Association of Madrid.

    Science.gov (United States)

    Peña, E; Mata, M; López-Manzanares, L; Kurtis, M; Eimil, M; Martínez-Castrillo, J C; Navas, I; Posada, I J; Prieto, C; Ruíz-Huete, C; Vela, L; Venegas, B

    2016-03-19

    Although antidepressants are widely used in Parkinson's disease (PD), few well-designed studies to support their efficacy have been conducted. These clinical guidelines are based on a review of the literature and the results of an AMN movement disorder study group survey. Evidence suggests that nortriptyline, venlafaxine, paroxetine, and citalopram may be useful in treating depression in PD, although studies on paroxetine and citalopram yield conflicting results. In clinical practice, however, selective serotonin reuptake inhibitors are usually considered the treatment of choice. Duloxetine may be an alternative to venlafaxine, although the evidence for this is less, and venlafaxine plus mirtazapine may be useful in drug-resistant cases. Furthermore, citalopram may be indicated for the treatment of anxiety, atomoxetine for hypersomnia, trazodone and mirtazapine for insomnia and psychosis, and bupropion for apathy. In general, antidepressants are well tolerated in PD. However, clinicians should consider the anticholinergic effect of tricyclic antidepressants, the impact of serotonin-norepinephrine reuptake inhibitors on blood pressure, the extrapyramidal effects of antidepressants, and any potential interactions between monoamine oxidase B inhibitors and other antidepressants. Copyright © 2016 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.

  10. Exostectomy for chronic midfoot plantar ulcer in Charcot deformity

    DEFF Research Database (Denmark)

    Laurinaviciene, R.; Kirketerp-Moeller, K.; Holstein, Per Evald

    2008-01-01

    Charcot midfoot ulcers are rare and very difficult to heal, with surgery being an option. This retrospective study assessed healing rates, complications, and the incidence of re-ulceration and other foot ulcer problems following exostectomies Udgivelsesdato: 2008/2......Charcot midfoot ulcers are rare and very difficult to heal, with surgery being an option. This retrospective study assessed healing rates, complications, and the incidence of re-ulceration and other foot ulcer problems following exostectomies Udgivelsesdato: 2008/2...

  11. Antioxidant and anticholinesterase investigations of Rumex hastatus D. Don: potential effectiveness in oxidative stress and neurological disorders

    Directory of Open Access Journals (Sweden)

    Sajjad Ahmad

    2015-01-01

    Full Text Available BACKGROUND: Rumex species are traditionally used for the treatment of neurological disorders including headache, migraine, depression, paralysis etc. Several species have been scientifically validated for antioxidant and anticholinestrase potentials. This study aims to investigate Rumex hastatus D. Don crude methanolic extract, subsequent fractions, saponins and flavonoids for acetylcholinestrase, butyrylcholinestrase inhibition and diverse antioxidant activities to validate its folkloric uses in neurological disorders. Rumexhastatus crude methanolic extract (Rh. Cr, subsequent fractions; n-hexane (Rh. Hex, chloroform (Rh. Chf, ethyl acetate (Rh. EtAc, aqueous fraction (Rh. Aq, crude saponins (Rh. Sp and flavonoids (Rh. Fl were investigated against acetylcholinesterase (AChE and butyrylcholinesterase (BChE at various concentrations (125, 250, 500,1000 μg/mL using Ellman's spectrophotometric analysis. Antioxidant potentials of Rh. Sp and Rh. Fl were evaluated using DPPH, H2O2 and ABTS free radical scavenging assays at 62.5, 125, 250, 500, 1000 μg/mL. RESULTS: All the test samples showed concentration dependent cholinesterase inhibition and radicals scavenging activity. The AChE inhibition potential of Rh. Sp and Rh. Fl were most prominent i.e., 81.67 ± 0.88 and 91.62 ± 1.67 at highest concentration with IC50 135 and 20 μg/mL respectively. All the subsequent fractions exhibited moderate to high AChE inhibition i.e., Rh. Cr, Rh. Hex, Rh. Chf, Rh. EtAc and Rh. Aq showed IC50 218, 1420, 75, 115 and 1210 μg/mL respectively. Similarly, against BChE various plant extracts i.e., Rh. Sp, Rh. Fl, Rh. Cr, Rh. Hex, Rh. Chf, Rh. EtAc and Rh. Aq resulted IC50 165,175, 265, 890, 92, 115 and 220 μg/mL respectively. In DPPH free radical scavenging assay, Rh. Sp and Rh. Fl showed comparable results with the positive control i.e., 63.34 ± 0.98 and 76.93 ± 1.13% scavenging at 1 mg/mL concentration (IC50 312 and 104 μg/mL respectively. The percent ABTS

  12. Neurologic Complications of Celiac Disease

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2004-06-01

    Full Text Available Patients with celiac disease (CD [n=l 11] and controls (n=211 were questioned regarding neurologic disorders, their charts were reviewed, and they received neurologic evaluations, including brain imaging or EEG if indicated, in a study of neurologic complications of CD at Carmel Medical Center, Technion-Israel Institute of Technology, Haifa, Israel.

  13. Bone mineral density in diabetes mellitus patients with and without a Charcot foot

    DEFF Research Database (Denmark)

    Christensen, Tomas M; Bülow, Jens; Simonsen, Lene

    2010-01-01

    To measure bone mineral density in patients with diabetes mellitus and the complication Charcot osteoarthropathy (CA).......To measure bone mineral density in patients with diabetes mellitus and the complication Charcot osteoarthropathy (CA)....

  14. Neurologic complications of vaccinations.

    Science.gov (United States)

    Miravalle, Augusto A; Schreiner, Teri

    2014-01-01

    This chapter reviews the most common neurologic disorders associated with common vaccines, evaluates the data linking the disorder with the vaccine, and discusses the potential mechanism of disease. A literature search was conducted in PubMed using a combination of the following terms: vaccines, vaccination, immunization, and neurologic complications. Data were also gathered from publications of the American Academy of Pediatrics Committee on Infectious Diseases, the World Health Organization, the US Centers for Disease Control and Prevention, and the Vaccine Adverse Event Reporting System. Neurologic complications of vaccination are rare. Many associations have been asserted without objective data to support a causal relationship. Rarely, patients with a neurologic complication will have a poor outcome. However, most patients recover fully from the neurologic complication. Vaccinations have altered the landscape of infectious disease. However, perception of risk associated with vaccinations has limited the success of disease eradication measures. Neurologic complications can be severe, and can provoke fear in potential vaccines. Evaluating whether there is causal link between neurologic disorders and vaccinations, not just temporal association, is critical to addressing public misperception of risk of vaccination. Among the vaccines available today, the cost-benefit analysis of vaccinations and complications strongly argues in favor of vaccination. © 2014 Elsevier B.V. All rights reserved.

  15. Peroxisomal disorders in neurology

    NARCIS (Netherlands)

    Wanders, R. J.; Heymans, H. S.; Schutgens, R. B.; Barth, P. G.; van den Bosch, H.; Tager, J. M.

    1988-01-01

    Although peroxisomes were initially believed to play only a minor role in mammalian metabolism, it is now clear that they catalyse essential reactions in a number of different metabolic pathways and thus play an indispensable role in intermediary metabolism. The metabolic pathways in which

  16. How music training enhances working memory: a cerebrocerebellar blending mechanism that can lead equally to scientific discovery and therapeutic efficacy in neurological disorders

    OpenAIRE

    Vandervert, Larry

    2015-01-01

    Background Following in the vein of studies that concluded that music training resulted in plastic changes in Einstein’s cerebral cortex, controlled research has shown that music training (1) enhances central executive attentional processes in working memory, and (2) has also been shown to be of significant therapeutic value in neurological disorders. Within this framework of music training-induced enhancement of central executive attentional processes, the purpose of this article is to argue...

  17. P2X7 Receptors as a Transducer in the Co-Occurrence of Neurological/Psychiatric and Cardiovascular Disorders: A Hypothesis

    Directory of Open Access Journals (Sweden)

    Stephen D. Skaper

    2009-01-01

    Full Text Available Background. Over-stimulation of the purinergic P2X7 receptor may bring about cellular dysfunction and injury in settings of neurodegeneration, chronic inflammation, as well as in psychiatric and cardiovascular diseases. Here we speculate how P2X7 receptor over-activation may lead to the co-occurrence of neurological and psychiatric disorders with cardiovascular disorders. Presentation. We hypothesize that proinflammatory cytokines, in particular interleukin-1, are key players in the pathophysiology of neurological, psychiatric, and cardiovascular diseases. Critically, this premise is based on a role for the P2X7 receptor in triggering a rise in these cytokines. Given the broad distribution of P2X7 receptors in nervous, immune, and vascular tissue cells, this receptor is proposed as central in linking the nervous, immune, and cardiovascular systems. Testing. Investigate, retrospectively, whether a bidirectional link can be established between illnesses with a proinflammatory component (e.g., inflammatory and chronic neuropathic pain and cardiovascular disease, for example, hypertension, and whether patients treated with anti-inflammatory drugs have a lower incidence of disease complications. Positive outcome would indicate a prospective study to evaluate therapeutic efficacy of P2X7 receptor antagonists. Implications. It should be stressed that sufficient direct evidence does not exist at present supporting our hypothesis. However, a positive outcome would encourage the further development of P2X7 receptor antagonists and their application to limit the co-occurrence of neurological, psychiatric, and cardiovascular disorders.

  18. Suicide in Neurologic Illness.

    Science.gov (United States)

    Arciniegas, David B.; Anderson, C. Alan

    2002-11-01

    The risk of attempted or completed suicide is increased in patients with migraine with aura, epilepsy, stroke, multiple sclerosis, traumatic brain injury, and Huntington's disease. Contrary to the general perception that the risk of suicide among patients with Alzheimer's disease and other dementing conditions is low, several reports suggest that the risk of suicide in these patients increases relative to the general population. Some patients at risk for neurologic disorders are also at increased risk for suicide; in particular, the risk of suicide is increased among persons at risk for Huntington's disease, independent of the presence or absence of the Huntington's gene mutation. The risk of attempted or completed suicide in neurologic illness is strongly associated with depression, feelings of hopelessness or helplessness, and social isolation. Additional suicide risk factors in persons with neurologic illness include cognitive impairment, relatively younger age (under 60 years), moderate physical disability, recent onset or change in illness, a lack of future plans or perceived meaning in life, recent losses (personal, occupational, or financial), and prior history of psychiatric illness or suicidal behavior. Substance dependence, psychotic disorders, anxiety disorders, and some personality disorders (eg, borderline personality disorder) may also contribute to increased risk of suicide among persons with neurologic illnesses. Identification and aggressive treatment of psychiatric problems, especially depression, as well as reduction of modifiable suicide risk factors among patients with neurologic illness is needed to reduce the risk of attempted and completed suicide in this population.

  19. Charcot-Marie-Tooth disease: The development of a diagnostic platform using next generation sequencing

    DEFF Research Database (Denmark)

    Christensen, Rikke; Væth, Signe; Thorsen, Kasper

    Background: Charcot-Marie-Tooth Disease (CMT) is one of the most common inherited neurological diseases. Today, more than 70 CMT related genes are known to cause inherited neuropathy. The diagnostic strategy in most laboratories is based on Sanger-sequencing of few genes. In our patient cohort......, Sanger sequencing of 4 genes have led to a diagnosis in approximately 30% of the patients. Aims: 1) Development of a targeted NGS platform containing 63 genes that currently are found to be associated with CMT. 2) Analysis of the increased diagnostic yield using this platform to analyze 200 CMT samples...... previously analyzed using Sanger sequencing without identification of a disease causing mutation. Materials and Methods: Libraries for 200 patient samples obtained for CMT diagnostics were prepared using Illumina Truseq and target enrichment using SeqCap EZ Choise Library (Nimblegen). The libraries were...

  20. A role for myosin Va in cerebellar plasticity and motor learning: a possible mechanism underlying neurological disorder in myosin Va disease.

    Science.gov (United States)

    Miyata, Mariko; Kishimoto, Yasushi; Tanaka, Masahiko; Hashimoto, Kouichi; Hirashima, Naohide; Murata, Yoshiharu; Kano, Masanobu; Takagishi, Yoshiko

    2011-04-20

    Mutations of the myosin Va gene cause the neurological diseases Griscelli syndrome type 1 and Elejalde syndrome in humans and dilute phenotypes in rodents. To understand the pathophysiological mechanisms underlying the neurological disorders in myosin Va diseases, we conducted an integrated analysis at the molecular, cellular, electrophysiological, and behavioral levels using the dilute-neurological (d-n) mouse mutant. These mice manifest an ataxic gait and clonic seizures during postnatal development, but the neurological disorders are ameliorated in adulthood. We found that smooth endoplasmic reticulum (SER) rarely extended into the dendritic spines of Purkinje cells (PCs) of young d-n mice, and there were few, if any, IP(3) receptors. Moreover, long-term depression (LTD) at parallel fiber-PC synapses was abolished, consistent with our previous observations in juvenile lethal dilute mutants. Young d-n mice exhibited severe impairment of cerebellum-dependent motor learning. In contrast, adult d-n mice showed restoration of motor learning and LTD, and these neurological changes were associated with accumulation of SER and IP(3) receptors in some PC spines and the expression of myosin Va proteins in the PCs. RNA interference-mediated repression of myosin Va caused a reduction in the number of IP(3) receptor-positive spines in cultured PCs. These findings indicate that myosin Va function is critical for subsequent processes in localization of SER and IP(3) receptors in PC spines, LTD, and motor learning. Interestingly, d-n mice had defects of motor coordination from young to adult ages, suggesting that the role of myosin Va in PC spines is not sufficient for motor coordination.

  1. Post-marketing observational program of the effectiveness of fluvoxamine for the treatment of depression in patients with neurological disorders: the FRIENDS study

    Directory of Open Access Journals (Sweden)

    Yahno NN

    2017-11-01

    Full Text Available Nikolay N Yahno,1 Anastasia V Fedotova2 1Neurology Department, I.M. Sechenov First Moscow State Medical University, 2Neurology Department, Additional Professional Education Faculty, Pirogov Russian National Research Medical University, Moscow, Russian Federation Abstract: In a prospective, non-blinded, uncontrolled, multicenter, post-marketing, observational study (FRIENDS; NCT02043197, fluvoxamine (50–300 mg/day for 90 days was effective for the treatment of depression in 299 adult patients (age ≥18 years with neurological disorders at baseline. The therapeutic effect of fluvoxamine was measured by means of changes in the Hospital Anxiety and Depression Scale depression and anxiety scores (HADS-D and HADS-A, respectively, global severity of illness, and clinical condition (measured using the Clinical Global Improvement [CGI] scale. The mean HADS-D subscale score at baseline in the per-protocol cohort (n=296 was 11.7±3.1 points and the corresponding mean HADS-A score was 12.6±3.2. Significant (P<0.0001 improvements in both scores were recorded during fluvoxamine treatment and later follow-up. Most patients (>85% recorded reductions versus baseline in both indices. In the CGI-based assessment, most evaluated patients (>200 experienced moderate to very substantial clinical improvement, with no or limited side effects. Significant improvements were also recorded in the exploratory outcomes of sleep quality, assessed using the Insomnia Severity Index, and cognitive function, assessed using the Montreal Cognitive Assessment (P<0.0001 vs baseline for both. No death or serious adverse drug reactions were reported during the study. The results of this observational study affirm that fluvoxamine is effective and well tolerated for the treatment of depression in the context of neurological disorders. The effects on the exploratory endpoints of this research merit evaluation in controlled trials. Keywords: depression, anxiety, fluvoxamine

  2. Potential of robots as next-generation technology for clinical assessment of neurological disorders and upper-limb therapy

    OpenAIRE

    Stephen H. Scott, PhD; Sean P. Dukelow, MD, PhD

    2011-01-01

    Robotic technologies have profoundly affected the identification of fundamental properties of brain function. This success is attributable to robots being able to control the position of or forces applied to limbs, and their inherent ability to easily, objectively, and reliably quantify sensorimotor behavior. Our general hypothesis is that these same attributes make robotic technologies ideal for clinically assessing sensory, motor, and cognitive impairments in stroke and other neurologi-cal ...

  3. Is shoulder arthroplasty an option for charcot arthropathy?

    Science.gov (United States)

    Schoch, Bradley; Werthel, Jean-David; Sperling, John W; Cofield, Robert H; Sanchez-Sotelo, Joaquin

    2016-12-01

    Charcot arthropathy is a rare cause of debilitating joint destruction. Shoulder arthroplasty for Charcot arthropathy is challenging secondary to local bone and soft tissue loss, lack of protective sensation, and altered muscle control. The purpose of this study is to review the outcomes, complications, and survivorship of shoulder arthroplasty for Charcot arthropathy. Between January 2000 and December 2011, ten shoulders with Charcot arthropathy were treated with shoulder arthroplasty at our Institution (six hemiarthroplasty, one total shoulder arthroplasty, three reverse shoulder arthroplasty). Shoulders were followed for a minimum of two years or until re-operation. Outcomes measures included pain, range of motion, complications, and reoperation. Shoulder arthroplasty improved pain to a mean score of 2.6 at follow up. However, gains in range of motion were not as substantial, with mean elevation of only 105°. External rotation improved from 20 to 43°. Subjectively, six of the ten patients rated the result as much better or better. Two shoulders underwent revision surgery at an average of five months after index arthroplasty. Shoulder arthroplasty for the treatment of the sequelae of a Charcot joint is a reasonable treatment option to provide substantial pain relief and improved motion. The relative indications of hemiarthroplasty, total shoulder arthroplasty, and reverse shoulder arthroplasty for this particular condition continue to evolve.

  4. Charcot spinal arthropathy in a paraplegic weight lifter: case report.

    Science.gov (United States)

    Rose, David M; Hilton, Andrew I; Tucker, Stewart K

    2006-05-15

    A case report of aggressive multilevel Charcot spinal arthropathy treated with staged spinal instrumentation. To report an unusual case of Charcot spinal arthropathy, given the rapidity of progression and extent of tissue destruction, and present the results of successful spinal instrumentation and stabilization. Charcot spinal arthropathy in the long-standing paraplegic patient is more commonly seen in those who have undergone prior spinal surgery and is usually restricted to 2 spinal levels. A 36-year-old amateur weight lifter with T6 complete paraplegia presented with lower thoracic back pain, a kyphotic deformity of the thoracolumbar region, and gross spinal instability on transferring. Imaging revealed extensive bony destruction from T10-T12 and complete absence of spinal tissue over the affected levels. Staged anterior and posterior spinal instrumentation from T3 to L4 was performed. Spinal stabilization was achieved, and the patient was pain free and able to resume light training at 6-month follow-up. We would advise a high index of suspicion of Charcot arthropathy in the active paraplegic patient presenting with back pain caudal to their sensory level. Staged spinal instrumentation is an effective treatment for multilevel Charcot spinal arthropathy.

  5. Chapter 30: historical aspects of the major neurological vitamin deficiency disorders: the water-soluble B vitamins.

    Science.gov (United States)

    Lanska, Douglas J

    2010-01-01

    This historical review addresses major neurological disorders associated with deficiencies of water-soluble B vitamins: beriberi, Wernicke-Korsakoff syndrome, pellagra, neural tube defects, and subacute combined degeneration of the spinal cord. Beriberi: Beriberi was known for millennia in Asia, but was not described by a European until the 17th century when Brontius in the Dutch East Indies reported the progressive sensorimotor polyneuropathy. The prevalence of beriberi increased greatly in Asia with a change in the milling process for rice in the late 19th century. In the 1880s, Takaki demonstrated the benefits of dietary modification in sailors, and later instituted dietary reforms in the Japanese Navy, which largely eradicated beriberi from the Japanese Navy by 1887. In 1889 Eijkman in Java serendipitously identified dietary factors as a major contributor to "chicken polyneuritis," which he took to be an animal model for beriberi; the polyneuritis could be cured or prevented by feeding the chickens either unpolished rice or rice polishings. By 1901, Grijns, while continuing studies of beriberi in Java, suggested a dietary deficiency explanation for beriberi after systematically eliminating deficiencies of known dietary components and excluding a toxic effect. Wernicke-Korsakoff syndrome: In the late 1870s, Wernicke identified a clinicopathological condition with ophthalmoparesis, nystagmus, ataxia, and encephalopathy, associated with punctate hemorrhages symmetrically arranged in the grey matter around the third and fourth ventricles and the aqueduct of Sylvius. In the late 1880s, Korsakoff described a spectrum of cognitive disorders, including a confabulatory amnestic state following an agitated delirium, occurring in conjunction with peripheral polyneuropathy. Beginning around 1900, investigators recognized the close relationship between Korsakoff's psychosis, delirium tremens, and Wernicke's encephalopathy, but not until several decades later were Wernicke

  6. How music training enhances working memory: a cerebrocerebellar blending mechanism that can lead equally to scientific discovery and therapeutic efficacy in neurological disorders.

    Science.gov (United States)

    Vandervert, Larry

    2015-01-01

    Following in the vein of studies that concluded that music training resulted in plastic changes in Einstein's cerebral cortex, controlled research has shown that music training (1) enhances central executive attentional processes in working memory, and (2) has also been shown to be of significant therapeutic value in neurological disorders. Within this framework of music training-induced enhancement of central executive attentional processes, the purpose of this article is to argue that: (1) The foundational basis of the central executive begins in infancy as attentional control during the establishment of working memory, (2) In accordance with Akshoomoff, Courchesne and Townsend's and Leggio and Molinari's cerebellar sequence detection and prediction models, the rigors of volitional control demands of music training can enhance voluntary manipulation of information in thought and movement, (3) The music training-enhanced blending of cerebellar internal models in working memory as can be experienced as intuition in scientific discovery (as Einstein often indicated) or, equally, as moments of therapeutic advancement toward goals in the development of voluntary control in neurological disorders, and (4) The blending of internal models as in (3) thus provides a mechanism by which music training enhances central executive processes in working memory that can lead to scientific discovery and improved therapeutic outcomes in neurological disorders. Within the framework of Leggio and Molinari's cerebellar sequence detection model, it is determined that intuitive steps forward that occur in both scientific discovery and during therapy in those with neurological disorders operate according to the same mechanism of adaptive error-driven blending of cerebellar internal models. It is concluded that the entire framework of the central executive structure of working memory is a product of the cerebrocerebellar system which can, through the learning of internal models

  7. Lower-extremity amputation risk after charcot arthropathy and diabetic foot ulcer.

    Science.gov (United States)

    Sohn, Min-Woong; Stuck, Rodney M; Pinzur, Michael; Lee, Todd A; Budiman-Mak, Elly

    2010-01-01

    To compare risks of lower-extremity amputation between patients with Charcot arthropathy and those with diabetic foot ulcers. A retrospective cohort of patients with incident Charcot arthropathy or diabetic foot ulcers in 2003 was followed for 5 years for any major and minor amputations in the lower extremities. After a mean follow-up of 37 +/- 20 and 43 +/- 18 months, the Charcot and ulcer groups had 4.1 and 4.7 amputations per 100 person-years, respectively. Among patients Charcot alone was 7 times higher for patients with ulcer alone and 12 times higher for patients with Charcot and ulcer. Charcot arthropathy by itself does not pose a serious amputation risk, but ulcer complication multiplicatively increases the risk. Early surgical intervention for Charcot patients in the absence of deformity or ulceration may not be advisable.

  8. Neurologic Diseases and Sleep.

    Science.gov (United States)

    Barone, Daniel A; Chokroverty, Sudansu

    2017-03-01

    Sleep disorders and neurologic illness are common and burdensome in their own right; when combined, they can have tremendous negative impact at an individual level as well as societally. The socioeconomic burden of sleep disorders and neurologic illness can be identified, but the real cost of these conditions lies far beyond the financial realm. There is an urgent need for comprehensive care and support systems to help with the burden of disease. Further research in improving patient outcomes in those who suffer with these conditions will help patients and their families, and society in general. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Perioperative Management of Neurological Conditions

    Directory of Open Access Journals (Sweden)

    Manjeet Singh Dhallu

    2017-06-01

    Full Text Available Perioperative care of the patients with neurological diseases can be challenging. Most important consideration is the management and understanding of pathophysiology of these disorders and evaluation of new neurological changes that occur perioperatively. Perioperative generally refers to 3 phases of surgery: preoperative, intraoperative, and postoperative. We have tried to address few commonly encountered neurological conditions in clinical practice, such as delirium, stroke, epilepsy, myasthenia gravis, and Parkinson disease. In this article, we emphasize on early diagnosis and management strategies of neurological disorders in the perioperative period to minimize morbidity and mortality of patients.

  10. Charcot-Leyden crystals in a prostatic adenocarcinoma.

    Science.gov (United States)

    Aydin, Ozgür

    2006-09-07

    A transrectal needle biopsy from a 63-year-old man was decided because of a high prostatic specific antigen in the blood, and a hard right lobe in rectal examination. 10 examples were taken from each lobe. In 1 of 4 of the examples from the left lobe, which contained a small focus of adenocarcinoma, numerous eosinophils were observed to surround the carcinomatous focus and attack the carcinoma cells. Uniquely, at the same focus Charcot-Leyden crystals could be seen in the intraluminal space and stromal area. A carcinoma oriented eosinophil accumulation, and Charcot-Leyden crystals in prostate was not described before.

  11. Charcot-Leyden crystals in a prostatic adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Aydın Özgür

    2006-09-01

    Full Text Available Abstract A transrectal needle biopsy from a 63-year-old man was decided because of a high prostatic spesific antigen in the blood, and a hard right lobe in rectal examination. 10 examples were taken from each lobe. In 1 of 4 of the examples from the left lobe, which contained a small focus of adenocarcinoma, numerous eosinophils were observed to surround the carcinomatous focus and attack the carcinoma cells. Uniquely, at the same focus Charcot-Leyden crystals could be seen in the intraluminal space and stromal area. A carcinoma oriented eosinophil accumulation, and Charcot-Leyden crystals in prostate was not described before.

  12. Functional Impairment of Patients Undergoing Surgical Correction for Charcot Foot Arthropathy.

    Science.gov (United States)

    Kroin, Ellen; Schiff, Adam; Pinzur, Michael S; Davis, Elissa S; Chaharbakhshi, Edwin; DiSilvio, Frank A

    2017-07-01

    Investigations using the Medical Outcomes Study Short Form 36 Healthy Survey (SF-36) and the American Orthopaedic Foot & Ankle Society Diabetic Foot Questionnaire (AOFAS-DFQ) have demonstrated a poor quality of life in patients with Charcot foot arthropathy. The Short Musculoskeletal Function Assessment (SMFA) questionnaire has been widely used in patients with a broad range of musculoskeletal disorders. Twenty-five consecutive patients undergoing operative correction for diabetes-related Charcot foot arthropathy of the midfoot completed the SMFA prior to undergoing surgery. There were 16 males and 9 females. The average body mass index (BMI) was 37.4 (range 25.8-50.2), and the average hemoglobin A1c was 7.5 (range 5.3-10.1) prior to surgery. All 25 patients exhibited significant impairment in all 6 domains of the SMFA ( P foot severely impaired the quality of life in patients beyond the impact of morbid obesity. This impairment equally impacted all of the functional and emotional domains measured with the SMFA as compared with population norms. This investigation provides a benchmark for measuring the impact of operative correction of the deformity. In addition, the SMFA appears to be a valid tool for measuring impairment in this complex patient population. Level II, prospective comparative investigation.

  13. Mutation analysis of PMP22 in Slovak patients with Charcot-Marie-Tooth disease and hereditary neuropathy with liability to pressure palsies.

    Science.gov (United States)

    Resko, Peter; Radvansky, Jan; Odnogova, Zuzana; Baldovic, Marian; Minarik, Gabriel; Polakova, Helena; Palffy, Roland; Kadasi, Ludevit

    2011-12-01

    Charcot-Marie-Tooth disease (CMT) and related peripheral neuropathies are the most commonly inherited neurological disorders in humans, characterized by clinical and genetic heterogeneity. The most prevalent clinical entities belonging to this group of disorders are CMT type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP). CMT1A and HNPP are predominantly caused by a 1.5 Mb duplication and deletion in the chromosomal region 17p11.2, respectively, and less frequently by other mutations in the peripheral myelin protein 22 (PMP22) gene. Despite being relatively common diseases, they haven't been previously studied in the Slovak population. Therefore, the aim of this study was to identify the spectrum and frequency of PMP22 mutations in the Slovak population by screening 119 families with CMT and 2 families with HNPP for causative mutations in this gene. The copy number determination of PMP22 resulted in the detection of CMT1A duplication in 40 families and the detection of HNPP deletion in 7 families, 6 of which were originally diagnosed as CMT. Consequent mutation screening of families without duplication or deletion using dHPLC and sequencing identified 6 single base changes (3 unpublished to date), from which only c.327C>A (Cys109X) present in one family was provably causative. These results confirm the leading role of PMP22 mutation analysis in the differential diagnosis of CMT and show that the spectrum and frequency of PMP22 mutations in the Slovak population is comparable to that seen in the global population.

  14. Toxoplasmosis and Polygenic Disease Susceptibility Genes: Extensive Toxoplasma gondii Host/Pathogen Interactome Enrichment in Nine Psychiatric or Neurological Disorders

    OpenAIRE

    C. J. Carter

    2013-01-01

    Toxoplasma gondii is not only implicated in schizophrenia and related disorders, but also in Alzheimer's or Parkinson's disease, cancer, cardiac myopathies, and autoimmune disorders. During its life cycle, the pathogen interacts with ~3000 host genes or proteins. Susceptibility genes for multiple sclerosis, Alzheimer's disease, schizophrenia, bipolar disorder, depression, childhood obesity, Parkinson's disease, attention deficit hyperactivity disorder (P??from??8.01E ? 05??(ADHD)??to??1.22E ?...

  15. Posttraumatic Charcot (Neuropathic) Spinal Arthropathy at the Cervicothoracic Junction.

    Science.gov (United States)

    Aydinli, Ufuk; Mohan, Nilesh Kumar; Kara, Kursat

    2016-10-01

    Charcot spine arthropathy (posttraumatic neuroarthropathy of the spine) has been reported to be a very late and rare complication of spinal cord injury. Charcot of the cervicothoracic and upper thoracic region rarely is reported in the literature. Charcot spinal arthropathy is a cause of progressive deformity and may present as late as 30 years after the original spinal cord injury. This is more common in paraplegic patients who are actively ambulating. A 56-year-old patient with complete paraplegia for approximately 20 years after spinal cord injury presented with severe kyphous deformity and instability of thoracolumbar spine. His sensory level to deep pain was at thoracic (D4). He kept developing new neuroarthropathies at different segments within a span of 5-6 months after every decompression and fusion with anterior cage and posterior instrumentation done. A total of 3 surgeries had been done in span of 2 years, initially thoracic, then lumbar and finally cervicothoracic junction. We present this case because of the challenges in surgery for instrumentation of new Charcot spinal arthropathy. Reports of neuroarthropathy developing above the level of spinal cord injury and at the cervicothoracic junction are rare. The treating surgeon should be cognizant of the possibility of developing secondary levels of neuroarthropathy above and below a previously successful fusion. Copyright © 2016 Elsevier Inc. All rights reserved.

  16. Hybrid Fixation for Ankle Fusion in Diabetic Charcot Arthropathy.

    Science.gov (United States)

    El-Mowafi, Hani; Abulsaad, Mazen; Kandil, Yasser; El-Hawary, Ahmed; Ali, Samer

    2017-10-01

    Ankle fusion is difficult to achieve in the diabetic Charcot ankle Brodsky type 3a because of the poor quality of the bone and the inability to achieve a stable biomechanical construct. The aim of this study was to report the outcome of ankle fusion using a combination of an intramedullary nail and a circular external fixator in patients with diabetic Charcot arthropathy. We prospectively studied 24 patients with diabetic Charcot arthropathy of the ankle who were treated by fusion of the tibiotalar joint using a combined retrograde intramedullary nail and Ilizarov external fixator. Their mean age was 50.7 ± 6.9 (range, 43-62) years. The mean follow-up after surgery was 36.4 ± 5.8 (range, 24-98) months. Twenty-two patients (92%) achieved clinical and radiographic solid bony fusion. No patients in this series needed amputation. All the patients were pain free, and the mean American Orthopaedic Foot and Ankle Society Score (AOFAS) improved significantly from 34.6 ± 6.8 to 66.4 ± 4.5 at the last follow-up. Two patients developed an ulcer over the heel due to a prominent nail. The ulcer healed after nail removal. Eight patients developed pin tract infection. We report a successful outcomes of ankle fusions using combined intramedullary nail locked only proximally and ring external fixator (hybrid fixation) in patients with diabetic Charcot arthropathy. Level IV, case series.

  17. Jean-Baptiste Charcot, the French Antarctic expedition and scurvy

    Directory of Open Access Journals (Sweden)

    Hélio Afonso Ghizoni Teive

    2014-07-01

    Full Text Available During the second expedition to the South Pole, Commander Jean-Baptiste Charcot and some members of the crew of “Pourquoi Pas?” developed symptoms suggestive of scurvy. The clinical picture was totally reversed after dietary changes.

  18. Charcot-Marie-Tooth disease type 1A

    DEFF Research Database (Denmark)

    Hertz, Jens Michael; Børglum, A D; Brandt, C A

    1994-01-01

    Charcot-Marie-Tooth disease type 1A (CMT1A) is an autosomal dominant peripheral neuropathy associated with a DNA duplication on chromosome 17p11.2-p12 in the majority of cases. Most of the sporadic cases are due to a de novo duplication. We have screened for this duplication in 11 Danish patients...

  19. Circulating Charcot-Leyden crystals in the hypereosinophilic syndrome.

    Science.gov (United States)

    Dincsoy, H P; Burton, T J; van der Bel-Kahn, J M

    1981-02-01

    A patient who had malignant melanoma associated with the hypereosinophilic syndrome died of cardiovascular thrombotic lesions. Widespread tissue eosinophilia was accompanied by numerous Charcot-Leyden crystals in the tumor as well as in various organs, including the renal tubules. A unique observation, not previously described, is the finding of Charcot-Leyden crystals in the thrombi and vessels, including the renal glomeruli. While little is known of the significance of Charcot-Leyden crystals, it is speculated that the circulating crystals injure the endothelium and trigger intravascular coagulation, resulting in thrombosis. A search for the crystals in blood and/or urine may be of additional aid in the evaluation of the extent of the thrombotic process. A special staining method is proposed to facilitate recognition of the crystals, since these are virtually not visualized by routine stain. With the use of such a staining method, future observations in other cases of the hypereosinophilic syndrome may elucidate the role of Charcot-Leyden crystals in the pathogenesis of the thrombotic cardiovascular lesions of this syndrome.

  20. Charcot-Leyden crystals within a periapical lesion.

    Science.gov (United States)

    Silver, G K; Simon, J H

    2000-11-01

    Transmission electron microscopy revealed the presence of Charcot-Leyden crystals within a periapical lesion, which was assessed histopathologically as consistent with a periapical granuloma that failed to resolve after conventional endodontic treatment. This paper presents the clinical, radiographic, histological, and ultrastructural findings of this case and discusses their potential clinical significance.

  1. [Neurology and literature].

    Science.gov (United States)

    Iniesta, I

    2010-10-01

    Literature complements medical literature in the academic and clinical development of neurologists. The present article explores the contributions of writers of fiction on neurology. Literary works of fiction with particular reference to neurology. A symbiosis between writers of fiction and doctors has been well recognised. From Shakespeare to Cervantes by way of Dickens and Cela to writer - physicians such as Anton Chekhov or António Lobo Antunes have contributed through their medically informed literature to the better understanding of neurology. Some writers like Dostoevsky, Machado de Assis and Margiad Evans have written about their own experiences with disease thus bringing new insights to medicine. Furthermore, some neurological disorders have been largely based on literary descriptions. For instance, Dostoevsky's epilepsy has been retrospectively analysed by famous neurologists including Freud, Alajouanine or Gastaut, whilst his writings and biography have prompted others like Waxman and Geschwind to describe typical behavioural changes in temporal lobe epilepsy, finding their source of inspiration in Dostoevsky. Likewise, Cirignotta et al have named an unusual type of seizure after the Russian novelist. Inspired by Lewis Carroll, Todd introduced the term Alice in Wonderland Syndrome to refer to visual distortions generally associated with migraine. Writers of fiction offer a humanised perception of disease by contributing new insights into the clinical history, informing about the subjective experience of the illness and helping to eradicate the stigma associated to neurological disorders.

  2. Linking of the quality of life in neurological disorders (Neuro-QoL) to the international classification of functioning, disability and health.

    Science.gov (United States)

    Wong, Alex W K; Lau, Stephen C L; Cella, David; Lai, Jin-Shei; Xie, Guanli; Chen, Lidian; Chan, Chetwyn C H; Heinemann, Allen W

    2017-05-05

    The quality of life in neurological disorders (Neuro-QoL) is a U.S. National Institutes of Health initiative that produced a set of self-report measures of physical, mental, and social health experienced by adults or children who have a neurological condition or disorder. To describe the content of the Neuro-QoL at the item level using the World Health Organization's international classification of functioning, disability and health (ICF). We assessed the Neuro-QoL for its content coverage of functioning and disability relative to each of the four ICF domains (i.e., body functions, body structures, activities and participation, and environment). We used second-level ICF three-digit codes to classify items into categories within each ICF domain and computed the percentage of categories within each ICF domain that were represented in the Neuro-QoL items. All items of Neuro-QoL could be mapped to the ICF categories at the second-level classification codes. The activities and participation domain and the mental functions category of the body functions domain were the areas most often represented by Neuro-QoL. Neuro-QoL provides limited coverage of the environmental factors and body structure domains. Neuro-QoL measures map well to the ICF. The Neuro-QoL-ICF-mapped items provide a blueprint for users to select appropriate measures in ICF-based measurement applications.

  3. CNS penetration of intrathecal-lumbar idursulfase in the monkey, dog and mouse: implications for neurological outcomes of lysosomal storage disorder.

    Science.gov (United States)

    Calias, Pericles; Papisov, Mikhail; Pan, Jing; Savioli, Nancy; Belov, Vasily; Huang, Yan; Lotterhand, Jason; Alessandrini, Mary; Liu, Nan; Fischman, Alan J; Powell, Jan L; Heartlein, Michael W

    2012-01-01

    A major challenge for the treatment of many central nervous system (CNS) disorders is the lack of convenient and effective methods for delivering biological agents to the brain. Mucopolysaccharidosis II (Hunter syndrome) is a rare inherited lysosomal storage disorder resulting from a deficiency of iduronate-2-sulfatase (I2S). I2S is a large, highly glycosylated enzyme. Intravenous administration is not likely to be an effective therapy for disease-related neurological outcomes that require enzyme access to the brain cells, in particular neurons and oligodendrocytes. We demonstrate that intracerebroventricular and lumbar intrathecal administration of recombinant I2S in dogs and nonhuman primates resulted in widespread enzyme distribution in the brain parenchyma, including remarkable deposition in the lysosomes of both neurons and oligodendrocytes. Lumbar intrathecal administration also resulted in enzyme delivery to the spinal cord, whereas little enzyme was detected there after intraventricular administration. Mucopolysaccharidosis II model is available in mice. Lumbar administration of recombinant I2S to enzyme deficient animals reduced the storage of glycosaminoglycans in both superficial and deep brain tissues, with concurrent morphological improvements. The observed patterns of enzyme transport from cerebrospinal fluid to the CNS tissues and the resultant biological activity (a) warrant further investigation of intrathecal delivery of I2S via lumbar catheter as an experimental treatment for the neurological symptoms of Hunter syndrome and (b) may have broader implications for CNS treatment with biopharmaceuticals.

  4. CNS penetration of intrathecal-lumbar idursulfase in the monkey, dog and mouse: implications for neurological outcomes of lysosomal storage disorder.

    Directory of Open Access Journals (Sweden)

    Pericles Calias

    Full Text Available A major challenge for the treatment of many central nervous system (CNS disorders is the lack of convenient and effective methods for delivering biological agents to the brain. Mucopolysaccharidosis II (Hunter syndrome is a rare inherited lysosomal storage disorder resulting from a deficiency of iduronate-2-sulfatase (I2S. I2S is a large, highly glycosylated enzyme. Intravenous administration is not likely to be an effective therapy for disease-related neurological outcomes that require enzyme access to the brain cells, in particular neurons and oligodendrocytes. We demonstrate that intracerebroventricular and lumbar intrathecal administration of recombinant I2S in dogs and nonhuman primates resulted in widespread enzyme distribution in the brain parenchyma, including remarkable deposition in the lysosomes of both neurons and oligodendrocytes. Lumbar intrathecal administration also resulted in enzyme delivery to the spinal cord, whereas little enzyme was detected there after intraventricular administration. Mucopolysaccharidosis II model is available in mice. Lumbar administration of recombinant I2S to enzyme deficient animals reduced the storage of glycosaminoglycans in both superficial and deep brain tissues, with concurrent morphological improvements. The observed patterns of enzyme transport from cerebrospinal fluid to the CNS tissues and the resultant biological activity (a warrant further investigation of intrathecal delivery of I2S via lumbar catheter as an experimental treatment for the neurological symptoms of Hunter syndrome and (b may have broader implications for CNS treatment with biopharmaceuticals.

  5. Neurologic manifestations of hypothyroidism in dogs.

    Science.gov (United States)

    Bertalan, Abigail; Kent, Marc; Glass, Eric

    2013-03-01

    Hypothyroidism is a common endocrine disease in dogs. A variety of clinicopathologic abnormalities may be present; however, neurologic deficits are rare. In some instances, neurologic deficits may be the sole manifestation of hypothyroidism. Consequent ly, the diagnosis and management of the neurologic disorders associated with hypothyroidism can be challenging. This article describes several neurologic manifestations of primary hypothyroidism in dogs; discusses the pathophysiology of hypothyroidism-induced neurologic disorders affecting the peripheral and central nervous systems; and reviews the evidence for the neurologic effects of hypothyroidism.

  6. Epidemiology of neurological disorders in India: review of background, prevalence and incidence of epilepsy, stroke, Parkinson's disease and tremors

    National Research Council Canada - National Science Library

    Gourie-Devi, M

    2014-01-01

    ...). Prevalence and incidence rates of common disorders including epilepsy, stroke, Parkinson's disease and tremors determined through population-based surveys show considerable variation across different...

  7. Plantar Temperature Response to Walking in Diabetes with and without Acute Charcot: The Charcot Activity Response Test

    Directory of Open Access Journals (Sweden)

    Bijan Najafi

    2012-01-01

    Full Text Available Objective. Asymmetric plantar temperature differences secondary to inflammation is a hallmark for the diagnosis and treatment response of Charcot foot syndrome. However, little attention has been given to temperature response to activity. We examined dynamic changes in plantar temperature (PT as a function of graduated walking activity to quantify thermal responses during the first 200 steps. Methods. Fifteen individuals with Acute Charcot neuroarthropathy (CN and 17 non-CN participants with type 2 diabetes and peripheral neuropathy were recruited. All participants walked for two predefined paths of 50 and 150 steps. A thermal image was acquired at baseline after acclimatization and immediately after each walking trial. The PT response as a function of number of steps was examined using a validated wearable sensor technology. The hot spot temperature was identified by the 95th percentile of measured temperature at each anatomical region (hind/mid/forefoot. Results. During initial activity, the PT was reduced in all participants, but the temperature drop for the nonaffected foot was 1.9 times greater than the affected side in CN group (P=0.04. Interestingly, the PT in CN was sharply increased after 50 steps for both feet, while no difference was observed in non-CN between 50 and 200 steps. Conclusions. The variability in thermal response to the graduated walking activity between Charcot and non-Charcot feet warrants future investigation to provide further insight into the correlation between thermal response and ulcer/Charcot development. This stress test may be helpful to differentiate CN and its response to treatment earlier in its course.

  8. Charcots artropati som årsag til hypoparatyroid hyperkalcæmi

    DEFF Research Database (Denmark)

    Engberg, Susanne; Jensen, Jens-Erik Beck; Kønig, Karen Bay

    2012-01-01

    A 45-year-old woman with type 2 diabetes and multiple diabetic complications was diagnosed with hypoparathyroid hypercalcaemia. The bone scintigraphy showed Charcots arthropathy. Blood tests, computer tomography and mammography did not give any indication of malignancy, vitamin-D intoxication nor...... hyperthyroidism. Charcots arthropathy is not a recognized cause of hypoparathyroid hypercalcaemia, but the mechanism might be increased boneresorption. We recommend that Charcots arthropathy is considered a cause of hypoparathyroid hypercalcaemia in patients with diabetic neuropathy....

  9. An overview of conservative treatment options for diabetic Charcot foot neuroarthropathy

    Directory of Open Access Journals (Sweden)

    Crystal L. Ramanujam

    2011-05-01

    Full Text Available Conservative management of Charcot foot neuroarthropathy remains efficacious for certain clinical scenarios. Treatment of the patient should take into account the stage of the Charcot neuroarthopathy, site(s of involvement, presence or absence of ulceration, presence or absence of infection, overall medical status, and level of compliance. The authors present an overview of evidence-based non-operative treatment for diabetic Charcot neuroarthropathy with an emphasis on the most recent developments in therapy.

  10. Mortality Risk of Charcot Arthropathy Compared With That of Diabetic Foot Ulcer and Diabetes Alone

    OpenAIRE

    Sohn, Min-Woong; Lee, Todd A.; Stuck, Rodney M.; Frykberg, Robert G.; Budiman-Mak, Elly

    2009-01-01

    OBJECTIVE The purpose of this study was to compare mortality risks of patients with Charcot arthropathy with those of patients with diabetic foot ulcer and those of patients with diabetes alone (no ulcer or Charcot arthropathy). RESEARCH DESIGN AND METHODS A retrospective cohort of 1,050 patients with incident Charcot arthropathy in 2003 in a large health care system was compared with patients with foot ulcer and those with diabetes alone. Mortality was determined during a 5-year follow-up pe...

  11. Adult neurology training during child neurology residency.

    Science.gov (United States)

    Schor, Nina F

    2012-08-21

    As it is currently configured, completion of child neurology residency requires performance of 12 months of training in adult neurology. Exploration of whether or not this duration of training in adult neurology is appropriate for what child neurology is today must take into account the initial reasons for this requirement and the goals of adult neurology training during child neurology residency.

  12. Genetic dysfunction of MT-ATP6 causes axonal Charcot-Marie-Tooth disease.

    LENUS (Irish Health Repository)

    Pitceathly, Robert D S

    2012-09-11

    Charcot-Marie-Tooth (CMT) disease is the most common inherited neuromuscular disorder, affecting 1 in 2,500 individuals. Mitochondrial DNA (mtDNA) mutations are not generally considered within the differential diagnosis of patients with uncomplicated inherited neuropathy, despite the essential requirement of ATP for axonal function. We identified the mtDNA mutation m.9185T>C in MT-ATP6, encoding the ATP6 subunit of the mitochondrial ATP synthase (OXPHOS complex V), at homoplasmic levels in a family with mitochondrial disease in whom a severe motor axonal neuropathy was a striking feature. This led us to hypothesize that mutations in the 2 mtDNA complex V subunit encoding genes, MT-ATP6 and MT-ATP8, might be an unrecognized cause of isolated axonal CMT and distal hereditary motor neuropathy (dHMN).

  13. The Role of Rehabilitation in the Management of Patients with Charcot-Marie-Tooth Disease: Report of Two Cases.

    Science.gov (United States)

    Dimitrova, Erieta Nikolikj; Božinovikj, Ivana; Ristovska, Simona; Pejcikj, Aleksandra Hadzieva; Kolevska, Aleksandra; Hasani, Mirjeta

    2016-09-15

    Charcot-Marie-Tooth (CMT) disease is a hereditary disease with signs of chronic non-progressive motor-sensory neuropathy which is characterised by symmetric muscle atrophy and weakness of the distal portion of lower extremities. The aim is to present two cases with peroneal muscular atrophy, applied rehabilitation procedures and rehabilitation outcome. Patient DR, aged 51, and patient KH, aged 78. Both patients had weakness and pronounced atrophy of the distal portion of lower extremities, numbness down the legs, contractures in the ankles and walking difficulties. Evaluation of patients included a clinical examination, Barthel Index, Time Up and Go test, measurement of the ankle range of motion, and a manual muscle test. On admission, the Barthel Index score was 60 in the first case, and 80 in the second. The rehabilitation program included exercise therapy with for lower extremity, occupational therapy, stationary bicycle riding, galvanic current, water exercises, and ankle-foot orthoses for both legs. The therapy applied had no significant changes in the clinical neurological status of the patients, but yet it provided some improvement in ankle contractures, better mobility, and a more stable gait. The application of rehabilitation procedures in patients with Charcot-Marie-Tooth disease can improve their functional status and walking stability.

  14. The seminal role played by Pierre Marie in Neurology and Internal Medicine

    Directory of Open Access Journals (Sweden)

    Gustavo M Almeida

    2015-10-01

    Full Text Available The authors review the most important contributions of Pierre Marie to the elucidation and description of several neurological diseases, such as Charcot-Marie-Tooth’s disease and hereditary cerebellar ataxia, as well as his contributions to Internal Medicine, including his pioneering studies on acromegaly, ankylosing spondylitis, and hypertrophic pulmonary osteoarthropathy. His works led to incontestable advances in the medical sciences that transcended his time.

  15. Launching Effectiveness Research to Guide Practice in Neurosurgery: A National Institute Neurological Disorders and Stroke Workshop Report.

    Science.gov (United States)

    Walicke, Patricia; Abosch, Aviva; Asher, Anthony; Barker, Fred G; Ghogawala, Zoher; Harbaugh, Robert; Jehi, Lara; Kestle, John; Koroshetz, Walter; Little, Roderick; Rubin, Donald; Valadka, Alex; Wisniewski, Stephen; Chiocca, E Antonio

    2017-04-01

    This workshop addressed challenges of clinical research in neurosurgery. Randomized controlled clinical trials (RCTs) have high internal validity, but often insufficiently generalize to real-world practice. Observational studies are inclusive but often lack sufficient rigor. The workshop considered possible solutions, such as (1) statistical methods for demonstrating causality using observational data; (2) characteristics required of a registry supporting effectiveness research; (3) trial designs combining advantages of observational studies and RCTs; and (4) equipoise, an identified challenge for RCTs. In the future, advances in information technology potentially could lead to creation of a massive database where clinical data from all neurosurgeons are integrated and analyzed, ending the separation of clinical research and practice and leading to a new "science of practice." Copyright © 2017 by the Congress of Neurological Surgeons.

  16. Effect of the functional caregivers Plan implementation on the anxiety and quality of life for the family caregivers of dependent people with neurological disorders

    Directory of Open Access Journals (Sweden)

    Ruth Molina Fuillerat

    2012-01-01

    Full Text Available In January 2005 the Andalusian Health Service Improvement Plan prepared: Caring for the Caregiver include actions to be taken to promote equity, to recognize and facilitate the work of family carers. From our perspective of formal caregivers, it seems necessary to consider not only themselves need care patients with the disease, but also makes it mandatory caring individuals usually relatives, facilitators of the provision of care. In the Unit of Neurology, the daily observation of these family situations, has guided and network relationship between the two formal and informal systems of care, and we have tried the approach of the caregivers as clients to treat them as co-participants the experience of caring. Hypothesis: The Implementation of Functional Plan caregiver positive impact on hospitalization decreased anxiety and improved quality of life of caregivers of a dependent patient. Overall objective: To determine the effect of applying functional caregiver Plan on anxiety and quality of life of family caregivers of dependent people with neurological disorders. Study Design: Experimental study of the clinical trial such an intervention group and a control group randomly assigned.

  17. Complications in the management of Charcot spinal arthropathy.

    Science.gov (United States)

    Hong, Joseph; Sanfilippo, James A; Rihn, Jeffrey; Fernandez, Carmella; Winegar, Corbett D; Friel, Brian; Ludwig, Steven; Gelb, Daniel; Vaccaro, Alexander R

    2009-09-01

    Charcot spinal arthropathy is a relatively rare, destructive process characterized by a cycle of progressive deformity, destruction, and worsening instability as a result of repetitive trauma and inflammation. It may result from nontraumatic as well as traumatic causes. Historically, patients with severe symptomatic instability have been successfully treated with combined anterior and posterior fusion techniques. The long-term outcomes and potential complications, however, have not been well reported. The authors report on 2 such cases of Charcot spinal arthropathy treated surgically, one with a traumatic and one with a nontraumatic etiology. They include the unique pitfalls encountered while treating these patients, as well as their surgical treatments, complications, and long-term results.

  18. Neuropathic arthropathy (Charcot's joint) in dialysis patients

    Energy Technology Data Exchange (ETDEWEB)

    Meneghello, A.; Bertoli, M.

    1984-08-01

    To the author's knowledge, uraemic neuropathy has not been previously reported as a cause of Charcot's joint. In this paper they present three cases in which the association between clinical and radiographic patterns suggest the diagnosis of neuropathic arthropathy. The features of uraemic neuropathy are stressed and the role of secondary hyperparathyroidism in the development of this type of arthropathy is discussed. The extremely severe hyperparathyroidism reported here, may cause tendon and ligament disease, especially at the site of their bone insertion. Uraemic tendon and ligament failures weaken joints and produce further instability, which may be a precipitating factor of uraemic Charcot's joint in patients undergoing chronic haemodialysis. 5 figs.

  19. Mortality risk of Charcot arthropathy compared with that of diabetic foot ulcer and diabetes alone.

    Science.gov (United States)

    Sohn, Min-Woong; Lee, Todd A; Stuck, Rodney M; Frykberg, Robert G; Budiman-Mak, Elly

    2009-05-01

    The purpose of this study was to compare mortality risks of patients with Charcot arthropathy with those of patients with diabetic foot ulcer and those of patients with diabetes alone (no ulcer or Charcot arthropathy). A retrospective cohort of 1,050 patients with incident Charcot arthropathy in 2003 in a large health care system was compared with patients with foot ulcer and those with diabetes alone. Mortality was determined during a 5-year follow-up period. Patients with Charcot arthropathy were matched to individuals in the other two groups using propensity score matching based on patient age, sex, race, marital status, diabetes duration, and diabetes control. During follow-up, 28.0% of the sample died; 18.8% with diabetes alone and 37.0% with foot ulcer died compared with 28.3% with Charcot arthropathy. Multivariable Cox regression shows that, compared with Charcot arthropathy, foot ulcer was associated with 35% higher mortality risk (hazard ratio 1.35 [95% CI 1.18-1.54]) and diabetes alone with 23% lower risk (0.77 [0.66-0.90]). Of the patients with Charcot arthropathy, 63% experienced foot ulceration before or after the onset of the Charcot arthropathy. Stratified analyses suggest that Charcot arthropathy is associated with a significantly increased mortality risk independent of foot ulcer and other comorbidities. Charcot arthropathy was significantly associated with higher mortality risk than diabetes alone and with lower risk than foot ulcer. Patients with foot ulcers tended to have a higher prevalence of peripheral vascular disease and macrovascular diseases than patients with Charcot arthropathy. This finding may explain the difference in mortality risks between the two groups.

  20. [Vitamin D and neurology].

    Science.gov (United States)

    Thouvenot, Éric; Camu, William

    2013-10-01

    Vitamin D deficiency is associated with a higher risk of multiple sclerosis and also with a higher relapse rate as well as a higher number of MRI lesions. Elders with vitamin D deficiency have worse cognitive performance. Vitamin D deficiency is a risk factor for developing Alzheimer's disease. Ischemic stroke are more frequent and more severe in patients with low vitamin D levels. Carotid atherosclerosis is more frequent and more severe in patients with vitamin D deficiency. Vitamin D deficiency is associated with a higher risk and worse prognosis of Parkinson's disease. In the different neurological disorders discussed herein, gene polymorphisms that could alter vitamin D metabolism are also associated with a higher incidence or a worse disease prognosis. Despite the links between vitamin D deficiency and the risks of developing neurological disorders, there is, to date, no proof that supplementation could alter the course of these diseases. Copyright © 2013. Published by Elsevier Masson SAS.

  1. Ultrastructural localization of Charcot-Leyden crystal protein (lysophospholipase) to intracytoplasmic crystals in tumor cells of primary solid and papillary epithelial neoplasm of the pancreas.

    Science.gov (United States)

    Dvorak, A M; Letourneau, L; Weller, P F; Ackerman, S J

    1990-05-01

    Charcot-Leyden crystals (CLC), composed of a single protein with lysophospholipase activity, have been traditionally associated with eosinophil-rich disorders. Atypically shaped and typical CLC were noted by electron microscopy in the surgical sample from a patient with solid and papillary epithelial neoplasm of the pancreas. This rare primary tumor of the pancreas with limited aggressive behavior also contained damaged and partially or completely degranulated eosinophils in the tumor stroma. We localized CLC protein by ultrastructural immunogold staining to the CLC within vacuolar structures and in vesicles of tumor cell cytoplasm as well as to the cytoplasm and nucleus of eosinophils in the tumor stroma. These findings provide evidence that epithelial tumor cells contain Charcot-Leyden crystal protein that most likely originates from tumor stroma eosinophils. Tumor stroma eosinophils have generally been associated with improved prognoses in a wide variety of epithelial neoplasms. The role of tumor CLC protein (lysophospholipase) in these settings deserves further investigation.

  2. Charcot arthropathy of the knee. A case-based review.

    Science.gov (United States)

    Kucera, Tomas; Urban, Karel; Sponer, Pavel

    2011-03-01

    The diagnosis of Charcot arthropathy in the knee is rare. However, there is an increasing number of diabetic patients, and they are living longer due to improvements in treatment. Because neuropathic arthropathy is a late effect of peripheral neuropathy, we can expect an increasing incidence of neuropathic arthropathy. Total knee arthroplasty is the preferred choice of treatment by patients, although it may also be associated with a high incidence of serious complications. This case report presents a young female with diabetes mellitus and Charcot arthropathy of the knee managed by total knee arthroplasty and a literature review. The report encompasses a 5-year follow-up of the patient, from the first contact after knee distortion through diagnosis of Charcot arthropathy and the performance of total knee arthroplasty with outpatient controls. The diagnosis was established on the basis of the rapid destruction of the medial tibial condyle after knee distortion in a patient with neuropathy. Neuropathic arthropathy was confirmed by histology. The patient refused knee fusion and total knee arthroplasty was performed. The patient quickly achieved a painless, stable knee with a 130-degree range of motion. However, a radiolucent line appeared under the tibial component due to premature weight-bearing. The patient was ordered to refrain from weight-bearing for the next 3 months, and the knee was healed. While the management of Charcot arthropathy in the knee remains controversial, total knee arthroplasty is not a contraindication. Early diagnosis, appropriate choice of implant and operative technique, and long-term weight protection are essential.

  3. Use of implantable bone growth stimulation in Charcot ankle arthrodesis.

    Science.gov (United States)

    Hockenbury, R Todd; Gruttadauria, Michael; McKinney, Ivan

    2007-09-01

    The purpose of this study was to review the results of arthrodesis of the Charcot hindfoot when an implantable bone growth stimulator was added to the procedure. Arthrodesis of the Charcot hindfoot has a high nonunion and complication rate. Ten patients (ages 50 to 69 years) with Charcot neuroarthropathy of the ankle, hindfoot, or both had arthrodesis with use of rigid internal fixation and an implantable bone growth stimulator. There were six tibiotalocalcaneal, two pantalar, and two tibiocalcaneal arthrodeses. An intramedullary nail was used in nine patients and a blade plate was used in one patient. All but one patient was diabetic. Four of the ten patients had preoperative osteomyelitis or postoperative infection. Another patient had purulent drainage, although cultures were negative. Four patients had a preoperative ulceration. Five patients had a two-stage procedure for debridement of infected bone, removal of hardware, and placement of antibiotic beads. Autogenous bone graft from the distal fibula or proximal tibia was used in all patients. One patient with a preoperative osteomyelitis developed a stable ankle pseudarthrosis. The other nine patients fused at an average of 3.7 months after surgery for a fusion rate of 90%. There were two major complications and eight minor complications. There were no amputations. All patients were ambulatory in a double upright brace or shoes for diabetic patients and were free of ulceration at the time of followup. Average American Orthopaedic Foot and Ankle Society (AOFAS) Ankle-Hindfoot score improved from 21 preoperatively to 59 postoperatively. The adjunctive use of an implantable bone growth stimulator in conjunction with rigid internal fixation, autogenous bone grafting, and sound operative technique may enhance the outcome and fusion rate in patients undergoing arthrodesis for Charcot neuroarthropathy of the ankle and hindfoot.

  4. Formation of Charcot-Leyden crystals by human basophils

    OpenAIRE

    1982-01-01

    Charcot-Leyden crystals (CLC) are currently believed to be unique to the eosinophil and a hallmark of active eosinophilic inflammation or proliferation. The distinctiveness of the CLC to the eosinophil was questioned in 1965 by Archer and Blackwood (9), but their demonstration of CLC formation in basophils was ignored and later dismissed (1) as being the result of eosinophil contamination of basophil-enriched cell suspensions. We reexamined this question and showed that basophils obtained fro...

  5. Toxoplasmosis and Polygenic Disease Susceptibility Genes: Extensive Toxoplasma gondii Host/Pathogen Interactome Enrichment in Nine Psychiatric or Neurological Disorders

    Directory of Open Access Journals (Sweden)

    C. J. Carter

    2013-01-01

    Full Text Available Toxoplasma gondii is not only implicated in schizophrenia and related disorders, but also in Alzheimer's or Parkinson's disease, cancer, cardiac myopathies, and autoimmune disorders. During its life cycle, the pathogen interacts with ~3000 host genes or proteins. Susceptibility genes for multiple sclerosis, Alzheimer's disease, schizophrenia, bipolar disorder, depression, childhood obesity, Parkinson's disease, attention deficit hyperactivity disorder (multiple sclerosis, and autism (, but not anorexia or chronic fatigue are highly enriched in the human arm of this interactome and 18 (ADHD to 33% (MS of the susceptibility genes relate to it. The signalling pathways involved in the susceptibility gene/interactome overlaps are relatively specific and relevant to each disease suggesting a means whereby susceptibility genes could orient the attentions of a single pathogen towards disruption of the specific pathways that together contribute (positively or negatively to the endophenotypes of different diseases. Conditional protein knockdown, orchestrated by T. gondii proteins or antibodies binding to those of the host (pathogen derived autoimmunity and metabolite exchange, may contribute to this disruption. Susceptibility genes may thus be related to the causes and influencers of disease, rather than (and as well as to the disease itself.

  6. [Complex reconstruction with internal locking plate fixation for Charcot arthropathy].

    Science.gov (United States)

    Ramadani, F; Härägus, H; Radu, P; Trieb, K; Hofstaetter, S

    2015-01-01

    Osteosynthesis and reposition of the Charcot foot is challenging with respect to choice of a proper implant. There is currently no international consensus regarding the optimal implant. Locking plates seem to be an innovative and stable method for reconstruction. The aim of this work is to analyze bone fusion, complications, pseudoarthrosis, and patient satisfaction. This paper presents a retrospective analysis of 63 consecutive Charcot feet treated between 2004 and 2014. The mean follow-up time was 2.4 years. All Charcot feet treated between 2004 and 2014 were Sanders type II or III. A bony fusion was achieved in 50 % of the cases, 26 % had a functional pseudoarthrosis with intact implants and pain-free mobility, and 22 % showed no healing with broken implants. Conclusion Internal fixation with locking plates is superior to screw fixation only with regard to biomechanics. We prefer internal fixation plates to external fixation because of stability even in the case of pseudoathrosis and because of the learning curve.

  7. Charcot arthropathy in ultrasound examination – a case report

    Science.gov (United States)

    Nowakowska-Płaza, Anna; Walentowska-Janowicz, Marta; Chojnowski, Marek; Sudoł-Szopińska, Iwona

    2016-01-01

    This article presents a patient with a long history of type 1 diabetes mellitus complicated with neuropathy and Charcot disease. The most common cause of neuropathic osteoarthropathy, called Charcot osteoarthropathy, is poorly controlled diabetes. The clinical picture is characterized by considerable edema, redness and increased skin temperature with relatively slight pain due to injury to nerve fibers responsible for pain sensation. The differential diagnosis should include bacterial or autoimmune arthritis, arthritis associated with gout as well as venous thrombosis and injury. The contribution of a local inflammatory reaction and abnormal bone turnover with excessive osteoclast activity might play a role in the etiopathogenesis of this disease. As a result, osseous and articular destruction progresses rapidly leading to irreversible deformity of the foot. Avoiding weight-bearing and resting the foot in a specially selected plaster cast is the most important part of treatment. Patients with the aforementioned complaints are referred to radiologists for imaging examinations. An ultrasonographer should pay attention to changes typical of Charcot arthropathy, such as: inflammatory and destructive changes in joints of the foot, uneven contour of bones with thickening and periosteal hyperemia as well as soft tissue swelling. PMID:27446605

  8. Charcot arthropathy in ultrasound examination – a case report

    Directory of Open Access Journals (Sweden)

    Mateusz Płaza

    2016-06-01

    Full Text Available This article presents a patient with a long history of type 1 diabetes mellitus complicated with neuropathy and Charcot disease. The most common cause of neuropathic osteoarthropathy, called Charcot osteoarthropathy, is poorly controlled diabetes. The clinical picture is characterized by considerable edema, redness and increased skin temperature with relatively slight pain due to injury to nerve fibers responsible for pain sensation. The differential diagnosis should include bacterial or autoimmune arthritis, arthritis associated with gout as well as venous thrombosis and injury. The contribution of a local inflammatory reaction and abnormal bone turnover with excessive osteoclast activity might play a role in the etiopathogenesis of this disease. As a result, osseous and articular destruction progresses rapidly leading to irreversible deformity of the foot. Avoiding weight-bearing and resting the foot in a specially selected plaster cast is the most important part of treatment. Patients with the aforementioned complaints are referred to radiologists for imaging examinations. An ultrasonographer should pay attention to changes typical of Charcot arthropathy, such as: inflammatory and destructive changes in joints of the foot, uneven contour of bones with thickening and periosteal hyperemia as well as soft tissue swelling.

  9. [Complex reconstruction in Charcot arthropathy using the Ilizarov ring fixator].

    Science.gov (United States)

    Wirth, S H; Espinosa, N; Berli, M; Jankauskas, L

    2015-01-01

    From the foot surgeon's point of view, the surgical treatment of Charcot neuro-osteopathy is considered challenging. Conservative treatment using plaster and orthoses is used to treat Charcot arthropathy, especially in the acute stage of a treatment strategy. Surgical treatment using the Ilizarov ring fixator is established for the correction of residual misalignments and also in the acute stage. Advantages of the ring fixator are great stability of the apparatus, low loading of the soft tissues caused by foreign material, and possible long-term treatment. The aim of this work is to explain the indication, planning of correction, assembly of Ilizarov ring fixator, and the postoperative treatment with respect to the literature and the authors' experience. The aim of treatment with the Ilizarov ring fixator is a plantigrade, resilient, ulcer-free foot. Treatment is lengthy for both the affected patients and the attending orthopedic surgeon, and great attention is required to achieve the desired corrections and a good result for the patient. According to the literature and the authors' experience, the results indicate that the Ilizarov ring fixator represents a viable method for the preservation of the affected limb in patients with Charcot neuro-osteopathy. The assembly of the apparatus must follow the principles of Ilizarov in order to avoid failure. Corrective osseous and soft tissue interventions require a detailed preoperative analysis.

  10. Charcot arthropathy in ultrasound examination - a case report.

    Science.gov (United States)

    Płaza, Mateusz; Nowakowska-Płaza, Anna; Walentowska-Janowicz, Marta; Chojnowski, Marek; Sudoł-Szopińska, Iwona

    2016-06-01

    This article presents a patient with a long history of type 1 diabetes mellitus complicated with neuropathy and Charcot disease. The most common cause of neuropathic osteoarthropathy, called Charcot osteoarthropathy, is poorly controlled diabetes. The clinical picture is characterized by considerable edema, redness and increased skin temperature with relatively slight pain due to injury to nerve fibers responsible for pain sensation. The differential diagnosis should include bacterial or autoimmune arthritis, arthritis associated with gout as well as venous thrombosis and injury. The contribution of a local inflammatory reaction and abnormal bone turnover with excessive osteoclast activity might play a role in the etiopathogenesis of this disease. As a result, osseous and articular destruction progresses rapidly leading to irreversible deformity of the foot. Avoiding weight-bearing and resting the foot in a specially selected plaster cast is the most important part of treatment. Patients with the aforementioned complaints are referred to radiologists for imaging examinations. An ultrasonographer should pay attention to changes typical of Charcot arthropathy, such as: inflammatory and destructive changes in joints of the foot, uneven contour of bones with thickening and periosteal hyperemia as well as soft tissue swelling.

  11. Natural history of Charcot-Marie-Tooth disease during childhood.

    Science.gov (United States)

    Cornett, Kayla M D; Menezes, Manoj P; Shy, Rosemary R; Moroni, Isabella; Pagliano, Emanuela; Pareyson, Davide; Estilow, Timothy; Yum, Sabrina W; Bhandari, Trupti; Muntoni, Francesco; Laura, Matilde; Reilly, Mary M; Finkel, Richard S; Eichinger, Kate J; Herrmann, David N; Bray, Paula; Halaki, Mark; Shy, Michael E; Burns, Joshua

    2017-09-01

    To determine the rate of disease progression in a longitudinal natural history study of children with Charcot-Marie-Tooth (CMT) disease. Two hundred six (103 female) participants aged 3 to 20 years enrolled in the Inherited Neuropathies Consortium were assessed at baseline and 2 years. Demographic, anthropometric, and diagnostic information were collected. Disease progression was assessed with the CMT Pediatric Scale (CMTPedS), a reliable Rasch-built linearly weighted disability scale evaluating fine and gross motor function, strength, sensation, and balance. On average, CMTPedS Total scores progressed at a rate of 2.4 ± 4.9 over 2 years (14% change from baseline; p < 0.001). There was no difference between males and females (mean difference, 0.5; 95% confidence interval [CI], -0.9 to 1.9; p = 0.49). The most responsive CMTPedS items were dorsiflexion strength (z-score change, -0.3; 95% CI, -0.6 to -0.05; p = 0.02), balance (z-score change, -1.0; 95% CI, -1.9 to -0.09; p = 0.03), and long jump (z-score change, -0.4; 95% CI, -0.7 to -0.02; p = 0.04). Of the most common genetic subtypes, 111 participants with CMT1A/PMP22 duplication progressed by 1.8 ± 4.2 (12% change from baseline; p < 0.001), 9 participants with CMT1B/MPZ mutation progressed by 2.2 ± 5.1 (11% change), 6 participants with CMT2A/MFN2 mutation progressed by 6.2 ± 7.9 (23% change), and 7 participants with CMT4C/SH3TC2 mutations progressed by 3.0 ± 4.5 (12% change). Participants with CMT2A progressed faster than CMT1A (mean difference, -4.4; 95% CI, -8.1 to -0.8; p = 0.02). Children with CMT1A progressed consistently through early childhood (3-10 years) and adolescence (11-20 years; mean difference, 1.1; 95% CI, -0.6 to 2.7; p = 0.19), whereas CMT2A appeared to progress faster during early childhood than adolescence (mean difference, 10.0; 95% CI, -2.2 to 22.2; p = 0.08). Using the CMTPedS as an outcome measure of disease severity, children

  12. Physical activity behavior change in persons with neurologic disorders: overview and examples from Parkinson disease and multiple sclerosis.

    Science.gov (United States)

    Ellis, Terry; Motl, Robert W

    2013-06-01

    Persons with chronic progressive neurologic diseases such as Parkinson disease (PD) and multiple sclerosis (MS) face significant declines in mobility and activities of daily living, resulting in a loss of independence and compromised health-related quality of life over the course of the disease. Such undesirable outcomes can be attenuated through participation in exercise and physical activity, yet there is profound and prevalent physical inactivity in persons with PD and MS that may initiate a cycle of deconditioning and worsening of disease consequences, independent of latent disease processes. This Special Interest article highlights the accruing evidence revealing the largely sedentary behaviors common among persons living with physically disabling conditions and summarizes the evidence on the benefits of physical activity in persons with PD and MS. We then examine the social cognitive theory as an approach to identifying the primary active ingredients for behavioral change and, hence, the targets of interventions for increasing physical activity levels. The design and efficacies of interventions based on the social cognitive theory for increasing physical activity in persons with PD and MS are discussed. Finally, a rationale for adopting a secondary prevention approach to delivering physical therapy services is presented, with an emphasis on the integration of physical activity behavior change interventions into the care of persons with chronic, progressive disabilities over the course of the disease.Video Abstract available (see Video, Supplemental Digital Content 1, http://links.lww.com/JNPT/A42) for more insights from the authors.

  13. Quality of clinical trials for selected priority mental and neurological disorders in sub-Saharan Africa: a systematic review

    OpenAIRE

    Mulugeta A; Medhin G; Yimer G; Jemal R; Fekadu A

    2016-01-01

    Anwar Mulugeta,1 Girmay Medhin,2 Getnet Yimer,1 Rahimush Jemal,3 Abebaw Fekadu,4,5 1Department of Pharmacology, School of Medicine, College of Health Sciences, Addis Ababa University, 2Aklilu Lemma Institute of Pathobiology, Addis Ababa University, 3Department of Pharmacy, Tikur Anbesa Hospital, 4Department of Psychiatry, School of Medicine, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia; 5Department of Psychological Medicine, Centre for Affective Disorders, Instit...

  14. Charcot Neuropathic Arthropathy of the Foot: A Literature Review and Single-Center Experience

    Directory of Open Access Journals (Sweden)

    Tomas Kucera

    2016-01-01

    Full Text Available Charcot neuropathic osteoarthropathy of the foot is a relatively common complication of diabetic neuropathy. Incorrect diagnosis and improper treatment often result in the extremity having to be amputated. This paper summarises the current view on the etiology, diagnostics, and treatment of diabetic Charcot neuropathic osteoarthropathy, with particular focus on preserving the extremity through surgical intervention from our own experiences.

  15. Charcot Neuropathic Arthropathy of the Foot: A Literature Review and Single-Center Experience.

    Science.gov (United States)

    Kucera, Tomas; Shaikh, Haroun Hassan; Sponer, Pavel

    Charcot neuropathic osteoarthropathy of the foot is a relatively common complication of diabetic neuropathy. Incorrect diagnosis and improper treatment often result in the extremity having to be amputated. This paper summarises the current view on the etiology, diagnostics, and treatment of diabetic Charcot neuropathic osteoarthropathy, with particular focus on preserving the extremity through surgical intervention from our own experiences.

  16. Charcot arthropathy of the lumbosacral spine mimicking a vertebral tumor after spinal cord injury.

    Science.gov (United States)

    Son, Soo-Bum; Lee, Sun-Ho; Kim, Eun-Sang; Eoh, Whan

    2013-12-01

    Charcot spinal arthropathy is a rare, progressive type of vertebral joint degeneration that occurs in the setting of any preexisting condition characterized by decreased afferent innervation to the extent that normal protective joint sensation in the vertebral column is impaired. The authors report on a case of Charcot arthropathy of the lower lumbar spine mimicking a spinal tumor following cervical cord injury.

  17. Charcot Arthropathy of the Lumbosacral Spine Mimicking a Vertebral Tumor after Spinal Cord Injury

    OpenAIRE

    Son, Soo-Bum; Lee, Sun-Ho; Kim, Eun-Sang; Eoh, Whan

    2013-01-01

    Charcot spinal arthropathy is a rare, progressive type of vertebral joint degeneration that occurs in the setting of any preexisting condition characterized by decreased afferent innervation to the extent that normal protective joint sensation in the vertebral column is impaired. The authors report on a case of Charcot arthropathy of the lower lumbar spine mimicking a spinal tumor following cervical cord injury.

  18. Phenotypic spectrum of dynamin 2 mutations in Charcot-Marie-Tooth neuropathy.

    NARCIS (Netherlands)

    Claeys, K.G.; Zuchner, S.; Kennerson, M.; Berciano, J.; Garcia, A.; Verhoeven, K.; Storey, E.; Merory, J.R.; Bienfait, H.M.; Lammens, M.M.Y.; Nelis, E.; Baets, J.; Vriendt, E. De; Berneman, Z.N.; Veuster, I. De; Vance, J.M.; Nicholson, G.; Timmerman, V.; Jonghe, P. de

    2009-01-01

    Dominant intermediate Charcot-Marie-Tooth neuropathy type B is caused by mutations in dynamin 2. We studied the clinical, haematological, electrophysiological and sural nerve biopsy findings in 34 patients belonging to six unrelated dominant intermediate Charcot-Marie-Tooth neuropathy type B

  19. Charcot-Marie-tooth disease presenting in a young Nigerian male: A ...

    African Journals Online (AJOL)

    Charcot-Marie-tooth disease presenting in a young Nigerian male: A case report. ... International Journal of Medicine and Health Development ... of a 31-year-old man presenting to the medical outpatient clinic of the University of Nigeria Teawching Hospital Enugu with features of Charcot-Marie-Tooth disease is presented.

  20. The neurological disease ontology.

    Science.gov (United States)

    Jensen, Mark; Cox, Alexander P; Chaudhry, Naveed; Ng, Marcus; Sule, Donat; Duncan, William; Ray, Patrick; Weinstock-Guttman, Bianca; Smith, Barry; Ruttenberg, Alan; Szigeti, Kinga; Diehl, Alexander D

    2013-12-06

    We are developing the Neurological Disease Ontology (ND) to provide a framework to enable representation of aspects of neurological diseases that are relevant to their treatment and study. ND is a representational tool that addresses the need for unambiguous annotation, storage, and retrieval of data associated with the treatment and study of neurological diseases. ND is being developed in compliance with the Open Biomedical Ontology Foundry principles and builds upon the paradigm established by the Ontology for General Medical Science (OGMS) for the representation of entities in the domain of disease and medical practice. Initial applications of ND will include the annotation and analysis of large data sets and patient records for Alzheimer's disease, multiple sclerosis, and stroke. ND is implemented in OWL 2 and currently has more than 450 terms that refer to and describe various aspects of neurological diseases. ND directly imports the development version of OGMS, which uses BFO 2. Term development in ND has primarily extended the OGMS terms 'disease', 'diagnosis', 'disease course', and 'disorder'. We have imported and utilize over 700 classes from related ontology efforts including the Foundational Model of Anatomy, Ontology for Biomedical Investigations, and Protein Ontology. ND terms are annotated with ontology metadata such as a label (term name), term editors, textual definition, definition source, curation status, and alternative terms (synonyms). Many terms have logical definitions in addition to these annotations. Current development has focused on the establishment of the upper-level structure of the ND hierarchy, as well as on the representation of Alzheimer's disease, multiple sclerosis, and stroke. The ontology is available as a version-controlled file at http://code.google.com/p/neurological-disease-ontology along with a discussion list and an issue tracker. ND seeks to provide a formal foundation for the representation of clinical and research data

  1. Duchenne, Charcot and Babinski, three neurologists of La Salpetrière Hospital, and their contribution to concepts of the central organization of motor synergy.

    Science.gov (United States)

    Clarac, François; Massion, Jean; Smith, Allan M

    2009-11-01

    Many currently accepted notions of motor control originate from a few seminal concepts developed in the latter half of the 19th century (see Bennett and Hacker, 2002). The goal of this review is to retrace some current ideas about motor control back to the thought of three French neurologists of Hospital of the Salpetrière hospital in Paris during the latter half of the 19th century and early 20th century (Fig. 1): Guillaume Duchenne de Boulogne (1806-1875), Jean-Martin Charcot (1825-1893), and Joseph Babinski (1857-1932). A common theoretical and methodological thread unites these three men as Charcot was taught neurology by Duchenne, and Babinski was trained by Charcot. The influential concepts developed by these pioneering French neurologists have been neglected for nearly a century and only rediscovered recently. We intend to highlight how these astute clinicians used their meticulous clinical observations of patients to reveal novel and original perspectives of motor co-ordination. Between 1850 and 1930, all three men played a major role in developing and shaping the entire field of normal and pathological motor control in addition to making important contributions to three major scientific issues; the centralist view of muscle sense, the emerging concept of muscle synergy in voluntary movements and in locomotion and finally the specific role of the cerebellum in muscle synergy. The important contributions of these men will be considered in the context of other significant schools of neurology from other countries. Finally, the concept of cerebellar asynergy as proposed by Babinski anticipated the development of the internal models which much later were able to provide a theoretical basis for understanding the mechanism of learned motor co-ordination involving the cerebellum.

  2. Combining Non-Pharmacological Treatments with Pharmacotherapies for Neurological Disorders: A Unique Interface of the Brain, Drug–Device, and Intellectual Property

    Science.gov (United States)

    Bulaj, Grzegorz

    2014-01-01

    Mobile medical applications (mHealth), music, and video games are being developed and tested for their ability to improve pharmacotherapy outcomes and medication adherence. Pleiotropic mechanism of music and gamification engages an intrinsic motivation and the brain reward system, supporting therapies in patients with neurological disorders, including neuropathic pain, depression, anxiety, or neurodegenerative disorders. Based on accumulating results from clinical trials, an innovative combination treatment of epilepsy seizures, comorbidities, and the medication non-adherence can be designed, consisting of antiepileptic drugs and disease self-management software delivering clinically beneficial music. Since creative elements and art expressed in games, music, and software are copyrighted, therefore clinical and regulatory challenges in developing copyrighted, drug–device therapies may be offset by a value proposition of the exclusivity due to the patent–independent protection, which can last for over 70 years. Taken together, development of copyrighted non-pharmacological treatments (e-therapies), and their combinations with pharmacotherapies, offer incentives to chronically ill patients and outcome-driven health care industries. PMID:25071711

  3. Combining non-pharmacological treatments with pharmacotherapies for neurological disorders: a unique interface of the brain, drug-device, and intellectual property.

    Science.gov (United States)

    Bulaj, Grzegorz

    2014-01-01

    Mobile medical applications (mHealth), music, and video games are being developed and tested for their ability to improve pharmacotherapy outcomes and medication adherence. Pleiotropic mechanism of music and gamification engages an intrinsic motivation and the brain reward system, supporting therapies in patients with neurological disorders, including neuropathic pain, depression, anxiety, or neurodegenerative disorders. Based on accumulating results from clinical trials, an innovative combination treatment of epilepsy seizures, comorbidities, and the medication non-adherence can be designed, consisting of antiepileptic drugs and disease self-management software delivering clinically beneficial music. Since creative elements and art expressed in games, music, and software are copyrighted, therefore clinical and regulatory challenges in developing copyrighted, drug-device therapies may be offset by a value proposition of the exclusivity due to the patent-independent protection, which can last for over 70 years. Taken together, development of copyrighted non-pharmacological treatments (e-therapies), and their combinations with pharmacotherapies, offer incentives to chronically ill patients and outcome-driven health care industries.

  4. Combining Non-pharmacological Treatments with Pharmacotherapies for Neurological Disorders: a Unique Interface of the Brain, Drug-Device and Intellectual Property

    Directory of Open Access Journals (Sweden)

    Grzegorz eBulaj

    2014-07-01

    Full Text Available Mobile medical applications (mHealth, music and video games are being developed and tested for their ability to improve pharmacotherapy outcomes and medication adherence. Pleiotropic mechanism of music and gamification engage an intrinsic motivation and the brain reward system, supporting therapies in patients with neurological disorders, including neuropathic pain, depression, anxiety, or neurodegenerative disorders. Based on accumulating results from clinical trials, an innovative combination treatment of epilepsy seizures, comorbidities and the medication non-adherence can be designed, consisting of antiepileptic drugs and disease self-management software delivering clinically beneficial music. Since creative elements and art expressed in games, music and software are copyrighted, therefore clinical and regulatory challenges in developing copyrighted, drug-device therapies may be offset by a value proposition of the exclusivity due to the patent-independent protection which can last for over 70 years. Taken together, development of copyrighted non-pharmacological treatments (e-therapies, and their combinations with pharmacotherapies, offers incentives to chronically-ill patients and outcome-driven health care industries.

  5. Application of whole exome sequencing to a rare inherited metabolic disease with neurological and gastrointestinal manifestations: a congenital disorder of glycosylation mimicking glycogen storage disease.

    Science.gov (United States)

    Choi, Rihwa; Woo, Hye In; Choe, Byung-Ho; Park, Seungman; Yoon, Yeomin; Ki, Chang-Seok; Lee, Soo-Youn; Kim, Jong-Won; Song, Junghan; Kim, Dong Sub; Kwon, Soonhak; Park, Hyung-Doo

    2015-04-15

    Rare inherited metabolic diseases with neurological and gastrointestinal manifestations can be misdiagnosed as other diseases or remain as disorders with indeterminate etiologies. This study aims to provide evidence to recommend the utility of whole exome sequencing in clinical diagnosis of a rare inherited metabolic disease. A 4-month-old female baby visited an outpatient clinic due to poor weight gain, repeated seizure-like episodes, developmental delay, and unexplained hepatomegaly with abnormal liver function test results. Although liver biopsy revealed moderate fibrosis with a suggested diagnosis of glycogen storage disease (GSD), no mutations were identified either by single gene approach for GSD (G6PC and GAA) or by next generation sequencing panels for GSD (including 21 genes). Whole exome sequencing of the patient revealed compound heterozygous mutations of PMM2: c.580C>T (p.Arg194*) and c.713G>C (p.Arg238Pro) which mutations were associated with congenital disorder of glycosylation Ia (CDG-Ia: PMM2-CDG). We successfully applied exome sequencing to diagnose the first reported Korean patient with CDG-Ia, which was misdiagnosed as GSD. Whole exome sequencing may prove to be the preferred strategy for analysis of clinical features that do not readily suggest a specific diagnosis, such as those observed in inherited metabolic diseases, including CDG. Copyright © 2015 Elsevier B.V. All rights reserved.

  6. Assessment: Botulinum neurotoxin for the treatment of movement disorders (an evidence-based review): report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology.

    Science.gov (United States)

    Simpson, D M; Blitzer, A; Brashear, A; Comella, C; Dubinsky, R; Hallett, M; Jankovic, J; Karp, B; Ludlow, C L; Miyasaki, J M; Naumann, M; So, Y

    2008-05-06

    To perform an evidence-based review of the safety and efficacy of botulinum neurotoxin (BoNT) in the treatment of movement disorders. A literature search was performed including MEDLINE and Current Contents for therapeutic articles relevant to BoNT and selected movement disorders. Authors reviewed, abstracted, and classified articles based on American Academy of Neurology criteria (Class I-IV). The highest quality literature available for the respective indications was as follows: blepharospasm (two Class II studies); hemifacial spasm (one Class II and one Class III study); cervical dystonia (seven Class I studies); focal upper extremity dystonia (one Class I and three Class II studies); focal lower extremity dystonia (one Class II study); laryngeal dystonia (one Class I study); motor tics (one Class II study); and upper extremity essential tremor (two Class II studies). Botulinum neurotoxin should be offered as a treatment option for the treatment of cervical dystonia (Level A), may be offered for blepharospasm, focal upper extremity dystonia, adductor laryngeal dystonia, and upper extremity essential tremor (Level B), and may be considered for hemifacial spasm, focal lower limb dystonia, and motor tics (Level C). While clinicians' practice may suggest stronger recommendations in some of these indications, evidence-based conclusions are limited by the availability of data.

  7. Differences in quality between privately and publicly banked umbilical cord blood units: a pilot study of autologous cord blood infusion in children with acquired neurologic disorders

    Science.gov (United States)

    Sun, Jessica; Allison, June; McLaughlin, Colleen; Sledge, Linda; Waters-Pick, Barbara; Wease, Stephen; Kurtzberg, Joanne

    2013-01-01

    BACKGROUND A pilot study was conducted to determine the safety and feasibility of intravenous administration of autologous umbilical cord blood (CB) in young children with acquired neurologic disorders. Most CB units (CBUs) were electively stored in private CB banks. Unlike public banks, which utilize specific criteria and thresholds for banking, private banks generally store all collected CBUs. STUDY DESIGN AND METHODS CBUs of eligible patients containing more than 1 × 107 cells/kg were shipped to Duke from the banks of origin after confirming identity by HLA typing. On the day of infusion, CBUs were thawed and washed in dextran-albumin and infused intravenously. Patients were medicated with acetaminophen, diphenhydramine, and methylprednisolone before transfusion. Data regarding patients, infusions, and CBUs were collected retrospectively. Characteristics of CBUs were compared to existing data from CBUs publicly banked at the Carolinas Cord Blood Bank. RESULTS From March 2004 to December 2009, 184 children received 198 CB infusions. Three patients had infusion reactions, all responsive to medical therapy and stopping the infusion. Median precryopreservation volume (60 mL vs. 89 mL, p < 0.0001), total nucleated cell count (4.7 × 108 vs. 10.8 × 108, p < 0.0001), and CD34 count (1.8 × 106 vs. 3.0 × 106, p < 0.0001) were significantly lower than publicly stored CBUs. Postthaw sterility cultures were positive in 7.6% of infused CBUs. CONCLUSION IV infusion of autologous CB is safe and feasible in young children with neurologic injuries. Quality parameters of privately banked CBUs are inferior to those stored in public banks. If efficacy of autologous CB is established clinically, the quality of autologous units should be held to the same standards as those stored in public banks. PMID:20546200

  8. Measurements of C-reactive protein (CRP) and nerve-growth-factor (NGF) concentrations in serum and urine samples of dogs with neurologic disorders.

    Science.gov (United States)

    Kordass, Ulrike; Carlson, Regina; Stein, Veronika Maria; Tipold, Andrea

    2016-01-08

    The purpose of this study was to prove the hypothesis that C-reactive protein (CRP) and nerve growth factor (NGF) may be potential biomarkers for lower urinary tract disorders and may be able to distinguish between micturition dysfunctions of different origin in dogs with spinal cord diseases. NGF- and CRP- concentrations were measured in serum and urine samples using specific ELISA-Kits. Results in urine were standardized by urine-creatinine levels. CRP in serum was detectable in 32/76 and in urine samples in 40/76 patients. NGF could be measured in all serum and in 70/76 urine samples. Urinary CRP concentrations were significantly higher in dogs with micturition dysfunction (p = 0.0009) and in dogs with different neurological diseases (p = 0.0020) compared to the control group. However, comparing dogs with spinal cord disorders with and without associated micturition dysfunction no significant difference could be detected for NGF and CRP values in urine or serum samples. Additionally, levels did not decrease significantly, when measured at the time when the dogs regained the ability to urinate properly (urinary NGF p = 0.7962; urinary CRP p = 0.078). Urine samples with bacteria and/or leukocytes had no significant increase in urinary NGF (p = 0.1112) or CRP (p = 0.0534) concentrations, but higher CRP-levels in urine from dogs with cystitis were found compared to dogs without signs of cystitis. From these data we conclude that neither CRP nor NGF in urine or serum can be considered as reliable biomarkers for micturition disorders in dogs with spinal cord disorders in a clinical setting, but their production might be part of the pathogenesis of such disorders. Significantly higher levels of CRP could be found in the urine of dogs with micturition dysfunctions compared to control dogs. This phenomenon could potentially be explained by unspecific extrahepatic CRP production by smooth muscle cells in the dilated bladder.

  9. The neurology of proverbs.

    Science.gov (United States)

    Van Lancker, D

    1990-01-01

    Although proverb tests are commonly used in the mental status examination surprisingly little is known about either normal comprehension or the interpretation of proverbial expressions. Current proverbs tests have conceptual and linguistic shortcomings, and few studies have been done to investigate the specific effects of neurological and psychiatric disorders on the interpretation of proverbs. Although frontal lobes have traditionally been impugned in patients who are "concrete", recent studies targeting deficient comprehension of non literal language (e.g. proverbs, idioms, speech formulas, and indirect requests) point to an important role of the right hemisphere (RH). Research describing responses of psychiatrically and neurologically classified groups to tests of proverb and idiom usage is needed to clarify details of aberrant processing of nonliteral meanings. Meanwhile, the proverb test, drawing on diverse cognitive skills, is a nonspecific but sensitive probe of mental status.

  10. The Neurology of Proverbs

    Directory of Open Access Journals (Sweden)

    Diana Van Lancker

    1990-01-01

    Full Text Available Although proverb tests are commonly used in the mental status examination surprisingly little is known about either normal comprehension or the interpretation of proverbial expressions. Current proverbs tests have conceptual and linguistic shortcomings, and few studies have been done to investigate the specific effects of neurological and psychiatric disorders on the interpretation of proverbs. Although frontal lobes have traditionally been impugned in patients who are “concrete”, recent studies targeting deficient comprehension of non literal language (e.g. proverbs, idioms, speech formulas, and indirect requests point to an important role of the right hemisphere (RH. Research describing responses of psychiatrically and neurologically classified groups to tests of proverb and idiom usage is needed to clarify details of aberrant processing of nonliteral meanings. Meanwhile, the proverb test, drawing on diverse cognitive skills, is a nonspecific but sensitive probe of mental status.

  11. Neurological legal disability

    Directory of Open Access Journals (Sweden)

    Radhakrishna H

    2006-01-01

    Full Text Available Neurological disorders with a prolonged course, either remediable or otherwise are being seen increasingly in clinical practice and many such patients are young and are part of some organization or other wherein their services are needed if they were healthy and fit. The neurologists who are on the panel of these organizations are asked to certify whether these subjects are fit to work or how long they should be given leave. These certificates may be produced in the court of law and may be subjected to verification by another neurologist or a medical board. At present there are no standard guidelines in our country to effect such certification unlike in orthopedic specialty or in ophthalmology. The following is a beginning, based on which the neurologist can certify the neurological disability of such subjects and convey the same meaning to all neurologists across the country.

  12. Neurological evaluation of the selection stage of metaphor comprehension in individuals with and without autism spectrum disorder.

    Science.gov (United States)

    Chouinard, Brea; Volden, Joanne; Cribben, Ivor; Cummine, Jacqueline

    2017-10-11

    Because of their difficulties with figurative language in conversation, it is commonly thought that individuals with autism spectrum disorder (ASD) do not understand figurative meaning. However, recent research indicates that individuals with and without ASD are similar in the first two stages of metaphor comprehension, up to and including successful generation of the figurative meaning. In the current study, we used a sentence decision task to evaluate the subsequent stage of metaphor comprehension, the selection stage, which requires suppression/inhibition of the unintended meaning as part of selecting the intended meaning. fMRI activation and functional connectivity were used to compare the selection stage of metaphor comprehension between high-functioning individuals with ASD and carefully matched controls. Cortical and subcortical regions of interest were selected based on the basal-ganglia model of cognitive control. Compared to controls, individuals with ASD recruited greater activation in regions related to verbal memory (thalamus), semantic associations (medial temporal gyrus), and basic visual processing (middle occipital gyrus). Functional connectivity analysis revealed fewer overall connections and cortical-subcortical connections in the ASD group compared to controls. There was a novel finding of maintenance of subcortical-subcortical connectivity in the ASD group, specific to the selection condition, despite differences in cortically involved connections. Reduced cortical-subcortical connectivity in the ASD group compared to controls may reflect a more global impairment in cognitive control pathways, while consistent subcortical-subcortical connectivity may reflect systemic inflexibility or preserved subcortical function and dissociation between subcortical and cortical systems. Further investigation is required. Copyright © 2017 IBRO. Published by Elsevier Ltd. All rights reserved.

  13. Prevalence of polymorphisms in OPG, RANKL and RANK as potential markers for Charcot arthropathy development.

    Science.gov (United States)

    Bruhn-Olszewska, Bożena; Korzon-Burakowska, Anna; Węgrzyn, Grzegorz; Jakóbkiewicz-Banecka, Joanna

    2017-03-29

    Charcot arthropathy is one of the most serious complications of diabetic foot syndrome that leads to amputation of the affected limb. Since there is no cure for Charcot arthropathy, early diagnosis and implementation preventive care are the best available treatment. However, diagnosis is hindered by obscure clinical picture of the disease and lack of molecular markers for its early detection. Results of recent research suggest that OPG-RANKL-RANK axis regulating bone metabolism can be associated with Charcot arthropathy and that SNPs in OPG gene are associated with the disease. Here we report the results of comprehensive analysis of ten SNPs in OPG, RANKL and RANK genes in 260 subjects divided into diabetes, neuropathy and Charcot arthropathy groups. Besides genotype analysis we performed linkage disequilibrium and hierarchical clustering to obtain information about correlation between SNPs. Our results show that OPG 245T/G (rs3134069) and OPG 1217C/T (rs3102734) polymorphisms co-occur in patients with Charcot arthropathy (r2 = 0.99). Moreover, hierarchical clustering revealed a characteristic profile of all SNPs in Charcot arthropathy and neuropathy, which is distinct from control group. Our results suggest that analysis of multiple SNPs can be used as potential marker of Charcot arthropathy and provide insight into possible molecular mechanisms of its development.

  14. Identification of human eosinophil lysophospholipase as the constituent of Charcot-Leyden crystals.

    Science.gov (United States)

    Weller, P F; Goetzl, E J; Austen, K F

    1980-12-01

    Since the initial descriptions of Charcot-Leyden crystals more than 100 years ago, the presence of these slender, dipyramidal crystals in human tissues and biologic fluids has become a hallmark of eosinophilic leukocyte infiltration, especially in association with allergic and helminthic diseases. The formation of these crystals in vitro after disruption of human eosinophils, but not of other cell types, in hypotonic saline or detergent established the eosinophil as the unique cellular source of the crystalline protein. Charcot-Leyden crystals have now been found to express lysophospholipase activity (lysolecithin acylhydrolase, EC 3.1.1.5), and the solubilized Charcot-Leyden crystal protein presents a single stained protein band that is coincident with the lysophospholipase activity eluted from replicate gels on alkaline polyacrylamide gel electrophoresis. On sodium dodecyl sulfate/polyacrylamide gel electrophoresis, the solubilized Charcot-Leyden crystal protein migrates with a molecular weight of 17,400, which is comparable to that of eosinophil lysophospholipase purified chromatographically to homogeneity; further, on combination, the two proteins comigrate as a single staining band. Finally, the chromatographically purified eosinophil lysophospholipase in hypotonic buffer forms dipyramidal crystals morphologically identical to Charcot-Leyden crystals. The findings that chromatographically purified, homogeneous eosinophil lysophospholipase and Charcot-Leyden crystal protein express the same enzymatic activity, are of the same size and charge, and form crystals of identical morphology indicate that human eosinophil lysophospholipase is the constituent of Charcot-Leyden crystals.

  15. Sarcopenia and body composition in diabetic Charcot osteoarthropathy

    DEFF Research Database (Denmark)

    Jansen, Rasmus Bo; Christensen, Tomas Møller; Bülow, Jens

    2015-01-01

    BACKGROUND: Treatment of Charcot osteoarthropathy (COA) requires restricted walking and offloading for several months, which lead to fat re-distribution and increased sarcopenia. OBJECTIVES/AIM: To investigate whether subjects with COA have an altered body composition compared to controls. METHODS....../total body fat distribution ratios. RESULTS: Sarcopenia frequency was higher in the total population with diabetes overall (9-40%), compared to normal materials. Using two different models for correlating appendicular lean mass to sarcopenia, there were no differences in sarcopenia-rates between the groups...

  16. Surgical Treatment Options for the Diabetic Charcot Midfoot Deformity.

    Science.gov (United States)

    Alrashidi, Yousef; Hügle, Thomas; Wiewiorski, Martin; Herrera-Perez, Mario; Valderrabano, Victor

    2017-01-01

    Management of diabetic Charcot midfoot deformity is one of the most demanding aspects of foot and ankle surgery. Its treatment should aim at reducing the rate of complications, including foot and ankle amputations or limb loss. Attempting reconstruction at Eichenholtz stages I and II carries the risk of infection and loss of fixation. It is advisable to limit surgical reconstruction to Eichenholtz stage III in the absence of any evidence of infection or vascular insufficiency. Achilles lengthening or gastrocnemius-soleus release is an essential initial step in surgery. Addressing the medial foot column first is a key to a successful reconstruction. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. Diabetic charcot neuroarthropathy of the foot and ankle with osteomyelitis.

    Science.gov (United States)

    Ramanujam, Crystal L; Stapleton, John J; Zgonis, Thomas

    2014-10-01

    One of the most devastating foot and/or ankle complications in the diabetic population with peripheral neuropathy is the presence of Charcot neuroarthropathy (CN). In recent years, diabetic limb salvage has been attempted more frequently as opposed to major lower extremity amputation for CN of the foot and ankle with ulceration and/or deep infection. Treatment strategies for osteomyelitis in the diabetic population have evolved. This article reviews some of the most common surgical strategies recommended for the diabetic patient with CN of the foot and/or ankle and concomitant osteomyelitis. Copyright © 2014 Elsevier Inc. All rights reserved.

  18. Charcot-marie-tooth disease complicating type 2 diabetes.

    LENUS (Irish Health Repository)

    Win, Htet Htet Ne

    2011-07-01

    Although both conditions are relatively common, there are very few descriptions of type 2 diabetes mellitus coexisting with Charcot-Marie-Tooth disease (CMT). This case report and literature review describes a 53-year-old Irish man who presented with type 2 diabetes and significant neuropathy, and who was subsequently diagnosed with CMT type 1A. This case report will also discuss how to differentiate diabetic neuropathy from a progressive hereditary neuropathy and how coexistence aggravates the progression of neuropathy thus necessitating early diagnosis.

  19. Charcot-Marie-Tooth disease complicating type 2 diabetes.

    LENUS (Irish Health Repository)

    Win, Htet Htet Ne

    2012-02-01

    Although both conditions are relatively common, there are very few descriptions of type 2 diabetes mellitus coexisting with Charcot-Marie-Tooth disease (CMT). This case report and literature review describes a 53-year-old Irish man who presented with type 2 diabetes and significant neuropathy, and who was subsequently diagnosed with CMT type 1A. This case report will also discuss how to differentiate diabetic neuropathy from a progressive hereditary neuropathy and how coexistence aggravates the progression of neuropathy thus necessitating early diagnosis.

  20. Charcot-Leyden crystals: pathology and diagnostic utility.

    Science.gov (United States)

    Pantanowitz, Liron; Balogh, Karoly

    2004-07-01

    Eosinophilia is associated with several diseases of the upper respiratory tract. The predominance of eosinophils in nasal tissue, secretions, and lavage fluid has been used as an aid in the diagnosis of allergic rhinitis. Charcot-Leyden crystals, considered to be a morphologic hallmark of eosinophil-related disease, are often also found in inflamed nasal tissue and paranasal sinus contents of patients with allergic rhinitis. These bipyramidal-shaped crystals are composed of the enzyme lysolecithin acylhydrolase, one of several eosinophilic proteins that damage respiratory epithelium and contribute to the pathology of allergy in the upper respiratory tract.

  1. Charcot-Leyden crystals in a prostatic adenocarcinoma

    OpenAIRE

    Aydın Özgür

    2006-01-01

    Abstract A transrectal needle biopsy from a 63-year-old man was decided because of a high prostatic spesific antigen in the blood, and a hard right lobe in rectal examination. 10 examples were taken from each lobe. In 1 of 4 of the examples from the left lobe, which contained a small focus of adenocarcinoma, numerous eosinophils were observed to surround the carcinomatous focus and attack the carcinoma cells. Uniquely, at the same focus Charcot-Leyden crystals could be seen in the intralumina...

  2. Captopril-induced pemphigus vegetans with Charcot-Leyden crystals.

    Science.gov (United States)

    Pinto, G M; Lamarão, P; Vale, T

    1992-08-01

    A 78-year-old woman developed intertriginous vegetating plaques, mouth ulcers, and a cerebriform tongue after 11 months of captopril therapy. Findings of clinical, histologic, and immunofluorescence testing were consistent with a diagnosis of pemphigus vegetans. In addition, Charcot-Leyden crystals were observed within some of the intraepidermal, eosinophilic abscess cavities, which were bordered by granular cells undergoing keratinization. The skin lesions cleared rapidly after discontinuation of captopril. To the best of our knowledge, this report describes the first case of pemphigus vegetans induced by captopril.

  3. Does the Brief-BESTest Meet Classical Test Theory and Rasch Analysis Requirements for Balance Assessment in People With Neurological Disorders?

    Science.gov (United States)

    Bravini, Elisabetta; Nardone, Antonio; Godi, Marco; Guglielmetti, Simone; Franchignoni, Franco; Giordano, Andrea

    2016-10-01

    The Brief Balance Evaluation Systems Test (Brief-BESTest) was recently proposed as a clinical tool for quickly measuring balance disorders, but its measurement properties warrant investigation. The study objective was to perform a detailed analysis of the psychometric properties of the Brief-BESTest by means of Classical Test Theory and Rasch analysis. This was an observational measurement study. Brief-BESTest data were collected from a sample of 244 participants. Internal consistency was analyzed with the Cronbach α and item-to-total correlations. Test-retest reliability and interrater reliability were investigated in a subgroup of 21 participants. The minimum detectable change at the 95% confidence level was calculated. Scale dimensionality was examined through Horn parallel analysis; this step was followed by exploratory factor analysis for ordinal data. Finally, data were examined using Rasch analysis (rating scale model). The Cronbach α was .89, and all item-to-total correlations were greater than .40. Test-retest reliability had an intraclass correlation coefficient (ICC) (2,1) of .94, and interrater reliability had an ICC (2,1) of .90. The minimum detectable change at the 95% confidence level was 4.30 points. The unidimensionality of the test was confirmed, but 1 item showed low communality. Rasch analysis revealed the inadequacy of response categories, 5 misfitting items, minor mistargeting, moderate person reliability (.80), and 2 pairs of locally dependent items. The sample was a cross-section of people who had balance disorders from different neurological etiologies and were recruited consecutively at a single rehabilitation facility. The Brief-BESTest was confirmed to have some acceptable-to-good reliability indexes when calculated according to Classical Test Theory, but the scale showed fairly limited sensitivity to change. Rasch analysis indicated that item selection should be improved from a psychometric point of view. Item redundancy needs to be

  4. Efficient CNS targeting in adult mice by intrathecal infusion of single-stranded AAV9-GFP for gene therapy of neurological disorders.

    Science.gov (United States)

    Bey, K; Ciron, C; Dubreil, L; Deniaud, J; Ledevin, M; Cristini, J; Blouin, V; Aubourg, P; Colle, M-A

    2017-05-01

    Adeno-associated virus (AAV) gene therapy constitutes a powerful tool for the treatment of neurodegenerative diseases. While AAVs are generally administered systemically to newborns in preclinical studies of neurological disorders, in adults the maturity of the blood-brain barrier (BBB) must be considered when selecting the route of administration. Delivery of AAVs into the cerebrospinal fluid (CSF) represents an attractive approach to target the central nervous system (CNS) and bypass the BBB. In this study, we investigated the efficacy of intra-CSF delivery of a single-stranded (ss) AAV9-CAG-GFP vector in adult mice via intracisternal (iCist) or intralumbar (it-Lumb) administration. It-Lumb ssAAV9 delivery resulted in greater diffusion throughout the entire spinal cord and green fluorescent protein (GFP) expression mainly in the cerebellum, cortex and olfactory bulb. By contrast, iCist delivery led to strong GFP expression throughout the entire brain. Comparison of the transduction efficiency of ssAAV9-CAG-GFP versus ssAAV9-SYN1-GFP following it-Lumb administration revealed widespread and specific GFP expression in neurons and motoneurons of the spinal cord and brain when the neuron-specific synapsin 1 (SYN1) promoter was used. Our findings demonstrate that it-Lumb ssAAV9 delivery is a safe and highly efficient means of targeting the CNS in adult mice.

  5. A census of P. longum's phytochemicals and their network pharmacological evaluation for identifying novel drug-like molecules against various diseases, with a special focus on neurological disorders.

    Science.gov (United States)

    Choudhary, Neha; Singh, Vikram

    2018-01-01

    Piper longum (P. longum, also called as long pepper) is one of the common culinary herbs that has been extensively used as a crucial constituent in various indigenous medicines, specifically in traditional Indian medicinal system known as Ayurveda. For exploring the comprehensive effect of its constituents in humans at proteomic and metabolic levels, we have reviewed all of its known phytochemicals and enquired about their regulatory potential against various protein targets by developing high-confidence tripartite networks consisting of phytochemical-protein target-disease association. We have also (i) studied immunomodulatory potency of this herb; (ii) developed subnetwork of human PPI regulated by its phytochemicals and could successfully associate its specific modules playing important role in diseases, and (iii) reported several novel drug targets. P10636 (microtubule-associated protein tau, that is involved in diseases like dementia etc.) was found to be the commonly screened target by about seventy percent of these phytochemicals. We report 20 drug-like phytochemicals in this herb, out of which 7 are found to be the potential regulators of 5 FDA approved drug targets. Multi-targeting capacity of 3 phytochemicals involved in neuroactive ligand receptor interaction pathway was further explored via molecular docking experiments. To investigate the molecular mechanism of P. longum's action against neurological disorders, we have developed a computational framework that can be easily extended to explore its healing potential against other diseases and can also be applied to scrutinize other indigenous herbs for drug-design studies.

  6. Evidence-based guideline: intravenous immunoglobulin in the treatment of neuromuscular disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology.

    Science.gov (United States)

    Patwa, H S; Chaudhry, V; Katzberg, H; Rae-Grant, A D; So, Y T

    2012-03-27

    To assess the evidence for the efficacy of IV immunoglobulin (IVIg) to treat neuromuscular disorders. The MEDLINE, Web of Science, and EMBASE databases were searched (1966-2009). Selected articles were rated according to the American Academy of Neurology's therapeutic classification of evidence scheme; recommendations were based on the evidence level. IVIg is as efficacious as plasmapheresis and should be offered for treating Guillain-Barré syndrome (GBS) in adults (Level A). IVIg is effective and should be offered in the long-term treatment of chronic inflammatory demyelinating polyneuropathy (Level A). IVIg is probably effective and should be considered for treating moderate to severe myasthenia gravis and multifocal motor neuropathy (Level B). IVIg is possibly effective and may be considered for treating nonresponsive dermatomyositis in adults and Lambert-Eaton myasthenic syndrome (Level C). Evidence is insufficient to support or refute use of IVIg in the treatment of immunoglobulin M paraprotein-associated neuropathy, inclusion body myositis, polymyositis, diabetic radiculoplexoneuropathy, or Miller Fisher syndrome, or in the routine treatment of postpolio syndrome or in children with GBS (Level U). IVIg combined with plasmapheresis should not be considered for treating GBS (Level B). More data are needed regarding IVIg efficacy as compared with other treatments/treatment combinations. Most studies concluded IVIg-related serious adverse effects were rare. Given the variable nature of these diseases, individualized treatments depending on patient need and physician judgment are important.

  7. Volumetric magnetic resonance imaging correlates of the National Institute of Neurological Disorders and Stroke-Canadian Stroke Network vascular cognitive impairment neuropsychology protocols.

    Science.gov (United States)

    Wong, Adrian; Wang, Defeng; Black, Sandra E; Nyenhuis, David L; Shi, Lin; Chu, Winnie C W; Xiong, Yun-yun; Au, Lisa; Lau, Alexander; Chan, Anne Y Y; Wong, Lawrence K S; Mok, Vincent

    2015-01-01

    Vascular cognitive impairment (VCI) refers to the entire spectrum of cognitive dysfunction attributable to vascular changes in the brain. The objective of this study is to evaluate magnetic resonance imaging (MRI) correlates of performance on the National Institute of Neurological Disorders and Stroke-Canadian Stroke Network (NINDS-CSN) VCI neuropsychology protocols. Fifty ischemic stroke patients and 50 normal elderly persons completed the VCI protocols and MRI. Relationships between the four cognitive domains (executive/activation, language, visuospatial, and memory) and three protocol (60-, 30-, and 5-min) summary scores with MRI measures of volumes of white matter hyperintensities (WMH) and global brain and hippocampal atrophy were assessed using linear regression. All cognitive domain scores were associated with WMH volume and, with the exception of language domain, with global atrophy. Additional relationships were found between executive/activation and language domains with left hippocampal volume, visuospatial domain with right hippocampal volume, and memory domain with bilateral hippocampal volumes. All protocol summary scores showed comparable relationships with WMH and hippocampal volumes, with additional relationships found between the 60- and 30-min protocols with global brain volume. Performance on the NINDS-CSN VCI protocols reflects underlying volumetric brain changes implicated in cognitive dysfunctions in VCI.

  8. Ultrastructural observation of Charcot-Leyden crystals in mechlorethamine-treated cutaneous lesions of histiocytosis X.

    Science.gov (United States)

    Kanitakis, J; Schmitt, D; Euvrard, S; Thivolet, J

    1986-03-01

    We were recently able to observe, by electron microscopy, Charcot-Leyden crystals in the cutaneous lesions of histiocytosis X (Letterer-Siwe disease) that had been treated by local applications of mechlorethamine. The occurrence of Charcot-Leyden crystals in skin lesions is infrequent; so far, these structures have been observed only in cases of facial (Lever's) eosinophilic granuloma and in incontinentia pigmenti. Although Charcot-Leyden crystals can at times be formed spontaneously in tissue lesions, the possibility that their formation was favored by the local action of mechlorethamine is considered.

  9. AAV1.NT-3 gene therapy for charcot-marie-tooth neuropathy.

    Science.gov (United States)

    Sahenk, Zarife; Galloway, Gloria; Clark, Kelly Reed; Malik, Vinod; Rodino-Klapac, Louise R; Kaspar, Brian K; Chen, Lei; Braganza, Cilwyn; Montgomery, Chrystal; Mendell, Jerry R

    2014-03-01

    Charcot-Marie-Tooth (CMT) neuropathies represent a heterogeneous group of peripheral nerve disorders affecting 1 in 2,500 persons. One variant, CMT1A, is a primary Schwann cell (SC) disorder, and represents the single most common variant. In previous studies, we showed that neurotrophin-3 (NT-3) improved the trembler(J) (Tr(J)) mouse and also showed efficacy in CMT1A patients. Long-term treatment with NT-3 was not possible related to its short half-life and lack of availability. This led to considerations of NT-3 gene therapy via adenoassociated virus (AAV) delivery to muscle, acting as secretory organ for widespread distribution of this neurotrophic agent. In the Tr(J) model of demyelinating CMT, rAAV1.NT-3 therapy resulted in measurable NT-3 secretion levels in blood sufficient to provide improvement in motor function, histopathology, and electrophysiology of peripheral nerves. Furthermore, we showed that the compound muscle action potential amplitude can be used as surrogate for functional improvement and established the therapeutic dose and a preferential muscle-specific promoter to achieve sustained NT-3 levels. These studies of intramuscular (i.m.) delivery of rAAV1.NT-3 serve as a template for future CMT1A clinical trials with a potential to extend treatment to other nerve diseases with impaired nerve regeneration.

  10. Neurological caricatures since the 15th century.

    Science.gov (United States)

    Lorusso, Lorenzo

    2008-01-01

    During the Renaissance, different artists began to draw medical illustrations from various viewpoints. Leonardo da Vinci was among those who sought to portray the emotional as well as the physical qualities of man. Other European artists described caricatural aspects of medical activities. In Northern Europe, Albrecht Durer, Hieronymus Bosch, and Pieter Brueghel were also famous for drawing caricatures. Later English artists, notably William Hogarth, Thomas Rowlandson, James Gillray, and the Cruikshanks, satirized life in general and the medical profession in particular. In Spain, Francisco Goya's works became increasingly macabre and satirical following his own mysterious illness and, in France, Honore Daumier used satire and humor to expose medical quackery. Also physicians such as Charles Bell and Jean-Martin Charcot were talented caricaturists. Their own personal artistic styles reflected their approach and gave a different "image" of neurology. Caricatures were popular portraits of developments in science and medicine and were frequently used whenever scientific language was too difficult to disseminate, in particular in the field of neurology.

  11. Neurologic complications of alcoholism.

    Science.gov (United States)

    Noble, James M; Weimer, Louis H

    2014-06-01

    This review serves as an overview of neurologic conditions associated with alcohol abuse or withdrawal, including epidemiology, clinical symptoms, diagnostic approach, and treatment. Frequent alcohol abuse and frank alcoholism are very common among adults in the United States. Although rates decline with each decade, as many as 10% of the elderly drink excessively. Given the ubiquitous nature of alcoholism in society, its complications have been clinically recognized for generations, with recent advances focusing on improved understanding of ethanol's biochemical targets and the pathophysiology of its complications. The chronic effects of alcohol abuse are myriad and include neurologic complications through both direct and indirect effects on the central and peripheral nervous systems. These disorders include several encephalopathic states related to alcohol intoxication, withdrawal, and related nutritional deficiencies; acute and chronic toxic and nutritional peripheral neuropathies; and myopathy. Although prevention of alcoholism and its neurologic complications is the optimal strategy, this article reviews the specific treatment algorithms for alcohol withdrawal and its related nutritional deficiency states.

  12. Neurological and autoimmune disorders after vaccination against pandemic influenza A (H1N1) with a monovalent adjuvanted vaccine: population based cohort study in Stockholm, Sweden.

    Science.gov (United States)

    Bardage, Carola; Persson, Ingemar; Ortqvist, Ake; Bergman, Ulf; Ludvigsson, Jonas F; Granath, Fredrik

    2011-10-12

    To examine the risk of neurological and autoimmune disorders of special interest in people vaccinated against pandemic influenza A (H1N1) with Pandemrix (GlaxoSmithKline, Middlesex, UK) compared with unvaccinated people over 8-10 months. Retrospective cohort study linking individualised data on pandemic vaccinations to an inpatient and specialist database on healthcare utilisation in Stockholm county for follow-up during and after the pandemic period. Stockholm county, Sweden. Population All people registered in Stockholm county on 1 October 2009 and who had lived in this region since 1 January 1998; 1,024,019 were vaccinated against H1N1 and 921,005 remained unvaccinated. Neurological and autoimmune diagnoses according to the European Medicines Agency strategy for monitoring of adverse events of special interest defined using ICD-10 codes for Guillain-Barré syndrome, Bell's palsy, multiple sclerosis, polyneuropathy, anaesthesia or hypoaesthesia, paraesthesia, narcolepsy (added), and autoimmune conditions such as rheumatoid arthritis, inflammatory bowel disease, and type 1 diabetes; and short term mortality according to vaccination status. Excess risks among vaccinated compared with unvaccinated people were of low magnitude for Bell's palsy (hazard ratio 1.25, 95% confidence interval 1.06 to 1.48) and paraesthesia (1.11, 1.00 to 1.23) after adjustment for age, sex, socioeconomic status, and healthcare utilisation. Risks for Guillain-Barré syndrome, multiple sclerosis, type 1 diabetes, and rheumatoid arthritis remained unchanged. The risks of paraesthesia and inflammatory bowel disease among those vaccinated in the early phase (within 45 days from 1 October 2009) of the vaccination campaign were significantly increased; the risk being increased within the first six weeks after vaccination. Those vaccinated in the early phase were at a slightly reduced risk of death than those who were unvaccinated (0.94, 0.91 to 0.98), whereas those vaccinated in the late phase

  13. Infections with Pseudomonas aeruginosa in Charcot arthropathy of the foot.

    Science.gov (United States)

    Illgner, Ulrich; Uekoetter, Andreas; Runge, Sabrina; Wetz, Hans Henning

    2013-02-01

    Patients with Charcot arthropathy present a high risk for ulcers with secondary bone infection. Infections with Pseudomonas aeruginosa represent a severe threat to the patients. We hypothesized that infections with P aeruginosa result in a longer stay in hospital and more operations than infections with other bacteria. All patients who underwent surgery for Charcot arthropathy of the feet between 1996 and 2006 (n = 205) in our clinic were included. The duration of hospitalization and number of surgeries for infections due to methicillin-resistant Staphylococcus aureus (MRSA) versus P aeruginosa were compared to infections with other bacteria. All patients were scanned for MRSA and were isolated when tested positive and treated according to a defined algorithm. Seventy-nine intraoperative samples exhibited bacterial growth: 12 cases of MRSA, 14 cases of P aeruginosa, and 53 case of other bacteria. Patients with deep infections due to P aeruginosa stayed significantly longer in the hospital (52 vs 35 days, P < .041) and needed significantly more surgery (1.71 vs 1.28 surgeries, P < .027). There was no significant difference between patients with MRSA infections and those without MRSA or P aeruginosa. Infections with P aeruginosa resulted in more surgeries and a longer stay in the hospital. Early debridement is the basic treatment. A specific algorithm for isolation and operative and antibiotic treatment for P aeruginosa infections is proposed similar to an algorithm for MRSA that has been shown to be successful. Level IV, retrospective case Series.

  14. A proposed treatment algorithm for midfoot Charcot arthropathy.

    Science.gov (United States)

    Smith, W Bret; Moore, Chris A

    2012-02-01

    Charcot arthropathy (CA) is an insidious, destructive, and disabling pathology of the foot and ankle. Efficient and effective treatment is therefore required to improve clinical outcomes and to reduce overall mortality risk. The purpose of this case series report is to propose a treatment algorithm for correcting deformity and restoring a plantigrade, shoeable foot in patients with midfoot CA. While there is no clear evidence in the literature favoring either internal or external fixation methods, it may be appropriate to use both techniques during most deformity correction cases. Depending on the presence of ulceration, single-stage and multi-stage reconstruction with either biplanar or aggressive resection can effectively address the presentation of nonviable tissue and a wide degree of variable deformity. Following midfoot reduction, medial column plating and circular external frame can be used together to effectively stabilize and protect the correction and construct. In all cases, the aim is to immediately place the patient into a Charcot Restraint Orthotic Walker boot, with the ultimate goal of transitioning to diabetic shoeing as quickly as possible.

  15. Charcot-Leyden crystals in fine needle aspiration cytology.

    Science.gov (United States)

    Arora, V K; Singh, N; Bhatia, A

    1997-01-01

    To study the occurrence and significance of Charcot-Leyden crystals (CLC) in routinely processed, eosinophil-rich smears. Fine needle aspiration (FNA) smears of 258 cases of cysticercosis, 35 of lymphatic filariasis and 19 of nonparasitic lesions with significant eosinophils were screened for the presence of CLC and correlated with the cytomorphology of the underlying disease. Charcot-Leyden crystals were seen in lymphatic filariasis (4), eosinophilic granuloma (1), chronic myelogenous leukemia (1) and pyogenic abscess (1). CLC were conspicuous by their absence in smears of cysticercosis, although cholesterol crystals were frequently observed. CLC were present in both May-Grünwald-Giemsa- and Papanicolaou-stained smears. Necrosis was a prominent cytologic feature in all cases with CLC. CLC encountered in routinely processed aspirates containing eosinophils, albeit infrequently, indicate in vivo formation. Necrosis probably provides a milieu for suspension of eosinophils, facilitating the formation of CLC. Absence of CLC in cysticercosis could be attributed to the high lipid content of these lesions, as indicated by the frequent occurrence of cholesterol crystals.

  16. Neurologic presentation of celiac disease.

    Science.gov (United States)

    Bushara, Khalafalla O

    2005-04-01

    Celiac disease (CD) long has been associated with neurologic and psychiatric disorders including cerebellar ataxia, peripheral neuropathy, epilepsy, dementia, and depression. Earlier reports mainly have documented the involvement of the nervous system as a complication of prediagnosed CD. However, more recent studies have emphasized that a wider spectrum of neurologic syndromes may be the presenting extraintestinal manifestation of gluten sensitivity with or without intestinal pathology. These include migraine, encephalopathy, chorea, brain stem dysfunction, myelopathy, mononeuritis multiplex, Guillain-Barre-like syndrome, and neuropathy with positive antiganglioside antibodies. The association between most neurologic syndromes described and gluten sensitivity remains to be confirmed by larger epidemiologic studies. It further has been suggested that gluten sensitivity (as evidenced by high antigliadin antibodies) is a common cause of neurologic syndromes (notably cerebellar ataxia) of otherwise unknown cause. Additional studies showed high prevalence of gluten sensitivity in genetic neurodegenerative disorders such as hereditary spinocerebellar ataxia and Huntington's disease. It remains unclear whether gluten sensitivity contributes to the pathogenesis of these disorders or whether it represents an epiphenomenon. Studies of gluten-free diet in patients with gluten sensitivity and neurologic syndromes have shown variable results. Diet trials also have been inconclusive in autism and schizophrenia, 2 diseases in which sensitivity to dietary gluten has been implicated. Further studies clearly are needed to assess the efficacy of gluten-free diet and to address the underlying mechanisms of nervous system pathology in gluten sensitivity.

  17. PET and SPECT in neurology

    Energy Technology Data Exchange (ETDEWEB)

    Dierckx, Rudi A.J.O. [Groningen University Medical Center (Netherlands). Dept. of Nuclear Medicine and Molecular Imaging; Ghent Univ. (Belgium). Dept. of Radiology and Nuclear Medicine; Vries, Erik F.J. de; Waarde, Aren van [Groningen University Medical Center (Netherlands). Dept. of Nuclear Medicine and Molecular Imaging; Otte, Andreas (ed.) [Univ. of Applied Sciences Offenburg (Germany). Faculty of Electrical Engineering and Information Technology

    2014-07-01

    PET and SPECT in Neurology highlights the combined expertise of renowned authors whose dedication to the investigation of neurological disorders through nuclear medicine technology has achieved international recognition. Classical neurodegenerative disorders are discussed as well as cerebrovascular disorders, brain tumors, epilepsy, head trauma, coma, sleeping disorders, and inflammatory and infectious diseases of the CNS. The latest results in nuclear brain imaging are detailed. Most chapters are written jointly by a clinical neurologist and a nuclear medicine specialist to ensure a multidisciplinary approach. This state-of-the-art compendium will be valuable to anybody in the field of neuroscience, from the neurologist and the radiologist/nuclear medicine specialist to the interested general practitioner and geriatrician. It is the second volume of a trilogy on PET and SPECT imaging in the neurosciences, the other volumes covering PET and SPECT in psychiatry and in neurobiological systems.

  18. Pé cavo adquirido na doença de Charcot-Marie-Tooth Acquired pes cavus in Charcot-Marie-Tooth disease

    Directory of Open Access Journals (Sweden)

    Daniel Augusto Carvalho Maranho

    2009-01-01

    Full Text Available As neuropatias sensitivomotoras hereditárias, principalmente a doença de Charcot-Marie-Tooth, manifestam-se frequentemente com o aparecimento de pé cavovaro, deformidade caracterizada pela acentuação fixa do arco plantar e inversão do retropé. O diagnóstico da doença de base e a cuidadosa avaliação do paciente fornecem os elementos-chave para decisão do tratamento. O cavo pode situar-se no antepé, retropé ou ser o resultado da associação das duas localizações. Deformidades combinadas, principalmente varismo e garras dos artelhos, devem ser bem avaliadas; as características clínicas como grau das alterações, acometimento da força muscular, flexibilidade e idade são fatores importantes para a decisão da conduta. O tratamento conservador do pé cavovaro por meio de fisioterapia, palmilhas e adaptação nos calçados é reservado ao paciente mais jovem ou casos levemente acometidos. Entretanto, há tendência de agravamento das deformidades devido à característica progressiva da doença neurológica de base. Assim, o tratamento cirúrgico pelas técnicas clássicas é indicado precocemente, sendo importante identificar as alterações primárias, diferenciá-las das secundárias e corrigi-las, se possível. As transferências musculares são usadas no sentido de minimizar o desequilíbrio, estruturas retraídas são seccionadas ou alongadas e osteotomias localizadas devem ser preferíveis às artrodeses, que são reservadas para pés rígidos e muito deformados de pacientes adultos.Hereditary motor and sensory neuropathies, especially Charcot-Marie-Tooth disease, are frequently expressed with an acquired cavusvarus foot which is characterized by a fixed increase of the plantar arch and hindfoot inversion. Diagnosis of the underlying condition achieved through careful patient assessment and local evaluations is the keystone for decision-making about the adequate treatment. The cavus may present as an isolated deformity of

  19. Charcot arthropathy of the foot and ankle: modern concepts and management review.

    Science.gov (United States)

    Wukich, Dane K; Sung, Wenjay

    2009-01-01

    Charcot arthropathy (Charcot neuroarthropathy, diabetic neuropathic osteoarthropathy, or neuropathic arthropathy) remains a poorly understood disease, although recent research has improved our level of knowledge regarding its etiology and treatment. The effects of Charcot arthropathy are almost exclusively seen in the foot and ankle, and the diagnosis is commonly missed upon initial presentation. It has been well established that this complication of diabetes mellitus severely reduces the overall quality of life and dramatically increases the morbidity and mortality of patients. However, there are few high-level evidence studies to support management and treatment options at this point in time. The goal of this study is to evaluate the modern concepts of Charcot arthropathy through a review of the available literature and to integrate a perspective of management from the authors' extensive experience.

  20. Chapter 20: neurological illustration from photography to cinematography.

    Science.gov (United States)

    Aubert, Geneviève

    2010-01-01

    This chapter explores iconography in neurology from the birth of photography up to the early medical applications of cinematography before 1914. The important visual part of neurological diagnosis explains why these techniques were adopted very early by neurologists. Duchenne published the first medical book illustrated with photographs of patients. The first and most famous photographic laboratory was created in Charcot's department, at the Salpêtrière in Paris, under the direction of Albert Londe. Londe published the first book dedicated to medical photography. The physiologist Marey and the photographer Muybridge, in association with neurologists, played key roles in the development of chronophotography and cinematography. Germany was the first country to welcome cinematography in a neurology department. Independently, neurologists began to film patients in other countries in Europe and in America. In 1905, Arthur Van Gehuchten (1861-1914), Belgian anatomist and neurologist, began systematically to film neurologic patients, with the intention of building up a complete neurological iconographic collection. This collection has survived and has been restored in the laboratory of the Royal Belgian Film Archive where the films are now safely stored in their vaults.