Science.gov

Sample records for advisory neurological disorders

  1. 76 FR 2129 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    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... Committee: National Advisory Neurological Disorders and Stroke Council, Basic and Preclinical...

  2. Genomics in Neurological Disorders

    PubMed Central

    Han, Guangchun; Sun, Jiya; Wang, Jiajia; Bai, Zhouxian; Song, Fuhai; Lei, Hongxing

    2014-01-01

    Neurological disorders comprise a variety of complex diseases in the central nervous system, which can be roughly classified as neurodegenerative diseases and psychiatric disorders. The basic and translational research of neurological disorders has been hindered by the difficulty in accessing the pathological center (i.e., the brain) in live patients. The rapid advancement of sequencing and array technologies has made it possible to investigate the disease mechanism and biomarkers from a systems perspective. In this review, recent progresses in the discovery of novel risk genes, treatment targets and peripheral biomarkers employing genomic technologies will be discussed. Our major focus will be on two of the most heavily investigated neurological disorders, namely Alzheimer’s disease and autism spectrum disorder. PMID:25108264

  3. 77 FR 27239 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-05-09

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... ] Advisory Neurological Disorders and Stroke. The meeting will be closed to the public as indicated below in... of Neurological Disorders and Stroke, including consideration of personnel qualifications...

  4. 75 FR 3475 - National Institute Of Neurological Disorders and Stroke; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-01-21

    ... HUMAN SERVICES National Institutes of Health National Institute Of Neurological Disorders and Stroke.... App.), notice is hereby given of meetings of the National Advisory Neurological Disorders and Stroke... Neurological Disorders and Stroke Council; Training, Career Development, and Special Programs...

  5. 76 FR 81951 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-12-29

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... Advisory Neurological Disorders and Stroke. The meeting will be closed to the public as indicated below in... of Neurological Disorders and Stroke, including consideration of personnel qualifications...

  6. 75 FR 53319 - National Institute of Neurological Disorders and Stroke; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-08-31

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is ] hereby given of meetings of the National Advisory Neurological Disorders and Stroke... Neurological Disorders and Stroke Council Training, Career Development, and Special Programs Subcommittee....

  7. 78 FR 22273 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-04-15

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... Advisory Neurological Disorders and Stroke. The meeting will be closed to the public as indicated below in... of Neurological Disorders and Stroke, including consideration of personnel qualifications...

  8. 77 FR 52337 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-08-29

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... Advisory Neurological Disorders and Stroke. The meeting will be closed to the public as indicated below in... of Neurological Disorders and Stroke, including consideration of personnel qualifications...

  9. 76 FR 59414 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-09-26

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... Advisory Neurological Disorders and Stroke. The meeting will be closed to the public as indicated below in... of Neurological Disorders and Stroke, including consideration of personnel qualifications...

  10. Wikipedia and neurological disorders.

    PubMed

    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. PMID:25890773

  11. Key sleep neurologic disorders

    PubMed Central

    St. Louis, Erik K.

    2014-01-01

    Summary Sleep disorders are frequent comorbidities in neurologic patients. This review focuses on clinical aspects and prognosis of 3 neurologic sleep disorders: narcolepsy, restless legs syndrome/Willis-Ekbom disease (RLS/WED), and REM sleep behavior disorder (RBD). Narcolepsy causes pervasive, enduring excessive daytime sleepiness, adversely affecting patients' daily functioning. RLS/WED is characterized by an uncomfortable urge to move the legs before sleep, often evolving toward augmentation and resulting in daylong bothersome symptoms. RBD causes potentially injurious dream enactment behaviors that often signify future evolution of overt synucleinopathy neurodegeneration in as many as 81% of patients. Timely recognition, referral for polysomnography, and longitudinal follow-up of narcolepsy, RLS/WED, and RBD patients are imperatives for neurologists in providing quality comprehensive patient care. PMID:24605270

  12. [Renogenic neurologic disorders].

    PubMed

    Barbas, I M; Kodzaev, Iu K; Rudenko, T V; Skoromets, A A

    1985-01-01

    A total of 137 patients with chronic diseases of the kidneys were examined, including 34 without and 103 with chronic renal insufficiency. The neurologic syndromes under study included encephalomyelopathy with a predominant damage to the coordination systems, polyneuropathy and myopathy. These neurological changes were expressed irrespective of chronic renal failure, while their degree directly correlated with its severity. Stabilography and tremorography proved adequate and objective methods of assessing coordination disorders and made it possible to detect the above changes at the preclinical stage. PMID:3002077

  13. [Neurological Disorders and Pregnancy].

    PubMed

    Berlit, P

    2016-02-01

    Neurological disorders caused by pregnancy and puerperium include the posterior reversible encephalopathy syndrome, the amniotic fluid embolism syndrome (AFES), the postpartum angiopathy due to reversible vasoconstriction syndrome, and the Sheehan syndrome. Hypertension and proteinuria are the hallmarks of preeclampsia, seizures define eclampsia. Hemolysis, elevated liver enzymes and low platelets constitute the HELLP syndrome. Vision disturbances including cortical blindness occur in the posterior reversible encephalopathy syndrome (PRES). The Sheehan syndrome presents with panhypopituitarism post partum due to apoplexia of the pituitary gland in severe peripartal blood loss leading to longstanding hypotension. Some neurological disorders occur during pregnancy and puerperium with an increased frequency. These include stroke, sinus thrombosis, the restless legs syndrome and peripheral nerve syndromes, especially the carpal tunnel syndrome. Chronic neurologic diseases need an interdisciplinary approach during pregnancy. Some anticonvulsants double the risk of birth defects. The highest risk exists for valproic acid, the lowest for lamotrigine and levetiracetam. For MS interval treatment, glatiramer acetate and interferones seem to be safe during pregnancy. All other drugs should be avoided. PMID:26953551

  14. Coprophagia in neurologic disorders.

    PubMed

    Josephs, Keith A; Whitwell, Jennifer L; Parisi, Joseph E; Lapid, Maria I

    2016-05-01

    We report on the unusual behavior of coprophagia (eating one's own feces) in neurologic disorders. The Mayo Clinic Health Sciences-computerized clinical database was queried for all patients evaluated at our institution between 1995 and 2015 in which coprophagia was documented in the medical records. Twenty-six patients were identified of which 17 had coprophagia. Of the 17 patients, five were excluded due to age at onset less than 10 years, leaving 12 adult patients for this study. The median age at onset of coprophagia in the 12 patients was 55 years (range 20-88 years), and half were female. Additional behaviors were common including scatolia (fecal smearing), hypersexuality, aggression, and pica (eating objects of any kind). Coprophagia was associated with neurodegenerative dementia in six patients, developmental delay in two, and one each with seizures, steroid psychosis, frontal lobe tumor, and schizoaffective disorder. Brain imaging in the six patients with dementia showed moderate-to-severe medial temporal lobe atrophy, as well as mild frontal lobe atrophy. Autopsy examination was performed in one patient and revealed frontotemporal lobar degeneration pathology. Many different behavioral and pharmacologic therapies were implemented, yet only haloperidol was associated with discontinuation of the behavior. Coprophagia is associated with different neurologic disorders, particularly neurodegenerative dementias. The behavior may be related to medial temporal lobe atrophy, similar to the Klüver-Bucy syndrome. Haloperidol appears to be effective in treating the behavior, at least in some patients. PMID:27017341

  15. 75 FR 2149 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-01-14

    ... 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... personal privacy. Name of Committee: National Advisory Neurological Disorders and Stroke Council,...

  16. Paraneoplastic neurological disorders.

    PubMed

    Blaes, Franz; Tschernatsch, Marlene

    2010-10-01

    The article provides an overview on the diagnosis and pathogenesis of paraneoplastic neurological disorders (PNDs), and subsequently the current therapeutic strategies in these patients. PNDs are nervous system dysfunctions in cancer patients, which are not due to a local effect of the tumor or its metastases. Most of these clinically defined syndromes in adults are associated with lung cancer, especially small-cell lung cancer, lymphoma and gynecological tumors. In a part of the PND, an overlapping of different clinical syndromes can be observed. Highly specific autoantibodies directed against onconeuronal antigens led to the current hypothesis of an autoimmune pathophysiology. Whereas the most central nervous PNDs are more T-cell-mediated, limbic encephalitis can be caused by pathogenic receptor autoantibodies. The PND of the neuromuscular junction and paraneoplastic autonomic neuropathy are mainly associated with receptor or ion channel autoantibodies. The childhood opsoclonus-myoclonus syndrome and the PNDs associated with receptor/ion channel autoantibodies often respond to immunosuppressive therapies, plasmapheresis and intravenous immunoglobulins. By contrast, most CNS PNDs associated with defined antineuronal antibodies directed against intracellular antigens only stabilize after tumor treatment. PMID:20925471

  17. Occupational neurological disorders in Korea.

    PubMed

    Kim, Eun-A; Kang, Seong-Kyu

    2010-12-01

    The purpose of this article was to provide a literature review of occupational neurological disorders and related research in Korea, focusing on chemical hazards. We reviewed occupational neurological disorders investigated by the Occupational Safety and Health Research Institute of Korean Occupational Safety and Health Agency between 1992 and 2009, categorizing them as neurological disorders of the central nervous system (CNS), of the peripheral nervous system (PNS) or as neurodegenerative disorders. We also examined peer-reviewed journal articles related to neurotoxicology, published from 1984 to 2009. Outbreaks of occupational neurological disorder of the CNS due to inorganic mercury and carbon disulfide poisoning had helped prompt the development of the occupational safety and health system of Korea. Other major neurological disorders of the CNS included methyl bromide intoxication and chronic toxic encephalopathy. Most of the PNS disorders were n-hexane-induced peripheral neuritis, reported from the electronics industry. Reports of manganese-induced Parkinsonism resulted in the introduction of neuroimaging techniques to occupational medicine. Since the late 1990s, the direction of research has been moving toward degenerative disorder and early effect of neurotoxicity. To understand the early effects of neurotoxic chemicals in the preclinical stage, more follow-up studies of a longer duration are necessary. PMID:21258587

  18. Occupational Neurological Disorders in Korea

    PubMed Central

    Kang, Seong-Kyu

    2010-01-01

    The purpose of this article was to provide a literature review of occupational neurological disorders and related research in Korea, focusing on chemical hazards. We reviewed occupational neurological disorders investigated by the Occupational Safety and Health Research Institute of Korean Occupational Safety and Health Agency between 1992 and 2009, categorizing them as neurological disorders of the central nervous system (CNS), of the peripheral nervous system (PNS) or as neurodegenerative disorders. We also examined peer-reviewed journal articles related to neurotoxicology, published from 1984 to 2009. Outbreaks of occupational neurological disorder of the CNS due to inorganic mercury and carbon disulfide poisoning had helped prompt the development of the occupational safety and health system of Korea. Other major neurological disorders of the CNS included methyl bromide intoxication and chronic toxic encephalopathy. Most of the PNS disorders were n-hexane-induced peripheral neuritis, reported from the electronics industry. Reports of manganese-induced Parkinsonism resulted in the introduction of neuroimaging techniques to occupational medicine. Since the late 1990s, the direction of research has been moving toward degenerative disorder and early effect of neurotoxicity. To understand the early effects of neurotoxic chemicals in the preclinical stage, more follow-up studies of a longer duration are necessary. PMID:21258587

  19. 75 FR 21643 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-04-26

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... privacy. Name of Committee: National Advisory Neurological Disorders and Stroke Council. Date: May 27..., National Institute of Neurological Disorders and Stroke, NIH, 6001 Executive Blvd., Suite 3309, MSC...

  20. 75 FR 992 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-01-07

    ... 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... Neurological Disorders and Stroke Council. Date: February 4-5, 2010. Open: February 4, 2010, 10:30 a.m. to...

  1. 76 FR 2129 - National Institute of Neurological Disorders And Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    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... Neurological Disorders and Stroke Council Clinical Trials Subcommittee. Date: February 2-3, 2011....

  2. 78 FR 45932 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-07-30

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of the National Advisory Neurological Disorders and Stroke Council. The... Neurological Disorders and Stroke Council. Date: September 12-13, 2013. Open: September 12, 2013, 8:00 a.m....

  3. 75 FR 52010 - National Institute of Neurological Disorders and Stroke; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-08-24

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of meetings of the National Advisory Neurological Disorders and Stroke... Neurological Disorders and Stroke Council; Clinical Trials Subcommittee. Date: September 22-23, 2010....

  4. 76 FR 28054 - National Institute of Neurological Disorders and Stroke; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-05-13

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of meetings of the National Advisory Neurological Disorders and Stroke... Neurological Disorders and Stroke Council, Clinical Trials Subcommittee. Date: May 25, 2011. Closed: 6:30...

  5. 75 FR 22607 - National Institute of Neurological Disorders and Stroke; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-04-29

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of meetings of the National Advisory Neurological Disorders and Stroke... Neurological Disorders and Stroke Council, Clinical Trials Subcommittee. Date: May 26-27, 2010. Closed: May...

  6. Another Neurological Disorder Tied to Zika

    MedlinePlus

    ... fullstory_157678.html Another Neurological Disorder Tied to Zika It may cause meningoencephalitis, an infection and swelling ... list of neurological disorders potentially associated with the Zika virus continues to grow, health officials reported Wednesday. ...

  7. 77 FR 48999 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-08-15

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of the National Advisory Neurological Disorders and Stroke Council. The... Disorders and Stroke Council. Date: September 20-21, 2012. Open: September 20, 2012, 8 a.m. to 2:15...

  8. 76 FR 51043 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-08-17

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of the National Advisory Neurological Disorders and Stroke Council. The... Disorders and Stroke Council. Date: September 15-16, 2011. Open: September 15, 2011, 8:30 a.m. to 2...

  9. 77 FR 24725 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-04-25

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of the National Advisory Neurological Disorders and Stroke Council. The... Disorders and Stroke Council. Date: May 24-25, 2012. Open: May 24, 2012, 8 a.m. to 2:15 p.m. Agenda:...

  10. 77 FR 72872 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-12-06

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of the National Advisory Neurological Disorders and Stroke Council. The... Disorders and Stroke Council. Date: January 31-February 1, 2013. Open: January 31, 2013, 8:00 a.m. to 2:45...

  11. 78 FR 76633 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-12-18

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of the National Advisory Neurological Disorders and Stroke Council. The... Disorders and Stroke Council. Date: January 30-31, 2014. Open: January 30, 2014, 8:00 a.m. to 3:00...

  12. 75 FR 80510 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-12-22

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... privacy. Name of Committee: National Advisory Neurological Disorders and Stroke Council. Date: February 3... Disorders and Stroke, NIH, 6001 Executive Blvd., Suite 3309, MSC 9531, Bethesda, MD 20892, (301)...

  13. 78 FR 22274 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-04-15

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of the National Advisory Neurological Disorders and Stroke Council. The... Disorders and Stroke Council. Date: May 23-24, 2013. Open: May 23, 2013, 8:00 a.m. to 2:45 p.m....

  14. 77 FR 1702 - National Institute of Neurological Disorders and Stroke Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-01-11

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of the National Advisory Neurological Disorders and Stroke Council. The... Disorders and Stroke Council. Date: February 16-17, 2012. Open: February 16, 2012, 8 a.m. to 2:30...

  15. 76 FR 20691 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-04-13

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke.... App.), notice is hereby given of the National Advisory Neurological Disorders and Stroke Council. The... Disorders and Stroke Council. Date: May 26-27, 2011. Open: May 26, 2011, 10:45 a.m. to 4:45 p.m....

  16. [Neurology of hysteria (conversion disorder)].

    PubMed

    Sonoo, Masahiro

    2014-07-01

    Hysteria has served as an important driving force in the development of both neurology and psychiatry. Jean Martin Charcot's devotion to mesmerism for treating hysterical patients evoked the invention of psychoanalysis by Sigmund Freud. Meanwhile, Joseph Babinski took over the challenge to discriminate between organic and hysterical patients from Charcot and found Babinski's sign, the greatest milestone in modern neurological symptomatology. Nowadays, the usage of the term hysteria is avoided. However, new terms and new classifications are complicated and inconsistent between the two representative taxonomies, the DSM-IV and ICD-10. In the ICD-10, even the alternative term conversion disorder, which was becoming familiar to neurologists, has also disappeared as a group name. The diagnosis of hysteria remains important in clinical neurology. Extensive exclusive diagnoses and over investigation, including various imaging studies, should be avoided because they may prolong the disease course and fix their symptoms. Psychological reasons that seem to explain the conversion are not considered reliable. Positive neurological signs suggesting nonorganic etiologies are the most reliable measures for diagnosing hysteria, as Babinski first argued. Hysterical paresis has several characteristics, such as giving-way weakness or peculiar distributions of weakness. Signs to uncover nonorganic paresis utilizing synergy include Hoover's test and the Sonoo abductor test. PMID:24998831

  17. Autoimmune neurologic disorders in children.

    PubMed

    Lim, Ming; Gorman, Mark

    2016-01-01

    Autoimmune neurologic diseases are of major clinical importance in children. Antibody-mediated diseases of the central nervous system are now increasingly recognized in childhood, where the antibodies bind to cell surface epitopes on neuronal or glial proteins, and the patients demonstrate either focal or more generalized clinical signs depending on the extent of brain regions targeted by the antibodies. The antibodies are directed towards ion channels, receptors, and membrane proteins; and the diseases include limbic encephalitis and N-methyl-d-aspartate receptor-antibody encephalitis, among many others. Additionally there are conditions where the wider immune system is implicated. Neurologic features like seizures, movement disorders, autonomic dysfunction, and sleep disorders, with neuroimaging and electrophysiologic features, may indicate a specific antibody-mediated or immune disorder. Often, phenotypic overlap is observed between these conditions, and phenotypic variation seen in children with the same condition. Nevertheless, many patients benefit from immunotherapy with substantial improvement, although huge efforts are still required to optimize the outcome for many patients. In many patients no antibodies have yet been identified, even though they respond to immunotherapies. Here we describe the known antibodies and associated diseases, discuss conditions that are thought to be immune-mediated but have no known immunologic biomarker, and provide guidelines for the investigation and classification of these disorders. PMID:27112693

  18. Neurological causes of taste disorders.

    PubMed

    Heckmann, J G; Lang, C J G

    2006-01-01

    In caring for patients with taste disorders, the clinical assessment should include complete examination of the cranial nerves and, in particular, gustatory testing. Neurophysiological methods such as blink reflex and masseter reflex allow the testing of trigeminofacial and trigeminotrigeminal pathways. Modern imaging methods (MRI and computed tomography) enable the delineation of the neuroanatomical structures which are involved in taste and their relation to the bony skull base. From a neurological point of view, gustatory disorders can result from damage at any location of the neural gustatory pathway from the taste buds via the peripheral (facial, glossopharyngeal and vagal nerve) and central nervous system (brainstem, thalamus) to its representation within the cerebral cortex. Etiopathogenetically, a large number of causes has to be considered, e.g. drugs and physical agents, cerebrovascular disorders including dissection of the carotid artery and pontine/thalamic lesions, space-occupying processes - in particular tumors compressing the cerebellopontine angle and the jugular foramen of the skull base - head trauma and skull base fractures, isolated cranial mononeuropathy (e.g. Bell's palsy) or polyneuropathy, epilepsy, dementia, multiple sclerosis and major depression. In addition to this, aging can also lead to diminished taste perception. Due to the broad differential diagnostic considerations, it is essential to look for additional, even mild, neurological signs and symptoms. Treatment must relate to the underlying cause. Zinc may be tried in idiopathic dysgeusia. PMID:16733343

  19. Neurology of inherited glycosylation disorders

    PubMed Central

    Freeze, HH; Eklund, E A; Ng, BG; Patterson, M C

    2013-01-01

    Congenital disorders of glycosylation comprise most of the nearly 70 genetic disorders known to be caused by impaired synthesis of glycoconjugates. The effects are expressed in most organ systems, and most involve the nervous system. Typical manifestations include structural abnormalities, (eg, rapidly progressive cerebellar atrophy), myopathies (including congenital muscular dystrophies and limb-girdle dystrophies), strokes and stroke-like episodes, epileptic seizures, developmental delay, and demyelinating neuropathy. Patients can have neurological symptoms associated with coagulopathies, immune dysfunction with or without infections, and cardiac, renal, or hepatic failure, which are common features of glycosylation disorders. The diagnosis of congenital disorders of glycosylation should be considered for any patient with multisystem disease and in those with more specific phenotypic features. Measurement of concentrations of selected glycoconjugates can be used to screen for many of these disorders, and molecular diagnosis is becoming more widely available in clinical practice. Disease-modifying treatments are available for only a few disorders, but all affected individuals benefit from early diagnosis and aggressive management. PMID:22516080

  20. Functional Disorders in Neurology: Case Studies.

    PubMed

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

    2016-08-01

    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 consensus term Persistent Posturo-Perceptual Dizziness (PPPD), axial jerking/myoclonus as a functional movement disorder, functional speech symptoms, post-concussion disorder with functional cognitive symptoms and finally advances in treatment of dissociative seizures and functional motor disorders. PMID:27445247

  1. Endocrine disorders and the neurologic manifestations

    PubMed Central

    2014-01-01

    The nervous system and the endocrine system are closely interrelated and both involved intimately in maintaining homeostasis. Endocrine dysfunctions may lead to various neurologic manifestations such as headache, myopathy, and acute encephalopathy including coma. It is important to recognize the neurologic signs and symptoms caused by the endocrine disorders while managing endocrine disorders. This article provides an overview of the neurologic manifestations found in various endocrine disorders that affect pediatric patients. It is valuable to think about 'endocrine disorder' as a cause of the neurologic manifestations. Early diagnosis and treatment of hormonal imbalance can rapidly relieve the neurologic symptoms. Better understanding of the interaction between the endocrine system and the nervous system, combined with the knowledge about the pathophysiology of the neurologic manifestations presented in the endocrine disorders might allow earlier diagnosis and better treatment of the endocrine disorders. PMID:25654063

  2. 75 FR 51278 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-08-19

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... privacy. Name of Committee: National Advisory Neurological Disorders and Stroke Council. Date: September... Stroke, NIH, 6001 Executive Blvd., Suite 3309, MSC 9531, Bethesda, MD 20892, (301) 496-9248....

  3. Brain banking for neurological disorders.

    PubMed

    Samarasekera, Neshika; Al-Shahi Salman, Rustam; Huitinga, Inge; Klioueva, Natasja; McLean, Catriona A; Kretzschmar, Hans; Smith, Colin; Ironside, James W

    2013-11-01

    Brain banks are used to gather, store, and provide human brain tissue for research and have been fundamental to improving our knowledge of the brain in health and disease. To maintain this role, the legal and ethical issues relevant to the operations of brain banks need to be more widely understood. In recent years, researchers have reported that shortages of high-quality brain tissue samples from both healthy and diseased people have impaired their efforts. Closer collaborations between brain banks and improved strategies for brain donation programmes will be essential to overcome these problems as the demand for brain tissue increases and new research techniques become more widespread, with the potential for substantial scientific advances in increasingly common neurological disorders. PMID:24074724

  4. Addressing neurological disorders with neuromodulation.

    PubMed

    Oluigbo, Chima O; Rezai, Ali R

    2011-07-01

    Neurological disorders are becoming increasingly common in developed countries as a result of the aging population. In spite of medications, these disorders can result in progressive loss of function as well as chronic physical, cognitive, and emotional disability that ultimately places enormous emotional and economic on the patient, caretakers, and the society in general. Neuromodulation is emerging as a therapeutic option in these patients. Neuromodulation is a field, which involves implantable devices that allow for the reversible adjustable application of electrical, chemical, or biological agents to the central or peripheral nervous system with the objective of altering its functioning with the objective of achieving a therapeutic or clinically beneficial effect. It is a rapidly evolving field that brings together many different specialties in the fields of medicine, materials science, computer science and technology, biomedical, and neural engineering as well as the surgical or interventional specialties. It has multiple current and emerging indications, and an enormous potential for growth. The main challenges before it are in the need for effective collaboration between engineers, basic scientists, and clinicians to develop innovations that address specific problems resulting in new devices and clinical applications. PMID:21193369

  5. Functional neurological disorders: mechanisms and treatment.

    PubMed

    Lehn, Alexander; Gelauff, Jeannette; Hoeritzauer, Ingrid; Ludwig, Lea; McWhirter, Laura; Williams, Stevie; Gardiner, Paula; Carson, Alan; Stone, Jon

    2016-03-01

    Functional neurological disorders are common problems in neurologic practice. In the past decade there has been an increasing interest in this group of disorders both from a clinical as well as research point of view. In this review, we highlight some of the most salient and exciting publications from recent years focusing especially on new findings illuminating mechanism and studies examining treatment. PMID:26410744

  6. Comorbidity between neurological illness and psychiatric disorders.

    PubMed

    Hesdorffer, Dale C

    2016-06-01

    Psychiatric disorders are common in many neurological disorders, including epilepsy, migraine, Alzheimer's disease, Parkinson's disease, essential tremor, and stroke. These comorbidities increase disease burden and may complicate the treatment of the combined disorders. Initial studies of the comorbidity of psychiatric and neurological disorders were cross-sectional, and time order of the associations was impossible to elucidate. More recent work has clarified time associations between psychiatric disorders and neurological disorders, particularly in epilepsy and stroke where epidemiological evidence suggests that there is a bidirectional relationship. This article takes an epidemiological approach to understanding these relationships and focuses mostly on epilepsy. Although, these relationships are understood in many neurological disorders, routine screening for psychiatric disorders in neurological disorders is infrequent, mostly due to the lack of partnerships between psychiatrists and neurologists and the paucity of neuropsychiatrists. Much more needs to be done to improve the detection and treatment of patients affected by neurological and psychiatric disorders. Understanding the scope of this overlap may inspire collaborations to improve the lives of people affected by both disorders. PMID:26898322

  7. Complementary and alternative medicine for neurologic disorders.

    PubMed

    Kline, Karen L

    2002-02-01

    The use of complementary and alternative veterinary medicine in treating neurologic disorders has increased in popularity in response to advances in human alternative and integrative therapies. Neurolocalization of lesions to the brain, spinal cord, and neuromuscular systems is discussed, as well as the diagnostics and therapeutics used to treat such disorders. Emphasis is placed on integrative and alternative treatments for such neurologic diseases as seizures, cerebrovascular accidents, canine cognitive disorder, meningitis, intervertebral disc disease, fibrocartilagenous embolism, degenerative myelopathy, and myopathies. Thorough physical and neurologic examinations, establishment of a correct diagnosis, and integrative therapeutics are aimed at improving the overall quality of life of the veterinary patient. PMID:11890124

  8. Neurological disorders and celiac disease.

    PubMed

    Casella, Giovanni; Bordo, Bianca M; Schalling, Renzo; Villanacci, Vincenzo; Salemme, Marianna; DI Bella, Camillo; Baldini, Vittorio; Bassotti, Gabrio

    2016-06-01

    Celiac disease (CD) determines neurologic manifestations in 10% of all CD patients. We describe the most common clinical manifestations as cerebellar ataxia, gluten encephalopathy, multiple sclerosis, peripheral neuropathies, sensorineural hearing loss, epilepsy, headache, depression, cognitive deficiencies and other less described clinical conditions. Our aim is to perform, as more as possible, a review about the most recent update on the topics in international literature. It is important to consider clinical neurological manifestations in celiac patients and to research these conditions also in the follow-up because they may start also one year after the start of gluten free diet (GFD) as peripheral neuropathy. The association with autism is analysed and possible new association with non-celiac gluten sensitivity (NCGS) are considered. PMID:26619901

  9. 76 FR 12973 - Neurological Devices Panel of the Medical Devices Advisory Committee; Amendment of Notice

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-03-09

    ... HUMAN SERVICES Food and Drug Administration Neurological Devices Panel of the Medical Devices Advisory... Medical Devices Advisory Committee. This meeting was announced in the Federal Register of February 7, 2011... meeting of the Neurological Devices Panel of the Medical Devices Advisory Committee would be held on...

  10. Neurological disorders presenting mainly in adolescence

    PubMed Central

    Macleod, S; Appleton, R E

    2007-01-01

    The aim of this review is to discuss some of the neurological diseases that present mainly in the adolescent period. The article focuses on the usual presentation and course of the more common, and some uncommon, epilepsies, neuromuscular disorders, neurodegenerative disorders, inflammatory disorders of the central nervous system and some other, miscellaneous conditions. The article ends with a very brief and general discussion about management issues in this age group. PMID:17264287

  11. Preimplantation genetic diagnosis for inherited neurological disorders.

    PubMed

    Tur-Kaspa, Ilan; Jeelani, Roohi; Doraiswamy, P Murali

    2014-07-01

    Preimplantation genetic diagnosis (PGD) is an option for couples at risk of having offspring with an inherited debilitating or fatal neurological disorder who wish to conceive a healthy child. PGD has been carried out for conditions with various modes of inheritance, including spinal muscular atrophy, Huntington disease, fragile X syndrome, and chromosomal or mitochondrial disorders, and for susceptibility genes for cancers with nervous system involvement. Most couples at risk of transmitting a genetic mutation would opt for PGD over prenatal testing and possible termination of a pregnancy. The aim of this Perspectives article is to assist neurologists in counselling and treating patients who wish to explore the option of PGD to enable conception of an unaffected child. PGD can be accomplished for most disorders in which the genetic basis is known, and we argue that it is time for clinicians and neurological societies to consider the evidence and to formulate guidelines for the responsible integration of PGD into modern preventative neurology. PMID:24866878

  12. Functional neurological disorders: the neurological assessment as treatment.

    PubMed

    Stone, Jon

    2016-02-01

    The neurologist's role in patients with functional disorders has traditionally been limited to making the diagnosis, excluding a 'disease' and pronouncing the symptoms to be 'non-organic' or 'psychogenic'. In this article, I argue that there are multiple opportunities during routine assessment of a patient with a functional disorder for the neurologist to take the lead with treatment. These opportunities occur throughout history taking, during the examination and, with greatest potential for treatment, at the end of the consultation. Elements of the neurologist's discussion that may be most useful include (a) emphasis that symptoms are genuine, common and potentially reversible; (b) explanation of the positive nature of the diagnosis (ie, not a diagnosis of exclusion); (c) simple advice about distraction techniques, self-help techniques and sources of information; (d) referral on to appropriate physiotherapy and/or psychological services; and (e) offering outpatient review. I also discuss how new diagnostic criteria for Diagnostic and Statistical Manual of Mental Disorders-5 and changes proposed for International Classification of Diseases may facilitate changes that allow neurologists to bring their management of patients with functional disorders in line with other multidisciplinary neurological disorders in the outpatient clinic. PMID:26715762

  13. Astrogliopathology in neurological, neurodevelopmental and psychiatric disorders.

    PubMed

    Verkhratsky, Alexei; Parpura, Vladimir

    2016-01-01

    Astroglial cells represent a main element in the maintenance of homeostasis and providing defense to the brain. Consequently, their dysfunction underlies many, if not all, neurological, neurodevelopmental and neuropsychiatric disorders. General astrogliopathy is evident in diametrically opposing morpho-functional changes in astrocytes, i.e. their hypertrophy along with reactivity or atrophy with asthenia. Neurological disorders with astroglial participation can be genetic, of which Alexander disease is a primary sporadic astrogliopathy, environmentally caused, such as heavy metal encephalopathies, or neurodevelopmental in origin. Astroglia contribute to neurodegenerative processes seen in amyotrophic lateral sclerosis, Alzheimer's and Huntington's diseases. Furthermore, astroglia also play a role in major neuropsychiatric disorders, ranging from schizophrenia to depression, as well as in addictive disorders. PMID:25843667

  14. Mitochondria in Neuroplasticity and Neurological Disorders

    PubMed Central

    Mattson, Mark P.; Gleichmann, Marc; Cheng, Aiwu

    2009-01-01

    Mitochondrial electron transport generates the ATP that is essential for the excitability and survival of neurons, and the protein phosphorylation reactions that mediate synaptic signaling and related long-term changes in neuronal structure and function. Mitochondria are highly dynamic organelles that divide, fuse and move purposefully within axons and dendrites. An Major functions of mitochondria in neurons include the regulation of Ca2+ and redox signaling, developmental and synaptic plasticity, and the arbitration of cell survival and death. The importance of mitochondria in neurons is evident in the neurological phenotypes in rare diseases caused by mutations in mitochondrial genes. Mitochondria-mediated oxidative stress, perturbed Ca2+ homeostasis and apoptosis may also contribute to the pathogenesis of prominent neurological diseases including Alzheimer’s, Parkinson’s and Huntington’s diseases, stroke, ALS and psychiatric disorders. Advances in understanding the molecular and cell biology of mitochondria are leading to novel approaches for the prevention and treatment of neurological disorders. PMID:19081372

  15. Neurological disorders and inflammatory bowel diseases

    PubMed Central

    Casella, Giovanni; Tontini, Gian Eugenio; Bassotti, Gabrio; Pastorelli, Luca; Villanacci, Vincenzo; Spina, Luisa; Baldini, Vittorio; Vecchi, Maurizio

    2014-01-01

    Extraintestinal manifestations occur in about one-third of patients living with inflammatory bowel disease (IBD) and may precede the onset of gastrointestinal symptoms by many years. Neurologic disorders associated with IBD are not frequent, being reported in 3% of patients, but they often represent an important cause of morbidity and a relevant diagnostic issue. In addition, the increasing use of immunosuppressant and biological therapies for IBD may also play a pivotal role in the development of neurological disorders of different type and pathogenesis. Hence, we provide a complete and profound review of the main features of neurological complications associated with IBD, with particular reference to those related to drugs and with a specific focus on their clinical presentation and possible pathophysiological mechanisms. PMID:25083051

  16. [Preventing swallowing disorders in neurological patients].

    PubMed

    Poindessous, Jean-Luc; Basta, Martial; Da Silva, José; Tillard, Audrey; Rasquier, Stéphanie; Héron, Anne

    2015-12-01

    Swallowing disorders in neurological rehabilitation are common and important as they can have harmful consequences. A multi-disciplinary hospital team was created to study ways of preventing their occurrence. This article presents the areas to focus on and the main orientations of patient management. PMID:26654505

  17. 76 FR 14979 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-03-18

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke; Notice of Meeting Pursuant to section 10(a) of the Federal Advisory Committee Act, as amended (5 U.S.C. App.), notice is hereby given of a meeting...

  18. 78 FR 51196 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-08-20

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting Pursuant to section 10(d) of the Federal Advisory Committee Act, as amended (5...

  19. 75 FR 2146 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-01-14

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting Pursuant to section 10(d) of the Federal Advisory Committee Act, as ] amended...

  20. 78 FR 59945 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-09-30

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting Pursuant to section 10(d) of the Federal Advisory Committee Act, as ] amended...

  1. 77 FR 24727 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-04-25

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting Pursuant to section 10(d) of the Federal Advisory Committee Act, as amended (5...

  2. 78 FR 42969 - National Institute of Neurological Disorders and Stroke; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-07-18

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke; Notice of Meeting Pursuant to section 10(a) of the Federal Advisory Committee Act, as amended (5 U.S.C. App.), notice is hereby given of a meeting...

  3. 75 FR 11187 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-03-10

    ... From the Federal Register Online via the Government Publishing Office ] DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting Pursuant to section 10(d) of the Federal Advisory Committee Act, as amended (5 U.S.C. App.), notice is hereby given of...

  4. 76 FR 6485 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-02-04

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting Pursuant to section 10(d) of the Federal Advisory Committee Act, as amended (5 U.S.C. App.), notice is hereby given of...

  5. Protective effects of ginseng on neurological disorders

    PubMed Central

    Ong, Wei-Yi; Farooqui, Tahira; Koh, Hwee-Ling; Farooqui, Akhlaq A.; Ling, Eng-Ang

    2015-01-01

    Ginseng (Order: Apiales, Family: Araliaceae, Genus: Panax) has been used as a traditional herbal medicine for over 2000 years, and is recorded to have antianxiety, antidepressant and cognition enhancing properties. The protective effects of ginseng on neurological disorders are discussed in this review. Ginseng species and ginsenosides, and their intestinal metabolism and bioavailability are briefly introduced. This is followed by molecular mechanisms of effects of ginseng on the brain, including glutamatergic transmission, monoamine transmission, estrogen signaling, nitric oxide (NO) production, the Keap1/Nrf2 adaptive cellular stress pathway, neuronal survival, apoptosis, neural stem cells and neuroregeneration, microglia, astrocytes, oligodendrocytes and cerebral microvessels. The molecular mechanisms of the neuroprotective effects of ginseng in Alzheimer’s disease (AD) including β-amyloid (Aβ) formation, tau hyperphosphorylation and oxidative stress, major depression, stroke, Parkinson’s disease and multiple sclerosis are presented. It is hoped that this discussion will stimulate more studies on the use of ginseng in neurological disorders. PMID:26236231

  6. 77 FR 68788 - Neurological Devices Panel of the Medical Devices Advisory Committee; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-11-16

    ... HUMAN SERVICES Food and Drug Administration Neurological Devices Panel of the Medical Devices Advisory... of Committee: Neurological Devices Panel of the Medical Devices Advisory Committee. General Function... intervention for patients who have failed maximal medical management. Of note, the CoAxia NeuroFlo Catheter...

  7. 75 FR 72832 - Neurological Devices Panel of the Medical Devices Advisory Committee; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-11-26

    ... HUMAN SERVICES Food and Drug Administration Neurological Devices Panel of the Medical Devices Advisory...). The meeting will be open to the public. Name of Committee: Neurological Devices Panel of the Medical Devices Advisory Committee. General Function of the Committee: To provide advice and recommendations...

  8. 77 FR 73034 - Neurological Devices Panel of the Medical Devices Advisory Committee; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-12-07

    ... HUMAN SERVICES Food and Drug Administration Neurological Devices Panel of the Medical Devices Advisory...). The meeting will be open to the public. Name of Committee: Neurological Devices Panel of the Medical Devices Advisory Committee. General Function of the Committee: To provide advice and recommendations...

  9. Genetics of hereditary neurological disorders in children

    PubMed Central

    Yu, Sui; Wu, Zhanhe; Tang, Beisha

    2014-01-01

    Hereditary neurological disorders (HNDs) are relatively common in children compared to those occurring in adulthood. Recognising clinical manifestations of HNDs is important for the selection of genetic testing, genetic testing results interpretation, and genetic consultation. Meanwhile, advances in next generation sequencing (NGS) technologies have significantly enabled the discovery of genetic causes of HNDs and also challenge paediatricians on applying genetic investigation. Combination of both clinical information and advanced technologies will enhance the genetic test yields in clinical setting. This review summarises the clinical presentations as well as genetic causes of paediatric neurological disorders in four major areas including movement disorders, neuropsychiatric disorders, neuron peripheral disorders and epilepsy. The aim of this review is to help paediatric neurologists not only to see the clinical features but also the complex genetic aspect of HNDs in order to utilise genetic investigation confidently in their clinical practice. A smooth transition from research based to clinical use of comprehensive genetic testing in HNDs in children could be foreseen in the near future while genetic testing, genetic counselling and genetic data interpretation are in place appropriately. PMID:26835329

  10. NEUROLOGICAL ASPECTS OF HUMAN GLYCOSYLATION DISORDERS

    PubMed Central

    Freeze, Hudson H.; Eklund, Erik A.; Ng, Bobby G.; Patterson, Marc C.

    2016-01-01

    This review will present principles of glycosylation, describe the relevant glycosylation pathways and their related disorders, and highlight some of the neurological aspects and issues that continue to challenge researchers. Over 100 rare human genetic disorders that result from deficiencies in the different glycosylation pathways are known today. Most of these disorders impact the central and/or peripheral nervous systems. Patients typically have developmental delay/intellectual disability, hypotonia, seizures, neuropathy, and metabolic abnormalities in multiple organ systems. Between these disorders there is great clinical diversity because all cell types differentially glycosylate proteins and lipids. The patients have hundreds of mis-glycosylated products afflicting a myriad of processes including cell signaling, cell-cell interaction and cell migration. This vast complexity in glycan composition and function, along with limited analytic tools has impeded the identification of key glycosylated molecules that cause pathologies, and to date few critical target proteins have been pinpointed. PMID:25840006

  11. Compensation for occupational neurological and mental disorders.

    PubMed

    Kang, Dong-Mug; Kim, Inah

    2014-06-01

    Standards for the recognition of occupational diseases (ODs) in Korea were established in 1954 and have been amended several times. In 2013, there was a significant change in these standards. On the basis of scientific evidence and causality, the International Labour Organization list, European Commission schedule, and compensated cases in Korea were reviewed to revise the previous standards for the recognition of ODs in Korea. A disease-based approach using the International Classification of Diseases (10th version) was added on the previous standards, which were agent-specific approaches. The amended compensable occupational neurological disorders and occupational mental disorders (OMDs) in Korea are acute and chronic central nervous system (CNS) disorders, toxic neuropathy, peripheral neuropathy, manganese-related disorders, and post-traumatic stress disorder. Several agents including trichloroethylene (TCE), benzene, vinyl chloride, organotin, methyl bromide, and carbon monoxide (CO) were newly included as acute CNS disorders. TCE, lead, and mercury were newly included as chronic CNS disorders. Mercury, TCE, methyl n-butyl ketone, acrylamide, and arsenic were newly included in peripheral neuropathy. Post-traumatic stress disorders were newly included as the first OMD. This amendment makes the standard more comprehensive and practical. However, this amendment does not perfectly reflect the recent scientific progress and social concerns. Ongoing effort, research, and expert consensus are needed to improve the standard. PMID:25006326

  12. Molecular Targets of Cannabidiol in Neurological Disorders.

    PubMed

    Ibeas Bih, Clementino; Chen, Tong; Nunn, Alistair V W; Bazelot, Michaël; Dallas, Mark; Whalley, Benjamin J

    2015-10-01

    Cannabis has a long history of anecdotal medicinal use and limited licensed medicinal use. Until recently, alleged clinical effects from anecdotal reports and the use of licensed cannabinoid medicines are most likely mediated by tetrahydrocannabinol by virtue of: 1) this cannabinoid being present in the most significant quantities in these preparations; and b) the proportion:potency relationship between tetrahydrocannabinol and other plant cannabinoids derived from cannabis. However, there has recently been considerable interest in the therapeutic potential for the plant cannabinoid, cannabidiol (CBD), in neurological disorders but the current evidence suggests that CBD does not directly interact with the endocannabinoid system except in vitro at supraphysiological concentrations. Thus, as further evidence for CBD's beneficial effects in neurological disease emerges, there remains an urgent need to establish the molecular targets through which it exerts its therapeutic effects. Here, we conducted a systematic search of the extant literature for original articles describing the molecular pharmacology of CBD. We critically appraised the results for the validity of the molecular targets proposed. Thereafter, we considered whether the molecular targets of CBD identified hold therapeutic potential in relevant neurological diseases. The molecular targets identified include numerous classical ion channels, receptors, transporters, and enzymes. Some CBD effects at these targets in in vitro assays only manifest at high concentrations, which may be difficult to achieve in vivo, particularly given CBD's relatively poor bioavailability. Moreover, several targets were asserted through experimental designs that demonstrate only correlation with a given target rather than a causal proof. When the molecular targets of CBD that were physiologically plausible were considered for their potential for exploitation in neurological therapeutics, the results were variable. In some cases

  13. Profile of neurological disorders in an adult neurology clinic in Kumasi, Ghana

    PubMed Central

    Sarfo, Fred Stephen; Akassi, John; Badu, Elizabeth; Okorozo, Aham; Ovbiagele, Bruce; Akpalu, Albert

    2016-01-01

    Background Although the burden of neurological disorders is highest among populations in developing countries there is a dearth of data on the clinical spectrum of these disorders. Objective To profile the frequency of neurologic disorders and basic demographic data in an adult neurology out-patient service commissioned in 2011 in Kumasi, Ghana. Methods The study was conducted at the neurology clinic of the Komfo Anokye Teaching Hospital in Kumasi, Ghana. Over a three year period, all medical records of patients enrolled at the out-patient neurology clinic was reviewed by a neurologist and neurological diagnoses classified according to ICD-10. Results 1812 adults enrolled for care in the neurology out-patient service between 2011 and 2013. This comprised of 882 males and 930 females (male: female ratio of 1.0: 1.1) with an overall median age of 54 (IQR, 39–69) years. The commonest primary neurological disorders seen were strokes, epilepsy and seizure disorders, and movement disorders at frequencies of 57.1%, 19.8%, and 8.2% respectively. Conclusions Cerebrovascular diseases, epilepsy and movement disorders were among the commonest neurological disorders and the major contributors to neurologic morbidity among Ghanaians in an urban neurology clinic. PMID:27110596

  14. Sleep Disorders in Neurologic Practice: A Case-based Approach.

    PubMed

    Panossian, Lori Ani; Avidan, Alon Y

    2016-08-01

    Sleep disorders are common in neurology practice, but are often undiagnosed and untreated. Specific patient cohorts, such as older adults, patients residing in nursing homes, and patients with underlying chronic neurologic and psychiatric disorders, are at particular risk. If these sleep problems are not properly evaluated and managed the patient may experience exacerbation of the underlying neurologic disorder. This article highlights some of the key sleep disorders relevant to practicing neurologists, emphasizing hypersomnolence, insomnia, and sleep-related movement disorders in the setting of neurologic disorders to enhance the tools available for evaluation, and discusses management strategies. PMID:27445242

  15. Immunotherapeutic approaches to paraneoplastic neurological disorders.

    PubMed

    Blaes, Franz

    2002-04-01

    The review presents an overview on the pathogenesis of paraneoplastic neurological disorders (PNDs) and the current therapeutic immunosuppressive or immunomodulatory strategies used in these patients. PNDs are disturbances in the functioning of the nervous system in cancer patients, where the disturbances are not due to a local effect of the tumour or its metastases. Most of these clinically, well-defined syndromes in adults are associated with lung cancer (especially small cell lung cancer), lymphomas and gynaecological tumours. Since autoantibodies directed against proteins expressed in neurons and tumour cells have been found, PNDs are suspected to be autoimmune. In neuromuscular PND, immunosuppressive therapies, plasmapheresis and intravenous immunoglobulins are effective treatments. In contrast, central nervous system PNDs seen in adults are by far the most problematic group to treat. With exception of the stiff-man syndrome, immunosuppression appears to have little effect on these neurological disorders. Tumour therapy stabilises PNDs but does not cause improvement. Plasmapheresis reduces the autoantibody titre in the sera of these patients but, like tumour therapy, does not lead to a clinical improvement. In children with paraneoplastic opsoclonus-myoclonus syndrome, steroids and intravenous immunoglobulins may lead to a complete or partial remission of PNDs. PMID:11955279

  16. Community-Acquired Pneumonia Hospitalization among Children with Neurologic Disorders

    PubMed Central

    Millman, Alexander J.; Finelli, Lyn; Bramley, Anna M.; Peacock, Georgina; Williams, Derek J.; Arnold, Sandra R.; Grijalva, Carlos G.; Anderson, Evan J.; McCullers, Jonathan A.; Ampofo, Krow; Pavia, Andrew T.; Edwards, Kathryn M.; Jain, Seema

    2016-01-01

    Objective To describe and compare the clinical characteristics, outcomes, and etiology of pneumonia among children hospitalized with community-acquired pneumonia (CAP) with neurologic disorders, non-neurologic underlying conditions, and no underlying conditions. Study design Children <18 years old hospitalized with clinical and radiographic CAP were enrolled at 3 US children’s hospitals. Neurologic disorders included cerebral palsy, developmental delay, Down syndrome, epilepsy, non-Down syndrome chromosomal abnormalities, and spinal cord abnormalities. We compared the epidemiology, etiology, and clinical outcomes of CAP in children with neurologic disorders with those with non-neurologic underlying conditions, and those with no underlying conditions using bivariate, age-stratified, and multivariate logistic regression analyses. Results From January 2010–June 2012, 2358 children with radiographically confirmed CAP were enrolled; 280 (11.9%) had a neurologic disorder (52.1% of these individuals also had non-neurologic underlying conditions), 934 (39.6%) had non-neurologic underlying conditions only, and 1144 (48.5%) had no underlying conditions. Children with neurologic disorders were older and more likely to require intensive care unit (ICU) admission than children with non-neurologic underlying conditions and children with no underlying conditions; similar proportions were mechanically ventilated. In age-stratified analysis, children with neurologic disorders were less likely to have a pathogen detected than children with non-neurologic underlying conditions. In multivariate analysis, having a neurologic disorder was associated with ICU admission for children ≥2 years of age. Conclusions Children with neurologic disorders hospitalized with CAP were less likely to have a pathogen detected and more likely to be admitted to the ICU than children without neurologic disorders. PMID:27017483

  17. Etiology of Attention Disorders: A Neurological/Genetic Perspective.

    ERIC Educational Resources Information Center

    Grantham, Madeline Kay

    This paper explores the historical origins of attention deficit disorder/attention deficit hyperactivity disorder (ADD/ADHD) as a neurological disorder, current neurological and genetic research concerning the etiology of ADD/ADHD, and implications for diagnosis and treatment. First, ADD/ADHD is defined and then the origins of ADD/ADHD as a…

  18. 76 FR 9587 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-02-18

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... personal privacy. Name of Committee: National Institute of Neurological Disorders and Stroke Initial Review...: National Institute of Neurological Disorders and Stroke Initial Review Group; Neurological Sciences...

  19. Immunotherapeutic approaches to paraneoplastic neurological disorders.

    PubMed

    Blaes, F

    2000-04-01

    Paraneoplastic neurological disorders (PNDs) are disturbances of the nervous system function in cancer patients, which are not due to a local effect of the tumour or its metastases. This review focuses on the neuromuscular PND and central nervous system (CNS) PND, which are by far the most problematic group to treat. Most of these clinically well-defined syndromes are associated with lung cancer, especially small cell lung cancer (SCLC), lymphoma and gynaecological tumours. Since auto-antibodies directed against proteins expressed in neurones and tumour cells have been found, PNDs are suspected to be autoimmune. In neuromuscular PND, immunosuppressive therapies, plasmapheresis and iv. immunoglobulins (iv. Ig) have proven to be effective. In PND of the CNS, therapy of the tumour itself or immunosuppressive drugs seem to have little effect on the neurological syndromes. Plasmapheresis reduces the auto-antibody titre in the sera of these patients, but does not lead to a clinical improvement. This review presents an overview on the pathogenesis of PND and the possible benefit of an early immunosuppressive or immunomodulatory treatment, especially treatment with iv. immunoglobulins. PMID:11060705

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

    PubMed Central

    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

  1. 76 FR 6625 - Neurological Devices Panel of the Medical Devices Advisory Committee; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-02-07

    ... HUMAN SERVICES Food and Drug Administration Neurological Devices Panel of the Medical Devices Advisory... Devices Advisory Committee. General Function of the Committee: To provide advice and recommendations to... Embolization Device (PED), sponsored by Chestnut Medical. The PED is indicated for the endovascular...

  2. Functional Neuroanatomy and Neurophysiology of Functional Neurological Disorders (Conversion Disorder).

    PubMed

    Voon, Valerie; Cavanna, Andrea E; Coburn, Kerry; Sampson, Shirlene; Reeve, Alya; LaFrance, W Curt

    2016-01-01

    Much is known regarding the physical characteristics, comorbid symptoms, psychological makeup, and neuropsychological performance of patients with functional neurological disorders (FNDs)/conversion disorders. Gross neurostructural deficits do not account for the patients' deficits or symptoms. This review describes the literature focusing on potential neurobiological (i.e. functional neuroanatomic/neurophysiological) findings among individuals with FND, examining neuroimaging and neurophysiological studies of patients with the various forms of motor and sensory FND. In summary, neural networks and neurophysiologic mechanisms may mediate "functional" symptoms, reflecting neurobiological and intrapsychic processes. PMID:26900733

  3. Hyperhomocysteinemia and Neurologic Disorders: a Review

    PubMed Central

    Ansari, Ramin; Mallack, Eric; Luo, Jin Jun

    2014-01-01

    Homocysteine (Hcy) is a sulfur-containing amino acid that is generated during methionine metabolism. It has a physiologic role in DNA metabolism via methylation, a process governed by the presentation of folate, and vitamins B6 and B12. Physiologic Hcy levels are determined primarily by dietary intake and vitamin status. Elevated plasma levels of Hcy (eHcy) can be caused by deficiency of either vitamin B12 or folate, or a combination thereof. Certain genetic factors also cause eHcy, such as C667T substitution of the gene encoding methylenetetrahydrofolate reductase. eHcy has been observed in several medical conditions, such as cardiovascular disorders, atherosclerosis, myocardial infarction, stroke, minimal cognitive impairment, dementia, Parkinson's disease, multiple sclerosis, epilepsy, and eclampsia. There is evidence from laboratory and clinical studies that Hcy, and especially eHcy, exerts direct toxic effects on both the vascular and nervous systems. This article provides a review of the current literature on the possible roles of eHcy relevant to various neurologic disorders. PMID:25324876

  4. 75 FR 55803 - Neurological Devices Panel of the Medical Devices Advisory Committee; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-09-14

    ... HUMAN SERVICES Food and Drug Administration Neurological Devices Panel of the Medical Devices Advisory...). The meeting will be open to the public. Name of Committee: Neurological Devices Panel of the Medical... a disability, please contact AnnMarie Williams, Conference Management Staff, at 301-796-5966...

  5. Neurologic Disorders in Immunocompetent Patients with Autochthonous Acute Hepatitis E

    PubMed Central

    Perrin, H. Blasco; Cintas, P.; Abravanel, F.; Gérolami, R.; d'Alteroche, L.; Raynal, J.-N.; Alric, L.; Dupuis, E.; Prudhomme, L.; Vaucher, E.; Couzigou, P.; Liversain, J.-M.; Bureau, C.; Vinel, J.-P.; Kamar, N.; Izopet, J.

    2015-01-01

    Neurologic disorders, mainly Guillain-Barré syndrome and Parsonage–Turner syndrome (PTS), have been described in patients with hepatitis E virus (HEV) infection in industrialized and developing countries. We report a wider range of neurologic disorders in nonimmunocompromised patients with acute HEV infection. Data from 15 French immunocompetent patients with acute HEV infection and neurologic disorders were retrospectively recorded from January 2006 through June 2013. The disorders could be divided into 4 main entities: mononeuritis multiplex, PTS, meningoradiculitis, and acute demyelinating neuropathy. HEV infection was treated with ribavirin in 3 patients (for PTS or mononeuritis multiplex). One patient was treated with corticosteroids (for mononeuropathy multiplex), and 5 others received intravenous immunoglobulin (for PTS, meningoradiculitis, Guillain-Barré syndrome, or Miller Fisher syndrome). We conclude that pleiotropic neurologic disorders are seen in HEV-infected immunocompetent patients. Patients with acute neurologic manifestations and aminotransferase abnormalities should be screened for HEV infection. PMID:26490255

  6. Control of Abnormal Synchronization in Neurological Disorders

    PubMed Central

    Popovych, Oleksandr V.; Tass, Peter A.

    2014-01-01

    In the nervous system, synchronization processes play an important role, e.g., in the context of information processing and motor control. However, pathological, excessive synchronization may strongly impair brain function and is a hallmark of several neurological disorders. This focused review addresses the question of how an abnormal neuronal synchronization can specifically be counteracted by invasive and non-invasive brain stimulation as, for instance, by deep brain stimulation for the treatment of Parkinson’s disease, or by acoustic stimulation for the treatment of tinnitus. On the example of coordinated reset (CR) neuromodulation, we illustrate how insights into the dynamics of complex systems contribute to successful model-based approaches, which use methods from synergetics, non-linear dynamics, and statistical physics, for the development of novel therapies for normalization of brain function and synaptic connectivity. Based on the intrinsic multistability of the neuronal populations induced by spike timing-dependent plasticity (STDP), CR neuromodulation utilizes the mutual interdependence between synaptic connectivity and dynamics of the neuronal networks in order to restore more physiological patterns of connectivity via desynchronization of neuronal activity. The very goal is to shift the neuronal population by stimulation from an abnormally coupled and synchronized state to a desynchronized regime with normalized synaptic connectivity, which significantly outlasts the stimulation cessation, so that long-lasting therapeutic effects can be achieved. PMID:25566174

  7. Functional neurological disorders in outpatient practice: An Australian cohort.

    PubMed

    Ahmad, Omar; Ahmad, Kate E

    2016-06-01

    Functional disorders are defined as neurological symptoms without causative organic pathology identified. They are a diverse and often neglected group of disorders. The aim of this was to determine the incidence and outcome of functional neurological disorders in an Australian neurology practice. Over a 17month period, all patients presenting to a single outpatient neurology service were evaluated to determine the incidence and outcome of these disorders. A total of 884 patients were assessed and of these, 137 had a final diagnosis of functional neurological illness, equating to an incidence of 15% of all patients seen. Functional disorders were the third most common presentation overall. Patients with functional disorders were younger, more likely to be female and had a higher rate of current psychiatric comorbidity compared to other neurology patients. Sensory symptoms were the most common manifestation (48%) followed by limb weakness (37%) and psychogenic non-epileptic seizures (14%). Outcome information was available for 49% of patients at an average of 3months follow-up. 45% had some improvement in their symptoms, 43% had static symptoms and 12% had worsening of symptoms. This study confirms the high incidence of functional disorders in outpatient neurology practice. Early improvement was seen in a substantial proportion of patients and is influenced by duration of symptoms. PMID:26754851

  8. Quantifying anatomical shape variations in neurological disorders.

    PubMed

    Singh, Nikhil; Fletcher, P Thomas; Preston, J Samuel; King, Richard D; Marron, J S; Weiner, Michael W; Joshi, Sarang

    2014-04-01

    proposed methodology thus holds promise for discovering new patterns of shape changes in the human brain that could add to our understanding of disease progression in neurological disorders. PMID:24667299

  9. Microbiota and Neurological Disorders: A Gut Feeling.

    PubMed

    Moos, Walter H; Faller, Douglas V; Harpp, David N; Kanara, Iphigenia; Pernokas, Julie; Powers, Whitney R; Steliou, Kosta

    2016-01-01

    In the past century, noncommunicable diseases have surpassed infectious diseases as the principal cause of sickness and death, worldwide. Trillions of commensal microbes live in and on our body, and constitute the human microbiome. The vast majority of these microorganisms are maternally derived and live in the gut, where they perform functions essential to our health and survival, including: digesting food, activating certain drugs, producing short-chain fatty acids (which help to modulate gene expression by inhibiting the deacetylation of histone proteins), generating anti-inflammatory substances, and playing a fundamental role in the induction, training, and function of our immune system. Among the many roles the microbiome ultimately plays, it mitigates against untoward effects from our exposure to the environment by forming a biotic shield between us and the outside world. The importance of physical activity coupled with a balanced and healthy diet in the maintenance of our well-being has been recognized since antiquity. However, it is only recently that characterization of the host-microbiome intermetabolic and crosstalk pathways has come to the forefront in studying therapeutic design. As reviewed in this report, synthetic biology shows potential in developing microorganisms for correcting pathogenic dysbiosis (gut microbiota-host maladaptation), although this has yet to be proven. However, the development and use of small molecule drugs have a long and successful history in the clinic, with small molecule histone deacetylase inhibitors representing one relevant example already approved to treat cancer and other disorders. Moreover, preclinical research suggests that epigenetic treatment of neurological conditions holds significant promise. With the mouth being an extension of the digestive tract, it presents a readily accessible diagnostic site for the early detection of potential unhealthy pathogens resident in the gut. Taken together, the data outlined

  10. Microbiota and Neurological Disorders: A Gut Feeling

    PubMed Central

    Moos, Walter H.; Faller, Douglas V.; Harpp, David N.; Kanara, Iphigenia; Pernokas, Julie; Powers, Whitney R.; Steliou, Kosta

    2016-01-01

    Abstract In the past century, noncommunicable diseases have surpassed infectious diseases as the principal cause of sickness and death, worldwide. Trillions of commensal microbes live in and on our body, and constitute the human microbiome. The vast majority of these microorganisms are maternally derived and live in the gut, where they perform functions essential to our health and survival, including: digesting food, activating certain drugs, producing short-chain fatty acids (which help to modulate gene expression by inhibiting the deacetylation of histone proteins), generating anti-inflammatory substances, and playing a fundamental role in the induction, training, and function of our immune system. Among the many roles the microbiome ultimately plays, it mitigates against untoward effects from our exposure to the environment by forming a biotic shield between us and the outside world. The importance of physical activity coupled with a balanced and healthy diet in the maintenance of our well-being has been recognized since antiquity. However, it is only recently that characterization of the host–microbiome intermetabolic and crosstalk pathways has come to the forefront in studying therapeutic design. As reviewed in this report, synthetic biology shows potential in developing microorganisms for correcting pathogenic dysbiosis (gut microbiota–host maladaptation), although this has yet to be proven. However, the development and use of small molecule drugs have a long and successful history in the clinic, with small molecule histone deacetylase inhibitors representing one relevant example already approved to treat cancer and other disorders. Moreover, preclinical research suggests that epigenetic treatment of neurological conditions holds significant promise. With the mouth being an extension of the digestive tract, it presents a readily accessible diagnostic site for the early detection of potential unhealthy pathogens resident in the gut. Taken together, the

  11. 78 FR 11898 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-02-20

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  12. 77 FR 33470 - National Institute of Neurological Disorders and Stroke Notice of Closed Meetings

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  13. 75 FR 5093 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

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  14. 77 FR 37421 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

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  15. 77 FR 65005 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

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  16. 75 FR 26268 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

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  17. 77 FR 2740 - National Institute of Neurological Disorders and Stroke Notice of Closed Meeting

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  18. 75 FR 16153 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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    2010-03-31

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  19. 76 FR 66732 - National Institute of Neurological Disorders and Stroke Notice of Closed Meetings

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  20. 78 FR 13359 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

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    2013-02-27

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  1. [Symptoms of obsessive-compulsive disorder in neurological diseases].

    PubMed

    Kutlubaev, M A

    2016-01-01

    Obsessive-compulsive disorder (OCD) is a common form of neurosis. Symptoms of OCD could develop as a sign of focal brain lesion, particularly in multiple sclerosis, extrapyramidal disorders, epilepsy, less frequently - in other diseases. Timely diagnosis and treatment of the symptoms of OCD is an important aspect in the management of mentioned neurological disorders. PMID:27240053

  2. Optogenetic cell control in experimental models of neurological disorders.

    PubMed

    Tønnesen, Jan

    2013-10-15

    The complexity of the brain, in which different neuronal cell types are interspersed and complexly interconnected, has posed a major obstacle in identifying pathophysiological mechanisms underlying prevalent neurological disorders. This is largely based in the inability of classical experimental approaches to target defined neural populations at sufficient temporal and spatial resolution. As a consequence, effective clinical therapies for prevalent neurological disorders are largely lacking. Recently developed optogenetic probes are genetically expressed photosensitive ion channels and pumps that in principal overcome these limitations. Optogenetic probes allow millisecond resolution functional control over selected optogenetically transduced neuronal populations targeted based on promoter activity. This optical cell control scheme has already been applied to answer fundamental questions pertaining to neurological disorders by allowing researchers to experimentally intercept, or induce, pathophysiological neuronal signaling activity in a highly controlled manner. Offering high temporal resolution control over neural activity at high cellular specificity, optogenetic tools constitute a game changer in research aiming at understanding pathophysiological signaling mechanisms in neurological disorders and in developing therapeutic strategies to correct these. In this regard, recent experimental work has provided new insights in underlying mechanisms, as well as preliminary proof-of-principle for optogenetic therapies, of several neurological disorders, including Parkinson's disease, epilepsy and progressive blindness. This review synthesizes experimental work where optogenetic tools have been applied to explore pathologic neural network activity in models of neurological disorders. PMID:23871610

  3. Bridging neuroanatomy, neuroradiology and neurology: three-dimensional interactive atlas of neurological disorders.

    PubMed

    Nowinski, W L; Chua, B C

    2013-06-01

    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. Normal neuroanatomy is divided into about 2,300 components, including the cerebrum, cerebellum, brainstem, spinal cord, arteries, veins, dural sinuses, tracts, cranial nerves (CN), white matter, deep gray nuclei, ventricles, visual system, muscles, glands and cervical vertebrae (C1-C5). The brain pathology database contains 144 focal and distributed synthesized lesions (70 vascular, 36 CN-related, and 38 regional anatomy-related), each lesion labeled with the resulting disorder and associated signs, symptoms, and/or syndromes compiled from materials reported in the literature. The initial view of each lesion was preset in terms of its location and size, surrounding surface and sectional (magnetic resonance) neuroanatomy, and labeling of lesion and neuroanatomy. In addition, a glossary of neurological disorders was compiled and for each disorder materials from textbooks were included to provide neurological description. This atlas of neurological disorders is potentially useful to a wide variety of users ranging from medical students, residents and nurses to general practitioners, neuroanatomists, neuroradiologists and neurologists, as it contains both normal (surface and sectional) brain anatomy and pathology correlated with neurological disorders presented in a visual and interactive way. PMID:23859280

  4. 77 FR 61768 - Neurological Devices Panel of the Medical Devices Advisory Committee; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-11

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES Food and Drug Administration Neurological Devices Panel of the Medical Devices Advisory Committee; Notice of Meeting AGENCY: Food and Drug Administration, HHS. ACTION: Notice. This notice announces a forthcoming meeting of a...

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

    PubMed

    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. PMID:15608775

  6. 77 FR 59939 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-01

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... personal privacy. Name of Committee: National Institute of Neurological Disorders and Stroke Initial Review... Neurological Disorders and Stroke Initial Review Group; Neurological Sciences and Disorders A. Date: November...

  7. 78 FR 59041 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-09-25

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... personal privacy. Name of Committee: National Institute of Neurological Disorders and Stroke Initial Review... Neurological Disorders and Stroke Initial Review, Group Neurological Sciences and Disorders A. Date: October...

  8. 75 FR 51279 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-08-19

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke...: National Institute of Neurological Disorders and Stroke Initial Review Group, Neurological Sciences and... Disorders and Stroke Initial Review Group, Neurological Sciences and Disorders K. Date: October 28-29,...

  9. [Neurological development disorders in very low birth weight newborns].

    PubMed

    Domínguez-Dieppa, F; Soriano-Puig, J A; Roca-Molina, M; Dueñas-Gómez, E

    1992-04-01

    Thirty very low birth weight newborns (less than 1,500 g) were evaluated at term from the neurological point of view, and were followed-up during the first 2 years of life by a multidisciplinary team, in order to detect long term sequelaes. There were 3 patients with major neurological disorders, and one third presented minor alterations. Neurological evaluation at term was a good sequelae predictor. No significative differences were found in perinatal variables: birthweight, gestational age, Apgar's score, ventilotherapy and with presence of intraventricular hemorrhage when normal outcome prematures were compared with those with neurological disorders. Blindness or deafness were not detected. It's important to continue the follow-up study up to the school age. PMID:1305391

  10. Drug treatment of vertigo in neurological disorders.

    PubMed

    Berisavac, Ivana I; Pavlović, Aleksandra M; Trajković, Jasna J Zidverc; Šternić, Nadežda M Čovičković; Bumbaširević, Ljiljana G Beslać

    2015-01-01

    Vertigo is a common symptom in everyday clinical practice. The treatment depends on the specific etiology. Vertigo may be secondary to inner ear pathology, or any existing brainstem or cerebellar lesion but may also be psychogenic. Central vertigo is a consequence of a central nervous system lesion. It is often associated with a focal neurological deficit. Peripheral vertigo is secondary to dysfunction of the peripheral vestibular system and is usually characterized by an acute vertigo with loss of balance, sensation of spinning in the space or around self, and is exaggerated with changes of the head and body position; no other neurological deficit is present. Some medications may also cause vertigo. Depending on the cause of the vertigo, drugs with different mechanisms of action, physical therapy, psychotherapy, as well as surgery may be used to combat this disabling malady. Symptomatic treatment has a particularly important role, regardless of the etiology of vertigo. We reviewed the current medications recommended for patients with vertigo, their mechanisms of action and their most frequent side effects. PMID:26588629

  11. The use of aminopyridines in neurological disorders.

    PubMed

    Sedehizadeh, Saam; Keogh, Michael; Maddison, Paul

    2012-01-01

    Aminopyridines are members of a family of monoamino and diamino derivatives of pyridine, and their principal mechanism of action is dose-dependent blockade of voltage-gated potassium channels, in particular, fast voltage-gated potassium channels. To date, only 2 main broad-spectrum potassium channel blockers, 4-aminopyridine (4-AP) and 3,4-diaminopyridine (3,4-DAP), have been used as investigational new drugs in various neurological diseases. More recently, licensed versions of these compounds including dalfampridine extended release (Fampyra, Biogen Idec) for the improvement of walking in adult patients with multiple sclerosis, and amifampridine (Firdapse, Biomarin Europe Ltd) for the treatment of Lambert-Eaton myasthenic syndrome have been released, and the costs associated with using these new products highlights the importance of evaluating the clinically meaningful treatment effects of these drugs.The current review summarizes the evidence of aminopyridine use in neurological conditions and in particular presents a systematic review of all randomized trials of 3,4-DAP in Lambert-Eaton myasthenic syndrome to determine the efficacy of this treatment using meta-analysis of clinical and electrophysiological end points. PMID:22805230

  12. The role of lipid peroxidation in neurological disorders

    PubMed Central

    Shichiri, Mototada

    2014-01-01

    There has been much evidence demonstrating the involvement of oxidative stress in the pathology of neurological disorders. Moreover, the vulnerability of the central nervous system to reactive oxygen species mediated injury is well established since neurons consume large amounts of oxygen, the brain has many areas containing high iron content, and neuronal mitochondria generate large amounts of hydrogen peroxide. Furthermore, neuronal membranes are rich in polyunsaturated fatty acids, which are particularly susceptible to oxidative stress. Recently, the biological roles of products produced by lipid peroxidation have received much attention, not only for their pathological mechanisms associated with neurological disorders, but also for their practical clinical applications as biomarkers. Here, we discuss the production mechanisms of reactive oxygen species in some neurological disorders, including Alzheimer’s disease, Down syndrome, Parkinson’s disease, and stroke. We also describe lipid peroxidation biomarkers for evaluating oxidative stress. PMID:24895477

  13. [Pediatric neurological disorders and genetic counseling].

    PubMed

    Fukushima, Yoshimitsu

    2003-07-01

    Genetic counseling provides medical and genetic information of the disease including its natural history, recurrence risk, availability and usefulness of genetic testing, as well as psycho-social support. In the field of pediatric neurology, the majority of genetic counseling seems to be a simple risk estimation of the next child and unrelated to ethical issues. However, in some cases requiring prenatal diagnosis or presymptomatic testing, we have to address serious ethical issues. Genetic counseling should be provided by an educated medical doctor at a suitable genetics clinic. In Japan, we have "Japanese Board of Medical Genetics, Clinical Geneticist" as an education system of clinical genetics for medical doctors. Pediatric neurologists should know the special issues of genetic information and contact with clinical geneticists in selected cases. PMID:12875203

  14. Revised Medical Criteria for Evaluating Neurological Disorders. Final rule.

    PubMed

    2016-07-01

    We are revising the criteria in the Listing of Impairments (listings) that we use to evaluate disability claims involving neurological disorders in adults and children under titles II and XVI of the Social Security Act (Act). These revisions reflect our program experience; advances in medical knowledge, treatment, and methods of evaluating neurological disorders; comments we received from medical experts and the public at an outreach policy conference; responses to an advance notice of proposed rulemaking (ANPRM); and public comments we received in response to a Notice of Proposed Rulemaking (NPRM) and a Federal Register notice that reopened the NPRM comment period. PMID:27373016

  15. Neurological implications of urea cycle disorders

    PubMed Central

    Summar, M.; Leonard, J. V.

    2013-01-01

    Summary The urea cycle disorders constitute a group of rare congenital disorders caused by a deficiency of the enzymes or transport proteins required to remove ammonia from the body. Via a series of biochemical steps, nitrogen, the waste product of protein metabolism, is removed from the blood and converted into urea. A consequence of these disorders is hyperammonaemia, resulting in central nervous system dysfunction with mental status changes, brain oedema, seizures, coma, and potentially death. Both acute and chronic hyperammonaemia result in alterations of neurotransmitter systems. In acute hyperammonaemia, activation of the NMDA receptor leads to excitotoxic cell death, changes in energy metabolism and alterations in protein expression of the astrocyte that affect volume regulation and contribute to oedema. Neuropathological evaluation demonstrates alterations in the astrocyte morphology. Imaging studies, in particular 1H MRS, can reveal markers of impaired metabolism such as elevations of glutamine and reduction of myoinositol. In contrast, chronic hyperammonaemia leads to adaptive responses in the NMDA receptor and impairments in the glutamate–nitric oxide–cGMP pathway, leading to alterations in cognition and learning. Therapy of acute hyperammonaemia has relied on ammonia-lowering agents but in recent years there has been considerable interest in neuroprotective strategies. Recent studies have suggested restoration of learning abilities by pharmacological manipulation of brain cGMP with phosphodiesterase inhibitors. Thus, both strategies are intriguing areas for potential investigation in human urea cycle disorders. PMID:18038189

  16. 77 FR 65896 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-31

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... personal privacy. Name of Committee: National Institute of Neurological Disorders and Stroke Special... Disorders and Stroke Special Emphasis Panel; Summer Research Experience Programs (R25). Date: November...

  17. [Neurologic disorders in acute dichloroethane poisoning].

    PubMed

    Akimov, G A; Buchko, V M; Kolesnichenko, I P

    1978-01-01

    The paper deals with a study of the nervous system in 121 patients with acute poisening with dichlorethane. Among the studied contingent there were 110 males and 11 females. According to the severity of the intoxication the patients were divided into 3 groups: mild--23 cases, moderate--11 cases, severe--87 cases. The following 6 neurological syndromes were distinguished: comatose, convulsive, atactic, extrapyramidal, psychotic and asthenic with vegetative-vascular insufficiency. Morphological studies detected the following: congestion plethora, vascular dystonia, microfoci hemorrhages, acute swelling of the nervous cells with signs of chromatolyses, shrunk cells, severe and ischemic change of the nervous cells. The treatment consisted in an accelerated elimination of dichlorethane from the organism and symptomatic therapy. The results of these studies demonstrated that in poisoning with dichlorethane there were diffuse, mainly dystrophic changes in the cells of the brain and spinal cord, which clinically may be expressed by symptoms of a lesion of many systems and may be qualified as toxic encephalomyelopathy. PMID:665065

  18. Functional (Psychogenic) Cognitive Disorders: A Perspective from the Neurology Clinic.

    PubMed

    Stone, Jon; Pal, Suvankar; Blackburn, Daniel; Reuber, Markus; Thekkumpurath, Parvez; Carson, Alan

    2015-09-24

    Cognitive symptoms such as poor memory and concentration represent a common cause of morbidity among patients presenting to general practitioners and may result in referral for a neurological opinion. In many cases, these symptoms do not relate to an underlying neurological disease or dementia. In this article we present a personal perspective on the differential diagnosis of cognitive symptoms in the neurology clinic, especially as this applies to patients who seek advice about memory problems but have no neurological disease process. These overlapping categories include the following 'functional' categories: 1) cognitive symptoms as part of anxiety or depression; 2) "normal" cognitive symptoms that become the focus of attention; 3) isolated functional cognitive disorder in which symptoms are outwith 'normal' but not explained by anxiety; 4) health anxiety about dementia; 5) cognitive symptoms as part of another functional disorder; and 6) retrograde dissociative (psychogenic) amnesia. Other 'non-dementia' diagnoses to consider in addition are 1) cognitive symptoms secondary to prescribed medication or substance misuse; 2) diseases other than dementia causing cognitive disorders; 3) patients who appear to have functional cognitive symptoms but then go on to develop dementia/another neurological disease; and finally 4) exaggeration/malingering. We discuss previous attempts to classify the problem of functional cognitive symptoms, the importance of making a positive diagnosis for the patient, and the need for large cohort studies to better define and manage this large group of patients. PMID:26445274

  19. Secondary Abnormalities of Neurotransmitters in Infants with Neurological Disorders

    ERIC Educational Resources Information Center

    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…

  20. Collaborative Screening of Psychiatric and Language Disorders in Pediatric Neurology.

    ERIC Educational Resources Information Center

    Spratt, Eve G.; Anderson, Deborah; Pagano, Maria; Macias, Michelle

    This study evaluated 102 patients (ages 5 to 13) at the Medical University of South Carolina's Pediatric Neurology Clinic for incidence of psychiatric or language disorders. Parents completed the Pediatric Symptom Checklist (PSC), the Child Behavior Checklist, and the Language Problems Scale; phone interviews were conducted to determine child…

  1. Minor Neurological Dysfunction in Children with Autism Spectrum Disorder

    ERIC Educational Resources Information Center

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

    2011-01-01

    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 705 children (513 males, 192 females; mean age…

  2. The Neurological Basis of Attention Deficit Hyperactivity Disorder.

    ERIC Educational Resources Information Center

    Ballard, Shirley; Bolan, Morna; Burton, Michael; Snyder, Sherry; Pasterczyk-Seabolt, Claire; Martin, Don

    1997-01-01

    Reviews research on attention deficit hyperactivity disorder (ADHD) and examines the role of neurochemical stimulation and signs of neurological deficits. Describes the chemical action of drugs used to treat ADHD, along with cognitive, affective, and behavioral effects, and side effects. Elaborates on drug treatment and basic behavior modification…

  3. Are neurological and psychiatric disorders different?†

    PubMed Central

    David, Anthony S.; Nicholson, Timothy

    2015-01-01

    There have been recent calls to abandon the distinction between neurological and psychiatric disorders on philosophical and moral grounds. Crossley and colleagues, in this issue, meta-analyse published structural brain imaging data and prove that they are different after all – or do they? PMID:26527662

  4. Mesenchymal Stem Cells as Cellular Vectors for Pediatric Neurological Disorders

    PubMed Central

    Phinney, Donald G.; Isakova, Iryna A.

    2014-01-01

    Lysosomal storage diseases are a heterogeneous group of hereditary disorders characterized by a deficiency in lysosomal function. Although these disorders differ in their etiology and phenotype those that affect the nervous system generally manifest as a profound deterioration in neurologic function with age. Over the past several decades implementation of various treatment regimens including bone marrow and cord blood cell transplantation, enzyme replacement, and substrate reduction therapy have proved effective for managing some clinical manifestations of these diseases but their ability to ameliorate neurologic complications remains unclear. Consequently, there exists a need to develop alternative therapies that more effectively target the central nervous system. Recently, direct intracranial transplantation of tissue-specific stem and progenitor cells has been explored as a means to reconstitute metabolic deficiencies in the CNS. In this chapter we discuss the merits of bone marrow-derived mesenchymal stem cells (MSCs) for this purpose. Originally identified as progenitors of connective tissue cell lineages, recent findings have revealed several novel aspects of MSC biology that make them attractive as therapeutic agents in the CNS. We relate these advances in MSC biology to their utility as cellular vectors for treating neurologic sequelae associated with pediatric neurologic disorders. PMID:24858930

  5. AMPA Receptors as Therapeutic Targets for Neurological Disorders.

    PubMed

    Lee, Kevin; Goodman, Lucy; Fourie, Chantelle; Schenk, Susan; Leitch, Beulah; Montgomery, Johanna M

    2016-01-01

    Almost every neurological disease directly or indirectly affects synapse function in the brain. However, these diseases alter synapses through different mechanisms, ultimately resulting in altered synaptic transmission and/or plasticity. Glutamate is the major neurotransmitter that mediates excitatory synaptic transmission in the brain through activation of alpha-amino-3-hydroxy-5-methyl-4-isoxazole-propionate (AMPA) receptors. These receptors have therefore been identified as a target for the development of therapeutic treatments for neurological disorders including epilepsy, neurodegenerative diseases, autism, and drug addiction. The fact that AMPA receptors play a dominant role throughout the brain raises the significant challenge of selectively targeting only those regions affected by disease, and clinical trials have raised doubt regarding the feasibility of specifically targeting AMPA receptors for new therapeutic options. Benzamide compounds that act as positive allosteric AMPA receptor modulators, known as AMPAkines, can act on specific brain regions and were initially proposed to revolutionize the treatment of cognitive deficits associated with neurological disorders. Their therapeutic potential has since declined due to inconsistent results in clinical trials. However, recent advances in basic biomedical research are significantly increasing our knowledge of AMPA receptor structure, binding sites, and interactions with auxiliary proteins. In particular, the large complex of postsynaptic proteins that interact with AMPA receptor subunits have been shown to control AMPA receptor insertion, location, pharmacology, synaptic transmission, and plasticity. These proteins are now being considered as alternative therapeutic target sites for modulating AMPA receptors in neurological disorders. PMID:26920691

  6. Neurological and psychiatric disorders as a neuroglial failure

    PubMed Central

    VERKHRATSKY, ALEXEI; PARPURA, VLADIMIR

    2014-01-01

    Neuroglia are a diverse non-neuronal population of cells in the central and peripheral nervous system. These cells have a variety of functions that can all be summed up as the maintenance of homeostasis of the nervous system. It is the loss of homeostasis that represents the culprit of all disorders. Thus, neuroglia can be envisioned as the pivotal element in all neural disorders, be that neurological or psychiatric. In this review, we discuss the role of glia in homeostasis and defence of the nervous system as well as changes in the morpho-functional characteristics of these cells in various disorders. PMID:25544781

  7. The role of CELF proteins in neurological disorders.

    PubMed

    Gallo, Jean-Marc; Spickett, Carl

    2010-01-01

    CELF (CUG-BP and ETR-3-like factors) proteins are structurally related RNA-binding proteins involved in various aspects of RNA processing including splicing and mRNA stability. The first member of the family, CELF1/CUG-BP1, was identified through its role in myotonic dystrophy, type 1. Several recent studies have uncovered the recurrent implication, to various extents, of CELF proteins or of the functionally related muscleblind-like 1 protein in a number of neurological conditions. This is particularly clear for inherited neurodegenerative disorders caused by expansions of translated or untranslated triplet repeats in the causative gene. Here we review the role played by CELF proteins, at least as modifiers of the pathological phenotype, in a number of neurological diseases. The involvement of CELF proteins suggest that individual pathogenic pathways in a number of neurological conditions overlap at the level of RNA processing. PMID:20622515

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

    PubMed Central

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

    2015-01-01

    Summary 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. PMID:26336632

  9. 77 FR 70791 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

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    2012-11-27

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  10. 76 FR 369 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

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  11. 75 FR 4577 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  12. 76 FR 18230 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  13. 76 FR 66730 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  15. 76 FR 10038 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  16. 77 FR 45644 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  17. 75 FR 20370 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  18. 77 FR 43343 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  19. 76 FR 47595 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  20. 78 FR 24763 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  1. 78 FR 24764 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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    2013-04-26

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  2. 78 FR 45933 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  3. 78 FR 42528 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  4. 75 FR 49501 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

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  5. 76 FR 25702 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  6. 75 FR 30409 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

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  7. 76 FR 34716 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  8. 75 FR 54162 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  9. 78 FR 18996 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  10. 77 FR 35987 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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    2012-06-15

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  11. 78 FR 66372 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

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  12. 78 FR 15727 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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  13. 78 FR 77476 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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    2013-12-23

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  14. 78 FR 17420 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

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    2013-03-21

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  15. Large Animal Models of Neurological Disorders for Gene Therapy

    PubMed Central

    Gagliardi, Christine; Bunnell, Bruce A.

    2009-01-01

    The development of therapeutic interventions for genetic disorders and diseases that affect the central nervous system (CNS) has proven challenging. There has been significant progress in the development of gene therapy strategies in murine models of human disease, but gene therapy outcomes in these models do not always translate to the human setting. Therefore, large animal models are crucial to the development of diagnostics, treatments, and eventual cures for debilitating neurological disorders. This review focuses on the description of large animal models of neurological diseases such as lysosomal storage diseases, Parkinson’s disease, Huntington’s disease, and neuroAIDS. The review also describes the contributions of these models to progress in gene therapy research. PMID:19293458

  16. Telomere shortening in neurological disorders: an abundance of unanswered questions.

    PubMed

    Eitan, Erez; Hutchison, Emmette R; Mattson, Mark P

    2014-05-01

    Telomeres, ribonucleoprotein complexes that cap eukaryotic chromosomes, typically shorten in leukocytes with aging. Aging is a primary risk factor for neurodegenerative disease (ND), and a common assumption has arisen that leukocyte telomere length (LTL) can serve as a predictor of neurological disease. However, the evidence for shorter LTL in Alzheimer's and Parkinson's patients is inconsistent. The diverse causes of telomere shortening may explain variability in LTL between studies and individuals. Additional research is needed to determine whether neuronal and glial telomeres shorten during aging and in neurodegenerative disorders, if and how LTL is related to brain cell telomere shortening, and whether telomere shortening plays a causal role in or exacerbates neurological disorders. PMID:24698125

  17. Telomere Shortening in Neurological Disorders: An Abundance of Unanswered Questions

    PubMed Central

    Eitan, Erez; Hutchison, Emmette R.; Mattson, Mark P.

    2014-01-01

    Telomeres, ribonucleoprotein complexes that cap eukaryotic chromosomes, typically shorten in leukocytes with aging. Aging is a primary risk factor for neurodegenerative disease (ND), and a common assumption has arisen that leukocyte telomere length (LTL) can serve as a predictor of neurological disease. However, the evidence for shorter LTL in Alzheimer’s and Parkinson’s patients is inconsistent. The diverse causes of telomere shortening may explain variability in LTL between studies and individuals. Additional research is needed to determine whether neuronal and glial telomeres shorten during aging and in neurodegenerative disorders, if and how LTL is related to brain cell telomere shortening, and whether telomere shortening plays a causal role in or exacerbates neurological disorders. PMID:24698125

  18. Evaluating suspected work-related neurologic disorders (clinical diagnosis).

    PubMed

    Lotti, Marcello; Aminoff, Michael J

    2015-01-01

    The clinical diagnosis of work-related neurologic disorders is essentially one of exclusion because symptoms and signs are often nonspecific. The clinical reasoning requires a three-step approach: (1) establish the characteristics of the presenting disease; (2) ascertain that observed clinical features are consistent with those caused by the suspected agent(s); and (3) assess occupational exposures. A detailed history is of paramount importance in evaluating patients with suspected work-related neurologic disorders as it is in other clinical contexts, especially because in some circumstances it may represent the only criterion to establish causality. Thus, besides characterization of neurologic symptoms, including their location, quality, timecourse, and possible other associated symptoms, the work environment of the patient should be understood in full detail. In this respect, when a neurotoxin is suspected, then the history collection can be guided by the knowledge of the likely syndromes it produces. Similarly, physical examination should be directed to the target of toxicity/entrapment based on information from the work history. Although specific sites and elements of the nervous system may be affected depending on the offending agent, most neurotoxic disorders are characterized by generalized rather than focal neurologic abnormalities. Laboratory toxicologic tests have limited application for the etiologic diagnosis of neurotoxic disorders, except in cases of acute poisoning and in patients exposed to neurotoxic chemicals with prolonged half-life. In most cases examination takes place after the end of exposure, when the offending chemical is no longer detectable in body fluids. Electrophysiologic studies, in particular evoked potentials, electromyography, and conduction velocities, are important to confirm the organic basis of symptoms, particularly to detect subclinical or early neurologic involvement and to reduce the number of disorders to be considered in

  19. Auditory brainstem responses (ABR) in children with neurological disorders.

    PubMed

    el Khateeb, I; Abdul Razzak, B; Moosa, A

    1988-01-01

    Auditory brainstem responses (ABR) were studied in 35 children with neurological disorders and 24 controls. Abnormal results were obtained in 16 patients. All 5 of the patients with metachromatic leukodystrophy had evidence of peripheral and/or central delay in transmission in keeping with evidence of demyelination of both peripheral (i.e. auditory nerve) and central (i.e. brainstem) pathways as occurs in this disorder. Two children with lead poisoning had delayed conduction in the peripheral pathways only and in these there was good correlation between the degree of delay and the ulnar nerve conduction velocity; both improved after chelation therapy. One infant with lead poisoning had central delay only. One infant with osteopetrosis manifested progressive damage to the auditory nerves. Delayed conduction was also found in one patient each with hydrocephalus, spinal muscular atrophy, and in 2 infants with cerebral palsy. No responses were obtained in one infant with congenital rubella, one deaf-mute and one child with an undiagnosed degenerative neurological disease. Auditory brainstem responses are of value in detecting disturbances of the auditory nerve or brainstem in children with various neurological disorders. PMID:3218703

  20. 76 FR 57062 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

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    2011-09-15

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... personal privacy. Name of Committee: National Institute of Neurological Disorders and Stroke Initial Review... Neurological Disorders and Stroke Initial Review Group, NST-2 Subcommittee. Date: October 31-November 1,...

  1. Gene Therapy for the Treatment of Neurological Disorders: Metabolic Disorders

    PubMed Central

    Gessler, Dominic J.; Gao, Guangping

    2016-01-01

    Metabolic disorders comprise a large group of heterogeneous diseases ranging from very prevalent diseases such as diabetes mellitus to rare genetic disorders like Canavan Disease. Whether either of these diseases is amendable by gene therapy depends to a large degree on the knowledge of their pathomechanism, availability of the therapeutic gene, vector selection, and availability of suitable animal models. In this book chapter, we review three metabolic disorders of the central nervous system (CNS; Canavan Disease, Niemann–Pick disease and Phenylketonuria) to give examples for primary and secondary metabolic disorders of the brain and the attempts that have been made to use adeno-associated virus (AAV) based gene therapy for treatment. Finally, we highlight commonalities and obstacles in the development of gene therapy for metabolic disorders of the CNS exemplified by those three diseases. PMID:26611604

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

    PubMed

    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

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

    PubMed Central

    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

  4. Neurology Academic Advisory Committee: a strategy for faculty retention and advancement.

    PubMed

    Schenkenberg, T; Foster, N L; Bromberg, M B; DeWitt, L D; Flanigan, K M

    2011-08-16

    Major effort and expense are devoted to faculty recruitment. Subsequent direction, support, and guidance of faculty members for retention and academic advancement are often inconsistent and ineffective. Individual mentorship is widely endorsed as an important element in advancement but often does not occur or is uneven in its pragmatic benefit. We formed a Departmental Academic Advisory Committee to provide individualized advice and guidance about career development and institutional promotion, retention, and tenure procedures. To assess the effectiveness of this process, a survey was sent to faculty members. A 100% response rate was achieved. The results of the survey demonstrated high levels of acceptance by faculty members and described benefits experienced by faculty, including better understanding of promotion and tenure policies and specific actions taken to achieve professional goals. An academic advisory committee can be a valuable adjunct to individual mentorship and to meetings with department chairs to enhance faculty satisfaction and advancement of neurology faculty members. PMID:21795659

  5. Zika virus-associated neurological disorders: a review.

    PubMed

    Araujo, Abelardo Q C; Silva, Marcus Tulius T; Araujo, Alexandra P Q C

    2016-08-01

    Zika virus, an arbovirus transmitted by mosquitoes of the Aedes species, is now rapidly disseminating throughout the Americas and the ongoing Brazilian outbreak is the largest Zika virus epidemic so far described. In addition to being associated with a non-specific acute febrile illness, a number of neurological manifestations, mainly microcephaly and Guillain-Barré syndrome, have been associated with infection. These with other rarer neurological conditions suggest that Zika virus, similar to other flaviviruses, is neuropathogenic. The surge of Zika virus-related microcephaly cases in Brazil has received much attention and the role of the virus in this and in other neurological manifestations is growing. Zika virus has been shown to be transmitted perinatally and the virus can be detected in amniotic fluid, placenta and foetus brain tissue. A significant increase in Guillain-Barré syndrome incidence has also been reported during this, as well as in previous outbreaks. More recently, meningoencephalitis and myelitis have also been reported following Zika virus infection. In summary, while preliminary studies have suggested a clear relationship between Zika virus infection and certain neurological conditions, only longitudinal studies in this epidemic, as well as experimental studies either in animal models or in vitro, will help to better understand the role of the virus and the pathogenesis of these disorders. PMID:27357348

  6. Biological factors underlying sex differences in neurological disorders.

    PubMed

    Loke, Hannah; Harley, Vincent; Lee, Joohyung

    2015-08-01

    The prevalence, age of onset, pathophysiology, and symptomatology of many neurological and neuropsychiatric conditions differ significantly between males and females. Females suffer more from mood disorders such as depression and anxiety, whereas males are more susceptible to deficits in the dopamine system including Parkinson's disease (PD), attention-deficit hyperactivity disorder (ADHD), schizophrenia, and autism spectrum disorders (ASD). Until recently, these sex differences have been explained solely by the neuroprotective actions of sex hormones in females. Emerging evidence however indicates that the sex chromosome genes (i.e. X- and Y-linked genes) also contribute to brain sex differences. In particular, the Y-chromosome gene, SRY (Sex-determining Region on the Y chromosome) is an interesting candidate as it is expressed in dopamine-abundant brain regions, where it regulates dopamine biosynthesis and dopamine-mediated functions such as voluntary movement in males. Furthermore, SRY expression is dysregulated in a toxin-induced model of PD, suggesting a role for SRY in the pathogenesis of dopamine cells. Taken together, these studies highlight the importance of understanding the interplay between sex-specific hormones and sex-specific genes in healthy and diseased brain. In particular, better understanding of regulation and function of SRY in the male brain could provide entirely novel and important insights into genetic factors involved in the susceptibility of men to neurological disorders, as well as development of novel sex-specific therapies. PMID:26028290

  7. Neurological signs in congenital iodine-deficiency disorder (endemic cretinism).

    PubMed

    DeLong, G R; Stanbury, J B; Fierro-Benitez, R

    1985-06-01

    Neurological examinations were made of 67 children and adults with congenital iodine-deficiency disorder (endemic cretinism) in four rural villages in highland Ecuador. There was a distinct and readily identifiable pattern of neurological deficits. These included, to varying degrees: deaf-mutism or lesser degrees of bilateral hearing-loss or dysarthria; spasticity, particularly involving the proximal lower extremities; mental deficiency of a characteristic type; and rigidity and bradykinesia. Not all of these elements were found in all cases. Less common features were strabismus, kyphoscoliosis and frontal-lobe signs. There were exceptional cases with hypotonia. In contrast, cerebellar function was largely spared, as were functions of emotion and attention, vegetative and autonomic functions, social interaction, and probably memory, except in the most severely involved. PMID:4018426

  8. Metabolic phenotyping and systems biology approaches to understanding neurological disorders.

    PubMed

    Dumas, Marc-Emmanuel; Davidovic, Laetitia

    2013-01-01

    The development of high-throughput metabolic profiling and the study of the metabolome are particularly important in brain research where small molecules or metabolites play fundamental signalling roles: neurotransmitters, signalling lipids, osmolytes and even ions. Metabolic profiling has shown that metabolic perturbations in the brain go beyond alterations of neurotransmission and that variations in brain metabolic homeostasis are associated with neurological disorders. In this report, we will focus on recent developments in the field of metabolic phenotyping that have contributed to unravelling the pathophysiology of neurological diseases. Also, we will highlight the necessity of implementing systems biology approaches to integrate metabolic data and tackle the structural and functional complexity of the brain in normal and pathological conditions. PMID:23755365

  9. Potroom palsy? Neurologic disorder in three aluminum smelter workers.

    PubMed

    Longstreth, W T; Rosenstock, L; Heyer, N J

    1985-11-01

    We studied three patients with a progressive neurologic disorder, all of whom had worked for over 12 years in the same potroom of an aluminum smelting plant. All had incoordination and an intention tremor. Two of the three patients had cognitive deficits, and the most severely affected patient also had spastic paraparesis. None had involvement of the peripheral nervous system. Despite extensive evaluations, the cause of these patients' problems remains obscure. It is tempting to implicate one of the numerous substances to which the patients were exposed in the potroom, but none is known to cause the neurologic problems seen in these patients. Neurotoxic effects of aluminum in animals are directed at the central nervous system, and theoretically long-term low-level exposure to aluminum in the potroom could explain the findings in our patients. PMID:4062445

  10. Potroom palsy. Neurologic disorder in three aluminum smelter workers

    SciTech Connect

    Longstreth, W.T. Jr.; Rosenstock, L.; Heyer, N.J.

    1985-11-01

    The authors studied three patients with a progressive neurologic disorder, all of whom had worked for over 12 years in the same potroom of an aluminum smelting plant. All had incoordination and an intention tremor. Two of the three patients had cognitive deficits, and the most severely affected patient also had spastic paraparesis. None had involvement of the peripheral nervous system. Despite extensive evaluations, the cause of these patients problems remains obscure. It is tempting to implicate one of the numerous substances to which the patients were exposed in the potroom, but none is known to cause the neurologic problems seen in these patients. Neurotoxic effects of aluminum in animals are directed at the central nervous system, and theoretically long-term low-level exposure to aluminum in the potroom could explain the findings in our patients.

  11. Sleep loss as risk factor for neurologic disorders: a review.

    PubMed

    Palma, Jose-Alberto; Urrestarazu, Elena; Iriarte, Jorge

    2013-03-01

    Sleep loss refers to sleep of shorter duration than the average baseline need of seven to eight hours per night. Sleep loss and sleep deprivation have severe effects on human health. In this article, we review the main aspects of sleep loss, taking into account its effects on the central nervous system. The neurocognitive and behavioral effects of sleep loss are well known. However, there is an increasing amount of research pointing to sleep deprivation as a risk factor for neurologic diseases, namely stroke, multiple sclerosis, Alzheimer's disease, headache, epilepsy, pain, and somnambulism. Conversely, sleep loss has been reported to be a potential protective factor against Parkinson's disease. The pathophysiology involved in this relationship is multiple, comprising immune, neuroendocrine, autonomic, and vascular mechanisms. It is extremely important to identify the individuals at risk, since recognition and adequate treatment of their sleep problems may reduce the risk of certain neurologic disorders. PMID:23352029

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

    PubMed

    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. PMID:25307589

  13. Induced pluripotent stem cells for modeling neurological disorders.

    PubMed

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

    2015-12-24

    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

  14. Connectivity Analysis is Essential to Understand Neurological Disorders

    PubMed Central

    Rowe, James B.

    2010-01-01

    Neurological and neuropsychiatric disorders are major causes of morbidity worldwide. A systems level analysis including functional and structural neuroimaging is particularly useful when the pathology leads to disorders of higher order cognitive functions in human patients. However, an analysis that is restricted to regional effects is impoverished and insensitive, compared to the analysis of distributed brain networks. We discuss the issues to consider when choosing an appropriate connectivity method, and compare the results from several different methods that are relevant to fMRI and PET data. These include psychophysiological interactions in general linear models, structural equation modeling, dynamic causal modeling, and independent components analysis. The advantages of connectivity analysis are illustrated with a range of structural and neurodegenerative brain disorders. We illustrate the sensitivity of these methods to the presence or severity of disease and/or treatment, even where analyses of voxel-wise activations are insensitive. However, functional and structural connectivity methods should be seen as complementary to, not a substitute for, other imaging and behavioral approaches. The functional relevance of changes in connectivity, to motor or cognitive performance, are considered alongside the complex relationship between structural and functional changes and neuropathology. Finally some of the problems associated with connectivity analysis are discussed. We suggest that the analysis of brain connectivity is an essential complement to the analysis of regionally specific dysfunction, in order to understand neurological and neuropsychiatric disease, and to evaluate the mechanisms of effective therapies. PMID:20948582

  15. Induced pluripotent stem cells for modeling neurological disorders

    PubMed Central

    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

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

    PubMed Central

    Cherney, Leora R.; van Vuuren, Sarel

    2013-01-01

    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 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’s 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. PMID:22851346

  17. Vestibular evoked myogenic potentials (VEMPs) in central neurological disorders.

    PubMed

    Venhovens, J; Meulstee, J; Verhagen, W I M

    2016-01-01

    Several types of acoustic stimulation (i.e. tone bursts or clicks), bone-conducted vibration, forehead taps, and galvanic stimulation elicit myogenic potentials. These can be recorded in cervical and ocular muscles, the so called vestibular evoked myogenic potentials (VEMPs). The cervical VEMP (cVEMP) resembles the vestibulo-collic reflex and the responses can be recorded from the ipsilateral sternocleidomastoid muscle. The ocular VEMP resembles the vestibulo-ocular reflex and can be recorded from extra-ocular muscles by a surface electrode beneath the contralateral infraorbital margin. Initially, the literature concerning VEMPs was limited to peripheral vestibular disorders, however, the field of VEMP testing is rapidly expanding, with an increasing focus on central neurological disorders. The current literature concerning VEMP abnormalities in central neurological disorders is critically reviewed, especially regarding the methodological aspects in relation to quality as well as the clinical interpretation of the VEMP results. Suggestions for further research are proposed as well as some clinically useful indications. PMID:25649969

  18. Voltage-gated sodium channels in neurological disorders.

    PubMed

    Chahine, Mohamed; Chatelier, Aurélien; Babich, Olga; Krupp, Johannes J

    2008-04-01

    Voltage-gated sodium channels play an essential biophysical role in many excitable cells such as neurons. They transmit electrical signals through action potential (AP) generation and propagation in the peripheral (PNS) and central nervous systems (CNS). Each sodium channel is formed by one alpha-subunit and one or more beta-subunits. There is growing evidence indicating that mutations, changes in expression, or inappropriate modulation of these channels can lead to electrical instability of the cell membrane and inappropriate spontaneous activity observed during pathological states. This review describes the biochemical, biophysical and pharmacological properties of neuronal voltage-gated sodium channels (VGSC) and their implication in several neurological disorders. PMID:18537643

  19. Stem cells for the treatment of neurological disorders

    NASA Astrophysics Data System (ADS)

    Lindvall, Olle; Kokaia, Zaal

    2006-06-01

    Many common neurological disorders, such as Parkinson's disease, stroke and multiple sclerosis, are caused by a loss of neurons and glial cells. In recent years, neurons and glia have been generated successfully from stem cells in culture, fuelling efforts to develop stem-cell-based transplantation therapies for human patients. More recently, efforts have been extended to stimulating the formation and preventing the death of neurons and glial cells produced by endogenous stem cells within the adult central nervous system. The next step is to translate these exciting advances from the laboratory into clinically useful therapies.

  20. The neurological basis of attention deficit hyperactivity disorder.

    PubMed

    Ballard, S; Bolan, M; Burton, M; Snyder, S; Pasterczyk-Seabolt, C; Martin, D

    1997-01-01

    Attention deficit hyperactivity disorder (ADHD) is a serious disability with long-term consequences. At present the disorder is considered organic in pathology, particularly in regard to central nervous system functioning. This paper reviews research on ADHD. The role of neurochemical stimulation is discussed, and the signs of neurological deficits are explored. Nearly 600,000 young people in the United States receive medication daily for ADHD, and these drugs mimic brain neurotransmitters. The chemical action of these drugs and the cognitive, affective, and behavioral effects are discussed. Side effects and dosage levels are also examined. Basic behavior modification with ADHD children and how these techniques can be combined with effective drug treatment are elaborated. PMID:9426808

  1. Neurological soft signs in homicidal men with antisocial personality disorder.

    PubMed

    Lindberg, Nina; Tani, Pekka; Stenberg, Jan-Henry; Appelberg, Björn; Porkka-Heiskanen, Tarja; Virkkunen, Matti

    2004-11-01

    Neurological soft signs (NSS) are characterized by abnormalities in motor, sensory, and integrative functions. NSS have been regarded as a result of neurodevelopmental dysfunction, and as evidence of a central nervous system defect, resulting in considerable sociopsychological dysfunction. During the last decade there has been growing evidence of brain dysfunction in severe aggressive behavior. As a symptom, aggression overlaps a number of psychiatric disorders, but it is commonly associated with antisocial personality disorder. The aim of the present study was to examine NSS in an adult criminal population using the scale by Rossi et al. [29]. Subjects comprised 14 homicidal men with antisocial personality disorder recruited from a forensic psychiatric examination. Ten age- and gender-matched healthy volunteers as well as eight patients with schizophrenia, but no history of physical aggression, served as controls. The NSS scores of antisocial offenders were significantly increased compared with those of the healthy controls, whereas no significant differences were observed between the scores of offenders and those of patients with schizophrenia. It can be speculated that NSS indicate a nonspecific vulnerability factor in several psychiatric syndromes, which are further influenced by a variety of genetic and environmental components. One of these syndromes may be antisocial personality disorder with severe aggression. PMID:15504651

  2. 77 FR 8268 - National Institute of Neurological Disorders and Stroke Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-02-14

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... personal privacy. Name of Committee: National Institute of Neurological Disorders and Stroke Special... Disorders and Stroke Special Emphasis Panel; K99R00 Conflict Review. Date: March 6, 2012. Time: 2 p.m. to...

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

    PubMed Central

    El Tallawy, Hamdy N; Farghaly, Wafaa MA; Rageh, Tarek A; Saleh, Ahmed O; Mestekawy, Taha AH; Darwish, Manal MM; Abd El Hamed, Mohamed A; Ali, Anwar M; Mahmoud, Doaa M

    2016-01-01

    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. PMID:27621635

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

    PubMed

    El Tallawy, Hamdy N; Farghaly, Wafaa Ma; Rageh, Tarek A; Saleh, Ahmed O; Mestekawy, Taha Ah; Darwish, Manal Mm; Abd El Hamed, Mohamed A; Ali, Anwar M; Mahmoud, Doaa M

    2016-01-01

    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. PMID:27621635

  5. I-123 Iofetamine SPECT scan in children with neurological disorders

    SciTech Connect

    Flamini, J.R.; Konkol, R.J.; Wells, R.G.; Sty, J.R. )

    1990-10-01

    I-123 Iofetamine (IMP) single photon emission computed tomography (SPECT) imaging of the brain in 42 patients (ages 14 days to 23 years) was compared with other localizing studies in children with neurological diseases. All had an EEG and at least one imaging study of the brain (computed tomography (CT) or magnetic resonance imaging (MRI), or both). Seventy-eight percent of the patients had an EEG within 24-72 hours of the IMP-SPECT scan. Thirty-five (83%) had a history of seizures, and the remainder had other neurological conditions without a history of seizures. In most cases, a normal EEG reading with normal CT or MRI result predicted a normal SPECT study. When the EEG was abnormal the majority of the IMP-SPECT scans were abnormal and localized the abnormality to the same region. A comparison with CT and MRI showed that structural abnormalities involving the cortex were usually well demonstrated with IMP-SPECT imaging. Structural lesions confined to the white matter were generally not detectable with IMP-SPECT. In a few cases, SPECT scans revealed abnormalities in deep brain areas not identified by EEG. IMP-SPECT imaging is a valuable technique for the detection and localization of abnormal cerebral metabolic activity in children with seizure disorders. A correlation with CT or MRI is essential for proper interpretation of abnormalities detected with IMP SPECT imaging.

  6. Neurological Disorders Associated with Striatal Lesions: Classification and Diagnostic Approach.

    PubMed

    Tonduti, Davide; Chiapparini, Luisa; Moroni, Isabella; Ardissone, Anna; Zorzi, Giovanna; Zibordi, Federica; Raspante, Sergio; Panteghini, Celeste; Garavaglia, Barbara; Nardocci, Nardo

    2016-06-01

    Neostriatal abnormalities can be observed in a very large number of neurological conditions clinically dominated by the presence of movement disorders. The neuroradiological picture in some cases has been described as "bilateral striatal necrosis" (BSN). BSN represents a condition histo-pathologically defined by the involvement of the neostriata and characterized by initial swelling of putamina and caudates followed by degeneration and cellular necrosis. After the first description in 1975, numerous acquired and hereditary conditions have been associated with the presence of BSN. At the same time, a large number of disorders involving neostriata have been described as BSN, in some cases irrespective of the presence of signs of cavitation on MRI. As a consequence, the etiological spectrum and the nosographic boundaries of the syndrome have progressively become less clear. In this study, we review the clinical and radiological features of the conditions associated with MRI evidence of bilateral striatal lesions. Based on MRI findings, we have distinguished two groups of disorders: BSN and other neostriatal lesions (SL). This distinction is extremely helpful in narrowing the differential diagnosis to a small group of known conditions. The clinical picture and complementary exams will finally lead to the diagnosis. We provide an update on the etiological spectrum of BSN and propose a diagnostic flowchart for clinicians. PMID:27074771

  7. 76 FR 73653 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-11-29

    ..., Scientific Review Branch, Division of Extramural Research, NINDS/NIH/DHHS/Neuroscience Center, 6001 Executive... Related to Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences,...

  8. Synaptic Plasticity and Neurological Disorders in Neurotropic Viral Infections

    PubMed Central

    Atluri, Venkata Subba Rao; Hidalgo, Melissa; Samikkannu, Thangavel; Kurapati, Kesava Rao Venkata; Nair, Madhavan

    2015-01-01

    Based on the type of cells or tissues they tend to harbor or attack, many of the viruses are characterized. But, in case of neurotropic viruses, it is not possible to classify them based on their tropism because many of them are not primarily neurotropic. While rabies and poliovirus are considered as strictly neurotropic, other neurotropic viruses involve nervous tissue only secondarily. Since the AIDS pandemic, the interest in neurotropic viral infections has become essential for all clinical neurologists. Although these neurotropic viruses are able to be harbored in or infect the nervous system, not all the neurotropic viruses have been reported to cause disrupted synaptic plasticity and impaired cognitive functions. In this review, we have discussed the neurotropic viruses, which play a major role in altered synaptic plasticity and neurological disorders. PMID:26649202

  9. The therapeutic value of yoga in neurological disorders

    PubMed Central

    Mishra, Shri K.; Singh, Parampreet; Bunch, Steven J.; Zhang, Ray

    2012-01-01

    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. PMID:23349587

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

    PubMed Central

    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. Fatty acids and their therapeutic potential in neurological disorders.

    PubMed

    Lei, Enie; Vacy, Kristina; Boon, Wah Chin

    2016-05-01

    There is little doubt that we are what we eat. Fatty acid supplementation and diets rich in fatty acids are being promoted as ways to a healthier brain. Short chain fatty acids are a product of intestinal microbiota metabolism of dietary fibre; and their derivatives are used as an anti-convulstant. They demonstrated therapeutic potential in neurodegenerative conditions as HDAC inhibitors; and while the mechanism is not well understood, have been shown to lower amyloid β in Alzheimer's Disease in preclinical studies. Medium chain fatty acids consumed as a mixture in dietary oils can induce ketogenesis without the need for a ketogentic diet. Hence, this has the potential to provide an alternative energy source to prevent neuronal cell death due to lack of glucose. Long chain fatty acids are commonly found in the diet as omega fatty acids. They act as an anti-oxidant protecting neuronal cell membranes from oxidative damage and as an anti-inflammatory mediator in the brain. We review which agents, from each fatty acid class, have the most therapeutic potential for neurological disorders (primarily Alzheimer's disease, Parkinson's disease, Autism Spectrum Disorder as well as possible applications to traumatic brain injury), by discussing what is known about their biological mechanisms from preclinical studies. PMID:26939763

  12. Common mechanisms of compensatory respiratory plasticity in spinal neurological disorders.

    PubMed

    Johnson, Rebecca A; Mitchell, Gordon S

    2013-11-01

    In many neurological disorders that disrupt spinal function and compromise breathing (e.g. ALS, cervical spinal injury, MS), patients often maintain ventilatory capacity well after the onset of severe CNS pathology. In progressive neurodegenerative diseases, patients ultimately reach a point where compensation is no longer possible, leading to catastrophic ventilatory failure. In this brief review, we consider evidence that common mechanisms of compensatory respiratory plasticity preserve breathing capacity in diverse clinical disorders, despite the onset of severe pathology (e.g. respiratory motor neuron denervation and/or death). We propose that a suite of mechanisms, operating at distinct sites in the respiratory control system, underlies compensatory respiratory plasticity, including: (1) increased (descending) central respiratory drive, (2) motor neuron plasticity, (3) plasticity at the neuromuscular junction or spared respiratory motor neurons, and (4) shifts in the balance from more to less severely compromised respiratory muscles. To establish this framework, we contrast three rodent models of neural dysfunction, each posing unique problems for the generation of adequate inspiratory motor output: (1) respiratory motor neuron death, (2) de- or dysmyelination of cervical spinal pathways, and (3) cervical spinal cord injury, a neuropathology with components of demyelination and motor neuron death. Through this contrast, we hope to understand the multilayered strategies used to "fight" for adequate breathing in the face of mounting pathology. PMID:23727226

  13. Noise in models of neurological and psychiatric disorders.

    PubMed

    Spitzer, M; Neumann, M

    1996-09-01

    The concept of noise has only recently been applied to modelling neuropsychiatric disorders. Two examples of such models are presented. 1. A phantom limb is a neurological condition after the amputation of an extremity. It consists of sensations of the presence of the lost limb and has been attributed to cortical as well as non-cortical mechanisms. A neural network model of phantom limbs is proposed which can parsimoniously account for a large number of clinical features and recent findings of cortical map plasticity after deafferentation. In trained self-organizing feature maps, deafferentation was simulated. Reorganization is shown to be driven by input noise. According to the model, the production of input noise by the deafferented primary sensory neuron drives cortical reorganization in amputees. No such noise is generated and/or conducted to the cortex in paraplegics. 2. Several clinical features of schizophrenia have been related to the ratio of signal to noise in neuronal information processing. In particular, dopamine--which has been implicated in the causation of schizophrenia for decades--has been proposed to modulate signal-to-noise ratio. Data are presented which suggest that schizophrenic thought disorder is the result of a hypodopaminergic state and concomitant increased effects of noise in semantic information processing. Possible functions of noise in the nervous systems are discussed. PMID:8968824

  14. Neurologic bases for comorbidity of balance disorders, anxiety disorders and migraine: neurotherapeutic implications

    PubMed Central

    Balaban, Carey D; Jacob, Rolf G; Furman, Joseph M

    2011-01-01

    The comorbidity among balance disorders, anxiety disorders and migraine has been studied extensively from clinical and basic research perspectives. From a neurological perspective, the comorbid symptoms are viewed as the product of sensorimotor, interoceptive and cognitive adaptations that are produced by afferent interoceptive information processing, a vestibulo–parabrachial nucleus network, a cerebral cortical network (including the insula, orbitofrontal cortex, prefrontal cortex and anterior cingulate cortex), a raphe nuclear–vestibular network, a coeruleo–vestibular network and a raphe–locus coeruleus loop. As these pathways overlap extensively with pathways implicated in the generation, perception and regulation of emotions and affective states, the comorbid disorders and effective treatment modalities can be viewed within the contexts of neurological and psychopharmacological sites of action of current therapies. PMID:21375443

  15. 76 FR 23613 - National Institute of Neurological Disorders and Stroke; Amended Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-04-27

    ... published in the Federal Register on April 1, 2011, 76 FR 18230. The date and time of the meeting was... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... Neurological Disorders and Stroke Special Emphasis Panel, April 19, 2011, 3:15 p.m. to April 19, 2011, 7:15...

  16. 75 FR 22818 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-04-30

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... personal privacy. Name of Committee: National Institute of Neurological Disorders and Stroke Initial Review... Stroke Initial Review Group, NST-1 Subcommittee. Date: June 3-4, 2010. Time: 8 a.m. to 6 p.m. Agenda:...

  17. 78 FR 19498 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-04-01

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... Stroke Initial Review Group; Neurological Sciences and Disorders C. Date: June 20-21, 2013. Time: 8:00...

  18. 77 FR 19025 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-03-29

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... personal privacy. Name of Committee: National Institute of Neurological Disorders and Stroke Special Emphasis Panel; Translational Stroke SEP. Date: April 18, 2012. Time: 10 a.m. to 12 p.m. Agenda: To...

  19. 78 FR 72683 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-12-03

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... personal privacy. Name of Committee: National Institute of Neurological Disorders and Stroke, Special Emphasis Panel, Stroke Trials Network-NDMC. Date: December 18, 2013. Time: 9:00 a.m. to 2:30 p.m....

  20. 78 FR 29144 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-05-17

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke..., neuhuber@ninds.nih.gov . Name of Committee: National Institute of Neurological Disorders and Stroke...

  1. 77 FR 72362 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-12-05

    ... HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... personal privacy. Name of Committee: National Institute of Neurological Disorders and Stroke Special... Stroke Special Emphasis Panel; Ancillary Studies SEP. Date: December 21, 2012. Time: 9:30 a.m. to 12:30...

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

    PubMed Central

    Hove, Michael J.; Keller, Peter E.

    2014-01-01

    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 Parkinson 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. PMID:25773624

  3. Dynamic regulation of aquaporin-4 water channels in neurological disorders

    PubMed Central

    Hsu, Ying; Tran, Minh; Linninger, Andreas A.

    2015-01-01

    Aquaporin-4 water channels play a central role in brain water regulation in neurological disorders. Aquaporin-4 is abundantly expressed at the astroglial endfeet facing the cerebral vasculature and the pial membrane, and both its expression level and subcellular localization significantly influence brain water transport. However, measurements of aquaporin-4 levels in animal models of brain injury often report opposite trends of change at the injury core and the penumbra. Furthermore, aquaporin-4 channels play a beneficial role in brain water clearance in vasogenic edema, but a detrimental role in cytotoxic edema and exacerbate cell swelling. In light of current evidence, we still do not have a complete understanding of the role of aquaporin-4 in brain water transport. In this review, we propose that the regulatory mechanisms of aquaporin-4 at the transcriptional, translational, and post-translational levels jointly regulate water permeability in the short and long time scale after injury. Furthermore, in order to understand why aquaporin-4 channels play opposing roles in cytotoxic and vasogenic edema, we discuss experimental evidence on the dynamically changing osmotic gradients between blood, extracellular space, and the cytosol during the formation of cytotoxic and vasogenic edema. We conclude with an emerging picture of the distinct osmotic environments in cytotoxic and vasogenic edema, and propose that the directions of aquaporin-4-mediated water clearance in these two types of edema are distinct. The difference in water clearance pathways may provide an explanation for the conflicting observations of the roles of aquaporin-4 in edema resolution. PMID:26526878

  4. Carriers in Cell-Based Therapies for Neurological Disorders

    PubMed Central

    Wong, Francisca S. Y.; Chan, Barbara P.; Lo, Amy C. Y.

    2014-01-01

    There is a pressing need for long-term neuroprotective and neuroregenerative therapies to promote full function recovery of injuries in the human nervous system resulting from trauma, stroke or degenerative diseases. Although cell-based therapies are promising in supporting repair and regeneration, direct introduction to the injury site is plagued by problems such as low transplanted cell survival rate, limited graft integration, immunorejection, and tumor formation. Neural tissue engineering offers an integrative and multifaceted approach to tackle these complex neurological disorders. Synergistic therapeutic effects can be obtained from combining customized biomaterial scaffolds with cell-based therapies. Current scaffold-facilitated cell transplantation strategies aim to achieve structural and functional rescue via offering a three-dimensional permissive and instructive environment for sustainable neuroactive factor production for prolonged periods and/or cell replacement at the target site. In this review, we intend to highlight important considerations in biomaterial selection and to review major biodegradable or non-biodegradable scaffolds used for cell transplantation to the central and peripheral nervous system in preclinical and clinical trials. Expanded knowledge in biomaterial properties and their prolonged interaction with transplanted and host cells have greatly expanded the possibilities for designing suitable carrier systems and the potential of cell therapies in the nervous system. PMID:24933636

  5. Therapeutic effects of progesterone in animal models of neurological disorders.

    PubMed

    De Nicola, Alejandro F; Coronel, Florencia; Garay, Laura I; Gargiulo-Monachelli, Gisella; Gonzalez Deniselle, Maria Claudia; Gonzalez, Susana L; Labombarda, Florencia; Meyer, Maria; Guennoun, Rachida; Schumacher, Michael

    2013-12-01

    Substantial evidence supports that progesterone exerts many functions in the central and peripheral nervous system unrelated to its classical role in reproduction. In this review we first discussed progesterone effects following binding to the classical intracellular progesterone receptors A and B and several forms of membrane progesterone receptors, the modulation of intracellular signalling cascades and the interaction of progesterone reduced metabolites with neurotransmitter receptors. We next described our results involving animal models of human neuropathologies to elucidate the protective roles of progesterone. We described: (a) the protective and promyelinating effects of progesterone in experimental spinal cord injury; (b) the progesterone protective effects exerted upon motoneurons in the degenerating spinal cord of Wobbler mouse model of amyotropic lateral sclerosis; (c) the protective and anti-inflammatory effects of progesterone in the murine experimental autoimmune encephalomyelitis model of multiple sclerosis and after lysolecithin demyelination; (d) the progesterone prevention of nociception and neuropathic pain which follow spinal cord injury; and (e) the protective effect of progesterone in experimental ischemic stroke. Whenever available, the molecular mechanisms involved in these progesterone effects were examined. The multiplicity of progesterone beneficial effects has opened new venues of research for neurological disorders. In this way, results obtained in animal models could provide the basis for novel therapeutic strategies and pre-clinical studies. PMID:24040821

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

    PubMed

    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. PMID:17952657

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

    PubMed

    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. PMID:25773624

  8. The Therapeutic Effects of Singing in Neurological Disorders.

    PubMed

    Wan, Catherine Y; Rüber, Theodor; Hohmann, Anja; Schlaug, Gottfried

    2010-04-01

    Music making (playing an instrument or singing) is a multimodal activity that involves the integration of auditory and sensorimotor processes. The ability to sing in humans is evident from infancy, and does not depend on formal vocal training but can be enhanced by training. Given the behavioral similarities between singing and speaking, as well as the shared and distinct neural correlates of both, researchers have begun to examine whether singing can be used to treat some of the speech-motor abnormalities associated with various neurological conditions. This paper reviews recent evidence on the therapeutic effects of singing, and how it can potentially ameliorate some of the speech deficits associated with conditions such as stuttering, Parkinson's disease, acquired brain lesions, and autism. By reviewing the status quo, it is hoped that future research can help to disentangle the relative contribution of factors to why singing works. This may ultimately lead to the development of specialized or "gold-standard" treatments for these disorders, and to an improvement in the quality of life for patients. PMID:21152359

  9. Dynamic regulation of aquaporin-4 water channels in neurological disorders.

    PubMed

    Hsu, Ying; Tran, Minh; Linninger, Andreas A

    2015-10-01

    Aquaporin-4 water channels play a central role in brain water regulation in neurological disorders. Aquaporin-4 is abundantly expressed at the astroglial endfeet facing the cerebral vasculature and the pial membrane, and both its expression level and subcellular localization significantly influence brain water transport. However, measurements of aquaporin-4 levels in animal models of brain injury often report opposite trends of change at the injury core and the penumbra. Furthermore, aquaporin-4 channels play a beneficial role in brain water clearance in vasogenic edema, but a detrimental role in cytotoxic edema and exacerbate cell swelling. In light of current evidence, we still do not have a complete understanding of the role of aquaporin-4 in brain water transport. In this review, we propose that the regulatory mechanisms of aquaporin-4 at the transcriptional, translational, and post-translational levels jointly regulate water permeability in the short and long time scale after injury. Furthermore, in order to understand why aquaporin-4 channels play opposing roles in cytotoxic and vasogenic edema, we discuss experimental evidence on the dynamically changing osmotic gradients between blood, extracellular space, and the cytosol during the formation of cytotoxic and vasogenic edema. We conclude with an emerging picture of the distinct osmotic environments in cytotoxic and vasogenic edema, and propose that the directions of aquaporin-4-mediated water clearance in these two types of edema are distinct. The difference in water clearance pathways may provide an explanation for the conflicting observations of the roles of aquaporin-4 in edema resolution. PMID:26526878

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

    PubMed Central

    Nasiri, Hamid; Ebrahimi, Amrollah; Zahed, Arash; Arab, Mostafa; Samouei, Rahele

    2015-01-01

    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. PMID:26487881

  11. Update on the role of p75NTR in neurological disorders: A novel therapeutic target.

    PubMed

    Shu, Ya-Hai; Lu, Xiu-Min; Wei, Jing-Xiang; Xiao, Lan; Wang, Yong-Tang

    2015-12-01

    As a low-affinity neurotrophins receptor, p75 neurotrophin receptor (p75NTR) is a transmembrane receptor involved in a diverse array of cellular responses, including apoptosis, survival, neurite outgrowth, migration, and cell cycle arrest, which may be related to some neurological disorders, such as Alzheimer's disease (AD), schizophrenia, major depressive disorder (MDD), posttraumatic stress disorder (PTSD), amyotrophic lateral sclerosis (ALS), and Parkinson's disease (PD). Indeed, a series of studies during the last decade has demonstrated that the p75NTR signaling plays key roles in most aspects of the neurological disorder diseases. In spite of the limited information available, this review still tried to summary the relationship between p75NTR and diverse neurological disorder diseases, and tried to further clarify the possible mechanism, which may provide a novel therapeutic target for the treatment of neurological disorders. PMID:26653545

  12. Survey of Neurological Disorders in Children Aged 9-15 Years in Northern India.

    PubMed

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

    2016-04-01

    The prevalence of neurological disorders in resource-poor settings, although likely to be high, is largely unexplored. The prevalence and risk factors for neurological disorders, including epilepsy and intellectual, motor, vision, and hearing deficits, in children aged 9 to 15 years in the community were investigated. A new instrument was developed, validated, and used in a 2-stage community survey for neurological disorders in Lucknow, India. Screen-positives and random proportion of screen-negatives were validated using predefined criteria. Prevalence of different neurological disorders was calculated by weighted proportions. Of 6431 children screened, 221 were positive. A total of 214 screen-positives and 251 screen-negatives were validated. Prevalence of neurological disorders was 31.3 per 1000 children of this age group (weighted 95% confidence interval = 16.5, 46.4). The final model for risk factors included age, mud house, delayed cry at birth, and previous head injury. The prevalence of neurological disorders is high in this region. Predictors of neurological disorders are largely modifiable. PMID:26428662

  13. Differentiating cerebral ischemia from functional neurological symptom disorder: a psychosomatic perspective

    PubMed Central

    2014-01-01

    Background The differential diagnosis of pseudo-neurological symptoms often represents a clinical challenge. The Diagnostic and Statistical Manual of Mental Disorders, DSM-5, made an attempt to improve diagnostic criteria of conversion disorder (functional neurological symptom disorder). Incongruences of the neurological examination, i.e. positive neurological signs, indicate a new approach - whereas psychological factors are not necessary anymore. As the DSM-5 will influence the International Classification of Diseases, ICD-11, this is of importance. In the case presented, a history of psychological distress and adverse childhood experiences coexisted with a true neurological disorder. We discuss the relevance of an interdisciplinary assessment and of operationalized diagnostic criteria. Case presentation A 32-year-old man presented twice with neurological symptoms without obvious pathological organic findings. A conversion disorder was considered early on at the second admission by the neurology team. Sticking to ICD-10, this diagnosis was not supported by a specialist for psychosomatic medicine, due to missing hints of concurrent psychological distress in temporal association with neurological symptoms. Further investigations then revealed a deep vein thrombosis (though D-dimers had been negative), which had probably resulted in a crossed embolus. Conclusion The absence of a clear proof of biological dysfunction underlying neurological symptoms should not lead automatically to the diagnosis of a conversion disorder. In contrast, at least in more complex patients, the work-up should include repeated psychological and neurological assessments in close collaboration. According to ICD-10 positive signs of concurrent psychological distress are required, while DSM-5 emphasizes an incongruity between neurological symptoms and neurophysiological patterns of dysfunction. In the case presented, an extensive medical work-up was initially negative, and neither positive

  14. Ethical and policy issues in newborn screening of children for neurologic and developmental disorders.

    PubMed

    Ross, Lainie Friedman

    2015-06-01

    Genetic testing for neurologic and developmental disorders spans the spectrum from universal newborn screening for conditions like phenylketonuria to diagnostic testing for suspected genetic conditions, to predictive genetic testing for childhood-onset conditions. Given that virtually all children in the United States undergo genetic screening in the newborn period, this article focuses on 3 actual case studies of neurologic and developmental disorders that have been included or proposed for inclusion in newborn screening programs: Duchenne muscular dystrophy (a neuromuscular disorder), Krabbe disease (a neurodegenerative disorder), and fragile X syndrome (a neurodevelopmental disorder). PMID:26022175

  15. Stem cells as promising therapeutic options for neurological disorders.

    PubMed

    Yoo, Jongman; Kim, Han-Soo; Hwang, Dong-Youn

    2013-04-01

    Due to the limitations of pharmacological and other current therapeutic strategies, stem cell therapies have emerged as promising options for treating many incurable neurologic diseases. A variety of stem cells including pluripotent stem cells (i.e., embryonic stem cells and induced pluripotent stem cells) and multipotent adult stem cells (i.e., fetal brain tissue, neural stem cells, and mesenchymal stem cells from various sources) have been explored as therapeutic options for treating many neurologic diseases, and it is becoming obvious that each type of stem cell has pros and cons as a source for cell therapy. Wise selection of stem cells with regard to the nature and status of neurologic dysfunctions is required to achieve optimal therapeutic efficacy. To this aim, the stem cell-mediated therapeutic efforts on four major neurological diseases, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, and stroke, will be introduced, and current problems and future directions will be discussed. PMID:23097262

  16. 78 FR 64223 - National Institute of Neurological Disorders and Stroke Amended Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-10-28

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke Amended Notice of Meeting Notice is hereby given of a change in the meeting of the Neurological...

  17. 78 FR 64227 - National Institute of Neurological Disorders and Stroke; Amended Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-10-28

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke; Amended Notice of Meeting Notice is hereby given of a change in the meeting of the Neurological...

  18. Abnormalities on the Neurological Examination and EEG in Young Children with Pervasive Developmental Disorders

    ERIC Educational Resources Information Center

    Akshoomoff, Natacha; Farid, Nikdokht; Courchesne, Eric; Haas, Richard

    2007-01-01

    This study examined the nature and frequency of neurological and EEG abnormalities in 60 young children (ages 2-6 years) with pervasive developmental disorders. A number of standard neurological functions could not be adequately assessed due to the young age of the children and/or limited comprehension and cooperation. The most common neurological…

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

    PubMed Central

    Lepcha, Anjali; Chandran, Reni K.; Alexander, Mathew; Agustine, Ann Mary; Thenmozhi, K.; Balraj, Achamma

    2015-01-01

    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. PMID:26019414

  20. 77 FR 28886 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-05-16

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke...., as amended. The grant applications and the discussions could disclose confidential trade secrets...

  1. 76 FR 20695 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-04-13

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke...., as amended. The grant applications and the discussions could disclose confidential trade secrets...

  2. 78 FR 4423 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-01-22

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke...., as amended. The grant applications and the discussions could disclose confidential trade secrets...

  3. 75 FR 64316 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-19

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke...., as amended. The grant applications and the discussions could disclose confidential trade secrets...

  4. 75 FR 64315 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-19

    ... Executive Boulevard, MSC 9529, Neuroscience Center, Room 3203, Bethesda, MD 20892-9529. 301- 496-5388... Related to Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences,...

  5. 75 FR 57043 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-09-17

    ..., Neuroscience Center, 6001 Executive Blvd., Suite 3204, MSC 9529, Bethesda, MD 20892, 301-496-0660, Benzingw... to Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences,...

  6. 76 FR 16432 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-03-23

    ... review and evaluate grant applications. Place: National Institutes of Health, Neuroscience Center, 6001... Review Officer, Scientific Review Branch, Division of Extramural Research, NINDS/ NIH/DHHS/Neuroscience... to Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences,...

  7. Nonlanguage disorders of speech reflect complex neurologic apparatus.

    PubMed

    Valenstein, E

    1975-09-01

    jerk or gag reflex, and absence of other upper motor neuron signs, such as upgoing toes, indicate a lower motor neuron or neuromuscular junction problem. Appropriate tests to rule out myasthenia gravis should be done. The other conditions discussed here are often obvious from their clinical presentation. Although the specific disorder of speech sometimes is helpful in localizing the cause, in most patients, the associated deficits on neurologic examination are of greatest value. PMID:169183

  8. Distinct neurological disorders with ATP1A3 mutations

    PubMed Central

    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

  9. Distinct neurological disorders with ATP1A3 mutations.

    PubMed

    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

    2014-05-01

    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

  10. Methyl Bromide Poisoning—A Bizarre Neurological Disorder

    PubMed Central

    Collins, Raymond P.

    1965-01-01

    Methyl bromide, a widely used fumigant, may cause burns of the skin, fatalities accompanied by coma and convulsions, or prolonged neurologic and psychiatric symptoms. Burns are more likely to occur where evaporation is prevented under protective clothing. Symptoms of serious illness may not develop for hours after exposure. Since action appears to be one of methylation, especially of SH groups, B.A.L. may be helpful if used promptly. PMID:14347974

  11. Panic attacks and panic disorder: the great neurologic imposters.

    PubMed

    Stahl, S M; Soefje, S

    1995-06-01

    Patients who experience panic attacks, panic disorder, or agoraphobia have significant impairment associated with their disorders. The cost to society in health care costs as well as the human suffering and mortality is high and may be even higher than necessary because of misdiagnosis and inadequate treatment of these patients. Although we do not have many answers in the areas of pathophysiology or neurochemistry of panic disorder, we do have effective treatments for panic disorder and agoraphobia. If these are conceptualized as distinct disorders with specific symptoms, making a diagnosis of panic disorder or agoraphobia is relatively easy. Making the correct diagnosis may save the patient many months or years of suffering and many inappropriate tests or treatments. PMID:7481132

  12. Social correlates of mental, neurological, and substance use disorders in China and India: a review.

    PubMed

    Cheng, Hui G; Shidhaye, Rahul; Charlson, Fiona; Deng, Fei; Lyngdoh, Tanica; Chen, Shengnan; Nanda, Sharmishtha; Lacroix, Kimberly; Baxter, Amanda; Whiteford, Harvey

    2016-09-01

    Understanding the epidemiological profiles of mental, neurological, and substance use disorders provides opportunities for the identification of high-risk population subgroups and for the development of effective country-specific prevention and intervention strategies. Guided by the Conceptual Framework for Action on the Social Determinants of Health by WHO we reviewed the literature to examine the association between a range of social correlates (eg, sex, age, education, income, urbanicity, marital status, and regional differences) and mental, neurological, and substance use disorders in China and India, the most populous countries in the world. We looked for papers on mental, neurological, and substance use disorders with location identifiers and socioeconomic correlates published between 1990 and 2015 and our search found 65 relevant studies from China and 29 from India. Several association patterns between social correlates and mental, neurological, and substance use disorders were not consistent with those reported in high-income countries, including a high concentration of middle-aged men with alcohol use disorders in China and to a lesser extent in India, and a positive association between being married and depression among women in India. Consistent with previous global reports, low education and poverty were associated with higher occurrence of dementia in both China and India, although there is evidence of an interaction between education and income in the risk for dementia in China. Large variations across regions and ethnic groups were consistently documented in China. These unique correlation patterns for mental, neurological, and substance use disorders identified in China and India emphasise the importance of understanding the local social context when planning targeted strategies to reduce the burden of these disorders. High-quality, up-to-date information about the constantly changing pattern of societal factors correlated with mental, neurological

  13. Association between hepatitis E and neurological disorders: two case studies and literature review.

    PubMed

    Deroux, A; Brion, J P; Hyerle, L; Belbezier, A; Vaillant, M; Mosnier, E; Larrat, S; Morand, P; Pavese, P

    2014-05-01

    Hepatitis E (HEV) is an emerging disease in our developed countries, but is not routinely tested for in case of liver cytolysis. However, a growing number of extra-hepatic manifestations of HEV infection associated with acute hepatitis are reported. In this article, we discuss two cases of HEV with neurological symptoms, one with encephalitis, and the other with Parsonage Turner syndrome. All these disorders appeared concomitantly with liver cytolysis and disappeared quickly, following the viral kinetics. Only twenty cases of neurological manifestation of HEV have been described before. The use of HEV serology in patients with concurrent liver cytolysis and neurological symptoms has to be improved. PMID:24583064

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

    PubMed

    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. PMID:27023045

  15. Male sexual dysfunction and infertility associated with neurological disorders

    PubMed Central

    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

  16. [Macroscopic findings of brains are helpful in differential diagnosis of neurological disorders].

    PubMed

    Yoshida, Mari

    2013-01-01

    Neuropathological diagnosis is essential in neurological disorders. Neurological signs and neuroimaging play a major role in clinical diagnosis. Although neuroimaging indicates the location and size of lesions, which is useful to longitudinal evaluation of edema or atrophy, pathological diagnosis is absolutely necessary to qualitative diagnosis. The first step of pathological diagnosis starts to observe macroscopic findings of brains, which reveal the distribution of lesions specific to individual disorders. Since the macroscopic abnormal findings are based on the microscopic degenerative findings, it may be no exaggeration to say that macroscopic findings enable to make neuropathological diagnosis. Accuracy of macroscopic finding is corrected or revised with microscopic findings and finally compared with neuroimaging and clinical diagnosis. Therefore it is very important and useful to learn macroscopic findings of neurological disorders. PMID:24291833

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

    PubMed

    Thomas, Jaya; Seo, Dongmin; Sael, Lee

    2016-01-01

    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. PMID:27258269

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

    PubMed Central

    Thomas, Jaya; Seo, Dongmin; Sael, Lee

    2016-01-01

    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. PMID:27258269

  19. The role of electromagnetic fields in neurological disorders.

    PubMed

    Terzi, Murat; Ozberk, Berra; Deniz, Omur Gulsum; Kaplan, Suleyman

    2016-09-01

    In the modern world, people are exposed to electromagnetic fields (EMFs) as part of their daily lives; the important question is "What is the effect of EMFs on human health?" Most previous studies are epidemiological, and we still do not have concrete evidence of EMF pathophysiology. Several factors may lead to chemical, morphological, and electrical alterations in the nervous system in a direct or indirect way. It is reported that non-ionizing EMFs have effects on animals and cells. The changes they bring about in organic systems may cause oxidative stress, which is essential for the neurophysiological process; it is associated with increased oxidization in species, or a reduction in antioxidant defense systems. Severe oxidative stress can cause imbalances in reactive oxygen species, which may trigger neurodegeneration. This review aims to detail these changes. Special attention is paid to the current data regarding EMFs' effects on neurological disease and associated symptoms, such as headache, sleep disturbances, and fatigue. PMID:27083321

  20. Current therapeutic techniques and rehabilitation from neurological disorders

    NASA Technical Reports Server (NTRS)

    Nickel, V. L.; Hsu, J. D.

    1974-01-01

    Rancho Los Amigos Hospital is a 1100-bed teaching hospital that is primarily oriented toward rehabilitation. The individual services that deal with neuromuscular disorders are categorically disease entity oriented: They are directed toward the major problems, such as spinal cord injuries, amputations, stroke, cerebral palsy, and rheumatoid arthritis. The services at Rancho cross many traditional medical specialty barriers.

  1. Advances in Reprogramming-Based Study of Neurologic Disorders

    PubMed Central

    Baldwin, Kristin K.

    2015-01-01

    The technology to convert adult human non-neural cells into neural lineages, through induced pluripotent stem cells (iPSCs), somatic cell nuclear transfer, and direct lineage reprogramming or transdifferentiation has progressed tremendously in recent years. Reprogramming-based approaches aimed at manipulating cellular identity have enormous potential for disease modeling, high-throughput drug screening, cell therapy, and personalized medicine. Human iPSC (hiPSC)-based cellular disease models have provided proof of principle evidence of the validity of this system. However, several challenges remain before patient-specific neurons produced by reprogramming can provide reliable insights into disease mechanisms or be efficiently applied to drug discovery and transplantation therapy. This review will first discuss limitations of currently available reprogramming-based methods in faithfully and reproducibly recapitulating disease pathology. Specifically, we will address issues such as culture heterogeneity, interline and inter-individual variability, and limitations of two-dimensional differentiation paradigms. Second, we will assess recent progress and the future prospects of reprogramming-based neurologic disease modeling. This includes three-dimensional disease modeling, advances in reprogramming technology, prescreening of hiPSCs and creating isogenic disease models using gene editing. PMID:25749371

  2. Clinical assessment of social cognitive function in neurological disorders.

    PubMed

    Henry, Julie D; von Hippel, William; Molenberghs, Pascal; Lee, Teresa; Sachdev, Perminder S

    2016-01-01

    Social cognition broadly refers to the processing of social information in the brain that underlies abilities such as the detection of others' emotions and responding appropriately to these emotions. Social cognitive skills are critical for successful communication and, consequently, mental health and wellbeing. Disturbances of social cognition are early and salient features of many neuropsychiatric, neurodevelopmental and neurodegenerative disorders, and often occur after acute brain injury. Its assessment in the clinic is, therefore, of paramount importance. Indeed, the most recent edition of the American Psychiatric Association's Diagnostic and Statistical Manual for Mental Disorders (DSM-5) introduced social cognition as one of six core components of neurocognitive function, alongside memory and executive control. Failures of social cognition most often present as poor theory of mind, reduced affective empathy, impaired social perception or abnormal social behaviour. Standard neuropsychological assessments lack the precision and sensitivity needed to adequately inform treatment of these failures. In this Review, we present appropriate methods of assessment for each of the four domains, using an example disorder to illustrate the value of these approaches. We discuss the clinical applications of testing for social cognitive function, and finally suggest a five-step algorithm for the evaluation and treatment of impairments, providing quantitative evidence to guide the selection of social cognitive measures in clinical practice. PMID:26670297

  3. Gross cerebellar paraneoplastic neurological disorder in a patient with an occult breast cancer

    PubMed Central

    Poudel, Chandra K; Achar, K N

    2013-01-01

    Paraneoplastic neurological disorders are relatively rare conditions posing both diagnostic as well as therapeutic challenges. A previously fit 66-year-old woman presented with subtle cerebellar symptoms which progressed rapidly over the course of days. Chest x-ray and routine blood tests were unremarkable. CT of the head with contrast showed no abnormality. Lumbar puncture showed no evidence of infection or oligoclonal bands. She was transferred to a neurological centre from a remote and rural setting. Subsequent MRI was reported to be normal as well. Tumour markers were negative but the paraneoplastic anti-Yo antibody was positive. A whole body CT scan revealed a spiculated left breast lesion which turned out to be malignant on fine needle aspiration. She underwent left mastectomy, had plasmapharesis and received high-dose intravenous Ig for her paraneoplastic neurological symptoms. She remained neurologically stable and underwent rehabilitation in her local hospital before getting discharged home. PMID:23595173

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

    PubMed

    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. PMID:27505016

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

    PubMed Central

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

    2016-01-01

    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.

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

    PubMed Central

    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

  7. Lymphatics in Neurological Disorders: A Neuro-Lympho-Vascular Component of Multiple Sclerosis and Alzheimer's Disease?

    PubMed

    Louveau, Antoine; Da Mesquita, Sandro; Kipnis, Jonathan

    2016-09-01

    Lymphatic vasculature drains interstitial fluids, which contain the tissue's waste products, and ensures immune surveillance of the tissues, allowing immune cell recirculation. Until recently, the CNS was considered to be devoid of a conventional lymphatic vasculature. The recent discovery in the meninges of a lymphatic network that drains the CNS calls into question classic models for the drainage of macromolecules and immune cells from the CNS. In the context of neurological disorders, the presence of a lymphatic system draining the CNS potentially offers a new player and a new avenue for therapy. In this review, we will attempt to integrate the known primary functions of the tissue lymphatic vasculature that exists in peripheral organs with the proposed function of meningeal lymphatic vessels in neurological disorders, specifically multiple sclerosis and Alzheimer's disease. We propose that these (and potentially other) neurological afflictions can be viewed as diseases with a neuro-lympho-vascular component and should be therapeutically targeted as such. PMID:27608759

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

    PubMed Central

    Kaneko, Miki; Yamashita, Yushiro; Iramina, Keiji

    2016-01-01

    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. PMID:26797613

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

    PubMed

    Kaneko, Miki; Yamashita, Yushiro; Iramina, Keiji

    2016-01-01

    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. PMID:26797613

  10. Management of Lower Urinary Tract Dysfunction in Patients with Neurological Disorders

    PubMed Central

    Li, Wen Ji

    2012-01-01

    The proper performance of the lower urinary tract is dependent on an intact neural innervation of the individual structures involved. Therefore, any congenital neurological anomalies, diseases, or lesions of the central, peripheral, or autonomic nervous systems can result in lower urinary tract symptoms. Lower urinary tract dysfunction (LUTD) secondary to neurological disorders can significantly reduce quality of life (QoL) and may also give rise to serious complications and psychological and social sequelae. The goals of management of LUTD in patients with neurological disorders are to prevent serious complications and to improve the patient's QoL. Understanding the physiology and pathophysiology of micturition is critical to selecting appropriate treatment options. This article provides an overview of the clinical characteristics, diagnosis, and management of LUTD in patients with certain central and peripheral neuropathies and common lesions. PMID:23060994

  11. From brain connectivity models to identifying foci of a neurological disorder.

    PubMed

    Venkataraman, Archana; Kubicki, Marek; Golland, Polina

    2012-01-01

    We propose a novel approach to identify the foci of a neurological disorder based on anatomical and functional connectivity information. Specifically, we formulate a generative model that characterizes the network of abnormal functional connectivity emanating from the affected foci. We employ the variational EM algorithm to fit the model and to identify both the afflicted regions and the differences in connectivity induced by the disorder. We demonstrate our method on a population study of schizophrenia. PMID:23285615

  12. 75 FR 9421 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-03-02

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... Emphasis Panel; Epilepsy Clinical Trial. Date: March 11, 2010. Time: 8 a.m. to 6 p.m. Agenda: To review...

  13. 78 FR 21615 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-04-11

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... Emphasis Panel; Epilepsy Genetics Review. Date: May 1, 2013. Time: 8:00 a.m. to 12:00 p.m. Agenda:...

  14. Educational Programming for Pupils with Neurologically Based Language Disorders. Final Report.

    ERIC Educational Resources Information Center

    Zedler, Empress Y.

    To investigate procedures whereby schools may achieve maximal results with otherwise normal underachieving pupils with neurologically based language-learning disorders, 100 such subjects were studied over a 2-year period. Fifty experimental subjects remained in regular classes in school and received individualized teaching outside of school hours…

  15. A Unique Team Approach to the Total Education of the Student with a Neurological Disorder.

    ERIC Educational Resources Information Center

    Cant, Malcolm J.

    The paper outlines the program of services provided by a multidisciplinary professional team for the neurologically disordered child from preschool to young adulthood. Noted among the services offered are the following: an infant stimulation program, preschool prep program, group sensory integration program, special educational assistance, summer…

  16. 76 FR 69747 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-11-09

    ... Emphasis Panel; Pilot Clinical Trial. Date: December 5, 2011. Time: 2:15 p.m. to 4 p.m. Agenda: To review... Stroke Special Emphasis Panel; Stroke Clinical Trial. Date: December 8, 2011. Time: 2:15 p.m. to 4 p.m....853, Clinical Research Related to Neurological Disorders; 93.854, Biological Basis Research in...

  17. 78 FR 70310 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    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...

  18. 76 FR 52961 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-08-24

    ... review and evaluate contract proposals. Place: National Institutes of Health, Neuroscience Center, 6001..., Scientific Review Officer, DHHS/NIH/NINDS/DER/SRB, 6001 Executive Boulevard, MSC 9529, Neuroscience Center... Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences, National Institutes of...

  19. 77 FR 15112 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-03-14

    ... review and evaluate contract proposals. Place: National Institutes of Health, Neuroscience Center, 6001.../Neuroscience Center, 6001 Executive Blvd., Suite 3208, MSC 9529, Bethesda, MD 20892, 301-496-5324, mcconnej... to Neurological Disorders; 93.854, Biological Basis Research in the ] Neurosciences,...

  20. 75 FR 39548 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-07-09

    ... and evaluate grant applications. Place: National Institutes of Health, Neuroscience Center, 6001.../Neuroscience Center, 6001 Executive Blvd., Suite 3208, Msc 9529, Bethesda, MD 20852, 301-435-6033, rajarams... to Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences,...

  1. 76 FR 41273 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-07-13

    ... review and evaluate grant applications. Place: National Institutes of Health, Neuroscience Center, 6001.../DHHS/Neuroscience Center, 6001 Executive Blvd., Suite 3208, MSC 9529, Bethesda, MD 20892-9529, 301-594... Related to Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences,...

  2. 75 FR 67380 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-11-02

    ...: To review and evaluate grant applications. Place: National Institutes of Health, Neuroscience Center..., NINDS/NIH/DHHS, Neuroscience Center, 6001 Executive Blvd., Room 3204, MSC 9529, Bethesda, MD 20892, 301..., Clinical Research Related to Neurological Disorders; 93.854, Biological Basis Research in the...

  3. 76 FR 10381 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-02-24

    ... evaluate contract proposals. Place: National Institutes of Health, Neuroscience Center, 6001 Executive... Review Officer, DHHS/NIH/NINDS/DER/SRB, 6001 Executive Boulevard; MSC 9529, Neuroscience Center; Room... Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences, National Institutes of...

  4. 76 FR 10382 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-02-24

    ... evaluate grant applications. Place: National Institutes of Health, Neuroscience Center, 6001 Executive.../Neuroscience Center, 6001 Executive Boulevard, Suite 3208, MSC 9529, Bethesda, MD 20892-9529, 301-594-0635... to Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences,...

  5. 75 FR 64315 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-19

    ... grant applications. Place: National Institutes of Health, Neuroscience Center, 6001 Executive Boulevard..., Neuroscience Center, 6001 Executive Blvd., Room 3208, MSC 9529, Bethesda, MD 20892-9529. 301-496-0635. Rc218u... Neurological Disorders; 93.854, Biological Basis Research in the Neurosciences, National Institutes of...

  6. 77 FR 6570 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-02-08

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke... Palomar, 2121 P Street NW., Washington, DC 20037. Contact Person: JoAnn McConnell, Ph.D.,...

  7. Translational Meta-analytical Methods to Localize the Regulatory Patterns of Neurological Disorders in the Human Brain

    PubMed Central

    Sochat, Vanessa; David, Maude; Wall, Dennis P

    2015-01-01

    The task of mapping neurological disorders in the human brain must be informed by multiple measurements of an individual’s phenotype - neuroimaging, genomics, and behavior. We developed a novel meta-analytical approach to integrate disparate resources and generated transcriptional maps of neurological disorders in the human brain yielding a purely computational procedure to pinpoint the brain location of transcribed genes likely to be involved in either onset or maintenance of the neurological condition. PMID:26958307

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

    ERIC Educational Resources Information Center

    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…

  9. Targeting the neurovascular unit for treatment of neurological disorders

    PubMed Central

    VanGilder, Reyna L.; Rosen, Charles L.; Barr, Taura L.; Huber, Jason D.

    2011-01-01

    Drug discovery for CNS disorders has been restricted by the inability for therapeutic agents to cross the blood-brain barrier (BBB). Moreover, current drugs aim to correct neuron cell signaling, thereby neglecting pathophysiological changes affecting other cell types of the neurovascular unit (NVU). Components of the NVU (pericytes, microglia, astrocytes, and neurons, and basal lamina) act as an intricate network to maintain the neuronal homeostatic microenvironment. Consequently, disruptions to this intricate cell network lead to neuron malfunction and symptoms characteristic of CNS diseases. A lack of understanding in NVU signaling cascades may explain why current treatments for CNS diseases are not curative. Current therapies treat symptoms by maintaining neuron function. Refocusing drug discovery to sustain NVU function may provide a better method of treatment by promoting neuron survival. In this review, we will examine current therapeutics for common CNS diseases, describe the importance of the NVU in cerebral homeostasis and discuss new possible drug targets and technologies that aim to improve treatment and drug delivery to the diseased brain. PMID:21172386

  10. Practical considerations on the use of rituximab in autoimmune neurological disorders

    PubMed Central

    Kosmidis, Mixalis L.; Dalakas, Marinos C.

    2010-01-01

    Rituximab (Mabthera, Rituxan) is a chimeric human/murine monoclonal antibody against CD-20 surface antigen expressed on B-cells. Rituximab, by causing B-cell depletion, appears to be effective in several autoimmune disorders; it has been approved for rheumatoid arthritis and is a promising new agent in the treatment of several autoimmune neurological disorders. A controlled study in patients with relapsing remitting multiple sclerosis has shown that rituximab significantly reduces the number of new MRI lesions and improves clinical outcome; it also showed some promise in a subset of patients with primary progressive MS. The drug is also effective in a number of patients with Devic’s disease, myasthenia gravis, autoimmune neuropathies, and inflammatory myopathies. The apparent effectiveness of rituximab has moved B-cells into the center stage of clinical and laboratory investigation of autoimmune neurological disorders. We review the evidence-based effectiveness of rituximab in neurological disorders based on controlled trials and anecdotal reports, including our own experience, and address the immunobiology of B-cells in autoimmune central nervous system (CNS) and peripheral nervous system (PNS) disorders. In addition, we provide practical guidelines on how best to use this drug in clinical practice and highlight its potential toxicity. PMID:21179602

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

    PubMed

    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. PMID:26257776

  12. Myeloid cell-based therapies in neurological disorders: How far have we come?

    PubMed

    Böttcher, Chotima; Priller, Josef

    2016-03-01

    The pathogenesis of neurological disorders such as multiple sclerosis (MS), amyotrophic lateral sclerosis (ALS) and Alzheimer's disease (AD) is multifactorial and incompletely understood. The development of therapies for these disorders of the central nervous system (CNS) is thus far very challenging. Neuroinflammation is one of the processes that contribute to the pathogenesis of CNS diseases, and therefore represents an important therapeutic target. Myeloid cells derived from the bone marrow are ideal candidates for cell therapy in the CNS as they are capable of targeting the brain and providing neuroprotective and anti-inflammatory effects. In this review, experimental and clinical evidence for the therapeutic potential of myeloid cells in neurological disorders will be discussed. This article is part of a Special Issue entitled: Neuro Inflammation edited by Helga E. de Vries and Markus Schwaninger. PMID:26455341

  13. Neurological disorders and therapeutics targeted to surmount the blood–brain barrier

    PubMed Central

    Kanwar, Jagat R; Sriramoju, Bhasker; Kanwar, Rupinder K

    2012-01-01

    We are now in an aging population, so neurological disorders, particularly the neurodegenerative diseases, are becoming more prevalent in society. As per the epidemiological studies, Europe alone suffers 35% of the burden, indicating an alarming rate of disease progression. Further, treatment for these disorders is a challenging area due to the presence of the tightly regulated blood–brain barrier and its unique ability to protect the brain from xenobiotics. Conventional therapeutics, although effective, remain critically below levels of optimum therapeutic efficacy. Hence, methods to overcome the blood–brain barrier are currently a focus of research. Nanotechnological applications are gaining paramount importance in addressing this question, and yielding some promising results. This review addresses the pathophysiology of the more common neurological disorders and novel drug candidates, along with targeted nanoparticle applications for brain delivery. PMID:22848160

  14. An overview on the correlation of neurological disorders with cardiovascular disease

    PubMed Central

    Firoz, C.K.; Jabir, Nasimudeen R.; Khan, Mohd Shahnawaz; Mahmoud, Maged; Shakil, Shazi; Damanhouri, Ghazi A.; Zaidi, Syed Kashif; Tabrez, Shams; Kamal, Mohammad A.

    2014-01-01

    Neurological disorders (NDs) are one of the leading causes of death especially in the developed countries. Among those NDs, Alzheimer’s disease (AD) and Parkinson disease (PD) are heading the table. There have been several reports in the scientific literatures which suggest the linkage between cardiovascular disorders (CVDs) and NDs. In the present communication, we have tried to compile NDs (AD and PD) association with CVDs reported in the literature. Based on the available scientific literature, we believe that further comprehensive study needs to be done to elucidate the molecular linking points associated with the above mentioned disorders. PMID:25561878

  15. Key sleep neurologic disorders: Narcolepsy, restless legs syndrome/Willis-Ekbom disease, and REM sleep behavior disorder.

    PubMed

    St Louis, Erik K

    2014-02-01

    Sleep disorders are frequent comorbidities in neurologic patients. This review focuses on clinical aspects and prognosis of 3 neurologic sleep disorders: narcolepsy, restless legs syndrome/Willis-Ekbom disease (RLS/WED), and REM sleep behavior disorder (RBD). Narcolepsy causes pervasive, enduring excessive daytime sleepiness, adversely affecting patients' daily functioning. RLS/WED is characterized by an uncomfortable urge to move the legs before sleep, often evolving toward augmentation and resulting in daylong bothersome symptoms. RBD causes potentially injurious dream enactment behaviors that often signify future evolution of overt synucleinopathy neurodegeneration in as many as 81% of patients. Timely recognition, referral for polysomnography, and longitudinal follow-up of narcolepsy, RLS/WED, and RBD patients are imperatives for neurologists in providing quality comprehensive patient care. PMID:24605270

  16. The Ketogenic Diet as a Treatment Paradigm for Diverse Neurological Disorders

    PubMed Central

    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. PMID:22509165

  17. Pilot Testing Behavior Therapy for Chronic Tic Disorders in Neurology and Developmental Pediatrics Clinics.

    PubMed

    Ricketts, Emily J; Gilbert, Donald L; Zinner, Samuel H; Mink, Jonathan W; Lipps, Tara D; Wiegand, Geoffrey A; Vierhile, Amy E; Ely, Laura J; Piacentini, John; Walkup, John T; Woods, Douglas W

    2016-03-01

    Comprehensive Behavioral Intervention for Tics (CBIT) is an efficacious treatment with limited regional availability. As neurology and pediatric clinics are often the first point of therapeutic contact for individuals with tics, the present study assessed preliminary treatment response, acceptability, and feasibility of an abbreviated version, modified for child neurology and developmental pediatrics clinics. Fourteen youth (9-17) with Tourette disorder across 2 child neurology clinics and one developmental pediatrics clinic participated in a small case series. Clinician-rated tic severity (Yale Global Tic Severity Scale) decreased from pre- to posttreatment, z = -2.0, P < .05, r = -.48, as did tic-related impairment, z = -2.4, P < .05, r = -.57. Five of the 9 completers (56%) were classified as treatment responders. Satisfaction ratings were high, and therapeutic alliance ratings were moderately high. Results provide guidance for refinement of this modified CBIT protocol. PMID:26271790

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

    PubMed

    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. PMID:26781943

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

    PubMed Central

    Song, Yu; Kwak, Shin; Yoshida, Sohei; Yamamoto, Yoshiharu

    2014-01-01

    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. PMID:25214709

  20. The Global Burden of Mental, Neurological and Substance Use Disorders: An Analysis from the Global Burden of Disease Study 2010

    PubMed Central

    Whiteford, Harvey A.; Ferrari, Alize J.; Degenhardt, Louisa; Feigin, Valery; Vos, Theo

    2015-01-01

    Background The Global Burden of Disease Study 2010 (GBD 2010), estimated that a substantial proportion of the world’s disease burden came from mental, neurological and substance use disorders. In this paper, we used GBD 2010 data to investigate time, year, region and age specific trends in burden due to mental, neurological and substance use disorders. Method For each disorder, prevalence data were assembled from systematic literature reviews. DisMod-MR, a Bayesian meta-regression tool, was used to model prevalence by country, region, age, sex and year. Prevalence data were combined with disability weights derived from survey data to estimate years lived with disability (YLDs). Years lost to premature mortality (YLLs) were estimated by multiplying deaths occurring as a result of a given disorder by the reference standard life expectancy at the age death occurred. Disability-adjusted life years (DALYs) were computed as the sum of YLDs and YLLs. Results In 2010, mental, neurological and substance use disorders accounted for 10.4% of global DALYs, 2.3% of global YLLs and, 28.5% of global YLDs, making them the leading cause of YLDs. Mental disorders accounted for the largest proportion of DALYs (56.7%), followed by neurological disorders (28.6%) and substance use disorders (14.7%). DALYs peaked in early adulthood for mental and substance use disorders but were more consistent across age for neurological disorders. Females accounted for more DALYs in all mental and neurological disorders, except for mental disorders occurring in childhood, schizophrenia, substance use disorders, Parkinson’s disease and epilepsy where males accounted for more DALYs. Overall DALYs were highest in Eastern Europe/Central Asia and lowest in East Asia/the Pacific. Conclusion Mental, neurological and substance use disorders contribute to a significant proportion of disease burden. Health systems can respond by implementing established, cost effective interventions, or by supporting the

  1. Dextromethorphan: An update on its utility for neurological and neuropsychiatric disorders.

    PubMed

    Nguyen, Linda; Thomas, Kelan L; Lucke-Wold, Brandon P; Cavendish, John Z; Crowe, Molly S; Matsumoto, Rae R

    2016-03-01

    Dextromethorphan (DM) is a commonly used antitussive and is currently the only FDA-approved pharmaceutical treatment for pseudobulbar affect. Its safety profile and diverse pharmacologic actions in the central nervous system have stimulated new interest for repurposing it. Numerous preclinical investigations and many open-label or blinded clinical studies have demonstrated its beneficial effects across a variety of neurological and psychiatric disorders. However, the optimal dose and safety of chronic dosing are not fully known. This review summarizes the preclinical and clinical effects of DM and its putative mechanisms of action, focusing on depression, stroke, traumatic brain injury, seizure, pain, methotrexate neurotoxicity, Parkinson's disease and autism. Moreover, we offer suggestions for future research with DM to advance the treatment for these and other neurological and psychiatric disorders. PMID:26826604

  2. FROM REINFORCEMENT LEARNING MODELS OF THE BASAL GANGLIA TO THE PATHOPHYSIOLOGY OF PSYCHIATRIC AND NEUROLOGICAL DISORDERS

    PubMed Central

    Maia, Tiago V.; Frank, Michael J.

    2013-01-01

    Over the last decade and a half, reinforcement learning models have fostered an increasingly sophisticated understanding of the functions of dopamine and cortico-basal ganglia-thalamo-cortical (CBGTC) circuits. More recently, these models, and the insights that they afford, have started to be used to understand key aspects of several psychiatric and neurological disorders that involve disturbances of the dopaminergic system and CBGTC circuits. We review this approach and its existing and potential applications to Parkinson’s disease, Tourette’s syndrome, attention-deficit/hyperactivity disorder, addiction, schizophrenia, and preclinical animal models used to screen novel antipsychotic drugs. The approach’s proven explanatory and predictive power bodes well for the continued growth of computational psychiatry and computational neurology. PMID:21270784

  3. The potential of induced pluripotent stem cells in models of neurological disorders: implications on future therapy.

    PubMed

    Crook, Jeremy Micah; Wallace, Gordon; Tomaskovic-Crook, Eva

    2015-03-01

    There is an urgent need for new and advanced approaches to modeling the pathological mechanisms of complex human neurological disorders. This is underscored by the decline in pharmaceutical research and development efficiency resulting in a relative decrease in new drug launches in the last several decades. Induced pluripotent stem cells represent a new tool to overcome many of the shortcomings of conventional methods, enabling live human neural cell modeling of complex conditions relating to aberrant neurodevelopment, such as schizophrenia, epilepsy and autism as well as age-associated neurodegeneration. This review considers the current status of induced pluripotent stem cell-based modeling of neurological disorders, canvassing proven and putative advantages, current constraints, and future prospects of next-generation culture systems for biomedical research and translation. PMID:25664599

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

    PubMed

    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. PMID:20441539

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

    PubMed Central

    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. PMID:25333848

  6. 77 FR 22791 - Secretary's Advisory Committee on Heritable Disorders in Newborns and Children; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-04-17

    ... mortality in newborns and children having (or at risk for) heritable disorders. The Advisory Committee's..., Maternal and Child Health Bureau, Health Resources and Services Administration, Room 18A-19,...

  7. 76 FR 51375 - Secretary's Advisory Committee on Heritable Disorders in Newborns and Children; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-08-18

    ... mortality in newborns and children having (or at risk) for heritable disorders. The Advisory Committee, as... obtaining other relevant information should write or contact Alaina M. Harris, Maternal and Child...

  8. Repetitive Transcranial Magnetic Stimulation for Clinical Applications in Neurological and Psychiatric Disorders: An Overview

    PubMed Central

    Machado, Sergio; Arias-Carrión, Oscar; Paes, Flávia; Vieira, Renata Teles; Caixeta, Leonardo; Novaes, Felipe; Marinho, Tamires; Almada, Leonardo Ferreira; Silva, Adriana Cardoso; Nardi, Antonio Egidio

    2013-01-01

    Neurological and psychiatric disorders are characterized by several disabling symptoms for which effective, mechanism-based treatments remain elusive. Consequently, more advanced non-invasive therapeutic methods are required. A method that may modulate brain activity and be viable for use in clinical practice is repetitive transcranial magnetic stimulation (rTMS). It is a non-invasive procedure whereby a pulsed magnetic field stimulates electrical activity in the brain. Here, we focus on the basic foundation of rTMS, the main stimulation parametters, the factors that influence individual responses to rTMS and the experimental advances of rTMS that may become a viable clinical application to treat neurological and psychiatric disorders. The findings showed that rTMS can improve some symptoms associated with these conditions and might be useful for promoting cortical plasticity in patients with neurological and psychiatric disorders. However, these changes are transient and it is premature to propose these applications as realistic therapeutic options, even though the rTMS technique has been evidenced as a potential modulator of sensorimotor integration and neuroplasticity. Functional imaging of the region of interest could highlight the capacity of rTMS to bring about plastic changes of the cortical circuitry and hint at future novel clinical interventions. Thus, we recommend that further studies clearly determine the role of rTMS in the treatment of these conditions. Finally, we must remember that however exciting the neurobiological mechanisms might be, the clinical usefulness of rTMS will be determined by its ability to provide patients with neurological and psychiatric disorders with safe, long-lasting and substantial improvements in quality of life. PMID:25610279

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

    PubMed Central

    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. PMID:27064956

  10. Spasmodic dysphonia in an adolescent patient with an autoimmune neurologic disorder.

    PubMed

    Boseley, Mark E; Gherson, Shirley; Hartnick, Christopher J

    2007-01-01

    Pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS) has been primarily described in the neurology and psychiatry literature. The symptoms of this syndrome typically are a range of obsessive compulsive disorders and neuromuscular tics. The otolaryngologist occasionally becomes involved with these children when it is deemed that chronic tonsil infections are the source. We report here on a child diagnosed with PANDAS who presented with severe ventricular hyperfunction and adductor spasmodic dysphonia. She was treated with botulinum toxin, which resulted in a significant improvement in subjective voice as well as reduced jitter and shimmer on objective voice measurements. PMID:17362824

  11. GAD65 epitope mapping and search for novel autoantibodies in GAD-associated neurological disorders.

    PubMed

    Fouka, P; Alexopoulos, H; Akrivou, S; Trohatou, O; Politis, P K; Dalakas, M C

    2015-04-15

    Antibodies against Glutamic-acid-decarboxylase (GAD65) are seen in various CNS excitability disorders including stiff-person syndrome, cerebellar ataxia, encephalitis and epilepsy. To explore pathogenicity, we examined whether distinct epitope specificities or other co-existing antibodies may account for each disorder. The epitope recognized by all 27 tested patients, irrespective of clinical phenotype, corresponded to the catalytic core of GAD. No autoantibodies against known GABAergic antigens were found. In a screen for novel specificities using live hippocampal neurons, three epilepsy patients, but no other, were positive. We conclude that no GAD-specific epitope defines any neurological syndrome but other antibody specificities may account for certain phenotypes. PMID:25867471

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

    SciTech Connect

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

    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.

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

    PubMed Central

    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. PMID:25783229

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

    PubMed

    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. PMID:25783229

  15. Neurological manifestations of gastrointestinal disorders, with particular reference to the differential diagnosis of multiple sclerosis.

    PubMed

    Ghezzi, A; Zaffaroni, M

    2001-11-01

    Neurological manifestations of gastrointestinal disorders are described, with particular reference to those resembling multiple sclerosis (MS) on clinical or MRI grounds. Patients with celiac disease can present cerebellar ataxia, progressive myoclonic ataxia, myelopathy, or cerebral, brainstem and peripheral nerve involvement. Antigliadin antibodies can be found in subjects with neurological dysfunction of unknown cause, particularly in sporadic cerebellar ataxia ("gluten ataxia"). Patients with Whipple's disease can develop mental and psychiatric changes, supranuclear gaze palsy, upper motoneuron signs, hypothalamic dysfunction, cranial nerve abnormalities, seizures, ataxia, myorhythmia and sensory deficits. Neurological manifestations can complicate inflammatory bowel disease (e.g. ulcerative colitis and Crohn's disease) due to vascular or vasculitic mechanisms. Cases with both Crohn's disease and MS or cerebral vasculitis are described. Epilepsy, chronic inflammatory polyneuropathy, muscle involvement and myasthenia gravis are also reported. The central nervous system can be affected in patients with hepatitis C virus (HCV) infection because of vasculitis associated with HCV-related cryoglobulinemia. Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is a disease caused by multiple deletions of mitochondrial DNA. It is characterized by peripheral neuropathy, ophthalmoplegia, deafness, leukoencephalopathy, and gastrointestinal symptoms due to visceral neuropathy. Neurological manifestations can be the consequence of vitamin B1, nicotinamide, vitamin B12, vitamin D, or vitamin E deficiency and from nutritional deficiency states following gastric surgery. PMID:11794474

  16. [Somatoform disorders in neurology visits: history and circumstances: retrospective study of 124 cases].

    PubMed

    Dubas, F; Thomas-Antérion, C

    2012-12-01

    We report 124 cases of somatoform disorders, considering psychogenic disorders at the same level as neurological disorders. We noted any psychic, somatic or social condition (history taking) and facilitating circumstances. The patients were aged 16 to 84 years old; 71.7% were women. We observed pain (35.4%), psychogenic headache (25%), sensorimotor loss (27.4%), gait and psychogenic tremor (17.7%), cognitive disorders (11.8%), ocular symptoms (7.2%), and urogenital symptoms (2.4%). Delay to consultation ranged from a few days to 20 years. Psychiatric comorbidity was noted in 30.6% of the cases. In 55.6% of 124 cases, we observed a psychological background. It was a childhood trauma in 15.3% of these cases. In one-third of the 124 situations, we noted an underlying somatic or social condition. Facilitation conditions were frequently mixed. Somatic and/or psychological conditions were noted in one-third of the 124 cases and social conditions in half of them. The neurologist is faced with the challenge of naming the symptom (most often labelled a functional disorder) and of making the decision to stop or limit investigations. Visits by patients with psychogenic disorders make up a significant percentage of neurology speciality appointments. The neurologist should not limit the consultation to differentiating "real" symptoms from psychogenic somatoform disorders, but should also propose a straightforward compassionate approach for effective therapeutic care. By carefully listening to the patient's dialogue, the neurologist can help the patient give meaning to the symptoms, and progress towards improved well-being. PMID:23153685

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

    PubMed Central

    Vela, Guillermo; Stark, Peter; Socha, Michael; Sauer, Ann Katrin; Hagmeyer, Simone; Grabrucker, Andreas M.

    2015-01-01

    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. PMID:25878905

  18. Zinc in gut-brain interaction in autism and neurological disorders.

    PubMed

    Vela, Guillermo; Stark, Peter; Socha, Michael; Sauer, Ann Katrin; Hagmeyer, Simone; Grabrucker, Andreas M

    2015-01-01

    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. PMID:25878905

  19. Structural basis for early-onset neurological disorders caused by mutations in human selenocysteine synthase

    PubMed Central

    Puppala, Anupama K.; French, Rachel L.; Matthies, Doreen; Baxa, Ulrich; Subramaniam, Sriram; Simonović, Miljan

    2016-01-01

    Selenocysteine synthase (SepSecS) catalyzes the terminal reaction of selenocysteine, and is vital for human selenoproteome integrity. Autosomal recessive inheritance of mutations in SepSecS–Ala239Thr, Thr325Ser, Tyr334Cys and Tyr429*–induced severe, early-onset, neurological disorders in distinct human populations. Although harboring different mutant alleles, patients presented remarkably similar phenotypes typified by cerebellar and cerebral atrophy, seizures, irritability, ataxia, and extreme spasticity. However, it has remained unclear how these genetic alterations affected the structure of SepSecS and subsequently elicited the development of a neurological pathology. Herein, our biophysical and structural characterization demonstrates that, with the exception of Tyr429*, pathogenic mutations decrease protein stability and trigger protein misfolding. We propose that the reduced stability and increased propensity towards misfolding are the main causes for the loss of SepSecS activity in afflicted patients, and that these factors contribute to disease progression. We also suggest that misfolding of enzymes regulating protein synthesis should be considered in the diagnosis and study of childhood neurological disorders. PMID:27576344

  20. Structural basis for early-onset neurological disorders caused by mutations in human selenocysteine synthase.

    PubMed

    Puppala, Anupama K; French, Rachel L; Matthies, Doreen; Baxa, Ulrich; Subramaniam, Sriram; Simonović, Miljan

    2016-01-01

    Selenocysteine synthase (SepSecS) catalyzes the terminal reaction of selenocysteine, and is vital for human selenoproteome integrity. Autosomal recessive inheritance of mutations in SepSecS-Ala239Thr, Thr325Ser, Tyr334Cys and Tyr429*-induced severe, early-onset, neurological disorders in distinct human populations. Although harboring different mutant alleles, patients presented remarkably similar phenotypes typified by cerebellar and cerebral atrophy, seizures, irritability, ataxia, and extreme spasticity. However, it has remained unclear how these genetic alterations affected the structure of SepSecS and subsequently elicited the development of a neurological pathology. Herein, our biophysical and structural characterization demonstrates that, with the exception of Tyr429*, pathogenic mutations decrease protein stability and trigger protein misfolding. We propose that the reduced stability and increased propensity towards misfolding are the main causes for the loss of SepSecS activity in afflicted patients, and that these factors contribute to disease progression. We also suggest that misfolding of enzymes regulating protein synthesis should be considered in the diagnosis and study of childhood neurological disorders. PMID:27576344

  1. Patients receiving lithium therapy have a reduced prevalence of neurological and cardiovascular disorders.

    PubMed

    Prosser, James M; Fieve, Ronald R

    2016-11-01

    A variety of evidence from laboratory and animal studies suggests that lithium has neurotrophic and cytoprotective properties, and may ameliorate or prevent some disease states. We investigated whether such a protective effect can be observed in human psychiatric patients receiving lithium therapy. We carried out a retrospective chart review of 1028 adult psychiatric male and female outpatients attending four lithium clinics in metropolitan New York City. Patients were divided into two groups based on lithium usage, and the prevalence of neurological and cardiovascular disorders was compared. The main outcome measures were the occurrence in the two patient groups of a variety of neurological disorders and myocardial infarction. Odds ratios were calculated to assess the risk of having a disorder for patients receiving lithium compared to patients not receiving lithium: for seizures, the odds ratio was 0.097; for amyotrophic lateral sclerosis, the odds ratio was 0.112; for dementia not otherwise specified, the odds ratio was 0.112; and for myocardial infarction, the odds ratio was 0.30. Logistical regression analysis showed that lithium treatment is a significant negative predictive factor in the prevalence of each of these disease states, when age, duration of clinic attendance, and use of anti-psychotic medications are taken into account. Our results show that patients receiving regular lithium treatment have a reduced prevalence of some neurological disorders and myocardial infarctions. One possible explanation of these results is that a protective effect of lithium observed in laboratory and animal studies may also be present in human patients receiving regular lithium therapy. PMID:27328427

  2. Treatment of neurological disorders by introducing mRNA in vivo using polyplex nanomicelles.

    PubMed

    Baba, Miyuki; Itaka, Keiji; Kondo, Kenji; Yamasoba, Tatsuya; Kataoka, Kazunori

    2015-03-10

    Sensory nerve disorders are difficult to cure completely considering poor nerve regeneration capacity and difficulties in accurately targeting neural tissues. Administering mRNA is a promising approach for treating neurological disorders because mRNA can provide proteins and peptides in their native forms for mature non-dividing neural cells, without the need of entering their nuclei. However, direct mRNA administration into neural tissues in vivo has been challenging due to too unstable manner of mRNA and its strong immunogenicity. Thus, using a suitable carrier is essential for effective mRNA administration. For this purpose, we established a novel carrier based on the self-assembly of polyethylene glycol (PEG)-polyamino acid block copolymer, i.e. polyplex nanomicelles. To investigate the feasibility and efficacy of mRNA administration for the treatment of sensory nerve disorders, we used a mouse model of experimentally induced olfactory dysfunction. Intranasal administration of mRNA-loaded nanomicelles provided an efficient and sustained protein expression for nearly two days in nasal tissues, particularly in the lamina propria which contains olfactory nerve fibers, with effectively regulating the immunogenicity of mRNA. Consequently, once-daily intranasal administration of brain-derived neurotrophic factor (BDNF)-expressing mRNA using polyplex nanomicelles remarkably enhanced the neurological recovery of olfactory function along with repairing the olfactory epithelium to a nearly normal architecture. To the best of our knowledge, this is the first study to show the therapeutic potential of introducing exogenous mRNA for the treatment of neurological disorders. These results indicate the feasibility and safety of using mRNA, and provide a novel strategy of mRNA-based therapy. PMID:25599855

  3. Facial Palsy, a Disorder Belonging to Influential Neurological Dynasty: Review of Literature.

    PubMed

    Newadkar, Ujwala R; Chaudhari, Lalit; Khalekar, Yogita K

    2016-07-01

    Facial paralysis is one of the common problem leading to facial deformation. Bell's palsy (BP) is defined as a lower motor neuron palsy of acute onset and idiopathic origin. BP is regarded as a benign common neurological disorder of unknown cause. It has an acute onset and is almost always a mononeuritis. The facial nerve is a mixed cranial nerve with a predominant motor component, which supplies all muscles concerned with unilateral facial expression. Knowledge of its course is vital for anatomic localization and clinical correlation. BP accounts for approximately 72% of facial palsies. Almost a century later, the management and etiology of BP is still a subject of controversy. Here, we present a review of literature on this neurologically significant entity. PMID:27583233

  4. Facial Palsy, a Disorder Belonging to Influential Neurological Dynasty: Review of Literature

    PubMed Central

    Newadkar, Ujwala R.; Chaudhari, Lalit; Khalekar, Yogita K.

    2016-01-01

    Facial paralysis is one of the common problem leading to facial deformation. Bell's palsy (BP) is defined as a lower motor neuron palsy of acute onset and idiopathic origin. BP is regarded as a benign common neurological disorder of unknown cause. It has an acute onset and is almost always a mononeuritis. The facial nerve is a mixed cranial nerve with a predominant motor component, which supplies all muscles concerned with unilateral facial expression. Knowledge of its course is vital for anatomic localization and clinical correlation. BP accounts for approximately 72% of facial palsies. Almost a century later, the management and etiology of BP is still a subject of controversy. Here, we present a review of literature on this neurologically significant entity. PMID:27583233

  5. [Neurological disorders related to vitamin B12 deficiency in prisons in Guinea: a 22-case study].

    PubMed

    Cisse, F A; Bah, H; Konate, F; Camara, N; Camara, M I; Conde, K; Kassory, I F B; Sanogo, A; Diakhate, I; Cisse, A

    2013-08-01

    Neurological disorders related to vitamin B12 deficiency are common in prisons of tropical Africa. We collected 22 cases (20 men and 2 women). They all showed vitamin B12 deficiency associated with neurological signs that were represented by sclerosis combined with bone marrow (n = 9), peripheral neuropathy (n = 6), cerebellar syndrome (n = 2), a pyramidal syndrome of the lower limbs (n = 4) and optic neuropathy (n = 1). Laboratory tests showed a mean hemoglobin concentration of 7.2 ± 1.5 g/dl, mean 104 ± 28 fl, macrocytic anemia in 10 patients. Biermer's disease was identified in 9 patients, 3 patients showed the syndrome of non dissociation of vitamin B12, a gastrectomy in 2 patients and no etiology was identified in 8 patients. PMID:23793871

  6. Enteral nutrition feeding alters antioxidant activity in unstimulated whole saliva composition of patients with neurological disorders.

    PubMed

    Cunha-Correia, Adriana Sales; Neto, Antonio Hernandes; Pereira, Ariana Ferreira; Aguiar, Sandra Maria Herondina Coelho Ávila; Nakamune, Ana Cláudia de Melo Stevanato

    2014-06-01

    Patients with neurological disorders have an increased risk of oral and systemic diseases due to compromised oral hygiene. If patients lose the ability to swallow and chew food as a result of their disorder, enteral nutrition is often utilized. However, this type of feeding may modify salivary antioxidant defenses, resulting in increased oxidative damage and the emergence of various diseases. The aim of this study was to evaluate the effects of enteral nutrition on biochemical parameters in the unstimulated whole saliva composition of patients with neurological disorders. For this, enzymatic (superoxide dismutase - SOD; glutathione peroxidase - GPx) and non-enzymatic (uric acid; ferric ion reducing antioxidant power - FRAP) antioxidant activity, as well as a marker for oxidative damage (thiobarbituric acid reactive substances - TBARS) were analyzed. Unstimulated whole saliva was collected from 12 patients with neurological disorders and tube-feeding (tube-fed group - TFG), 15 patients with neurological disorders and normal feeding via the mouth (non-tube-fed group - NTFG), and 12 volunteers without neurological disorders (control group - CG). The daily oral hygiene procedures of TFG and NTFG patients were similar and dental care was provided monthly by the same institution's dentist. All patients exhibited adequate oral health conditions. The salivary levels of FRAP, uric acid, SOD, GPx, TBARS, and total protein were compared between studied groups. FRAP was increased (p<0.05) in the NTFG (4,651 ± 192.5 mmol/mL) and the TFG (4,743 ± 116.7 mmol/mL) when compared with the CG (1,844 ± 343.8 mmol/mL). GPx values were lower (p<0.05) in the NTGF (8.24 ± 1.09 mmol/min/mg) and the TFG (8.37 ± 1.60 mmol/min/mg) than in the CG (15.30 ± 2.61 mmol/min/mg). Uric acid in the TFG (1.57 ± 0.23 mg/dL) was significantly lower than in the NTFG (2.34 ± 0.20mg/dL) and the CG (3.49 ± 0.21 mg/dL). Protein was significantly lower in the TFG (5.35 ± 0.27 g/dL) than in the NTFG (7

  7. Inhibitors of catechol-O-methyltransferase in the treatment of neurological disorders.

    PubMed

    Jatana, Nidhi; Apoorva, N; Malik, Sonika; Sharma, Aditya; Latha, Narayanan

    2013-01-01

    Catechol-O-methyltransferase (COMT) is the enzyme which catalyzes the transfer of a methyl group from S-adenosylmethionine to catechols and catecholamines, like the neurotransmitters dopamine, epinephrine and norepinephrine. COMT has implications in many neurological and psychiatric disorders like schizophrenia, Parkinson's disease (PD), bipolar disorders, etc. and therefore, it serves as an important drug target. Since its characterization in 1957, many inhibitors were designed where the first generation inhibitors were found to be highly toxic, short acting and had poor bioavailability. Currently, two of the second generation inhibitors, tolcapone and entacapone have been used for treatment of PD but are associated with various dopaminergic and gastro-intestinal side-effects. There have been several approaches for the design of novel COMT inhibitors with a good and safe therapeutic profile. The focus of this article is to review the current knowledge on COMT and the role of COMT inhibitors in the treatment of neurological disorders. The inhibitors have been classified into six different classes based on the structural framework. A historical overview of the discovery and development of COMT inhibitors is presented with a special emphasis on new generation of inhibitors till date. PMID:24450388

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

    PubMed

    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. PMID:26988283

  9. The use of pluripotent stem cell for personalized cell therapies against neurological disorders.

    PubMed

    Ha, Hye-Yeong; Jang, Si-Hyong; Jung, Ji-Won

    2011-01-01

    Although there are a number of weaknesses for clinical use, pluripotent stem cells are valuable sources for patient-specific cell therapies against various diseases. Backed-up by a huge number of basic researches, neuronal differentiation mechanism is well established and pluripotent stem cell therapies against neurological disorders are getting closer to clinical application. However, there are increasing needs for standardization of the sourcing pluripotent stem cells by establishing stem cell registries and banking. Global harmonization will accelerate practical use of personalized therapies using pluripotent stem cells. PMID:22203784

  10. DNA microarray gene expression analysis technology and its application to neurological disorders.

    PubMed

    Greenberg, S A

    2001-09-11

    DNA microarray technology is currently an area of great interest. Also called "genechip" technology, it incorporates molecular genetics and computer science on a massive scale. This technology can rapidly provide a detailed view of the simultaneous expression of entire genomes and provide new insights into gene function, disease pathophysiology, disease classification, and drug development. In this review, the author discusses the basic theory behind genechip and the other biologic chip technologies, their limitations given the current state of biologic knowledge and computational abilities, and their potential applications to the understanding of neurologic disorders. PMID:11575306

  11. Synaptopathies: synaptic dysfunction in neurological disorders - A review from students to students.

    PubMed

    Lepeta, Katarzyna; Lourenco, Mychael V; Schweitzer, Barbara C; Martino Adami, Pamela V; Banerjee, Priyanjalee; Catuara-Solarz, Silvina; de La Fuente Revenga, Mario; Guillem, Alain Marc; Haidar, Mouna; Ijomone, Omamuyovwi M; Nadorp, Bettina; Qi, Lin; Perera, Nirma D; Refsgaard, Louise K; Reid, Kimberley M; Sabbar, Mariam; Sahoo, Arghyadip; Schaefer, Natascha; Sheean, Rebecca K; Suska, Anna; Verma, Rajkumar; Vicidomini, Cinzia; Wright, Dean; Zhang, Xing-Ding; Seidenbecher, Constanze

    2016-09-01

    Synapses are essential components of neurons and allow information to travel coordinately throughout the nervous system to adjust behavior to environmental stimuli and to control body functions, memories, and emotions. Thus, optimal synaptic communication is required for proper brain physiology, and slight perturbations of synapse function can lead to brain disorders. In fact, increasing evidence has demonstrated the relevance of synapse dysfunction as a major determinant of many neurological diseases. This notion has led to the concept of synaptopathies as brain diseases with synapse defects as shared pathogenic features. In this review, which was initiated at the 13th International Society for Neurochemistry Advanced School, we discuss basic concepts of synapse structure and function, and provide a critical view of how aberrant synapse physiology may contribute to neurodevelopmental disorders (autism, Down syndrome, startle disease, and epilepsy) as well as neurodegenerative disorders (Alzheimer and Parkinson disease). We finally discuss the appropriateness and potential implications of gathering synapse diseases under a single term. Understanding common causes and intrinsic differences in disease-associated synaptic dysfunction could offer novel clues toward synapse-based therapeutic intervention for neurological and neuropsychiatric disorders. In this Review, which was initiated at the 13th International Society for Neurochemistry (ISN) Advanced School, we discuss basic concepts of synapse structure and function, and provide a critical view of how aberrant synapse physiology may contribute to neurodevelopmental (autism, Down syndrome, startle disease, and epilepsy) as well as neurodegenerative disorders (Alzheimer's and Parkinson's diseases), gathered together under the term of synaptopathies. Read the Editorial Highlight for this article on page 783. PMID:27333343

  12. Soft Neurological Signs and Cognitive Function in Obsessive-compulsive Disorder Patients

    PubMed Central

    Dhuri, Chetali Vijay; Parkar, Shubhangi R.

    2016-01-01

    Objective: Modern research on obsessive-compulsive disorder (OCD) indicates that the primary cause of OCD, which was earlier explained only on basis of psychoanalytical theories, is biological. Our study attempts to investigate the neurobiological signs in form of soft neurological signs and cognitive function in OCD. Methods: A cross sectional study was conducted at psychiatric facility of Seth G.S. Medical College and KEM Hospital. Materials and Method: 50 OCD patients and age- and education-matched controls were selected for the study. Established instruments were used to assess the neurological soft signs (NSS) and the cognitive deficits. Results: OCD patients had significant more NSS in tests for motor coordination, sensory integration, complex motor tasks, hard signs, and right/left and spatial orientation. Cognitive deficits in the domains of visuospatial ability, executive function, attention, and working memory were significantly more in OCD patients compared to controls. Conclusion: Our study highlights the role of biological factors in form of soft neurological signs and cognitive dysfunction in the development of the OCD. PMID:27570338

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

    PubMed Central

    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

  14. Learning Disorders as a School Health Problem—Neurological and Psychiatric Aspects

    PubMed Central

    Whitsell, Leon J.

    1969-01-01

    Broadened concepts of intellectual functions have shown that many varieties of mental subnormality may be preventable or subject to improvement with proper treatment. Many types of neurologic dysfunction are accompanied by learning disorders based on specific intellectual deficits. A more refined delineation of the higher cerebral functions of each child with a learning disorder provides the basis for improved specific remedial educational techniques. Such detailed assessment of higher functions of the nervous system can be greatly enhanced by the appropriate special evaluations carried out by well trained psychologists, speech pathologists and educational consultants, working in cooperation with physicians. The varieties of adjustment problems of children and emotional impact of a learning disorder should be recognized as early as possible and treated appropriately. Motor and perceptual-motor therapies may have limited value in some cases but may be harmful if indiscriminately applied. Psychotropic drugs have a relatively limited place in the management of learning disorders but may be immensely valuable in some cases by helping to control specific behavior problems which interfere with learning processes. Physicians have a major responsibility to provide help and leadership in dealing with learning disorders. PMID:4902291

  15. Functional Neurological Symptom Disorder: Mismanagement, Misdiagnosis, Chronic Cough Following Sexual Abuse: A Rare Case Report.

    PubMed

    Bidaki, Reza; Zarepur, Ehsan; Akrami, Maryam; Mohammad, Mohammad

    2016-01-01

    Objective Conversion disorder (CD) is a mental disorder in which patient displays neurological symptoms such as blindness, mutism, paralysis and seizure. It starts when our mind converts our mental stress into a physical symptom. A 15-year-old single white female with chronic cough, which had begun 5 months ago, was brought to our clinic. She had no history of hospitalization. His daily cough was without sputum production or fever, rhinorrhea and stopped during sleep. There was no recent exposure to tobacco smoke or a person with a chronic productive cough. Laboratory tests were normal. She had engaged 4 months ago. Doing sex during engagement is prohibited in her culture but and had anal sex, because of her spouse's trend. Psychotherapy was done and complete recovery was accomplished. PMID:27247590

  16. Evaluation of the somatosensory evoked blink response in patients with neurological disorders.

    PubMed Central

    Miwa, H; Yamaji, Y; Abe, H; Mizuno, Y

    1996-01-01

    BACKGROUND--The somatosensory evoked blink response (SBR) is a characteristic reflex blink elicited by electrical stimulation of peripheral nerves or other anatomical sites. METHODS--139 patients with neurological disorders were examined for presence of the SBR. Although the SBR was not usually elicitable, it was present in a subset of patients with Parkinson's disease and with hemifacial spasm. It was also present in a patient with Guillain-Barré syndrome before the recovery phase. The latency of the EMG activities responsible for the SBR was significantly shorter than that of the startle blink. CONCLUSIONS--The SBR is not a variant of the startle blink, but is a release phenomenon transmitted via the brainstem reticular formation. This response may be clinically relevant in disorders associated with brainstem lesions and abnormal blinking. PMID:8778259

  17. Functional Neurological Symptom Disorder: Mismanagement, Misdiagnosis, Chronic Cough Following Sexual Abuse: A Rare Case Report

    PubMed Central

    BIDAKI, Reza; ZAREPUR, Ehsan; AKRAMI, Maryam; Mohammad, Mohammad

    2016-01-01

    Objective Conversion disorder (CD) is a mental disorder in which patient displays neurological symptoms such as blindness, mutism, paralysis and seizure. It starts when our mind converts our mental stress into a physical symptom. A 15-year-old single white female with chronic cough, which had begun 5 months ago, was brought to our clinic. She had no history of hospitalization. His daily cough was without sputum production or fever, rhinorrhea and stopped during sleep. There was no recent exposure to tobacco smoke or a person with a chronic productive cough. Laboratory tests were normal. She had engaged 4 months ago. Doing sex during engagement is prohibited in her culture but and had anal sex, because of her spouse’s trend. Psychotherapy was done and complete recovery was accomplished. PMID:27247590

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

    PubMed

    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. PMID:24975171

  19. Frontiers in therapeutic development of allopregnanolone for Alzheimer’s disease and other neurological disorders

    PubMed Central

    Irwin, Ronald W.; Solinsky, Christine M.; Brinton, Roberta Diaz

    2014-01-01

    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 (AD) that have the potential to accelerate therapeutic translation for multiple unmet

  20. Role of long purine stretches in controlling the expression of genes associated with neurological disorders.

    PubMed

    Singh, Himanshu Narayan; Rajeswari, Moganty R

    2015-11-10

    Purine repeat sequences present in the human genome are known to act as hotspots for mutations leading to chromosomal imbalances. It is established that large purine repeats (PRs) form stable DNA triplex structure which can inhibit gene expression. Friedreich's ataxia (FRDA), the autosomal neurodegenerative disorder is the only human disease known so far, where a large purine (GAA) repeat in the FXN gene is known to inhibit the expression of frataxin protein. We explored the hidden purine repeats (PRn with n ≥ 200) if any, in the human genome to find out how they are associated with neurological disorders. The results showed 28 PRs, which are mostly restricted to the intronic regions. Interestingly, the transcriptome expression analysis of PR-carrying genes (PR-genes) revealed that most of them are down-regulated in neurological disorders (autism, Alzheimer's disease, schizophrenia, epilepsy, mental retardation, Parkinson's disease, brain tumor) as compared to that in healthy controls. The altered gene expression in brain disorders can be interpreted in terms of a possible expansion of purine repeats leading to formation of very stable DNA-triplex and/or alleviation of the repair enzymes and/or other unknown cellular factors. Interactome analysis identified four PR-genes in signaling pathways whose dysregulation is correlated directly with pathogenesis: GRK5 and KLK6 in Alzheimer's disease; FGF14 in craniosynostosis, mental retardation and FLT1 in neuroferritinopathy. By virtue of being mutational hotspots and their ability to form DNA-triplex, purine repeats in genome disturb the genome integrity and interfere with the transcriptional regulation. However, validation of the disease linkage of PR-genes can be validated using knock-out techniques. PMID:26149656

  1. Current understanding of the circadian clock and the clinical implications for neurological disorders.

    PubMed

    Turek, F W; Dugovic, C; Zee, P C

    2001-11-01

    The changes in behavior that occur on a 24-hour basis to match the 24-hour changes in the physical environment due to the rotation of the earth on its axis are a hallmark of life on the planet Earth. The nervous system of both lower and higher organisms has evolved over millions of years to meet the demands of the dramatic changes in the physical environment that occur in relation to the changes in the light-dark cycle, optimizing the survival and reproductive success of the organism. During the past 50 years, it has been clearly established that the 24-hour nature of life was not simply a response to the 24-hour changes in the physical environment imposed by celestial mechanics, but instead was due to an internal time-keeping system in the brain. Many neurological disorders are associated with abnormal 24-hour rhythms, including the sleep-wake cycle. The recent discovery of the molecular basis of the neural clock in animals offers neurologists new avenues for studying the pathophysiology of neurological disorders. PMID:11708984

  2. Blockade of N-acetylaspartylglutamate peptidases: a novel protective strategy for brain injuries and neurological disorders.

    PubMed

    Zhong, Chunlong; Luo, Qizhong; Jiang, Jiyao

    2014-12-01

    The peptide neurotransmitter N-acetylaspartylglutamate (NAAG) is reported to suppress glutamate release mainly through selective activation of presynaptic Group II metabotropic glutamate receptor subtype 3 (mGluR3). Therefore, strategies of inhibition of NAAG peptidases and subsequent NAAG hydrolysis to elevate levels of NAAG could reduce glutamate release under pathological conditions and be neuroprotective by attenuating excitotoxic cell injury. A series of potent inhibitors of NAAG peptidases has been synthesized and demonstrated efficacy in experimental models of ischemic-hypoxic brain injury, traumatic brain injury, inflammatory pain, diabetic neuropathy, amyotrophic lateral sclerosis and phencyclidine-induced schizophrenia-like behaviors. The excessive glutamatergic transmission has been implicated in all of these neurological disorders. Thus, blockade of NAAG peptidases may augment an endogenous protective mechanism and afford neuroprotection in the brain. This review aims to summarize and provide insight into the current understanding of the novel neuroprotective strategy based on limiting glutamate excitotoxicity for a wide variety of brain injuries and neurological disorders. PMID:24494725

  3. The bowel and beyond: the enteric nervous system in neurological disorders.

    PubMed

    Rao, Meenakshi; Gershon, Michael D

    2016-09-01

    The enteric nervous system (ENS) is large, complex and uniquely able to orchestrate gastrointestinal behaviour independently of the central nervous system (CNS). An intact ENS is essential for life and ENS dysfunction is often linked to digestive disorders. The part the ENS plays in neurological disorders, as a portal or participant, has also become increasingly evident. ENS structure and neurochemistry resemble that of the CNS, therefore pathogenic mechanisms that give rise to CNS disorders might also lead to ENS dysfunction, and nerves that interconnect the ENS and CNS can be conduits for disease spread. We review evidence for ENS dysfunction in the aetiopathogenesis of autism spectrum disorder, amyotrophic lateral sclerosis, transmissible spongiform encephalopathies, Parkinson disease and Alzheimer disease. Animal models suggest that common pathophysiological mechanisms account for the frequency of gastrointestinal comorbidity in these conditions. Moreover, the neurotropic pathogen, varicella zoster virus (VZV), unexpectedly establishes latency in enteric and other autonomic neurons that do not innervate skin. VZV reactivation in these neurons produces no rash and is therefore a clandestine cause of gastrointestinal disease, meningitis and strokes. The gut-brain alliance has raised consciousness as a contributor to health, but a gut-brain axis that contributes to disease merits equal attention. PMID:27435372

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

    PubMed

    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

    2016-05-01

    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

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

    PubMed Central

    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

  6. Neurological and cerebellar soft signs do not discriminate schizophrenia from bipolar disorder patients.

    PubMed

    Chrobak, Adrian Andrzej; Siwek, Grzegorz Przemysław; Siuda-Krzywicka, Katarzyna; Arciszewska, Aleksandra; Starowicz-Filip, Anna; Siwek, Marcin; Dudek, Dominika

    2016-01-01

    Patients with schizophrenia (SZ) and bipolar disorder (BD) share subtle motor abnormalities called the neurological soft signs (NSS). Since in both diseases there is evidence for alterations in cerebellar functions, structure and connectivity, we expected that the cerebellar soft signs (CSS), analogue of NSS focusing strictly on cerebellar symptoms, would be also a common trait in SZ and BD. We examined 30 patients with BD, 30 patients with SZ and 28 control subjects using the Neurological Evaluation Scale (NES, for NSS) and International Cooperative Ataxia Rating Scale (ICARS, for CSS). SZ and BD did not differ in total and subscales' scores in both NES and ICARS. Subscale analysis revealed that SZ performed significantly worse than controls in all the subscales of both NES and ICARS. BD patients scored significantly worse than controls in all NES subscales and in oculomotor and kinetic subscales of the ICARS, while other ICARS subscales did not differentiate those two groups. To our knowledge this is the first study to show that CSS constitute common symptoms in BD and SZ. We recommend a special focus on those diseases in further research regarding structural and functional changes of cerebellum and their clinical outcome. PMID:26241859

  7. Effectiveness of music therapy as an aid to neurorestoration of children with severe neurological disorders.

    PubMed

    Bringas, Maria L; Zaldivar, Marilyn; Rojas, Pedro A; Martinez-Montes, Karelia; Chongo, Dora M; Ortega, Maria A; Galvizu, Reynaldo; Perez, Alba E; Morales, Lilia M; Maragoto, Carlos; Vera, Hector; Galan, Lidice; Besson, Mireille; Valdes-Sosa, Pedro A

    2015-01-01

    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 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, confirm the importance of MT for the rehabilitation of patients across a wide range of dysfunctions. PMID:26582974

  8. Quantitative evaluations of ankle spasticity and stiffness in neurological disorders using manual spasticity evaluator

    PubMed Central

    Peng, Qiyu; Park, Hyung-Soon; Shah, Parag; Wilson, Nicole; Ren, Yupeng; Wu, Yi-Ning; Liu, Jie; Gaebler-Spira, Deborah J.; Zhang, Li-Qun

    2013-01-01

    Spasticity and contracture are major sources of disability in people with neurological impairments that have been evaluated using various instruments: the Modified Ashworth Scale, tendon reflex scale, pendulum test, mechanical perturbations, and passive joint range of motion (ROM). These measures generally are either convenient to use in clinics but not quantitative or they are quantitative but difficult to use conveniently in clinics. We have developed a manual spasticity evaluator (MSE) to evaluate spasticity/contracture quantitatively and conveniently, with ankle ROM and stiffness measured at a controlled low velocity and joint resistance and Tardieu catch angle measured at several higher velocities. We found that the Tardieu catch angle was linearly related to the velocity, indicating that increased resistance at higher velocities was felt at further stiffer positions and, thus, that the velocity dependence of spasticity may also be position-dependent. This finding indicates the need to control velocity in spasticity evaluation, which is achieved with the MSE. Quantitative measurements of spasticity, stiffness, and ROM can lead to more accurate characterizations of pathological conditions and outcome evaluations of interventions, potentially contributing to better healthcare services for patients with neurological disorders such as cerebral palsy, spinal cord injury, traumatic brain injury, and stroke. PMID:21674395

  9. Effectiveness of music therapy as an aid to neurorestoration of children with severe neurological disorders

    PubMed Central

    Bringas, Maria L.; Zaldivar, Marilyn; Rojas, Pedro A.; Martinez-Montes, Karelia; Chongo, Dora M.; Ortega, Maria A.; Galvizu, Reynaldo; Perez, Alba E.; Morales, Lilia M.; Maragoto, Carlos; Vera, Hector; Galan, Lidice; Besson, Mireille; Valdes-Sosa, Pedro A.

    2015-01-01

    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 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, confirm the importance of MT for the rehabilitation of patients across a wide range of dysfunctions. PMID:26582974

  10. Distinct neurological disorders with C9orf72 mutations: genetics, pathogenesis, and therapy.

    PubMed

    Chi, Song; Jiang, Teng; Tan, Lan; Yu, Jin-Tai

    2016-07-01

    The G4C2 repeat expansion within C9orf72 has been recently identified as the most common genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis. This mutation has also been detected in a variety of other neurological diseases with distinct clinical manifestations. The exact mechanisms of how this mutation leads to the wide spectrum of clinical syndromes remain unknown. A series of molecular changes together with some potential modifiers may play a key role. Nucleolar stress, nucleocytoplasmic transport defect, oxidative damage, inhibited stress granules assembly, activated endoplasmic reticulum stress, and inhibited proteasome activity are mechanisms that contribute to the pathogenesis of these diseases. Additional mutations, epigenetic modifiers, and repeat size are potential modifiers that modulate specific phenotypes on the basis of the molecular changes. Here, we summarize distinct C9orf72-related neurological disorders and their corresponding neuropathological changes. Then, we elucidate the existing molecular knowledge and the potential modifiers. Finally, we detail the main target of treatment aiming at controlling expanded RNA transcripts. PMID:27139021

  11. Diversity and plasticity of microglial cells in psychiatric and neurological disorders.

    PubMed

    Nakagawa, Yutaka; Chiba, Kenji

    2015-10-01

    Recent advanced immunological analyses have revealed that the diversity and plasticity of macrophages lead to the identification of functional polarization states (classically activated M1 type and alternatively activated M2 type) which are dependent on the extracellular environment. M1 and M2 polarization states of macrophages play an important role in controlling the balance between pro-inflammatory and anti-inflammatory conditions. Microglial cells are resident mononuclear phagocytes in the central nervous system (CNS), express several macrophage-associated markers, and appear to display functional polarization states similar to macrophages. Like M1 macrophages, M1 polarized microglia can produce pro-inflammatory cytokines and mediators such as interleukin (IL) 1β, IL-6, tumor necrosis factor-α, CC-chemokine ligand 2, nitric oxide, and reactive oxygen species, suggesting that these molecules contribute to dysfunction of neural network in the CNS. On the other hand, M2 polarized microglia can produce anti-inflammatory cytokine, IL-10 and express several receptors that are implicated in inhibiting inflammation and restoring homeostasis. In this review, we summarize the diversity, plasticity, and immunoregulatory functions of M1 and M2 microglia in psychiatric and neurological disorders. Based on these aspects, we propose a contribution of imbalance between M1 and M2 polarization of microglia in bipolar disorder, obesity, amyotrophic lateral sclerosis, and Rett syndrome. Consequently, molecules that normalize the imbalance between M1 and M2 microglial polarization states may provide a beneficial therapeutic target for the treatment of these disorders. PMID:26129625

  12. Treatment of neurological and psychiatric disorders with deep brain stimulation; raising hopes and future challenges.

    PubMed

    Sharifi, Mohammad Sharif

    2013-01-01

    The technology of Neural Stimulation in recent years has become the focus of the research and treatment, although it has been around for many years. The potential use of stimulating the brain and nerves ranges from the spinal cord stimulation to the implantations of cochlear and bionic eyes with a large discrepancy between the clinical readiness for these various uses. Electrical high-frequency Deep Brain Stimulation (DBS) was developed as an alternative option to treat a few neurological disorders. However, with advancing in surgical procedures, technologies and safeties, the applications of DBS are expanding not only for therapeutic purposes but also for research. Although the exact mechanisms of action/s are not fully understood, the outcome of the ongoing research and clinical trials are promising. DBS has been used to treat the essential tremor since 1997, Parkinson's disease (PD) since 2002 and dystonia since 2003. It has also been used to treat various disorders, including major depression. The therapeutic effect of DBS in PD is well established but for other diseases such as epilepsy the outcomes are unclear and ambiguous. This article is a succinct review of the literature, focusing on PD, epilepsy and Obsessive Compulsive Disorder (OCD). PMID:25337356

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

    PubMed Central

    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

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

    PubMed Central

    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

  15. 78 FR 76320 - Advisory Opinion Proceeding; Certain Sleep-Disordered Breathing Treatment Systems and Components...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-12-17

    ..., California; and ResMed Ltd. of Australia (collectively, ``ResMed''). 78 FR 25475 (May 1, 2013). The complaint... COMMISSION Advisory Opinion Proceeding; Certain Sleep-Disordered Breathing Treatment Systems and Components... importation, importation, or sale within the United States after importation of certain...

  16. Altered galectin glycosylation: potential factor for the diagnostics and therapeutics of various cardiovascular and neurological disorders.

    PubMed

    Ashraf, Ghulam Md; Perveen, Asma; Tabrez, Shams; Jabir, Nasimudeen R; Damanhouri, Ghazi A; Zaidi, Syed Kashif; Banu, Naheed

    2015-01-01

    Galectins are β-galactoside binding mammalian proteins characterized by the presence of a conserved carbohydrate recognition domain, expressed in almost all taxa of living organisms and involved in broad range of significant biological and physiological functions. Previously, we reported the purification and extensive characterization of galectin-1 from goat (Capra hircus) heart. Interestingly, the purified protein was found to have significant level of glycosylation. This intrigued us to evaluate the involvement of glycosylation in relation to protein's structural and functional integrity in its purified form. In the present study, an extensive comparative physicochemical characterization has been performed between the glycosylated and deglycosylated form of the purified protein. Deglycosylation resulted in an enhanced fluorescence quenching and marked reduction in pH and thermal stability of the purified galectin. Exposure to various biologically active chemicals showed significant differences in the properties and stability profile, causing significant deviations from its regular secondary structure in the deglycosylated form. These results clearly indicated enhanced structural and functional stabilization in the glycosylated galectin. The data revealed herein adds a vital facet demonstrating the significance of galectin expression and glycosylation in causation, progression, and possible therapeutics of associated clinical disorders. Our approach also allowed us to define some key interactions between the purified galectin and carbohydrate ligands that could well serve as an important landmark for designing new drug protocols for various cardiovascular and neurological disorders. PMID:25416978

  17. Looking Inside the Matrix: Perineuronal Nets in Plasticity, Maladaptive Plasticity and Neurological Disorders.

    PubMed

    De Luca, Ciro; Papa, Michele

    2016-07-01

    The integrity of the central nervous system (CNS) matrix is crucial for its proper function. Loss of the lattice-like structure compromise synaptic stability and can lead to the disruption of the excitatory/inhibitory balance, astrocytosis, maladaptive plasticity and neuronal death. Perineuronal nets (PNNs) in the extracellular matrix (ECM) provide synaptic integration and control the functional wiring between neurons. These nets are significantly modified during CNS disorders, such as neurodegenerative, cerebrovascular and inflammatory diseases. The breakdown or the modification of PNNs could be due to the activity of matrix metalloproteinases (MMPs) or to the deposition of proteoglycans, glycoproteins, and hyaluronic acid. The expression and the activity of ECM-degrading enzymes can be regulated with tissue inhibitors of MMPs or via transcriptional and epigenetic silencing or enhancement (i.e. via histone deacetylases). The identification of molecules and mechanisms able to modify these processes will be essential for a new perspective on brain functioning in health and disease, leading to a target-directed approach with drugs directly interfering with the molecular mechanism underlying neurological disorders. PMID:26935742

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

    PubMed Central

    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

    2013-01-01

    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

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

    ERIC Educational Resources Information Center

    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…

  20. Rey's 15-Item Visual Memory Test for the Detection of Malingering: Normative Observations on Patients with Neurological Disorders.

    ERIC Educational Resources Information Center

    Lee, Gregory P.; And Others

    1992-01-01

    To gather normative observations on a visual memory test developed by A. Rey (1964), it was administered to 100 temporal-lobe epilepsy patients with memory deficits and 56 outpatients with neurological disorders. Results suggest a cutoff score of 7 on the memory test may alert the clinician to possible factitious memory complaints. (SLD)

  1. Autistic Traits, ADHD Symptoms, Neurological Soft Signs and Regional Cerebral Blood Flow in Adults with Autism Spectrum Disorders

    ERIC Educational Resources Information Center

    Manouilenko, Irina; Pagani, Marco; Stone-Elander, Sharon; Odh, Richard; Brolin, Fredrik; Hatherly, Robert; Jacobsson, Hans; Larsson, Stig A.; Bejerot, Susanne

    2013-01-01

    The resting regional cerebral blood flow (rCBF) patterns related to co-occurring symptoms such as inattention, hyperactivity, neurological soft signs and motor problems have not yet been disclosed in autism spectrum disorders (ASD). In this study thirteen adults with ASD and ten matched neurotypical controls underwent PET. The scores of rating…

  2. Astrocytic adrenoceptors: a major drug target in neurological and psychiatric disorders?

    PubMed

    Hertz, L; Chen, Y; Gibbs, M E; Zang, P; Peng, L

    2004-06-01

    Considerable attention has recently been paid to astrocyte functions, which are briefly summarized. A large amount of data is available about adrenoceptor expression and function in astrocytes, some of it dating back to the 1970's and some of it very recent. This material is reviewed in the present paper. The brain is innervated by noradrenergic fibers extending from locus coeruleus in the brain stem, which in turn is connected to a network of adrenergic and noradrenergic nuclei in the medulla and pons, contributing to the control of (nor)adrenergic, serotonergic, dopaminergic and cholinergic function, both in the central nervous system (CNS) and in the periphery. In the CNS astrocytes constitute a major target for noradrenergic innervation, which regulates morphological plasticity, energy metabolism, membrane transport, gap junction permeability and immunological responses in these cells. Noradrenergic effects on astrocytes are essential during consolidation of episodic, long-term memory, which is reinforced by beta-adrenergic activation. Glycogenolysis and synthesis of glutamate and glutamine from glucose, both of which are metabolic processes restricted to astrocytes, occur at several time-specific stages during the consolidation. Astrocytic abnormalities are almost certainly important in the pathogenesis of multiple sclerosis and in all probability contribute essentially to inflammation and malfunction in Alzheimer's disease and to mood disturbances in affective disorders. Noradrenergic function in astrocytes is severely disturbed by chronic exposure to cocaine, which also changes astrocyte morphology. Development of drugs modifying noradrenergic receptor activity and/or down-stream signaling is advocated for treatment of several neurological/psychiatric disorders and for neuroprotection. Astrocytic preparations are suggested for study of mechanism(s) of action of antidepressant drugs and pathophysiology of mood disorders. PMID:15180484

  3. Etiological beliefs of patients with neurological disorders attending a tertiary care center: A cross-sectional study

    PubMed Central

    Bajaj, Bhupender Kumar; Chaudhary, Shipra; Shrestha, Runa

    2013-01-01

    Background: The understanding and management of neurological disorders is undergoing revolutionary changes over the last three decades in the background of ever increasing advances in medical technologies, diagnostic techniques, therapeutic processes and, molecular and genetic medicine. The fruits of these advances can reach patients only if the psychosocial hurdles in their delivery are identified, acknowledged and addressed. Aim: To explore the beliefs and practices of patients with neurological disorders in a tertiary care center in the eastern Nepal. Materials and Methods: One hundred patients attending neurology/medicine outpatient for neurological disorders were interviewed about their beliefs regarding the triggering factors, causation and treatment-seeking behavior particularly from traditional healers. Result: Of the 100 patients (49 males, 51 females) recruited in the study, 51% expressed having ‘no idea’ about their illness. Only 20% patients gave medically congruent explanation for their illness. Psychological factors were attributed as triggering factors by 16% of patients, of which two-thirds were females. Chance, destiny and ‘jadu tona’ topped the list of triggering factors. Forty-four percent patients had sought help of traditional faith healers (‘Dhami Jhakri’) before seeking medical help. Traditional faith healers were approached by patients irrespective of their educational background. Fifty-nine percent of patients who first sought traditional faith healers, believed in ‘jadu-tona’. Of those interviewed, 16% were planning to go to a faith healer in near future. Conclusion: The beliefs of patients with neurological disorders frequently do not conform to current medical opinion. There is need for greater communication and education of patients by their treating physicians. PMID:24347941

  4. Common Neurological Disorders Involving Inpatient Liaisons at a Secondary Referral Hospital in Taiwan: A Retrospective Cross-Sectional Study

    PubMed Central

    Liu, Chih-Yang; Chiang, Han-Lin; Fu, Ser-Chen; Su, Yu-Chin; Hsiao, Cheng-Lun; Yang, Fu-Yi

    2016-01-01

    Background and Purpose The requirement for neurology liaison is increasing in accordance with the growing health care demands associated with aging populations. The aim of this study was to characterize the nature of neurological inpatient liaisons (NILs) to help plan for the appropriate use of neurology resources. Methods This was a retrospective cross-sectional study of NILs in a secondary referral hospital over a 12-month period. Results There were 853 neurological consultations with a liaison rate of 3% per admission case. Chest medicine, gastroenterology, and infectious disease were the three most frequent specialties requesting liaison, and altered consciousness, seizure, and stroke were the three most frequent disorders for which a NIL was requested. Infection was the most common cause of altered consciousness. Epilepsy, infection, and previous stroke were common causes of seizure disorders. Acute stroke accounted for 44% of all stroke disorders. Electroencephalography was the most recommended study, and was also the most frequently performed. Ninety-five percent of emergency consultations were completed within 2 hours, and 85% of regular consultations were completed within 24 hours. The consult-to-visit times for emergency and regular consultations were 44±47 minutes (mean±standard deviation) and 730±768 minutes, respectively, and were shorter for regular consultations at intensive care units (p=0.0151) and for seizure and stroke disorders (p=0.0032). Conclusions Altered consciousness, seizure, and stroke were the most common reasons for NILs. Half of the patients had acute neurological diseases warranting immediate diagnosis and treatment by the consulting neurologists. Balancing increasing neurologist workloads and appropriate health-care resources remains a challenge. PMID:26754782

  5. A Novel Predicted Calcium-Regulated Kinase Family Implicated in Neurological Disorders

    PubMed Central

    Dudkiewicz, Małgorzata; Lenart, Anna; Pawłowski, Krzysztof

    2013-01-01

    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 Ca2+-binding domain in FAM69A and FAM69B proteins, inserted within the structure of the kinase domain, suggests they may function as Ca2+-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. PMID:23840464

  6. The crystal structure of human GlnRS provides basis for the development of neurological disorders

    PubMed Central

    Ognjenović, Jana; Wu, Jiang; Matthies, Doreen; Baxa, Ulrich; Subramaniam, Sriram; Ling, Jiqiang; Simonović, Miljan

    2016-01-01

    Cytosolic glutaminyl-tRNA synthetase (GlnRS) is the singular enzyme responsible for translation of glutamine codons. Compound heterozygous mutations in GlnRS cause severe brain disorders by a poorly understood mechanism. Herein, we present crystal structures of the wild type and two pathological mutants of human GlnRS, which reveal, for the first time, the domain organization of the intact enzyme and the structure of the functionally important N-terminal domain (NTD). Pathological mutations mapping in the NTD alter the domain structure, and decrease catalytic activity and stability of GlnRS, whereas missense mutations in the catalytic domain induce misfolding of the enzyme. Our results suggest that the reduced catalytic efficiency and a propensity of GlnRS mutants to misfold trigger the disease development. This report broadens the spectrum of brain pathologies elicited by protein misfolding and provides a paradigm for understanding the role of mutations in aminoacyl-tRNA synthetases in neurological diseases. PMID:26869582

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

    PubMed Central

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

    2015-01-01

    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, 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. PMID:25656172

  8. Endocannabinoids and endocannabinoid-related mediators: Targets, metabolism and role in neurological disorders.

    PubMed

    Iannotti, Fabio Arturo; Di Marzo, Vincenzo; Petrosino, Stefania

    2016-04-01

    The endocannabinoid system (ECS) is composed of two G protein-coupled receptors (GPCRs), the cannabinoid CB1 and CB2 receptors, and the two main endogenous lipid ligands of such receptors (also known as the "endocannabinoids"), anandamide and 2-arachidonoyl-glycerol. The ECS is a pleiotropic signalling system involved in all aspects of mammalian physiology and pathology, and for this reason it represents a potential target for the design and development of new therapeutic drugs. However, the endocannabinoids as well as some of their congeners also interact with a much wider range of receptors, including members of the Transient Receptor Potential (TRP) channels, Peroxisome Proliferator-Activated Receptors (PPARs), and other GPCRs. Indeed, following the discovery of the endocannabinoids, endocannabinoid-related lipid mediators, which often share the same metabolic pathways of the endocannabinoids, have also been identified or rediscovered. In this review article, we discuss the role of endocannabinoids and related lipids during physiological functions, as well as their involvement in some of the most common neurological disorders. PMID:26965148

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

    PubMed

    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. PMID:25656172

  10. The crystal structure of human GlnRS provides basis for the development of neurological disorders

    DOE PAGESBeta

    Ognjenovic, Jana; Wu, Jiang; Matthies, Doreen; Baxa, Ulrich; Subramaniam, Sriram; Ling, Jiqiang; Simonovic, Miljan

    2016-02-10

    Cytosolic glutaminyl-tRNA synthetase (GlnRS) is the singular enzyme responsible for translation of glutamine codons. Compound heterozygous mutations in GlnRS cause severe brain disorders by a poorly understood mechanism. Herein, we present crystal structures of the wild type and two pathological mutants of human GlnRS, which reveal, for the first time, the domain organization of the intact enzyme and the structure of the functionally important N-terminal domain (NTD). Pathological mutations mapping in the NTD alter the domain structure, and decrease catalytic activity and stability of GlnRS, whereas missense mutations in the catalytic domain induce misfolding of the enzyme. Our results suggestmore » that the reduced catalytic efficiency and a propensity of GlnRS mutants to misfold trigger the disease development. As a result, this report broadens the spectrum of brain pathologies elicited by protein misfolding and provides a paradigm for understanding the role of mutations in aminoacyl-tRNA synthetases in neurological diseases. Keywords« less

  11. National Survey and Community Advisory Board Development for a Bipolar Disorder Biobank

    PubMed Central

    Frye, Mark A; Doederlein, Allen; Koenig, Barbara; McElroy, Susan L; Nassan, Malik; Seymour, Lisa R; Biernacka, Joanna M; Daniels, Allen S

    2015-01-01

    Objectives To engage a national advocacy group and local stakeholders for guidance in developing a bipolar disorder biobank through a web-based survey and a community advisory board. Methods The Depression and Bipolar Support Alliance and the Mayo Clinic Bipolar Biobank conducted a national web-based survey inquiring about interest in participating in a biobank (i.e., giving DNA and clinical information). A community advisory board was convened to guide establishment of the biobank and identify key deliverables from the research project and for the community. Results Among 385 survey respondents, funding source (87%), professional opinion (76%), mental health consumer opinion (79%), and return of research results (91%) were believed to be important for considering study participation. Significantly more patients were willing to participate in a biobank managed by a university or clinic (78.2%) than one managed by government (63.4%) or industry (58.2%; both p < 0.001). The nine-member community advisory board expressed interest in research to help predict the likelihood of bipolar disorder developing in a child of an affected parent and which medications to avoid. The advisory board endorsed the use of a comprehension questionnaire to evaluate participants' understanding of the study (e.g., longevity of DNA specimens, right to remove samples, accessing medical records) as a means to strengthen the informed-consent process. Conclusions These national survey and community advisory data support the merit of establishing a biobank to enable studies of disease risk, provided that health records and research results are adequately protected. The goals of earlier diagnosis and individualized treatment of bipolar disorder were endorsed. PMID:26291791

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

    PubMed Central

    Meng, Xiangling; Wang, Wei; Lu, Hui; He, Ling-jie; Chen, Wu; Chao, Eugene S; Fiorotto, Marta L; Tang, Bin; Herrera, Jose A; Seymour, Michelle L; Neul, Jeffrey L; Pereira, Fred A; Tang, Jianrong; Xue, Mingshan; Zoghbi, Huda Y

    2016-01-01

    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-function mutations in MECP2, display impaired excitatory neurotransmission, the RTT phenotype can be largely reproduced in mice simply by removing MeCP2 from inhibitory GABAergic neurons. To determine what role excitatory signaling impairment might play in RTT pathogenesis, we generated conditional mouse models with Mecp2 either removed from or expressed solely in glutamatergic neurons. MeCP2 deficiency in glutamatergic neurons leads to early lethality, obesity, tremor, altered anxiety-like behaviors, and impaired acoustic startle response, which is distinct from the phenotype of mice lacking MeCP2 only in inhibitory neurons. These findings reveal a role for excitatory signaling impairment in specific neurobehavioral abnormalities shared by RTT and other postnatal neurological disorders. DOI: http://dx.doi.org/10.7554/eLife.14199.001 PMID:27328325

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

    PubMed

    Meng, Xiangling; Wang, Wei; Lu, Hui; He, Ling-Jie; Chen, Wu; Chao, Eugene S; Fiorotto, Marta L; Tang, Bin; Herrera, Jose A; Seymour, Michelle L; Neul, Jeffrey L; Pereira, Fred A; Tang, Jianrong; Xue, Mingshan; Zoghbi, Huda Y

    2016-01-01

    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-function mutations in MECP2, display impaired excitatory neurotransmission, the RTT phenotype can be largely reproduced in mice simply by removing MeCP2 from inhibitory GABAergic neurons. To determine what role excitatory signaling impairment might play in RTT pathogenesis, we generated conditional mouse models with Mecp2 either removed from or expressed solely in glutamatergic neurons. MeCP2 deficiency in glutamatergic neurons leads to early lethality, obesity, tremor, altered anxiety-like behaviors, and impaired acoustic startle response, which is distinct from the phenotype of mice lacking MeCP2 only in inhibitory neurons. These findings reveal a role for excitatory signaling impairment in specific neurobehavioral abnormalities shared by RTT and other postnatal neurological disorders. PMID:27328325

  14. Primary sleep disorders seen at a Neurology service-based sleep clinic in India: Patterns over an 8-year period

    PubMed Central

    Sharma, Piyush Kumar; Shukla, Garima; Gupta, Anupama; Goyal, Vinay; Srivastava, Achal; Behari, Madhuri

    2013-01-01

    There is an increasing awareness for recognition of sleep disorders in India; however, there is still a huge gap in the number of people suffering from various sleep disorders, in the community versus those visiting hospital clinics for the same. Ours is a neurology services-based sleep disorders clinic, which has evolved successfully over the last decade. In this study, we aimed to evaluate the changes in referral patterns and distribution of various sleep disorders in the patients presenting to the clinic. Materials and Methods: This is a retrospective chart review-based study on all patients seen over an 8-year period, divided into 2 groups comprising of patients seen during the first 4 years versus those seen over the next 4 years. Only those patients who had the sleep disorder as their presenting manifestation and those who had been formally interviewed with a pre-structured questionnaire detailing about the main features of the common sleep disorders according to the ICSD-R were included. Patients, in whom the sleep disorder could be clearly attributable to another neurological or systemic disorder, were excluded. Statistical analysis was carried out to identify the differences between the two groups as regards the distribution of various sleep disorders and other clinical data. Results: Among 710 patients registered in the clinic, 469 were included for analysis and 222 patients formed group 1 while 247 formed group 2. The main differences observed were in the form of a clear increase in the percentage of patients with sleep-related breathing disorders, sleep-related movement disorder, and the hypersomnias on comparison of distribution over the first 4 years versus the last 4 years; while a clear decline was seen in the number of patients with insomnia and parasomnias. A 3-fold increase was observed in the number of patients in whom polysomnography was obtained. Conclusion: The distribution of various sleep disorders as seen in a neurology service-based sleep

  15. Neurologic disorders in Medicaid vs privately insured children and working-age adults

    PubMed Central

    Geer, Joseph P.; Frick, Kevin; Carone, Marco

    2014-01-01

    Summary This retrospective, observational study reports health utilization and access patterns of Medicaid recipients for neurologic diseases compared to privately insured individuals seen in 2 hospitals at a single institution in the same time period. We reviewed records of patients and compared demographic characteristics, visit types, neurologic diagnoses, and all-cause mortality, by age group, when seen with Medicaid vs private insurance. Adults insured by Medicaid were more likely to present as inpatients and with life-threatening neurologic disease compared to privately insured patients. Moreover, adult patients presenting with neurologic disease on Medicaid had a higher all-cause mortality rate than privately insured patients. Similar disparities in neurologic disease were not observed in children. The relationship of these findings to patient educational status, household income, comorbidities, and the reasons prompting Medicaid eligibility require additional study. PMID:24790798

  16. Parallel fluctuations of psychiatric and neurological symptoms in a patient with multiple sclerosis and bipolar affective disorder.

    PubMed

    Salmaggi, A; Eoli, M; La Mantia, L; Erbetta, A

    1995-11-01

    The case of a female patient affected by simultaneously onsetting multiple sclerosis and bipolar affective disorder at age 33 is reported. Over the following years, the patient displayed minor mood fluctuations but, at the ages of 41 and 42 years, respectively, she suffered from a major depressive and a manic episode, both of which were concomitant with a marked worsening in her neurological condition. PMID:8613416

  17. A Learning Based Fiducial-driven Registration Scheme for Evaluating Laser Ablation Changes in Neurological Disorders

    PubMed Central

    Wan, Tao; Bloch, B.Nicolas; Danish, Shabbar; Madabhushi, Anant

    2014-01-01

    In this work, we present a novel learning based fiducial driven registration (LeFiR) scheme which utilizes a point matching technique to identify the optimal configuration of landmarks to better recover deformation between a target and a moving image. Moreover, we employ the LeFiR scheme to model the localized nature of deformation introduced by a new treatment modality - laser induced interstitial thermal therapy (LITT) for treating neurological disorders. Magnetic resonance (MR) guided LITT has recently emerged as a minimally invasive alternative to craniotomy for local treatment of brain diseases (such as glioblastoma multiforme (GBM), epilepsy). However, LITT is currently only practised as an investigational procedure world-wide due to lack of data on longer term patient outcome following LITT. There is thus a need to quantitatively evaluate treatment related changes between post- and pre-LITT in terms of MR imaging markers. In order to validate LeFiR, we tested the scheme on a synthetic brain dataset (SBD) and in two real clinical scenarios for treating GBM and epilepsy with LITT. Four experiments under different deformation profiles simulating localized ablation effects of LITT on MRI were conducted on 286 pairs of SBD images. The training landmark configurations were obtained through 2000 iterations of registration where the points with consistently best registration performance were selected. The estimated landmarks greatly improved the quality metrics compared to a uniform grid (UniG) placement scheme, a speeded-up robust features (SURF) based method, and a scale-invariant feature transform (SIFT) based method as well as a generic free-form deformation (FFD) approach. The LeFiR method achieved average 90% improvement in recovering the local deformation compared to 82% for the uniform grid placement, 62% for the SURF based approach, and 16% for the generic FFD approach. On the real GBM and epilepsy data, the quantitative results showed that Le

  18. Parental quality of life in complex paediatric neurologic disorders of unknown aetiology.

    PubMed

    van Nimwegen, K J M; Kievit, W; van der Wilt, G J; Schieving, J H; Willemsen, M A A P; Donders, A R T; Verhaak, C M; Grutters, J P C

    2016-09-01

    Complex paediatric neurology (CPN) patients generally present with non-specific symptoms, such as developmental delay, impaired movement and epilepsy. The diagnostic trajectory in these disorders is usually complicated and long-lasting, and may be burdensome to the patients and their parents. Additionally, as caring for a chronically ill child can be stressful and demanding, parents of these patients may experience impaired health-related quality of life (HRQoL). This study aims to assess parental HRQoL and factors related to it in CPN. Physical and mental HRQoL of 120 parents was measured and compared to the general population using the SF-12 questionnaire. Parents also completed this questionnaire for the measurement of patient HRQoL. Additional questionnaires were used to measure parental uncertainty (Visual Analogue Scale) and worry phenomena (Penn State Worry Questionnaire), and to obtain socio-demographic data. A linear mixed model with random effect was used to investigate which of these variables were associated with parental HRQoL. As compared to the general population, HRQoL of these parents appeared diminished. Fathers showed both lowered physical (51.76, p < 0.05) and mental (49.41, p < 0.01) HRQoL, whereas mothers only showed diminished mental (46.46, p < 0.01) HRQoL. Patient HRQoL and parental worry phenomena were significantly correlated with overall and mental parental HRQoL. The reduction in parental mental HRQoL is alarming, also because children strongly rely on their parents and parental mental health is known to influence children's health. Awareness of these problems among clinicians, and supportive care if needed are important to prevent exacerbation of the problems. PMID:27321953

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

    PubMed Central

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

    2016-01-01

    Background 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. Methods 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. Results 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. Conclusions 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.

  20. Neurological channelopathies

    PubMed Central

    Graves, T; Hanna, M

    2005-01-01

    Ion channels are membrane-bound proteins that perform key functions in virtually all human cells. Such channels are critically important for the normal function of the excitable tissues of the nervous system, such as muscle and brain. Until relatively recently it was considered that dysfunction of ion channels in the nervous system would be incompatible with life. However, an increasing number of human diseases associated with dysfunctional ion channels are now recognised. Such neurological channelopathies are frequently genetically determined but may also arise through autoimmune mechanisms. In this article clinical, genetic, immunological, and electrophysiological aspects of this expanding group of neurological disorders are reviewed. Clinical situations in which a neurological channelopathy should enter into the differential diagnosis are highlighted. Some practical guidance on how to investigate and treat this complex group of disorders is also included. PMID:15640425

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

    PubMed Central

    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

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

    ERIC Educational Resources Information Center

    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…

  3. Immune Parameters That Distinguish Multiple Sclerosis Patients from Patients with Other Neurological Disorders at Presentation

    PubMed Central

    Mouzaki, Athanasia; Rodi, Maria; Dimisianos, Nikolaos; Emmanuil, Andreas; Kalavrizioti, Dimitra; Lagoudaki, Rosa; Grigoriadis, Nikolaos C.; Papathanasopoulos, Panagiotis

    2015-01-01

    indices revealed higher intrathecal IgG synthesis in MS patients, and higher blood-CSF barrier dysfunction in IND patients. The IgG index correlated with CSF IL-4 in MS patients only. Conclusions We found no signature cytokines or profiles thereof in MS patients at presentation. Only IND patients presented with a clear Th1 cytokine polarization in serum and CSF. The parameters that distinguished MS patients from patients with other neurological disorders were IgG intrathecal synthesis, the IgG index and its correlation with CSF IL-4 levels. PMID:26317430

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

    NASA Technical Reports Server (NTRS)

    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

  5. Use of botulinum toxin type A in the management of patients with neurological disorders: a national survey

    PubMed Central

    Smania, Nicola; Colosimo, Carlo; Bentivoglio, Anna Rita; Sandrini, Giorgio; Picelli, Alessandro

    2013-01-01

    Summary The aim of this survey was to provide an overview of important issues relating to therapeutic strategies based on botulinum toxin type A injection for the treatment of patients with neurological disorders. Two hundred and ten physicians from neurology and neurorehabilitation units in Italian hospitals answered a questionnaire exploring some clinical aspects of the use of botulinum toxin type A in patients with spasticity/dystonia. 66% of the physicians treated patients with dystonia, 80% treated adults with spasticity, and 35% treated children with cerebral palsy. Palpation with no instrumental guidance was the injection technique most commonly used for treating patients with dystonia, spasticity and cerebral palsy; 57% of the physicians evaluated patients instrumentally before toxin injection, while 45% assessed post-injection improvements by instrumental means; 78% of the physicians prescribed (when appropriate) rehabilitation procedures after toxin injection. Our results seem to show that the routine use of botulinum toxin in clinics is far from standardized. PMID:24598392

  6. REM Sleep Behavior Disorder and REM Sleep Without Atonia as an Early Manifestation of Degenerative Neurological Disease

    PubMed Central

    McCarter, Stuart J.; St Louis, Erik K.

    2013-01-01

    Rapid eye movement (REM) sleep behavior disorder (RBD) is a parasomnia characterized by repeated episodes of dream enactment behavior and REM sleep without atonia (RSWA) during polysomnography recording. RSWA is characterized by increased phasic or tonic muscle activity seen on polysomnographic electromyogram channels. RSWA is a requisite diagnostic feature of RBD, but may also be seen in patients without clinical symptoms or signs of dream enactment as an incidental finding in neurologically normal individuals, especially in patients receiving antidepressant therapy. RBD may be idiopathic or symptomatic. Patients with idiopathic RBD often later develop other neurological features including parkinsonism, orthostatic hypotension, anosmia, or cognitive impairment. RSWA without clinical symptoms as well as clinically overt RBD also often occurs concomitantly with the α-synucleinopathy family of neurodegenerative disorders, which includes idiopathic Parkinson disease, Lewy body dementia, and multiple system atrophy. This review article considers the epidemiology of RBD, clinical and polysomnographic diagnostic standards for both RBD and RSWA, previously reported associations of RSWA and RBD with neurodegenerative disorders and other potential causes, the pathophysiology of which brain structures and networks mediate dysregulation of REM sleep muscle atonia, and considerations for the effective and safe management of RBD. PMID:22328094

  7. Teaching Neurophysiology, Neuropharmacology, and Experimental Design Using Animal Models of Psychiatric and Neurological Disorders

    ERIC Educational Resources Information Center

    Morsink, Maarten C.; Dukers, Danny F.

    2009-01-01

    Animal models have been widely used for studying the physiology and pharmacology of psychiatric and neurological diseases. The concepts of face, construct, and predictive validity are used as indicators to estimate the extent to which the animal model mimics the disease. Currently, we used these three concepts to design a theoretical assignment to…

  8. Tourette's Disorder: Genetic Update, Neurological Correlates, and Evidence-Based Interventions

    ERIC Educational Resources Information Center

    Phelps, LeAdelle

    2008-01-01

    This article provides an update of the search for genetic markers related to Tourette's Disorder. The probable neurophysiology of the disorder is reviewed. Frequently prescribed medications are related to the probable biological bases of the disorder. Behavioral interventions and assessment tools are examined. It is concluded that evidence based…

  9. Intrathecal-specific glutamic acid decarboxylase antibodies at low titers in autoimmune neurological disorders.

    PubMed

    Sunwoo, Jun-Sang; Chu, Kon; Byun, Jung-Ick; Moon, Jangsup; Lim, Jung-Ah; Kim, Tae-Joon; Lee, Soon-Tae; Jung, Keun-Hwa; Park, Kyung-Il; Jeon, Daejong; Jung, Ki-Young; Kim, Manho; Lee, Sang Kun

    2016-01-15

    Autoantibodies to glutamic acid decarboxylase (Gad-Abs) are implicated in various neurological syndromes. The present study aims to identify intrathecal-specific GAD-Abs and to determine clinical manifestations and treatment outcomes. Nineteen patients had GAD-Abs in cerebrospinal fluid but not in paired serum samples. Neurological syndromes included limbic encephalitis, temporal lobe epilepsy, cerebellar ataxia, autonomic dysfunction, and stiff-person syndrome. Immunotherapy had beneficial effects in 57.1% of patients, and the patients with limbic encephalitis responded especially well to immunotherapy. Intrathecal-specific antibodies to GAD at low titers may appear as nonspecific markers of immune activation within the central nervous system rather than pathogenic antibodies causing neuronal dysfunction. PMID:26711563

  10. Gene Prioritization by Integrated Analysis of Protein Structural and Network Topological Properties for the Protein-Protein Interaction Network of Neurological Disorders.

    PubMed

    Paul, Yashna; Hasija, Yasha

    2016-01-01

    Neurological disorders are known to show similar phenotypic manifestations like anxiety, depression, and cognitive impairment. There is a need to identify shared genetic markers and molecular pathways in these diseases, which lead to such comorbid conditions. Our study aims to prioritize novel genetic markers that might increase the susceptibility of patients affected with one neurological disorder to other diseases with similar manifestations. Identification of pathways involving common candidate markers will help in the development of improved diagnosis and treatments strategies for patients affected with neurological disorders. This systems biology study for the first time integratively uses 3D-structural protein interface descriptors and network topological properties that characterize proteins in a neurological protein interaction network, to aid the identification of genes that are previously not known to be shared between these diseases. Results of protein prioritization by machine learning have identified known as well as new genetic markers which might have direct or indirect involvement in several neurological disorders. Important gene hubs have also been identified that provide an evidence for shared molecular pathways in the neurological disease network. PMID:27034906

  11. Gene Prioritization by Integrated Analysis of Protein Structural and Network Topological Properties for the Protein-Protein Interaction Network of Neurological Disorders

    PubMed Central

    Paul, Yashna

    2016-01-01

    Neurological disorders are known to show similar phenotypic manifestations like anxiety, depression, and cognitive impairment. There is a need to identify shared genetic markers and molecular pathways in these diseases, which lead to such comorbid conditions. Our study aims to prioritize novel genetic markers that might increase the susceptibility of patients affected with one neurological disorder to other diseases with similar manifestations. Identification of pathways involving common candidate markers will help in the development of improved diagnosis and treatments strategies for patients affected with neurological disorders. This systems biology study for the first time integratively uses 3D-structural protein interface descriptors and network topological properties that characterize proteins in a neurological protein interaction network, to aid the identification of genes that are previously not known to be shared between these diseases. Results of protein prioritization by machine learning have identified known as well as new genetic markers which might have direct or indirect involvement in several neurological disorders. Important gene hubs have also been identified that provide an evidence for shared molecular pathways in the neurological disease network. PMID:27034906

  12. 38 CFR 4.124a - Schedule of ratings-neurological conditions and convulsive disorders.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... result of such review, VA will apply 38 CFR 3.114, if applicable. 8046Cerebral arteriosclerosis: Purely...-psychotic organic psychiatric disturbance (psychotic, psychoneurotic or personality disorder) if diagnosed... psychotic or psychroneurotic disorder will be rated under the appropriate diagnostic code. The...

  13. Autoimmune neurological disorders associated with group-A beta-hemolytic streptococcal infection.

    PubMed

    Hachiya, Yasuo; Miyata, Rie; Tanuma, Naoyuki; Hongou, Kazuhisa; Tanaka, Keiko; Shimoda, Konomi; Kanda, Sachiko; Hoshino, Ai; Hanafusa, Yukiko; Kumada, Satoko; Kurihara, Eiji; Hayashi, Masaharu

    2013-08-01

    Although central nervous system (CNS) disorders associated with group-A beta-hemolytic streptococcal (GABHS) infection occur only rarely, Sydenham's chorea is a well-recognized disease that can arise following infection. Children may develop a tic, obsessive compulsive disorder (OCD), and extrapyramidal movement subsequent to GABHS infection. These disorders have been termed pediatric autoimmune neuropsychiatric disorders associated with streptococci (PANDAS). Herein we report one case each of acute disseminated encephalomyelitis (ADEM), PANDAS and subacute encephalitis associated with GABHS infection. To evaluate the pathogenesis of the CNS disorders associated with GABHS infection, we measured levels of neurotransmitters, cytokines, anti-neuronal autoantibodies, and performed immunohistochemistry using patient sera to stain human brain sections. All three cases showed psychiatric behavioral disorders. Immunotherapy was effective, and homovanillic acid levels in the cerebrospinal fluid (CSF) were elevated at the acute stage in all three cases. In each case of ADEM and PANDAS, immunohistochemistry demonstrated neuronal impairment in the basal ganglia during the acute stage. Neuronal immunoreactivity was visualized in the cerebral cortex at the acute stage in the case of subacute encephalitis. There was no direct correlation between immunoreactivity of patient sera on the brain sections and positivity of anti-neuronal autoantibodies or CSF biomarkers. The results suggest that autoimmune responses may modulate neurotransmission, and the use of patient serum for immunohistochemistry is a sensitive screening method for the detection of anti-neuronal autoantibodies in CNS disorders associated with GABHS infection. PMID:23142103

  14. Neurology and orthopaedics

    PubMed Central

    Houlden, Henry; Charlton, Paul; Singh, Dishan

    2007-01-01

    Neurology encompasses all aspects of medicine and surgery, but is closer to orthopaedic surgery than many other specialities. Both neurological deficits and bone disorders lead to locomotor system abnormalities, joint complications and limb problems. The main neurological conditions that require the attention of an orthopaedic surgeon are disorders that affect the lower motor neurones. The most common disorders in this group include neuromuscular disorders and traumatic peripheral nerve lesions. Upper motor neurone disorders such as cerebral palsy and stroke are also frequently seen and discussed, as are chronic conditions such as poliomyelitis. The management of these neurological problems is often coordinated in the neurology clinic, and this group, probably more than any other, requires a multidisciplinary team approach. PMID:17308288

  15. Regulation of ABC efflux transporters at blood-brain barrier in health and neurological disorders.

    PubMed

    Qosa, Hisham; Miller, David S; Pasinelli, Piera; Trotti, Davide

    2015-12-01

    The strength of the blood-brain barrier (BBB) in providing protection to the central nervous system from exposure to circulating chemicals is maintained by tight junctions between endothelial cells and by a broad range of transporter proteins that regulate exchange between CNS and blood. The most important transporters that restrict the permeability of large number of toxins as well as therapeutic agents are the ABC transporters. Among them, P-gp, BCRP, MRP1 and MRP2 are the utmost studied. These efflux transporters are neuroprotective, limiting the brain entry of neurotoxins; however, they could also restrict the entry of many therapeutics and contribute to CNS pharmacoresistance. Characterization of several regulatory pathways that govern expression and activity of ABC efflux transporters in the endothelium of brain capillaries have led to an emerging consensus that these processes are complex and contain several cellular and molecular elements. Alterations in ABC efflux transporters expression and/or activity occur in several neurological diseases. Here, we review the signaling pathways that regulate expression and transport activity of P-gp, BCRP, MRP1 and MRP2 as well as how their expression/activity changes in neurological diseases. This article is part of a Special Issue entitled SI: Neuroprotection. PMID:26187753

  16. Nose-to-brain drug delivery by nanoparticles in the treatment of neurological disorders.

    PubMed

    Ong, Wei-Yi; Shalini, Suku-Maran; Costantino, Luca

    2014-01-01

    Many potential drugs for the treatment of neurological diseases are unable to reach the brain in sufficient enough concentrations to be therapeutic because of the blood brain barrier. On the other hand, direct delivery of drugs to the brain provides the possibility of a greater therapeutic-toxic ratio than with systemic drug delivery. The use of intranasal delivery of therapeutic agents to the brain provides a means of bypassing the blood brain barrier in a non-invasive manner. In this respect, nanosized drug carriers were shown to enhance the delivery of drugs to CNS compared to equivalent drug solution formulations. Neurological conditions that have been studied in animal models that could benefit from nose-to-brain delivery of nanotherapeutics include pain, epilepsy, neurodegenerative disease and infectious diseases. The delivery of drugs to the brain via the nose-to-brain route holds great promise, on the basis of preclinical research by means of drug delivery systems such as polymeric nanoparticles and clinical data related to intranasal delivery to CNS of large molecular weight biologics administered in solution, but safety issues about toxicity on nasal mucosa, Np transport into the brain, delivery only to specific brain regions and variability in the adsorbed dose still represent research topics that need to be considered, with a view of clinical translation of these delivery systems. PMID:25039773

  17. Manic depressive psychosis and schizophrenia are neurological disorders at the extremes of CNS maturation and nutritional disorders associated with a deficit in marine fat.

    PubMed

    Saugstad, L F

    2001-12-01

    The maturational theory of brain development comprises manic depressive psychosis and schizophrenia. It holds that the disorders are part of human diversity in growth and maturation, which explains their ubiquity, shared susceptibility genes and multifactorial inheritance. Rate of maturation and age at puberty are the genotype; the disorders are localized at the extremes with normality in between. This is based on the association between onset of puberty and the final regressive event, with pruning of 40% of excitatory synapses leaving the inhibitory ones fairly unchanged. This makes excitability, a fundamental property of nervous tissue, a distinguishing factor: the earlier puberty, the greater excitability--the later puberty, the greater deficit. Biological treatment supports deviation from the norm: neuroleptics are convulsant; antidepressives are anti-epiletogenic. There is an association between onset of puberty and body-build: early maturers are pyknic broad-built, late ones linearly leptosomic. This discrepancy is similar to that in the two disorders, supporting the theory that body-build is the phenotype. Standard of living is the environmental factor, which affects pubertal age and shifts the panorama of mental illness accordingly. Unnatural death has increased with antipsychotics. Other treatment is needed. PUFA deficit has been observed in RBC in both disorders and striking improvements with addition of minor amounts of PUFA. This supports that dietary deficit might cause psychotic development and that prevention is possible. Other neurological disorders also profit from PUFA, underlining a general deficit in the diet. PMID:11918426

  18. Videofluoroscopy in the assessment of feeding disorders of children with neurological problems.

    PubMed

    Morton, R E; Bonas, R; Fourie, B; Minford, J

    1993-05-01

    A multidisciplinary assessment, including videofluoroscopy, was carried out on 14 children with feeding difficulties associated with neurological problems. Recommendations were made on all aspects of feeding and the trunk position was changed for half of the patients. A later interview with the parents confirmed that the recommendations had been helpful. The optimum trunk position for feeding was determined during videofluoroscopy by positioning the patient in the erect or reclined position, and also by analysis of relative difficulties during the oral and pharyngeal phases of swallowing. Those with difficulties mainly in the oral phase fed best in the reclined position; those with difficulties mainly in the pharyngeal phase fed best in the erect position, particularly if they had upper-oesophageal sphincter spasm in association with a tonic labyrinthine reflex. PMID:8495819

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

    NASA Astrophysics Data System (ADS)

    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.

  20. Apotemnophilia, body integrity identity disorder or xenomelia? Psychiatric and neurologic etiologies face each other

    PubMed Central

    Sedda, Anna; Bottini, Gabriella

    2014-01-01

    This review summarizes the available studies of a rare condition in which individuals seek the amputation of a healthy limb or desire to be paraplegic. Since 1977, case reports and group studies have been produced, trying to understand the cause of this unusual desire. The main etiological hypotheses are presented, from the psychological/psychiatric to the most recent neurologic explanation. The paradigms adopted and the clinical features are compared across studies and analyzed in detail. Finally, future directions and ethical implications are discussed. A proposal is made to adopt a multidisciplinary approach that comprises state-of-the-art technologies and a variety of theoretical models, including both body representation and psychological and sexual components. PMID:25045269

  1. Removal kinetics of antibodies against glutamic acid decarboxylase by various plasmapheresis modalities in the treatment of neurological disorders.

    PubMed

    Ohkubo, Atsushi; Okado, Tomokazu; Kurashima, Naoki; Maeda, Takuma; Miyamoto, Satoko; Nakamura, Ayako; Seshima, Hiroshi; Iimori, Soichiro; Sohara, Eisei; Uchida, Shinichi; Rai, Tatemitsu

    2014-06-01

    Plasmapheresis is one of the acute treatment modalities for neurological disorders associated with antibodies against glutamic acid decarboxylase (anti-GAD). However, there is little information about the removal kinetics of anti-GAD by various plasmapheresis modalities. Here, we investigated the removal rate of anti-GAD and fibrinogen (Fib) by immunoadsorption (IA), plasma exchange using a conventional plasma separator (OP-PE), and plasma exchange using a high cut-off selective membrane plasma separator (EC-PE) in two cases of anti-GAD-associated neurological diseases. In case 1, IA and OP-PE were used, and the percent reductions were as follows: anti-GAD: 38.2% and 69.1% and Fib: 67.7% and 68.2%, respectively. In case 2, OP-PE and EC-PE were used, and the percent reductions were as follows: anti-GAD: 65.8% and 48.5% and Fib: 68.5% and 19.8%, respectively. OP-PE could remove anti-GAD more efficiently than IA. Further, EC-PE could maintain coagulation factors such as Fib better than IA and OP-PE. It is important to select the appropriate plasmapheresis modality on the basis of the removal kinetics. PMID:24965288

  2. [Transition to adult care for children with chronic neurological disorders; which is the best way to make it?].

    PubMed

    Moreno Villares, José Manuel

    2014-01-01

    Chronic neurological disorders in children have significant effects on adult medical and social function. Transition from pediatric to adult services is a complex process. No objective data are available to inform physicians about the most effective approach. Nevertheless the most recommended approach is a joint pediatric/adult transition clinic. Malnutrition, either under or overnutrition, is a common condition among neurologically impaired children. Undernutrition is most prevalent, and its causes are diverse: insufficient caloric intake, excessive nutrient losses and abnormal energy metabolism. Malnutrition is associated with significant morbidity, while nutritional rehabilitation improves overall health as well as quality of life. It is not easy to determine which the nutritional needs in these patients are. Besides, they often present difficulties for oral feeding, mainly due to oromotor dysfunction. Gastrointestinal symptoms, gastro esophageal reflux and constipation, as well as spasticity, scoliosis and joint deformities contribute to these difficulties. Because of that, an assessment of nutritional status should be performed periodically, and to assess efficacy and security of oral intake. If modifying oral diet we cannot confirm an adequate support, a nasogastric tube or a gastrostomy need to be considered. Often, a fundoplication is associated to the placement of a gastrostomy. Although the outcomes in a better nutritional status and quality of life are often obtained, it is not an easy decision for families. PMID:25077342

  3. The Involvement of Neuron-Specific Factors in Dendritic Spinogenesis: Molecular Regulation and Association with Neurological Disorders.

    PubMed

    Hu, Hsiao-Tang; Shih, Pu-Yun; Shih, Yu-Tzu; Hsueh, Yi-Ping

    2016-01-01

    Dendritic spines are the location of excitatory synapses in the mammalian nervous system and are neuron-specific subcellular structures essential for neural circuitry and function. Dendritic spine morphology is determined by the F-actin cytoskeleton. F-actin remodeling must coordinate with different stages of dendritic spinogenesis, starting from dendritic filopodia formation to the filopodia-spines transition and dendritic spine maturation and maintenance. Hundreds of genes, including F-actin cytoskeleton regulators, membrane proteins, adaptor proteins, and signaling molecules, are known to be involved in regulating synapse formation. Many of these genes are not neuron-specific, but how they specifically control dendritic spine formation in neurons is an intriguing question. Here, we summarize how ubiquitously expressed genes, including syndecan-2, NF1 (encoding neurofibromin protein), VCP, and CASK, and the neuron-specific gene CTTNBP2 coordinate with neurotransmission, transsynaptic signaling, and cytoskeleton rearrangement to control dendritic filopodia formation, filopodia-spines transition, and dendritic spine maturation and maintenance. The aforementioned genes have been associated with neurological disorders, such as autism spectrum disorders (ASDs), mental retardation, learning difficulty, and frontotemporal dementia. We also summarize the corresponding disorders in this report. PMID:26819769

  4. [Neurologic and mental disorders in patients with migraine and in their children].

    PubMed

    Sviridova, E I; Kalashnikova, L A; Asanova, L M

    1990-01-01

    To specify indications for differentiated therapy, a study was made of the characteristic features of the psychopathological picture of the interictal period of migraine in 50 women aged 28 to 60 years suffering from different forms of migrainous attacks. Besides, the neuropsychic disorders were also examined in those patients' children. The first group was made up of migraine patients in whom paroxysmal psychic disorders resembling convulsion-free epileptic attacks ranked first in the clinical structure of the interictal period. In the second group patients, of paramount importance was the hysterical symptomatology. The third group patients suffered from somatized depressions. The differentiated therapy of psychic disorders of the interictal period favoured the deceleration of migrainous attacks in all the three groups patients. PMID:2175132

  5. Genetic disruption of voltage-gated calcium channels in psychiatric and neurological disorders

    PubMed Central

    Heyes, Samuel; Pratt, Wendy S.; Rees, Elliott; Dahimene, Shehrazade; Ferron, Laurent; Owen, Michael J.; Dolphin, Annette C.

    2015-01-01

    This review summarises genetic studies in which calcium channel genes have been connected to the spectrum of neuropsychiatric syndromes, from bipolar disorder and schizophrenia to autism spectrum disorders and intellectual impairment. Among many other genes, striking numbers of the calcium channel gene superfamily have been implicated in the aetiology of these diseases by various DNA analysis techniques. We will discuss how these relate to the known monogenic disorders associated with point mutations in calcium channels. We will then examine the functional evidence for a causative link between these mutations or single nucleotide polymorphisms and the disease processes. A major challenge for the future will be to translate the expanding psychiatric genetic findings into altered physiological function, involvement in the wider pathology of the diseases, and what potential that provides for personalised and stratified treatment options for patients. PMID:26386135

  6. Role of histaminergic system in blood-brain barrier dysfunction associated with neurological disorders.

    PubMed

    Bañuelos-Cabrera, Ivette; Valle-Dorado, María Guadalupe; Aldana, Blanca Irene; Orozco-Suárez, Sandra Adela; Rocha, Luisa

    2014-11-01

    Blood-brain barrier (BBB) disruption has been associated with several acute and chronic brain disorders such as Alzheimer's disease, Parkinson's disease and epilepsy. This represents a critical situation because damaged integrity of the BBB is related to the influx of immune mediators, plasma proteins and other outside elements from blood to the central nervous system (CNS) that may trigger a cascade of events that leads to neuroinflammation. In this review, evidence that mast cells and the release of factors such as histamine play an important role in the neuroinflammatory process associated with brain disorders such as Alzheimer's disease, Parkinson's disease and epilepsy is presented. PMID:25446620

  7. Central Nervous System-Peripheral Immune System Dialogue in Neurological Disorders: Possible Application of Neuroimmunology in Urology.

    PubMed

    Park, Hyun-Sun; Park, Min-Jung; Kwon, Min-Soo

    2016-05-01

    Previous concepts of immune-privileged sites obscured the role of peripheral immune cells in neurological disorders and excluded the consideration of the potential benefits of immunotherapy. Recently, however, numerous studies have demonstrated that the blood-brain barrier in the central nervous system is an educational barrier rather than an absolute barrier to peripheral immune cells. Emerging knowledge of immune-privileged sites suggests that peripheral immune cells can infiltrate these sites via educative gates and that crosstalk can occur between infiltrating immune cells and the central nervous system parenchyma. This concept can be expanded to the testis, which has long been considered an immune-privileged site, and to neurogenic bladder dysfunction. Thus, we propose that the relationship between peripheral immune cells, the brain, and the urologic system should be considered as an additional possible mechanism in urologic diseases, and that immunotherapy might be an alternative therapeutic strategy in treating neurogenic bladder dysfunction. PMID:27230462

  8. Neuropeptide Y (NPY) and the central nervous system: distribution effects and possible relationship to neurological and psychiatric disorders.

    PubMed

    Wahlestedt, C; Ekman, R; Widerlöv, E

    1989-01-01

    1. NPY is a 36 amino acid tyrosine-rich peptide. It is one of the most abundant and widely distributed neuropeptides known today within the central nervous system with particularly high concentrations in the hypothalamus and in several limbic regions. 2. NPY seems to coexist with other on neurotransmitters like somatostatin, galanin, GABA and the catecholamines noradrenaline and adrenaline in discrete brain regions. 3. NPY binding sites are widely distributed in the brain. However they do not always overlap with the distribution of NPY-like immunoreactivity. 4. NPY is suggested to be involved in a large number of neuroendocrine functions, stress responses, circadian rhythms, central autonomic functions, eating and drinking behaviour, and sexual and motor behaviour. 5. Psychotropic drugs and neurotoxins can alter the NPY concentrations in discrete brain regions. 6. It is possible that NPY is related to various neurological and psychiatric illnesses, like Huntington's chorea, Alzheimer's disease, Parkinson's disease, eating disorders, and major depressive illness. PMID:2664885

  9. Central Nervous System-Peripheral Immune System Dialogue in Neurological Disorders: Possible Application of Neuroimmunology in Urology

    PubMed Central

    2016-01-01

    Previous concepts of immune-privileged sites obscured the role of peripheral immune cells in neurological disorders and excluded the consideration of the potential benefits of immunotherapy. Recently, however, numerous studies have demonstrated that the blood–brain barrier in the central nervous system is an educational barrier rather than an absolute barrier to peripheral immune cells. Emerging knowledge of immune-privileged sites suggests that peripheral immune cells can infiltrate these sites via educative gates and that crosstalk can occur between infiltrating immune cells and the central nervous system parenchyma. This concept can be expanded to the testis, which has long been considered an immune-privileged site, and to neurogenic bladder dysfunction. Thus, we propose that the relationship between peripheral immune cells, the brain, and the urologic system should be considered as an additional possible mechanism in urologic diseases, and that immunotherapy might be an alternative therapeutic strategy in treating neurogenic bladder dysfunction. PMID:27230462

  10. The pharmacology of neurotrophic treatment with Cerebrolysin: brain protection and repair to counteract pathologies of acute and chronic neurological disorders.

    PubMed

    Masliah, E; Díez-Tejedor, E

    2012-04-01

    Neurotrophic factors are considered as part of the therapeutic strategy for neurological disorders like dementia, stroke and traumatic brain injury. Cerebrolysin is a neuropeptide preparation which mimics the action of endogenous neurotrophic factors on brain protection and repair. In dementia models, Cerebrolysin decreases β-amyloid deposition and microtubule-associated protein tau phosphorylation by regulating glycogen synthase kinase-3β and cyclin-dependent kinase 5 activity, increases synaptic density and restores neuronal cytoarchitecture. These effects protect integrity of the neuronal circuits and thus result in improved cognitive and behavioral performance. Furthermore, Cerebrolysin enhances neurogenesis in the dentate gyrus, the basis for neuronal replacement therapy in neurodegenerative diseases. Experimental studies in stroke animal models have shown that Cerebrolysin stabilizes the structural integrity of cells by inhibition of calpain and reduces the number of apoptotic cells after ischemic lesion. Cerebrolysin induces restorative processes, decreases infarct volume and edema formation and promotes functional recovery. Stroke-induced neurogenesis in the subventricular zone was also promoted by Cerebrolysin, thus supporting the brain's self-repair after stroke. Both, traumatic brain and spinal cord injury conditions stimulate the expression of natural neurotrophic factors to promote repair and regeneration processes -axonal regeneration, neuronal plasticity and neurogenesis- that is considered to be crucial for the future recovery. Neuroprotective effects of Cerebrolysin on experimentally induced traumatic spinal cord injury have shown that Cerebrolysin prevents apoptosis of lesioned motoneurons and promotes functional recovery. This section summarizes the most relevant data on the pharmacology of Cerebrolysin obtained from in vitro assays (biochemical and cell cultures) and in vivo animal models of acute and chronic neurological disorders. PMID

  11. Fasting and Systemic Insulin Signaling Regulate Phosphorylation of Brain Proteins That Modulate Cell Morphology and Link to Neurological Disorders.

    PubMed

    Li, Min; Quan, Chao; Toth, Rachel; Campbell, David G; MacKintosh, Carol; Wang, Hong Yu; Chen, Shuai

    2015-12-11

    Diabetes is strongly associated with cognitive decline, but the molecular reasons are unknown. We found that fasting and peripheral insulin promote phosphorylation and dephosphorylation, respectively, of specific residues on brain proteins including cytoskeletal regulators such as slit-robo GTPase-activating protein 3 (srGAP3) and microtubule affinity-regulating protein kinases (MARKs), in which deficiency or dysregulation is linked to neurological disorders. Fasting activates protein kinase A (PKA) but not PKB/Akt signaling in the brain, and PKA can phosphorylate the purified srGAP3. The phosphorylation of srGAP3 and MARKs were increased when PKA signaling was activated in primary neurons. Knockdown of PKA decreased the phosphorylation of srGAP3. Furthermore, WAVE1, a protein kinase A-anchoring protein, formed a complex with srGAP3 and PKA in the brain of fasted mice to facilitate the phosphorylation of srGAP3 by PKA. Although brain cells have insulin receptors, our findings are inconsistent with the down-regulation of phosphorylation of target proteins being mediated by insulin signaling within the brain. Rather, our findings infer that systemic insulin, through a yet unknown mechanism, inhibits PKA or protein kinase(s) with similar specificity and/or activates an unknown phosphatase in the brain. Ser(858) of srGAP3 was identified as a key regulatory residue in which phosphorylation by PKA enhanced the GAP activity of srGAP3 toward its substrate, Rac1, in cells, thereby inhibiting the action of this GTPase in cytoskeletal regulation. Our findings reveal novel mechanisms linking peripheral insulin sensitivity with cytoskeletal remodeling in neurons, which may help to explain the association of diabetes with neurological disorders such as Alzheimer disease. PMID:26499801

  12. 78 FR 15024 - National Institute of Neurological Disorders and Stroke, Interagency Pain Research Coordinating...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-03-08

    ... the 2011 Institute of Medicine report titled ``Relieving Pain in America: A Blueprint for Transforming... and disorders associated with pain; (b) identify critical gaps in basic and clinical research on the..., management, and research as recommended in the 2011 Institute of Medicine Report titled ``Relieving Pain...

  13. 75 FR 70014 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-11-16

    ... grant applications. Place: National Institutes of Health, Neuroscience Center, 6001 Executive Boulevard... Administrator, Scientific Review Branch, Division of Extramural Research, NINDS/NIH/DHHS, Neuroscience Center... Disorders; 93.854, Biological Basis Research in the Neurosciences, National Institutes of Health, HHS)...

  14. 75 FR 42758 - National Institute of Neurological Disorders and Stroke; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-07-22

    ... grant applications. Place: National Institutes of Health, Neuroscience Center, 6001 Executive Boulevard... Administrator, Scientific Review Branch, Division of Extramural Research, NINDS/NIH/DHHS/Neuroscience Center... Disorders; 93.854, Biological Basis Research in the Neurosciences, National Institutes of Health, HHS)...

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

    PubMed

    Beggs, Clive B

    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

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

    PubMed Central

    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

  17. The mammalian tachykinin ligand-receptor system: an emerging target for central neurological disorders

    PubMed Central

    Pantaleo, Nick; Chadwick, Wayne; Park, Sung-Soo; Wang, Liyun; Zhou, Yu; Martin, Bronwen; Maudsley, Stuart

    2010-01-01

    Our understanding of the complex signaling neurophysiology of the central nervous system has facilitated the exploration of potential novel receptor-ligand system targets for disorders of this most complex organ. In recent years, many relatively neglected receptor-ligand systems have been re-evaluated with respect to their ability to potently modulate discrete tracts in the central nervous system. One such system is the tachykinin (previously neurokinin) system. The multiple heptahelical G protein-coupled receptors and neuropeptide ligands that comprise this system may be significantly involved in more central nervous systems actions than previously thought, including sleep disorders, amyotrophic lateral sclerosis, Alzheimer’s and Machado-Joseph disease. The development of our understanding of the role of the tachykinin receptor-ligand system in higher order central functions is likely to allow the creation of more specific and selective tachykinin-related neurotherapeutics. PMID:20632965

  18. Neurology of sleep and sleep-related breathing disorders and their relationships to sleep bruxism.

    PubMed

    Simmons, Jerald H

    2012-02-01

    Conditions that affect sleep can impact overall health. More than 70 million Americans suffer from problems with sleep. The purpose of this article is to provide the basic science of sleep physiology and how it relates to disorders that are pertinent to dentistry. Concepts are presented that explain airway dynamics and how the jaw and tongue influence airway obstruction. Additionally, explanation is given on an association between temporomandibularj aw dysfunction and bruxism during sleep. PMID:22416635

  19. [Rehabilitative treatment of patients with complicated spinal injuries and trophic disorders in specialized neurological center].

    PubMed

    Khashchuk, A V; Bur'ianov, O A; Nen'ko, A M; Laksha, A M

    2014-01-01

    The analysis of treatment results 132 patients with consequences with spine injury and the presence of venous disorders which in conditions of specialized health resort management system applied in complex restorative treatment, which includes patogeneti no-reasonable comprehensive preparation, surgery and further restorative treatment. Based on the analysis and systematization of the results developed diagnostic and therapeutic algorithm and algorithm for planning surgical tactics. PMID:24908972

  20. Bridging the Gap in Neurotherapeutic Discovery and Development: The Role of the National Institute of Neurological Disorders and Stroke in Translational Neuroscience.

    PubMed

    Mott, Meghan; Koroshetz, Walter

    2015-07-01

    The mission of the National Institute of Neurological Disorders and Stroke (NINDS) is to seek fundamental knowledge about the brain and nervous system and to use that knowledge to reduce the burden of neurological disease. NINDS supports early- and late-stage therapy development funding programs to accelerate preclinical discovery and the development of new therapeutic interventions for neurological disorders. The NINDS Office of Translational Research facilitates and funds the movement of discoveries from the laboratory to patients. Its grantees include academics, often with partnerships with the private sector, as well as small businesses, which, by Congressional mandate, receive > 3% of the NINDS budget for small business innovation research. This article provides an overview of NINDS-funded therapy development programs offered by the NINDS Office of Translational Research. PMID:26081907

  1. Neurological disorder in dairy cattle associated with consumption of beer residues contaminated with Aspergillus clavatus.

    PubMed

    Loretti, Alexandre Paulino; Colodel, Edson Moleta; Driemeier, David; Corrêa, André Mendes; Bangel, Jorge José; Ferreiro, Laerte

    2003-03-01

    A neurological syndrome in dairy cattle associated with consumption of moldy beer residues is described. The disease occurred on 1 farm in late June 2001, during winter. Six heifers and 1 cow out of 45 cattle were affected during a 3-week period. The affected animals died spontaneously or were euthanized approximately 2-14 days after the onset of clinical signs. The clinical signs were characterized by flaccid paralysis and gait abnormalities. Clinical signs were more pronounced after exercise and included stiff and unsteady gait, knuckling at the fetlocks of the hind limbs, frequent falling, inability to rise, muscular tremors, especially of the head and the hindquarters, and drooling. Main necropsy findings included degenerative and necrotic changes of the larger medial muscle groups of the hindquarters, i.e., adductor, pectineus, quadriceps femoris, rectus femuris, sartorius, semimembranosus, semitendinosus, and vastus medialis, and of the forequarters, including pectoralis descendens, pectoralis ascendens, and transversus pectoralis. The main histologic findings consisted of degenerative and necrotic neuronal changes (chromatolysis) of varying severity and extent affecting selected nuclei of the brainstem and neurons of the ventral horns of the spinal cord. Similar microscopic lesions were observed in the neurons of the spinal cord of 1 experimental sheep force-fed for 35 days with 1 kg/day of the same batch of foodstuff that was originally fed to the cattle. Coarse white or gray lumps, interpreted as mycelia, were observed in the beer by-product. Aspergillus clavatus was the dominant fungus isolated. Deaths ceased after the consumption of beer residue was discontinued. Recovery from illness was observed in 1 animal. The diagnosis was based on epidemiological data, clinical signs, necropsy findings, histological lesions, dosing trial, and mycology. A similar condition caused by consumption of barley by-products, sprouted wheat, corn sprouts, and beetroot

  2. The body electric: a long view of electrical therapy for functional neurological disorders.

    PubMed

    McWhirter, Laura; Carson, Alan; Stone, Jon

    2015-04-01

    The use of electricity in medical treatment has always been technology-driven, rather than aetiology-driven; as new techniques have appeared, clinicians have quickly looked to try them in the treatment of all sorts of conditions where existing treatment options are limited. Functional disorders--as identified anachronistically in our analysis--have been key contenders for emerging electrical treatments: with Leyden jars, with galvanic and electromagnetic machines, and more recently with TMS and TENS. Parallels can be drawn with the history of electrical treatments for migraine and headache (Koehler and Boes, 2010). Regardless of the mode of delivery of electricity, stimulating a limb to produce movement has repeatedly been found to aid and assist recovery in functional motor disorders. This may also be true of non-electrical methods: we have found benefits using both therapeutic sedation and explanatory demonstration of a positive Hoover's sign as therapeutic methods of demonstrating normal movement in functionally weak limbs (Stone et al., 2014). Each surge in enthusiasm for new electrical treatments has been followed by questions about the nature of the disorder and validity of the treatment response. Physicians have tended to attribute therapeutic success initially to powerful biological or even metaphysical effects, but with time and experience these explanations have been replaced by views that the treatment works through suggestion and placebo. Discomfort with these conclusions has in the past discouraged ongoing development of electrical treatments, even if the end result for patients has been encouraging. In Edwards's Bayesian model, functional motor and sensory symptoms are hypothesized to arise when 'pathologically precise prior beliefs' mediated by attentional processes cause experience of symptoms via a hierarchy of false inferences (Edwards, 2012). It can be argued that use of TMS or peripheral stimulation to produce movement of a functionally weak

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

    PubMed

    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

  4. XY sex reversal and a nonprogressive neurologic disorder: a new syndrome?

    PubMed

    Mahbubul Huq, A H; Nigro, M A

    2000-10-01

    We report a patient with a unique combination of clinical findings: XY sex reversal, spastic paraplegia, mental retardation, dysmorphism, and infantile-onset olivopontocerebellar hypoplasia. The phenotype of our patient did not coincide with any of the described forms of XY reversal syndromes, hereditary or sporadic spastic paraplegias, or congenital or infantile-onset cerebellar or olivopontocerebellar atrophies or hypoplasias. The disorder of this patient likely represents a genetic condition with pleiotropic effects on brain development and sex determination and adds further evidence for the heterogeneity of spastic paraplegia/infantile olivopontocerebellar hypoplasia syndromes and sex reversal syndromes. PMID:11068172

  5. William Shakespeare's neurology.

    PubMed

    Paciaroni, Maurizio; Bogousslavsky, Julien

    2013-01-01

    Many of Shakespeare's plays contain characters who appear to be afflicted by neurological or psychiatric disorders. Shakespeare, in his descriptive analysis of his protagonists, was contributing to the understanding of these disorders. In fact, Charcot frequently used Shakespearean references in his neurological teaching sessions, stressing how acute objective insight is essential to achieving expert clinical diagnosis. Charcot found in Shakespeare the same rigorous observational techniques for which he himself became famous. This chapter describes many of Shakespearean characters suffering from varied neurological disorders, including Parkinsonism, epilepsy, sleeping disturbances, dementia, headache, prion disease, and paralyses. PMID:24290473

  6. Reliability and Validity of the Assessment of Neurological Soft-Signs in Children with and without Attention-Deficit-Hyperactivity Disorder

    ERIC Educational Resources Information Center

    Gustafsson, Peik; Svedin, Carl Goran; Ericsson, Ingegerd; Linden, Christian; Karlsson, Magnus K.; Thernlund, Gunilla

    2010-01-01

    Aim: To study the value and reliability of an examination of neurological soft-signs, often used in Sweden, in the assessment of children with attention-deficit-hyperactivity disorder (ADHD), by examining children with and without ADHD, as diagnosed by an experienced clinician using the DSM-III-R. Method: We have examined interrater reliability…

  7. The Collaborative Study on Cerebral Palsy, Mental Retardation, and Other Neurological and Sensory Disorders of Infancy and Childhood. Bibliography No. 8. July 1974 through June 1975.

    ERIC Educational Resources Information Center

    National Inst. of Neurological and Communicative Disorders and Stroke (NIH), Bethesda, MD.

    The eighth in a series of annual bibliographies of the Collaborative Perinatal Project lists 30 manuscripts and journal articles from studies on cerebral palsy, mental retardation, and other neurological and sensory disorders of infancy and childhood. Entries are grouped under the categories of core and non-core data publications (based on…

  8. Regulation of Neuroinflammation through Programed Death-1/Programed Death Ligand Signaling in Neurological Disorders

    PubMed Central

    Zhao, Shangfeng; Li, Fengwu; Leak, Rehana K.; Chen, Jun; Hu, Xiaoming

    2014-01-01

    Immune responses in the central nervous system (CNS), which involve both resident glial cells and infiltrating peripheral immune cells, play critical roles in the progress of brain injuries and neurodegeneration. To avoid inflammatory damage to the compromised brain, the immune cell activities in the CNS are controlled by a plethora of chemical mediators and signal transduction cascades, such as inhibitory signaling through programed death-1 (PD-1) and programed death ligand (PD-L) interactions. An increasing number of recent studies have highlighted the importance of PD-1/PD-L pathway in immune regulation in CNS disorders such as ischemic stroke, multiple sclerosis, and Alzheimer’s disease. Here, we review the current knowledge of the impact of PD-1/PD-L signaling on brain injury and neurodegeneration. An improved understanding of the function of PD-1/PD-L in the cross-talk between peripheral immune cells, CNS glial cells, and non-immune CNS cells is expected to shed further light on immunomodulation and help develop effective and safe immunotherapies for CNS disorders. PMID:25232304

  9. Insights into the Pathology of the α2-Na(+)/K(+)-ATPase in Neurological Disorders; Lessons from Animal Models.

    PubMed

    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

  10. Insights into the Pathology of the α2-Na+/K+-ATPase in Neurological Disorders; Lessons from Animal Models

    PubMed Central

    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 to

  11. Congenital disorder of glycosylation type 1a: three siblings with a mild neurological phenotype.

    PubMed

    Coman, D; McGill, J; MacDonald, R; Morris, D; Klingberg, S; Jaeken, J; Appleton, D

    2007-07-01

    We report 3 siblings (1 male and 2 female) recently diagnosed with congenital disorder of glycosylation type Ia (CDG-Ia) in their mid-20s. They experience mild mental retardation but manage to function independently in society. Their professions are library assistant, professional artistic painter and secretarial work. All three siblings have cerebellar hypoplasia and ataxia, but are able to ambulate easily. Two of the siblings have required strabismus surgical repairs. All have antithrombin III deficiency, osteoporosis, and mild dysmorphic features. Hypergonadotrophic hypogonadism was a feature of the two female siblings. A type 1 sialotransferrin pattern and phosphomannomutase (PMM) deficiency have been demonstrated. They are compound heterozygotes for R141H and L32R mutations in the PMM2 gene. While there is clinical heterogeneity in CDG-Ia, we believe that our patients are among the mildest of intellectually affected CDG-Ia patients reported to date. PMID:17451957

  12. Moieties in antidiabetic drugs as a target of insulin receptors in association with common neurological disorders

    PubMed Central

    GUZMÁN, DAVID CALDERÓN; OLGUÍN, HUGO JUÁREZ; GARCÍA, ERNESTINA HERNÁNDEZ; HERRERA, MARIBEL ORTIZ; BRIZUELA, NORMA OSNAYA

    2016-01-01

    Insulin is a peptide that can be harmful with regards to neuroplasticity, neuroprotection and neuromodulation. Furthermore, the role of insulin highlights its relevance in the progress of diverse clinical disorders as well as in the mechanisms associated with certain pathogenesis and their evolution towards diabetes, obesity and neurodegenerative diseases. The precise molecular mechanisms by which these diseases are induced remain to be elucidated. The benefits in knowing/discovering these mechanisms in animal models and humans cannot be undermined. An in depth understanding of the principal risk factors leading to obesity and their management is vital in the implementation of early-life strategies of intervention and prevention, with a view to avoid adverse late-life outcomes. Therefore, the aim of the present study was to review their possible association with antidiabetic drugs. PMID:27073619

  13. Plasticity and modular control of locomotor patterns in neurological disorders with motor deficits

    PubMed Central

    Ivanenko, Y. P.; Cappellini, G.; Solopova, I. A.; Grishin, A. A.; MacLellan, M. J.; Poppele, R. E.; Lacquaniti, F.

    2013-01-01

    Human locomotor movements exhibit considerable variability and are highly complex in terms of both neural activation and biomechanical output. The building blocks with which the central nervous system constructs these motor patterns can be preserved in patients with various sensory-motor disorders. In particular, several studies highlighted a modular burst-like organization of the muscle activity. Here we review and discuss this issue with a particular emphasis on the various examples of adaptation of locomotor patterns in patients (with large fiber neuropathy, amputees, stroke and spinal cord injury). The results highlight plasticity and different solutions to reorganize muscle patterns in both peripheral and central nervous system lesions. The findings are discussed in a general context of compensatory gait mechanisms, spatiotemporal architecture and modularity of the locomotor program. PMID:24032016

  14. KTX 0101: a potential metabolic approach to cytoprotection in major surgery and neurological disorders.

    PubMed

    Smith, Sharon L; Heal, David J; Martin, Keith F

    2005-01-01

    KTX 0101 is the sodium salt of the physiological ketone, D-beta-hydroxybutyrate (betaOHB). This neuroprotectant, which has recently successfully completed clinical Phase IA evaluation, is being developed as an intravenous infusion fluid to prevent the cognitive deficits caused by ischemic foci in the brain during cardiopulmonary bypass (CPB) surgery. KTX 0101 maintains cellular viability under conditions of physiological stress by acting as a "superfuel" for efficient ATP production in the brain and peripheral tissues. Unlike glucose, this ketone does not require phosphorylation before entering the TCA cycle, thereby sparing vital ATP stores. Although no reliable models of CPB-induced ischemia exist, KTX 0101 is powerfully cytoprotectant under the more severe ischemic conditions of global and focal cerebral ischemia, cardiac ischemia and lung hemorrhage. Neuroprotection has been demonstrated by reductions in infarct volume, edema, markers of apoptosis and functional impairment. One significant difference between KTX 0101 and other potential neuroprotectants in development is that betaOHB is a component of human metabolic physiology which exploits the body's own neuroprotective mechanisms. KTX 0101 also protects hippocampal organotypic cultures against early and delayed cell death in an in vitro model of status epilepticus, indicating that acute KTX 0101 intervention in this condition could help prevent the development of epileptiform foci, a key mechanism in the etiology of intractable epilepsy. In models of chronic neurodegenerative disorders, KTX 0101 protects neurons against damage caused by dopaminergic neurotoxins and by the fragment of beta-amyloid, Abeta(1-42), implying possible therapeutic applications for ketogenic strategies in treating Parkinson's and Alzheimer's diseases. Major obstacles to the use of KTX 0101 for long term therapy in chronic disorders, e.g., Parkinson's and Alzheimer's diseases, are the sodium loading problem and the need to administer

  15. Defects of mutant DNMT1 are linked to a spectrum of neurological disorders

    PubMed Central

    Baets, Jonathan; Duan, Xiaohui; Wu, Yanhong; Smith, Gordon; Seeley, William W.; Mademan, Inès; McGrath, Nicole M.; Beadell, Noah C.; Khoury, Julie; Botuyan, Maria-Victoria; Mer, Georges; Worrell, Gregory A.; Hojo, Kaori; DeLeon, Jessica; Laura, Matilde; Liu, Yo-Tsen; Senderek, Jan; Weis, Joachim; Van den Bergh, Peter; Merrill, Shana L.; Reilly, Mary M.; Houlden, Henry; Grossman, Murray; Scherer, Steven S.; De Jonghe, Peter; Dyck, Peter J.

    2015-01-01

    We report a broader than previously appreciated clinical spectrum for hereditary sensory and autonomic neuropathy type 1E (HSAN1E) and a potential pathogenic mechanism for DNA methyltransferase (DNMT1) mutations. The clinical presentations and genetic characteristics of nine newly identified HSAN1E kinships (45 affected subjects) were investigated. Five novel mutations of DNMT1 were discovered; p.C353F, p.T481P, p.P491L, p.Y524D and p.I531N, all within the target-sequence domain, and two mutations (p.T481P, p.P491L) arising de novo. Recently, HSAN1E has been suggested as an allelic disorder of autosomal dominant cerebellar ataxia, deafness and narcolepsy. Our results indicate that all the mutations causal for HSAN1E are located in the middle part or N-terminus end of the TS domain, whereas all the mutations causal for autosomal dominant cerebellar ataxia, deafness and narcolepsy are located in the C-terminus end of the TS domain. The impact of the seven causal mutations in this cohort was studied by cellular localization experiments. The binding efficiency of the mutant DNMT proteins at the replication foci and heterochromatin were evaluated. Phenotypic characterizations included electromyography, brain magnetic resonance and nuclear imaging, electroencephalography, sural nerve biopsies, sleep evaluation and neuropsychometric testing. The average survival of HSAN1E was 53.6 years. [standard deviation = 7.7, range 43–75 years], and mean onset age was 37.7 years. (standard deviation = 8.6, range 18–51 years). Expanded phenotypes include myoclonic seizures, auditory or visual hallucinations, and renal failure. Hypersomnia, rapid eye movement sleep disorder and/or narcolepsy were identified in 11 subjects. Global brain atrophy was found in 12 of 14 who had brain MRI. EEGs showed low frequency (delta waves) frontal-predominant abnormality in five of six patients. Marked variability in cognitive deficits was observed, but the majority of patients (89%) developed

  16. Defects of mutant DNMT1 are linked to a spectrum of neurological disorders.

    PubMed

    Baets, Jonathan; Duan, Xiaohui; Wu, Yanhong; Smith, Gordon; Seeley, William W; Mademan, Inès; McGrath, Nicole M; Beadell, Noah C; Khoury, Julie; Botuyan, Maria-Victoria; Mer, Georges; Worrell, Gregory A; Hojo, Kaori; DeLeon, Jessica; Laura, Matilde; Liu, Yo-Tsen; Senderek, Jan; Weis, Joachim; Van den Bergh, Peter; Merrill, Shana L; Reilly, Mary M; Houlden, Henry; Grossman, Murray; Scherer, Steven S; De Jonghe, Peter; Dyck, Peter J; Klein, Christopher J

    2015-04-01

    We report a broader than previously appreciated clinical spectrum for hereditary sensory and autonomic neuropathy type 1E (HSAN1E) and a potential pathogenic mechanism for DNA methyltransferase (DNMT1) mutations. The clinical presentations and genetic characteristics of nine newly identified HSAN1E kinships (45 affected subjects) were investigated. Five novel mutations of DNMT1 were discovered; p.C353F, p.T481P, p.P491L, p.Y524D and p.I531N, all within the target-sequence domain, and two mutations (p.T481P, p.P491L) arising de novo. Recently, HSAN1E has been suggested as an allelic disorder of autosomal dominant cerebellar ataxia, deafness and narcolepsy. Our results indicate that all the mutations causal for HSAN1E are located in the middle part or N-terminus end of the TS domain, whereas all the mutations causal for autosomal dominant cerebellar ataxia, deafness and narcolepsy are located in the C-terminus end of the TS domain. The impact of the seven causal mutations in this cohort was studied by cellular localization experiments. The binding efficiency of the mutant DNMT proteins at the replication foci and heterochromatin were evaluated. Phenotypic characterizations included electromyography, brain magnetic resonance and nuclear imaging, electroencephalography, sural nerve biopsies, sleep evaluation and neuropsychometric testing. The average survival of HSAN1E was 53.6 years. [standard deviation = 7.7, range 43-75 years], and mean onset age was 37.7 years. (standard deviation = 8.6, range 18-51 years). Expanded phenotypes include myoclonic seizures, auditory or visual hallucinations, and renal failure. Hypersomnia, rapid eye movement sleep disorder and/or narcolepsy were identified in 11 subjects. Global brain atrophy was found in 12 of 14 who had brain MRI. EEGs showed low frequency (delta waves) frontal-predominant abnormality in five of six patients. Marked variability in cognitive deficits was observed, but the majority of patients (89%) developed

  17. Defects of mutant DNMT1 are linked to a spectrum of neurological disorders

    PubMed Central

    Baets, Jonathan; Duan, Xiaohui; Wu, Yanhong; Smith, Gordon; Seeley, William W.; Mademan, Inès; McGrath, Nicole M.; Beadell, Noah C.; Khoury, Julie; Botuyan, Maria-Victoria; Mer, Georges; Worrell, Gregory A.; Hojo, Kaori; DeLeon, Jessica; Laura, Matilde; Liu, Yo-Tsen; Senderek, Jan; Weis, Joachim; Van den Bergh, Peter; Merrill, Shana L.; Reilly, Mary M.; Houlden, Henry; Grossman, Murray; Scherer, Steven S.; De Jonghe, Peter; Dyck, Peter J.

    2015-01-01

    We report a broader than previously appreciated clinical spectrum for hereditary sensory and autonomic neuropathy type 1E (HSAN1E) and a potential pathogenic mechanism for DNA methyltransferase (DNMT1) mutations. The clinical presentations and genetic characteristics of nine newly identified HSAN1E kinships (45 affected subjects) were investigated. Five novel mutations of DNMT1 were discovered; p.C353F, p.T481P, p.P491L, p.Y524D and p.I531N, all within the target-sequence domain, and two mutations (p.T481P, p.P491L) arising de novo. Recently, HSAN1E has been suggested as an allelic disorder of autosomal dominant cerebellar ataxia, deafness and narcolepsy. Our results indicate that all the mutations causal for HSAN1E are located in the middle part or N-terminus end of the TS domain, whereas all the mutations causal for autosomal dominant cerebellar ataxia, deafness and narcolepsy are located in the C-terminus end of the TS domain. The impact of the seven causal mutations in this cohort was studied by cellular localization experiments. The binding efficiency of the mutant DNMT proteins at the replication foci and heterochromatin were evaluated. Phenotypic characterizations included electromyography, brain magnetic resonance and nuclear imaging, electroencephalography, sural nerve biopsies, sleep evaluation and neuropsychometric testing. The average survival of HSAN1E was 53.6 years. [standard deviation = 7.7, range 43–75 years], and mean onset age was 37.7 years. (standard deviation = 8.6, range 18–51 years). Expanded phenotypes include myoclonic seizures, auditory or visual hallucinations, and renal failure. Hypersomnia, rapid eye movement sleep disorder and/or narcolepsy were identified in 11 subjects. Global brain atrophy was found in 12 of 14 who had brain MRI. EEGs showed low frequency (delta waves) frontal-predominant abnormality in five of six patients. Marked variability in cognitive deficits was observed, but the majority of patients (89%) developed

  18. The role of nanotechnology and nano and micro-electronics in monitoring and control of cardiovascular diseases and neurological disorders

    NASA Astrophysics Data System (ADS)

    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

  19. Low-dose fenfluramine in the treatment of neurologic disorders: experience in Dravet syndrome

    PubMed Central

    Schoonjans, An-Sofie; Lagae, Lieven; Ceulemans, Berten

    2015-01-01

    In this paper, we review the experience with fenfluramine in epileptic and other paroxysmal disorders. Since the best available data are from the treatment of Dravet syndrome, we will focus primarily on this condition. Originally fenfluramine was launched as an anorectic agent. As early as 1985, seizure reduction in children could be demonstrated in a few cases with photosensitive, self-induced epilepsy. Hereafter, a small study was launched in patients with self-induced epilepsy. Results showed a significant seizure reduction, and review of the patient data showed that 5 of the 12 patients had Dravet syndrome. During that observation period, fenfluramine was withdrawn from the market because of cardiovascular side effects associated with prescribing higher doses in combination with phentermine for weight loss. In March 2002, a Belgian Royal Decree was issued permitting further study of fenfluramine in pediatric patients with intractable epilepsy. In 2011 under the Royal Decree, a prospective study of patients with Dravet syndrome treated with low-dose fenfluramine was initiated and is currently ongoing. The initial results are promising in terms of reduction of seizure frequency and overall tolerability. PMID:26600876

  20. Systems Biology in the study of Neurological Disorders: Focus on Alzheimer’s Disease

    PubMed Central

    Pasinetti, Giulio M.; Hiller-Sturmhöfel, Susanne

    2008-01-01

    Systems biology approaches may be useful for studying the mechanisms underlying alcohol’s harmful effects on the brain. Such approaches already are used in the study of Alzheimer’s disease (AD), a progressive neurodegenerative disorder that, with the overall increase in life expectancy, will affect an increasing proportion of the population and become an increasingly serious public health concern. Systems biology approaches such as complementary DNA (cDNA) microarray analyses have helped identify several genes whose expression is altered in patients exhibiting the earliest stages of AD. Several of these genes are involved in the release of messenger molecules from the ends of nerve cells (i.e., in synaptic vesicle functioning), and their particular role in AD must be investigated further using conventional molecular biological approaches. Similarly, protein array analyses have identified candidate proteins that may play a role in the development of AD. Finally, proteomic approaches, such as certain mass spectrometry techniques, have been used to search for biomarkers of the progression from normal cognitive functioning to mild cognitive impairment and AD, which eventually may allow early and reliable diagnosis of the disease. These approaches already have yielded some candidate molecules whose validity and reliability as biomarkers of AD, however, still need to be confirmed. PMID:23584752

  1. [Some neurologic and psychiatric complications in endocrine disorders: the thyroid gland].

    PubMed

    Aszalós, Zsuzsa

    2007-02-18

    Thyroid hormones are of primary importance for the perinatal development of the central nervous system, and for normal function of the adult brain. These hormones primarily regulate the transcription of specific target genes. They increase the cortical serotonergic neurotransmission, and play an important role in regulating central noradrenergic and GABA function. Thyroid deficiency during the perinatal period results in mental retardation. Hypothyroidism of the adults causes most frequently dementia and depression. Other less common clinical pictures include myxoedema coma, dysfunction of cerebellum and cranial nerves. Hypothyroidism also increases predisposition of stroke. Peripheral diseases frequently include polyneuropathy, carpal tunnel syndrome, myalgic state, and rarely myokymia. Nearly all the hyperthyroid patients show minor psychiatric signs, and infrequently psychosis, dementia, confusion state, depression, apathetic thyrotoxicosis, thyrotoxic crisis, seizures, pyramidal signs, or chorea occur. The peripheral complications may be indicated by chronic thyrotoxic myopathy, infiltrative ophthalmopathy, myasthenia gravis, periodic hypokalemic paralysis and polyneuropathy. Generalized resistance to thyroid hormone was confirmed in a number of patients with attention deficit-hyperactivity disorder. Significantly elevated antithyroid antibody titers characterize Hashimoto's encephalopathy. This condition is a rare, acute - subacute, serious, life threatening, but steroid-responsive, relapsing-remitting, autoimmune disease. PMID:17344150

  2. Low-dose fenfluramine in the treatment of neurologic disorders: experience in Dravet syndrome.

    PubMed

    Schoonjans, An-Sofie; Lagae, Lieven; Ceulemans, Berten

    2015-11-01

    In this paper, we review the experience with fenfluramine in epileptic and other paroxysmal disorders. Since the best available data are from the treatment of Dravet syndrome, we will focus primarily on this condition. Originally fenfluramine was launched as an anorectic agent. As early as 1985, seizure reduction in children could be demonstrated in a few cases with photosensitive, self-induced epilepsy. Hereafter, a small study was launched in patients with self-induced epilepsy. Results showed a significant seizure reduction, and review of the patient data showed that 5 of the 12 patients had Dravet syndrome. During that observation period, fenfluramine was withdrawn from the market because of cardiovascular side effects associated with prescribing higher doses in combination with phentermine for weight loss. In March 2002, a Belgian Royal Decree was issued permitting further study of fenfluramine in pediatric patients with intractable epilepsy. In 2011 under the Royal Decree, a prospective study of patients with Dravet syndrome treated with low-dose fenfluramine was initiated and is currently ongoing. The initial results are promising in terms of reduction of seizure frequency and overall tolerability. PMID:26600876

  3. Ischemic perinatal stroke: summary of a workshop sponsored by the National Institute of Child Health and Human Development and the National Institute of Neurological Disorders and Stroke.

    PubMed

    Raju, Tonse N K; Nelson, Karin B; Ferriero, Donna; Lynch, John Kylan

    2007-09-01

    Ischemic perinatal stroke is a disorder associated with significant long-term neurologic morbidity. With an estimated incidence of 1 in 2300 to 5000 births, stroke is more likely to occur in the perinatal period than at any time in childhood. The incidence of ischemic perinatal stroke ranks second only to that of strokes in the elderly population. Although ischemic perinatal stroke is a well-recognized disorder, many aspects remain to be studied. There is no consensus on its terminology, definition, or classification. Several risk factors have been identified, but their precise roles in causing stroke are not well understood. There are no reliable predictors of ischemic perinatal stroke on which to base prevention or treatment strategies. To review these important issues and propose a research agenda, the National Institute of Child Health and Human Development and the National Institute of Neurological Disorders and Stroke convened a workshop in August 2006. This article provides a summary of the workshop. PMID:17766535

  4. Mannose-binding Lectin Mediated Complement Pathway in Autoimmune Neurological Disorders.

    PubMed

    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. PMID:27424141

  5. Role of PRRT2 in common paroxysmal neurological disorders: a gene with remarkable pleiotropy.

    PubMed

    Heron, Sarah E; Dibbens, Leanne M

    2013-03-01

    Mutations in the gene PRRT2 encoding proline-rich transmembrane protein 2 have recently been identified as the cause of three clinical entities: benign familial infantile epilepsy (BFIE), infantile convulsions with choreoathetosis (ICCA) syndrome, and paroxysmal kinesigenic dyskinesia (PKD). Patients with ICCA have both BFIE and PKD and families with ICCA may contain individuals who exhibit all three phenotypes. These three phenotypes were all mapped by linkage analyses to the pericentromeric region of chromosome 16, and were hypothesised to have the same genetic basis due to the co-occurrence of the disorders in some families. Despite considerable effort, the gene or genes for BFIE, ICCA, and PKD were not identified for many years after the linkage region was identified. Mutations in the gene PRRT2 were identified in several Chinese families with PKD, suggesting that the gene may also be responsible for ICCA and BFIE in families linked to the chromosome 16 locus. This was demonstrated to be the case, with the majority of families with ICCA and BFIE found to have PRRT2 mutations. The vast majority of these mutations are truncating and are predicted to lead to haploinsufficiency. PRRT2 is a largely uncharacterised protein. It is expressed in the brain and has been demonstrated to interact with SNAP-25, a component of the molecular machinery involved in the release of neurotransmitters at the presynaptic membrane. Therefore, the PRRT2 protein may play a role in this process. However, the molecular mechanisms underlying the remarkable pleiotropy associated with PRRT2 mutations have still to be determined. PMID:23343561

  6. Prospects for Engineering Neurons from Local Neocortical Cell Populations as Cell-Mediated Therapy for Neurological Disorders

    PubMed Central

    Bazarek, Stanley; Peterson, Daniel A.

    2014-01-01

    There is little cell replacement following neurological injury, limiting the regenerative response of the CNS. Progress in understanding the biology of neural stem cells has raised interest in using stem cells for replacing neurons lost to injury or to disease. Stem cell therapy may also have a role in rebuilding deficient neural circuitry underlying mood disorders, epilepsy, and pain modulation among others. In vitro expansion of stem cells with directed differentiation prior to transplantation is one approach to stem cell therapy. Emerging evidence suggests that it may be possible to directly convert in vivo endogenous neural cells to a neuronal fate, providing an alternative strategy for stem cell therapy to the CNS. This review assesses the evidence for engineering subtype-specific neuronal fate of endogenous neural cells in the cerebral cortex as a function of initial cell lineage, reactive response to injury, conversion factors, and environmental context. We conclude with a discussion of some of the challenges that need to be overcome to move this alternative in vivo engineered conversion process towards becoming a viable therapeutic option. PMID:24756774

  7. Fornix as an imaging marker for episodic memory deficits in healthy aging and in various neurological disorders

    PubMed Central

    Douet, Vanessa; Chang, Linda

    2015-01-01

    The fornix is a part of the limbic system and constitutes the major efferent and afferent white matter tracts from the hippocampi. The underdevelopment of or injuries to the fornix are strongly associated with memory deficits. Its role in memory impairments was suggested long ago with cases of surgical forniceal transections. However, recent advances in brain imaging techniques, such as diffusion tensor imaging, have revealed that macrostructural and microstructural abnormalities of the fornix correlated highly with declarative and episodic memory performance. This structure appears to provide a robust and early imaging predictor for memory deficits not only in neurodegenerative and neuroinflammatory diseases, such as Alzheimer's disease and multiple sclerosis, but also in schizophrenia and psychiatric disorders, and during neurodevelopment and “typical” aging. The objective of the manuscript is to present a systematic review regarding published brain imaging research on the fornix, including the development of its tracts, its role in various neurological diseases, and its relationship to neurocognitive performance in human studies. PMID:25642186

  8. Functional neuroimaging of human central auditory processing in normal subjects and patients with neurological and neuropsychiatric disorders.

    PubMed

    Engelien, A; Stern, E; Silbersweig, D

    2001-02-01

    Auditory sensory processing in the human cerebral cortex is disturbed in several neurological and neuropsychiatric disorders, ranging from devastating perceptual deficits in neuropsychological syndromes such as cortical deafness and auditory agnosia to the problem of involuntary hallucinatory perception in schizophrenia. With modern non-invasive functional imaging techniques (e.g., PET, fMRI, and MEG), the normal auditory cortical functional anatomy can now be studied in humans in vivo, as well as its disruption in pathological conditions. This article will summarize current knowledge on human central auditory perception in health and disease, with an emphasis on recent functional neuroimaging studies, in the context of clinical and basic neuroscientific knowledge. New strategies include a focus on the role of other, non-temporal brain areas for auditory processing, particularly in the frontal lobes, and the combined use of techniques offering both precise spatial and temporal resolution. One step towards this goal has been the recent development of a silent, event-related fMRI scanning technique. PMID:11320447

  9. Common Data Elements for Spinal Cord Injury Clinical Research: A National Institute for Neurological Disorders and Stroke Project

    PubMed Central

    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

  10. Effects of Bee Venom Injections at Acupoints on Neurologic Dysfunction Induced by Thoracolumbar Intervertebral Disc Disorders in Canines: A Randomized, Controlled Prospective Study

    PubMed Central

    Tsai, Li-Chuan; Lin, Yi-Wen; Hsieh, Ching-Liang

    2015-01-01

    Intervertebral disk disease (IVDD) is a major spine disorder in canines that causes neurological dysfunction, particularly in the thoracolumbar area. Analgesic and anti-inflammatory drugs are typically used to reduce nociceptive signals to decrease canine suffering. Bee venom (BV) has been reported to exert anti-inflammatory and analgesic effects. Injection of BV at acupoints has been widely used to treat clinical disorders including inflammation, pain, and arthritis. The current study was intended to determine whether BV injections at acupoints can enhance treatment of canine neurological dysfunction caused by IVDD. A single-blind controlled trial involving 40 adult canines with neurological dysfunction induced by IVDD subdivided into 2 groups was designed, and 36 canines finished the study. The myelopathy scoring system (MSS) grade and functional numeric scale (FNS) scores improved further after BV treatment than after control treatment. BV injection exerted a particularly strong effect on canines with moderate to severe IVDD and dramatically reduced clinical rehabilitation time. The results indicate that BV injections at acupoints are more effective at protecting canines from IVDD-induced neurological dysfunction and pain than is treatment alone. PMID:26693480

  11. Linking the Activity Measure for Post-acute Care and the Quality of Life Outcomes in Neurological Disorders

    PubMed Central

    Ni, Pengsheng; Lai, Jin-shei; Tian, Feng; Coster, Wendy J.; Jette, Alan M.; Straub, Donald; Cella, David

    2012-01-01

    Objective To use item response theory (IRT) methods to link physical functioning items in the Activity Measure for Post-acute Care (AM-PAC) and the Quality of Life Outcomes in Neurological Disorders (Neuro-QOL) Design Secondary data analysis of the physical functioning items of AM-PAC and Neuro-QOL. We used a non-equivalent group design with 36 core items common to both instruments. We used a test characteristic curve transformation method to for linking AM-PAC and Neuro-QOL scores. Linking was conducted so that both raw scores and scaled AM-PAC and Neuro-QOL scores (converted-logit scores with mean = 50 and SD = 10) could be compared. Setting AM-PAC items were administered to rehabilitation patients in post-acute care settings. Neuro-QOL items were administered to a community sample of adults via the Internet. Participants The AM-PAC sample consisted of 1,041 post acute care patients; the Neuro-QOL sample was 549 community-dwelling adults. Interventions Not applicable. Main Outcome Measures 25 Mobility items and 11 ADL items common to both instruments were included in the analysis. Results Neuro-QOL items were linked to the AM-PAC scale using the Generalized Partial Credit Model. Mobility and ADL subscale scores from the two instruments were calibrated to the AM-PAC metric. Conclusions An IRT-based linking method placed AM-PAC and NeuroQOL Mobility and ADL scores on a common metric. This linking allowed estimation of AM-PAC Mobility and ADL subscale scores based on Neuro-QOL Mobility and ADL subscale scores, and vice versa. The accuracy of these results should be validated in a future sample in which participants respond to both instruments. PMID:21958921

  12. Molecular cloning of human T-cell lymphotrophic virus type I-like proviral genome from the peripheral lymphocyte DNA of a patient with chronic neurologic disorders

    SciTech Connect

    Reddy, E.P.; Mettus, R.V.; DeFreitas, E.; Wroblewska, Z.; Cisco, M.; Koprowski, H. )

    1988-05-01

    Human T-cell lymphotropic virus type 1 (HTLV-I), the etiologic agent of human T-cell leukemia, has recently been shown to be associated with neurologic disorders such as tropical spastic paraparesis, HTLV-associated myelopathy, and possibly with multiple sclerosis. In this communication, the authors have examined one specific case of neurologic disorder that can be classified as multiple sclerosis or tropical spastic paraparesis. The patient suffering from chronic neurologic disorder was found to contain antibodies to HTLV-I envelope and gag proteins in his serum and cerebrospinal fluid. Lymphocytes from peripheral blood and cerebrospinal fluid of the patient were shown to express viral RNA sequences by in situ hybridization. Southern blot analysis of the patient lymphocyte DNA revealed the presence of HTLV-I-related sequences. Blot-hybridization analysis of the RNA from fresh peripheral lymphocytes stimulated with interleukin 2 revealed the presence of abundant amounts of genomic viral RNA with little or no subgenomic RNA. They have clones the proviral genome from the DNA of the peripheral lymphocytes and determined its restriction map. This analysis shows that this proviral genome is very similar if not identical to that of the prototype HTLV-I genome.

  13. [Palliative care in neurology].

    PubMed

    Provinciali, Leandro; Tarquini, Daniela; De Falco, Fabrizio A; Carlini, Giulia; Zappia, Mario; Toni, Danilo

    2015-07-01

    Palliative care in neurology is characterized by the need of taking into account some distinguishing features which supplement and often differ from the general palliative approach to cancer or to severe organ failures. Such position is emphasized by a new concept of palliative assistance which is not limited to the "end of life" stage, as it was the traditional one, but is applied along the entire course of progressive, life-limiting, and disabling conditions. There are various reasons accounting for a differentiation of palliative care in neurology and for the development of specific expertise; the long duration of the advanced stages of many neurological diseases and the distinguishing features of some clinical problems (cognitive disorders, psychic disorders, etc.), in addition to the deterioration of some general aspects (nutrition, etc.), make the general criteria adopted for cancer, severe respiratory, hepatic or renal failures and heart failure inadequate. The neurological diseases which could benefit from the development of a specific palliative approach are dementia, cerebrovascular diseases, movement disorders, neuromuscular diseases, severe traumatic brain injury, brain cancers and multiple sclerosis, as well as less frequent conditions. The growing literature on palliative care in neurology provides evidence of the neurological community's increasing interest in taking care of the advanced and terminal stages of nervous system diseases, thus encouraging research, training and updating in such direction. This document aims to underline the specific neurological requirements concerning the palliative assistance. PMID:26228722

  14. Neurologic effects of alcoholism.

    PubMed Central

    Diamond, I; Messing, R O

    1994-01-01

    Alcoholism, a worldwide disorder, is the cause of a variety of neurologic disorders. In this article we discuss the cellular pathophysiology of ethanol addition and abuse as well as evidence supporting and refuting the role of inheritance in alcoholism. A genetic marker for alcoholism has not been identified, but neurophysiologic studies may be promising. Some neurologic disorders related to longterm alcoholism are due predominantly to inadequate nutrition (the thiamine deficiency that causes Wernicke's encephalopathy), but others appear to involve the neurotoxicity of ethanol on brain (alcohol withdrawal syndrome and dementia) and peripheral nerves (alcoholic neuropathy and myopathy). Images PMID:7975567

  15. Chapter 30: historical aspects of the major neurological vitamin deficiency disorders: the water-soluble B vitamins.

    PubMed

    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

  16. Neuropsychological, electrophysiological and neurological impairments in patients with obsessive compulsive disorder, their healthy siblings and healthy controls: Identifying potential endophenotype(s).

    PubMed

    Ozcan, Halil; Ozer, Suzan; Yagcioglu, Suha

    2016-06-30

    The etiology of obsessive-compulsive disorder (OCD) has not been clarified. This study aimed to investigate the cognitive, neurological, electrophysiological functions which are reflected in executive functions, memory, visuospatial integration; neurological examination and auditory event related potentials (AERP) (N100, N200, P200 and P300) in patients with OCD, their siblings, and control subjects and to determine potential endophenotypic markers. Thirty-three patients with OCD, 18 siblings and 21 controls; matched for age, gender and years of education were included. Yale Brown Obsessive Compulsive Symptoms Checklist Scale, Hamilton Depression-Rating Scale, an exhaustive neuropscyhological test battery and Neurological Evaluation Scale were administered. Their AERP recordings were obtained. Executive functions and visuospatial integration were highly impaired in patients and slightly in their siblings compared to controls. P200 amplitude was sorted as siblings>patients>controls. P300 amplitude was sorted as patientsNeurological Evaluation Scale scores were lower in patients compared to siblings and controls. The logistic regression analysis showed that, higher P300 amplitude, better performance on block design test and faster completion of Stroop test would predict being in the control group, whereas higher P200 amplitude would predict being in the case (patient and sibling) groups. We suggest that these seem to be the potential endophenotypes of OCD. PMID:27100062

  17. Neurologic emergencies in pregnancy.

    PubMed

    Donaldson, J O

    1991-06-01

    Any one neurologic emergency is rare during pregnancy. As a group, neurologic disorders are a major cause of maternal mortality. Optimal management requires a multidisciplinary approach and ready access to the collective experience of other clinicians. This article discusses the management of status epilepticus, eclamptic hypertensive encephalopathy, stroke, including subarachnoid hemorrhage, myasthenic crisis, porphyric crisis, acute Guillain-Barré syndrome, autonomic hyperreflexia, malignant hyperthermia, chorea gravidarum, and Wernicke's encephalopathy. PMID:1945251

  18. Neurological complications of transplantation.

    PubMed

    Pustavoitau, Aliaksei; Bhardwaj, Anish; Stevens, Robert

    2011-01-01

    Recipients of solid organ or hematopoietic cell transplants are at risk of life-threatening neurological disorders including encephalopathy, seizures, infections and tumors of the central nervous system, stroke, central pontine myelinolysis, and neuromuscular disorders-often requiring admission to, or occurring in, the intensive care unit (ICU). Many of these complications are linked directly or indirectly to immunosuppressive therapy. However, neurological disorders may also result from graft versus host disease, or be an expression of the underlying disease which prompted transplantation, as well as injury induced during radiation, chemotherapy, surgery, and ICU stay. In rare cases, neuroinfectious pathogens may be transmitted with the transplanted tissue or organ. Diagnosis may be a challenge because clinical symptoms and findings on neuroimaging lack specificity, and a biological specimen or tissue diagnosis is often needed for definitive diagnosis. Management is centered on preventing further neurological injury, etiology-targeted therapy, and balancing the benefits and toxicities of specific immunosuppressive agents. PMID:21764765

  19. Neuroimaging distinction between neurological and psychiatric disorders†

    PubMed Central

    Crossley, Nicolas A.; Scott, Jessica; Ellison-Wright, Ian; Mechelli, Andrea

    2015-01-01

    Background It is unclear to what extent the traditional distinction between neurological and psychiatric disorders reflects biological differences. Aims To examine neuroimaging evidence for the distinction between neurological and psychiatric disorders. Method We performed an activation likelihood estimation meta-analysis on voxel-based morphometry studies reporting decreased grey matter in 14 neurological and 10 psychiatric disorders, and compared the regional and network-level alterations for these two classes of disease. In addition, we estimated neuroanatomical heterogeneity within and between the two classes. Results Basal ganglia, insula, sensorimotor and temporal cortex showed greater impairment in neurological disorders; whereas cingulate, medial frontal, superior frontal and occipital cortex showed greater impairment in psychiatric disorders. The two classes of disorders affected distinct functional networks. Similarity within classes was higher than between classes; furthermore, similarity within class was higher for neurological than psychiatric disorders. Conclusions From a neuroimaging perspective, neurological and psychiatric disorders represent two distinct classes of disorders. PMID:26045351

  20. Current neurology

    SciTech Connect

    Appel, S.H. )

    1988-01-01

    The topics covered in this book include: Duchenne muscular dystrophy: DNA diagnosis in practice; Central nervous system magnetic resonance imaging; and Magnetic resonance spectroscopy of neurologic diseases.

  1. Design of dual inhibitors of ROCK-I and NOX2 as potential leads for the treatment of neuroinflammation associated with various neurological diseases including autism spectrum disorder.

    PubMed

    Alokam, Reshma; Singhal, Sarthak; Srivathsav, Geetha Sai; Garigipati, Sowmya; Puppala, Sripriya; Sriram, Dharmarajan; Perumal, Yogeeswari

    2015-02-01

    Inhibition of both Rho kinase (ROCK-I) and NADPH oxidase (NOX2) to treat neuroinflammation could be very effective in the treatment of progressive neurological diseases like Alzheimer's disease, autism spectral disorder, and fragile X syndrome. NOX2 being a multi-enzyme component is activated during host defense in phagocytes such as microglia, to catalyze the production of superoxide from oxygen, while ROCK is an important mediator of fundamental cell processes like adhesion, proliferation and migration. Phosphorylated ROCK was found to activate NOX2 assembly via Ras related C3 botulinum toxin substrate (Rac) in disease conditions. Overexpression of ROCK-I and NOX2 in innate immune cells like microglial cells contribute to progressive neuronal damage early in neurological disease development. In the present study we employed a computer-aided methodology combining pharmacophores and molecular docking to identify new chemical entities that could inhibit ROCK-I as well as NOX2 (p47 phox). Among the huge dataset of a commercial database, top 18 molecules with crucial binding interactions were selected for biological evaluation. Seven among the lead molecules exhibited inhibitory potential against ROCK-I and NOX2 with IC50s ranging from 1.588 to 856.2 nM and 0.8942 to 10.24 μM, respectively, and emerged as potential hits as dual inhibitors with adequate selectivity index (SI = CC50/GIC50) in cell-based assays. The most active compound 3 was further found to show reduction of the pro-inflammatory mediators such as TNFα, interleukin-6 (IL-6) and interleukin-1beta (IL-1β) mRNA expression levels in activated (MeHg treated) human neuroblastoma (IMR32) cell lines. Hence the present work documented the utility of these dual inhibitors as prototypical leads to be useful for the treatment of neurological disorders including autism spectrum disorder and Alzheimer's disease. PMID:25465055

  2. 78 FR 51195 - Discretionary Advisory Committee on Heritable Disorders in Newborns and Children; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-08-20

    ... HUMAN SERVICES Health Resources and Services Administration Discretionary Advisory Committee on... or committees, was established to advise the Secretary of the Department of Health and Human Services... athletes, (3) a presentation on genome sequencing, (4) a panel on the Affordable Care Act, and (5)...

  3. Child neurology services in Africa.

    PubMed

    Wilmshurst, Jo M; Badoe, Eben; Wammanda, Robinson D; Mallewa, Macpherson; Kakooza-Mwesige, Angelina; Venter, Andre; Newton, Charles R

    2011-12-01

    The first African Child Neurology Association meeting identified key challenges that the continent faces to improve the health of children with neurology disorders. The capacity to diagnose common neurologic conditions and rare disorders is lacking. The burden of neurologic disease on the continent is not known, and this lack of knowledge limits the ability to lobby for better health care provision. Inability to practice in resource-limited settings has led to the migration of skilled professionals away from Africa. Referral systems from primary to tertiary are often unpredictable and chaotic. There is a lack of access to reliable supplies of basic neurology treatments such as antiepileptic drugs. Few countries have nationally accepted guidelines either for the management of epilepsy or status epilepticus. There is a great need to develop better training capacity across Africa in the recognition and management of neurologic conditions in children, from primary health care to the subspecialist level. PMID:22019842

  4. Child Neurology Services in Africa

    PubMed Central

    Wilmshurst, Jo M.; Badoe, Eben; Wammanda, Robinson D.; Mallewa, Macpherson; Kakooza-Mwesige, Angelina; Venter, Andre; Newton, Charles R.

    2013-01-01

    The first African Child Neurology Association meeting identified key challenges that the continent faces to improve the health of children with neurology disorders. The capacity to diagnose common neurologic conditions and rare disorders is lacking. The burden of neurologic disease on the continent is not known, and this lack of knowledge limits the ability to lobby for better health care provision. Inability to practice in resource-limited settings has led to the migration of skilled professionals away from Africa. Referral systems from primary to tertiary are often unpredictable and chaotic. There is a lack of access to reliable supplies of basic neurology treatments such as antiepileptic drugs. Few countries have nationally accepted guidelines either for the management of epilepsy or status epilepticus. There is a great need to develop better training capacity across Africa in the recognition and management of neurologic conditions in children, from primary health care to the subspecialist level. PMID:22019842

  5. CNS Penetration of Intrathecal-Lumbar Idursulfase in the Monkey, Dog and Mouse: Implications for Neurological Outcomes of Lysosomal Storage Disorder

    PubMed Central

    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. PMID:22279584

  6. Ravel's neurological illness.

    PubMed

    Alonso, R J; Pascuzzi, R M

    1999-01-01

    In the last 10 years of his life, Maurice Ravel (1875-1937) experienced a gradually progressive decline in neurological function. Dr. Alajouanine examined Ravel, noting the presence of aphasia and apraxia with relative preservation of comprehension and memory. The exact diagnosis remains unclear, but the likelihood of a progressive degenerative disorder, such as frontotemporal dementia, is herein discussed. PMID:10718529

  7. Neurologic deficit

    MedlinePlus

    ... neurologic deficit refers to abnormal function of a body area due to weaker function of the brain, spinal cord, muscles, or nerves. Examples include: Abnormal reflexes Inability to speak Decreased sensation Loss of balance ...

  8. Neurological Assessment.

    PubMed

    Fritz, Deborah; Musial, Maryann K

    2016-01-01

    Reasons for completing a neurological exam include: detecting life-threatening conditions, identifying nervous system dysfunction and the effects of this dysfunction on activities of daily living, comparing current data to previous exams to determine trends, and to provide a database upon which to base collaborative care across disciplines. In this third article of a four-part series, subjective and objective assessment of the neurological exam is reviewed. PMID:26645839

  9. Neurological assessment.

    PubMed

    Maher, Ann Butler

    2016-08-01

    Neurological system assessment is an important skill for the orthopaedic nurse because the nervous system has such an overlap with the musculoskeletal system. Nurses whose scope of practice includes such advanced evaluation, e.g. nurse practitioners, may conduct the examination described here but the information will also be useful for nurses caring for patients who have abnormal neurological assessment findings. Within the context of orthopaedic physical assessment, possible neurological findings are evaluated as they complement the patient's history and the examiner's findings. Specific neurological assessment is integral to diagnosis of some orthopaedic conditions such as carpal tunnel syndrome. In other situations such as crushing injury to the extremities, there is high risk of associated neurological or neurovascular injury. These patients need anticipatory examination and monitoring to prevent complications. This article describes a basic neurological assessment; emphasis is on sensory and motor findings that may overlap with an orthopaedic presentation. The orthopaedic nurse may incorporate all the testing covered here or choose those parts that further elucidate specific diagnostic questions suggested by the patient's history, general evaluation and focused musculoskeletal examination. Abnormal findings help to suggest further testing, consultation with colleagues or referral to a specialist. PMID:27118633

  10. Creativity and neurological disease.

    PubMed

    Acosta, Lealani Mae Y

    2014-08-01

    Although humans have long valued creativity, the generation of such innovation is still incompletely understood. Looking at the healthy brain, researchers have localized certain parts for a basic understanding of these mechanisms. By researching the brain affected by neurological disease, scientists have observed unique manifestations of creativity, such as in frontotemporal lobar degeneration, Alzheimer's disease, Parkinson's disease and parkinsonian spectrum disorders, and stroke, which help clarify these creative underpinnings. Incorporating both healthy and disease models of cerebral functioning, neurological and neuroscientific research from recent years has built on established theories and expanded current knowledge. PMID:24938215

  11. Neurologic Manifestations of Blood Dyscrasias.

    PubMed

    Couriel, Daniel R; Ricker, Holly; Steinbach, Mary; Lee, Catherine J

    2016-08-01

    Neurologic manifestations are common in blood diseases, and they can be caused by the hematologic disorder or its treatment. This article discusses hematologic diseases in adult patients, and categorizes them into benign and malignant conditions. The more common benign hematologic diseases associated with neurologic manifestations include anemias, particularly caused by B12 deficiency and sickle cell disease, and a variety of disorders of hemostasis causing bleeding or thrombosis, including thrombotic microangiopathy. Malignant conditions like multiple myeloma, leukemias, and lymphomas can have neurologic complications resulting from direct involvement, or caused by the different therapies to treat these cancers. PMID:27443994

  12. Genomic medicine and neurology.

    PubMed

    Vance, Jeffery M; Tekin, Demet

    2011-04-01

    The application of genetics to the understanding of neurology has been highly successful over the past several decades. During the past 10 years, tools were developed to begin genetic investigations into more common disorders such as Alzheimer disease, multiple sclerosis, autism, and Parkinson disease. The era of genomic medicine now has begun and will have an increasing effect on the daily care of common neurologic diseases. Thus it is important for neurologists to have a basic understanding of genomic medicine and how it differs from the traditional clinical genetics of the past. This article provides some basic information about genomic medicine and pharmacogenetics in neurology to help neurologists to begin to adopt these principles into their practice. PMID:22810818

  13. Bone marrow-derived macrophages and the CNS: An update on the use of experimental chimeric mouse models and bone marrow transplantation in neurological disorders.

    PubMed

    Larochelle, Antoine; Bellavance, Marc-André; Michaud, Jean-Philippe; Rivest, Serge

    2016-03-01

    The central nervous system (CNS) is a very unique system with multiple features that differentiate it from systemic tissues. One of the most captivating aspects of its distinctive nature is the presence of the blood brain barrier (BBB), which seals it from the periphery. Therefore, to preserve tissue homeostasis, the CNS has to rely heavily on resident cells such as microglia. These pivotal cells of the mononuclear lineage have important and dichotomous roles according to various neurological disorders. However, certain insults can overwhelm microglia as well as compromising the integrity of the BBB, thus allowing the infiltration of bone marrow-derived macrophages (BMDMs). The use of myeloablation and bone marrow transplantation allowed the generation of chimeric mice to study resident microglia and infiltrated BMDM separately. This breakthrough completely revolutionized the way we captured these 2 types of mononuclear phagocytic cells. We now realize that microglia and BMDM exhibit distinct features and appear to perform different tasks. Since these cells are central in several pathologies, it is crucial to use chimeric mice to analyze their functions and mechanisms to possibly harness them for therapeutic purpose. This review will shed light on the advent of this methodology and how it allowed deciphering the ontology of microglia and its maintenance during adulthood. We will also compare the different strategies used to perform myeloablation. Finally, we will discuss the landmark studies that used chimeric mice to characterize the roles of microglia and BMDM in several neurological disorders. This article is part of a Special Issue entitled: Neuro Inflammation edited by Helga E. de Vries and Markus Schwaninger. PMID:26432480

  14. Quantitative EEG Magnitudes in Children with and without Attention Deficit Disorder during Neurological Screening and Cognitive Tasks.

    ERIC Educational Resources Information Center

    Crawford, Helen J.; Barabasz, Marianne

    1996-01-01

    Quantitative EEG magnitude data were obtained from children with and without attention deficit disorder (ADD). The data suggest that the right fronto-centro-temporal region is not as "cognitively activated" relative to the left hemisphere in those children with ADD. Neurotherapy training of the right frontal and central regions in ADD children was…

  15. Motor Proficiency and Emotional/Behavioural Disturbance in Autism and Asperger's Disorder: Another Piece of the Neurological Puzzle?

    ERIC Educational Resources Information Center

    Papadopoulos, Nicole; McGinley, Jennifer; Tonge, Bruce; Bradshaw, John; Saunders, Kerryn; Murphy, Anna; Rinehart, Nicole

    2012-01-01

    The relationship of motor proficiency with emotional/behavioural disturbance, autistic symptoms and communication disturbance was investigated in children diagnosed with autism and Asperger's disorder (AD). The Movement Assessment Battery for Children was used as a measure of motor impairment, and the Developmental Behavioural Checklist was used…

  16. Toxoplasmosis and Polygenic Disease Susceptibility Genes: Extensive Toxoplasma gondii Host/Pathogen Interactome Enrichment in Nine Psychiatric or Neurological Disorders

    PubMed Central

    Carter, C. J.

    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 − 71) (multiple sclerosis), and autism (P = 0.013), 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. PMID:23533776

  17. Fellowship programs in behavioral neurology.

    PubMed

    Green, R C; Benjamin, S; Cummings, J L

    1995-03-01

    We sent a behavioral neurology fellowship questionnaire to each of the training directors of 160 neurology residency programs throughout the world, seeking information about programs offering advanced training in behavioral neurology (or similar fellowships in cognitive neurology, neurobehavior, or cognitive neuroscience). Response rate was 100%. Thirty-four respondents reported active fellowship programs in behavioral neurology, and 28 additional respondents indicated that a behavioral neurology fellowship was planned. Nine of the 34 programs (26.5%) defined themselves as exclusively or predominantly concerned with dementia and age-related neurobehavioral disorders. Directors of the 34 active fellowship programs estimated that their combined programs had graduated 199 fellows and were currently training fifty. Most fellowships concentrated on outpatient clinical training, with teaching required by 78.1% and research required by 81.8%. Specialty certification for behavioral neurology was favored by over 75% of behavioral neurology fellowship training directors but by only 30% of training directors in residency programs without behavioral neurology fellowships. Behavioral neurology training programs have grown dramatically in response to an increased recognition of the academic interest in and the clinical needs for these services. PMID:7898686

  18. Relationship between the chemokine receptor CCR5 and microglia in neurological disorders: consequences of targeting CCR5 on neuroinflammation, neuronal death and regeneration in a model of epilepsy.

    PubMed

    Louboutin, Jean-Pierre; Strayer, David S

    2013-09-01

    Chemokines may play a role in leukocyte migration across the blood-brain barrier (BBB) during neuroinflammation and other neuropathological processes, such as epilepsy. The CC chemokine receptor 5 (CCR5) is a member of CC-chemokine receptor family that binds several chemokines, including CCL3 (macrophage inflammatory protein-1alpha, MIP-1alpha), CCL4 (macrophage inflammatory protein-1beta, MIP-1beta) and CCL5 (RANTES). The current review examines the relationship between CCR5 and the microglia in different neurological disorders and models of CNS injury. CCR5 expression is upregulated in different neurological diseases, where it is often immunolocalized in microglial cells. A multistep cascade couples CCR5 activation by chemokines to Ca(2+) increases in human microglia. Because changes in [Ca(2+)] (i) affect chemotaxis, secretion, and gene expression, pharmacologic modulation of this pathway may alter inflammatory and degenerative processes in the CNS. Consequently, targeting CCR5 by using CCR5 antagonists may attenuate critical aspects of neuroinflammation in different models of neurological disorders. To illustrate the interaction between CCR5 and microglia in the CNS, we used a model of excitotoxicity, and demonstrate the intimate involvement of CCR5 in neuron injury and inflammation attendant to kainic acid (KA)-induced neurotoxicity. CCR5 participates in neuronal injury caused by the excitotoxin, KA, brings inflammatory cells to the sites of KA-induced CNS injury, defines the extent of tissue loss after KA exposure and limits reparative responses. We used a SV40-derived vector carrying an interfering RNA (RNAi) that targets CCR5. Delivered directly to the bone marrow, this vector decreased CCR5 expression in circulating cells. Animals so treated showed greatly reduced expression of CCR5 and its ligands (MIP-1alpha and RANTES) in the CNS, including in the brain vasculature, decreased BBB leakage, demonstrated greater KA-stimulated neurogenesis and increased

  19. Neurology and Don Quixote.

    PubMed

    Palma, Jose-Alberto; Palma, Fermin

    2012-01-01

    Don Quixote de la Mancha, which is considered one of the most important and influential works of Western modern prose, contains many references of interest for almost all of the medical specialties. In this regard, numerous references to neurology can be found in Cervantes' immortal work. In this study, we aimed to read Don Quixote from a neurologist's point of view, describing the neurological phenomena scattered throughout the novel, including tremors, sleep disturbances, neuropsychiatric symptoms, dementia, epilepsy, paralysis, stroke, syncope, traumatic head injury, and headache; we relate these symptoms with depictions of those conditions in the medical literature of the time. We also review Cervantes' sources of neurological information, including the works by renowned Spanish authors such as Juan Huarte de San Juan, Dionisio Daza Chacón and Juan Valverde de Amusco, and we hypothesize that Don Quixote's disorder was actually a neurological condition. Although Cervantes wrote it four centuries ago, Don Quixote contains plenty of references to neurology, and many of the ideas and concepts reflected in it are still of interest. PMID:23006630

  20. 78 FR 23770 - Establishment of the Discretionary Advisory Committee on Heritable Disorders in Newborns and...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-04-22

    ..., counseling, testing, or specialty services for newborns and children at risk for heritable disorders; (b) experts in ethics and heritable disorders who have worked and published material in the area of public health and genetic conditions; and (c) members from the public sector who have expertise,...

  1. Visual mismatch negativity (vMMN): A review and meta-analysis of studies in psychiatric and neurological disorders.

    PubMed

    Kremláček, Jan; Kreegipuu, Kairi; Tales, Andrea; Astikainen, Piia; Põldver, Nele; Näätänen, Risto; Stefanics, Gábor

    2016-07-01

    The visual mismatch negativity (vMMN) response is an event-related potential (ERP) component, which is automatically elicited by events that violate predictions based on prior events. VMMN experiments use visual stimulus repetition to induce predictions, and vMMN is obtained by subtracting the response to rare unpredicted stimuli from those to frequent stimuli. One increasingly popular interpretation of the mismatch response postulates that vMMN, similar to its auditory counterpart (aMMN), represents a prediction error response generated by cortical mechanisms forming probabilistic representations of sensory signals. Here we discuss the physiological and theoretical basis of vMMN and review thirty-three studies from the emerging field of its clinical applications, presenting a meta-analysis of findings in schizophrenia, mood disorders, substance abuse, neurodegenerative disorders, developmental disorders, deafness, panic disorder and hypertension. Furthermore, we include reports on aging and maturation as they bear upon many clinically relevant conditions. Surveying the literature we found that vMMN is altered in several clinical populations which is in line with aMMN findings. An important potential advantage of vMMN however is that it allows the investigation of deficits in predictive processing in cognitive domains which rely primarily on visual information; a principal sensory modality and thus of vital importance in environmental information processing and response, and a modality which arguably may be more sensitive to some pathological changes. However, due to the relative infancy of research in vMMN compared to aMMN in clinical populations its potential for clinical application is not yet fully appreciated. The aim of this review and meta-analysis therefore is to present, in a detailed systematic manner, the findings from clinically-based vMMN studies, to discuss their potential impact and application, to raise awareness of this measure and to improve our

  2. Report: Stem cell applications in neurological practice, an expert group consensus appraisal

    PubMed Central

    Devi, M. Gourie; Sharma, Alka; Mohanty, Sujata; Jain, Neeraj; Verma, Kusum; Padma, M. Vasantha; Pal, Pramod; Chabbra, H. S.; Khadilkar, Satish; Prabhakar, Sudesh; Singh, Gagandeep

    2016-01-01

    Introduction: Neurologists in their clinical practice are faced with inquiries about the suitability of stem cell approaches by patients with a variety of acute and chronic (namely neurodegenerative) disorders. The challenge is to provide these patients with accurate information about the scope of stem cell use as well as at the same time, empowering patients with the capacity to make an autonomous decision regarding the use of stem cells. Methods: The Indian Academy of Neurology commissioned an Expert Group Meeting to formulate an advisory to practicing neurologists to counsel patients seeking information and advice about stem cell approaches. Results and Conclusions: In the course of such counselling, it should be emphasized that the information provided by many lay websites might be unsubstantiated. Besides, standard recommendations for the stem cell research, in particular, the application of several layers of oversight should be strictly adhered in order to ensure safety and ethical use of stem cells in neurological disorders. PMID:27570390

  3. Combining non-pharmacological treatments with pharmacotherapies for neurological disorders: a unique interface of the brain, drug-device, and intellectual property.

    PubMed

    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

  4. Combining Non-Pharmacological Treatments with Pharmacotherapies for Neurological Disorders: A Unique Interface of the Brain, Drug–Device, and Intellectual Property

    PubMed Central

    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

  5. Motor proficiency and emotional/behavioural disturbance in autism and Asperger's disorder: another piece of the neurological puzzle?

    PubMed

    Papadopoulos, Nicole; McGinley, Jennifer; Tonge, Bruce; Bradshaw, John; Saunders, Kerryn; Murphy, Anna; Rinehart, Nicole

    2012-11-01

    The relationship of motor proficiency with emotional/behavioural disturbance, autistic symptoms and communication disturbance was investigated in children diagnosed with autism and Asperger's disorder (AD). The Movement Assessment Battery for Children was used as a measure of motor impairment, and the Developmental Behavioural Checklist was used as a measure of emotional/behavioural disturbance in the following groups: AD (n = 22), high functioning autism (HFA) (n = 23), LFA (n = 8) and typically developing children (n = 20). The HFA group had more difficulty with motor items, such as ball skills and balance, than did the AD group. There were significant positive correlations between impairments in motor proficiency (in particular ball skills and balance) and emotional/behavioural disturbance, autistic symptoms and communication disturbance. These findings are consistent with the hypothesis that there are qualitative and quantitative differences in the motor profile between autism and AD. In addition, the association between motor proficiency impairment and emotional/behavioural disturbance in autism and AD emphasizes the importance for screening of co-occurring emotional/behavioural symptoms in individuals with motor difficulties. These findings have implications for the potential use of adjunct motor measures in the diagnosis and definition of autism spectrum disorders. PMID:21949004

  6. 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

    PubMed Central

    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

  7. Neurological diseases and pain

    PubMed Central

    2012-01-01

    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 frequently becomes centralized through maladaptive responses within the central nervous system that can profoundly alter brain systems and thereby behaviour (e.g. depression). Chronic pain should thus be considered a brain disease in which alterations in neural networks affect multiple aspects of brain function, structure and chemistry. The study and treatment of this disease is greatly complicated by the lack of objective measures for either the symptoms or the underlying mechanisms of chronic pain. In pain associated with neurological disease, it is sometimes difficult to obtain even a subjective evaluation of pain, as is the case for patients in a vegetative state or end-stage Alzheimer's disease. It is critical that neurologists become more involved in chronic pain treatment and research (already significant in the fields of migraine and peripheral neuropathies). To achieve this goal, greater efforts are needed to enhance training for neurologists in pain treatment and promote greater interest in the field. This review describes examples of pain in different neurological diseases including primary neurological pain conditions, discusses the therapeutic potential of brain-targeted therapies and highlights the need for objective measures of pain. PMID:22067541

  8. Transcriptomic analysis of the hippocampus from six inbred strains of mice suggests a basis for sex-specific susceptibility and severity of neurological disorders.

    PubMed

    Vied, Cynthia; Ray, Surjyendu; Badger, Crystal-Dawn; Bundy, Joseph L; Arbeitman, Michelle N; Nowakowski, Richard S

    2016-09-01

    Identifying sex differences in gene expression within the brain is critical for determining why multiple neurological and behavioral disorders differentially affect males and females. Several disorders are more common or severe in males (e.g., autism and schizophrenia) or in females (e.g., Alzheimer's disease and depression). We analyzed transcriptomic data from the mouse hippocampus of six inbred strains (129S1/SvImJ, A/J, C57BL/6J, DBA/1J, DBA/2J, and PWD/Ph) to provide a perspective on differences between male and female gene expression. Our data show that 1) gene expression differences in males vs. females varies substantially across the strains, 2) only a few genes are differentially expressed across all of the strains (termed core genes), and 3) >2,600 genes differ in the individual strain comparisons (termed noncore genes). We found that DBA/2J uniquely has a substantial majority (89%) of differentially expressed genes (DEGs) that are more highly expressed in females than in males (female-biased); 129/SvImJ has a majority (69%) of DEGs that are more highly expressed in males. To gain insight into the function of the DEGs, we examined gene ontology and pathway and phenotype enrichment and found significant enrichment in phenotypes related to abnormal nervous system morphology and physiology, among others. In addition, several pathways are enriched significantly, including Alzheimer's disease (AD), with 32 genes implicated in AD, eight of which are male-biased. Three of the male-biased genes have been implicated in a neuroprotective role in AD. Our transcriptomic data provide new insight into the possible genetic bases for sex-specific susceptibility and severity of brain disorders. J. Comp. Neurol. 524:2696-2710, 2016. © 2016 Wiley Periodicals, Inc. PMID:26917114

  9. Roles and effectiveness of lay community health workers in the prevention of mental, neurological and substance use disorders in low and middle income countries: a systematic review

    PubMed Central

    2013-01-01

    Background It has been suggested that lay community health workers (LHWs) could play a role in primary and secondary prevention of Mental, Neurological and Substance use (MNS) disorders in low resourced settings. We conducted a systematic review of the literature with the aim of assessing the existing evidence base for the roles and effectiveness of LHWs in primary and secondary prevention of MNS disorders in low and middle income countries (LMICs). Methods Internet searches of relevant electronic databases for articles published in English were done in August 2011 and repeated in June 2013. Abstracts and full text articles were screened according to predefined criteria. Authors were asked for additional information where necessary. Results A total of 15 studies, 11 of which were randomised, met our inclusion criteria. Studies were heterogeneous with respect to interventions, outcomes and LHWs’ roles. Reduction in symptoms of depression and improved child mental development were the common outcomes assessed. Primary prevention and secondary prevention strategies were carried out in 11 studies and 4 studies respectively .There was evidence of effectiveness of interventions however, most studies (n = 13) involved small sample sizes and all were judged to have an unclear or high risk of bias. Conclusions LHWs have the potential to provide psychosocial and psychological interventions as part of primary and secondary prevention of MNS disorders in LMICs, but there is currently insufficient robust evidence of effectiveness of LHW led preventive strategies in this setting. More studies need to be carried out in a wider range of settings in LMICs that control for risk of bias as far as possible, and that also collect indicators relating to the fidelity and cost of interventions. PMID:24119375

  10. Insights into the Pathology of the α3 Na(+)/K(+)-ATPase Ion Pump in Neurological Disorders; Lessons from Animal Models.

    PubMed

    Holm, Thomas H; Lykke-Hartmann, Karin

    2016-01-01

    The transmembrane Na(+)-/K(+) ATPase is located at the plasma membrane of all mammalian cells. The Na(+)-/K(+) ATPase utilizes energy from ATP hydrolysis to extrude three Na(+) cations and import two K(+) cations into the cell. The minimum constellation for an active Na(+)-/K(+) ATPase is one alpha (α) and one beta (β) subunit. Mammals express four α isoforms (α1-4), encoded by the ATP1A1-4 genes, respectively. The α1 isoform is ubiquitously expressed in the adult central nervous system (CNS) whereas α2 primarily is expressed in astrocytes and α3 in neurons. Na(+) and K(+) are the principal ions involved in action potential propagation during neuronal depolarization. The α1 and α3 Na(+)-/K(+) ATPases are therefore prime candidates for restoring neuronal membrane potential after depolarization and for maintaining neuronal excitability. The α3 isoform has approximately four-fold lower Na(+) affinity compared to α1 and is specifically required for rapid restoration of large transient increases in [Na(+)]i. Conditions associated with α3 deficiency are therefore likely aggravated by suprathreshold neuronal activity. The α3 isoform been suggested to support re-uptake of neurotransmitters. These processes are required for normal brain activity, and in fact autosomal dominant de novo mutations in ATP1A3 encoding the α3 isoform has been found to cause the three neurological diseases Rapid Onset Dystonia Parkinsonism (RDP), Alternating Hemiplegia of Childhood (AHC), and Cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS). All three diseases cause acute onset of neurological symptoms, but the predominant neurological manifestations differ with particularly early onset of hemiplegic/dystonic episodes and mental decline in AHC, ataxic encephalopathy and impairment of vision and hearing in CAPOS syndrome and late onset of dystonia/parkinsonism in RDP. Several mouse models have been generated to study the in vivo

  11. Insights into the Pathology of the α3 Na+/K+-ATPase Ion Pump in Neurological Disorders; Lessons from Animal Models

    PubMed Central

    Holm, Thomas H.; Lykke-Hartmann, Karin

    2016-01-01

    The transmembrane Na+-/K+ ATPase is located at the plasma membrane of all mammalian cells. The Na+-/K+ ATPase utilizes energy from ATP hydrolysis to extrude three Na+ cations and import two K+ cations into the cell. The minimum constellation for an active Na+-/K+ ATPase is one alpha (α) and one beta (β) subunit. Mammals express four α isoforms (α1−4), encoded by the ATP1A1-4 genes, respectively. The α1 isoform is ubiquitously expressed in the adult central nervous system (CNS) whereas α2 primarily is expressed in astrocytes and α3 in neurons. Na+ and K+ are the principal ions involved in action potential propagation during neuronal depolarization. The α1 and α3 Na+-/K+ ATPases are therefore prime candidates for restoring neuronal membrane potential after depolarization and for maintaining neuronal excitability. The α3 isoform has approximately four-fold lower Na+ affinity compared to α1 and is specifically required for rapid restoration of large transient increases in [Na+]i. Conditions associated with α3 deficiency are therefore likely aggravated by suprathreshold neuronal activity. The α3 isoform been suggested to support re-uptake of neurotransmitters. These processes are required for normal brain activity, and in fact autosomal dominant de novo mutations in ATP1A3 encoding the α3 isoform has been found to cause the three neurological diseases Rapid Onset Dystonia Parkinsonism (RDP), Alternating Hemiplegia of Childhood (AHC), and Cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS). All three diseases cause acute onset of neurological symptoms, but the predominant neurological manifestations differ with particularly early onset of hemiplegic/dystonic episodes and mental decline in AHC, ataxic encephalopathy and impairment of vision and hearing in CAPOS syndrome and late onset of dystonia/parkinsonism in RDP. Several mouse models have been generated to study the in vivo consequences of Atp1a3 modulation

  12. Neurology--the next 10 years.

    PubMed

    Baron, Ralf; Ferriero, Donna M; Frisoni, Giovanni B; Bettegowda, Chetan; Gokaslan, Ziya L; Kessler, John A; Vezzani, Annamaria; Waxman, Stephen G; Jarius, Sven; Wildemann, Brigitte; Weller, Michael

    2015-11-01

    Since the launch of our journal as Nature Clinical Practice Neurology in 2005, we have seen remarkable progress in many areas of neurology research, but what does the future hold? Will advances in basic research be translated into effective disease-modifying therapies, and will personalized medicine finally become a reality? For this special Viewpoint article, we invited a panel of Advisory Board members and other journal contributors to outline their research priorities and predictions in neurology for the next 10 years. PMID:26503922

  13. Neuro-psychopharmacogenetics and Neurological Antecedents of Posttraumatic Stress Disorder: Unlocking the Mysteries of Resilience and Vulnerability

    PubMed Central

    Bowirrat, Abdalla; Chen, Thomas J.H.; Blum, Kenneth; Madigan, Margaret; Bailey, John A.; Chuan Chen, Amanda Lih; Downs, B. William; Braverman, Eric R.; Radi, Shahien; Waite, Roger L.; Kerner, Mallory; Giordano, John; Morse, Siohban; Oscar-Berman, Marlene; Gold, Mark

    2010-01-01

    Background and Hypothesis: Although the biological underpinnings of immediate and protracted trauma-related responses are extremely complex, 40 years of research on humans and other mammals have demonstrated that trauma (particularly trauma early in the life cycle) has long-term effects on neurochemical responses to stressful events. These effects include the magnitude of the catecholamine response and the duration and extent of the cortisol response. In addition, a number of other biological systems are involved, including mesolimbic brain structures and various neurotransmitters. An understanding of the many genetic and environmental interactions contributing to stress-related responses will provide a diagnostic and treatment map, which will illuminate the vulnerability and resilience of individuals to Posttraumatic Stress Disorder (PTSD). Proposal and Conclusions: We propose that successful treatment of PTSD will involve preliminary genetic testing for specific polymorphisms. Early detection is especially important, because early treatment can improve outcome. When genetic testing reveals deficiencies, vulnerable individuals can be recommended for treatment with “body friendly” pharmacologic substances and/or nutrients. Results of our research suggest the following genes should be tested: serotoninergic, dopaminergic (DRD2, DAT, DBH), glucocorticoid, GABAergic (GABRB), apolipoprotein systems (APOE2), brain-derived neurotrophic factor, Monamine B, CNR1, Myo6, CRF-1 and CRF-2 receptors, and neuropeptide Y (NPY). Treatment in part should be developed that would up-regulate the expression of these genes to bring about a feeling of well being as well as a reduction in the frequency and intensity of the symptoms of PTSD. PMID:21629442

  14. PIPs in neurological diseases.

    PubMed

    Waugh, Mark G

    2015-08-01

    Phosphoinositide (PIP) lipids regulate many aspects of cell function in the nervous system including receptor signalling, secretion, endocytosis, migration and survival. Levels of PIPs such as PI4P, PI(4,5)P2 and PI(3,4,5)P3 are normally tightly regulated by phosphoinositide kinases and phosphatases. Deregulation of these biochemical pathways leads to lipid imbalances, usually on intracellular endosomal membranes, and these changes have been linked to a number of major neurological diseases including Alzheimer's, Parkinson's, epilepsy, stroke, cancer and a range of rarer inherited disorders including brain overgrowth syndromes, Charcot-Marie-Tooth neuropathies and neurodevelopmental conditions such as Lowe's syndrome. This article analyses recent progress in this area and explains how PIP lipids are involved, to varying degrees, in almost every class of neurological disease. This article is part of a Special Issue entitled Brain Lipids. PMID:25680866

  15. Could Local Dynamic Stability Serve as an Early Predictor of Falls in Patients with Moderate Neurological Gait Disorders? A Reliability and Comparison Study in Healthy Individuals and in Patients with Paresis of the Lower Extremities

    PubMed Central

    Reynard, Fabienne; Vuadens, Philippe; Deriaz, Olivier; Terrier, Philippe

    2014-01-01

    Falls while walking are frequent in patients with muscular dysfunction resulting from neurological disorders. Falls induce injuries that may lead to deconditioning and disabilities, which further increase the risk of falling. Therefore, an early gait stability index would be useful to evaluate patients in order to prevent the occurrence of future falls. Derived from chaos theory, local dynamic stability (LDS), defined by the maximal Lyapunov exponent, assesses the sensitivity of a dynamic system to small perturbations. LDS has already been used for fall risk prediction in elderly people. The aim of the present study was to provide information to facilitate future researches regarding gait stability in patients with neurological gait disorders. The main objectives were 1) to evaluate the intra-session repeatability of LDS in patients and 2) to assess the discriminative power of LDS to differentiate between healthy individuals and neurological patients. Eighty-three patients with mild to moderate neurological disorders associated with paresis of the lower extremities and 40 healthy controls participated in the study. The participants performed 2×30 s walking wearing a 3D accelerometer attached to the lower back, from which 2×35 steps were extracted. LDS was defined as the average exponential rate of divergence among trajectories in a reconstructed state-space that reflected the gait dynamics. LDS assessed along the medio-lateral axis offered the highest repeatability and discriminative power. Intra-session repeatability (intraclass correlation coefficient between the two repetitions) in the patients was 0.89 and the smallest detectable difference was 16%. LDS was substantially lower in the patients than in the controls (33% relative difference, standardized effect size 2.3). LDS measured in short over-ground walking tests seems sufficiently reliable. LDS exhibits good discriminative power to differentiate fall-prone individuals and opens up the possibility of

  16. Neurologic Diseases

    MedlinePlus

    The brain, spinal cord, and nerves make up the nervous system. Together they control all the workings of the body. When something goes wrong ... develops, such as spina bifida Degenerative diseases, where nerve cells are ... to the spinal cord and brain Seizure disorders, such as epilepsy ...

  17. Autism Spectrum Disorders in Africa: Current Challenges in Identification, Assessment, and Treatment: A Report on the International Child Neurology Association Meeting on ASD in Africa, Ghana, April 3-5, 2014.

    PubMed

    Ruparelia, Kavita; Abubakar, Amina; Badoe, Eben; Bakare, Muideen; Visser, Karren; Chugani, Diane C; Chugani, Harry T; Donald, Kirsten A; Wilmshurst, Jo M; Shih, Andy; Skuse, David; Newton, Charles R

    2016-07-01

    Prevalence of autism spectrum disorders has increased over recent years, however, little is known about the identification and management of autism spectrum disorder in Africa. This report summarizes a workshop on autism spectrum disorder in Africa under the auspices of the International Child Neurology Association and the African Child Neurology Association through guided presentations and working group reports, focusing on identification, diagnosis, management, and community support. A total of 47 delegates participated from 14 African countries. Although there was a huge variability in services across the countries represented, numbers of specialists assessing and managing autism spectrum disorder was small relative to populations served. Strategies were proposed to improve identification, diagnosis, management and support delivery for individuals with autism spectrum disorder across Africa in these culturally diverse, low-resource settings. Emphasis on raising public awareness through community engagement and improving access to information and training in autism spectrum disorder. Special considerations for the cultural, linguistic, and socioeconomic factors within Africa are discussed. PMID:26979098

  18. Selective targeting of MAPK family kinases JNK over p38 by rationally designed peptides as potential therapeutics for neurological disorders and epilepsy.

    PubMed

    Zhuo, Zhi-Hong; Sun, Yi-Zhen; Jin, Pei-Na; Li, Feng-Yan; Zhang, Yi-Le; Wang, Huai-Li

    2016-07-19

    Human mitogen-activated protein kinase (MAPK) family members JNK and p38 are two homologous protein-serine/threonine kinases but play distinct roles in the pathological process of neurological disorders. Selective targeting of JNK over p38 has been established as a potential therapeutic approach to epilepsy and other nervous system diseases. Herein, we describe an integrated in vitro-in silico protocol to rationally design kinase-peptide interaction specificity based on crystal structure data. In the procedure, a simulated annealing (SA) iteration optimization strategy is described to improve peptide selectivity between the two kinases. The optimization accepts moderate compromise in peptide affinity to JNK in order to maximize the affinity difference between peptide interactions with JNK and p38. The structural basis, energetic properties and dynamic behavior of SA-improved peptides bound with the peptide-docking sites of JNK and p38 kinase domains are investigated in detail using atomistic molecular dynamics (MD) simulations and post binding free energy analysis. The theoretical findings and computational designs are then confirmed by fluorescence polarization assays. Using the integrated protocol we successfully obtain three decapeptide ligands, namely RLHPSMTDFL, RAKLPTSVDY and KPSRPWNLEI, that exhibit both potent affinity to JNK (K = 8.0, 5.4 and 12.1 μM, respectively) and high selectivity for JNK over p38 (K/K = 9.2, 17.9 and 6.3 fold, respectively). We also demonstrate that a JNK-over-p38 selective peptide should have a positively charged N-terminus, a polar central region and a negatively charged C-terminus, in which a number of hydrophobic residues distribute randomly along the peptide sequence. In particular, the residue positions 1, 6 and 9 play a crucial role in shaping peptide selectivity; the presence of, respectively, Arg, Thr and Asp at the three positions confers high specificity to kinase-peptide interactions. PMID:27263470

  19. Population-Level Retrospective Study of Neurologically Expressed Disorders in Ruminants before the Onset of Bovine Spongiform Encephalopathy (BSE) in Belgium, a BSE Risk III Country

    PubMed Central

    Saegerman, C.; Berkvens, D.; Claes, L.; Dewaele, A.; Coignoul, F.; Ducatelle, R.; Cassart, D.; Brochier, B.; Costy, F.; Roels, S.; Deluyker, H.; Vanopdenbosch, E.; Thiry, E.

    2005-01-01

    A retrospective epidemiological study (n = 7,875) of neurologically expressed disorders (NED) in ruminants before the onset of the bovine spongiform encephalopathy epidemic (years studied, 1980 to 1997) was carried out in Belgium. The archives of all veterinary laboratories and rabies and transmissible spongiform encephalopathy (TSE) epidemiosurveillance networks were consulted. For all species, a significantly higher number of NED with virological causes (rabies) was reported south of the Sambre-Meuse Valley. During the period 1992 to 1997, for which the data were complete, (i) the predicted annual incidence of NED varied significantly as a function of species and area (higher numbers in areas where rabies was present) but was always above 100 cases per million, and (ii) the mean incidence of suspected TSE cases and, among them, those investigated by histopathological examination varied significantly as a function of species and area. The positive predictive value of a presumptive clinical diagnosis of NED ranged from 0.13 (game) to 0.63 (sheep). Knowledge of the positive predictive value permits the definition of a reference point before certain actions (e.g., awareness and training campaigns) are undertaken. It also shows the usefulness of a systematic necropsy or complementary laboratory tests to establish an etiological diagnosis. TSE analysis of a small, targeted historical sampling (n = 48) permitted the confirmation of one case and uncovered another case of scrapie. The results of the present study help to develop and maintain the quality of the worldwide clinical epidemiological networks for TSE, especially in countries that in the past imported live animals, animal products, and feedstuffs from countries with TSE cases. PMID:15695693

  20. Nutritional disorders in tropical neurology.

    PubMed

    Román, Gustavo C

    2013-01-01

    About three-fourths of the total world population live in the tropics but consume only 6% of worldwide food production and contribute 15% of the world's net revenue explaining the short life expectancy, high infantile mortality, and poor daily caloric intake; moreover, lack of clean drinking water and deficient sanitation promote water-borne infections, diarrhea, and risk of malabsorption that contribute to the prevalence of malnutrition in the tropics. One-third of the world's population consumes insufficient iodine increasing the risk for mental retardation and deafness due to maternal hypothyroidism. The main nutritional syndromes comprise protein-energy malnutrition (marasmus and kwashiorkor); nutritional neuropathies, myelopathies and neuromyelopathies, as well as specific deficiencies of vitamins and micronutrients including iodine, iron, zinc, and selenium. PMID:23829926

  1. Paraneoplastic neurological syndromes.

    PubMed

    Honnorat, Jérôme; Antoine, Jean-Christophe

    2007-01-01

    Paraneoplastic neurological syndromes (PNS) can be defined as remote effects of cancer that are not caused by the tumor and its metastasis, or by infection, ischemia or metabolic disruptions. PNS are rare, affecting less than 1/10,000 patients with cancer. Only the Lambert-Eaton myasthenic syndrome is relatively frequent, occurring in about 1% of patients with small cell lung cancer. PNS can affect any part of the central and peripheral nervous system, the neuromuscular junction, and muscle. They can be isolated or occur in association. In most patients, the neurological disorder develops before the cancer becomes clinically overt and the patient is referred to the neurologist who has the charge of identifying a neurological disorder as paraneoplastic. PNS are usually severely disabling. The most common PNS are Lambert-Eaton myasthenic syndrome (LEMS), subacute cerebellar ataxia, limbic encephalitis (LE), opsoclonus-myoclonus (OM), retinopathies (cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR), Stiff-Person syndrome (SPS), chronic gastrointestinal pseudoobstruction (CGP), sensory neuronopathy (SSN), encephalomyelitis (EM) and dermatomyositis. PNS are caused by autoimmune processes triggered by the cancer and directed against antigens common to both the cancer and the nervous system, designated as onconeural antigens. Due to their high specificity (> 90%), the best way to diagnose a neurological disorder as paraneoplastic is to identify one of the well-characterized anti-onconeural protein antibodies in the patient's serum. In addition, as these antibodies are associated with a restricted range of cancers, they can guide the search for the underlying tumor at a stage when it is frequently not clinically overt. This is a critical point as, to date, the best way to stabilize PNS is to treat the cancer as soon as possible. Unfortunately, about one-third of patients do not have detectable antibodies and 5% to 10% have an atypical antibody

  2. [Cerebrolysin in pediatric neurology practice].

    PubMed

    Petrukhin, A S; Pylaeva, O A

    2014-01-01

    Мany aspects of сerebrolysin treatment in a wide range of nervous system disorders in children are described. High efficacy and well tolerated therapy are revealed. These findings expand the perspectives of using сerebrolysin in pediatric neurology. PMID:24637827

  3. Neurology Case Studies: Cerebrovascular Disease.

    PubMed

    Farooq, Muhammad U; Gorelick, Philip B

    2016-08-01

    This article discusses interesting vascular neurology cases including the management of intracranial stenosis, migraine headache and stroke risk, retinal artery occlusions associated with impaired hearing, intracranial occlusive disease, a heritable cause of stroke and vascular cognitive impairment, and an interesting clinico-neuroradiologic disorder associated with eclampsia. PMID:27445238

  4. Fly model causes neurological rethink

    PubMed Central

    Sadanandappa, Madhumala K

    2013-01-01

    A Drosophila model for a neurological disorder called type 2B Charcot-Marie-Tooth disease reveals that it has its origins in a partial loss of function, rather than a gain of function, which points to the need for a new therapeutic approach. PMID:24336781

  5. Edgar Allan Poe and neurology.

    PubMed

    Teive, Hélio Afonso Ghizoni; Paola, Luciano de; Munhoz, Renato Puppi

    2014-06-01

    Edgar Allan Poe was one of the most celebrated writers of all time. He published several masterpieces, some of which include references to neurological diseases. Poe suffered from recurrent depression, suggesting a bipolar disorder, as well as alcohol and drug abuse, which in fact led to his death from complications related to alcoholism. Various hypotheses were put forward, including Wernicke's encephalopathy. PMID:24964115

  6. Historical perspective of Indian neurology

    PubMed Central

    Mishra, Shrikant; Trikamji, Bhavesh; Singh, Sandeep; Singh, Parampreet; Nair, Rajasekharan

    2013-01-01

    Objective: To chronicle the history of medicine and neurology in India with a focus on its establishment and evolution. Background: The history of neurology in India is divided into two periods: ancient and modern. The ancient period dates back to the mid-second millennium Before Christ (B.C.) during the creation of the Ayurvedic Indian system of Medicine, which detailed descriptions of neurological disorders called Vata Vyadhi. The early 20th century witnessed the birth of modern Indian medicine with the onset of formal physician training at the nation's first allopathic medical colleges located in Madras (1835), Calcutta (1835) and Mumbai (1848). Prior to India's independence from Britain in 1947, only 25 medical schools existed in the entire country. Today, there are over 355. In 1951, physicians across the field of neurology and neurosurgery united to create the Neurological Society of India (NSI). Four decades later in 1991, neurologists branched out to establish a separate organization called the Indian Academy of Neurology (IAN). Design/Methods: Information was gathered through literature review using PubMed, MD Consult, OVID, primary texts and research at various academic institutions in India. Results: Neurological disorders were first described in ancient India under Ayurveda. The transition to modern medicine occurred more recently through formal training at medical schools beginning in the 1930's. Early pioneers and founders of the NSI (1951) include Dr. Jacob Chandy, Dr. B Ramamurthi, Dr. S. T. Narasimhan and Dr. Baldev Singh. Later, Dr. J. S. Chopra, a prominent neurologist and visionary, recognized the need for primary centers of collaboration and subsequently established the IAN (1991). The future of Neurology in India is growing rapidly. Currently, there are 1100 practicing neurologists and more than 150 post-graduate trainees who join the ranks every year. As the number of neurologists rises across India, there is an increase in the amount of

  7. Role of a redox-based methylation switch in mRNA life cycle (pre- and post-transcriptional maturation) and protein turnover: implications in neurological disorders.

    PubMed

    Trivedi, Malav S; Deth, Richard C

    2012-01-01

    Homeostatic synaptic scaling in response to neuronal stimulus or activation, and due to changes in cellular niche, is an important phenomenon for memory consolidation, retrieval, and other similar cognitive functions (Turrigiano and Nelson, 2004). Neurological disorders and cognitive disabilities in autism, Rett syndrome, schizophrenia, dementia, etc., are strongly correlated to alterations in protein expression (both synaptic and cytoplasmic; Cajigas et al., 2010). This correlation suggests that efficient temporal regulation of synaptic protein expression is important for synaptic plasticity. In addition, equilibrium between mRNA processing, protein translation, and protein turnover is a critical sensor/trigger for recording synaptic information, normal cognition, and behavior (Cajigas et al., 2010). Thus a regulatory switch, which controls the lifespan, maturation, and processing of mRNA, might influence cognition and adaptive behavior. Here, we propose a two part novel hypothesis that methylation might act as this suggested coordinating switch to critically regulate mRNA maturation at (1) the pre-transcription level, by regulating precursor-RNA processing into mRNA, via other non-coding RNAs and their influence on splicing phenomenon, and (2) the post-transcription level by modulating the regulatory functions of ribonucleoproteins and RNA binding proteins in mRNA translation, dendritic translocation as well as protein synthesis and synaptic turnover. DNA methylation changes are well recognized and highly correlated to gene expression levels as well as, learning and memory; however, RNA methylation changes are recently characterized and yet their functional implications are not established. This review article provides some insight on the intriguing consequences of changes in methylation levels on mRNA life-cycle. We also suggest that, since methylation is under the control of glutathione anti-oxidant levels (Lertratanangkoon et al., 1997), the redox status of

  8. Role of a Redox-Based Methylation Switch in mRNA Life Cycle (Pre- and Post-Transcriptional Maturation) and Protein Turnover: Implications in Neurological Disorders

    PubMed Central

    Trivedi, Malav S.; Deth, Richard C.

    2012-01-01

    Homeostatic synaptic scaling in response to neuronal stimulus or activation, and due to changes in cellular niche, is an important phenomenon for memory consolidation, retrieval, and other similar cognitive functions (Turrigiano and Nelson, 2004). Neurological disorders and cognitive disabilities in autism, Rett syndrome, schizophrenia, dementia, etc., are strongly correlated to alterations in protein expression (both synaptic and cytoplasmic; Cajigas et al., 2010). This correlation suggests that efficient temporal regulation of synaptic protein expression is important for synaptic plasticity. In addition, equilibrium between mRNA processing, protein translation, and protein turnover is a critical sensor/trigger for recording synaptic information, normal cognition, and behavior (Cajigas et al., 2010). Thus a regulatory switch, which controls the lifespan, maturation, and processing of mRNA, might influence cognition and adaptive behavior. Here, we propose a two part novel hypothesis that methylation might act as this suggested coordinating switch to critically regulate mRNA maturation at (1) the pre-transcription level, by regulating precursor-RNA processing into mRNA, via other non-coding RNAs and their influence on splicing phenomenon, and (2) the post-transcription level by modulating the regulatory functions of ribonucleoproteins and RNA binding proteins in mRNA translation, dendritic translocation as well as protein synthesis and synaptic turnover. DNA methylation changes are well recognized and highly correlated to gene expression levels as well as, learning and memory; however, RNA methylation changes are recently characterized and yet their functional implications are not established. This review article provides some insight on the intriguing consequences of changes in methylation levels on mRNA life-cycle. We also suggest that, since methylation is under the control of glutathione anti-oxidant levels (Lertratanangkoon et al., 1997), the redox status of

  9. Recent imaging advances in neurology.

    PubMed

    Rocchi, Lorenzo; Niccolini, Flavia; Politis, Marios

    2015-09-01

    Over the recent years, the application of neuroimaging techniques such as magnetic resonance imaging (MRI) and positron emission tomography (PET) has considerably advanced the understanding of complex neurological disorders. PET is a powerful molecular imaging tool, which investigates the distribution and binding of radiochemicals attached to biologically relevant molecules; as such, this technique is able to give information on biochemistry and metabolism of the brain in health and disease. MRI uses high intensity magnetic fields and radiofrequency pulses to provide structural and functional information on tissues and organs in intact or diseased individuals, including the evaluation of white matter integrity, grey matter thickness and brain perfusion. The aim of this article is to review the most recent advances in neuroimaging research in common neurological disorders such as movement disorders, dementia, epilepsy, traumatic brain injury and multiple sclerosis, and to evaluate their contribution in the diagnosis and management of patients. PMID:25808503

  10. [Present and future of neurology in Spain].

    PubMed

    Illa Sendra, I; García De Yébenes Prous, J; Ramo Tello, C; Polo Esteban, J M; Molinuevo Guix, J L; Robles Bayón, A; Mulas Delgado, F; Alvarez Sabín, J; Aguilar Barbera, M; Berciano Blanco JA, J A; Blesa González, R; Carnero Pardo, C; Castillo Sánchez, J; Del Ser Quijano, T; Ferrer Abizanda, I; García-Albea Ristol, E; Gómez Isla, T; Graus Ribas, F; Jiménez Hernández, M D; Liaño Martínez, H; Matías Guiu-Guia, J; Zarranz Imirizaldu, J J; Paradas López, C; Elena Martínez, G; Maltas Pérez, G; Ponce Rodríguez, M T

    2001-11-01

    This is a document prepared by the Spanish Society of Neurology (SEN), which was given to the President of Spain (Mr. José María Aznar) last September with the main aim of examining the current situation of Neurology in our country. It analyses the present and future of Neurology in clinical assistance, teaching and research. To prepare this document the criteria of patients' associations has been considered, including the Declaration of Madrid which has been subscribed by thirty of these associations. In spite of its relevant development in the previous decades, the current situation of Neurology in Spain is far from the ideal. To reach the recommendable menber of 3 or 4 neurologists per 100,000 inhabitants it is necessary to duplicate the present number of neurologists which has been estimated around 2/100,000; this situation is especially urgent in some Autonomous Communities. The most important problems in neurological assistance are: inadequate follow-up of the chronic outpatients, low numbers of neurological beds and of duties of Neurology, as well as of neurological case of patients with urgent neurological disorders. It is also necessary to increase the number of professors of Neurology to adequately cover pregraduate teaching; again there are important differences in teaching positions among Autonomous Communities. Neurology residence should be prolonged from 4 to 5 years. Finally, it is necessary to support the appearance of superespecialised units and to promote a coordinated research with other close specialities including basic neuroscience. PMID:11742621

  11. Neurologic diseases in HIV-infected patients.

    PubMed

    Bilgrami, Mohammed; O'Keefe, Paul

    2014-01-01

    Since the introduction of highly active antiretroviral therapy there has been an improvement in the quality of life for people with HIV infection. Despite the progress made, about 70% of HIV patients develop neurologic complications. These originate either in the central or the peripheral nervous system (Sacktor, 2002). These neurologic disorders are divided into primary and secondary disorders. The primary disorders result from the direct effects of the virus and include HIV-associated neurocognitive disorder (HAND), HIV-associated vacuolar myelopathy (VM), and distal symmetric polyneuropathy (DSP). Secondary disorders result from marked immunosuppression and include opportunistic infections and primary central nervous system lymphoma (PCNSL). A differential diagnosis which can be accomplished by detailed history, neurologic examination, and by having a good understanding of the role of HIV in various neurologic disorders will help physicians in approaching these problems. The focus of this chapter is to discuss neuropathogenesis of HIV, the various opportunistic infections, primary CNS lymphoma, neurosyphilis, CNS tuberculosis, HIV-associated peripheral neuropathies, HIV-associated neurocognitive disorder (HAND), and vacuolar myelopathy (VM). It also relies on the treatment recommendations and guidelines for the above mentioned neurologic disorders proposed by the US Centers for Disease Control and Prevention (CDC) and the Infectious Diseases Society of America. PMID:24365422

  12. Neurology and psychiatry in Babylon.

    PubMed

    Reynolds, Edward H; Wilson, James V Kinnier

    2014-09-01

    We here review Babylonian descriptions of neurological and psychiatric disorders, including epilepsy, stroke, psychoses, obsessive compulsive disorder, phobias, psychopathic behaviour, depression and anxiety. Most of these accounts date from the first Babylonian dynasty of the first half of the second millennium BC, within a millennium and a half of the origin of writing. The Babylonians were remarkably acute and objective observers of medical disorders and human behaviour. Their detailed descriptions are surprisingly similar to modern 19th and 20th century AD textbook accounts, with the exception of subjective thoughts and feelings which are more modern fields of enquiry. They had no knowledge of brain or psychological function. Some neuropsychiatric disorders, e.g. stroke or facial palsy, had a physical basis requiring the attention of a physician or asû, using a plant and mineral based pharmacology; some disorders such as epilepsy, psychoses, depression and anxiety were regarded as supernatural due to evil demons or spirits, or the anger of personal gods, and thus required the intervention of the priest or ašipu; other disorders such as obsessive compulsive disorder and psychopathic behaviour were regarded as a mystery. The Babylonians were the first to describe the clinical foundations of neurology and psychiatry. We discuss these accounts in relation to subsequent and more modern clinical descriptions. PMID:25037816

  13. Sports neurology topics in neurologic practice

    PubMed Central

    Conidi, Francis X.; Drogan, Oksana; Giza, Christopher C.; Kutcher, Jeffery S.; Alessi, Anthony G.; Crutchfield, Kevin E.

    2014-01-01

    Summary We sought to assess neurologists' interest in sports neurology and learn about their experience in treating sports-related neurologic conditions. A survey was sent to a random sample of American Academy of Neurology members. A majority of members (77%) see at least some patients with sports-related neurologic issues. Concussion is the most common sports-related condition neurologists treat. More than half of survey participants (63%) did not receive any formal or informal training in sports neurology. At least two-thirds of respondents think it is very important to address the following issues: developing evidence-based return-to-play guidelines, identifying risk factors for long-term cognitive-behavioral sequelae, and developing objective diagnostic criteria for concussion. Our findings provide an up-to-date view of the subspecialty of sports neurology and identify areas for future research. PMID:24790800

  14. Antisense Therapy in Neurology

    PubMed Central

    Lee, Joshua J.A.; Yokota, Toshifumi

    2013-01-01

    Antisense therapy is an approach to fighting diseases using short DNA-like molecules called antisense oligonucleotides. Recently, antisense therapy has emerged as an exciting and promising strategy for the treatment of various neurodegenerative and neuromuscular disorders. Previous and ongoing pre-clinical and clinical trials have provided encouraging early results. Spinal muscular atrophy (SMA), Huntington’s disease (HD), amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), Fukuyama congenital muscular dystrophy (FCMD), dysferlinopathy (including limb-girdle muscular dystrophy 2B; LGMD2B, Miyoshi myopathy; MM, and distal myopathy with anterior tibial onset; DMAT), and myotonic dystrophy (DM) are all reported to be promising targets for antisense therapy. This paper focuses on the current progress of antisense therapies in neurology. PMID:25562650

  15. Astrocytes: The missing link in neurological disease?

    PubMed Central

    Lin, Chia-Ching John; Deneen, Benjamin

    2013-01-01

    The central nervous system (CNS) is comprised of numerous cell types that work in concert to facilitate proper function and homeostasis. Disruption of these carefully orchestrated networks results in neuronal dysfunction, manifesting itself in a variety of neurological disorders. While neuronal dysregulation is causative of symptoms manifest in the clinic, the etiology of these disorders is often more complex than simply a loss of neurons or intrinsic dysregulation of their function. In the adult brain, astrocytes comprise the most abundant cell type and play key roles in CNS physiology, therefore it stands to reason that dysregulation of normal astrocyte function contributes to the etiology and progression of varied neurological disorders. We review here some neurological disorders associated with an astrocyte factor and discuss how the related astrocyte dysfunction contributes to the etiology and/or progression of these disorders. PMID:24365571

  16. Neurotrophic factors and neurologic disease.

    PubMed Central

    Holtzman, D M; Mobley, W C

    1994-01-01

    Discovered only 40 years ago, nerve growth factor is the prototypic neurotrophic factor. By binding to specific receptors on certain neurons in the peripheral nervous system and brain, nerve growth factor acts to enhance their survival, differentiation, and maintenance. In recent years, many additional neurotrophic factors have been discovered; some are structurally related to nerve growth factor while others are distinct from it. The robust actions of neurotrophic factors have suggested their use in preventing or lessening the dysfunction and death of neurons in neurologic disorders. We review the progress in defining neurotrophic factors and their receptors and in characterizing their actions. We also discuss some of the uses of neurotrophic factors in animal models of disease. Finally, we discuss how neurotrophic factors could be implicated in the pathogenesis of neurologic disorders. Images PMID:7975562

  17. Clinical neurology and executive dysfunction.

    PubMed

    Filley, C M

    2000-01-01

    Executive function is a uniquely human ability that permits an individual to plan, carry out, and monitor a sequence of actions that is intended to accomplish a goal. This crucial neurobehavioral capacity depends on the integrity of the frontal lobes, most importantly the dorsolateral prefrontal cortices and their connections. Executive dysfunction is associated with a wide range of neurologic disorders that affect these regions. In this paper, executive dysfunction is considered from the perspective of behavioral neurology, and the lesion method is employed to illustrate this impairment in a diverse group of disorders. Frontal system damage leading to disturbed executive function is common and clinically significant. Recognition of this syndrome is critical for ensuring the correct diagnosis, accurate prognosis, and appropriate treatment of affected patients. Executive dysfunction also represents an intriguing aspect of brain-behavior relationships and offers important insights into one of the highest cerebral functions. PMID:10879543

  18. Neurological diseases in famous painters.

    PubMed

    Piechowski-Jozwiak, Bartlomiej; Bogousslavsky, Julien

    2013-01-01

    Visual art production involves multiple processes including basic motor skills, such as coordination of movements, visual-spatial processing, emotional output, sociocultural context, and creativity. Thus, the relationship between artistic output and brain diseases is particularly complex, and brain disorders may lead to impairment of artistic production in multiple domains. Neurological conditions may also occasionally modify artistic style and lead to surprisingly innovative features in people with an initial loss of creativity. This chapter focuses on anecdotal reports of various neurological disorders and their potential consequences on works produced by famous or well-established artists, including Carl Frederik Reutersward, Giorgio de Chirico, Krystyna Habura, Leo Schnug, Ignatius Brennan, and many others. PMID:24041285

  19. Neurology and neurologic practice in China

    PubMed Central

    2011-01-01

    In the wake of dramatic economic success during the past 2 decades, the specialized field of neurology has undergone a significant transformation in China. With an increase in life expectancy, the problems of aging and cognition have grown. Lifestyle alterations have been associated with an epidemiologic transition both in the incidence and etiology of stroke. These changes, together with an array of social issues and institution of health care reform, are creating challenges for practicing neurologists throughout China. Notable problems include overcrowded, decrepit facilities, overloaded physician schedules, deteriorating physician-patient relationships, and an insufficient infrastructure to accommodate patients who need specialized neurologic care. Conversely, with the creation of large and sophisticated neurology centers in many cities across the country, tremendous opportunities exist. Developments in neurologic subspecialties enable delivery of high-quality care. Clinical and translational research based on large patient populations as well as highly sophisticated technologies are emerging in many neurologic centers and pharmaceutical companies. Child neurology and neurorehabilitation will be fast-developing subdisciplines. Given China's extensive population, the growth and progress of its neurology complex, and its ever-improving quality control, it is reasonable to anticipate that Chinese neurologists will contribute notably to unraveling the pathogenic factors causing neurologic diseases and to providing new therapeutic solutions. PMID:22123780

  20. Learning Disorders

    MedlinePlus

    ... new information. People with learning disorders may have problems Listening or paying attention Speaking Reading or writing ... around them can make them less of a problem. NIH: National Institute of Neurological Disorders and Stroke