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Sample records for juvenile neuronal ceroid

  1. Vision loss in juvenile neuronal ceroid lipofuscinosis (CLN3 disease).

    PubMed

    Ouseph, Madhu M; Kleinman, Mark E; Wang, Qing Jun

    2016-05-01

    Juvenile neuronal ceroid lipofuscinosis (JNCL; also known as CLN3 disease) is a devastating neurodegenerative lysosomal storage disorder and the most common form of Batten disease. Progressive visual and neurological symptoms lead to mortality in patients by the third decade. Although ceroid-lipofuscinosis, neuronal 3 (CLN3) has been identified as the sole disease gene, the biochemical and cellular bases of JNCL and the functions of CLN3 are yet to be fully understood. As severe ocular pathologies manifest early in disease progression, the retina is an ideal tissue to study in the efforts to unravel disease etiology and design therapeutics. There are significant discrepancies in the ocular phenotypes between human JNCL and existing murine models, impeding investigations on the sequence of events occurring during the progression of vision impairment. This review focuses on current understanding of vision loss in JNCL and discusses future research directions toward molecular dissection of the pathogenesis of the disease and associated vision problems in order to ultimately improve the quality of patient life and cure the disease.

  2. Psychiatric Symptoms of Children and Adolescents with Juvenile Neuronal Ceroid Lipofuscinosis

    ERIC Educational Resources Information Center

    Backman, M. L.; Santavuori, P. R.; Aberg, L. E.; Aronen, E. T.

    2005-01-01

    Juvenile neuronal ceroid lipofuscinosis (JNCL) is one of the most common neurodegenerative disorders in childhood and adolescence. The clinical picture includes diverse and complex psychiatric symptoms that are difficult to treat. Only symptomatic treatment is available. To improve symptomatic therapy, it is important to recognize the symptoms.…

  3. Late onset juvenile neuronal ceroid-lipofuscinosis with granular osmiophilic deposits (GROD)

    SciTech Connect

    Hofman, I.L.; Taschner, P.E.M.

    1995-06-05

    The juvenile-onset subtype of the neuronal ceroid lipofuscinoses (JNCL) is well known and ultra-structurally characterized by fingerprints and/or curvilinear bodies in many cell types. Linkage studies indicated a most likely location for CLN3, the gene involved in JNCL, in the interval between loci D16S297 and D16S57, within close proximity of the loci D16S298 and D16S299. We present two sibs with a late onset progressive disease of mental deterioration, progressive macular degeneration, motor disturbances, and epilepsy. Histological symptoms of neuronal ceroid lipofuscinosis and ultrastructural granular osmiophilic deposits (GROD) in lymphocytes and neurons are found. Individual haplotypes at polymorphic marker loci on chromosome 16 were constructed to determine whether JNCL with GROD is linked to the CLN3 locus. 8 refs., 3 figs.

  4. Standardized assessment of seizures in patients with juvenile neuronal ceroid lipofuscinosis

    PubMed Central

    Augustine, Erika F; Adams, Heather R; Beck, Christopher A; Vierhile, Amy; Kwon, Jennifer; Rothberg, Paul G; Marshall, Frederick; Block, Robert; Dolan, James; Mink, Jonathan W

    2015-01-01

    AIM To evaluate seizure phenomenology, treatment, and course in individuals with juvenile neuronal ceroid lipofuscinosis (JNCL). METHOD Data from an ongoing natural history study of JNCL were analyzed using cross-sectional and longitudinal methods. Seizures were evaluated with the Unified Batten Disease Rating Scale, a disease-specific quantitative assessment tool. RESULTS Eighty-six children (44 males, 42 females) with JNCL were assessed at an average of three annual visits (range 1–11y). Eighty-six percent (n=74) experienced at least one seizure, most commonly generalized tonic-clonic, with mean age at onset of 9 years 7 months (SD 2y 10mo). Seizures were infrequent, typically occurring less often than once every 3 months, and were managed with one to two medications for most participants. Valproate (49%, n=36) and levetiracetam (41%, n=30) were the most commonly used seizure medications. Myoclonic seizures occurred infrequently (16%, n=14). Seizure severity did not vary by sex or genotype. Seizures showed mild worsening with increasing age. INTERPRETATION The neuronal ceroid lipofuscinoses (NCLs) represent a group of disorders unified by neurodegeneration and symptoms of blindness, seizures, motor impairment, and dementia. While NCLs are considered in the differential diagnosis of progressive myoclonus epilepsy, we show that myoclonic seizures are infrequent in JNCL. This highlights the NCLs as consisting of genetically distinct disorders with differing natural history. PMID:25387857

  5. Chromosome 16 microdeletion in a patient with juvenile neuronal ceroid lipofuscinosis (Batten disease)

    SciTech Connect

    Taschner, P.E.M.; Vos, N. de; Thompson, A.D.; Callen, D.F.; Doggett, N.; Mole, S.E.; Dooley, T.P.; Barth, P.G.; Breuning, M.H. |

    1995-03-01

    The gene that is involved in juvenile neuronal ceroid lipofuscinosis (JNCL), or Batten disease - CLN3 - has been localized to 16p12, and the mutation shows a strong association with alleles of microsatellite markers D16S298, D16S299, and D16S288. Recently, haplotype analysis of a Batten patient from a consanguineous relationship indicated homozygosity for a D16S298 null allele. PCR analysis with different primers on DNA from the patient and his family suggests the presence of a cytogenetically undetectable deletion, which was confirmed by Southern blot analysis. The microdeletion is embedded in a region containing chromosome 16-specific repeated sequences. However, putative candidates for CLN3, members of the highly homologous sulfotransferase gene family, which are also present in this region in several copies, were not deleted in the patient. If the microdeletion in this patient is responsible for Batten disease, then we conclude that the sulfotransferase genes are probably not involved in JNCL. By use of markers and probes flanking D15S298, the maximum size of the microdeletion was determined to be {approximately}29 kb. The microdeletion may affect the CLN3 gene, which is expected to be in close proximity to D16S298. 27 refs., 6 figs.

  6. The Neuronal Ceroid-Lipofuscinoses

    ERIC Educational Resources Information Center

    Bennett, Michael J.; Rakheja, Dinesh

    2013-01-01

    The neuronal ceroid-lipofuscinoses (NCL's, Batten disease) represent a group of severe neurodegenerative diseases, which mostly present in childhood. The phenotypes are similar and include visual loss, seizures, loss of motor and cognitive function, and early death. At autopsy, there is massive neuronal loss with characteristic storage in…

  7. Linkage disequilibrium between the juvenile neuronal ceroid lipofuscinosis gene and marker loci on chromosome 16p12. 1

    SciTech Connect

    Lerner, T.J.; MacCormack, K.; Gleitsman, J.; Schlumpf, K.; Breakefield, X.O.; Gusella, J.F.; Haines, J.L. )

    1994-01-01

    The neuronal ceroid lipofuscinoses (NCL; Batten disease) are a collection of autosomal recessive disorders characterized by the accumulation of autofluorescent lipopigments in the neurons and other cell types. Clinically, these disorders are characterized by progressive encephalopathy, loss of vision, and seizures. CLN3, the gene responsible for juvenile NCL, has been mapped to a 15-cM region flanked by the marker loci D16S148 and D16S150 on human chromosome 16. CLN2, the gene causing the late-infantile form of NCL (LNCL), is not yet mapped. The authors have used highly informative dinucleoide repeat markers mapping between D16S148 and D16S150 to refine the localization of CLN3 and to test for linkage to CLN2. The authors find significant linkage disequilibrium between CLN3 and the dinucleotide repeat marker loci D16S288 (X[sup 2](7) = 46.5, P < .005), D16S298 (X[sup 2](6) = 36.6, P < .005), and D16S299 (X[sup 2](7) = 73.8, P < .005), and also a novel RFLP marker at the D16S272 locus (X[sup 2](1) = 5.7, P = .02). These markers all map to 16p12.1. The D16S298/D16S299 haplotype [open quotes]5/4[close quotes] is highly overrepresented, accounting for 54% of CLN3 chromosomes as compared with 8% of control chromosomes (X[sup 2] = 117, df = 1, P < .001). Examination of the haplotypes suggests that the CLN3 locus can be narrowed to the region immediately surrounding these markers in 16p12.1. Analysis of D16S299 in LNCL pedigrees supports the previous finding that CLN3 and CLN2 are different genetic loci. This study also indicates that dinucleotide repeat markers play a valuable role in disequilibrium studies. 23 refs., 1 fig., 4 tabs.

  8. Neuronal Ceroid Lipofuscinosis (Batten's Disease)

    PubMed Central

    Gordon, N. S.; Marsden, H. B.; Noronha, M. J.

    1972-01-01

    Four patients are described, who on clinical, histological, and biochemical criteria are considered to be suffering from neuronal ceroid lipofuscinosis. It is suggested that this may be the commonest condition included under the term amaurotic family idiocy. A number of gangliosidoses can be classified on a biochemical basis and considerable advances have been made in identifying the enzyme deficiencies. The aetiology of neuronal ceroid lipofuscinosis is unknown, and it is possible that there is more than one cause. Visual symptoms and signs are not always present. Though generalized convulsions occur at the start of the illness, myoclonus tends increasingly to dominate the clinical picture. An abnormal sensitivity to photic stimulation at a very slow frequency is a suggestive finding. Evidence of cerebral atrophy on air-encephalography favours this diagnosis, as the brain tends to be enlarged in the gangliosidoses. A definite diagnosis can only be made in life by examination of a cortical biopsy. Biochemical analysis will show a normal ganglioside pattern, and histological examination by light and electron microscopy will reveal characteristic changes. An age dependent classification of amaurotic family idiocy is no longer justifiable, and if full investigations are carried out, an increasing number of these patients can be diagnosed as suffering from a specific type of disorder. ImagesFIG. 1FIG. 2 PMID:5023478

  9. Genetics Home Reference: infantile neuronal ceroid lipofuscinosis

    MedlinePlus

    ... Batten Disease Foundation CLIMB: Children Living with Inherited Metabolic Diseases ... Sources for This Page Getty AL, Pearce DA. Interactions of the proteins of neuronal ceroid lipofuscinosis: clues to function. Cell ...

  10. Molecular genetics of neuronal ceroid lipofuscinoses.

    PubMed

    Järvelä, I; Vesa, J; Santavuori, P; Hellsten, E; Peltonen, L

    1992-12-01

    This overview describes recent advances in molecular biology of neuronal ceroid lipofuscinoses (CLN). Despite intensive research during last 20 years, the basic defects of these autosomal recessive-progressive encephalopathies of childhood remain unknown. Consequently, no specific cure is available. Methods of positional cloning (reverse genetics) starting from random linkage approach have been applied to search for gene defects in the infantile and juvenile forms of the disease. The results of this random search for disease loci have for the first time revealed molecular heterogeneity of CLN diseases. The gene defect causing the infantile form has been assigned to 1p32 in the Finnish family material, whereas the disease locus of the juvenile form has been localized to 16p12 in European and Canadian families. Finally, the gene defect causing the late infantile form has been excluded from both 1p32 and 16p12 chromosomal regions, referring to a third, still unknown locus causing CLN disease. Consequently, reliable prenatal and carrier diagnostics have now become possible in families with the infantile and juvenile forms of the disease, and DNA-based prenatal diagnostics have been successfully applied in the infantile form. Most importantly, the assignment of gene loci has brought these fatal brain diseases within the reach of molecular cloning strategies that eventually will result in revealing both the infantile and juvenile CLN genes and in identifying corresponding gene products. PMID:1287553

  11. Genetics Home Reference: congenital neuronal ceroid lipofuscinosis

    MedlinePlus

    ... AE, Tyynelä J. Cathepsin D deficiency underlies congenital human neuronal ceroid-lipofuscinosis. Brain. 2006 Jun;129(Pt 6):1438-45. Epub 2006 May 2. Citation on PubMed Steinfeld ... deficiency is associated with a human neurodegenerative disorder. Am J Hum Genet. 2006 Jun; ...

  12. Neuronal ceroid lipofuscinosis (Batten disease) in Newfoundland

    SciTech Connect

    Frecker, M.F.; Jacob, J.C.; Ives, E.J.

    1994-09-01

    The neuronal ceroid lipofuscinoses (NCL) are a group of recessively inherited neurodegenerative disorders. The most common type found in Newfoundland is late infantile NCL; 1 in 63 of the population is estimated to be a carrier. The incidence has decreased over the years with fewer affected siblings born in families and migration away from smaller communities. For the 30 late infantile cases (24 families), most presented with generalized convulsive seizures; all had curvilinear inclusion bodies in several cell types. The mean age at onset was 2.8 {plus_minus} 0.6 yr and they lived a mean of 7.5 {plus_minus} 2.1 yr. Three of these families have been used to exclude linkage to markers on chromosome 1 and 16. There is one typical juvenile NCL family; it has been used to further define the localization of the gene on chromosome 16p. Genetic counseling for carrier status has been offered in this family. A single case of adult NCL (Kufs) has been identified. Reevaluation of the cases indicated that there are many who have clinical and neuropathological features of both late infantile and juvenile NCL. Unusual findings in these 13 cases (11 families) included the coexistence of both types of inclusion bodies. In patients with juvenile fingerprint inclusion bodies, atypical presentations were delayed milestones at age 8 mo, visual loss before 4 yr, psychomotor retardation before loss of vision and an enhanced response to photic stimulation on EEG. Molecular studies will determine the basis for this heterogeneity. Extensive family histories showed only 53% (19/36) of families were consanguinous or interelated indicating that the frequency of the gene is probably quite high.

  13. Childhood neuronal ceroid-lipofuscinoses in Argentina.

    PubMed

    Taratuto, A L; Saccoliti, M; Sevlever, G; Ruggieri, V; Arroyo, H; Herrero, M; Massaro, M; Fejerman, N

    1995-06-01

    We report on 30 cases of neuronal ceroid lipofuscinoses (NCL), mainly diagnosed in 1985-1993 in Argentina, whose population is predominantly of European descent. Twenty-four cases were late infantile Jansky-Bielschowsky (LINCL) and 6 were juvenile Spielmeyer-Vogt (JNCL). Sex ratio was female:male, 20:10. Age range and mean at onset and at diagnosis for the LINCL cases were 1-6 years, mean 3.1, and 2-11 years, mean 5.5, and for the JNCL cases, 5-9 years, mean 7, and 9-18 years, mean 13, respectively. Cases were referred for biopsy after neurological examination, and most included complete electrophysiological [electroencephalography (EEG) with photic stimulation, electroretinography (ERG), and visual-evoked potential (VEP)], neuroimaging, and neurometabolic investigation. NCL was the first suspected clinical diagnosis, followed by mitochondrial encephalopathy in some cases of recent onset. Except for 1 case, clinical findings were homogeneous in LINCL, characterized by refractive epilepsy, mental regression and progressive deterioration, ataxia, myoclonia, and visual loss. Abnormal VEP, ERG, and EEG, with polyphasic high-voltage spikes when photic stimulation was performed at low frequency, were observed. Visual impairment and retinitis pigmentosa were early manifestations in 4/6 JNCL, followed by mental abnormalities, motor deterioration, and myoclonic jerks, while 2/4 followed an atypical course. In both variants inheritance was autosomal-recessive. Five out of 27 families had more than 1 affected member, 3 of whom were included in our series. Diagnosis was initially performed in conjunctival biopsy in 3 cases, skin in 5, muscle in 17, and brain in 5, though most cases had a concomitant biopsy from another tissue including nerve, and there was a single brain autopsy.(ABSTRACT TRUNCATED AT 250 WORDS)

  14. Moving towards effective therapeutic strategies for Neuronal Ceroid Lipofuscinosis.

    PubMed

    Geraets, Ryan D; Koh, Seung yon; Hastings, Michelle L; Kielian, Tammy; Pearce, David A; Weimer, Jill M

    2016-01-01

    The Neuronal Ceroid Lipofuscinoses (NCLs) are a family of autosomal recessive neurodegenerative disorders that annually affect 1:100,000 live births worldwide. This family of diseases results from mutations in one of 14 different genes that share common clinical and pathological etiologies. Clinically, the diseases are subcategorized into infantile, late-infantile, juvenile and adult forms based on their age of onset. Though the disease phenotypes may vary in their age and order of presentation, all typically include progressive visual deterioration and blindness, cognitive impairment, motor deficits and seizures. Pathological hallmarks of NCLs include the accumulation of storage material or ceroid in the lysosome, progressive neuronal degeneration and massive glial activation. Advances have been made in genetic diagnosis and counseling for families. However, comprehensive treatment programs that delay or halt disease progression have been elusive. Current disease management is primarily targeted at controlling the symptoms rather than "curing" the disease. Recognizing the growing need for transparency and synergistic efforts to move the field forward, this review will provide an overview of the therapeutic approaches currently being pursued in preclinical and clinical trials to treat different forms of NCL as well as provide insight to novel therapeutic approaches in development for the NCLs. PMID:27083890

  15. Remote Assessment of Cognitive Function in Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease): A Pilot Study of Feasibility and Reliability.

    PubMed

    Ragbeer, Shayne N; Augustine, Erika F; Mink, Jonathan W; Thatcher, Alyssa R; Vierhile, Amy E; Adams, Heather R

    2016-03-01

    Remote technology provides an opportunity to extend the reach of clinical care and research for pediatric rare disease. This pilot study evaluated the feasibility and reliability of neuropsychological evaluation, using remote audiovisual technology, in the assessment of children with juvenile Batten disease. Three children with Batten disease and 1 healthy sibling completed a standardized cognitive assessment. Results indicated high agreement between an in-person and a remote evaluator when comparing the subjects' cognitive test scores. This initial test of remote cognitive assessment suggests it is feasible and reliable in children with pediatric neurodegenerative disease, for whom disease burden may limit travel and access to expert care and/or clinical trials.

  16. Partial Correction of the CNS Lysosomal Storage Defect in a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis by Neonatal CNS Administration of an Adeno-Associated Virus Serotype rh.10 Vector Expressing the Human CLN3 Gene

    PubMed Central

    Sondhi, Dolan; Scott, Emma C.; Chen, Alvin; Hackett, Neil R.; Wong, Andrew M.S.; Kubiak, Agnieszka; Nelvagal, Hemanth R.; Pearse, Yewande; Cotman, Susan L.; Cooper, Jonathan D.

    2014-01-01

    Abstract Juvenile neuronal ceroid lipofuscinosis (JNCL or CLN3 disease) is an autosomal recessive lysosomal storage disease resulting from mutations in the CLN3 gene that encodes a lysosomal membrane protein. The disease primarily affects the brain with widespread intralysosomal accumulation of autofluorescent material and fibrillary gliosis, as well as the loss of specific neuronal populations. As an experimental treatment for the CNS manifestations of JNCL, we have developed a serotype rh.10 adeno-associated virus vector expressing the human CLN3 cDNA (AAVrh.10hCLN3). We hypothesized that administration of AAVrh.10hCLN3 to the Cln3Δex7/8 knock-in mouse model of JNCL would reverse the lysosomal storage defect, as well as have a therapeutic effect on gliosis and neuron loss. Newborn Cln3Δex7/8 mice were administered 3×1010 genome copies of AAVrh.10hCLN3 to the brain, with control groups including untreated Cln3Δex7/8 mice and wild-type littermate mice. After 18 months, CLN3 transgene expression was detected in various locations throughout the brain, particularly in the hippocampus and deep anterior cortical regions. Changes in the CNS neuronal lysosomal accumulation of storage material were assessed by immunodetection of subunit C of ATP synthase, luxol fast blue staining, and periodic acid-Schiff staining. For all parameters, Cln3Δex7/8 mice exhibited abnormal lysosomal accumulation, but AAVrh.10hCLN3 administration resulted in significant reductions in storage material burden. There was also a significant decrease in gliosis in AAVrh.10hCLN3-treated Cln3Δex7/8 mice, and a trend toward improved neuron counts, compared with their untreated counterparts. These data demonstrate that AAVrh.10 delivery of a wild-type cDNA to the CNS is not harmful and instead provides a partial correction of the neurological lysosomal storage defect of a disease caused by a lysosomal membrane protein, indicating that this may be an effective therapeutic strategy for JNCL and

  17. Application of chromosome 16 markers in the differential diagnosis of neuronal ceroid-lipofuscinosis

    SciTech Connect

    Taschner, P.E.M.; Vos, N. de; Breuning, M.H.

    1995-06-05

    Accurate diagnosis of neuronal ceroid lipofuscinosis (NCL) is important for a correct prognosis of the disease and for genetic counseling. Up to now, no direct diagnostic test has been available for NCL. The clinical diagnosis is made on the basis of symptoms, neurophysiological, neuroradiological, and specific lipopigment pattern data. Recent advances in the genetics of NCL have enabled us to use polymorphic DNA markers linked to the CLN1 and CLN3 loci as a tool in the differential diagnosis of NCL. We have applied genetic analysis with polymorphic DNA markers flanking the CLN3 gene on chromosome 16 to two consanguineous families in which NCL occurs. In the first family, which is of Turkish extraction, two patients suffering from a protracted form of juvenile NCL previously had been diagnosed with juvenile NCL. Haplotypes from this family indicate that the patients and their healthy sibling are haplo-identical, suggesting that this protracted form of juvenile NCL is not linked to the CLN3 locus. In the second family, which is Moroccan origin, one patient suffers from the early juvenile variant of NCL (Lake-Cavanagh). In this family, the patient and one of the healthy siblings have identical haplotypes, excluding linkage of early juvenile NCL to the CLN3 locus on 16p12.1-11.2. Therefore, these cases from different populations demonstrate that haplotype analysis can be used as an additional method to exclude the diagnosis of juvenile NCL. 21 refs., 4 figs., 1 tab.

  18. [Adult neuronal ceroid lipofuscinosis (Kufs disease)--a rare cause of dementia].

    PubMed

    Kozian, R; Kiszka, T; Peter, K

    1994-11-01

    Kufs' disease is a very rare type of neuronal ceroid lipofuscinosis. A case of a 52 year old man with dementia is described. The cause for patient's dementia was the adult type (Kufs' disease) of neuronal ceroid lipofuscinosis. The diagnosis based on the histopathological post mortem-examination of the brain-tissue. A brother of our patient became ill with the same symptoms and at the same age of onset. So we conclude that there is a accumulation in the family.

  19. Characterization of neuronal ceroid-lipofuscinosis in 3 cats.

    PubMed

    Chalkley, M D; Armien, A G; Gilliam, D H; Johnson, G S; Zeng, R; Wünschmann, A; Kovi, R C; Katz, M L

    2014-07-01

    Three young domestic shorthair cats were presented for necropsy with similar histories of slowly progressive visual dysfunction and neurologic deficits. Macroscopic examination of each cat revealed cerebral and cerebellar atrophy, dilated lateral ventricles, and slight brown discoloration of the gray matter. Histologically, there was bilateral loss of neurons within the limbic, motor, somatosensory, visual, and, to a lesser extent, vestibular systems with extensive astrogliosis in the affected regions of all 3 cases. Many remaining neurons and glial cells throughout the entire central nervous system were distended by pale yellow to eosinophilic, autofluorescent cytoplasmic inclusions with ultrastructural appearances typical of neuronal ceroid-lipofuscinoses (NCLs). Differences in clinical presentation and neurological lesions suggest that the 3 cats may have had different variants of NCL. Molecular genetic characterization in the 1 cat from which DNA was available did not reveal any plausible disease-causing mutations of the CLN1 (PPT1), CLN3, CLN5, CLN8, and CLN10 (CTSD) genes. Further investigations will be required to identify the mutations responsible for NCLs in cats. PMID:24026940

  20. Therapeutic Approaches to the Challenge of Neuronal Ceroid Lipofuscinoses

    PubMed Central

    Kohan, R.; Cismondi, I.A.; Oller-Ramirez, A.M.; Guelbert, N.; Anzolini, V. Tapia; Alonso, G.; Mole, S.E.; de Kremer, R. Dodelson; de Halac, I. Noher

    2013-01-01

    The Neuronal Ceroid Lipofuscinoses (NCLs) are lysosomal storage diseases (LSDs) affecting the central nervous system (CNS), with generally with recessive inheritance. They are characterized by pathological lipofuscin-like material accumulating in cells. The clinical phenotypes at all onset ages show progressive loss of vision, decreasing cognitive and motor skills, epileptic seizures and premature death, with dementia without visual loss prominent in the rarer adult forms. Eight causal genes, CLN10/CTSD, CLN1/PPT1, CLN2/TPP1, CLN3, CLN5, CLN6, CLN7/MFSD8, CLN8, with more than 269 mutations and 49 polymorphisms (http://www.ucl.ac.uk/ncl) have been described. Other NCL genes are hypothesized, including CLN4 and CLN9; CLCN6, CLCN7 and possibly SGSH are under study. Some therapeutic strategies applied to other LSDs with significant systemic involvement would not be effective in NCLs due to the necessity of passing the blood brain barrier to prevent the neurodegeneration, repair or restore the CNS functionality. There are therapies for the NCLs currently at preclinical stages and under phase 1 trials to establish safety in affected children. These approaches involve enzyme replacement, gene therapy, neural stem cell replacement, immune therapy and other pharmacological approaches. In the next decade, progress in the understanding of the natural history and the biochemical and molecular cascade of events relevant to the pathogenesis of these diseases in humans and animal models will be required to achieve significant therapeutic advances. PMID:21235444

  1. Astrocytosis in infantile neuronal ceroid lipofuscinosis: friend or foe?

    PubMed

    Shyng, Charles; Sands, Mark S

    2014-10-01

    Infantile neuronal ceroid lipofuscinosis (INCL; infantile Batten disease) is an inherited paediatric neurodegenerative disease. INCL is caused by a deficiency in the lysosomal enzyme palmitoyl-protein thioesterase-1 (PPT1) and is thus classified as a lysosomal storage disease. Pathological examination of both human and murine INCL brains reveals progressive, widespread neuroinflammation. In fact, astrocyte activation appears to be the first histological sign of disease. However, the role of astrocytosis in INCL was poorly understood. The hallmark of astrocyte activation is the up-regulation of intermediate filaments, such as glial fibrillary acidic protein (GFAP) and vimentin. The role of astrocytosis in INCL was studied in a murine model lacking PPT1 and the intermediate filaments GFAP and vimentin (triple-knockout). This murine model of INCL with attenuated astrocytosis had an exacerbated pathological and clinical phenotype. The triple-knockout mouse had a significantly shortened lifespan, and accelerated cellular and humoural neuroinflammatory response compared with the parental PPT1(-/-) mouse. The data obtained from the triple-knockout mouse strongly suggest that astrocyte activation plays a beneficial role in early INCL disease progression. A more thorough understanding of the glial responses to lysosomal enzyme deficiencies and the accumulation of undergraded substrates will be crucial to developing effective therapeutics.

  2. A novel mutation af Cln3 associated with delayed-classic juvenile ceroid lipofuscinois and autophagic vacuolar myopathy.

    PubMed

    Licchetta, L; Bisulli, F; Fietz, M; Valentino, M L; Morbin, M; Mostacci, B; Oliver, K L; Berkovic, S F; Tinuper, P

    2015-10-01

    Juvenile neuronal-ceroid-lipofuscinosis (JNCL) is a lysosomal storage disease caused by mutations in CLN3. The most frequent mutation is a 1.02-kb deletion that, when homozygous, causes the classical clinical presentation. Patients harboring mutations different than the major deletion show a marked clinical heterogeneity, including protracted disease course with possible involvement of extraneuronal tissues. Cardiac involvement is relatively rare in JNCL and it is usually due to myocardial storage of ceroid-lipofuscinin. Only recently, histopathological findings of autophagic vacuolar myopathy (AVM) were detected in JNCL patients with severe cardiomyopathy. We describe a 35-year-old male showing a delayed-classic JNCL with visual loss in childhood and neurological manifestations only appearing in adult life. He had an unusual CLN3 genotype with an unreported deletion (p.Ala349_Leu350del) and the known p.His315Glnfs*67 mutation. Autophagic vacuolar myopathy was shown by muscle biopsy. At clinical follow-up, moderately increased CPK levels were detected whereas periodic cardiac assessments have been normal to date. Adult neurologists should be aware of protracted JNCL as cause of progressive neurological decline in adults. The occurrence of autophagic vacuolar myopathy necessitates periodic cardiac surveillance, which is not usually an issue in classic JNCL due to early neurological death.

  3. Evidence for processing of dolichol-linked oligosaccharides in patients with neuronal ceroid-lipofuscinosis.

    PubMed

    Daniel, P F; Sauls, D L; Boustany, R M

    1992-02-15

    In agreement with reports from other laboratories, we have shown that patients with the juvenile or late infantile forms of neuronal ceroid-lipofuscinosis (NCL) have greatly increased levels (5-fold to 20-fold) of dolichyl pyrophosphoryl oligosaccharides in their cerebral gray matter. Oligosaccharides containing 2 GlcNAc residues and 3 to 9 mannose residues were liberated by mild acid hydrolysis. The oligosaccharide profile given by brain tissue from 2 patients with infantile NCL was markedly different from that of late infantile and juvenile NCL brain, with Man9GlcNAc2 as the most abundant component and decreasing amounts of Man8- Man7- and Man6GlcNAc2. By contrast, Man5GlcNAc2 was the most abundant oligosaccharide present in all juvenile NCL brain samples analyzed. Both the susceptibility of the isolated Man5GlcNAc2 to endoglucosaminidase H digestion and permethylation analysis clearly indicated that it is not an intermediate in the biosynthesis of Glc3Man9GlcNAc2-PP-dolichol but has undergone catabolism, probably either in the endoplasmic reticulum or in the Golgi apparatus. Treatment of cultured skin fibroblasts for 7 days with N-methyldeoxynojirimycin, a potent inhibitor of the endoplasmic reticulum processing enzymes glucosidase I and II, resulted in an accumulation of the same Man5GlcNAc2-PP-dolichol species that was elevated in juvenile NCL brain. The level in untreated fibroblasts was undetectable, suggesting that inhibition of processing glucosidases has interfered with the regulation and compartmentalization of lipid-linked oligosaccharides. PMID:1609840

  4. CLN2 Disease (Classic Late Infantile Neuronal Ceroid Lipofuscinosis).

    PubMed

    Kohlschütter, Alfried; Schulz, Angela

    2016-06-01

    CLN2 disease is an inherited metabolic storage disorder caused by the deficiency of the lysosomal enzyme tripeptidyl peptidase 1 (TPP1). The disease affects mainly the brain and the retina and is characterized by progressive dysfunction of the central nervous system, leading to dementia, epilepsy, loss of motor function and blindness. The classical late infantile type begins at around three years of age with epilepsy and/or a standstill of psychomotor development, followed by a rapid loss of all abilities and death in childhood. A late onset form in a small proportion of patients starts at the age of 4 to 10 years, but also leads to severe neurological deterioration. The deficiency of TPP1 causes the lysosomal accumulation of a material called ceroid lipofuscin. The natural substrate of TPP1 is not known, nor is the connection between storage process and neurodegeneration, which is characterized by loss of neurons. Among various experimental approaches to treatment, enzyme replacement therapy (ERT) and gene therapy have developed remarkably. Enzyme delivery through the cerebrospinal fluid led to wide distribution of enzyme activity in the brain and to attenuated neuropathology and disease progression in a TPP1-deficient mouse model as well as in a natural TPP1-deficient dog model. Safety of the intrathecal delivery, pharmacokinetics, and tissue distribution of the administered enzyme studied in non-human primates were encouraging, and a phase I/II clinical trial for intraventricular ERT in CLN2 patients is ongoing. A second approach uses intracerebral injection of viral vectors containing normal coding segments of the CLN2 gene. In a CLN2 mouse model, this procedure resulted in cerebral enzyme expression, reduced brain pathology and increased survival. A small number of patients have been treated the same way using an AAV2-vector for gene transfer to the brain. Although there were no serious adverse events unequivocally attributable to the vector used, there were

  5. Multifocal retinopathy in Dachshunds with CLN2 neuronal ceroid lipofuscinosis.

    PubMed

    Whiting, Rebecca E H; Pearce, Jacqueline W; Castaner, Leilani J; Jensen, Cheryl A; Katz, Rebecca J; Gilliam, Douglas H; Katz, Martin L

    2015-05-01

    The CLN2 form of neuronal ceroid lipofuscinosis is an autosomal recessively inherited lysosomal storage disease that is characterized by progressive vision loss culminating in blindness, cognitive and motor decline, neurodegeneration, and premature death. CLN2 disease results from mutations in the gene that encodes the soluble lysosomal enzyme tripeptidyl peptidase-1. A null mutation in the TPP1 gene encoding this enzyme causes a CLN2-like disease in Dachshunds. Dachshunds that are homozygous for this mutation serve as a model for human CLN2 disease, exhibiting clinical signs and neuropathology similar to those of children with this disorder. Affected dogs reach end-stage terminal disease status at 10-11 months of age. In addition to retinal changes typical of CLN2 disease, a retinopathy consisting of multifocal, bullous retinal detachment lesions was identified in 65% of (TPP1-/-) dogs in an established research colony. These lesions did not occur in littermates that were heterozygous or homozygous for the normal TPP1 allele. Retinal changes and the functional effects of this multifocal retinopathy were examined objectively over time using ophthalmic examinations, fundus photography, electroretinography (ERG), quantitative pupillary light response (PLR) recording, fluorescein angiography, optical coherence tomography (OCT) and histopathology. The retinopathy consisted of progressive multifocal serous retinal detachments. The severity of the disease-related retinal thinning was no more serious in most detached areas than in adjacent areas of the retina that remained in close apposition to the retinal pigment epithelium. The retinopathy observed in these dogs was somewhat similar to canine multifocal retinopathy (CMR), a disease caused by a mutation of the bestrophin gene BEST1. ERG a-wave amplitudes were relatively preserved in the Dachshunds with CLN2 disease, whether or not they developed the multifocal retinopathy. The retinopathy also had minimal effects on the

  6. Canine ceroid lipofuscinosis, a model for ageing of the human isocortex.

    PubMed

    Braak, H; Braak, E; Strenge, H; Koppang, N

    1984-01-01

    In canine ceroid lipofuscinosis (one case studied), isocortical layer IIIab pyramidal cells develop spindle-shaped enlargements of their proximal axon filled with lipopigment, a feature that can be observed in juvenile and adult type of human neuronal ceroid lipofuscinosis and in normal ageing of the human isocortex as well. PMID:6479601

  7. Recent studies of ovine neuronal ceroid lipofuscinoses from BARN, the Batten Animal Research Network.

    PubMed

    Palmer, David N; Neverman, Nicole J; Chen, Jarol Z; Chang, Chia-Tien; Houweling, Peter J; Barry, Lucy A; Tammen, Imke; Hughes, Stephanie M; Mitchell, Nadia L

    2015-10-01

    Studies on naturally occurring New Zealand and Australian ovine models of the neuronal ceroid-lipofuscinoses (Batten disease, NCLs) have greatly aided our understanding of these diseases. Close collaborations between the New Zealand groups at Lincoln University and the University of Otago, Dunedin, and a group at the University of Sydney, Australia, led to the formation of BARN, the Batten Animal Research Network. This review focusses on presentations at the 14th International Conference on Neuronal Ceroid Lipofuscinoses (Batten Disease), recent relevant background work, and previews of work in preparation for publication. Themes include CLN5 and CLN6 neuronal cell culture studies, studies on tissues from affected and control animals and whole animal in vivo studies. Topics include the effect of a CLN6 mutation on endoplasmic reticulum proteins, lysosomal function and the interactions of CLN6 with other lysosomal activities and trafficking, scoping gene-based therapies, a molecular dissection of neuroinflammation, identification of differentially expressed genes in brain tissue, an attempted therapy with an anti-inflammatory drug in vivo and work towards gene therapy in ovine models of the NCLs. This article is part of a Special Issue entitled: "Current Research on the Neuronal Ceroid Lipofuscinoses (Batten Disease)".

  8. CNS-expressed cathepsin D prevents lymphopenia in a murine model of congenital neuronal ceroid lipofuscinosis.

    PubMed

    Shevtsova, Zinayida; Garrido, Manuel; Weishaupt, Jochen; Saftig, Paul; Bähr, Mathias; Lühder, Fred; Kügler, Sebastian

    2010-07-01

    Deficiency in Cathepsin D (CtsD), the major cellular lysosomal aspartic proteinase, causes the congenital form of neuronal ceroid lipofuscinoses (NCLs). CtsD-deficient mice show severe visceral lesions like lymphopenia in addition to their central nervous system (CNS) phenotype of ceroid accumulation, microglia activation, and seizures. Here we demonstrate that re-expression of CtsD within the CNS but not re-expression of CtsD in visceral organs prevented both central and visceral pathologies of CtsD(-/-) mice. Our results suggest that CtsD was substantially secreted from CNS neurons and drained from CNS to periphery via lymphatic routes. Through this drainage, CNS-expressed CtsD acts as an important modulator of immune system maintenance and peripheral tissue homeostasis. These effects depended on enzymatic activity and not on proposed functions of CtsD as an extracellular ligand. Our results furthermore demonstrate that the prominent accumulation of ceroid/lipofuscin and activation of microglia in brains of CtsD(-/-) are not lethal factors but can be tolerated by the rodent CNS. PMID:20489146

  9. Mutations in DNAJC5, Encoding Cysteine-String Protein Alpha, Cause Autosomal-Dominant Adult-Onset Neuronal Ceroid Lipofuscinosis

    PubMed Central

    Nosková, Lenka; Stránecký, Viktor; Hartmannová, Hana; Přistoupilová, Anna; Barešová, Veronika; Ivánek, Robert; Hůlková, Helena; Jahnová, Helena; van der Zee, Julie; Staropoli, John F.; Sims, Katherine B.; Tyynelä, Jaana; Van Broeckhoven, Christine; Nijssen, Peter C.G.; Mole, Sara E.; Elleder, Milan; Kmoch, Stanislav

    2011-01-01

    Autosomal-dominant adult-onset neuronal ceroid lipofuscinosis (ANCL) is characterized by accumulation of autofluorescent storage material in neural tissues and neurodegeneration and has an age of onset in the third decade of life or later. The genetic and molecular basis of the disease has remained unknown for many years. We carried out linkage mapping, gene-expression analysis, exome sequencing, and candidate-gene sequencing in affected individuals from 20 families and/or individuals with simplex cases; we identified in five individuals one of two disease-causing mutations, c.346_348delCTC and c.344T>G, in DNAJC5 encoding cysteine-string protein alpha (CSPα). These mutations—causing a deletion, p.Leu116del, and an amino acid exchange, p.Leu115Arg, respectively—are located within the cysteine-string domain of the protein and affect both palmitoylation-dependent sorting and the amount of CSPα in neuronal cells. The resulting depletion of functional CSPα might cause in parallel the presynaptic dysfunction and the progressive neurodegeneration observed in affected individuals and lysosomal accumulation of misfolded and proteolysis-resistant proteins in the form of characteristic ceroid deposits in neurons. Our work represents an important step in the genetic dissection of a genetically heterogeneous group of ANCLs. It also confirms a neuroprotective role for CSPα in humans and demonstrates the need for detailed investigation of CSPα in the neuronal ceroid lipofuscinoses and other neurodegenerative diseases presenting with neuronal protein aggregation. PMID:21820099

  10. Retention of lysosomal protein CLN5 in the endoplasmic reticulum causes neuronal ceroid lipofuscinosis in Asian sibship.

    PubMed

    Lebrun, Anne-Hélène; Storch, Stephan; Rüschendorf, Franz; Schmiedt, Mia-Lisa; Kyttälä, Aija; Mole, Sara E; Kitzmüller, Claudia; Saar, Kathrin; Mewasingh, Leena D; Boda, Volker; Kohlschütter, Alfried; Ullrich, Kurt; Braulke, Thomas; Schulz, Angela

    2009-05-01

    The neuronal ceroid lipofuscinoses (NCLs) form a group of autosomal recessively inherited neurodegenerative disorders that mainly affect children. Ten NCL forms can be distinguished by age at onset, clinicopathologic features, and genetics. In eight of these forms, the underlying genes have been identified. At present, approximately 10% of all patients do not fall into one of the eight known genetic forms of NCL. We have identified two Asian families with two novel homozygous mutations in the CLN5 gene. In the first Pakistani family, two children developed symptoms of an early juvenile NCL. After exclusion of mutations in genes known to be associated with this age of onset in families from many different countries (CLN1, CLN2, CLN3, CLN6, CLN8 and CLN10) SNP array-based homozygosity mapping led to the identification of a novel homozygous mutation c.1072_1073delTT (p.Leu358AlafsX4) in CLN5. In the second Afghan family, two children developed symptoms of a late infantile NCL. The mutation c.1137G>T (p.Trp379Cys) in CLN5 was identified. The affected children in these families represent the first reported CLN5 patients originating in Asian sibships. Expression analysis showed that mutant p.Leu358AlafsX4 CLN5 is truncated and lacks a used N-glycosylation site at Asn401. The missense mutation p.Trp379Cys affected neither the size nor glycosylation of the CLN5 protein. Double immunofluorescence microscopy showed that while the wild-type CLN5 protein is localized in lysosomes, both mutant CLN5 proteins are retained in the endoplasmic reticulum rather than reaching the lysosome.

  11. The Chihuahua dog: A new animal model for neuronal ceroid lipofuscinosis CLN7 disease?

    PubMed

    Faller, Kiterie M E; Bras, Jose; Sharpe, Samuel J; Anderson, Glenn W; Darwent, Lee; Kun-Rodrigues, Celia; Alroy, Joseph; Penderis, Jacques; Mole, Sara E; Gutierrez-Quintana, Rodrigo; Guerreiro, Rita J

    2016-04-01

    Neuronal ceroid lipofuscinoses (NCLs) are a group of incurable lysosomal storage disorders characterized by neurodegeneration and accumulation of lipopigments mainly within the neurons. We studied two littermate Chihuahua dogs presenting with progressive signs of blindness, ataxia, pacing, and cognitive impairment from 1 year of age. Because of worsening of clinical signs, both dogs were euthanized at about 2 years of age. Postmortem examination revealed marked accumulation of autofluorescent intracellular inclusions within the brain, characteristic of NCL. Whole-genome sequencing was performed on one of the affected dogs. After sequence alignment and variant calling against the canine reference genome, variants were identified in the coding region or splicing regions of four previously known NCL genes (CLN6, ARSG, CLN2 [=TPP1], and CLN7 [=MFSD8]). Subsequent segregation analysis within the family (two affected dogs, both parents, and three relatives) identified MFSD8:p.Phe282Leufs13*, which had previously been identified in one Chinese crested dog with no available ancestries, as the causal mutation. Because of the similarities of the clinical signs and histopathological changes with the human form of the disease, we propose that the Chihuahua dog could be a good animal model of CLN7 disease. PMID:26762174

  12. Native and Complexed IGF-1: Biodistribution and Pharmacokinetics in Infantile Neuronal Ceroid Lipofuscinosis

    PubMed Central

    Huhtala, Tuulia; Rytkönen, Jussi; Jalanko, Anu; Kaasalainen, Martti; Salonen, Jarno; Riikonen, Raili; Närvänen, Ale

    2012-01-01

    Infantile neuronal ceroid lipofuscinosis (INCL) is a severe neurodegenerative disorder of childhood characterized by selective death of cortical neurons. Insulin-like growth factor 1 (IGF-1) is important in embryonic development and is considered as a potential therapeutic agent for several disorders of peripheral and central nervous systems. In circulation IGF-1 is mainly bound to its carrier protein IGFBP-3. As a therapeutic agent IGF-1 has shown to be more active as free than complexed form. However, this may cause side effects during the prolonged treatment. In addition to IGFBP-3 the bioavailability of IGF-1 can be modulated by using mesoporous silicon nanoparticles (NPs) which are optimal carriers for sustained release of unstable peptide hormones like IGF-1. In this study we compared biodistribution, pharmacokinetics, and bioavailability of radiolabeled free IGF-1, IGF-1/IGFBP-3, and IGF-1/NP complexes in a Cln1-/- knockout mouse model. IGF-1/NP was mainly accumulated in liver and spleen in all studied time points, whereas minor and more constant amounts were measured in other organs compared to free IGF-1 or IGF-1/IGFBP-3. Also concentration of IGF-1/NP in blood was relatively high and stable during studied time points suggesting continuous release of IGF-1 from the particles. PMID:22778966

  13. Granulin Knock Out Zebrafish Lack Frontotemporal Lobar Degeneration and Neuronal Ceroid Lipofuscinosis Pathology

    PubMed Central

    Solchenberger, Barbara; Russell, Claire; Kremmer, Elisabeth; Haass, Christian; Schmid, Bettina

    2015-01-01

    Loss of function mutations in granulin (GRN) are linked to two distinct neurological disorders, frontotemporal lobar degeneration (FTLD) and neuronal ceroid lipofuscinosis (NCL). It is so far unknown how a complete loss of GRN in NCL and partial loss of GRN in FTLD can result in such distinct diseases. In zebrafish, there are two GRN homologues, Granulin A (Grna) and Granulin B (Grnb). We have generated stable Grna and Grnb loss of function zebrafish mutants by zinc finger nuclease mediated genome editing. Surprisingly, the grna and grnb single and double mutants display neither spinal motor neuron axonopathies nor a reduced number of myogenic progenitor cells as previously reported for Grna and Grnb knock down embryos. Additionally, grna−/−;grnb−/− double mutants have no obvious FTLD- and NCL-related biochemical and neuropathological phenotypes. Taken together, the Grna and Grnb single and double knock out zebrafish lack any obvious morphological, pathological and biochemical phenotypes. Loss of zebrafish Grna and Grnb might therefore either be fully compensated or only become symptomatic upon additional challenge. PMID:25785851

  14. A novel mutation in the MFSD8 gene in late infantile neuronal ceroid lipofuscinosis.

    PubMed

    Stogmann, E; El Tawil, S; Wagenstaller, J; Gaber, A; Edris, S; Abdelhady, A; Assem-Hilger, E; Leutmezer, F; Bonelli, S; Baumgartner, C; Zimprich, F; Strom, T M; Zimprich, A

    2009-02-01

    Neuronal ceroid lipofuscinoses (NCL) are lysosomal storage disorders and constitute the most common group of progressive neurodegenerative diseases in childhood. Most NCLs are inherited in a recessive manner and are clinically characterised by a variable age at onset, epileptic seizures, psychomotor decline, visual impairment and premature death. To date, eight causative genes have been identified to underlie various clinical forms of NCL. We performed a genome-wide linkage analysis followed by sequencing the recently described NCL gene MFSD8 in three affected and three unaffected members of a consanguineous Egyptian family with an autosomal recessively inherited progressive neurodegenerative disorder. The clinical picture of the patients was compatible with a late infantile NCL (LINCL); however, impairment of the visual system was not a cardinal symptom in the respective family. By linkage analysis, we identified two putative loci on chromosome 1p36.11-p35.1 and 4q28.1-q28.2. The latter locus (4q28.1-q28.2) contained the MFSD8 gene, comprising a novel homozygous missense mutation in exon 5 (c.362a>g /p.Tyr121Cys), which segregated with the disease in the three affected sibs. We describe a novel mutation in the previously identified MFSD8 gene in a family with a common phenotype of LINCL, but no clinical report of vision loss. Our results enlarge the mutational and perhaps the nosological spectrum of one of the recently identified subtypes of NCL, called CLN7. PMID:18850119

  15. A Missense Mutation in Canine CLN6 in an Australian Shepherd with Neuronal Ceroid Lipofuscinosis

    PubMed Central

    Katz, Martin L.; Farias, Fabiana H.; Sanders, Douglas N.; Zeng, Rong; Khan, Shahnawaz; Johnson, Gary S.; O'Brien, Dennis P.

    2011-01-01

    The childhood neuronal ceroid lipofuscinoses (NCLs) are inherited neurodegenerative diseases that are progressive and ultimately fatal. An Australian Shepherd that exhibited a progressive neurological disorder with signs similar to human NCL was evaluated. The cerebral cortex, cerebellum, and retina were found to contain massive accumulations of autofluorescent inclusions characteristic of the NCLs. Nucleotide sequence analysis of DNA from the affected dog identified a T to C variant (c.829T>C) in exon 7 of CLN6. Mutations in the human ortholog underlie a late-infantile form of NCL in humans. The T-to-C transition results in a tryptophan to arginine amino acid change in the predicted protein sequence. Tryptophans occur at homologous positions in the CLN6 proteins from all 13 other vertebrates evaluated. The c.829T>C transition is a strong candidate for the causative mutation in this NCL-affected dog. Dogs with this mutation could serve as a model for the analogous human disorder. PMID:21234413

  16. Mutated CTSF in adult-onset neuronal ceroid lipofuscinosis and FTD

    PubMed Central

    van der Zee, Julie; Mariën, Peter; Crols, Roeland; Van Mossevelde, Sara; Dillen, Lubina; Perrone, Federica; Engelborghs, Sebastiaan; Verhoeven, Jo; D'aes, Tine; Ceuterick-De Groote, Chantal; Sieben, Anne; Versijpt, Jan; Cras, Patrick; Martin, Jean-Jacques

    2016-01-01

    Objective: To investigate the molecular basis of a Belgian family with autosomal recessive adult-onset neuronal ceroid lipofuscinosis (ANCL or Kufs disease [KD]) with pronounced frontal lobe involvement and to expand the findings to a cohort of unrelated Belgian patients with frontotemporal dementia (FTD). Methods: Genetic screening in the ANCL family and FTD cohort (n = 461) was performed using exome sequencing and targeted massive parallel resequencing. Results: We identified a homozygous mutation (p.Ile404Thr) in the Cathepsin F (CTSF) gene cosegregating in the ANCL family. No other mutations were found that could explain the disease in this family. All 4 affected sibs developed motor symptoms and early-onset dementia with prominent frontal features. Two of them evolved to akinetic mutism. Disease presentation showed marked phenotypic variation with the onset ranging from 26 to 50 years. Myoclonic epilepsy in one of the sibs was suggestive for KD type A, while epilepsy was not present in the other sibs who presented with clinical features of KD type B. In a Belgian cohort of unrelated patients with FTD, the same heterozygous p.Arg245His mutation was identified in 2 patients who shared a common haplotype. Conclusions: A homozygous CTSF mutation was identified in a recessive ANCL pedigree. In contrast to the previous associations of CTSF with KD type B, our findings suggest that CTSF genetic testing should also be considered in patients with KD type A as well as in early-onset dementia with prominent frontal lobe and motor symptoms.

  17. The role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis.

    PubMed

    Macauley, Shannon L; Pekny, Milos; Sands, Mark S

    2011-10-26

    Infantile neuronal ceroid lipofuscinosis (INCL) is an inherited neurodegenerative disorder affecting the CNS during infancy. INCL is caused by mutations in the CLN1 gene that lead to a deficiency in the lysosomal hydrolase, palmitoyl protein thioesterase 1 (PPT1). A murine model of INCL, the PPT1-deficient (PPT1(-/-)) mouse, is an accurate phenocopy of the human disease. The first pathological change observed in the PPT1(-/-) brain is regional areas of glial fibrillary acidic protein (GFAP) upregulation, which predicts future areas of neurodegeneration. We hypothesized that preventing GFAP and vimentin upregulation in reactive astrocytes will alter the CNS disease. To test this hypothesis, we generated mice simultaneously carrying null mutations in the GFAP, Vimentin, and PPT1 genes (GFAP(-/-)Vimentin(-/-)PPT1(-/-)). Although the clinical and pathological features of the GFAP(-/-)Vimentin(-/-)PPT1(-/-) mice are similar to INCL, the disease appears earlier and progresses more rapidly. One mechanism underlying this accelerated phenotype is a profound neuroinflammatory response within the CNS. Thus, our data identify a protective role for intermediate filament upregulation during astrocyte activation in INCL, a model of chronic neurodegeneration.

  18. Exome sequencing is an efficient tool for variant late-infantile neuronal ceroid lipofuscinosis molecular diagnosis.

    PubMed

    Patiño, Liliana Catherine; Battu, Rajani; Ortega-Recalde, Oscar; Nallathambi, Jeyabalan; Anandula, Venkata Ramana; Renukaradhya, Umashankar; Laissue, Paul

    2014-01-01

    The neuronal ceroid-lipofuscinoses (NCL) is a group of neurodegenerative disorders characterized by epilepsy, visual failure, progressive mental and motor deterioration, myoclonus, dementia and reduced life expectancy. Classically, NCL-affected individuals have been classified into six categories, which have been mainly defined regarding the clinical onset of symptoms. However, some patients cannot be easily included in a specific group because of significant variation in the age of onset and disease progression. Molecular genetics has emerged in recent years as a useful tool for enhancing NCL subtype classification. Fourteen NCL genetic forms (CLN1 to CLN14) have been described to date. The variant late-infantile form of the disease has been linked to CLN5, CLN6, CLN7 (MFSD8) and CLN8 mutations. Despite advances in the diagnosis of neurodegenerative disorders mutations in these genes may cause similar phenotypes, which rends difficult accurate candidate gene selection for direct sequencing. Three siblings who were affected by variant late-infantile NCL are reported in the present study. We used whole-exome sequencing, direct sequencing and in silico approaches to identify the molecular basis of the disease. We identified the novel c.1219T>C (p.Trp407Arg) and c.1361T>C (p.Met454Thr) MFSD8 pathogenic mutations. Our results highlighted next generation sequencing as a novel and powerful methodological approach for the rapid determination of the molecular diagnosis of NCL. They also provide information regarding the phenotypic and molecular spectrum of CLN7 disease.

  19. Exome-Sequencing Confirms DNAJC5 Mutations as Cause of Adult Neuronal Ceroid-Lipofuscinosis

    PubMed Central

    Benitez, Bruno A.; Alvarado, David; Cai, Yefei; Mayo, Kevin; Chakraverty, Sumitra; Norton, Joanne; Morris, John C.; Sands, Mark S.; Goate, Alison; Cruchaga, Carlos

    2011-01-01

    We performed whole-exome sequencing in two autopsy-confirmed cases and an elderly unaffected control from a multigenerational family with autosomal dominant neuronal ceroid lipofuscinosis (ANCL). A novel single-nucleotide variation (c.344T>G) in the DNAJC5 gene was identified. Mutational screening in an independent family with autosomal dominant ANCL found an in-frame single codon deletion (c.346_348 delCTC) resulting in a deletion of p.Leu116del. These variants fulfill all genetic criteria for disease-causing mutations: they are found in unrelated families with the same disease, exhibit complete segregation between the mutation and the disease, and are absent in healthy controls. In addition, the associated amino acid substitutions are located in evolutionarily highly conserved residues and are predicted to functionally affect the encoded protein (CSPα). The mutations are located in a cysteine-string domain, which is required for membrane targeting/binding, palmitoylation, and oligomerization of CSPα. We performed a comprehensive in silico analysis of the functional and structural impact of both mutations on CSPα. We found that these mutations dramatically decrease the affinity of CSPα for the membrane. We did not identify any significant effect on palmitoylation status of CSPα. However, a reduction of CSPα membrane affinity may change its palmitoylation and affect proper intracellular sorting. We confirm that CSPα has a strong intrinsic aggregation propensity; however, it is not modified by the mutations. A complementary disease-network analysis suggests a potential interaction with other NCLs genes/pathways. This is the first replication study of the identification of DNAJC5 as the disease-causing gene for autosomal dominant ANCL. The identification of the novel gene in ANCL will allow us to gain a better understanding of the pathological mechanism of ANCLs and constitutes a great advance toward the development of new molecular diagnostic tests and may

  20. Survival Advantage of Neonatal CNS Gene Transfer for Late Infantile Neuronal Ceroid Lipofuscinosis

    PubMed Central

    Sondhi, Dolan; Peterson, Daniel A.; Edelstein, Andrew M.; del Fierro, Katrina; Hackett, Neil R.; Crystal, Ronald G.

    2009-01-01

    Summary Late infantile neuronal ceroid lipofuscinosis (LINCL), a fatal autosomal recessive neurodegenerative lysosomal storage disorder of childhood, is caused by mutations in the CLN2 gene, resulting in deficiency of the protein tripeptidyl peptidase I (TPP-I). We have previously shown that direct CNS administration of AAVrh.10hCLN2 to adult CLN2 knockout mice, a serotype rh.10 adeno-associated virus expressing the wild type CLN2 cDNA, will partially improve neurological function and survival. In this study, we explore the hypothesis that administration of AAVrh.10hCLN2 to the neonatal brain will significantly improve the results of AAVrh.10hCLN2 therapy. To assess this concept, AAVrh.10hCLN2 vector was administered directly to the CNS of CLN2 knockout mice at 2 days, 3 wk and 7 wk of age. While all treatment groups show a marked increase in total TPP-I activity over wild-type mice, neonatally treated mice displayed high levels of TPP-I activity in the CNS 1 yr after administration which was spread throughout the brain. Using behavioral markers, 2 day treated mice demonstrate marked improvement over 3 wk, 7 wk or untreated mice. Finally, neonatal administration of AAVrh.10hCLN2 was associated with markedly enhanced survival, with a median time of death 376 days for neonatal treated mice, 277 days for 3 wk treated mice, 168 days for 7 wk treated mice, and 121 days for untreated mice. These data suggest that neonatal treatment offers many unique advantages, and that early detection and treatment may be essential for maximal gene therapy for childhood lysosomal storage disorders affecting the CNS. PMID:18639872

  1. Novel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteins

    PubMed Central

    2009-01-01

    Background Neuronal ceroid lipofuscinoses (NCLs) comprise at least eight genetically characterized neurodegenerative disorders of childhood. Despite of genetic heterogeneity, the high similarity of clinical symptoms and pathology of different NCL disorders suggest cooperation between different NCL proteins and common mechanisms of pathogenesis. Here, we have studied molecular interactions between NCL proteins, concentrating specifically on the interactions of CLN5, the protein underlying the Finnish variant late infantile form of NCL (vLINCLFin). Results We found that CLN5 interacts with several other NCL proteins namely, CLN1/PPT1, CLN2/TPP1, CLN3, CLN6 and CLN8. Furthermore, analysis of the intracellular targeting of CLN5 together with the interacting NCL proteins revealed that over-expression of PPT1 can facilitate the lysosomal transport of mutated CLN5FinMajor, normally residing in the ER and in the Golgi complex. The significance of the novel interaction between CLN5 and PPT1 was further supported by the finding that CLN5 was also able to bind the F1-ATPase, earlier shown to interact with PPT1. Conclusion We have described novel interactions between CLN5 and several NCL proteins, suggesting a modifying role for these proteins in the pathogenesis of individual NCL disorders. Among these novel interactions, binding of CLN5 to CLN1/PPT1 is suggested to be the most significant one, since over-expression of PPT1 was shown to influence on the intracellular trafficking of mutated CLN5, and they were shown to share a binding partner outside the NCL protein spectrum. PMID:19941651

  2. Mutated CTSF in adult-onset neuronal ceroid lipofuscinosis and FTD

    PubMed Central

    van der Zee, Julie; Mariën, Peter; Crols, Roeland; Van Mossevelde, Sara; Dillen, Lubina; Perrone, Federica; Engelborghs, Sebastiaan; Verhoeven, Jo; D'aes, Tine; Ceuterick-De Groote, Chantal; Sieben, Anne; Versijpt, Jan; Cras, Patrick; Martin, Jean-Jacques

    2016-01-01

    Objective: To investigate the molecular basis of a Belgian family with autosomal recessive adult-onset neuronal ceroid lipofuscinosis (ANCL or Kufs disease [KD]) with pronounced frontal lobe involvement and to expand the findings to a cohort of unrelated Belgian patients with frontotemporal dementia (FTD). Methods: Genetic screening in the ANCL family and FTD cohort (n = 461) was performed using exome sequencing and targeted massive parallel resequencing. Results: We identified a homozygous mutation (p.Ile404Thr) in the Cathepsin F (CTSF) gene cosegregating in the ANCL family. No other mutations were found that could explain the disease in this family. All 4 affected sibs developed motor symptoms and early-onset dementia with prominent frontal features. Two of them evolved to akinetic mutism. Disease presentation showed marked phenotypic variation with the onset ranging from 26 to 50 years. Myoclonic epilepsy in one of the sibs was suggestive for KD type A, while epilepsy was not present in the other sibs who presented with clinical features of KD type B. In a Belgian cohort of unrelated patients with FTD, the same heterozygous p.Arg245His mutation was identified in 2 patients who shared a common haplotype. Conclusions: A homozygous CTSF mutation was identified in a recessive ANCL pedigree. In contrast to the previous associations of CTSF with KD type B, our findings suggest that CTSF genetic testing should also be considered in patients with KD type A as well as in early-onset dementia with prominent frontal lobe and motor symptoms. PMID:27668283

  3. Exome sequencing is an efficient tool for variant late-infantile neuronal ceroid lipofuscinosis molecular diagnosis.

    PubMed

    Patiño, Liliana Catherine; Battu, Rajani; Ortega-Recalde, Oscar; Nallathambi, Jeyabalan; Anandula, Venkata Ramana; Renukaradhya, Umashankar; Laissue, Paul

    2014-01-01

    The neuronal ceroid-lipofuscinoses (NCL) is a group of neurodegenerative disorders characterized by epilepsy, visual failure, progressive mental and motor deterioration, myoclonus, dementia and reduced life expectancy. Classically, NCL-affected individuals have been classified into six categories, which have been mainly defined regarding the clinical onset of symptoms. However, some patients cannot be easily included in a specific group because of significant variation in the age of onset and disease progression. Molecular genetics has emerged in recent years as a useful tool for enhancing NCL subtype classification. Fourteen NCL genetic forms (CLN1 to CLN14) have been described to date. The variant late-infantile form of the disease has been linked to CLN5, CLN6, CLN7 (MFSD8) and CLN8 mutations. Despite advances in the diagnosis of neurodegenerative disorders mutations in these genes may cause similar phenotypes, which rends difficult accurate candidate gene selection for direct sequencing. Three siblings who were affected by variant late-infantile NCL are reported in the present study. We used whole-exome sequencing, direct sequencing and in silico approaches to identify the molecular basis of the disease. We identified the novel c.1219T>C (p.Trp407Arg) and c.1361T>C (p.Met454Thr) MFSD8 pathogenic mutations. Our results highlighted next generation sequencing as a novel and powerful methodological approach for the rapid determination of the molecular diagnosis of NCL. They also provide information regarding the phenotypic and molecular spectrum of CLN7 disease. PMID:25333361

  4. Chromatography and spectrofluorometry of brain fluorophores in neuronal ceroid lipofuscinosis (NCL).

    PubMed

    Armstrong, D; Wilhelm, J; Smid, F; Elleder, M

    1992-07-15

    The aim of the present work was to develop a chromatographic system for the separation of individual fluorophores extracted from neuronal ceroid lipofuscinosis (NCL) brain and isolated storage bodies. Extracts from gray matter were best resolved on silica-gel HPTLC plates using a mixture of chloroform/methanol/water (55:45:10 by vol.). Two other chromatographic systems were tested which gave poorer separation. Corrected fluorescence spectra were obtained on the original extract and fluorescence intensity, especially at longer wavelengths was increased in both samples. Yellow and blue fluorophores were detected on HPTLC plates using a primary violet and secondary yellow filter with cut-off levels of 400 and 520 nm, respectively. Plates were photographed at 20 min, 2 h and 1 week after chromatography. With this filter system, up to 12 yellow bands of differing intensity were observed at 20 min but with time, some of these changed to blue as a result of autoxidation. NCL tissues emit yellow fluorescence when viewed under light microscopy, however extracted material did not demonstrate a distinct peak in this region of the spectrum which should be around 575 nm. HPTLC confirmed this observation and time studies revealed that autoxidation changes occur and must be carefully controlled to reduce artifacts. The discrepancy between extracted and non-extracted observations may be the result of superposition of multiple fluorophores with differing maxima and/or a self-absorption phenomenon. The combination of chromatographic separation and spectral analysis as described in this study, may be a valuable technique to further clarify the characteristics of compound fluorescent lipopigments. It is suggested that NCL fluorophores of human brain differ in their properties from other models.

  5. Adult-onset autosomal recessive ataxia associated with neuronal ceroid lipofuscinosis type 5 gene (CLN5) mutations.

    PubMed

    Mancini, Cecilia; Nassani, Stefano; Guo, Yiran; Chen, Yulan; Giorgio, Elisa; Brussino, Alessandro; Di Gregorio, Eleonora; Cavalieri, Simona; Lo Buono, Nicola; Funaro, Ada; Pizio, Nicola Renato; Nmezi, Bruce; Kyttala, Aija; Santorelli, Filippo Maria; Padiath, Quasar Salem; Hakonarson, Hakon; Zhang, Hao; Brusco, Alfredo

    2015-01-01

    Autosomal recessive inherited ataxias are a growing group of genetic disorders. We report two Italian siblings presenting in their mid-50s with difficulty in walking, dysarthria and progressive cognitive decline. Visual loss, ascribed to glaucoma, manifested a few years before the other symptoms. Brain MRI showed severe cerebellar atrophy, prevalent in the vermis, with marked cortical atrophy of both hemispheres. Exome sequencing identified a novel homozygous mutation (c.935G > A;p.Ser312Asn) in the ceroid neuronal lipofuscinosis type 5 gene (CLN5). Bioinformatics predictions and in vitro studies showed that the mutation was deleterious and likely affects ER-lysosome protein trafficking. Our findings support CLN5 hypomorphic mutations cause autosomal recessive cerebellar ataxia, confirming other reports showing CLN mutations are associated with adult-onset neurodegenerative disorders. We suggest CLN genes should be considered in the molecular analyses of patients presenting with adult-onset autosomal recessive cerebellar ataxia.

  6. Diagnosis of neuronal ceroid lipofuscinosis type 2 (CLN2 disease): Expert recommendations for early detection and laboratory diagnosis.

    PubMed

    Fietz, Michael; AlSayed, Moeenaldeen; Burke, Derek; Cohen-Pfeffer, Jessica; Cooper, Jonathan D; Dvořáková, Lenka; Giugliani, Roberto; Izzo, Emanuela; Jahnová, Helena; Lukacs, Zoltan; Mole, Sara E; Noher de Halac, Ines; Pearce, David A; Poupetova, Helena; Schulz, Angela; Specchio, Nicola; Xin, Winnie; Miller, Nicole

    2016-09-01

    Neuronal ceroid lipofuscinoses (NCLs) are a heterogeneous group of lysosomal storage disorders. NCLs include the rare autosomal recessive neurodegenerative disorder neuronal ceroid lipofuscinosis type 2 (CLN2) disease, caused by mutations in the tripeptidyl peptidase 1 (TPP1)/CLN2 gene and the resulting TPP1 enzyme deficiency. CLN2 disease most commonly presents with seizures and/or ataxia in the late-infantile period (ages 2-4), often in combination with a history of language delay, followed by progressive childhood dementia, motor and visual deterioration, and early death. Atypical phenotypes are characterized by later onset and, in some instances, longer life expectancies. Early diagnosis is important to optimize clinical care and improve outcomes; however, currently, delays in diagnosis are common due to low disease awareness, nonspecific clinical presentation, and limited access to diagnostic testing in some regions. In May 2015, international experts met to recommend best laboratory practices for early diagnosis of CLN2 disease. When clinical signs suggest an NCL, TPP1 enzyme activity should be among the first tests performed (together with the palmitoyl-protein thioesterase enzyme activity assay to rule out CLN1 disease). However, reaching an initial suspicion of an NCL or CLN2 disease can be challenging; thus, use of an epilepsy gene panel for investigation of unexplained seizures in the late-infantile/childhood ages is encouraged. To confirm clinical suspicion of CLN2 disease, the recommended gold standard for laboratory diagnosis is demonstration of deficient TPP1 enzyme activity (in leukocytes, fibroblasts, or dried blood spots) and the identification of causative mutations in each allele of the TPP1/CLN2 gene. When it is not possible to perform both analyses, either demonstration of a) deficient TPP1 enzyme activity in leukocytes or fibroblasts, or b) detection of two pathogenic mutations in trans is diagnostic for CLN2 disease. PMID:27553878

  7. Neuronal ceroid lipofuscinosis type CLN2: A new rationale for the construction of phenotypic subgroups based on a survey of 25 cases in South America

    PubMed Central

    Kohan, Romina; Noelia Carabelos, María; Xin, Winnie; Sims, Katherine; Guelbert, Norberto; Adriana Cismondi, Inés; Pons, Patricia; Alonso, Graciela Irene; Troncoso, Mónica; Witting, Scarlet; Pearce, David A.; de Kremer, Raquel Dodelson; Oller-Ramírez, Ana María; de Halac, Inés Noher

    2013-01-01

    Tripeptidyl-peptidase 1 (TPP1) null or residual activity occurs in neuronal ceroid lipofuscinosis (NCL) with underlying TPP1/CLN2 mutations. A survey of 25 South American CLN2 affected individuals enabled the differentiation of two phenotypes: classical late-infantile and variant juvenile, each in approximately 50% of patients, with residual TPP1 activity occurring in approximately 32%. Each individual was assigned to one of three subgroups: (I) n=11, null TPP1 activity in leukocytes; (II) n=8, residual TPP1 activity of 0.60–15.85 nmol/h/mg (nr 110–476); (III) n=6, activity not measured in leukocytes. Curvilinear bodies (CB) appeared in almost all studied CLN2 subjects; the only exceptions occurred in cases of subgroup II: two individuals had combined CBs/fingerprints (FPs), and one case had pure FPs. There were 15 mutations (4 first published in this paper, 3 previously observed in South America by our group, and 8 previously observed by others). In subgroup I, mutations were either missense or nonsense; in subgroups II and III, mutations prevailed at the non-conserved intronic site, c.887-10A>G (intron 7), and to a lesser extent at c.89+5G>C (intron 2), in heterozygous combinations. Grouping phenotypically and genetically known individuals on the basis of TPP1 activity supported the concept that residual enzyme activity underlies a protracted disease course. The prevalence of intronic mutations at nonconserved sites in subgroup II individuals indicates that some alternative splicing might allow some residual TPP1 activity. PMID:23266810

  8. A murine model of infantile neuronal ceroid lipofuscinosis-ultrastructural evaluation of storage in the central nervous system and viscera.

    PubMed

    Galvin, Nancy; Vogler, Carole; Levy, Beth; Kovacs, Attila; Griffey, Megan; Sands, Mark S

    2008-01-01

    Infantile neuronal ceroid lipofuscinosis (INCL), also known as Santavuori-Haltia disease, is an inherited neurodegenerative disorder caused by a mutation in the gene encoding the lysosomal enzyme palmitoyl-protein-thioesterase-1 (PPT1). Fatty acid-modified proteins are not degraded and accumulate as granular osmiophilic deposits in cells in the central nervous system; patients have blindness, seizures, progressive psychomotor deterioration, and die in early childhood. Although the disease manifests clinically primarily with neurological symptoms, visceral storage also accumulates. A murine model of INCL due to PPT1 deficiency exhibits clinical findings and pathology similar to those seen in patients with INCL. Homozygous PPT1-deficient mice have a shortened life span and neurological abnormalities including seizures, blindness, and mental and motor deficits. Widespread granular osmiophilic deposits (GRODs) accumulate in lysosomes in neurons and glia in the brain, retinal cells, kidney glomerular cells, aortic smooth muscle cells, and, in lesser amounts, in the fixed-tissue macrophage system. Accumulation of GRODs in aortic smooth muscle cells is accompanied by abnormalities in cardiac function and aortic root dilatation. This PPT1-deficient murine model is a well-defined genetic system that can be used to test potential therapies for lysosomal storage disease and to study the pathophysiology of INCL. PMID:17990914

  9. Systemic administration of tripeptidyl peptidase I in a mouse model of late infantile neuronal ceroid lipofuscinosis: effect of glycan modification.

    PubMed

    Meng, Yu; Sohar, Istvan; Wang, Lingling; Sleat, David E; Lobel, Peter

    2012-01-01

    Late-infantile neuronal ceroid lipofuscinosis (LINCL) is a recessive genetic disease of childhood caused by deficiencies in the lysosomal protease tripeptidyl peptidase I (TPP1). Disease is characterized by progressive and extensive neuronal death. One hurdle towards development of enzyme replacement therapy is delivery of TPP1 to the brain. In this study, we evaluated the effect of modifying N-linked glycans on recombinant human TPP1 on its pharmacokinetic properties after administration via tail vein injection to a mouse model of LINCL. Unmodified TPP1 exhibited a dose-dependent serum half-life of 12 min (0.12 mg) to 45 min (2 mg). Deglycosylation or modification using sodium metaperiodate oxidation and reduction with sodium borohydride increased the circulatory half-life but did not improve targeting to the brain compared to unmodified TPP1. Analysis of liver, brain, spleen, kidney and lung demonstrated that for all preparations, >95% of the recovered activity was in the liver. Interestingly, administration of a single 2 mg dose (80 mg/kg) of unmodified TPP1 resulted in ∼10% of wild-type activity in brain. This suggests that systemic administration of unmodified recombinant enzyme merits further exploration as a potential therapy for LINCL.

  10. Increased Expression of the Large Conductance, Calcium-Activated K+ (BK) Channel in Adult-Onset Neuronal Ceroid Lipofuscinosis

    PubMed Central

    Donnelier, Julien; Braun, Samuel T.; Dolzhanskaya, Natalia; Ahrendt, Eva; Braun, Andrew P.; Velinov, Milen; Braun, Janice E. A.

    2015-01-01

    Cysteine string protein (CSPα) is a presynaptic J protein co-chaperone that opposes neurodegeneration. Mutations in CSPα (i.e., Leu115 to Arg substitution or deletion (Δ) of Leu116) cause adult neuronal ceroid lipofuscinosis (ANCL), a dominantly inherited neurodegenerative disease. We have previously demonstrated that CSPα limits the expression of large conductance, calcium-activated K+ (BK) channels in neurons, which may impact synaptic excitability and neurotransmission. Here we show by western blot analysis that expression of the pore-forming BKα subunit is elevated ~2.5 fold in the post-mortem cortex of a 36-year-old patient with the Leu116∆ CSPα mutation. Moreover, we find that the increase in BKα subunit level is selective for ANCL and not a general feature of neurodegenerative conditions. While reduced levels of CSPα are found in some postmortem cortex specimens from Alzheimer’s disease patients, we find no concomitant increase in BKα subunit expression in Alzheimer’s specimens. Both CSPα monomer and oligomer expression are reduced in synaptosomes prepared from ANCL cortex compared with control. In a cultured neuronal cell model, CSPα oligomers are short lived. The results of this study indicate that the Leu116∆ mutation leads to elevated BKα subunit levels in human cortex and extend our initial work in rodent models demonstrating the modulation of BKα subunit levels by the same CSPα mutation. While the precise sequence of pathogenic events still remains to be elucidated, our findings suggest that dysregulation of BK channels may contribute to neurodegeneration in ANCL. PMID:25905915

  11. Enzyme replacement therapy attenuates disease progression in a canine model of late-infantile neuronal ceroid lipofuscinosis (CLN2 disease)

    PubMed Central

    Katz, Martin L; Coates, Joan R; Sibigtroth, Christine M; Taylor, Jacob D; Carpentier, Melissa; Young, Whitney M; Wininger, Fred A; Kennedy, Derek; Vuillemenot, Brian R; O'Neill, Charles A

    2014-01-01

    Using a canine model of classical late-infantile neuronal ceroid lipofuscinosis (CLN2 disease), a study was conducted to evaluate the potential pharmacological activity of recombinant human tripeptidyl peptidase-1 (rhTPP1) enzyme replacement therapy administered directly to the cerebrospinal fluid (CSF). CLN2 disease is a hereditary neurodegenerative disorder resulting from mutations in CLN2, which encodes the soluble lysosomal enzyme tripeptidyl peptidase-1 (TPP1). Infants with mutations in both CLN2 alleles develop normally but in the late-infantile/early-childhood period undergo progressive neurological decline accompanied by pronounced brain atrophy. The disorder, a form of Batten disease, is uniformly fatal, with clinical signs starting between 2 and 4 years of age and death usually occurring by the early teenage years. Dachshunds homozygous for a null mutation in the canine ortholog of CLN2 (TPP1) exhibit a similar disorder that progresses to end stage at 10.5–11 months of age. Administration of rhTPP1 via infusion into the CSF every other week, starting at approximately 2.5 months of age, resulted in dose-dependent significant delays in disease progression, as measured by delayed onset of neurologic deficits, improved performance on a cognitive function test, reduced brain atrophy, and increased life span. Based on these findings, a clinical study evaluating the potential therapeutic value of rhTPP1 administration into the CSF of children with CLN2 disease has been initiated. PMID:24938720

  12. Neuronal Ceroid Lipofuscinosis in Border Collie Dogs in Japan: Clinical and Molecular Epidemiological Study (2000–2011)

    PubMed Central

    Mizukami, Keijiro; Kawamichi, Takuji; Koie, Hiroshi; Tamura, Shinji; Matsunaga, Satoru; Imamoto, Shigeki; Saito, Miyoko; Hasegawa, Daisuke; Matsuki, Naoaki; Tamahara, Satoshi; Sato, Shigenobu; Yabuki, Akira; Chang, Hye-Sook; Yamato, Osamu

    2012-01-01

    Neuronal ceroid lipofuscinosis (NCL) is an inherited, neurodegenerative lysosomal disease that causes premature death. The present study describes the clinical and molecular epidemiologic findings of NCL in Border Collies in Japan for 12 years, between 2000 and 2011. The number of affected dogs was surveyed, and their clinical characteristics were analyzed. In 4 kennels with affected dogs, the dogs were genotyped. The genetic relationships of all affected dogs and carriers identified were analyzed. The survey revealed 27 affected dogs, but there was a decreasing trend at the end of the study period. The clinical characteristics of these affected dogs were updated in detail. The genotyping survey demonstrated a high mutant allele frequency in examined kennels (34.8%). The pedigree analysis demonstrated that all affected dogs and carriers in Japan are related to some presumptive carriers imported from Oceania and having a common ancestor. The current high prevalence in Japan might be due to an overuse of these carriers by breeders without any knowledge of the disease. For NCL control and prevention, it is necessary to examine all breeding dogs, especially in kennels with a high prevalence. Such endeavors will reduce NCL prevalence and may already be contributing to the recent decreasing trend in Japan. PMID:22919312

  13. Guidelines for incorporating scientific knowledge and practice on rare diseases into higher education: neuronal ceroid lipofuscinoses as a model disorder.

    PubMed

    Cismondi, Inés Adriana; Kohan, Romina; Adams, Heather; Bond, Mike; Brown, Rachel; Cooper, Jonathan D; de Hidalgo, Perla K; Holthaus, Sophia-Martha Kleine; Mole, Sara E; Mugnaini, Julia; de Ramirez, Ana María Oller; Pesaola, Favio; Rautenberg, Gisela; Platt, Frances M; Noher de Halac, Inés

    2015-10-01

    This article addresses the educational issues associated with rare diseases (RD) and in particular the Neuronal Ceroid Lipofuscinoses (NCLs, or CLN diseases) in the curricula of Health Sciences and Professional's Training Programs. Our aim is to develop guidelines for improving scientific knowledge and practice in higher education and continuous learning programs. Rare diseases (RD) are collectively common in the general population with 1 in 17 people affected by a RD in their lifetime. Inherited defects in genes involved in metabolism are the commonest group of RD with over 8000 known inborn errors of metabolism. The majority of these diseases are neurodegenerative including the NCLs. Any professional training program on NCL must take into account the medical, social and economic burdens related to RDs. To address these challenges and find solutions to them it is necessary that individuals in the government and administrative authorities, academia, teaching hospitals and medical schools, the pharmaceutical industry, investment community and patient advocacy groups all work together to achieve these goals. The logistical issues of including RD lectures in university curricula and in continuing medical education should reflect its complex nature. To evaluate the state of education in the RD field, a summary should be periodically up dated in order to assess the progress achieved in each country that signed up to the international conventions addressing RD issues in society. It is anticipated that auditing current practice will lead to higher standards and provide a framework for those educators involved in establishing RD teaching programs world-wide.

  14. A reversal learning task detects cognitive deficits in a Dachshund model of late-infantile neuronal ceroid lipofuscinosis

    PubMed Central

    Sanders, Douglas N.; Kanazono, Shinichi; Wininger, Fred A.; Whiting, Rebecca E.H.; Flournoy, Camille A.; Coates, Joan R.; Castaner, Lani J.; O’Brien, Dennis P.; Katz, Martin L.

    2011-01-01

    The neuronal ceroid lipofuscinoses (NCLs) are autosomal recessive lysosomal storage diseases characterized by progressive neurodegeneration and by accumulation of autofluorescent storage material in the central nervous system and other tissues. One of the most prominent clinical signs of NCL is progressive decline in cognitive function. We previously described a frame shift mutation of TPP1 in miniature long-haired Dachshunds which causes an early-onset form of NCL analogous to classical late-infantile onset NCL (CLN2) in children. Dogs homozygous for the TPP1 mutation exhibit progressive neurological signs similar to those exhibited by human patients. In order to establish biomarkers for evaluating the efficacy of ongoing therapeutic studies in this canine model, we characterized phenotypic changes in 13 dogs through 9 months of age. Cognitive function was assessed using a T-maze reversal learning task. Cognitive dysfunction was detected in affected dogs as early as 6 months of age and worsened as the disease progressed. Physical and neurological examination, funduscopy, and electroretinography (ERG) were performed at regular intervals. Only changes in ERG responses revealed signs of disease progression earlier than the reversal learning task. In the later stages of the disease clinical signs of visual and motor deficits became evident. The visual and motor deficits were not severe enough to affect the performance of dogs in the T-maze. Declining performance on the reversal learning task is a sensitive measure of higher order cognitive dysfunction which can serve as a useful biomarker of disease progression. PMID:21745338

  15. Melatonin ineffective in neuronal ceroid lipofuscinosis patients with fragmented or normal motor activity rhythms recorded by wrist actigraphy.

    PubMed

    Hätönen, T; Kirveskari, E; Heiskala, H; Sainio, K; Laakso, M L; Santavuori, P

    1999-04-01

    Melatonin was tested as a sleeping pill in five patients with neuronal ceroid lipofuscinoses. The single-blind, placebo-controlled study consisted of motor activity recordings, sleep logs, and administration of placebo or melatonin (2.5 or 5 mg). Daily motor activity rhythms were measured by wrist actigraphy during four 7-day periods (baseline, placebo, melatonin 2.5 mg, and melatonin 5 mg). The placebo or melatonin was administered in the evenings for 3 weeks, and the recordings were made during the last week of the 3-week treatment. Sleep logs were kept by the caregivers during the recordings. Based on period analyses, the activity recordings were evaluated to display a normal (24-h) or fragmented rhythm. Three patients had normal motor activity patterns during the baseline recordings, and administration of placebo or melatonin did not affect their rest/activity rhythms. Two patients had abnormally fragmented activity rhythms during the baseline periods, and administration of placebo or melatonin did not induce synchronization. According to the actigraphic data, there were no changes in activity rhythms resulting from administration of melatonin. However, based on the observations, three families reported that melatonin slightly improved the sleep quality of the patients. These controversial findings show the difficulties involved in specifying the role of melatonin in modulating sleep. Thus, we conclude that more evidence is required before the significance of melatonin as a sleeping pill is defined. PMID:10191137

  16. Successful PGD for late infantile neuronal ceroid lipofuscinosis achieved by combined chromosome and TPP1 gene analysis.

    PubMed

    Shen, Jiandong; Cram, David Stephen; Wu, Wei; Cai, Lingbo; Yang, Xiaoyu; Sun, Xueping; Cui, Yugui; Liu, Jiayin

    2013-08-01

    Late infantile neuronal ceroid lipofuscinosis (NCL-2) is a severe debilitating autosomal recessive disease caused by mutations in TPP1. There are no effective treatments, resulting in early childhood death. A couple with two affected children presented for reproductive genetic counselling and chose to undertake IVF and preimplantation genetic diagnosis (PGD) to avoid the possibility of another affected child. However, DNA testing revealed only one mutation in the proband inherited from mother. Linkage analysis identified five informative linked short tandem repeat markers to aid the genetic diagnosis. Following IVF, five cleavage-stage embryos were biopsied and blastomeres were first subjected to whole-genome amplification, then a series of down-stream molecular genetic analyses to diagnose TPP1 genotype and finally array comparative genomic hybridization (CGH) to assess the chromosomal ploidy of each embryo. Two unaffected euploid embryos were identified for transfer. One was transferred on day 5 resulting in an ongoing pregnancy. Confirmatory prenatal diagnosis by amniocentesis showed concordance of the embryo and fetal diagnosis. As far as is known, this is the first successful report of PGD for NCL-2 using double-factor PGD with simultaneous single-gene testing and array CGH to identify an unaffected and chromosomally normal embryo for transfer.

  17. Melatonin ineffective in neuronal ceroid lipofuscinosis patients with fragmented or normal motor activity rhythms recorded by wrist actigraphy.

    PubMed

    Hätönen, T; Kirveskari, E; Heiskala, H; Sainio, K; Laakso, M L; Santavuori, P

    1999-04-01

    Melatonin was tested as a sleeping pill in five patients with neuronal ceroid lipofuscinoses. The single-blind, placebo-controlled study consisted of motor activity recordings, sleep logs, and administration of placebo or melatonin (2.5 or 5 mg). Daily motor activity rhythms were measured by wrist actigraphy during four 7-day periods (baseline, placebo, melatonin 2.5 mg, and melatonin 5 mg). The placebo or melatonin was administered in the evenings for 3 weeks, and the recordings were made during the last week of the 3-week treatment. Sleep logs were kept by the caregivers during the recordings. Based on period analyses, the activity recordings were evaluated to display a normal (24-h) or fragmented rhythm. Three patients had normal motor activity patterns during the baseline recordings, and administration of placebo or melatonin did not affect their rest/activity rhythms. Two patients had abnormally fragmented activity rhythms during the baseline periods, and administration of placebo or melatonin did not induce synchronization. According to the actigraphic data, there were no changes in activity rhythms resulting from administration of melatonin. However, based on the observations, three families reported that melatonin slightly improved the sleep quality of the patients. These controversial findings show the difficulties involved in specifying the role of melatonin in modulating sleep. Thus, we conclude that more evidence is required before the significance of melatonin as a sleeping pill is defined.

  18. Enzyme replacement therapy attenuates disease progression in a canine model of late-infantile neuronal ceroid lipofuscinosis (CLN2 disease).

    PubMed

    Katz, Martin L; Coates, Joan R; Sibigtroth, Christine M; Taylor, Jacob D; Carpentier, Melissa; Young, Whitney M; Wininger, Fred A; Kennedy, Derek; Vuillemenot, Brian R; O'Neill, Charles A

    2014-11-01

    Using a canine model of classical late-infantile neuronal ceroid lipofuscinosis (CLN2 disease), a study was conducted to evaluate the potential pharmacological activity of recombinant human tripeptidyl peptidase-1 (rhTPP1) enzyme replacement therapy administered directly to the cerebrospinal fluid (CSF). CLN2 disease is a hereditary neurodegenerative disorder resulting from mutations in CLN2, which encodes the soluble lysosomal enzyme tripeptidyl peptidase-1 (TPP1). Infants with mutations in both CLN2 alleles develop normally but in the late-infantile/early-childhood period undergo progressive neurological decline accompanied by pronounced brain atrophy. The disorder, a form of Batten disease, is uniformly fatal, with clinical signs starting between 2 and 4 years of age and death usually occurring by the early teenage years. Dachshunds homozygous for a null mutation in the canine ortholog of CLN2 (TPP1) exhibit a similar disorder that progresses to end stage at 10.5-11 months of age. Administration of rhTPP1 via infusion into the CSF every other week, starting at approximately 2.5 months of age, resulted in dose-dependent significant delays in disease progression, as measured by delayed onset of neurologic deficits, improved performance on a cognitive function test, reduced brain atrophy, and increased life span. Based on these findings, a clinical study evaluating the potential therapeutic value of rhTPP1 administration into the CSF of children with CLN2 disease has been initiated.

  19. Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase.

    PubMed Central

    Lu, J Y; Verkruyse, L A; Hofmann, S L

    1996-01-01

    Palmitoyl-protein thioesterase is a lysosomal long-chain fatty acyl hydrolase that removes fatty acyl groups from modified cysteine residues in proteins. Mutations in palmitoyl-protein thioesterase were recently found to cause the neurodegenerative disorder infantile neuronal ceroid lipofuscinosis, a disease characterized by accumulation of amorphous granular deposits in cortical neurons, leading to blindness, seizures, and brain death by the age of three. In the current study, we demonstrate that [35S]cysteine-labeled lipid thioesters accumulate in immortalized lymphoblasts of patients with infantile neuronal ceroid lipofuscinosis. The accumulation in cultured cells is reversed by the addition of recombinant palmitoyl-protein thioesterase that is competent for lysosomal uptake through the mannose-6-phosphate receptor. The [35S]cysteine-labeled lipids are substrates for palmitoyl-protein thioesterase in vitro, and their formation requires prior protein synthesis. These data support a role for palmitoyl-protein thioesterase in the lysosomal degradation of S-acylated proteins and define a major new pathway for the catabolism of acylated proteins in the lysosome. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 6 PMID:8816748

  20. Refined assignment of the infantile neuronal ceroid lipofuscinosis (INCL, CLN1) locus at 1p32: Incorporation of linkage disequilibrium in multipoint analysis

    SciTech Connect

    Hellsten, E.; Vesa, J.; Peltonen, L.; Jaervela, I. ); Speer, M.C.; Ott, J. New York State Psychiatric Institute, New York ); Maekelae, T.P.; Alitalo, K. )

    1993-06-01

    Infantile neuronal ceroid lipofuscinosis, INCL, CLN1, is an autosomally inherited progressive neuro-generative disorder. The disease results in the massive death of cortical neurons, suggesting an essential role for the CLN1 gene product in the normal neuronal maturation during the first years of life. Identification of new multiallelic markers has now made possible the construction of a refined genetic map encompassing the CLN1 locus at 1p32. Strong allelic association was detected with a new, highly polymorphic HY-TM1 marker. The authors incorporated this observed linkage disequilibrium into multipoint linkage analysis, which significantly increased the informativeness of the limited family material and facilitated refined assignment of the CLN1 locus. 23 refs., 2 figs., 4 tabs.

  1. Neurotoxic fragrance produces ceroid and myelin disease.

    PubMed

    Spencer, P S; Sterman, A B; Horoupian, D S; Foulds, M M

    1979-05-11

    Acetyl ethyl tetramethyl tetralin (AETT), a component of soaps, deodorants, and cosmetics, produces hyperirritability and limb weakness in rats repeatedly exposed to the compound. Brain, spinal cord, and peripheral nerves are discolored blue, show progressive neuronal ceroid degeneration, and develop spectacular myelin bubbling. These neurotoxic properties of AETT provide the basis for industry's decision to withdraw the compound from consumer products. In addition, AETT offers the experimentalist a new probe to explore the etiology and pathogeneses of human ceroid and myelin diseases.

  2. Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.

    PubMed

    Lu, Jui-Yun; Nelvagal, Hemanth R; Wang, Lingling; Birnbaum, Shari G; Cooper, Jonathan D; Hofmann, Sandra L

    2015-01-01

    The neuronal ceroid lipofuscinoses (NCLs) are a group of related hereditary lysosomal storage disorders characterized by progressive loss of neurons in the central nervous system resulting in dementia, loss of motor skills, seizures and blindness. A characteristic intralysosomal accumulation of autofluorescent storage material occurs in the brain and other tissues. Three major forms and nearly a dozen minor forms of NCL are recognized. Infantile-onset NCL (CLN1 disease) is caused by severe deficiency in a soluble lysosomal enzyme, palmitoyl-protein thioesterase-1 (PPT1) and no therapy beyond supportive care is available. Homozygous Ppt1 knockout mice reproduce the known features of the disease, developing signs of motor dysfunction at 5 months of age and death around 8 months. Direct delivery of lysosomal enzymes to the cerebrospinal fluid is an approach that has gained traction in small and large animal models of several other neuropathic lysosomal storage diseases, and has advanced to clinical trials. In the current study, Ppt1 knockout mice were treated with purified recombinant human PPT1 enzyme delivered to the lumbar intrathecal space on each of three consecutive days at 6 weeks of age. Untreated PPT1 knockout mice and wild-type mice served as additional controls. Four enzyme concentration levels (0, 2.6, 5.3 and 10.6 mg/ml of specific activity 20 U/mg) were administered in a volume of 80 μl infused over 8 min. Each group consisted of 16-20 mice. The treatment was well tolerated. Disease-specific survival was 233, 267, 272, and 284days for each of the four treatment groups, respectively, and the effect of treatment was highly significant (p<0.0001). The timing of motor deterioration was also delayed. Neuropathology was improved as evidenced by decreased autofluorescent storage material in the spinal cord and a decrease in CD68 staining in the cortex and spinal cord. The improvements in motor function and survival are similar to results reported for

  3. Neuronal ceroid lipofuscinoses (NCL)

    MedlinePlus

    ... NCL include: Abnormally increased muscle tone or spasm Blindness or vision problems Dementia Lack of muscle coordination ... early can have vision problems that progress to blindness and problems with mental function that get worse. ...

  4. Topographic variabilities of immunoreactivity to subunit c of mitochondrial ATP synthase and lectin binding in late infantile neuronal ceroid-lipofuscinosis.

    PubMed

    Kida, E; Wisniewski, K E; Connell, F

    1995-06-01

    A subset of lipophilic neurons in the brain tissue of late infantile neuronal ceroid lipofuscinosis (LINCL) cases shows in addition to finely granular storage lipopigment, larger spheroidal lysosomal inclusions, so called protein-type myoclonus bodies. Their incidence, significance, and biochemical composition have not been determined. To further characterize this type of lysosomal storage material, immunocytochemistry to subunit c of mitochondrial ATP synthase at the light and electron microscopy level, electron microscopy, and lectin histochemistry were applied. The majority of spheroidal inclusions were nonreactive to subunit c, the main protein component of the storage material in LINCL. These inclusions also showed no binding sites for the eight lectins examined, although six of the lectins used labeled finely granular storage material. According to electron and immunoelectron microscopy, spheroidal inclusions were composed of more homogeneous and more densely arranged material than typical curvilinear profiles, with shorter membranous profiles and sometime filamentous structures. The dissimilarities disclosed between finely granular lipopigment with curvilinear profiles and spheroidal inclusions in LINCL brain tissue suggest that either protein(s) other than subunit c are present in spheroidal inclusions, or subunit c in these sites undergoes conformational or proteolytic changes. These changes require further biochemical evaluations.

  5. Altered glutamate receptor function in the cerebellum of the Ppt1−/− mouse, a murine model of infantile neuronal ceroid lipofuscinosis

    PubMed Central

    Finn, Rozzy; Kovács, Attila D.; Pearce, David A.

    2011-01-01

    The neuronal ceroid lipofuscinoses (NCLs) are a family of devastating pediatric neurodegenerative disorders and currently represent the most common form of pediatric-onset neurodegeneration. Infantile NCL (INCL), the most aggressive of these disorders, is caused by mutations in the CLN1 gene that encodes the enzyme palmitoyl protein thioesterase 1 (PPT1). Previous studies have suggested that glutamatergic neurotransmission may be disrupted in INCL, and therefore, the present study investigates glutamate receptor function in the Ppt1−/− mouse model of INCL by comparing the sensitivity of cultured WT and Ppt1−/− cerebellar granule cells to glutamate receptor-mediated toxicity. Ppt1−/− neurons were significantly less sensitive to AMPA receptor-mediated toxicity but markedly more vulnerable to NMDA receptor-mediated cell death. Since glutamate receptor function is primarily regulated by the surface expression level of the receptor, the surface level of AMPA and NMDA receptor subunits in the cerebella of WT and Ppt1−/− mice was also examined. Western blotting of surface cross-linked cerebellar samples showed a significantly lower surface level of the GluR4 AMPA receptor subunit in Ppt1−/− mice, providing a plausible explanation for the decreased vulnerability of Ppt1−/− cerebellar neurons to AMPA receptor-mediated cell death. The surface expression of the NR1, NR2A and NR2B NMDA receptor subunits was similar in the cerebella of WT and Ppt1−/− mice, indicating that there is another mechanism behind the increased sensitivity of Ppt1−/− cerebellar granule cells to NMDA toxicity. Our results indicate an AMPA receptor hypo- and NMDA receptor hyperfunction phenotype in Ppt1−/− neurons and provide new therapeutic targets for INCL. PMID:21971706

  6. Expression profile of a Caenorhabditis elegans model of adult neuronal ceroid lipofuscinosis reveals down regulation of ubiquitin E3 ligase components

    PubMed Central

    McCue, Hannah V.; Chen, Xi; Barclay, Jeff W.; Morgan, Alan; Burgoyne, Robert D.

    2015-01-01

    Cysteine string protein (CSP) is a chaperone of the Dnaj/Hsp40 family of proteins and is essential for synaptic maintenance. Mutations in the human gene encoding CSP, DNAJC5, cause adult neuronal ceroid lipofucinosis (ANCL) which is characterised by progressive dementia, movement disorders, seizures and premature death. CSP null models in mice, flies and worms have been shown to also exhibit similar neurodegenerative phenotypes. Here we have explored the mechanisms underlying ANCL disease progression using Caenorhaditis elegans mutant strains of dnj-14, the worm orthologue of DNAJC5. Transcriptional profiling of these mutants compared to control strains revealed a broad down-regulation of ubiquitin proteasome system (UPS)-related genes, in particular, components of multimeric RING E3 ubiquitin ligases including F-Box, SKR and BTB proteins. These data were supported by the observation that dnj-14 mutant worm strains expressing a GFP-tagged ubiquitin fusion degradation substrate exhibited decreased ubiquitylated protein degradation. The results indicate that disruption of an essential synaptic chaperone leads to changes in expression levels of UPS-related proteins which has a knock-on effect on overall protein degradation in C. elegans. The specific over-representation of E3 ubiquitin ligase components revealed in our study, suggests that proteins and complexes upstream of the proteasome itself may be beneficial therapeutic targets. PMID:26395859

  7. Palmitoyl-protein thioesterase gene expression in the developing mouse brain and retina: implications for early loss of vision in infantile neuronal ceroid lipofuscinosis.

    PubMed

    Zhang, Z; Mandal, A K; Wang, N; Keck, C L; Zimonjic, D B; Popescu, N C; Mukherjee, A B

    1999-04-29

    Mutations in the palmitoyl-protein thioesterase (PPT) gene cause infantile neuronal ceroid lipofuscinosis (INCL), the clinical manifestations of which include the early loss of vision followed by deterioration of brain functions. To gain insight into the temporal onset of these clinical manifestations, we isolated and characterized a murine PPT (mPPT)-cDNA, mapped the gene on distal chromosome 4, and studied its expression in the eye and in the brain during development. Our results show that both cDNA and protein sequences of the murine and human PPTs are virtually identical and that the mPPT expression in the retina and in the brain is temporally regulated during development. Furthermore, the retinal expression of mPPT occurs much earlier and at a higher level than in the brain at all developmental stages investigated. Since many retinal and brain proteins are highly palmitoylated and depalmitoylation by PPT is essential for their effective recycling in the lysosomes, our results raise the possibility that inactivating mutations of the PPT gene, as occur in INCL, are likely to cause cellular accumulation of lipid-modified proteins in the retina earlier than in the brain. Consequently, the loss of vision occurs before the deterioration of brain functions in this disease. PMID:10231585

  8. Identification of YAC clones for human chromosome 1p32 and physical mapping of the infantile neuronal ceroid lipofuscinosis (INCL) locus

    SciTech Connect

    Hellsten, E.; Vesa, J.; Peltonen, L.

    1995-01-20

    Infantile neuronal ceroid lipofuscinosis (INCL, CLN1) is a neurodegenerative disorder in which the biochemical defect is unknown. We earlier assigned the disease locus to chromosome 1p32 in the immediate vicinity of the highly informative HY-TM1 marker by linkage and linkage disequilibrium analysis. Here we report the construction of PFGE maps on the CLN1 region covering a total of 4 Mb of this relatively poorly mapped chromosomal region. We established the order of loci at 1p32 as tel-D1S57-L-myc-HY-TM1-rlf-COL9A2-D1S193-D1S62-D1S211-cen by combining data obtained from analysis of a chromosome 1 somatic cell hybrid panel, PFGE, and interphase FISH. We isolated YACs and constructed two separate YAC contigs, the loci L-myc, HY-TM1, rlf, and COL9A2 being present on a 1000-kb contig and the markers D1S193, D1S62, and D1S211 on a YAC contig spanning a maximum of 860 kb. Within the 1000-kb contig we were able to identify five CpG islands in addition to those associated with the earlier cloned genes. The YAC contigs as well as the physical map provide us with tools for the identification of the INCL gene. 36 refs., 4 figs., 3 tabs.

  9. Partial genetic suppression of a loss-of-function mutant of the neuronal ceroid lipofuscinosis-associated protease TPP1 in Dictyostelium discoideum.

    PubMed

    Phillips, Jonathan E; Gomer, Richard H

    2015-02-01

    Neuronal ceroid lipofuscinosis (NCL) is the most common childhood-onset neurodegenerative disease. NCL is inevitably fatal, and there is currently no treatment available. Children with NCL show a progressive decline in movement, vision and mental abilities, and an accumulation of autofluorescent deposits in neurons and other cell types. Late-infantile NCL is caused by mutations in the lysosomal protease tripeptidyl peptidase 1 (TPP1). TPP1 cleaves tripeptides from the N-terminus of proteins in vitro, but little is known about the physiological function of TPP1. TPP1 shows wide conservation in vertebrates but it is not found in Drosophila, Caenorhabditis elegans or Saccharomyces cerevisiae. Here, we characterize ddTpp1, a TPP1 ortholog present in the social amoeba Dictyostelium discoideum. Lysates from cells lacking ddTpp1 show a reduced but not abolished ability to cleave a TPP1 substrate, suggesting that other Dictyostelium enzymes can perform this cleavage. ddTpp1 and human TPP1 localize to the lysosome in Dictyostelium, indicating conserved function and trafficking. Cells that lack ddTpp1 show precocious multicellular development and a reduced ability to form spores during development. When cultured in autophagy-stimulating conditions, cells lacking ddTpp1 rapidly decrease in size and are less viable than wild-type cells, suggesting that one function of ddTpp1 could be to limit autophagy. Cells that lack ddTpp1 exhibit strongly impaired development in the presence of the lysosome-perturbing drug chloroquine, and this phenotype can be suppressed through a secondary mutation in the gene that we name suppressor of tpp1(-) A (stpA), which encodes a protein with some similarity to mammalian oxysterol-binding proteins (OSBPs). Taken together, these results suggest that targeting specific proteins could be a viable way to suppress the effects of loss of TPP1 function. PMID:25540127

  10. Microdeletion in a Moroccan patient with juvenile neuronal ceroid lipofuscinosis (Batten disease) may affect the CLN3 gene

    SciTech Connect

    Taschner, P.E.M.; De Vos, N.; Briuning, M.H.

    1994-09-01

    CLN3 has been localized genetically to 16p12 and shows a strong association with microsatellite markers D16S298, D16S299, and D16S288. Recently, haplotype analysis of a Batten patient from a consanguineous, relationship with these markers indicated homozygosity for the D16S298 null allele. Null alleles for polymorphic microsatellite markers have been observed to arise from small deletions within one of the priming sequences. PCR analysis with different primers and Southern blot analysis using DNA from the patient and his family indicate a deletion of at least 160 bp. A cosmid contig containing D16S298 has been constructed. Cosmid NL 11A spans the deletion, which is located within a 25 kb EcoRI fragment. The isolation of cDNA clones from this region could lead to the identification of the CLN3 gene.

  11. Ceroid in fish

    USGS Publications Warehouse

    Wood, E.M.; Yasutake, W.T.

    1956-01-01

    Since the original description of ceroid in rats, many papers have appeared on the etiology and characteristics of this pigment. It was first seen as a yellow, granular pigment in hematoxylin and eosin sections of the cirrhotic livers of choline deficient rats. The pigment was more fully characterized by Endicott and Lillie, and additional stainging reactions were summarized recently by Lillie. The pigment is sudanophilic in paraffin sections, acid-fast, basophilic, isotropic, iron negative, and highly resistant to solution in water, alcohol, fat solvents, and dilute aqueous acids and alkalis. It is stained by Mallory's hemofuscin stain and Weigert's myelin stain. It reduces osmium tetraoxide and diamine silver carbonate but not ferric ferricyanide. The Gmelin reactions for bile pigments is negative. It has a greenish yellow fluorescence at 3650-3660 Å. It is Schiff positive with or without antecedent diastase digestion after performic or periodic acid oxidation.

  12. Defined chromosomal assignment of CLN5 demonstrates that at least four genetic loci are involved in the pathogenesis of human ceroid lipofuscinoses

    SciTech Connect

    Savukoski, M.; Peltonen, L.; Santavuori, P.; Kestilae, M. |; Williams, R.; Jaervelae, I.; Sharp, J.; Harris, J.; Gardiner, M.

    1994-10-01

    We demonstrate here that at least four genetically separate loci are involved in the pathogenesis of human neuronal ceroid lipofuscinoses (NCLs), fatal brain disorders of children. Earlier the assignments of the infantile and juvenile subtypes of NCL to 1p32 and 16p12 had revealed two loci; and here a variant subtype of the late-infantile form of NCL is mapped to a well-defined region on 13q21.1-q32, whereas the clinically similar, classical form of late-infantile NCL was found to represent the fourth, yet-unidentified NCL locus. The linkage disequilibrium was crucial for locus assignment in our highly limited family material, and the data exemplify the significance of this phenomenon in the random mapping of rare human diseases. 22 refs., 4 figs., 3 tabs.

  13. Activation of Strychnine-Sensitive Glycine Receptors by Shilajit on Preoptic Hypothalamic Neurons of Juvenile Mice.

    PubMed

    Bhattarai, Janardhan Prasad; Cho, Dong Hyu; Han, Seong Kyu

    2016-02-29

    Shilajit, a mineral pitch, has been used in Ayurveda and Siddha system of medicine to treat many human ailments, and is reported to contain at least 85 minerals in ionic form. This study examined the possible mechanism of Shilajit action on preoptic hypothalamic neurons using juvenile mice. The hypothalamic neurons are the key regulator of many hormonal systems. In voltage clamp mode at a holding potential of -60 mV, and under a high chloride pipette solution, Shilajit induced dose-dependent inward current. Shilajit-induced inward currents were reproducible and persisted in the presence of 0.5 μM tetrodotoxin (TTX) suggesting a postsynaptic action of Shilajit on hypothalamic neurons. The currents induced by Shilajit were almost completely blocked by 2 μM strychnine (Stry), a glycine receptor antagonist. In addition, Shilajit-induced inward currents were partially blocked by bicuculline. Under a gramicidin-perforated patch clamp mode, Shilajit induced membrane depolarization on juvenile neurons. These results show that Shilajit affects hypothalamic neuronal activities by activating the Stry-sensitive glycine receptor with α₂/α₂β subunit. Taken together, these results suggest that Shilajit contains some ingredients with possible glycine mimetic activities and might influence hypothalamic neurophysiology through activation of Stry-sensitive glycine receptor-mediated responses on hypothalamic neurons postsynaptically. PMID:26875561

  14. Juvenile-onset motor neuron disease caused by novel mutations in β-hexosaminidase

    PubMed Central

    Pierson, Tyler Mark; Torres, Paola A.; Zeng, Bei-Jin; Glanzman, Allan M.; Adams, David; Finkel, Richard S.; Mahuran, Don J.; Pastores, Gregory M.; Tennekoon, Gihan I.; Kolodny, Edwin H.

    2013-01-01

    A 12 year-old female presented with a seven-year history of progressive muscle weakness, atrophy, tremor and fasciculations. Cognition was normal. Rectal biopsy revealed intracellular storage material and biochemical testing indicated low hexosaminidase activity consistent with juvenile-onset GM2-gangliosidosis. Genetic evaluation revealed compound heterozygosity with two novel mutations in the hexosaminidase β-subunit (c.512-3 C>A and c.1613+15_1613+18dup). Protein analysis was consistent with biochemical findings and indicated only a small portion of β-subunits were properly processed. These results provide additional insight into juvenile-onset GM2-gangliosidoses and further expand the number of β-hexosaminidase mutations associated with motor neuron disease. PMID:23158871

  15. Morphological alterations in neocortical and cerebellar GABAergic neurons in a canine model of juvenile Batten disease.

    PubMed

    March, P A; Wurzelmann, S; Walkley, S U

    1995-06-01

    The pathogenesis of brain dysfunction in a canine model of juvenile Batten disease was studied with techniques designed to determine sequential changes in mitochondrial morphology and cytochrome oxidase (CO) activity, and in neurons and synapses using gamma-aminobutyric acid (GABA) as a neurotransmitter. Histochemical and immunocytochemical methods were employed. Mitochondrial alterations were found in a select population of nonpyramidal neurons in neocortex and claustrum, and in cerebellar basket cells. Proportions of affected neurons at any one time remained constant over the disease course, with morphologically-abnormal mitochondria first being recognized at age 6 months. Enlarged mitochondria were readily identifiable at the light microscope (LM) level as large CO-positive or mitochondrial antibody-positive granular structures. Colabelling with antibodies to GABA or to parvalbumin (PV) indicated that most of these cells were GABAergic. Ultrastructurally, atypical mitochondria were characterized by globular enlargement, intramitochondrial membranous inclusions, and disorganized internal structure. CO activity in all other cell somata and in neuropil was diminished compared with normal, age-matched tissue. Glutamic acid decarboxylase (GAD), PV, and GABA studies demonstrated loss of GABAergic neurons and synapses in cortex and cerebellum of affected dogs. These results indicate that abnormal mitochondria are present in neurons in Batten disease, and suggest that suboptimal mitochondrial function may play a role in the pathogenic mechanisms of brain dysfunction in this disorder.

  16. Genetic heterogeneity in juvenile NCL

    SciTech Connect

    Hart, Y.M.; Andermann, E.; Mitchison, H.M.

    1994-09-01

    The neuronal ceroid lipofuscinoses (NCL) are a group of related lysosomal storage diseases classified according to the age of onset, clinical syndrome, and pathology. The clinical syndromes include myoclonus, visual failure, progressive dementia, ataxia and generalized tonic clonic seizures in varying combinations depending on the age of onset and pathology. The mode of inheritance is autosomal recessive in most cases, except for several families with the adult form (Kufs` disease) which have autosomal dominant inheritance. Linkage for the infantile (Halatia-Santavuori) form (CLN1), characterized ultrastructurally by lysosomal granular osmiophilic deposits (GROD), has been demonstrated with markers on chromosome lp, while the gene for the typical juvenile (Spielmeyer-Vogt) form (CLN3), characterized by fingerprint-profile inclusions, has been linked to chromosome 16p. The gene locations of the late infantile (Jansky-Bielschowsky) and adult (Kufs` disease) forms are unknown, although it has recently been shown that the late infantile form does not link to chromosome 16p. We describe three siblings, including a pair of monozygotic twins, with juvenile onset NCL with GROD in whom linkage to the CLN3 region of chromsome 16p has been excluded. This would suggest that there is genetic heterogeneity not only among the different clinical syndromes, but also among identical clinical syndromes with different ultrastructural characteristics. Preliminary studies of linkage to chromosome 1p employing the microsatellite marker HY-TM1 have been uninformative. Further studies with other chromosome 1 markers are underway.

  17. Aberrant neuronal avalanches in cortical tissue removed from juvenile epilepsy patients.

    PubMed

    Hobbs, Jon P; Smith, Jodi L; Beggs, John M

    2010-12-01

    Some forms of epilepsy may arise as a result of pathologic interactions among neurons. Many forms of collective activity have been identified, including waves, spirals, oscillations, synchrony, and neuronal avalanches. All these emergent activity patterns have been hypothesized to show pathologic signatures associated with epilepsy. Here, the authors used 60-channel multielectrode arrays to record neuronal avalanches in cortical tissue removed from juvenile epilepsy patients. For comparison, they also recorded activity in rat cortical slices. The authors found that some human tissue removed from epilepsy patients exhibited prolonged periods of hyperactivity not seen in rat slices. In addition, they found a positive correlation between the branching parameter, a measure of network gain, and firing rate in human slices during periods of hyperactivity. This relationship was not present in rat slices. The authors suggest that this positive correlation between the branching parameter and the firing rate is part of a positive feedback loop and may contribute to some forms of epilepsy. These results also indicate that neuronal avalanches are abnormally regulated in slices removed from pediatric epilepsy patients.

  18. Quiescent neuronal progenitors are activated in the juvenile guinea pig lateral striatum and give rise to transient neurons.

    PubMed

    Luzzati, Federico; Nato, Giulia; Oboti, Livio; Vigna, Elisa; Rolando, Chiara; Armentano, Maria; Bonfanti, Luca; Fasolo, Aldo; Peretto, Paolo

    2014-11-01

    In the adult brain, active stem cells are a subset of astrocytes residing in the subventricular zone (SVZ) and the dentate gyrus (DG) of the hippocampus. Whether quiescent neuronal progenitors occur in other brain regions is unclear. Here, we describe a novel neurogenic system in the external capsule and lateral striatum (EC-LS) of the juvenile guinea pig that is quiescent at birth but becomes active around weaning. Activation of neurogenesis in this region was accompanied by the emergence of a neurogenic-like niche in the ventral EC characterized by chains of neuroblasts, intermediate-like progenitors and glial cells expressing markers of immature astrocytes. Like neurogenic astrocytes of the SVZ and DG, these latter cells showed a slow rate of proliferation and retained BrdU labeling for up to 65 days, suggesting that they are the primary progenitors of the EC-LS neurogenic system. Injections of GFP-tagged lentiviral vectors into the SVZ and the EC-LS of newborn animals confirmed that new LS neuroblasts originate from the activation of local progenitors and further supported their astroglial nature. Newborn EC-LS neurons existed transiently and did not contribute to neuronal addition or replacement. Nevertheless, they expressed Sp8 and showed strong tropism for white matter tracts, wherein they acquired complex morphologies. For these reasons, we propose that EC-LS neuroblasts represent a novel striatal cell type, possibly related to those populations of transient interneurons that regulate the development of fiber tracts during embryonic life. PMID:25336736

  19. Strong, reliable and precise synaptic connections between thalamic relay cells and neurones of the nucleus reticularis in juvenile rats

    PubMed Central

    Gentet, Luc J; Ulrich, Daniel

    2003-01-01

    The thalamic reticular nucleus (nRT) is composed entirely of GABAergic inhibitory neurones that receive input from pyramidal cortical neurones and excitatory relay cells of the ventrobasal complex of the thalamus (VB). It plays a major role in the synchrony of thalamic networks, yet the synaptic connections it receives from VB cells have never been fully physiologically characterised. Here, whole-cell current-clamp recordings were obtained from 22 synaptically connected VB-nRT cell pairs in slices of juvenile (P14–20) rats. At 34–36 °C, single presynaptic APs evoked unitary EPSPs in nRT cells with a peak amplitude of 7.4 ± 1.5 mV (mean ± s.e.m.) and a decay time constant of 15.1 ± 0.9 ms. Only four out of 22 pairs showed transmission failures at a mean rate of 6.8 ± 1.1 %. An NMDA receptor (NMDAR)-mediated component was significant at rest and subsequent EPSPs in a train were depressed. Only one out of 14 pairs tested was reciprocally connected; the observed IPSPs in the VB cell had a peak amplitude of 0.8 mV and were completely abolished in the presence of 10 μm bicuculline. Thus, synaptic connections from VB cells to nRT neurones are mainly ‘drivers’, while a small subset of cells form closed disynaptic loops. PMID:12563005

  20. Genetics Home Reference: late-infantile neuronal ceroid lipofuscinosis

    MedlinePlus

    ... endoplasmic reticulum . The endoplasmic reticulum is involved in protein production, processing, and transport. Within these cell structures, the ... CLN8 , MFSD8 , or PPT1 gene usually reduce the production or activity of the particular protein or enzyme made from the gene. In many ...

  1. Laminar Differences in Dendritic Structure of Pyramidal Neurons in the Juvenile Rat Somatosensory Cortex.

    PubMed

    Rojo, Concepción; Leguey, Ignacio; Kastanauskaite, Asta; Bielza, Concha; Larrañaga, Pedro; DeFelipe, Javier; Benavides-Piccione, Ruth

    2016-06-01

    Pyramidal cell structure varies between different cortical areas and species, indicating that the cortical circuits that these cells participate in are likely to be characterized by different functional capabilities. Structural differences between cortical layers have been traditionally reported using either the Golgi method or intracellular labeling, but the structure of pyramidal cells has not previously been systematically analyzed across all cortical layers at a particular age. In the present study, we investigated the dendritic architecture of complete basal arbors of pyramidal neurons in layers II, III, IV, Va, Vb, and VI of the hindlimb somatosensory cortical region of postnatal day 14 rats. We found that the characteristics of basal dendritic morphologies are statistically different in each cortical layer. The variations in size and branching pattern that exist between pyramidal cells of different cortical layers probably reflect the particular functional properties that are characteristic of the cortical circuit in which they participate. This new set of complete basal dendritic arbors of 3D-reconstructed pyramidal cell morphologies across each cortical layer will provide new insights into interlaminar information processing in the cerebral cortex.

  2. Laminar Differences in Dendritic Structure of Pyramidal Neurons in the Juvenile Rat Somatosensory Cortex.

    PubMed

    Rojo, Concepción; Leguey, Ignacio; Kastanauskaite, Asta; Bielza, Concha; Larrañaga, Pedro; DeFelipe, Javier; Benavides-Piccione, Ruth

    2016-06-01

    Pyramidal cell structure varies between different cortical areas and species, indicating that the cortical circuits that these cells participate in are likely to be characterized by different functional capabilities. Structural differences between cortical layers have been traditionally reported using either the Golgi method or intracellular labeling, but the structure of pyramidal cells has not previously been systematically analyzed across all cortical layers at a particular age. In the present study, we investigated the dendritic architecture of complete basal arbors of pyramidal neurons in layers II, III, IV, Va, Vb, and VI of the hindlimb somatosensory cortical region of postnatal day 14 rats. We found that the characteristics of basal dendritic morphologies are statistically different in each cortical layer. The variations in size and branching pattern that exist between pyramidal cells of different cortical layers probably reflect the particular functional properties that are characteristic of the cortical circuit in which they participate. This new set of complete basal dendritic arbors of 3D-reconstructed pyramidal cell morphologies across each cortical layer will provide new insights into interlaminar information processing in the cerebral cortex. PMID:26762857

  3. Laminar Differences in Dendritic Structure of Pyramidal Neurons in the Juvenile Rat Somatosensory Cortex

    PubMed Central

    Rojo, Concepción; Leguey, Ignacio; Kastanauskaite, Asta; Bielza, Concha; Larrañaga, Pedro; DeFelipe, Javier; Benavides-Piccione, Ruth

    2016-01-01

    Pyramidal cell structure varies between different cortical areas and species, indicating that the cortical circuits that these cells participate in are likely to be characterized by different functional capabilities. Structural differences between cortical layers have been traditionally reported using either the Golgi method or intracellular labeling, but the structure of pyramidal cells has not previously been systematically analyzed across all cortical layers at a particular age. In the present study, we investigated the dendritic architecture of complete basal arbors of pyramidal neurons in layers II, III, IV, Va, Vb, and VI of the hindlimb somatosensory cortical region of postnatal day 14 rats. We found that the characteristics of basal dendritic morphologies are statistically different in each cortical layer. The variations in size and branching pattern that exist between pyramidal cells of different cortical layers probably reflect the particular functional properties that are characteristic of the cortical circuit in which they participate. This new set of complete basal dendritic arbors of 3D-reconstructed pyramidal cell morphologies across each cortical layer will provide new insights into interlaminar information processing in the cerebral cortex. PMID:26762857

  4. Preparation of artificial ceroid/lipofuscin by UV-oxidation of subcellular organelles.

    PubMed

    Nilsson, E; Yin, D

    1997-12-01

    Recent studies have consistently shown that, during oxidative damage, glycation, and other oxygen stress-related reactions, various biomolecules are converted into ceroid- and lipofuscin-like fluorescent pigments. In this study, artificial ceroid/lipofuscin was produced by exposing rat liver fractions to UV-light overnight. Thiobarbituric acid reactive substances (TBARS) were formed in increasing amounts during the early stages of the process, but decreased as the material was later converted into a polymeric structure with few remaining peroxides. In the transmission electron microscope the artificial pigment showed lamellar structures and was osmiophilic. By energy-dispersive X-ray analysis the material was found to contain Ca and Fe in the same way as natural ceroid/lipofuscin. Moreover, it exhibited ceroid/lipofuscin-like, greenish-yellowish autofluorescence when assayed by microfluorometry, with a fluorescence maximum consistently found at 430 nm when excited at 350 nm. Identical fluorescence maxima were found for each fraction of rat liver that was used as the origin of the pigments, i.e. nuclei, mitochondria, lysosomes and microsomes. Extracts with either chloroform-methanol, or sodium dodecylsulphate, showed identical complex fluorescence. When the pigments were extracted by chloroform-methanol, five fluorescent bands were obtained after thin-layer chromatographic separation. Fibroblasts were found to endocytose the material, a process that converted them into lipofuscin-loaded cells of an aged phenotype as observed by light and electron microscopy. Similar fluorescence emission spectra were obtained from cells grown at 40% O2, in order to stimulate endogenous lipofuscin-formation, and from cells exposed to artificial ceroid/lipofuscin. The described technique for creating artificial ceroid/lipofuscin is relatively easy to perform and should provide a useful new tool to study the possible influences of ceroid/lipofuscin on lysosomal and cellular functions

  5. Immunoreactivity for neuronal NOS and fluorescent indication of NO formation in the NTS of juvenile rats submitted to chronic intermittent hypoxia.

    PubMed

    Pajolla, Gisela P; Accorsi-Mendonça, Daniela; Lunardi, Claure N; Bendhack, Lusiane M; Machado, Benedito H; Llewellyn-Smith, Ida J

    2009-06-15

    Exposure to chronic intermittent hypoxia (CIH) leads to significant autonomic and respiratory changes, similar to those observed in obstructive sleep apnea. The hypertension associated with CIH is due to sympathoexcitation triggered by long-term exposure to intermittent hypoxia. However, the mechanisms underlying these effects are unknown. Changes in central regulation of sympathetic activity may underlie CIH-induced hypertension. Since NO appears to be mainly sympathoinhibitory in the nucleus of the solitary tract (NTS), we hypothesized that CIH augments sympathetic activity, in part by reducing neuronal nitric oxide synthase (nNOS) expression and consequently nitric oxide (NO) production in this brain region. To test our hypothesis, juvenile male Wistar rats were exposed to CIH for 8 h/day for 10 days and sections of perfused brainstem were either stained to reveal nNOS-immunoreactivity or loaded with DAF 2-DA to label neurons containing NO. CIH rats showed a significant increase in mean arterial pressure and heart rate compared to controls. However, there was no significant difference in the distribution, staining intensity or numbers of nNOS-immunoreactive neurons in the NTS between experimental and control rats. We also found no significant change in NO content in the DAF 2-DA-loaded sections of NTS from CIH rats. Our data show that NO is not altered in the NTS of juvenile CIH rats, suggesting that nitrergic mechanisms, at least in the NTS, are unlikely to be involved in the sympathetic excitation that generates the hypertension observed after 10 days of CIH.

  6. Juvenile angiofibroma

    MedlinePlus

    Nasal tumor; Angiofibroma - juvenile; Benign nasal tumor; Juvenile nasal angiofibroma; JNA ... Juvenile angiofibroma is not very common. It is most often found in adolescent boys. The tumor contains ...

  7. Osmotic Edema Rapidly Increases Neuronal Excitability Through Activation of NMDA Receptor-Dependent Slow Inward Currents in Juvenile and Adult Hippocampus

    PubMed Central

    Lauderdale, Kelli; Murphy, Thomas; Tung, Tina; Davila, David; Binder, Devin K.

    2015-01-01

    Cellular edema (cell swelling) is a principal component of numerous brain disorders including ischemia, cortical spreading depression, hyponatremia, and epilepsy. Cellular edema increases seizure-like activity in vitro and in vivo, largely through nonsynaptic mechanisms attributable to reduction of the extracellular space. However, the types of excitability changes occurring in individual neurons during the acute phase of cell volume increase remain unclear. Using whole-cell patch clamp techniques, we report that one of the first effects of osmotic edema on excitability of CA1 pyramidal cells is the generation of slow inward currents (SICs), which initiate after approximately 1 min. Frequency of SICs increased as osmolarity decreased in a dose-dependent manner. Imaging of real-time volume changes in astrocytes revealed that neuronal SICs occurred while astrocytes were still in the process of swelling. SICs evoked by cell swelling were mainly nonsynaptic in origin and NMDA receptor-dependent. To better understand the relationship between SICs and changes in neuronal excitability, recordings were performed in increasingly physiological conditions. In the absence of any added pharmacological reagents or imposed voltage clamp, osmotic edema induced excitatory postsynaptic potentials and burst firing over the same timecourse as SICs. Like SICs, action potentials were blocked by NMDAR antagonists. Effects were more pronounced in adult (8–20 weeks old) compared with juvenile (P15–P21) mice. Together, our results indicate that cell swelling triggered by reduced osmolarity rapidly increases neuronal excitability through activation of NMDA receptors. Our findings have important implications for understanding nonsynaptic mechanisms of epilepsy in relation to cell swelling and reduction of the extracellular space. PMID:26489684

  8. Accumulation of ceroid in smooth muscle indicates severe malabsorption and vitamin E deficiency.

    PubMed Central

    Stamp, G W; Evans, D J

    1987-01-01

    Four patients had accumulation of ceroid in smooth muscle (lipofuscinosis), which indicated severe or uncontrolled malabsorption, with confirmed vitamin E deficiency in three cases. The distribution of the pigment was systematic, and there seemed to be an association between malabsorption syndrome and vitamin E deficiency. Vitamin E supplementation seems to be indicated in such patients, and it is suggested that studies of smooth muscle function should be made in cases of heavy accumulation of ceroid. Images Fig 1 Fig 2 Fig 3 Fig 4 Fig 5 PMID:3624501

  9. Biochemical basis of lipofuscin, ceroid, and age pigment-like fluorophores.

    PubMed

    Yin, D

    1996-01-01

    Serious studies of the formation mechanisms of age-related pigments and their possible cellular influence have been hampered for a long time by discrepancies and controversies over the definition, fluorescence emission, origin, and composition of these pigments. This review discusses several critical controversies in this field and lay special emphasis on the cellular and biochemical reactions related to the formation mechanisms of lipofuscin, ceroid, advanced glycation end-products (AGEs), and age pigment like fluorophores (APFs). Various amino compounds and their reaction with secondary aldehydic products of oxygen free radical-induced oxidation, particularly lipid peroxidation, are important sources of the fluorophores of ceroid/lipofuscin, which progressively accumulate as a result of phagocytosis and autophagocytosis of modified biomaterials within secondary lysosomes of postmitotic and other cells. Lipofuscin is the classical age pigment of postmitotic cells, while ceroid accumulates due to pathologic and experimental processes. There are good reasons to consider both ceroid and lipofuscin as materials of the same principal origin. The age-related intracellular fluorophores of retinal pigment epithelium (RPE) seems to represent a special class of lipofuscin, which partly contains derivatives of retinoids and carotenoids. Saccharide-originated fluorophores, principally AGEs formed during glycation/Maillard reactions, may be mainly responsible for the extracellular fluorescence of long-lived proteins, such as collagen, elastin, and lens crystalline. Although lipofuscin, ceroid, AGEs, and APFs can be produced from different types of biological materials due to different side reactions of essential biology, the crosslinking of carbonyl-amino compounds is recognized as a common process during their formation.

  10. Shifts in excitatory/inhibitory balance by juvenile stress: A role for neuron-astrocyte interaction in the dentate gyrus.

    PubMed

    Albrecht, Anne; Ivens, Sebastian; Papageorgiou, Ismini E; Çalışkan, Gürsel; Saiepour, Nasrin; Brück, Wolfgang; Richter-Levin, Gal; Heinemann, Uwe; Stork, Oliver

    2016-06-01

    Childhood trauma is a well-described risk factor for the development of stress-related psychopathology such as posttraumatic stress disorder or depression later in life. Childhood adversity can be modeled in rodents by juvenile stress (JS) protocols, resulting in impaired coping with stressful challenges in adulthood. In the current study, we investigated the long-lasting impact of JS on the expression of molecular factors for glutamate and γ-aminobutyric acid (GABA) uptake and turnover in sublayers of the dentate gyrus (DG) using laser microdissection and quantitative real-time polymerase chain reaction. We observed reduced mRNA expression levels after JS for factors mediating astrocytic glutamate and GABA uptake and degradation. These alterations were prominently observed in the dorsal but not ventral DG granule cell layer, indicating a lasting change in astrocytic GABA and glutamate metabolism that may affect dorsal DG network activity. Indeed, we observed increased inhibition and a lack of facilitation in response to paired-pulse stimulation at short interstimulus intervals in the dorsal DG after JS, while no alterations were evident in basal synaptic transmission or forms of long-term plasticity. The shift in paired-pulse response was mimicked by pharmacologically blocking the astrocytic GABA transporter GAT-3 in naïve animals. Accordingly, reduced expression levels of GAT-3 were confirmed at the protein level in the dorsal granule cell layer of rats stressed in juvenility. Together, these data demonstrate a lasting shift in the excitatory/inhibitory balance of dorsal DG network activity by JS that appears to be mediated by decreased GABA uptake into astrocytes. PMID:26875694

  11. Mechanism of ceroid formation in atherosclerotic plaque: in situ studies using a combination of Raman and fluorescence spectroscopy

    NASA Astrophysics Data System (ADS)

    Haka, Abigail S.; Kramer, John R.; Dasari, Ramachandra R.; Fitzmaurice, Maryann

    2011-01-01

    Accumulation of the lipid-protein complex ceroid is a characteristic of atherosclerotic plaque. The mechanism of ceroid formation has been extensively studied, because the complex is postulated to contribute to plaque irreversibility. Despite intensive research, ceroid deposits are defined through their fluorescence and histochemical staining properties, while their composition remains unknown. Using Raman and fluorescence spectral microscopy, we examine the composition of ceroid in situ in aorta and coronary artery plaque. The synergy of these two types of spectroscopy allows for identification of ceroid via its fluorescence signature and elucidation of its chemical composition through the acquisition of a Raman spectrum. In accordance with in vitro predictions, low density lipoprotein (LDL) appears within the deposits primarily in its peroxidized form. The main forms of modified LDL detected in both coronary artery and aortic plaques are peroxidation products from the Fenton reaction and myeloperoxidase-hypochlorite pathway. These two peroxidation products occur in similar concentrations within the deposits and represent ~40 and 30% of the total LDL (native and peroxidized) in the aorta and coronary artery deposits, respectively. To our knowledge, this study is the first to successfully employ Raman spectroscopy to unravel a metabolic pathway involved in disease pathogenesis: the formation of ceroid in atherosclerotic plaque.

  12. GABAergic neuron deficit as an idiopathic generalized epilepsy mechanism: the role of BRD2 haploinsufficiency in juvenile myoclonic epilepsy.

    PubMed

    Velíšek, Libor; Shang, Enyuan; Velíšková, Jana; Chachua, Tamar; Macchiarulo, Stephania; Maglakelidze, Giorgi; Wolgemuth, Debra J; Greenberg, David A

    2011-01-01

    Idiopathic generalized epilepsy (IGE) syndromes represent about 30% of all epilepsies. They have strong, but elusive, genetic components and sex-specific seizure expression. Multiple linkage and population association studies have connected the bromodomain-containing gene BRD2 to forms of IGE. In mice, a null mutation at the homologous Brd2 locus results in embryonic lethality while heterozygous Brd2+/- mice are viable and overtly normal. However, using the flurothyl model, we now show, that compared to the Brd2+/+ littermates, Brd2+/- males have a decreased clonic, and females a decreased tonic-clonic, seizure threshold. Additionally, long-term EEG/video recordings captured spontaneous seizures in three out of five recorded Brd2+/- female mice. Anatomical analysis of specific regions of the brain further revealed significant differences in Brd2+/- vs +/+ mice. Specifically, there were decreases in the numbers of GABAergic (parvalbumin- or GAD67-immunopositive) neurons along the basal ganglia pathway, i.e., in the neocortex and striatum of Brd2+/- mice, compared to Brd2+/+ mice. There were also fewer GABAergic neurons in the substantia nigra reticulata (SNR), yet there was a minor, possibly compensatory increase in the GABA producing enzyme GAD67 in these SNR cells. Further, GAD67 expression in the superior colliculus and ventral medial thalamic nucleus, the main SNR outputs, was significantly decreased in Brd2+/- mice, further supporting GABA downregulation. Our data show that the non-channel-encoding, developmentally critical Brd2 gene is associated with i) sex-specific increases in seizure susceptibility, ii) the development of spontaneous seizures, and iii) seizure-related anatomical changes in the GABA system, supporting BRD2's involvement in human IGE. PMID:21887291

  13. Unbiased Cell-based Screening in a Neuronal Cell Model of Batten Disease Highlights an Interaction between Ca2+ Homeostasis, Autophagy, and CLN3 Protein Function*

    PubMed Central

    Chandrachud, Uma; Walker, Mathew W.; Simas, Alexandra M.; Heetveld, Sasja; Petcherski, Anton; Klein, Madeleine; Oh, Hyejin; Wolf, Pavlina; Zhao, Wen-Ning; Norton, Stephanie; Haggarty, Stephen J.; Lloyd-Evans, Emyr; Cotman, Susan L.

    2015-01-01

    Abnormal accumulation of undigested macromolecules, often disease-specific, is a major feature of lysosomal and neurodegenerative disease and is frequently attributed to defective autophagy. The mechanistic underpinnings of the autophagy defects are the subject of intense research, which is aided by genetic disease models. To gain an improved understanding of the pathways regulating defective autophagy specifically in juvenile neuronal ceroid lipofuscinosis (JNCL or Batten disease), a neurodegenerative disease of childhood, we developed and piloted a GFP-microtubule-associated protein 1 light chain 3 (GFP-LC3) screening assay to identify, in an unbiased fashion, genotype-sensitive small molecule autophagy modifiers, employing a JNCL neuronal cell model bearing the most common disease mutation in CLN3. Thapsigargin, a sarco/endoplasmic reticulum Ca2+-ATPase (SERCA) Ca2+ pump inhibitor, reproducibly displayed significantly more activity in the mouse JNCL cells, an effect that was also observed in human-induced pluripotent stem cell-derived JNCL neural progenitor cells. The mechanism of thapsigargin sensitivity was Ca2+-mediated, and autophagosome accumulation in JNCL cells could be reversed by Ca2+ chelation. Interrogation of intracellular Ca2+ handling highlighted alterations in endoplasmic reticulum, mitochondrial, and lysosomal Ca2+ pools and in store-operated Ca2+ uptake in JNCL cells. These results further support an important role for the CLN3 protein in intracellular Ca2+ handling and in autophagic pathway flux and establish a powerful new platform for therapeutic screening. PMID:25878248

  14. Juvenile Arthritis

    MedlinePlus

    Juvenile arthritis (JA) is arthritis that happens in children. It causes joint swelling, pain, stiffness, and loss ... common type of JA that children get is juvenile idiopathic arthritis. There are several other forms of ...

  15. A comparative study of artificial ceroid/lipofuscin from different tissue materials of rats.

    PubMed

    Wang, Xinhui; Liao, Yanyang; Li, Guolin; Yin, Dazhong; Sheng, Shuli

    2008-01-01

    The artificial ceroid/lipofuscin pigments originated from different organ tissues, including liver, brain, heart, and kidney of rats, and biomaterials were studied with improved fluorometric techniques. With all tissue materials exposed under ultraviolet (UV) light, a series of similar fluorescent colors were observed under microfluorometer. Analogous fluorescence spectra were also demonstrated with a three-dimensional (3-D) front-surface fluorometric technique despite of the tissue differences. Measured with 3-D fluorometry, relatively simple lipofuscin-like fluorophores were observed from the reactions of malondialdehyde (MDA) with critical biological macromolecules, such as bovine serum albumin (BSA) and DNA. Our results demonstrated that the biomaterials from different tissues have a similar fate under accelerated oxidative/carbonyl stresses but may be differentiated by a fluorescence intensity ratio.

  16. Juvenile Firesetting.

    PubMed

    Peters, Brittany; Freeman, Bradley

    2016-01-01

    Juvenile firesetting is a significant cause of morbidity and mortality in the United States. Male gender, substance use, history of maltreatment, interest in fire, and psychiatric illness are commonly reported risk factors. Interventions that have been shown to be effective in juveniles who set fires include cognitive behavior therapy and educational interventions, whereas satiation has not been shown to be an effective intervention. Forensic assessments can assist the legal community in adjudicating youth with effective interventions. Future studies should focus on consistent assessment and outcome measures to create more evidence for directing evaluation and treatment of juvenile firesetters. PMID:26593122

  17. Juvenile Prostitution.

    ERIC Educational Resources Information Center

    Csapo, Marg

    1986-01-01

    Recent research and Canadian government committee reports concerning juvenile prostitution are reviewed. Proposals are made in the realms of law and social policy; and existing programs are described. (DB)

  18. Postnatal maternal separation enhances tonic GABA current of cortical layer 5 pyramidal neurons in juvenile rats and promotes genesis of GABAergic neurons in neocortical molecular layer and subventricular zone in adult rats.

    PubMed

    Feng, Mei; Sheng, Guoxia; Li, Zhongxia; Wang, Jiangping; Ren, Keming; Jin, Xiaoming; Jiang, Kewen

    2014-03-01

    Postnatal maternal separation (PMS) has been shown to be associated with an increased vulnerability to psychiatric illnesses in adulthood. However, the underlying neurological mechanisms are not well understood. Here we evaluated its effects on neurogenesis and tonic GABA currents of cortical layer 5 (L5) pyramidal neurons. PMS not only increased cell proliferation in the subventricular zone, cortical layer 1 and hippocampal dentate gyrus in the adult brain, but also promoted the newly generated cells to differentiate into GABAergic neurons, and PMS adult brain maintained higher ratios of GABAergic neurons in the survival of newly generated cells within 5 days immediately post PMS. Additionally, PMS increased the tonic currents at P7-10 and P30-35 in cortical L5 pyramidal cells. Our results suggest that the newly generated GABAergic neurons and the low GABA concentration-activated tonic currents may be involved in the development of psychiatric disorders after PMS.

  19. Glucose oxidation and low-density lipoprotein-induced macrophage ceroid accumulation: possible implications for diabetic atherosclerosis.

    PubMed Central

    Hunt, J V; Bottoms, M A; Clare, K; Skamarauskas, J T; Mitchinson, M J

    1994-01-01

    The exposure of proteins to high concentrations of glucose in vitro is widely considered a relevant model of the functional degeneration of tissue occurring in diabetes mellitus. In particular, the enhanced atherosclerosis in diabetes is often discussed in terms of glycation of low-density lipoprotein (LDL), the non-enzymic attachment of glucose to apolipoprotein amino groups. However, glucose can undergo transition-metal-catalysed oxidation under near-physiological conditions in vitro, producing oxidants that possess a reactivity similar to the hydroxyl radical. These oxidants can fragment protein, hydroxylate benzoic acid and induce lipid peroxidation in human LDL. In this study, glycation of LDL in vitro is accompanied by such oxidative processes. However, the oxidation of LDL varies with glucose concentration in a manner which does not parallel changes in protein glycation. Glycation increases in proportion to glucose concentration, whereas in our studies maximal oxidation occurs at a glucose concentration of approx. 25 mM. The modification of LDL resulting from exposure to glucose alters macrophage ceroid accumulation, a process which occurs in the human atherosclerotic plaque. The accumulation of ceroid in macrophages is shown to be related to LDL oxidation rather than LDL glycation, per se, as it too occurs at a maximum of approx. 25 mM. Oxidative sequelae of protein glycation appear to be a major factor in LDL-macrophage interactions, at least with respect to ceroid accumulation. Our observations are discussed in the context of the observed increase in the severity of atherosclerosis in diabetes. PMID:8198540

  20. Juvenile idiopathic arthritis

    MedlinePlus

    Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ... The cause of juvenile idiopathic arthritis (JIA) is not known. It is thought to be an autoimmune illness . This means the body attacks ...

  1. Juvenile Spondyloarthritis

    PubMed Central

    Gmuca, Sabrina; Weiss, Pamela F.

    2015-01-01

    Purpose of review To provide a comprehensive update of the pathogenesis, diagnostic imaging, treatments, and disease activity measurements of juvenile spondyloarthritis (JSpA). Recent findings Genetic and microbiome studies have provided new information regarding possible pathogenesis of JSpA. Recent work suggests that children with JSpA have decreased thresholds for pain in comparison to healthy children. Additionally, pain on physical examination and abnormalities on ultrasound of the entheses are not well correlated. Treatment guidelines for juvenile arthritis, including JSpA, were published by the American College of Rheumatology and are based on active joint count and presence of sacroiliitis. Recent studies have established the efficacy of tumor necrosis factor inhibitors in the symptomatic treatment of axial disease, though their efficacy for halting progression of structural damage is less clear. Newly developed disease activity measures for JSpA include the Juvenile Arthritis Disease Activity Score and the JSpA Disease Activity index. In comparison to other categories of juvenile arthritis, children with JSpA are less likely to attain and sustain inactive disease. Summary Further microbiome and genetic research may help elucidate JSpA pathogenesis. More randomized therapeutic trials are needed and the advent of new composite disease activity measurement tools will hopefully allow for the design of these greatly needed trials. PMID:26002028

  2. Baicalin Activates Glycine and γ-Aminobutyric Acid Receptors on Substantia Gelatinosa Neurons of the Trigeminal Subsnucleus Caudalis in Juvenile Mice.

    PubMed

    Yin, Hua; Bhattarai, Janardhan Prasad; Oh, Sun Mi; Park, Soo Joung; Ahn, Dong Kuk; Han, Seong Kyu

    2016-01-01

    The substantia gelatinosa (SG) of the trigeminal subnucleus caudalis (Vc) receives nociceptive afferent inputs from thin-myelinated A[Formula: see text] fibers and unmyelinated C fibers and has been shown to be involved in the processing of orofacial nociceptive information. Scutellaria baicalensis Georgi (Huang-Qin, SbG), one of the 50 fundamental herbs of Chinese herbology, has been used historically as anti-inflammatory and antineoplastic medicine. Baicalin, one of the major compounds of SbG, has been reported to have neuroprotective, anti-inflammatory and analgesic effects. However, the receptor type activated by baicalin and its precise action mechanism on the SG neurons of Vc have not yet been studied. The whole-cell patch clamp technique was performed to examine the ion channels activated by baicalin on the SG neurons of Vc. In high Cl[Formula: see text] pipette solution, the baicalin (300[Formula: see text][Formula: see text]M) induced repeatable inward currents ([Formula: see text][Formula: see text]pA, [Formula: see text]) without desensitization on all the SG neurons tested. Further, the inward currents showed a concentration (0.1-3[Formula: see text]mM) dependent pattern. The inward current was sustained in the presence of tetrodotoxin (0.5[Formula: see text][Formula: see text]M), a voltage sensitive Na[Formula: see text] channel blocker. In addition, baicalin-induced inward currents were reduced in the presence of picrotoxin (50[Formula: see text][Formula: see text]M), a GABAA receptor antagonist, flumazenil (100[Formula: see text][Formula: see text]M), a benzodiazepine-sensitive GABAA receptor antagonist, and strychnine (2[Formula: see text][Formula: see text]M), a glycine receptor antagonist, respectively. These results indicate that baicalin has inhibitory effects on the SG neurons of the Vc, which are due to the activation of GABAA and/or the glycine receptor. Our results suggest that baicalin may be a potential target for orofacial pain modulation

  3. Accumulation of dolichol-linked oligosaccharides in ceroid-lipofuscinosis (Batten disease).

    PubMed

    Hall, N A; Patrick, A D

    1988-01-01

    The accumulation of phosphorylated dolichol compounds in a number of tissues from cases of ceroid-lipofuscinosis (CL) is documented, together with an analysis of their complex carbohydrate structures. Oligosaccharides were released from dolichyl pyrophosphoryl compounds, partially purified from brain, either by mild acid hydrolysis or endoglucosaminidase digestion. The molar amounts of oligosaccharides released corresponded to the levels of P-dolichol in each brain analysed. Qualitative analysis indicated that the oligosaccharides from brain consist of a number of different components, ranging in size from four to fourteen monosaccharide units and containing chitobiose at the reducing terminal, and that the species containing seven or eight monosaccharides can be fully digested to a trisaccharide by alpha-mannosidase. The compounds that accumulate in CL tissues probably represent some of the lipid-linked intermediates known to be involved in the glycosylation of proteins, together with metabolites derived from these intermediates. The results suggest that CL might result from an impairment of the ability to metabolize dolichyl pyrophosphoryl oligosaccharides. PMID:3146320

  4. [Juvenile angiofibroma].

    PubMed

    Thuesen, Anne Daugaard; Jakobsen, John; Nepper-Rasmussen, Jørgen

    2005-08-22

    Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in Denmark each year. It sits in the foramen sphenopalatinum and occurs in boys from 14 to 25 years of age. The most frequent initial symptoms are nasal obstruction and epistaxis. Through the years, the treatment of juvenile angiofibroma has included many methods, including surgical excision, electrocoagulation, interstitial or external radiation therapy, cryosurgery, hormone administration and chemotherapy. Radiation, chemotherapy and surgery have proven to be the most effective treatments. The most serious complication has been preoperative bleeding, but since the introduction of preoperative particle embolization the blood loss has been greatly reduced. Today, surgery preceded by embolization is the primary standard treatment. It is important to diagnose the tumor early, when radical surgery is easier and the frequency of recurrence is lower.

  5. Kupffer cell structure in the juvenile Nile crocodile, Crocodylus niloticus.

    PubMed

    van Wilpe, Erna; Groenewald, Hermanus Bernardus

    2014-01-01

    The morphology of Kupffer cells was examined in the liver of the juvenile Nile crocodile using light microscopy and transmission electron microscopy. Pleomorphic Kupffer cells were located in the sinusoids, in the space of Disse, in the hepatic parenchyma and often connected adjacent sinusoids. The cell surfaces were irregular due to the presence of filopodia and lamelliapodia with phagocytosis of white blood cells, red blood cells and thrombocytes being evident. The cells were in close contact with endothelial cells and pit cells in the sinusoidal lumen and with stellate cells in the space of Disse. The cytoplasm contained large phagosomes comprising a combination of ceroid pigment, melanosomes and siderosomes. The nuclei were often indented and eccentrically placed due to the presence of the phagosomes. Conspicuous clusters of membrane-bound tubular organelles with a filamentous or crystalline interior were observed in the cytoplasm. The clusters were sometimes separated into smaller groups around phagosomes. A clear zone existed between the limiting membrane and the interior of these tubular organelles with the electron-dense interior profiles being, respectively, circular, angular or divided. The tubular organelles have not previously been described in Kupffer cells and possibly represent lysosomes with specialized functions. Mitochondria, microtubules, Golgi profiles, granular and smooth endoplasmic reticulum, and a few cytoplasmic lipid droplets were also present. The presence of the tubular organelles and the occurrence of the Kupffer cells in different locations in the liver of the juvenile Nile crocodile are indicative of particularly active and mobile cells.

  6. Characterization and chromosomal mapping of a mouse ortholog of the late-infantile ceroid-lipofuscinosis gene CLN2.

    PubMed

    Katz, M L; Liu, P C; Grob-Nunn, S E; Shibuya, H; Johnson, G S

    1999-11-01

    Late-infantile ceroid-lipofuscinosis (CLN2) is an autosomal recessively inherited, neurodegenerative disease in humans. The CLN2 locus has been mapped to Chromosome (Chr) 11p15, and its sequence and genomic organization have recently been reported. In the present study, the cDNA sequence, exon/intron organization, and chromosomal localization of a mouse ortholog of the CLN2 gene are described. The mouse cDNA contains an open reading frame that predicts a protein product of 562 amino acids. The mouse and human coding regions are 86% and 88% identical at the nucleic acid and amino acid levels, respectively. One less codon appears in the mouse cDNA when compared with the human ortholog. The mouse gene (Cln2) spans more than 6 kb and consists of 13 exons separated by introns ranging in size from 111 to 1259 bp. Length polymorphism in an (AC)(n) microsatellite in intron 3 of the mouse Cln2 gene was used to perform segregation analysis with The Jackson Laboratory DNA Panel Mapping Resource. On the basis of this analysis, the Cln2 gene was localized to a region of mouse Chr 7 that corresponds to human Chr 11p15. Characterization of the mouse Cln2 gene will facilitate generation of a mouse model for late-infantile ceroid-lipofuscinosis by gene targeting and identification of functionally important regions of the Cln2 protein.

  7. Fighting Juvenile Gun Violence. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Sheppard, David; Grant, Heath; Rowe, Wendy; Jacobs, Nancy

    This bulletin describes the Office of Juvenile Justice and Delinquency Prevention's efforts to fight juvenile gun violence. The Office awarded four community demonstration grants to implement "Partnerships To Reduce Juvenile Gun Violence." Partnership goals include increasing the effectiveness of existing strategies by enhancing and coordinating…

  8. Juvenile Justice & Youth Violence.

    ERIC Educational Resources Information Center

    Howell, James C.

    Youth violence and the juvenile justice system in the United States are explored. Part 1 takes stock of the situation. The first chapter discusses the origins and evaluation of the juvenile justice system, and the second considers the contributions of the Federal Juvenile Justice and Delinquency Prevention Act to the existing juvenile justice…

  9. Golden Retriever dogs with neuronal ceroid lipofuscinosis have a two-base-pair deletion and frameshift in CLN5.

    PubMed

    Gilliam, D; Kolicheski, A; Johnson, G S; Mhlanga-Mutangadura, T; Taylor, J F; Schnabel, R D; Katz, M L

    2015-01-01

    We studied a recessive, progressive neurodegenerative disease occurring in Golden Retriever siblings with an onset of signs at 15 months of age. As the disease progressed these signs included ataxia, anxiety, pacing and circling, tremors, aggression, visual impairment and localized and generalized seizures. A whole genome sequence, generated with DNA from one affected dog, contained a plausibly causal homozygous mutation: CLN5:c.934_935delAG. This mutation was predicted to produce a frameshift and premature termination codon and encode a protein variant, CLN5:p.E312Vfs*6, which would lack 39 C-terminal amino acids. Eighteen DNA samples from the Golden Retriever family members were genotyped at CLN5:c.934_935delAG. Three clinically affected dogs were homozygous for the deletion allele; whereas, the clinically normal family members were either heterozygotes (n = 11) or homozygous for the reference allele (n = 4). Among archived Golden Retrievers DNA samples with incomplete clinical records that were also genotyped at the CLN5:c.934_935delAG variant, 1053 of 1062 were homozygous for the reference allele, 8 were heterozygotes and one was a deletion-allele homozygote. When contacted, the owner of this homozygote indicated that their dog had been euthanized because of a neurologic disease that progressed similarly to that of the affected Golden Retriever siblings. We have collected and stored semen from a heterozygous Golden Retriever, thereby preserving an opportunity for us or others to establish a colony of CLN5-deficient dogs. PMID:25934231

  10. The Decline in Pulsatile GnRH Release, as Reflected by Circulating LH Concentrations, During the Infant-Juvenile Transition in the Agonadal Male Rhesus Monkey (Macaca mulatta) Is Associated With a Reduction in Kisspeptin Content of KNDy Neurons of the Arcuate Nucleus in the Hypothalamus

    PubMed Central

    Ramaswamy, Suresh; Dwarki, Karthik; Ali, Barkat; Gibbs, Robert B.

    2013-01-01

    Puberty in primates is timed by 2 hypothalamic events: during late infancy a decline in pulsatile GnRH release occurs, leading to a hypogonadotropic state that maintains quiescence of the prepubertal gonad; and in late juvenile development, pulsatile GnRH release is reactivated and puberty initiated, a phase of development that is dependent on kisspeptin signaling. In the present study, we determined whether the arrest of GnRH pulsatility in infancy was associated with a change in kisspeptin expression in the mediobasal hypothalamus (MBH). Kisspeptin was determined using immunohistochemistry in coronal hypothalamic sections from agonadal male rhesus monkeys during early infancy when GnRH release as reflected by circulating LH concentrations was robust and compared with that in juveniles in which GnRH pulsatility was arrested. The distribution of immunopositive kisspeptin neurons in the arcuate nucleus of the MBH of infants was similar to that previously reported for adults. Kisspeptin cell body number was greater in infants compared with juveniles, and at the middle to posterior level of the arcuate nucleus, this developmental difference was statistically significant. Neurokinin B in the MBH exhibited a similar distribution to that of kisspeptin and was colocalized with kisspeptin in approximately 60% of kisspeptin perikarya at both developmental stages. Intensity of GnRH fiber staining in the median eminence was robust at both stages. These findings indicate that the switch that shuts off pulsatile GnRH release during infancy and that guarantees the subsequent quiescence of the prepubertal gonad involves a reduction in a stimulatory kisspeptin tone to the GnRH neuronal network. PMID:23525220

  11. Juvenile dermatomyositis.

    PubMed

    Quartier, Pierre; Gherardi, Romain K

    2013-01-01

    Juvenile dermatomyositis (JDM) is a systemic, inflammatory, idiopathic disease, mainly affecting the skin and the muscles, starting before the age of 16, with an incidence around one case per 1 million children. Some patients display typical features of JDM without skin involvement, or even without muscle involvement; however, both tissues are affected over time in most cases. Diagnosis criteria were established by Bohan and Peter 35 years ago, based on the presence of typical skin rash and proximal muscle involvement. Other conditions have to be ruled out before making a diagnosis of JDM, such as other connective tissue diseases, polymyositis, infectious/postinfectious myositis, genetic diseases, or metabolic or drug-induced myopathies. Unlike adult-onset dermatomyositis, JDM is exceptionally associated with a malignant disease. JDM may also affect several organs, including the lungs and the digestive tract. In a subset of patients, glucose intolerance, lipodystrophia and/or calcinosis develop. Delay in treatment initiation or inadequate treatment may favor diffuse, debilitating calcinosis. JDM patients have to be referred to reference pediatric centers to properly assess disease activity and disease-related damage (including low bone density in most cases), and to define the best treatment. Long-lasting corticosteroid therapy remains the gold standard, together with physiotherapy. Ongoing clinical trials are assessing the effect of several immunosuppressive and immunomodulatory drugs, which may help to control the disease and possibly demonstrate a corticosteroid-sparing effect. Most patients respond to treatment; relapses are frequent but a complete disease remission is achieved in most cases before adulthood.

  12. Juvenile Delinquency: An Introduction

    ERIC Educational Resources Information Center

    Smith, Carolyn A.

    2008-01-01

    Juvenile Delinquency is a term which is often inaccurately used. This article clarifies definitions, looks at prevalence, and explores the relationship between juvenile delinquency and mental health. Throughout, differences between males and females are explored. (Contains 1 table.)

  13. Juvenile Arrests, 2000. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Snyder, Howard N.

    This bulletin examines the national and state juvenile arrest rate in 2000 using data reported annually by local law enforcement agencies nationwide to the FBI's Uniform Crime Reporting program. Results indicate that the murder rate in 2000 was the lowest since 1965; juvenile arrests for violence in 2000 were the lowest since 1988; few juveniles…

  14. Juvenile Arrests 1996. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Snyder, Howard N.

    In 1996, law enforcement agencies in the United States made an estimated 2.9 million arrests of persons under the age of 18. According to Federal Bureau of Investigation (FBI) figures, juveniles accounted for 19% of all arrests and 19% of all violent crime in 1996. The substantial growth in juvenile crime that began in the late 1980s peaked in…

  15. Juvenile Arrests, 1999. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Snyder, Howard N.

    This bulletin presents a summary and analysis of national and state juvenile arrest data for 1999. Data come from the FBI's annual "Crime in the United States" report, which offers the estimated number of crimes reported to law enforcement agencies. The 1999 murder rate was the lowest since 1966. Of the nearly 1,800 juveniles murdered in 1999, 33…

  16. Juvenile Arrests, 1998. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Snyder, Howard N.

    This report provides a summary and analysis of national and state juvenile arrest data in the United States. In 1998, law enforcement agencies made an estimated 2.6 million arrests of persons under age 18. Federal Bureau of Investigations statistics indicate that juveniles account for 18% of all arrests, and 17% of all violent crime arrests in…

  17. Juvenile Arrests, 2007. Juvenile Justice Bulletin

    ERIC Educational Resources Information Center

    Puzzanchera, Charles

    2009-01-01

    This Bulletin summarizes 2007 juvenile crime and arrest data reported by local law enforcement agencies across the country and cited in the FBI report, "Crime in the United States 2007." The Bulletin describes the extent and nature of juvenile crime that comes to the attention of the justice system. It serves as a baseline for comparison for…

  18. Concepts Shaping Juvenile Justice

    ERIC Educational Resources Information Center

    White, Rob

    2008-01-01

    Rob White's paper explores ways in which community building can be integrated into the practices of juvenile justice work. He provides a model of what can be called "restorative social justice", one that builds upon the juvenile conferencing model by attempting to fuse social justice concerns with progressive juvenile justice practices.

  19. Juveniles in court.

    PubMed

    Soulier, Matthew F; Scott, Charles L

    2010-01-01

    Nineteenth-century American reformers were concerned about the influence of immaturity and development in juvenile offenses. They responded to their delinquent youths through the creation of juvenile courts. This early American juvenile justice system sought to treat children as different from adults and to rehabilitate wayward youths through the state's assumption of a parental role. Although these rehabilitative goals were never fully realized, the field of American child psychiatry was spawned from these efforts on behalf of delinquent youths. Early child psychiatrists began by caring for juvenile offenders. The function of a child psychiatrist with juvenile delinquents expanded beyond strictly rehabilitation, however, as juvenile courts evolved to resemble criminal adult courts-due to landmark Supreme Court decisions and also juvenile legislation between 1966 and 1975. In response to dramatically increased juvenile violence and delinquency rates in the 1980s, juvenile justice became more retributional, and society was forced to confront issues such as capital punishment for juveniles, their transfer to adult courts, and their competency to stand trial. In the modern juvenile court, child psychiatrists are often asked to participate in the consideration of such issues because of their expertise in development. In that context we review the role of psychiatrists in assisting juvenile courts.

  20. Juveniles on trial.

    PubMed

    Quinn, Kathleen M

    2002-10-01

    This article describes common forensic evaluations requested of juvenile court mental health evaluators. There has been a legal shift toward criminalization of juvenile court, with a greater emphasis on rights, abandonment of the rehabilitative model, and greater movement of adolescents into the adult criminal court. A resulting shift has been the redefinition of juvenile court forensic evaluations toward the specificity of adult forensic work. The challenge for evaluators is to refine their knowledge of the forensic standards and bring knowledge of development, assessment, and diagnosis in juveniles and interview techniques appropriate to juveniles to improve the evaluation and forensic reports.

  1. Glycosylation, transport, and complex formation of palmitoyl protein thioesterase 1 (PPT1) – distinct characteristics in neurons

    PubMed Central

    Lyly, Annina; von Schantz, Carina; Salonen, Tarja; Kopra, Outi; Saarela, Jani; Jauhiainen, Matti; Kyttälä, Aija; Jalanko, Anu

    2007-01-01

    Background Neuronal ceroid lipofuscinoses (NCLs) are collectively the most common type of recessively inherited childhood encephalopathies. The most severe form of NCL, infantile neuronal ceroid lipofuscinosis (INCL), is caused by mutations in the CLN1 gene, resulting in a deficiency of the lysosomal enzyme, palmitoyl protein thioesterase 1 (PPT1). The deficiency of PPT1 causes a specific death of neocortical neurons by a mechanism, which is currently unclear. To understand the function of PPT1 in more detail, we have further analyzed the basic properties of the protein, especially focusing on possible differences in non-neuronal and neuronal cells. Results Our study shows that the N-glycosylation of N197 and N232, but not N212, is essential for PPT1's activity and intracellular transport. Deglycosylation of overexpressed PPT1 produced in neurons and fibroblasts demonstrates differentially modified PPT1 in different cell types. Furthermore, antibody internalization assays showed differences in PPT1 transport when compared with a thoroughly characterized lysosomal enzyme aspartylglucosaminidase (AGA), an important observation potentially influencing therapeutic strategies. PPT1 was also demonstrated to form oligomers by size-exclusion chromatography and co-immunoprecipitation assays. Finally, the consequences of disease mutations were analyzed in the perspective of our new results, suggesting that the mutations increase both the degree of glycosylation of PPT1 and its ability to form complexes. Conclusion Our current study describes novel properties for PPT1. We observe differences in PPT1 processing and trafficking in neuronal and non-neuronal cells, and describe for the first time the ability of PPT1 to form complexes. Understanding the basic characteristics of PPT1 is fundamental in order to clarify the molecular pathogenesis behind neurodegeneration in INCL. PMID:17565660

  2. Persistent Representation of Juvenile Experience in the Adult Songbird Brain

    PubMed Central

    Prather, JF; Peters, S; Nowicki, S; Mooney, R

    2010-01-01

    Juveniles sometimes learn behaviors that they cease to express as adults. Whether the adult brain retains a record of experiences associated with behaviors performed transiently during development remains unclear. We addressed this issue by studying neural representations of song in swamp sparrows, a species in which juveniles learn and practice many more songs than they retain in their adult vocal repertoire. We exposed juvenile swamp sparrows to a suite of tutor songs and confirmed that although many tutor songs were imitated during development, not all copied songs were retained into adulthood. We then recorded extracellularly in the sensorimotor nucleus HVC in anesthetized sparrows to assess neuronal responsiveness to songs in the adult repertoire, tutor songs, and novel songs. Individual HVC neurons almost always responded to songs in the adult repertoire and commonly responded even more strongly to a tutor song. Effective tutor songs were not simply those that were acoustically similar to songs in the adult repertoire. Moreover, the strength of tutor song responses was unrelated to the number of times that the bird sang copies of those songs in juvenile or adult life. Notably, several neurons responded most strongly to a tutor song performed only rarely and transiently during juvenile life, or even to a tutor song for which we could find no evidence of ever having been copied. Thus, HVC neurons representing songs in the adult repertoire also appear to retain a lasting record of certain tutor songs, including those imitated only transiently. PMID:20686001

  3. X-ray microprobe analysis of the retina and RPE in sheep with ovine ceroid-lipofuscinosis

    SciTech Connect

    Samuelson, D.A.; Armstrong, D.; Jolly, R. )

    1990-11-01

    Ovine ceroid-lipofuscinosis (OCL) is one animal model for the human condition, and because autofluorescent lipopigments are prominent in the brain and eye, it may also prove useful as a model for aging. For example, a progressive decline in electrical recording from brain and retina are observed in both aging and OCL. Samples of retinal and retinal pigment epithelial (RPE) tissues were obtained from a young control. 2 animals with OCL and a normal aged sheep. Specimens were cryo-fractured and examined by scanning electron microscopy/x-ray microanalysis. Measurements made of 6 individual cells in the ganglion layer of OCL specimens, the remainder of the retina, and RPE showed age-related changes in zinc, iron, and copper which were associated with lipopigment accumulation in the RPE. There was marked decrease in phosphate, sulfur, and manganese levels, as photoreceptor cells and their outer segments are lost in the disease process. This is the first report of metal analysis in the retina and RPE in a disease entity, and as a function of normal aging.

  4. Juvenile Confinement in Context

    ERIC Educational Resources Information Center

    Mendel, Richard A.

    2012-01-01

    For more than a century, the predominant strategy for the treatment and punishment of serious and sometimes not-so-serious juvenile offenders in the United States has been placement into large juvenile corrections institutions, alternatively known as training schools, reformatories, or youth corrections centers. America's heavy reliance on…

  5. Juvenile nasopharyngeal angiofibroma.

    PubMed

    Karthikeya, Patil; Mahima, V G; Bagewadi, Shivanand B

    2005-01-01

    Juvenile nasopharyngeal angiofibroma is a rare, histologically benign yet locally aggressive, vascular tumor that typically affects adolescent males. It accounts for 0.5 percent of all neoplasms of the head and neck. A case of juvenile nasopharyngeal angiofibroma manifesting in the oral cavity in a 20-year-old male patient is presented and discussed.

  6. Renewing Juvenile Justice

    ERIC Educational Resources Information Center

    Macallair, Daniel; Males, Mike; Enty, Dinky Manek; Vinakor, Natasha

    2011-01-01

    The Center on Juvenile and Criminal Justice (CJCJ) was commissioned by Sierra Health Foundation to critically examine California's juvenile justice system and consider the potential role of foundations in promoting systemic reform. The information gathered by CJCJ researchers for this report suggests that foundations can perform a key leadership…

  7. Vestibular Neuronitis

    MedlinePlus

    ... Prevent Painful Swimmer's Ear Additional Content Medical News Vestibular Neuronitis By Lawrence R. Lustig, MD NOTE: This ... Drugs Herpes Zoster Oticus Meniere Disease Purulent Labyrinthitis Vestibular Neuronitis Vestibular neuronitis is a disorder characterized by ...

  8. Reporting Crimes Against Juveniles. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Finkelhor, David; Ormrod, Richard

    This bulletin addresses the issue of reporting crimes against juveniles, describing findings from the National Crime Victimization Survey, which gathers information from citizens on crime, including whether and how they are reported. The survey also collects information about characteristics of victimizations, the nature of the incident location,…

  9. Neuronal beacon.

    PubMed

    Black, B; Mondal, A; Kim, Y; Mohanty, S K

    2013-07-01

    The controlled navigation of the axonal growth cone of a neuron toward the dendrite of its synaptic partner neuron is the fundamental process in forming neuronal circuitry. While a number of technologies have been pursued for axonal guidance over the past decades, they are either invasive or not controllable with high spatial and temporal resolution and are often limited by low guidance efficacy. Here, we report a neuronal beacon based on light for highly efficient and controlled guidance of cortical primary neurons.

  10. Juvenile Sex Offenders.

    PubMed

    Ryan, Eileen P; Otonichar, Joseph M

    2016-07-01

    Sexual offending by juveniles accounts for a sizable percentage of sexual offenses, especially against young children. In this article, recent research on female juvenile sex offenders (JSOs), risk factors for offending in juveniles, treatment, and the ways in which these youth may differ from general delinquents will be reviewed. Most JSOs do not go on to develop paraphilic disorders or to commit sex offenses during adulthood, and as a group, they are more similar to nonsexual offending juvenile delinquents than to adult sex offenders. Recent research has elucidated some differences between youth who commit sex offenses and general delinquents in the areas of atypical sexual interests, the use of pornography, and early sexual victimization during childhood. PMID:27222141

  11. The paediatric rheumatologist and orphan disease - a story without happy ending.

    PubMed

    Roszkiewicz, Justyna; Biernacka-Zielińska, Małgorzata; Smolewska, Elżbieta

    2016-01-01

    Orphan diseases are not a common challenge in the everyday practice of the rheumatologist. Despite their extremely rare occurrence one of the patients under our care developed one of them - neuronal ceroid lipofuscinosis, the most frequent neurodegenerative disease observed in the paediatric population. We report a case of 2-year-old girl diagnosed with oligoarticular form of juvenile idiopathic arthritis treated in our Department with steroids and methotrexate and staying in the stage of disease remission. During routine checkups at Outpatient Clinic we observed progressive deterioration of girls neurological condition resulting in ataxia, gait disturbances with no rheumatological cause behind and speech impairment. The appearance of the symptoms was accompanied by frequent episodes of epileptic seizures, with little clinical improvement on combined antiepileptic treatment. Magnetic resonance imaging that we performed showed a picture highly suggestive of neuronal ceroid lipofuscinosis - atrophy of the patients cerebrum and cerebellum. Genetic testing conducted resulted in the diagnosis of late infantile neuronal ceroid lipofuscinosis (LINCL).

  12. The paediatric rheumatologist and orphan disease - a story without happy ending.

    PubMed

    Roszkiewicz, Justyna; Biernacka-Zielińska, Małgorzata; Smolewska, Elżbieta

    2016-01-01

    Orphan diseases are not a common challenge in the everyday practice of the rheumatologist. Despite their extremely rare occurrence one of the patients under our care developed one of them - neuronal ceroid lipofuscinosis, the most frequent neurodegenerative disease observed in the paediatric population. We report a case of 2-year-old girl diagnosed with oligoarticular form of juvenile idiopathic arthritis treated in our Department with steroids and methotrexate and staying in the stage of disease remission. During routine checkups at Outpatient Clinic we observed progressive deterioration of girls neurological condition resulting in ataxia, gait disturbances with no rheumatological cause behind and speech impairment. The appearance of the symptoms was accompanied by frequent episodes of epileptic seizures, with little clinical improvement on combined antiepileptic treatment. Magnetic resonance imaging that we performed showed a picture highly suggestive of neuronal ceroid lipofuscinosis - atrophy of the patients cerebrum and cerebellum. Genetic testing conducted resulted in the diagnosis of late infantile neuronal ceroid lipofuscinosis (LINCL). PMID:27504025

  13. Genetics Home Reference: juvenile polyposis syndrome

    MedlinePlus

    ... In the third type, known as juvenile polyposis coli, affected individuals develop polyps only in their colon. People with generalized juvenile polyposis and juvenile polyposis coli typically develop polyps during childhood. Most juvenile polyps ...

  14. Juvenile Sex Offenders.

    PubMed

    Ryan, Eileen P

    2016-01-01

    Public policy has tended to treat juvenile sex offenders (JSOs) as adult sex offenders in waiting, despite research that contradicts this notion. Although as a group, JSOs are more similar to general delinquents than to adult sex offenders, atypical sexual interests and sexual victimization during childhood may be a pathway for sexual offending that differentiates some JSOs from their nonsexually delinquent peers. Developmental considerations must be considered in risk assessment evaluations of these youth. This article reviews theories of sexual offending in youth, risk factors for juvenile offending and reoffending, psychopathology in JSOs, risk assessment, and treatment. PMID:26593121

  15. Juvenile Justice in Maryland.

    ERIC Educational Resources Information Center

    Vetter, Donald P.; Frederick, Charles

    This four part guide provides secondary students with information about Maryland laws, courts, and legal system. The first section examines the nature and causes of increasing involvement of youth in crime, and identifies those crimes most commonly committed by juveniles. A special section on shoplifting is included. Section II examines the nature…

  16. Juvenile nasopharyngeal angiofibroma.

    PubMed

    Vadivel, S P; Bosch, A; Jose, B

    1980-01-01

    Seven cases of juvenile nasopharyngeal angiofibroma were seen in the Division of Radiation Oncology of the Department of Human Oncology, University of Wisconsin Hospitals from 1961 to 1977. The method of treatments and the end results are discussed. The clinical manifestations and the biological nature of this tumor are analyzed in detail, along with treatment recommendations.

  17. Treating the Juvenile Offender

    ERIC Educational Resources Information Center

    Hoge, Robert D., Ed.; Guerra, Nancy G., Ed.; Boxer, Paul, Ed.

    2008-01-01

    This authoritative, highly readable reference and text is grounded in the latest knowledge on how antisocial and criminal behavior develops in youth and how it can effectively be treated. Contributors describe proven ways to reduce juvenile delinquency by targeting specific risk factors and strengthening young people's personal, family, and…

  18. Neuronal polarization.

    PubMed

    Takano, Tetsuya; Xu, Chundi; Funahashi, Yasuhiro; Namba, Takashi; Kaibuchi, Kozo

    2015-06-15

    Neurons are highly polarized cells with structurally and functionally distinct processes called axons and dendrites. This polarization underlies the directional flow of information in the central nervous system, so the establishment and maintenance of neuronal polarization is crucial for correct development and function. Great progress in our understanding of how neurons establish their polarity has been made through the use of cultured hippocampal neurons, while recent technological advances have enabled in vivo analysis of axon specification and elongation. This short review and accompanying poster highlight recent advances in this fascinating field, with an emphasis on the signaling mechanisms underlying axon and dendrite specification in vitro and in vivo.

  19. Tripeptidyl peptidase I, the late infantile neuronal ceroid lipofuscinosis gene product, initiates the lysosomal degradation of subunit c of ATP synthase.

    PubMed

    Ezaki, J; Takeda-Ezaki, M; Kominami, E

    2000-09-01

    The specific accumulation of a hydrophobic protein, subunit c of ATP synthase, in lysosomes from the cells of patients with the late infantile form of NCL (LINCL) is caused by a defect in the CLN2 gene product, tripeptidyl peptidase I (TPP-I). The data here show that TPP-I is involved in the initial degradation of subunit c in lysosomes and suggest that its absence leads directly to the lysosomal accumulation of subunit c. The inclusion of a specific inhibitor of TPP-I, Ala-Ala-Phe-chloromethylketone (AAF-CMK), in the culture medium of normal fibroblasts induced the lysosomal accumulation of subunit c. In an in vitro incubation experiment the addition of AAF-CMK to mitochondrial-lysosomal fractions from normal cells inhibited the proteolysis of subunit c, but not the b-subunit of ATP synthase. The use of two antibodies that recognize the aminoterminal and the middle portion of subunit c revealed that the subunit underwent aminoterminal proteolysis, when TPP-I, purified from rat spleen, was added to the mitochondrial fractions. The addition of both purified TPP-I and the soluble lysosomal fractions, which contain various proteinases, to the mitochondrial fractions resulted in rapid degradation of the entire molecule of subunit c, whereas the degradation of subunit c was markedly delayed through the specific inhibition of TPP-I in lysosomal extracts by AAF-CMK. The stable subunit c in the mitochondrial-lysosomal fractions from cells of a patient with LINCL was degraded on incubation with purified TPP-I. The presence of TPP-I led to the sequential cleavage of tripeptides from the N-terminus of the peptide corresponding to the amino terminal sequence of subunit c.

  20. Juvenile nasopharyngeal angiofibroma.

    PubMed

    Sellars, S L

    1980-12-13

    The juvenile nasopharyngeal angiofibroma, a locally invasive, non-metastasizing tumour of male adolescence, occurs sporadically throughout the world. Its histiogenesis remains uncertain and its management controversial. These facets of a troublesome and dangerous conditions are discussed and the experiences from handling 9 such tumours seen at Groote Schuur Hospital over a 4-year period (1976-1979) are presented. Surgical excision, using a wide field exposure and pre-operative systemic oestrogen medication, is recommended as the treatment of choice.

  1. Intracranial juvenile nasopharyngeal angiofibroma.

    PubMed

    Wylie, J P; Slevin, N J; Johnson, R J

    1998-01-01

    We report the case history of a 26-year-old man who was diagnosed with advanced juvenile nasopharyngeal angiofibroma with intracranial extension. The lesion was considered to be inoperable and the patient was treated with radical radiotherapy. Serial magnetic resonance imaging has shown continued tumour regression and he remains well after 3 years. The literature is reviewed and radiotherapy recommended as the modality of choice for these patients.

  2. Juvenile idiopathic arthritis.

    PubMed

    Gowdie, Peter J; Tse, Shirley M L

    2012-04-01

    Juvenile idiopathic arthritis (JIA) encompasses a complex group of disorders with arthritis as a common feature. This article provides the pediatrician with a review of the epidemiology, classification, clinical manifestations, and complications of JIA. It also provides an update on the current understanding of the cause of JIA and recent developments in management and a recent review of the long-term outcome in JIA.

  3. Juvenile Onset HD

    MedlinePlus

    ... of the huntingtin gene called a “CAG repeat expansion”. The mutation results in gradual neuronal degeneration in ... Laboratory testing showing a fully-penetrant CAG repeat expansion in the HD gene (>39 CAG repeats). Brain ...

  4. Telepsychiatry in juvenile justice settings.

    PubMed

    Kaliebe, Kristopher E; Heneghan, James; Kim, Thomas J

    2011-01-01

    Telepsychiatry is emerging as a valuable means of providing mental health care in juvenile justice settings. Youth in the juvenile justice system have high levels of psychiatric morbidity. State and local juvenile justice systems frequently struggle to provide specialized psychiatric care, as these systems have limited resources and often operate in remote locations. Case studies in the use of telepsychiatry to provide improved care in juvenile corrections in 4 states are described, along with a review of advantages and disadvantages of telepsychiatry in these settings. PMID:21092916

  5. Telepsychiatry in juvenile justice settings.

    PubMed

    Kaliebe, Kristopher E; Heneghan, James; Kim, Thomas J

    2011-01-01

    Telepsychiatry is emerging as a valuable means of providing mental health care in juvenile justice settings. Youth in the juvenile justice system have high levels of psychiatric morbidity. State and local juvenile justice systems frequently struggle to provide specialized psychiatric care, as these systems have limited resources and often operate in remote locations. Case studies in the use of telepsychiatry to provide improved care in juvenile corrections in 4 states are described, along with a review of advantages and disadvantages of telepsychiatry in these settings.

  6. Improving Literacy Skills of Juvenile Detainees. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Hodges, Jane; And Others

    The Office of Juvenile and Delinquency Prevention funded a model designed to improve the literacy level of youth in juvenile detention and correctional facilities. The model specified training language arts teachers and relevant staff and volunteers in direct instruction methods for rapid improvement of students' comprehension, particularly for…

  7. Juvenile Mentoring Program: A Progress Review. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Novotney, Laurence C.; Mertinko, Elizabeth; Lange, James; Baker, Tara Kelley

    The greatest support offered by the Office of Juvenile Justice and Delinquency Prevention for youth mentoring has been through the Juvenile Mentoring Program (JUMP), which provides one-to-one mentoring for youth at risk of delinquency, gang involvement, educational failure, or dropping out of school. Information on JUMP has been collected through…

  8. Serious and Violent Juvenile Offenders. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Department of Justice, Washington, DC. Office of Juvenile Justice and Delinquency Prevention.

    This bulletin summarizes the latest and most comprehensive research and serious and violent juvenile (SJV) offenders taken from a report by the Study Group on Serious and Violent Juvenile Offenders. It describes characteristics of SVJ offenders and predictors of SVJ offending. Some interventions to prevent offenses by SVJs are reviewed. Recently…

  9. Juvenile hyaline fibromatosis.

    PubMed

    Larralde, M; Santos-Muñoz, A; Calb, I; Magariños, C

    2001-01-01

    Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease with onset in infancy or early childhood. It is characterized by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of the large joints. The light and electron microscopic features are very distinctive. Here we report an 8-month-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hypertrophy. Histologic and ultrastructural features were typical of JHF. Clinical features, pathology, and physiology are discussed.

  10. Juvenile psittacine environmental enrichment.

    PubMed

    Simone-Freilicher, Elisabeth; Rupley, Agnes E

    2015-05-01

    Environmental enrichment is of great import to the emotional, intellectual, and physical development of the juvenile psittacine and their success in the human home environment. Five major types of enrichment include social, occupational, physical, sensory, and nutritional. Occupational enrichment includes exercise and psychological enrichment. Physical enrichment includes the cage and accessories and the external home environment. Sensory enrichment may be visual, auditory, tactile, olfactory, or taste oriented. Nutritional enrichment includes variations in appearance, type, and frequency of diet, and treats, novelty, and foraging. Two phases of the preadult period deserve special enrichment considerations: the development of autonomy and puberty.

  11. Juvenile nasopharyngeal angiofibroma.

    PubMed

    Blount, Angela; Riley, Kristen O; Woodworth, Bradford A

    2011-08-01

    Juvenile nasopharyngeal angiofibromas (JNAs) are rare, benign, highly vascular, locally aggressive tumors that primarily affect male adolescents. Historical treatment of these neoplasms has been primarily surgical. In the past decade, endoscopic resection of JNAs has become a viable and promising surgical treatment option. Endoscopic resection has many advantages over traditional open techniques, including better cosmesis, decreased blood loss, shortened hospital stays, and equivalent or improved recurrence rates. Emerging endoscopic technology continues to push the boundaries of resection of skull base tumors and will no doubt become the surgical treatment of choice for most JNAs in the near future.

  12. Juvenile Dermatomyositis in Pregnancy

    PubMed Central

    Madu, Anthony Emeka; Omih, Edwin; Baguley, Elaine; Lindow, Stephen W.

    2013-01-01

    Juvenile dermatomyositis has variable clinical presentations both in and outside of pregnancy. A literature review indicated that optimal maternal and fetal outcomes can be anticipated when the pregnancy is undertaken while the disease is in remission. Poorer outcomes are associated with flare-up of the disease in early pregnancy compared with exacerbation in the second or third trimester, when fetal prognosis is usually good. We present a case of JDM in pregnancy with disease exacerbation late in pregnancy and review of the relevant literature. PMID:23662227

  13. Juvenile dermatomyositis in pregnancy.

    PubMed

    Madu, Anthony Emeka; Omih, Edwin; Baguley, Elaine; Lindow, Stephen W

    2013-01-01

    Juvenile dermatomyositis has variable clinical presentations both in and outside of pregnancy. A literature review indicated that optimal maternal and fetal outcomes can be anticipated when the pregnancy is undertaken while the disease is in remission. Poorer outcomes are associated with flare-up of the disease in early pregnancy compared with exacerbation in the second or third trimester, when fetal prognosis is usually good. We present a case of JDM in pregnancy with disease exacerbation late in pregnancy and review of the relevant literature.

  14. Juvenile Justice in Rural America.

    ERIC Educational Resources Information Center

    Jankovic, Joanne, Ed.; And Others

    Producing a much-needed organized body of literature about rural juvenile justice, 14 papers (largely from the 1979 National Symposium on Rural Justice) are organized to identify current issues, identify forces causing changes in current systems, review programs responding to rural juvenile justice problems, and provide planning models to aid…

  15. Psychopathology in Female Juvenile Offenders

    ERIC Educational Resources Information Center

    Dixon, Angela; Howie, Pauline; Starling, Jean

    2004-01-01

    Background: The aim was to document the spectrum of present and lifetime psychological disorders in female juvenile offenders, and to examine the relations between mental health status and socio-demographic, family and trauma variables. Method: One hundred juvenile offenders were matched with a comparison group of 100 females on age and…

  16. Iatrogenic Effect of Juvenile Justice

    ERIC Educational Resources Information Center

    Gatti, Uberto; Tremblay, Richard E.; Vitaro, Frank

    2009-01-01

    Background: The present study uses data from a community sample of 779 low-SES boys to investigate whether intervention by the juvenile justice system is determined, at least in part, by particular individual, familial and social conditions, and whether intervention by the juvenile courts during adolescence increases involvement in adult crime.…

  17. Mental Illness and Juvenile Offenders

    PubMed Central

    Underwood, Lee A.; Washington, Aryssa

    2016-01-01

    Within the past decade, reliance on the juvenile justice system to meet the needs of juvenile offenders with mental health concerns has increased. Due to this tendency, research has been conducted on the effectiveness of various intervention and treatment programs/approaches with varied success. Recent literature suggests that because of interrelated problems involved for youth in the juvenile justice system with mental health issues, a dynamic system of care that extends beyond mere treatment within the juvenile justice system is the most promising. The authors provide a brief overview of the extent to which delinquency and mental illness co-occur; why treatment for these individuals requires a system of care; intervention models; and the juvenile justice systems role in providing mental health services to delinquent youth. Current and future advancements and implications for practitioners are provided. PMID:26901213

  18. Mental Illness and Juvenile Offenders.

    PubMed

    Underwood, Lee A; Washington, Aryssa

    2016-02-18

    Within the past decade, reliance on the juvenile justice system to meet the needs of juvenile offenders with mental health concerns has increased. Due to this tendency, research has been conducted on the effectiveness of various intervention and treatment programs/approaches with varied success. Recent literature suggests that because of interrelated problems involved for youth in the juvenile justice system with mental health issues, a dynamic system of care that extends beyond mere treatment within the juvenile justice system is the most promising. The authors provide a brief overview of the extent to which delinquency and mental illness co-occur; why treatment for these individuals requires a system of care; intervention models; and the juvenile justice systems role in providing mental health services to delinquent youth. Current and future advancements and implications for practitioners are provided.

  19. Mental Illness and Juvenile Offenders.

    PubMed

    Underwood, Lee A; Washington, Aryssa

    2016-02-01

    Within the past decade, reliance on the juvenile justice system to meet the needs of juvenile offenders with mental health concerns has increased. Due to this tendency, research has been conducted on the effectiveness of various intervention and treatment programs/approaches with varied success. Recent literature suggests that because of interrelated problems involved for youth in the juvenile justice system with mental health issues, a dynamic system of care that extends beyond mere treatment within the juvenile justice system is the most promising. The authors provide a brief overview of the extent to which delinquency and mental illness co-occur; why treatment for these individuals requires a system of care; intervention models; and the juvenile justice systems role in providing mental health services to delinquent youth. Current and future advancements and implications for practitioners are provided. PMID:26901213

  20. Juvenile obesity enhances emotional memory and amygdala plasticity through glucocorticoids.

    PubMed

    Boitard, Chloé; Maroun, Mouna; Tantot, Frédéric; Cavaroc, Amandine; Sauvant, Julie; Marchand, Alain; Layé, Sophie; Capuron, Lucile; Darnaudery, Muriel; Castanon, Nathalie; Coutureau, Etienne; Vouimba, Rose-Marie; Ferreira, Guillaume

    2015-03-01

    In addition to metabolic and cardiovascular disorders, obesity is associated with adverse cognitive and emotional outcomes. Its growing prevalence during adolescence is particularly alarming since recent evidence indicates that obesity can affect hippocampal function during this developmental period. Adolescence is a decisive period for maturation of the amygdala and the hypothalamic-pituitary-adrenal (HPA) stress axis, both required for lifelong cognitive and emotional processing. However, little data are available on the impact of obesity during adolescence on amygdala function. Herein, we therefore evaluate in rats whether juvenile high-fat diet (HFD)-induced obesity alters amygdala-dependent emotional memory and whether it depends on HPA axis deregulation. Exposure to HFD from weaning to adulthood, i.e., covering adolescence, enhances long-term emotional memories as assessed by odor-malaise and tone-shock associations. Juvenile HFD also enhances emotion-induced neuronal activation of the basolateral complex of the amygdala (BLA), which correlates with protracted plasma corticosterone release. HFD exposure restricted to adulthood does not modify all these parameters, indicating adolescence is a vulnerable period to the effects of HFD-induced obesity. Finally, exaggerated emotional memory and BLA synaptic plasticity after juvenile HFD are alleviated by a glucocorticoid receptor antagonist. Altogether, our results demonstrate that juvenile HFD alters HPA axis reactivity leading to an enhancement of amygdala-dependent synaptic and memory processes. Adolescence represents a period of increased susceptibility to the effects of diet-induced obesity on amygdala function. PMID:25740536

  1. Juvenile obesity enhances emotional memory and amygdala plasticity through glucocorticoids.

    PubMed

    Boitard, Chloé; Maroun, Mouna; Tantot, Frédéric; Cavaroc, Amandine; Sauvant, Julie; Marchand, Alain; Layé, Sophie; Capuron, Lucile; Darnaudery, Muriel; Castanon, Nathalie; Coutureau, Etienne; Vouimba, Rose-Marie; Ferreira, Guillaume

    2015-03-01

    In addition to metabolic and cardiovascular disorders, obesity is associated with adverse cognitive and emotional outcomes. Its growing prevalence during adolescence is particularly alarming since recent evidence indicates that obesity can affect hippocampal function during this developmental period. Adolescence is a decisive period for maturation of the amygdala and the hypothalamic-pituitary-adrenal (HPA) stress axis, both required for lifelong cognitive and emotional processing. However, little data are available on the impact of obesity during adolescence on amygdala function. Herein, we therefore evaluate in rats whether juvenile high-fat diet (HFD)-induced obesity alters amygdala-dependent emotional memory and whether it depends on HPA axis deregulation. Exposure to HFD from weaning to adulthood, i.e., covering adolescence, enhances long-term emotional memories as assessed by odor-malaise and tone-shock associations. Juvenile HFD also enhances emotion-induced neuronal activation of the basolateral complex of the amygdala (BLA), which correlates with protracted plasma corticosterone release. HFD exposure restricted to adulthood does not modify all these parameters, indicating adolescence is a vulnerable period to the effects of HFD-induced obesity. Finally, exaggerated emotional memory and BLA synaptic plasticity after juvenile HFD are alleviated by a glucocorticoid receptor antagonist. Altogether, our results demonstrate that juvenile HFD alters HPA axis reactivity leading to an enhancement of amygdala-dependent synaptic and memory processes. Adolescence represents a period of increased susceptibility to the effects of diet-induced obesity on amygdala function.

  2. The Juvenile Justice System of the Future.

    ERIC Educational Resources Information Center

    Gladstone, William

    1995-01-01

    A former juvenile and family court judge reflects on experiences with juvenile issues, calling for critical changes in juvenile case handling. Advocates many changes for juvenile and family courts of the future, such as a new type of due process for children and the abolishment of labels such as "delinquent," runaway," or "abandoned" in favor of…

  3. Miranda Rights: Implications for Juveniles with Disabilities

    ERIC Educational Resources Information Center

    Katsiyannis, Antonis; Barrett, David E.; Losinski, Mickey L.

    2011-01-01

    Juvenile delinquency in the United States has been a persistent concern for decades. Consequently, because more juveniles have been referred to juvenile court and the arrest rate of preteen offenders has increased to almost three times that of older youth, the persistent and often controversial issue of the capacity of juvenile offenders to waive…

  4. [Mirror neurons].

    PubMed

    Rubia Vila, Francisco José

    2011-01-01

    Mirror neurons were recently discovered in frontal brain areas of the monkey. They are activated when the animal makes a specific movement, but also when the animal observes the same movement in another animal. Some of them also respond to the emotional expression of other animals of the same species. These mirror neurons have also been found in humans. They respond to or "reflect" actions of other individuals in the brain and are thought to represent the basis for imitation and empathy and hence the neurobiological substrate for "theory of mind", the potential origin of language and the so-called moral instinct.

  5. Juvenile striatal white matter is resistant to ischemia-induced damage.

    PubMed

    Ahrendsen, Jared T; Grewal, Himmat S; Hickey, Sean P; Culp, Cecilia M; Gould, Elizabeth A; Shimizu, Takeru; Strnad, Frank A; Traystman, Richard J; Herson, Paco S; Macklin, Wendy B

    2016-11-01

    White matter injury following ischemic stroke is a major cause of functional disability. Injury to both myelinated axons and oligodendrocytes, the myelin producing cells in the central nervous system, occurs in experimental models of ischemic stroke. Age-related changes in white matter vulnerability to ischemia have been extensively studied and suggest that both the perinatal and the aged periods are times of increased white matter vulnerability. However, sensitivity of white matter following stroke in the juvenile brain has not been evaluated. Interestingly, the late pediatric period is an important developmental stage, as it is the time of maximal myelination. The current study demonstrates that neurons in late pediatric/juvenile striatum are vulnerable to ischemic damage, with neuronal injury being comparable in juvenile and adult mice following ischemia. By contrast, actively myelinating striatal oligodendrocytes in the juvenile brain are resistant to ischemia, whereas adult oligodendrocytes are quite sensitive. As a result, myelin sheaths are remarkably intact and axons survive well in the injured striatum of juvenile mice. In addition to relative resistance of juvenile white matter, other glial responses were very different in juvenile and adult mice following cerebral ischemia, including differences in astrogliosis, fibrosis, NG2-cell reactivity, and vascular integrity. Together, these responses lead to long-term preservation of brain parenchyma in juvenile mice, compared to severe tissue loss and scarring in adult mice. Overall, the current study suggests that equivalent ischemic insults may result in less functional deficit in children compared to adults and an environment more conducive to long-term recovery. GLIA 2016;64:1972-1986.

  6. Juvenile striatal white matter is resistant to ischemia-induced damage.

    PubMed

    Ahrendsen, Jared T; Grewal, Himmat S; Hickey, Sean P; Culp, Cecilia M; Gould, Elizabeth A; Shimizu, Takeru; Strnad, Frank A; Traystman, Richard J; Herson, Paco S; Macklin, Wendy B

    2016-11-01

    White matter injury following ischemic stroke is a major cause of functional disability. Injury to both myelinated axons and oligodendrocytes, the myelin producing cells in the central nervous system, occurs in experimental models of ischemic stroke. Age-related changes in white matter vulnerability to ischemia have been extensively studied and suggest that both the perinatal and the aged periods are times of increased white matter vulnerability. However, sensitivity of white matter following stroke in the juvenile brain has not been evaluated. Interestingly, the late pediatric period is an important developmental stage, as it is the time of maximal myelination. The current study demonstrates that neurons in late pediatric/juvenile striatum are vulnerable to ischemic damage, with neuronal injury being comparable in juvenile and adult mice following ischemia. By contrast, actively myelinating striatal oligodendrocytes in the juvenile brain are resistant to ischemia, whereas adult oligodendrocytes are quite sensitive. As a result, myelin sheaths are remarkably intact and axons survive well in the injured striatum of juvenile mice. In addition to relative resistance of juvenile white matter, other glial responses were very different in juvenile and adult mice following cerebral ischemia, including differences in astrogliosis, fibrosis, NG2-cell reactivity, and vascular integrity. Together, these responses lead to long-term preservation of brain parenchyma in juvenile mice, compared to severe tissue loss and scarring in adult mice. Overall, the current study suggests that equivalent ischemic insults may result in less functional deficit in children compared to adults and an environment more conducive to long-term recovery. GLIA 2016;64:1972-1986. PMID:27463063

  7. Juvenile nasopharyngeal angiofibroma

    PubMed Central

    Makhasana, Jashika Adil Shroff; Kulkarni, Meena A; Vaze, Suhas; Shroff, Adil Sarosh

    2016-01-01

    Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. It is an aggressive neoplasm and shows a propensity for destructive local spread often extending to the base of the skull and into the cranium. Clinically, however, it is obscure with painless, progressive unilateral nasal obstruction being the common presenting symptom with or without epistaxis and rhinorrhea. Diagnosis of JNA is made by complete history, clinical examination, radiography, nasal endoscopy and by using specialized imaging techniques such as arteriography, computer tomography and magnetic resonance imaging. Histopathology reveals a fibrocellular stroma with spindle cells and haphazard arrangement of collagen interspersed with an irregular vascular pattern. A case report of JNA with rare intra-oral manifestation in a 17-year-old male patient is presented in the article. JNA being an aggressive tumor may recur posttreatment. Thus, early diagnosis, accurate staging, and adequate treatment are essential in the management of this lesion.

  8. [JUVENILE DERMATOMYOSITIS AND CALCINOSIS].

    PubMed

    Zhvania, M

    2015-01-01

    Juvenile Dermatomiositis (JD) is autoimmune disease that progresses with time; JD's main differentiated syndromes are rash on the skin, poor function of muscles, and often developing invalidism. If the health practitioners manage to diagnose the JD on an early stage and prescribe the adequate treatment the disease will not progress aggressively. This approach is tangible for practical rheumatology and pediatric. The article aims to present the reasons of the development of the JD and calcinosis. The study based on the description of the patients with JD. There are distinguished the main symptoms of the disease in children: frequent and acute developments of muscles calcinosis, occasionally with diffuse character followed with hypotrophy of the muscles, contractures and invalidism. One of the patient cases that describe the article is the thirteen-year boy with JD indicating repeated sequence of the disease, with diffusive calcinosis, cellulitis followed with secondary infection and impaired vision.

  9. Juvenile idiopathic arthritis.

    PubMed

    Espinosa, Maria; Gottlieb, Beth S

    2012-07-01

    Juvenile idiopathic arthrithis (JIA) is the most common rheumatic disease of childhood.JIA is a chronic disease that is associated with periods of disease flares and periods of disease inactivity.Early, aggressive treatment with nonsteroidal anti-inflammatory drugs, intra-articular corticosteroid injections, or methotrexate, has significantly improved the outcome of most children who have JIA. Biologics have been shown to be both safe and effective for the treatment of more aggressive forms of arthritis and for uveitis. Long-term safety data of biologics is still uncertain. In the near future, it is hoped that genetic testing will allow earlier diagnosis of JIA as well as help predict the disease course of children who have JIA. Genetic analysis also may allow physicians to target therapies more effectively. It is hoped that development of more specific therapies will decrease overall immunosuppression and other associated toxicities.

  10. Juvenile nasopharyngeal angiofibroma

    PubMed Central

    Makhasana, Jashika Adil Shroff; Kulkarni, Meena A; Vaze, Suhas; Shroff, Adil Sarosh

    2016-01-01

    Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. It is an aggressive neoplasm and shows a propensity for destructive local spread often extending to the base of the skull and into the cranium. Clinically, however, it is obscure with painless, progressive unilateral nasal obstruction being the common presenting symptom with or without epistaxis and rhinorrhea. Diagnosis of JNA is made by complete history, clinical examination, radiography, nasal endoscopy and by using specialized imaging techniques such as arteriography, computer tomography and magnetic resonance imaging. Histopathology reveals a fibrocellular stroma with spindle cells and haphazard arrangement of collagen interspersed with an irregular vascular pattern. A case report of JNA with rare intra-oral manifestation in a 17-year-old male patient is presented in the article. JNA being an aggressive tumor may recur posttreatment. Thus, early diagnosis, accurate staging, and adequate treatment are essential in the management of this lesion. PMID:27601836

  11. Juvenile nasopharyngeal angiofibroma.

    PubMed

    Makhasana, Jashika Adil Shroff; Kulkarni, Meena A; Vaze, Suhas; Shroff, Adil Sarosh

    2016-01-01

    Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. It is an aggressive neoplasm and shows a propensity for destructive local spread often extending to the base of the skull and into the cranium. Clinically, however, it is obscure with painless, progressive unilateral nasal obstruction being the common presenting symptom with or without epistaxis and rhinorrhea. Diagnosis of JNA is made by complete history, clinical examination, radiography, nasal endoscopy and by using specialized imaging techniques such as arteriography, computer tomography and magnetic resonance imaging. Histopathology reveals a fibrocellular stroma with spindle cells and haphazard arrangement of collagen interspersed with an irregular vascular pattern. A case report of JNA with rare intra-oral manifestation in a 17-year-old male patient is presented in the article. JNA being an aggressive tumor may recur posttreatment. Thus, early diagnosis, accurate staging, and adequate treatment are essential in the management of this lesion. PMID:27601836

  12. Juvenile homosexual homicide.

    PubMed

    Myers, Wade C; Chan, Heng Choon Oliver

    2012-01-01

    Limited information exists on juvenile homosexual homicide (JHH), that is, youths who perpetrate sexual homicides against same-sex victims. Only a handful of cases from the United States and internationally have been described in the literature. This study, the first of its kind, examines the epidemiology, victimology, victim-offender relationship, and weapon-use patterns in JHH offenders using a large U.S. database on homicide spanning three decades. The data for this study were derived from the Federal Bureau of Investigation's Supplementary Homicide Reports (SHRs) for the years 1976 through 2005. A total of 93 cases of JHH were identified. On average, three of these crimes occurred annually in the U.S., and there was a marked decline in its incidence over the study period. Ninety-five percent were male offender-male victim cases and 5% were female offender-female victim cases. JHH offenders were over-represented amongst all juvenile sexual murderers, similar to their adult counterparts. The majority of these boys were aged 16 or 17 and killed adult victims. They were significantly more likely to kill adult victims than other age groups, to be friends or acquaintances of the victims, and to use contact/edged weapons or firearms. Most offenders killed same-race victims, although Black offenders were significantly more likely than White offenders to kill interracially. A case report is provided to illustrate JHH. Further research is needed to promote our understanding of the pathogenesis, etiology, and associated risk factors for this aberrant form of murder by children.

  13. Transfer of Juvenile Cases to Criminal Court.

    PubMed

    Lee, Soo Jung; Kraus, Louis J

    2016-01-01

    The first juvenile court was founded in 1899 with the focus on rehabilitation of a juvenile offender as opposed to punishment in adult court. Determining culpability and disposition for adolescents has become a source of much discussion. With serious crimes, juvenile delinquents may be transferred from juvenile court to adult criminal court; this practice became more prevalent in the past century. However, growing knowledge of adolescent development has mitigated the culpability of youth offenders and resulted in judicial decisions influential to juvenile dispositions.

  14. Editor's Shelf: International Juvenile Titles.

    ERIC Educational Resources Information Center

    Mitchell-Powell, Brenda

    1994-01-01

    Provides an annotated list of international juvenile picture books and notes those that emphasize text over pictures. The 49 titles present international perspectives for educators, librarians, and parents seeking materials with alternative cultural content. The majority are folk tales. (SLD)

  15. Motor Neuron Diseases

    MedlinePlus

    ... called upper motor neurons ) are transmitted to nerve cells in the brain stem and spinal cord (called lower motor neurons ) and from them to particular muscles. Upper motor neurons direct the lower motor neurons ...

  16. Motor Neuron Diseases

    MedlinePlus

    ... Enhancing Diversity Find People About NINDS NINDS Motor Neuron Diseases Information Page Condensed from Motor Neuron Diseases ... and Information Publicaciones en Español What are Motor Neuron Diseases? The motor neuron diseases (MNDs) are a ...

  17. A giant juvenile nasopharyngeal angiofibroma.

    PubMed

    Yüce, Salim; Uysal, Ismail Önder; Doğan, Mansur; Polat, Kerem; Salk, Ismail; Müderris, Suphi

    2013-05-01

    Juvenile nasopharyngeal angiofibromas are locally growing and highly vascular tumors. They are primarily treated through surgical excision ranging from an open approach to an endoscopic approach. We presented a 20-year-old man with a giant juvenile nasopharyngeal angiofibroma that bilaterally obliterated the pterygopalatine fossa, invaded the sphenoid bone, and extended to the left nasal passage. His complaints were epistaxis and nasal obstruction. After embolization, the patient was treated surgically using the endoscopic approach and declared cured and discharged without any complications.

  18. A Giant Juvenile Nasopharyngeal Angiofibroma

    PubMed Central

    Yüce, Salim; Uysal, İsmail Önder; Doğan, Mansur; Polat, Kerem; Şalk, İsmail; Müderris, Suphi

    2012-01-01

    Juvenile nasopharyngeal angiofibroma (JNA) are locally growing highly vascular tumours. They are treated primarily by surgical excision ranging from open approach to endoscopic approach. We presented a 20-year-old male with a giant nasopharyngeal juvenile angiofibroma obliterating the pterygopalatine fossa bilaterally, invasing the sphenoid bone and extending to the left nasal passage. His complaints were epistaxis and nasal obstruction. After embolization, the patient was treated surgically with endoscopic approach and discharged as cured without any complication. PMID:23714961

  19. Juvenile xanthogranuloma with lichenoid appearance.

    PubMed

    Yoneda, Kozo; Demitsu, Toshio; Kubota, Yasuo

    2012-05-01

    Juvenile xanthogranulomas are benign histiocytic cell tumors that develop mainly in infancy and early childhood and then spontaneously regress. We report a 2-year-old boy who presented with generalized eruption of a mixture of micronodular and macronodular juvenile xanthogranuloma with a large number of widely distributed lichenoid papules. Light microscopic and immunocytochemical analyses of the lesion were consistent with juvenile xanthogranuloma. Abdominal ultrasonography did not detect any visceral lesions, and brain magnetic resonance imaging did not detect any mass lesions. We decided to observe the course without treatment in this case because there are no internal masses of juvenile xanthogranuloma. Regular follow up has therefore been scheduled. To our knowledge, this is the third report of a case demonstrating juvenile xanthogranuloma with lichenoid appearance. Future analyses of various cytokines such as granulocyte-macrophage colony-stimulating factor and/or tumor necrosis factor-α in juvenile xanthogranuloma lesions should be of great help in elucidating the pathogenesis of this disease. PMID:21980990

  20. A juvenile mouse pheromone inhibits sexual behaviour through the vomeronasal system.

    PubMed

    Ferrero, David M; Moeller, Lisa M; Osakada, Takuya; Horio, Nao; Li, Qian; Roy, Dheeraj S; Cichy, Annika; Spehr, Marc; Touhara, Kazushige; Liberles, Stephen D

    2013-10-17

    Animals display a repertoire of different social behaviours. Appropriate behavioural responses depend on sensory input received during social interactions. In mice, social behaviour is driven by pheromones, chemical signals that encode information related to age, sex and physiological state. However, although mice show different social behaviours towards adults, juveniles and neonates, sensory cues that enable specific recognition of juvenile mice are unknown. Here we describe a juvenile pheromone produced by young mice before puberty, termed exocrine-gland secreting peptide 22 (ESP22). ESP22 is secreted from the lacrimal gland and released into tears of 2- to 3-week-old mice. Upon detection, ESP22 activates high-affinity sensory neurons in the vomeronasal organ, and downstream limbic neurons in the medial amygdala. Recombinant ESP22, painted on mice, exerts a powerful inhibitory effect on adult male mating behaviour, which is abolished in knockout mice lacking TRPC2, a key signalling component of the vomeronasal organ. Furthermore, knockout of TRPC2 or loss of ESP22 production results in increased sexual behaviour of adult males towards juveniles, and sexual responses towards ESP22-deficient juveniles are suppressed by ESP22 painting. Thus, we describe a pheromone of sexually immature mice that controls an innate social behaviour, a response pathway through the accessory olfactory system and a new role for vomeronasal organ signalling in inhibiting sexual behaviour towards young. These findings provide a molecular framework for understanding how a sensory system can regulate behaviour.

  1. Juvenile temporal arteritis revisited.

    PubMed

    Tomlinson, F H; Lie, J T; Nienhuis, B J; Konzen, K M; Groover, R V

    1994-05-01

    We describe a case of arteritis involving the superficial temporal artery in an 8-year-old boy. After a 2-week prodrome of headache in the right temporal region, a painful pulsatile 6-mm nodule developed. No history of trauma or systemic disease was noted. The differential diagnosis included vasculitis or thrombosis of a vascular malformation of the temporal artery. The lesion was surgically excised for both diagnostic and cosmetic reasons. Histologic features of the nodule were diagnostic of juvenile temporal arteritis and characterized by non-giant cell granulomatous inflammation of the temporal artery, occlusive fibrous intimal proliferation, and microaneurysmal disruption of the media. At 12-month follow-up, the patient was well; no recurrent lesions or systemic disease was noted. Although rare, this disease should be recognized as arteritis that affects the external carotid circulation and should not be confused with classic giant cell temporal arteritis. If physicians are aware of this benign inflammatory disease of the temporal artery in children and young adults, unnecessary treatment will not be administered.

  2. Imaging for juvenile angiofibroma.

    PubMed

    Lloyd, G; Howard, D; Lund, V J; Savy, L

    2000-09-01

    Juvenile angiofibroma presents characteristic imaging signs, may of which allow diagnosis and accurate estimation of extent without recourse to the dangers of biopsy. The diagnosis by computed tomography (CT) is based upon the site of origin of the lesion in the pterygopalatine fossa. There are two constant features: (1) a mass in the posterior nasal cavity and pterygopalatine fossa; (2) erosion of bone behind the sphenopalatine foramen with extension to the upper medial pterygoid plate. Good bone imaging on CT is essential to show invasion of the cancellous bone of the sphenoid. This is the main predictor of recurrence: the deeper the extension, the larger the potential tumour remnant likely to be left following surgery. The characteristic features on magnetic resonance imaging (MRI) are due to the high vascularity of the tumour causing signal voids and strong post-contrast enhancement. MRI shows the pre-operative soft tissue extent of angiofibroma optimally, but its more important application is to provide post-operative surveillance: to show any residual or recurrent tumour, record tumour growth or natural involution and monitor the effects of radiotherapy.

  3. Managing juvenile Huntington's disease.

    PubMed

    Quarrell, Oliver W J; Nance, Martha A; Nopoulos, Peggy; Paulsen, Jane S; Smith, Jonathan A; Squitieri, Ferdinando

    2013-06-01

    Huntington's disease (HD) is a well-recognized progressive neurodegenerative disorder that follows an autosomal dominant pattern of inheritance. Onset is insidious and can occur at almost any age, but most commonly the diagnosis is made between the ages of 35 and 55 years. Onset ≤20 years of age is classified as juvenile HD (JHD). This age-based definition is arbitrary but remains convenient. There is overlap between the clinical pathological and genetic features seen in JHD and more traditional adult-onset HD. Nonetheless, the frequent predominance of bradykinesia and dystonia early in the course of the illness, more frequent occurrence of epilepsy and myoclonus, more widespread pathology, and larger genetic lesion means that the distinction is still relevant. In addition, the relative rarity of JHD means that the clinician managing the patient is often doing so for the first time. Management is, at best, symptomatic and supportive with few or no evidence-based guidelines. In this article, the authors will review what is known of the condition and present some suggestions based on their experience. PMID:24416077

  4. Farnesol-Detecting Olfactory Neurons in Drosophila

    PubMed Central

    Ronderos, David S.; Lin, Chun-Chieh; Potter, Christopher J.

    2014-01-01

    We set out to deorphanize a subset of putative Drosophila odorant receptors expressed in trichoid sensilla using a transgenic in vivo misexpression approach. We identified farnesol as a potent and specific activator for the orphan odorant receptor Or83c. Farnesol is an intermediate in juvenile hormone biosynthesis, but is also produced by ripe citrus fruit peels. Here, we show that farnesol stimulates robust activation of Or83c-expressing olfactory neurons, even at high dilutions. The CD36 homolog Snmp1 is required for normal farnesol response kinetics. The neurons expressing Or83c are found in a subset of poorly characterized intermediate sensilla. We show that these neurons mediate attraction behavior to low concentrations of farnesol and that Or83c receptor mutants are defective for this behavior. Or83c neurons innervate the DC3 glomerulus in the antennal lobe and projection neurons relaying information from this glomerulus to higher brain centers target a region of the lateral horn previously implicated in pheromone perception. Our findings identify a sensitive, narrowly tuned receptor that mediates attraction behavior to farnesol and demonstrates an effective approach to deorphanizing odorant receptors expressed in neurons located in intermediate and trichoid sensilla that may not function in the classical “empty basiconic neuron” system. PMID:24623773

  5. Forensic aspects of juvenile violence.

    PubMed

    Haller, L H

    2000-10-01

    The juvenile justice system was created because it was recognized that youthful offenders needed to be managed differently from adults. They were to receive habilitation services instead of punishment. It is now more than a century since the creation of the first juvenile court. After 67 years, the US Supreme Court, in Kent v United States stated that the model was not working because juveniles in the criminal justice system received no treatment and they had no rights. Because the issue that had been appealed was the lack of rights (not lack of treatment), the Court mandated that juveniles, like adults, be given certain rights. The following year, in In re Gault, the Court expanded these rights. Subsequent Supreme Court cases have dealt with these kinds of issues--that is, whether juvenile offenders are entitled to the same rights as adults and subject to the same penalties. The Supreme Court has never heard a "right to treatment" case, which is the other part of the juvenile court system. Cases have been brought in lower courts (e.g., Nelson v. Heyne, 1972) alleging inadequate treatment services, but no national impact has resulted. Thus, in general, children in the juvenile court system do not have an enforceable right to treatment and can obtain only what services are available in their jurisdictions. The services often are woefully inadequate. Sentencing a youth to probation, with the requirement that he or she participate in counseling or mental health treatment, is meaningless if services are not available. Community-based, model programs that provide effective treatment do exist. They are, as yet, the rare exception rather than the norm and, therefore, are not available to most youthful offenders. Incarcerated juveniles, obviously, cannot avail themselves of community programs. Litigation to give these youth the same rights as adults in penal institutions is not the answer because incarcerated adults don't have a right to treatment, only a right to be free

  6. The Heterogeneity of Juvenile Myositis

    PubMed Central

    Rider, Lisa G.

    2007-01-01

    Juvenile myositis is a heterogeneous group of systemic autoimmune diseases, in which clinical and serologic subgroups result in subsets of patients with distinct clinical manifestations, disease courses, immunogenetic associations, responses to therapy, and prognoses. A newly identified autoantibody of unknown specificity, anti-p155, is myositis-associated and seen in up to 20 – 30% of juvenile and adult DM patients. HLA DRB1*0301 and its linked allele DQA1*0501 have been identified as the major immunogenetic risk factor for juvenile and adult DM in both European- and African- American patients, and DQA1*0301 is an additional risk factor in European American patients. Several DQA1 alleles also are protective for juvenile DM. Environmental risk factors are poorly understood, but growing evidence suggests a role for infectious agents and ultraviolet radiation. The current therapy of juvenile DM consists of corticosteroids and other immunosuppressive agents, with the adjunctive treatment of cutaneous manifestations and rehabilitation. Therapeutic trials of biologic agents, including anti-TNFα and anti-CD20, may aid in developing promising new therapies for these disorders. PMID:17317616

  7. Genetics Home Reference: juvenile Paget disease

    MedlinePlus

    ... juvenile Paget disease: Genetic Testing Registry: Hyperphosphatasemia with bone disease These resources from MedlinePlus offer information about the ... familial osteoectasia hyperostosis corticalis deformans juvenilis hyperphosphatasemia ... idiopathic idiopathic hyperphosphatasia JPD juvenile Paget's ...

  8. New Treatments Helping Kids with Juvenile Arthritis

    MedlinePlus

    ... 159984.html New Treatments Helping Kids With Juvenile Arthritis Several biologics have been approved by the FDA ... 20, 2016 (HealthDay News) -- New treatments for juvenile arthritis offer hope to children with the chronic autoimmune ...

  9. Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions.

    PubMed

    Huang, Eric J; Zhang, Jiasheng; Geser, Felix; Trojanowski, John Q; Strober, Jonathan B; Dickson, Dennis W; Brown, Robert H; Shapiro, Barbara E; Lomen-Hoerth, Catherine

    2010-11-01

    Juvenile amyotrophic lateral sclerosis (ALS) with basophilic inclusions is a well-recognized entity. However, the molecular underpinnings of this devastating disease are poorly understood. Here, we present genetic and neuropathological characterizations in two young women with fatal rapidly progressive ALS with basophilic inclusions. In one case, a germline mutation (P525L) was detected in the fused in sarcoma/translocated in liposarcoma (FUS/TLS) gene, whereas no mutation was identified in the other case. Postmortem examination in both cases revealed severe loss of spinal motor neurons with remaining neurons showing basophilic inclusions that contain abnormal aggregates of FUS proteins and disorganized intracellular organelles, including mitochondria and endoplasmic reticulum. In both patients, the FUS-positive inclusions were also detected in neurons in layers IV-V of cerebral cortex and several brainstem nuclei. In contrast, spinal motor neurons in patients with late-onset sporadic ALS showed no evidence of abnormal accumulation of FUS protein. These results underscore the importance of FUS mutations and pathology in rapidly progressive juvenile ALS. Furthermore, our study represents the first detailed characterizations of neuropathological findings in rapidly progressive juvenile ALS patients with a mutation in the FUS/TLS gene.

  10. Characteristics of adopted juvenile delinquents.

    PubMed

    Kim, W J; Zrull, J P; Davenport, C W; Weaver, M

    1992-05-01

    There have been many reports describing the uniqueness of adopted children and adolescents' delinquent behaviors in terms of both their delinquent characteristics and courts' treatment of them. A total of 43 adopted juveniles, 32 extrafamilial (1.0%) and 11 intrafamilial (0.3%) adoptions were initially identified out of 3,280 juvenile delinquents. The adopted subjects were then compared with the demographically matched and offense matched nonadopted subjects. The family variables, such as marital and employment status of parents, were significantly different. However, there were only a few discernible trends, and in general there were no significant differences between the adopted and nonadopted juveniles in terms of their offense characteristics and dispositions. PMID:1592787

  11. Regulation of GABA Equilibrium Potential by mGluRs in Rat Hippocampal CA1 Neurons.

    PubMed

    Yang, Bo; Rajput, Padmesh S; Kumar, Ujendra; Sastry, Bhagavatula R

    2015-01-01

    The equilibrium potential for GABA-A receptor mediated currents (EGABA) in neonatal central neurons is set at a relatively depolarized level, which is suggested to be caused by a low expression of K+/Cl- co-transporter (KCC2) but a relatively high expression of Na+-K+-Cl- cotransporter (NKCC1). Theta-burst stimulation (TBS) in stratum radiatum induces a negative shift in EGABA in juvenile hippocampal CA1 pyramidal neurons. In the current study, the effects of TBS on EGABA in neonatal and juvenile hippocampal CA1 neurons and the underlying mechanisms were examined. Metabotropic glutamate receptors (mGluRs) are suggested to modulate KCC2 and NKCC1 levels in cortical neurons. Therefore, the involvement of mGluRs in the regulation of KCC2 or NKCC1 activity, and thus EGABA, following TBS was also investigated. Whole-cell patch recordings were made from Wistar rat hippocampal CA1 pyramidal neurons, in a slice preparation. In neonates, TBS induces a positive shift in EGABA, which was prevented by NKCC1 antisense but not NKCC1 sense mRNA. (RS)-a-Methyl-4-carboxyphenylglycine (MCPG), a group I and II mGluR antagonist, blocked TBS-induced shifts in both juvenile and neonatal hippocampal neurons. While blockade of mGluR1 or mGluR5 alone could interfere with TBS-induced shifts in EGABA in neonates, only a combined blockade could do the same in juveniles. These results indicate that TBS induces a negative shift in EGABA in juvenile hippocampal neurons but a positive shift in neonatal hippocampal neurons via corresponding changes in KCC2 and NKCC1 expressions, respectively. mGluR activation seems to be necessary for both shifts to occur while the specific receptor subtype involved seems to vary. PMID:26389591

  12. Regulation of GABA Equilibrium Potential by mGluRs in Rat Hippocampal CA1 Neurons.

    PubMed

    Yang, Bo; Rajput, Padmesh S; Kumar, Ujendra; Sastry, Bhagavatula R

    2015-01-01

    The equilibrium potential for GABA-A receptor mediated currents (EGABA) in neonatal central neurons is set at a relatively depolarized level, which is suggested to be caused by a low expression of K+/Cl- co-transporter (KCC2) but a relatively high expression of Na+-K+-Cl- cotransporter (NKCC1). Theta-burst stimulation (TBS) in stratum radiatum induces a negative shift in EGABA in juvenile hippocampal CA1 pyramidal neurons. In the current study, the effects of TBS on EGABA in neonatal and juvenile hippocampal CA1 neurons and the underlying mechanisms were examined. Metabotropic glutamate receptors (mGluRs) are suggested to modulate KCC2 and NKCC1 levels in cortical neurons. Therefore, the involvement of mGluRs in the regulation of KCC2 or NKCC1 activity, and thus EGABA, following TBS was also investigated. Whole-cell patch recordings were made from Wistar rat hippocampal CA1 pyramidal neurons, in a slice preparation. In neonates, TBS induces a positive shift in EGABA, which was prevented by NKCC1 antisense but not NKCC1 sense mRNA. (RS)-a-Methyl-4-carboxyphenylglycine (MCPG), a group I and II mGluR antagonist, blocked TBS-induced shifts in both juvenile and neonatal hippocampal neurons. While blockade of mGluR1 or mGluR5 alone could interfere with TBS-induced shifts in EGABA in neonates, only a combined blockade could do the same in juveniles. These results indicate that TBS induces a negative shift in EGABA in juvenile hippocampal neurons but a positive shift in neonatal hippocampal neurons via corresponding changes in KCC2 and NKCC1 expressions, respectively. mGluR activation seems to be necessary for both shifts to occur while the specific receptor subtype involved seems to vary.

  13. Regulation of GABA Equilibrium Potential by mGluRs in Rat Hippocampal CA1 Neurons

    PubMed Central

    Yang, Bo; Rajput, Padmesh S.; Kumar, Ujendra; Sastry, Bhagavatula R.

    2015-01-01

    The equilibrium potential for GABA-A receptor mediated currents (EGABA) in neonatal central neurons is set at a relatively depolarized level, which is suggested to be caused by a low expression of K+/Cl- co-transporter (KCC2) but a relatively high expression of Na+-K+-Cl- cotransporter (NKCC1). Theta-burst stimulation (TBS) in stratum radiatum induces a negative shift in EGABA in juvenile hippocampal CA1 pyramidal neurons. In the current study, the effects of TBS on EGABA in neonatal and juvenile hippocampal CA1 neurons and the underlying mechanisms were examined. Metabotropic glutamate receptors (mGluRs) are suggested to modulate KCC2 and NKCC1 levels in cortical neurons. Therefore, the involvement of mGluRs in the regulation of KCC2 or NKCC1 activity, and thus EGABA, following TBS was also investigated. Whole-cell patch recordings were made from Wistar rat hippocampal CA1 pyramidal neurons, in a slice preparation. In neonates, TBS induces a positive shift in EGABA, which was prevented by NKCC1 antisense but not NKCC1 sense mRNA. (RS)-a-Methyl-4-carboxyphenylglycine (MCPG), a group I and II mGluR antagonist, blocked TBS-induced shifts in both juvenile and neonatal hippocampal neurons. While blockade of mGluR1 or mGluR5 alone could interfere with TBS-induced shifts in EGABA in neonates, only a combined blockade could do the same in juveniles. These results indicate that TBS induces a negative shift in EGABA in juvenile hippocampal neurons but a positive shift in neonatal hippocampal neurons via corresponding changes in KCC2 and NKCC1 expressions, respectively. mGluR activation seems to be necessary for both shifts to occur while the specific receptor subtype involved seems to vary. PMID:26389591

  14. Guidelines for Juvenile Information Sharing. OJJDP Report

    ERIC Educational Resources Information Center

    Mankey, Jennifer; Baca, Patricia; Rondenell, Stephanie; Webb, Marilyn; McHugh, Denise

    2006-01-01

    The juvenile information sharing (JIS) guidelines were prepared by the Center for Network Development (CND) for the Office of Juvenile Justice and Delinquency Prevention (OJJDP). The guidelines suggest a course of action for key agency and organization stakeholders involved in a state or local effort to implement and sustain juvenile information…

  15. Disability and Juvenile Delinquency: Issues and Trends

    ERIC Educational Resources Information Center

    Morris, Kimberly A.; Morris, Richard J.

    2006-01-01

    The US juvenile justice system has gone through many changes since its inception in the late 1890s. Even with these changes and more than 100 years of empirical research, there is a paucity of literature published on juvenile delinquents with disabilities. The present article focuses on juvenile delinquents with disabilities, addressing…

  16. Families, Juvenile Justice and Children's Mental Health.

    ERIC Educational Resources Information Center

    McManus, Marilyn C., Ed.

    1997-01-01

    The theme issue of this bulletin is a discussion of youth with emotional disturbances who are in the juvenile justice system and how to meet their needs. Articles include: (1) "Responding to the Mental Health Needs of Youth in the Juvenile Justice System" (Susan Rotenberg); (2) "Prevalence of Mental Disorders among Youth in the Juvenile Justice…

  17. Counseling Juvenile Offenders in Institutional Settings.

    ERIC Educational Resources Information Center

    Chaneles, Sol, Ed.

    1982-01-01

    Reviews several aspects of counseling services for institutionalized juvenile offenders. The six articles include studies on the functional analysis of behavior in detention, vocational and social rehabilitation, the impact of a juvenile awareness program on personality traits, and the effectiveness of a juvenile transition center. (JAC)

  18. On the Prevention of Juvenile Crime

    ERIC Educational Resources Information Center

    Lelekov, V. A.; Kosheleva, E. V.

    2008-01-01

    Crimes committed by juveniles are among the most urgent social problems. Juvenile crime is as prevalent as crime itself is, and it has not been solved completely in any society and cannot be solved through law enforcement measures alone. In this article, the authors discuss the dynamics and structure of juvenile crime in Russia and present data…

  19. Intensive Reading Instruction in Juvenile Correctional Settings

    ERIC Educational Resources Information Center

    Williams, Jacob L.; Wexler, Jade; Roberts, Greg; Carpenter, Clint

    2011-01-01

    Despite 60 years of evidence linking juvenile illiteracy and delinquency, practitioners and policymakers have been painfully slow in the implementation of evidence-based reading interventions for incarcerated juveniles. We will present the Texas Juvenile Justice Tiered Instructional Model, an evidence-based reading program model created…

  20. Reforming Our Expectations about Juvenile Justice

    ERIC Educational Resources Information Center

    Rodriguez, Pamela F.; Baille, Daphne M.

    2010-01-01

    Typing the term "juvenile justice reform" into a Google[TM] search will result in 60 pages of entries. But what is meant by juvenile justice reform? What does it look like? How will one know when it is achieved? This article defines juvenile justice reform, discusses the principles of effective reform, and describes the practice of juvenile…

  1. Prevention of Serious and Violent Juvenile Offending. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Wasserman, Gail A.; Miller, Laurie S.; Cothern, Lynn

    This bulletin explores the proximal risk factors for juvenile offending, reviews the early developmental precursors to violent offending, and summarizes approaches to prevention. It also discusses components of intervention programs, limitations of single-focus prevention, examples of multi systemic interventions, and limitations of prevention…

  2. Special Education and the Juvenile Justice System. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Burrell, Sue; Warboys, Loren

    This bulletin summarizes provisions of federal law as they pertain to special education and juvenile justice. It discusses provisions of the Individuals with Disabilities Education Act 1997 including: the definition of disability; free appropriate public education; identification, referral, and evaluation; the individualized education program…

  3. Mobilizing Communities To Prevent Juvenile Crime. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Bownes, Donna; Ingersoll, Sarah

    Through Title V Incentive Grants for Local Delinquency Prevention Programs (Community Prevention Grants), the Office of Juvenile Justice and Delinquency Prevention (OJJDP) allocated $20 million in fiscal year 1997 to states to complement law enforcement and justice system efforts by helping local communities foster strong families and nurture…

  4. Race as a Factor in Juvenile Arrests. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Pope, Carl E.; Snyder, Howard E.

    This bulletin examines the effect of race on police decisions to take juvenile offenders into custody. Analysis of 1997 and 1998 data on 17 states from the Federal Bureau of Investigation's National Incident-Based Reporting System indicates that there is no evidence to support the hypothesis that police are more likely to arrest nonwhite juvenile…

  5. Oregon's Juvenile Psychiatric Security Review Board.

    PubMed

    Newman, Stewart S; Buckley, Mary Claire; Newman, Senia Pickering; Bloom, Joseph D

    2007-01-01

    In 2005, the Oregon Legislature passed a bill modifying the existing Psychiatric Security Review Board (PRSB) statute, creating a juvenile panel for management of juvenile insanity acquittees. Dubbed the Juvenile PSRB (JPSRB), it borrows heavily from the 30 years of experience of its adult predecessor. Statutory language was also modified to create a plea of "responsible except for insanity" for juveniles in Oregon. The authors discuss the similarities of the JPSRB to the adult PSRB system and highlight the differences that take into account the unique needs of juvenile defendants. They go on to discuss potential problems foreseen with implementation of the JPSRB system and to recommend possible solutions.

  6. Factors affecting attitudes toward juvenile sex offenders.

    PubMed

    Sahlstrom, Kimberly J; Jeglic, Elizabeth L

    2008-01-01

    This study investigated attitudes toward juvenile sex offenders and factors influencing those attitudes. Additionally, the influences of perpetrator characteristics such as age, gender, and ethnicity on societal attitudes towards intervention requirements were also investigated. Overall, attitudes toward juvenile sex offenders and their treatment amenability were negative. No differences in attitudes toward juvenile sex offenders were found between those who had been victims of sexual abuse and those that had not. Sex offenses committed by juvenile female sex offenders were viewed to be more serious and require more intervention than those committed by juvenile male sex offenders. PMID:19042245

  7. Juvenile Diabetes and Rehabilitation Counseling.

    ERIC Educational Resources Information Center

    Stone, J. Blair; Gregg, Charles H.

    1981-01-01

    Severe complications of diabetes are more likely to occur with the juvenile diabetic and problems of psychosocial adjustment are recurring and difficult. Implications for the rehabilitation counselor are discussed in terms of employment considerations, the effects of complications, genetic counseling, and cooperation with other professionals.…

  8. Juvenile Justice and Substance Use

    ERIC Educational Resources Information Center

    Chassin, Laurie

    2008-01-01

    Laurie Chassin focuses on the elevated prevalence of substance use disorders among young offenders in the juvenile justice system and on efforts by the justice system to provide treatment for these disorders. She emphasizes the importance of diagnosing and treating these disorders, which are linked both with continued offending and with a broad…

  9. [Juvenile angiofibroma. Results of radiotherapy].

    PubMed

    Rosset, A; Korzeniowski, S

    1990-01-01

    8 patients with the nasofibromata were treated by radiotherapy in Oncologic Center in Kraków. In most part of these patients tumors exceeded the nasopharynx or gave the massive postoperational recurrencies. Complete regression was obtained in 6 out of 8 cases. The radiation changes are described. The radiotherapy is effective in more advanced and recurrent stages of the juvenile nasofibroma.

  10. Juvenile Crime, Juvenile Justice. Panel on Juvenile Crime: Prevention, Treatment, and Control.

    ERIC Educational Resources Information Center

    McCord, Joan, Ed.; Widom, Cathy Spatz, Ed.; Crowell, Nancy A., Ed.

    This book discusses patterns and trends in crimes committed by children and adolescents, analyzing youth crime as a subset of general crime and studying the impact of race and gender. It evaluates different approaches to forecasting future crime rates. Data come from a national panel that examined what is known about juvenile crime and its…

  11. Juvenile amyotrophic lateral sclerosis: Classical wine glass sign on magnetic resonance imaging

    PubMed Central

    Kumar, Saurabh; Aga, Pallavi; Gupta, Aakansha; Kohli, Neera

    2016-01-01

    Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig disease, is a chronic degenerative neurologic disease and is characterized by the selective involvement of the motor system. Usually, patients present with upper motor neuron (UMN) and lower motor neuron compromise. Degeneration of the UMN in the cerebral cortex is one of the main pathologic changes in ALS. These changes usually affect corticospinal tracts leading to degeneration of the fibers which show characteristic hyperintensities along the tracts leading to the “wine glass sign.” Patients with ALS usually present in the sixth decade of life; presentation in pediatric age in the form of juvenile ALS being rare. PMID:27195035

  12. [Neuronal network].

    PubMed

    Langmeier, M; Maresová, D

    2005-01-01

    Function of the central nervous system is based on mutual relations among the nerve cells. Description of nerve cells and their processes, including their contacts was enabled by improvement of optical features of the microscope and by the development of impregnation techniques. It is associated with the name of Antoni van Leeuwenhoek (1632-1723), J. Ev. Purkyne (1787-1869), Camillo Golgi (1843-1926), and Ramón y Cajal (1852-1934). Principal units of the neuronal network are the synapses. The term synapse was introduced into neurophysiology by Charles Scott Sherrington (1857-1952). Majority of the interactions between nerve cells is mediated by neurotransmitters acting at the receptors of the postsynaptic membrane or at the autoreceptors of the presynaptic part of the synapse. Attachment of the vesicles to the presynaptic membrane and the release of the neurotransmitter into the synaptic cleft depend on the intracellular calcium concentration and on the presence of several proteins in the presynaptic element.

  13. MAPK signaling determines anxiety in the juvenile mouse brain but depression-like behavior in adults.

    PubMed

    Wefers, Benedikt; Hitz, Christiane; Hölter, Sabine M; Trümbach, Dietrich; Hansen, Jens; Weber, Peter; Pütz, Benno; Deussing, Jan M; de Angelis, Martin Hrabé; Roenneberg, Till; Zheng, Fang; Alzheimer, Christian; Silva, Alcino; Wurst, Wolfgang; Kühn, Ralf

    2012-01-01

    MAP kinase signaling has been implicated in brain development, long-term memory, and the response to antidepressants. Inducible Braf knockout mice, which exhibit protein depletion in principle forebrain neurons, enabled us to unravel a new role of neuronal MAPK signaling for emotional behavior. Braf mice that were induced during adulthood showed normal anxiety but increased depression-like behavior, in accordance with pharmacological findings. In contrast, the inducible or constitutive inactivation of Braf in the juvenile brain leads to normal depression-like behavior but decreased anxiety in adults. In juvenile, constitutive mutants we found no alteration of GABAergic neurotransmission but reduced neuronal arborization in the dentate gyrus. Analysis of gene expression in the hippocampus revealed nine downregulated MAPK target genes that represent candidates to cause the mutant phenotype.Our results reveal the differential function of MAPK signaling in juvenile and adult life phases and emphasize the early postnatal period as critical for the determination of anxiety in adults. Moreover, these results validate inducible gene inactivation as a new valuable approach, allowing it to discriminate between gene function in the adult and the developing postnatal brain. PMID:22529971

  14. Methylphenidate and the juvenile brain: enhancement of attention at the expense of cortical plasticity?

    PubMed

    Urban, Kimberly R; Gao, Wen-Jun

    2013-12-01

    Methylphenidate (Ritalin) is the most commonly prescribed psychoactive drug for juveniles and adolescents. Used to treat attention-deficit/hyperactivity disorder (ADHD) and for cognitive enhancement in healthy individuals, it has been regarded as a relatively safe medication for the past several decades. However, a thorough review of the literature reveals that the age-dependent activities of the drug, as well as potential developmental effects, are largely ignored. In addition, the diagnosis of ADHD is subjective, leaving open the possibility of misdiagnosis and excessive prescription of the drug. Recent studies have suggested that early life exposure of healthy rodent models to methylphenidate resulted in altered sleep/wake cycle, heightened stress reactivity, and, in fact, a dosage previously thought of as therapeutic depressed neuronal function in juvenile rats. Furthermore, juvenile rats exposed to low-dose methylphenidate displayed alterations in neural markers of plasticity, indicating that the drug might alter the basic properties of prefrontal cortical circuits. In this review of the current literature, we propose that juvenile exposure to methylphenidate may cause abnormal prefrontal function and impaired plasticity in the healthy brain, strengthening the case for developing a more thorough understanding of methylphenidate's actions on the developing, juvenile brain, as well as better diagnostic measures for ADHD.

  15. Juvenile morphology in baleen whale phylogeny.

    PubMed

    Tsai, Cheng-Hsiu; Fordyce, R Ewan

    2014-09-01

    Phylogenetic reconstructions are sensitive to the influence of ontogeny on morphology. Here, we use foetal/neonatal specimens of known species of living baleen whales (Cetacea: Mysticeti) to show how juvenile morphology of extant species affects phylogenetic placement of the species. In one clade (sei whale, Balaenopteridae), the juvenile is distant from the usual phylogenetic position of adults, but in the other clade (pygmy right whale, Cetotheriidae), the juvenile is close to the adult. Different heterochronic processes at work in the studied species have different influences on juvenile morphology and on phylogenetic placement. This study helps to understand the relationship between evolutionary processes and phylogenetic patterns in baleen whale evolution and, more in general, between phylogeny and ontogeny; likewise, this study provides a proxy how to interpret the phylogeny when fossils that are immature individuals are included. Juvenile individuals in the peramorphic acceleration clades would produce misleading phylogenies, whereas juvenile individuals in the paedomorphic neoteny clades should still provide reliable phylogenetic signals.

  16. Juvenile morphology in baleen whale phylogeny

    NASA Astrophysics Data System (ADS)

    Tsai, Cheng-Hsiu; Fordyce, R. Ewan

    2014-09-01

    Phylogenetic reconstructions are sensitive to the influence of ontogeny on morphology. Here, we use foetal/neonatal specimens of known species of living baleen whales (Cetacea: Mysticeti) to show how juvenile morphology of extant species affects phylogenetic placement of the species. In one clade (sei whale, Balaenopteridae), the juvenile is distant from the usual phylogenetic position of adults, but in the other clade (pygmy right whale, Cetotheriidae), the juvenile is close to the adult. Different heterochronic processes at work in the studied species have different influences on juvenile morphology and on phylogenetic placement. This study helps to understand the relationship between evolutionary processes and phylogenetic patterns in baleen whale evolution and, more in general, between phylogeny and ontogeny; likewise, this study provides a proxy how to interpret the phylogeny when fossils that are immature individuals are included. Juvenile individuals in the peramorphic acceleration clades would produce misleading phylogenies, whereas juvenile individuals in the paedomorphic neoteny clades should still provide reliable phylogenetic signals.

  17. Electroreceptive Prey-Location Coding by the Juvenile Paddlefish

    NASA Astrophysics Data System (ADS)

    van Hemmen, J. Leo

    2003-03-01

    The long rostrum of the paddlefish (Polyodon spathula), a Mississippi river inhabitant, supports an extensive array of ampullary electroreceptors that are used to locate its favorite prey, water fleas, in dark and muddy water. Neuronal coding of such real-world events is often considered to be optimal in the sense of minimizing a mean-square reconstruction error or maximizing likelihood. Implementation of these theoretically motivated optimality criteria is, however, computationally very costly. For the juvenile paddlefish we exhibit [1] a computationally cheap and geometrically simple algorithm with the neuronal activity νn of its electroreceptors 1<= n <= N as input and evaluating the turning direction \\varphi required to catch the prey through the so-called population-vector code \\varphi^estimate = arg [sumn νn exp(i \\varphi_n)]. This explains experimentally found prey-detection statistics more convincingly than minimization of the mean-square reconstruction error. The only assumption concerns the neuronal time scale of the sensory organs. [1] C. Leibold, K.B. Reuter, L. Voigts, W. Wojtenek, and J.L. van Hemmen, manuscript in preparation.

  18. Sex hormones in juvenile nasopharyngeal angiofibroma tissue.

    PubMed

    Kumagami, H

    1993-01-01

    Five cases of juvenile nasopharyngeal angiofibroma were studied in terms of the presence of progesterone, estradiol, testosterone, and dihydrotestosterone in the juvenile nasopharyngeal angiofibroma tissue using the peroxidase-antiperoxidase method. Progesterone and estradiol were positive in all cases. Testosterone was positive in 2 of the 5 patients. Dihydrotestosterone was positive in 3 of the 5 patients. Hormone in the juvenile nasopharyngeal angiofibroma tissue seems to change by the activity of nasopharyngeal angiofibroma.

  19. Black Juveniles in the Juvenile Justice System: A Cause for Alarm.

    ERIC Educational Resources Information Center

    LeFlore, Larry

    This report examines the representation of black youth in the juvenile justice system, describes changes in juvenile justice philosophy, and discusses policy implications. Black youth are overrepresented at all stages of the juvenile justice system compared to white youth. Positivist theories explain this overrepresentation as the result of…

  20. Juvenile Practice Is Not Child's Play: A Handbook for Attorneys Who Represent Juveniles in Texas.

    ERIC Educational Resources Information Center

    2002

    This handbook is an attempt to summarize the most important aspects of juvenile law for a new practitioner, and to offer some additional ideas and strategies to any juvenile defense attorney. The goal is to help improve representation of juveniles across the state of Texas. References to useful books, cases, and statutes are included. The handbook…

  1. JUVENILE DELINQUENCY AND YOUTH CRIME, TASK FORCE REPORT, REPORT ON JUVENILE JUSTICE AND CONSULTANTS PAPERS.

    ERIC Educational Resources Information Center

    President's Commission on Law Enforcement and Administration of Justice, Washington, DC.

    THIS REPORT CONSISTS OF A DETAILED DISCUSSION OF THE JUVENILE COURT SYSTEM AND THE PREVENTION OF DELINQUENCY. THE COMMISSION'S RECOMMENDATIONS ON JUVENILE DELINQUENCY INCLUDE THE AREAS OF THE JUVENILE JUSTICE SYSTEM, HOUSING AND RECREATION, FAMILIES, INVOLVING YOUTHS IN COMMUNITY LIFE, SCHOOLS, AND EMPLOYMENT. THE APPENDIXES, WHICH CONSTITUTE THE…

  2. A Handbook for Juveniles and Parents on Maine's Juvenile Justice System.

    ERIC Educational Resources Information Center

    Mehnert, Irene

    This guide explains Maine's juvenile justice system so that juveniles and/or their parents can know what to expect or what to do in a situation involving juveniles, public officials and the law. Although it is geographically specific, it could serve as a model to other states. The booklet can serve as a checklist to make sure law enforcement…

  3. Planning for Juvenile Detention Reforms: A Structured Approach. Pathways to Juvenile Detention Reform 1.

    ERIC Educational Resources Information Center

    Steinhart, David

    This report is a guide to juvenile detention planning, based largely on the experiences of Juvenile Detention Alternatives Initiative (JDAI) sites. Its eight chapters include: (1) "Why Is Comprehensive Juvenile Detention Planning Needed?"; (2) "Guiding Principles" (e.g., detention planning must be based on adequate data, must be collaborative,…

  4. Runaway Juvenile Crime? The Context of Juvenile Arrests in America. Research in Brief.

    ERIC Educational Resources Information Center

    Ziedenberg, Jason; Schiraldi, Vincent

    The Violent and Repeat Juvenile Offender Act of 1997 (S-10) was to be debated in the Senate in spring 1998. This bill would blur the distinction between juvenile and adult criminal systems, making it easier to imprison children as young as 14. Supporters of S-10 were citing statistics to indicate that juvenile crime was on the rise. In fact, the…

  5. National Implications in Juvenile Justice: The Influence of Juvenile Mentoring Programs on At Risk Youth.

    ERIC Educational Resources Information Center

    Belshaw, Scott H.; Kritsonis, William Allan

    2007-01-01

    In 1972 the federal government created the Juvenile Justice Delinquency Prevention Act that procured funding for various governmental programs to combat the sudden increase in juvenile crime. A provision of this Act set out the creation of mentoring programs to help decrease the juvenile crime rate and dropout rates in secondary schools. This…

  6. Developmental change in the contribution of voltage-gated Ca(2+) channels to the pacemaking of deep cerebellar nuclei neurons.

    PubMed

    Alviña, K; Tara, E; Khodakhah, K

    2016-05-13

    The activity of the deep cerebellar nuclei (DCN) neurons conveys the bulk of the output of the cerebellum. To generate these motor signals, DCN neurons integrate synaptic inputs with their own spontaneous activity. We have previously reported that N-type voltage-gated Ca(2+) channels modulate the spontaneous activity of the majority of juvenile DCN neurons in vitro. Specifically, pharmacologically blocking N-type Ca(2+) channels increases their firing rate causing DCN cells to burst. Adult DCN neurons however, behaved differently. To further investigate this change, we have studied here the effect of cadmium on the firing rate of DCN neurons in acute cerebellar slices obtained from adult (>2 months old) or juvenile (12-21 days old) rats and mice. Strikingly, and in contrast to juvenile DCN cells, cadmium did not affect the pacemaking of adult DCN cells. The activity of Purkinje cells (PCs) however was transformed into high-frequency bursting, regardless the age. Further, we questioned whether these findings could be due to an artifact associated with the added difficulty of preparing adult DCN slices. Hence we proceeded to examine the spontaneous activity of DCN neurons in anesthetized juvenile and adult rats and mice in vivo. When cadmium was injected into the DCN in vivo no significant change in firing rate was observed, conversely to most juvenile DCN neurons which showed high-frequency bursts after cadmium injection. In these same animals, PCs pacemaking showed no developmental difference. Thus our results demonstrate a remarkable age-dependent functional modification in the regulation of DCN neurons pacemaking. PMID:26902515

  7. Age-Dependent Neurogenesis and Neuron Numbers within the Olfactory Bulb and Hippocampus of Homing Pigeons

    PubMed Central

    Meskenaite, Virginia; Krackow, Sven; Lipp, Hans-Peter

    2016-01-01

    Many birds are supreme long-distance navigators that develop their navigational ability in the first months after fledgling but update the memorized environmental information needed for navigation also later in life. We studied the extent of juvenile and adult neurogenesis that could provide such age-related plasticity in brain regions known to mediate different mechanisms of pigeon homing: the olfactory bulb (OB), and the triangular area of the hippocampal formation (HP tr). Newly generated neurons (visualized by doublecortin, DCX) and mature neurons were counted stereologically in 35 pigeon brains ranging from 1 to 168 months of age. At the age of 1 month, both areas showed maximal proportions of DCX positive neurons, which rapidly declined during the first year of life. In the OB, the number of DCX-positive periglomerular neurons declined further over time, but the number of mature periglomerular cells appeared unchanged. In the hippocampus, the proportion of DCX-positive neurons showed a similar decline yet to a lesser extent. Remarkably, in the triangular area of the hippocampus, the oldest birds showed nearly twice the number of neurons as compared to young adult pigeons, suggesting that adult born neurons in these regions expanded the local circuitry even in aged birds. This increase might reflect navigational experience and, possibly, expanded spatial memory. On the other hand, the decrease of juvenile neurons in the aging OB without adding new circuitry might be related to the improved attachment to the loft characterizing adult and old pigeons. PMID:27445724

  8. Impairment of Oligodendroglia Maturation Leads to Aberrantly Increased Cortical Glutamate and Anxiety-Like Behaviors in Juvenile Mice

    PubMed Central

    Chen, Xianjun; Zhang, Weiguo; Li, Tao; Guo, Yu; Tian, Yanping; Wang, Fei; Liu, Shubao; Shen, Hai-Ying; Feng, Yue; Xiao, Lan

    2015-01-01

    Adolescence is the critical time for developing proper oligodendrocyte (OL)-neuron interaction and the peak of onset for many cognitive diseases, among which anxiety disorders display the highest prevalence. However, whether impairment of de novo OL development causes neuronal abnormalities and contributes to the early onset of anxiety phenotype in childhood still remains unexplored. In this study, we tested the hypothesis that defects in OL maturation manifests cortical neuron function and leads to anxiety-like behaviors in juvenile mice. We report here that conditional knockout of the Olig2 gene (Olig2 cKO) specifically in differentiating OLs in the mouse brain preferentially impaired OL maturation in the gray matter of cerebral cortex. Interestingly, localized proton magnetic resonance spectroscopy revealed that Olig2 cKO mice displayed abnormally elevated cortical glutamate levels. In addition, transmission electron microscopy demonstrated increased vesicle density in excitatory glutamatergic synapses in the cortex of the Olig2 cKO mice. Moreover, juvenile Olig2 cKO mice exhibited anxiety-like behaviors and impairment in behavioral inhibition. Taken together, our results suggest that impaired OL development affects glutamatergic neuron function in the cortex and causes anxiety-related behaviors in juvenile mice. These discoveries raise an intriguing possibility that OL defects may be a contributing mechanism for the onset of anxiety in childhood. PMID:26696827

  9. Glucocorticoids in juvenile idiopathic arthritis.

    PubMed

    Malattia, Clara; Martini, Alberto

    2014-05-01

    Although the use of corticosteroids in juvenile idiopathic arthritis (JIA) is now much more limited owing to the availability of methotrexate and biological agents, there are clinical scenarios where it is still indicated. For example, corticosteroids may be indicated for intraarticular injections to prevent joint deformities, as a "bridge" drug to relieve symptoms in polyarticular disease while waiting for methotrexate and biologics to exert their full therapeutic effects, and in the treatment of chronic iridocyclitis, macrophage activation syndrome, and systemic JIA, although the advent of interleukin (IL)-1 and IL-6 blockers has greatly reduced the latter indication.

  10. Juvenile Offenders and Victims: 2006 National Report

    ERIC Educational Resources Information Center

    Snyder, Howard N.; Sickmund, Melissa

    2006-01-01

    This report presents comprehensive information on juvenile crime, violence, and victimization and on the juvenile justice system. This report brings together the latest available statistics from a variety of sources and includes numerous tables, graphs, and maps, accompanied by analyses in clear, nontechnical language. The report offers Congress,…

  11. Juvenile Offenders and Victims: 1999 National Report.

    ERIC Educational Resources Information Center

    Snyder, Howard N.; Sickmund, Melissa

    This report offers the Congress, state legislators, and other state and local policymakers, professors and teachers, juvenile justice professionals, and concerned citizens solid answers to the most frequently asked questions about the nature of juvenile crime and victimization and about the justice system's response. Citing FBI and other data…

  12. Moral Development of Solo Juvenile Sex Offenders

    ERIC Educational Resources Information Center

    Van Vugt, Eveline; Stams, Geert Jan; Dekovic, Maja; Brugman, Daan; Rutten, Esther; Hendriks, Jan

    2008-01-01

    This study compared the moral development of solo juvenile male sex offenders (n = 20) and juvenile male non-offenders (n = 76), aged 13-19 years, from lower socioeconomic and educational backgrounds. The Moral Orientation Measure (MOM) was used to assess punishment- and victim-based moral orientation in sexual and non-sexual situations. Moral…

  13. Juvenile Delinquency: Research, Theory, and Comment.

    ERIC Educational Resources Information Center

    Moore, Bernice Milburn

    While this booklet on juvenile delinquency does not attempt a full review of the literature, it has been designed to further an understanding and appreciation of the social-psychological problems of deviant behavior. The booklet briefly covers the publicity which juvenile delinquency has been given in recent years, as well as the difficulties…

  14. Juveniles' Motivations for Remaining in Prostitution

    ERIC Educational Resources Information Center

    Hwang, Shu-Ling; Bedford, Olwen

    2004-01-01

    Qualitative data from in-depth interviews were collected in 1990-1991, 1992, and 2000 with 49 prostituted juveniles remanded to two rehabilitation centers in Taiwan. These data are analyzed to explore Taiwanese prostituted juveniles' feelings about themselves and their work, their motivations for remaining in prostitution, and their difficulties…

  15. Juvenile Obesity, Physical Activity, and Lifestyle Changes.

    ERIC Educational Resources Information Center

    Bar-Or, Oded

    2000-01-01

    Because many obese children become obese adults, the recent rapid increase in juvenile obesity poses a major public health challenge. Enhanced physical activity is a cornerstone in a multidisciplinary approach to preventing and treating juvenile obesity. Giving exercise recommendations focused for obese youth is critical. Cutting down on sedentary…

  16. Peer Relationships Among Institutionalized Juvenile Boys

    ERIC Educational Resources Information Center

    Preveaux, Neal E.; Ray, Glen E.; LoBello, Steven G.; Mehta, Sheila

    2004-01-01

    This study examined peer relationships (sociometric status and friendship) of institutionalized juvenile males ages 12 to 18. Results replicated previous studies using "normal" nondelinquent samples demonstrating that sociometrically popular status juveniles were evaluated higher on sociability and leadership than were average- or rejected-status…

  17. Wilderness/Adventure Programs for Juvenile Offenders.

    ERIC Educational Resources Information Center

    Kimball, Richard Owen

    Over 80 wilderness/adventure programs have emerged as a valuable alternative to traditional treatment for juvenile offenders, especially in combination with other services. Participants are referred from many points in the juvenile justice system by agents who should have a thorough understanding of wilderness programs so as to prepare the…

  18. Factors Affecting Attitudes toward Juvenile Sex Offenders

    ERIC Educational Resources Information Center

    Sahlstrom, Kimberly J.; Jeglic, Elizabeth L.

    2008-01-01

    This study investigated attitudes toward juvenile sex offenders and factors influencing those attitudes. Additionally, the influences of perpetrator characteristics such as age, gender, and ethnicity on societal attitudes towards intervention requirements were also investigated. Overall, attitudes toward juvenile sex offenders and their treatment…

  19. Literacy Levels of Male Juvenile Justice Detainees

    ERIC Educational Resources Information Center

    Wheldall, Kevin; Watkins, Renae

    2004-01-01

    The assessment records detailing the reading and spelling performance of a group of male juvenile justice detainees admitted over a 3-month period were examined in an attempt to quantify the basic literacy levels of juvenile offenders. Results of student self-ratings of their reading ability were also analysed. The participants comprised 68 males…

  20. Juvenile Offender Comprehensive Reentry Substance Abuse Treatment

    ERIC Educational Resources Information Center

    Watson, Donnie W.

    2004-01-01

    The literature provides ample evidence of the relationship of substance abuse to crime. Research over the last 20 years has established a strong correlation between substance abuse and juvenile delinquency (held, 1998). Currently, there are more than 350,000 juveniles on probation and in continuing care programs in the U.S. who have substance…

  1. Juvenile Anorexia Nervosa: Family Therapy's Natural Niche

    ERIC Educational Resources Information Center

    Fishman, H. Charles

    2006-01-01

    Juvenile Anorexia Nervosa (AN) is a severe problem both in terms of presenting symptomatology and its tendency toward chronicity. Researchers have consistently shown that family-based approaches are superior to individual approaches for the treatment of juvenile AN. This article addresses the capacity deficit of trained family therapists to treat…

  2. Learning and Memory Deficits in Male Adult Mice Treated with a Benzodiazepine Sleep-Inducing Drug during the Juvenile Period.

    PubMed

    Furukawa, Yusuke; Tanemura, Kentaro; Igarashi, Katsuhide; Ideta-Otsuka, Maky; Aisaki, Ken-Ichi; Kitajima, Satoshi; Kitagawa, Masanobu; Kanno, Jun

    2016-01-01

    Gamma-aminobutyric acid (GABA), the major inhibitory neurotransmitter in the mammalian central nervous system, is also known to be important for brain development. Therefore, disturbances of GABA receptor (GABA-R) mediated signaling (GABA-R signal) during brain development may influence normal brain maturation and cause late-onset brain malfunctions. In this study, we examined whether the stimulation of the GABA-R signal during brain development induces late-onset adverse effects on the brain in adult male mice. To stimulate the GABA-R signal, we used either the benzodiazepine sleep-inducing drug triazolam (TZ) or the non-benzodiazepine drug zolpidem (ZP). We detected learning and memory deficits in mice treated with TZ during the juvenile period, as seen in the fear conditioning test. On the other hand, ZP administration during the juvenile period had little effect. In addition, decreased protein expression of GluR1 and GluR4, which are excitatory neurotransmitter receptors, was detected in the hippocampi of mice treated with TZ during the juvenile period. We measured mRNA expression of the immediate early genes (IEGs), which are neuronal activity markers, in the hippocampus shortly after the administration of TZ or ZP to juvenile mice. Decreased IEG expression was detected in mice with juvenile TZ administration, but not in mice with juvenile ZP administration. Our findings demonstrate that TZ administration during the juvenile period can induce irreversible learning and memory deficits in adult mice. It may need to take an extra care for the prescription of benzodiazepine sleep-inducing drugs to juveniles because it might cause learning and memory deficits. PMID:27489535

  3. Learning and Memory Deficits in Male Adult Mice Treated with a Benzodiazepine Sleep-Inducing Drug during the Juvenile Period

    PubMed Central

    Furukawa, Yusuke; Tanemura, Kentaro; Igarashi, Katsuhide; Ideta-Otsuka, Maky; Aisaki, Ken-Ichi; Kitajima, Satoshi; Kitagawa, Masanobu; Kanno, Jun

    2016-01-01

    Gamma-aminobutyric acid (GABA), the major inhibitory neurotransmitter in the mammalian central nervous system, is also known to be important for brain development. Therefore, disturbances of GABA receptor (GABA-R) mediated signaling (GABA-R signal) during brain development may influence normal brain maturation and cause late-onset brain malfunctions. In this study, we examined whether the stimulation of the GABA-R signal during brain development induces late-onset adverse effects on the brain in adult male mice. To stimulate the GABA-R signal, we used either the benzodiazepine sleep-inducing drug triazolam (TZ) or the non-benzodiazepine drug zolpidem (ZP). We detected learning and memory deficits in mice treated with TZ during the juvenile period, as seen in the fear conditioning test. On the other hand, ZP administration during the juvenile period had little effect. In addition, decreased protein expression of GluR1 and GluR4, which are excitatory neurotransmitter receptors, was detected in the hippocampi of mice treated with TZ during the juvenile period. We measured mRNA expression of the immediate early genes (IEGs), which are neuronal activity markers, in the hippocampus shortly after the administration of TZ or ZP to juvenile mice. Decreased IEG expression was detected in mice with juvenile TZ administration, but not in mice with juvenile ZP administration. Our findings demonstrate that TZ administration during the juvenile period can induce irreversible learning and memory deficits in adult mice. It may need to take an extra care for the prescription of benzodiazepine sleep-inducing drugs to juveniles because it might cause learning and memory deficits. PMID:27489535

  4. Family transitions and juvenile delinquency.

    PubMed

    Schroeder, Ryan D; Osgood, Aurea K; Oghia, Michael J

    2010-01-01

    There is a large body of research that shows children from non-intact homes show higher rates of juvenile delinquency than children from intact homes, partially due to weaker parental control and supervision in non-intact homes. What has not been adequately addressed in the research is the influence of changes in family structure among individual adolescents over time on delinquent offending. Using the first and third waves of the National Youth Study, we assess the effect of family structure changes on changes in delinquent offending between waves through the intermediate process of changes in family time and parental attachment. Although prior research has documented adolescents in broken homes are more delinquent than youth in intact homes, the process of family dissolution is not associated with concurrent increases in offending. In contrast, family formation through marriage or cohabitation is associated with simultaneous increases in offending. Changes in family time and parental attachment account for a portion of the family formation effect on delinquency, and prior parental attachment and juvenile offending significantly condition the effect of family formation on offending. PMID:20879178

  5. Juvenile Offenders with Mental Health Needs: Reducing Recidivism Using Wraparound

    ERIC Educational Resources Information Center

    Pullmann, Michael D.; Kerbs, Jodi; Koroloff, Nancy; Veach-White, Ernie; Gaylor, Rita; Sieler, Dede

    2006-01-01

    The rate of youth with mental health needs is disproportionately high in juvenile justice. Wraparound planning involves families and providers in coordinating juvenile justice, mental health, and other services and supports. This study compares data from two groups of juvenile offenders with mental health problems: 106 youth in a juvenile justice…

  6. Challenging the Myths: 1999 National Report Series. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Snyder, Howard N.; Sickmund, Melissa

    This bulletin, extracted from "Juvenile Offenders and Victims: 1999 National Report," examines juvenile crime statistics, demonstrating that the predictions in the early 1990s of the emergence of juvenile superpredators (juveniles for whom violence is a way of life) is not supported by current data. Research indicates that levels of predatory…

  7. Profile of Incarcerated Juveniles: Comparison of Male and Female Offenders

    ERIC Educational Resources Information Center

    Martin, Don; Martin, Magy; Dell, Rex; Davis, Candice; Guerrieri, Karen

    2008-01-01

    Effective methods of identifying potential juvenile offenders are critical when developing prevention programs within both state and national juvenile justice systems. The characteristics of juvenile offenders in a large juvenile justice system are examined in this study. Participants live in a Midwestern city with a high rate of crime as…

  8. The NG2 Protein Is Not Required for Glutamatergic Neuron-NG2 Cell Synaptic Signaling.

    PubMed

    Passlick, Stefan; Trotter, Jacqueline; Seifert, Gerald; Steinhäuser, Christian; Jabs, Ronald

    2016-01-01

    NG2 glial cells (as from now NG2 cells) are unique in receiving synaptic input from neurons. However, the components regulating formation and maintenance of these neuron-glia synapses remain elusive. The transmembrane protein NG2 has been considered a potential mediator of synapse formation and alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) clustering, because it contains 2 extracellular Laminin G/Neurexin/Sex Hormone-Binding Globulin domains, which in neurons are crucial for formation of transsynaptic neuroligin-neurexin complexes. NG2 is connected via Glutamate Receptor-Interacting Protein with GluA2/3-containing AMPARs, thereby possibly mediating receptor clustering in glial postsynaptic density. To elucidate the role of NG2 in neuron-glia communication, we investigated glutamatergic synaptic transmission in juvenile and aged hippocampal NG2 cells of heterozygous and homozygous NG2 knockout mice. Neuron-NG2 cell synapses readily formed in the absence of NG2. Short-term plasticity, synaptic connectivity, postsynaptic AMPAR current kinetics, and density were not affected by NG2 deletion. During development, an NG2-independent acceleration of AMPAR current kinetics and decreased synaptic connectivity were observed. Our results indicate that the lack of NG2 does not interfere with genesis and basic properties of neuron-glia synapses. In addition, we demonstrate frequent expression of neuroligins 1-3 in juvenile and aged NG2 cells, suggesting a role of these molecules in synapse formation between NG2 glia and neurons.

  9. Dendritic spine density of prefrontal layer 6 pyramidal neurons in relation to apical dendrite sculpting by nicotinic acetylcholine receptors

    PubMed Central

    Kang, Lily; Tian, Michael K.; Bailey, Craig D. C.; Lambe, Evelyn K.

    2015-01-01

    Prefrontal layer 6 (L6) pyramidal neurons play an important role in the adult control of attention, facilitated by their strong activation by nicotinic acetylcholine receptors. These neurons in mouse association cortex are distinctive morphologically when compared to L6 neurons in primary cortical regions. Roughly equal proportions of the prefrontal L6 neurons have apical dendrites that are “long” (reaching to the pial surface) vs. “short” (terminating in the deep layers, as in primary cortical regions). This distinct prefrontal morphological pattern is established in the post-juvenile period and appears dependent on nicotinic receptors. Here, we examine dendritic spine densities in these two subgroups of prefrontal L6 pyramidal neurons under control conditions as well as after perturbation of nicotinic acetylcholine receptors. In control mice, the long neurons have significantly greater apical and basal dendritic spine density compared to the short neurons. Furthermore, manipulations of nicotinic receptors (chrna5 deletion or chronic developmental nicotine exposure) have distinct effects on these two subgroups of L6 neurons: apical spine density is significantly reduced in long neurons, and basal spine density is significantly increased in short neurons. These changes appear dependent on the α5 nicotinic subunit encoded by chrna5. Overall, the two subgroups of prefrontal L6 neurons appear positioned to integrate information either across cortex (long neurons) or within the deep layers (short neurons), and nicotinic perturbations differently alter spine density within each subgroup. PMID:26500498

  10. [Physiotherapy for juvenile idiopathic arthritis].

    PubMed

    Spamer, M; Georgi, M; Häfner, R; Händel, H; König, M; Haas, J-P

    2012-07-01

    Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.

  11. Law & psychiatry: punishing juveniles who kill.

    PubMed

    Appelbaum, Paul S

    2012-10-01

    Punishment of juvenile murderers forces policy makers to weigh the developmental immaturity of adolescents against the heinousness of their crimes. The U.S. Supreme Court has progressively limited the severity of punishments that can be imposed on juveniles, holding that their impulsivity, susceptibility to peer pressure, and more fluid character render them less culpable for their actions. Having eliminated the death penalty as a punishment, the Court recently struck down mandatory life sentences without prospect of parole. The decision is interesting for its emphasis on rehabilitation, opening the door to further restrictions on punitive sentences for juveniles-and perhaps for adults too.

  12. Law & psychiatry: punishing juveniles who kill.

    PubMed

    Appelbaum, Paul S

    2012-10-01

    Punishment of juvenile murderers forces policy makers to weigh the developmental immaturity of adolescents against the heinousness of their crimes. The U.S. Supreme Court has progressively limited the severity of punishments that can be imposed on juveniles, holding that their impulsivity, susceptibility to peer pressure, and more fluid character render them less culpable for their actions. Having eliminated the death penalty as a punishment, the Court recently struck down mandatory life sentences without prospect of parole. The decision is interesting for its emphasis on rehabilitation, opening the door to further restrictions on punitive sentences for juveniles-and perhaps for adults too. PMID:23032673

  13. Neuron adhesion and strengthening

    NASA Astrophysics Data System (ADS)

    Rocha, Aracely; Jian, Kuihuan; Ko, Gladys; Liang, Hong

    2010-07-01

    Understanding the neuron/material adhesion is important for neuron stimulation and growth. The current challenges remain in the lack of precision of measuring techniques and understanding the behavior of neuron. Here, we report a fluid shear method to investigate adhesion at the neuron/poly-D-lysine interface. In this study, the adhesion of 12-day-old chick embryo-retina neurons cultured on poly-D-lysine coated glass coverslips was measured via parallel disk rotational flow. The shear stress experienced by the cells increases with the disk radius. There is a critical point along the radius (Rc) where the stress experienced by the neurons equals their adhesion. The measured Rc can be used to calculate the neuron adhesion. Our results demonstrate that neurons adhered to the poly-D-lysine had a strain hardening effect. The adhesive shear stress of the neuron-material increased with applied shear (τa). When the τa reached or exceeded the value of 40 dyn/cm2, the adhesion remained constant at approximately 30 dyn/cm2. The present work allowed us not only to quantify the adhesive strength and force but also to evaluate the value of strain hardening at the neuron/poly-D-lysine interface.

  14. Juvenile western toads, Bufo boreas, avoid chemical cues of snakes fed juvenile, but not larval, conspecifics.

    PubMed

    Belden; Wildy; Hatch; Blaustein

    2000-04-01

    Previous investigations have demonstrated the importance of predator diet in chemically mediated antipredator behaviour. However, there are few data on responses to life-stage-specific predator diets, which could be important for animals like amphibians that undergo metamorphosis and must respond to different suites of predators at different life-history stages. In laboratory choice tests, we investigated the chemically mediated avoidance response of juvenile western toads, Bufo boreas, to four different chemical stimuli: (1) live conspecific juveniles; (2) live earthworms; (3) snakes fed juvenile conspecifics; and (4) snakes fed larval conspecifics (tadpoles). Juvenile toads avoided chemical cues from snakes that had eaten juvenile conspecifics, but did not respond to the other three stimuli, including chemical cues from snakes fed larval conspecifics. In addition, the response to cues from snakes fed juveniles differed significantly from that of snakes fed larvae. To our knowledge, this is the first study to demonstrate the importance of diet in predator avoidance of juvenile anurans and the ability of juvenile toads to distinguish between chemical cues from predators that have consumed larval versus juvenile conspecifics. Copyright 2000 The Association for the Study of Animal Behaviour. PMID:10792942

  15. Conceptualizing juvenile prostitution as child maltreatment: findings from the National Juvenile Prostitution Study.

    PubMed

    Mitchell, Kimberly J; Finkelhor, David; Wolak, Janis

    2010-02-01

    Two studies were conducted to identify the incidence (Study 1) and characteristics (Study 2) of juvenile prostitution cases known to law enforcement agencies in the United States. Study 1 revealed a national estimate of 1,450 arrests or detentions (95% confidence interval [CI]: 1,287-1,614) in cases involving juvenile prostitution during a 1-year period. In Study 2, exploratory data were collected from a subsample of 138 cases from police records in 2005. The cases are broadly categorized into three main types: (a) third-party exploiters, (b) solo prostitution, and (c) conventional child sexual abuse (CSA) with payment. Cases were classified into three initial categories based on police orientation toward the juvenile: (a) juveniles as victims (53%), (b) juveniles as delinquents (31%), and (c) juvenile as both victims and delinquents (16%). When examining the status of the juveniles by case type, the authors found that all the juveniles in CSA with payment cases were treated as victims, 66% in third-party exploiters cases, and 11% in solo cases. Findings indicate law enforcement responses to juvenile prostitution are influential in determining whether such youth are viewed as victims of commercial sexual exploitation or as delinquents.

  16. The paediatric rheumatologist and orphan disease – a story without happy ending

    PubMed Central

    Roszkiewicz, Justyna; Biernacka-Zielińska, Małgorzata

    2016-01-01

    Orphan diseases are not a common challenge in the everyday practice of the rheumatologist. Despite their extremely rare occurrence one of the patients under our care developed one of them – neuronal ceroid lipofuscinosis, the most frequent neurodegenerative disease observed in the paediatric population. We report a case of 2-year-old girl diagnosed with oligoarticular form of juvenile idiopathic arthritis treated in our Department with steroids and methotrexate and staying in the stage of disease remission. During routine checkups at Outpatient Clinic we observed progressive deterioration of girls neurological condition resulting in ataxia, gait disturbances with no rheumatological cause behind and speech impairment. The appearance of the symptoms was accompanied by frequent episodes of epileptic seizures, with little clinical improvement on combined antiepileptic treatment. Magnetic resonance imaging that we performed showed a picture highly suggestive of neuronal ceroid lipofuscinosis – atrophy of the patients cerebrum and cerebellum. Genetic testing conducted resulted in the diagnosis of late infantile neuronal ceroid lipofuscinosis (LINCL). PMID:27504025

  17. CO2-inhibited neurons in the medullary raphé are GABAergic

    PubMed Central

    Iceman, Kimberly E.; Corcoran, Andrea E.; Taylor, Barbara E.; Harris, Michael B.

    2014-01-01

    Previous studies have reported subsets of medullary raphé neurons that are either stimulated or inhibited by CO2/pH in vitro, in situ, and in vivo. We tested the hypothesis that medullary raphé CO2-inhibited neurons are GABAergic. Extracellular recordings in unanesthetized juvenile in situ rat preparations showed reversible hypercapnia-induced suppression of 19% (63/323) of medullary raphé neurons, and this suppression persisted after antagonism of NMDA, AMPA/kainate, and GABAA receptors. We stained a subset of CO2-inhibited cells and found that most (11/12) had glutamic acid decarboxylase 67 immunoreactivity (GAD67-ir). These data indicate that the majority of acidosis-inhibited medullary raphé neurons are GABAergic, and that their chemosensitivity is independent of major fast synaptic inputs. Thus, CO2-sensitive GABAergic neurons may play a role in central CO2/pH chemoreception. PMID:25087734

  18. CO2-inhibited neurons in the medullary raphé are GABAergic.

    PubMed

    Iceman, Kimberly E; Corcoran, Andrea E; Taylor, Barbara E; Harris, Michael B

    2014-11-01

    Previous studies have reported subsets of medullary raphé neurons that are either stimulated or inhibited by CO2/pH in vitro, in situ, and in vivo. We tested the hypothesis that medullary raphé CO2-inhibited neurons are GABAergic. Extracellular recordings in unanesthetized juvenile in situ rat preparations showed reversible hypercapnia-induced suppression of 19% (63/323) of medullary raphé neurons, and this suppression persisted after antagonism of NMDA, AMPA/kainate, and GABAA receptors. We stained a subset of CO2-inhibited cells and found that most (11/12) had glutamic acid decarboxylase 67 immunoreactivity (GAD67-ir). These data indicate that the majority of acidosis-inhibited medullary raphé neurons are GABAergic, and that their chemosensitivity is independent of major fast synaptic inputs. Thus, CO2-sensitive GABAergic neurons may play a role in central CO2/pH chemoreception.

  19. Genetics Home Reference: juvenile primary osteoporosis

    MedlinePlus

    ... caused by a shortage of calcium and other minerals in bones (decreased bone mineral density), which makes the bones brittle and prone ... protein is involved in the regulation of bone mineral density. LRP5 gene mutations that cause juvenile primary ...

  20. Group sexual offending by juvenile females.

    PubMed

    Wijkman, Miriam; Weerman, Frank; Bijleveld, Catrien; Hendriks, Jan

    2015-06-01

    This study examined all group sexual offending cases in the Netherlands between 1995 and 2009 (n = 26) in which at least one juvenile female offender (n = 35) had been adjudicated. Information from court files showed that the majority of juvenile female group sexual offenders have (inter)personal problems and (sexual) abuse experiences. The aims of the offender groups in committing the offense could be categorized in three themes: harassing the victim, sexual gratification, and taking revenge. The reasons why juvenile female offenders participated in a group could be categorized into group dynamics versus instrumental reasons. The findings are contrasted with findings on juvenile male group sexual offenders. Implications of the findings for research and treatment are discussed. PMID:25504258

  1. Defective neutrophil chemotaxis in juvenile periodontitis.

    PubMed Central

    Clark, R A; Page, R C; Wilde, G

    1977-01-01

    Neutrophil chemotaxis was evaluated in nine patients with juvenile periodontitis, with normal subjects and patients with the adult form of periodontitis as controls. Defective chemotactic responses were observed in neutrophils from seven of nine juvenile patients, and a reduced level of complement-derived chemotactic activity was demonstrated in serum from four patients. These determinations were normal in all the patients with adult periodontitis. Serum from five of the juvenile patients contained a heat-stable, non-dialyzable factor that markedly inhibited the chemotaxis of normal neutrophils. Thus the characteristic tissue destruction seen in juvenile periodontitis may be, at least in part, a consequence of a failure of host defense mechanisms. PMID:591063

  2. Screening Incarcerated Juveniles Using the MAYSI-2.

    PubMed

    Gilbert, Amy L; Grande, Todd L; Hallman, Janelle; Underwood, Lee A

    2015-01-01

    The high prevalence of mental health disorders among incarcerated juveniles is a matter of national and global concern. Juvenile justice personnel need accurate screening measures that identify youth requiring immediate mental health services. The purpose of this study was threefold: (a) to examine the utility of the Massachusetts Youth Screening Instrument, Version 2 (MAYSI-2) in identifying juveniles with mental health concerns in a large sample of juveniles (N = 4,009), (b) to provide data regarding rates of identified mental health needs in incarcerated youth, and (c) to provide descriptive comparisons to other studies using the MAYSI-2. Mean scores of subscales were compared with the MAYSI-2 normative samples and other recent studies. Results indicated that this population has a high occurrence of mental health symptoms and there is high variability in the severity of the symptoms. In addition, a multivariate analysis of variance test found significant differences in mental health problems across ethnic groups.

  3. Motor Neurons that Multitask

    PubMed Central

    Goulding, Martyn

    2013-01-01

    Animals use a form of sensory feedback termed proprioception to monitor their body position and modify the motor programs that control movement. In this issue of Neuron, Wen et al. (2012) provide evidence that a subset of motor neurons function as proprioceptors in C. elegans, where B-type motor neurons sense body curvature to control the bending movements that drive forward locomotion. PMID:23177952

  4. Mesmerising mirror neurons.

    PubMed

    Heyes, Cecilia

    2010-06-01

    Mirror neurons have been hailed as the key to understanding social cognition. I argue that three currents of thought-relating to evolution, atomism and telepathy-have magnified the perceived importance of mirror neurons. When they are understood to be a product of associative learning, rather than an adaptation for social cognition, mirror neurons are no longer mesmerising, but they continue to raise important questions about both the psychology of science and the neural bases of social cognition.

  5. Role of radiation therapy for 'juvenile' angiofibroma.

    PubMed

    Gudea, F; Vega, M; Canals, E; Montserrat, J M; Valdano, J

    1990-09-01

    Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign neoplasm which occurs primarily in male adolescents and is characterized by aggressive local growth. The controversy concerning appropriate treatment for patients with juvenile angiofibroma persists. Radiation therapy and surgical resection have both been reported to be effective to control a high proportion of these tumours. The case reported here demonstrates a locally advanced JNA controlled by radiation therapy.

  6. Genetics Home Reference: juvenile myoclonic epilepsy

    MedlinePlus

    ... the cell that inhibits signaling between nerve cells (neurons) and prevents the brain from being overloaded with ... receptors available. As a result, the signaling between neurons is not controlled, which can lead to overstimulation ...

  7. Genetics Home Reference: juvenile primary lateral sclerosis

    MedlinePlus

    ... this disorder are caused by damage to motor neurons , which are specialized nerve cells in the brain ... protein called alsin. Alsin is abundant in motor neurons , but its function is not fully understood. Mutations ...

  8. In vivo physiological recording from the lateral line of juvenile zebrafish

    PubMed Central

    Olt, Jennifer; Allen, Claire E.

    2016-01-01

    Key points Zebrafish provide a unique opportunity to investigate in vivo sensory transduction in mature hair cells.We have developed a method for studying the biophysical properties of mature hair cells from the lateral line of juvenile zebrafish.The method involves application of the anaesthetic benzocaine and intubation to maintain ventilation and oxygenation through the gills.The same approach could be used for in vivo functional studies in other sensory and non‐sensory systems from juvenile and adult zebrafish. Abstract Hair cells are sensory receptors responsible for transducing auditory and vestibular information into electrical signals, which are then transmitted with remarkable precision to afferent neurons. The zebrafish lateral line is emerging as an excellent in vivo model for genetic and physiological analysis of hair cells and neurons. However, research has been limited to larval stages because zebrafish become protected from the time of independent feeding under European law (from 5.2 days post‐fertilization (dpf) at 28.5°C). In larval zebrafish, the functional properties of most of hair cells, as well as those of other excitable cells, are still immature. We have developed an experimental protocol to record electrophysiological properties from hair cells of the lateral line in juvenile zebrafish. We found that the anaesthetic benzocaine at 50 mg l−1 was an effective and safe anaesthetic to use on juvenile zebrafish. Concentrations up to 300 mg l−1 did not affect the electrical properties or synaptic vesicle release of juvenile hair cells, unlike the commonly used anaesthetic MS‐222, which reduces the size of basolateral membrane K+ currents. Additionally, we implemented a method to maintain gill movement, and as such respiration and blood oxygenation, via the intubation of > 21 dpf zebrafish. The combination of benzocaine and intubation provides an experimental platform to investigate the physiology of mature hair cells from live

  9. Assessing the Parents of Juvenile Offenders: A Preliminary Validation Study of the Juvenile Offender Parent Questionnaire

    ERIC Educational Resources Information Center

    Rose, Clark C.; Glaser, Brian A.; Calhoun, Georgia B.; Bates, Jeffrey M.

    2004-01-01

    The present study is a preliminary investigation into the development of a parent self-report instrument, the Juvenile Offender Parent Questionnaire (JOPQ). A large pool of items was rationally derived from a model of parent competency and then administered to 243 parents of children who were making appearances in juvenile court. Exploratory…

  10. Collaboration and Leadership in Juvenile Detention Reform. Pathways to Juvenile Detention Reform 2.

    ERIC Educational Resources Information Center

    Feely, Kathleen

    This report addresses governance and leadership prerequisites for implementing specific strategies essential to juvenile detention reform. Chapter 1, "Why Are Collaboration and Leadership Essential to Detention Reform?" discusses principles of collaboration and leadership that emerged from the Juvenile Detention Alternatives Initiative (JDAI).…

  11. An Empirical Evaluation of Juvenile Awareness Programs in the United States: Can Juveniles Be "Scared Straight"?

    ERIC Educational Resources Information Center

    Klenowski, Paul M.; Bell, Keith J.; Dodson, Kimberly D.

    2010-01-01

    Juvenile awareness programs like Scared Straight became popular crime prevention strategies during the 1970s. Juvenile offenders and at-risk youth who participate in these programs are taken to prisons where inmates use confrontational methods to recount stories about violence, sex, and abuse perpetrated by fellow inmates while living a life…

  12. Characteristics of Crimes against Juveniles. Crimes against Children Series. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Finkelhor, David; Ormrod, Richard

    This Bulletin reviews data from the Federal Bureau of Investigation's 1997 National Incident-Based Reporting System (NIBRS) data file that pertain to juvenile victims, revealing that while juveniles made up 26% of the population of the 12 states participating in NIBRS in 1997, they accounted for only 12% of the reported crime victims. At the same…

  13. Tracking Juvenile Recidivists: Three Options for Creating Statewide, Longitudinal Records of Juvenile Offenders.

    ERIC Educational Resources Information Center

    Rooney, Teresa L.

    This document describes three options for a statewide statistical system for tracking recidivism of juvenile delinquents placed outside their homes in treatment programs. The information is intended for use by the state in allocating resources. The options described involve potential use of juvenile court records, placement data, and/or…

  14. Rhythmic Cortical Neurons Increase their Oscillations and Sculpt Basal Ganglia Signaling During Motor Learning

    PubMed Central

    Day, Nancy F.; Nick, Teresa A.

    2014-01-01

    The function and modulation of neural circuits underlying motor skill may involve rhythmic oscillations (Feller, 1999; Marder and Goaillard, 2006; Churchland et al., 2012). In the proposed pattern generator for birdsong, the cortical nucleus HVC, the frequency and power of oscillatory bursting during singing increases with development (Crandall et al., 2007; Day et al., 2009). We examined the maturation of cellular activity patterns that underlie these changes. Single unit ensemble recording combined with antidromic identification (Day et al., 2011) was used to study network development in anesthetized zebra finches. Autocovariance quantified oscillations within single units. A subset of neurons oscillated in the theta/alpha/mu/beta range (8–20 Hz), with greater power in adults compared to juveniles. Across the network, the normalized oscillatory power in the 8–20 Hz range was greater in adults than juveniles. In addition, the correlated activity between rhythmic neuron pairs increased with development. We next examined the functional impact of the oscillators on the output neurons of HVC. We found that the firing of oscillatory neurons negatively correlated with the activity of cortico-basal ganglia neurons (HVCXs), which project to Area X (the song basal ganglia). If groups of oscillators work together to tonically inhibit and precisely control the spike timing of adult HVCXs with coordinated release from inhibition, then the activity of HVCXs in juveniles should be decreased relative to adults due to uncorrelated, tonic inhibition. Consistent with this hypothesis, HVCXs had lower activity in juveniles. These data reveal network changes that shape cortical-to-basal ganglia signaling during motor learning. PMID:23776169

  15. Juvenile fibromyalgia: Guidance for management.

    PubMed

    Yokota, Shumpei; Kikuchi, Masako; Miyamae, Takako

    2013-08-01

    Juvenile fibromyalgia (JFM) is a disease in which patients complain of acute and chronic severe pain, an overt primary cause for which cannot be found or surmised. Although patients with JFM mainly complain of systemic pain or allodynia in the medical interview and physical examination, the concept of the disease is the total sum of painful illness, chronic fatigue, hypothermia and many other autonomic symptoms and signs. Many issues are interacting including individual traits (personality, temperament, sensitivity, memory of pain; age: early adolescence), individual states (self-esteem, anxiety, developmental level), and external stressors (parent especially mother, school environment). JFM is diagnosed on the combination of disease history, physical examination to determine the 18 tender points and allodynia, pain from gently touching their hair, and negative results of blood tests (inflammatory markers, thyroid function, myogenic enzymes). The goals of treatment are the following: restoration of function and relief of pain. Psychological support is advocated. Although the exact number of patients with JFM is still to be elucidated, it seems to be growing because pediatric rheumatologists in Japan encounter children with a wide variety of musculoskeletal pains. This guideline describes how to diagnose JFM in children and how to treat them appropriately.

  16. Corticospinal mirror neurons.

    PubMed

    Kraskov, A; Philipp, R; Waldert, S; Vigneswaran, G; Quallo, M M; Lemon, R N

    2014-01-01

    Here, we report the properties of neurons with mirror-like characteristics that were identified as pyramidal tract neurons (PTNs) and recorded in the ventral premotor cortex (area F5) and primary motor cortex (M1) of three macaque monkeys. We analysed the neurons' discharge while the monkeys performed active grasp of either food or an object, and also while they observed an experimenter carrying out a similar range of grasps. A considerable proportion of tested PTNs showed clear mirror-like properties (52% F5 and 58% M1). Some PTNs exhibited 'classical' mirror neuron properties, increasing activity for both execution and observation, while others decreased their discharge during observation ('suppression mirror-neurons'). These experiments not only demonstrate the existence of PTNs as mirror neurons in M1, but also reveal some interesting differences between M1 and F5 mirror PTNs. Although observation-related changes in the discharge of PTNs must reach the spinal cord and will include some direct projections to motoneurons supplying grasping muscles, there was no EMG activity in these muscles during action observation. We suggest that the mirror neuron system is involved in the withholding of unwanted movement during action observation. Mirror neurons are differentially recruited in the behaviour that switches rapidly between making your own movements and observing those of others.

  17. NEURON and Python.

    PubMed

    Hines, Michael L; Davison, Andrew P; Muller, Eilif

    2009-01-01

    The NEURON simulation program now allows Python to be used, alone or in combination with NEURON's traditional Hoc interpreter. Adding Python to NEURON has the immediate benefit of making available a very extensive suite of analysis tools written for engineering and science. It also catalyzes NEURON software development by offering users a modern programming tool that is recognized for its flexibility and power to create and maintain complex programs. At the same time, nothing is lost because all existing models written in Hoc, including graphical user interface tools, continue to work without change and are also available within the Python context. An example of the benefits of Python availability is the use of the xml module in implementing NEURON's Import3D and CellBuild tools to read MorphML and NeuroML model specifications.

  18. 75 FR 70216 - Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-11-17

    ... JUVENILE JUSTICE AND DELINQUENCY PREVENTION Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention AGENCY: Coordinating Council on Juvenile Justice and Delinquency Prevention. ACTION: Notice of meeting. SUMMARY: The Coordinating Council on Juvenile Justice and Delinquency...

  19. 76 FR 61672 - Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-10-05

    ... JUVENILE JUSTICE AND DELINQUENCY PREVENTION Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention AGENCY: Coordinating Council on Juvenile Justice and Delinquency Prevention. ACTION: Notice of meeting. SUMMARY: The Coordinating Council on Juvenile Justice and Delinquency...

  20. 76 FR 39075 - Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-07-05

    ... JUVENILE JUSTICE AND DELINQUENCY PREVENTION Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention AGENCY: Coordinating Council on Juvenile Justice and Delinquency Prevention. ACTION: Notice of meeting. SUMMARY: The Coordinating Council on Juvenile Justice and Delinquency...

  1. 78 FR 17184 - Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-03-20

    ... JUVENILE JUSTICE AND DELINQUENCY PREVENTION Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention AGENCY: Coordinating Council on Juvenile Justice and Delinquency Prevention. ACTION: Notice of meeting. SUMMARY: The Coordinating Council on Juvenile Justice and Delinquency...

  2. 77 FR 50486 - Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-08-21

    ... JUVENILE JUSTICE AND DELINQUENCY PREVENTION Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention AGENCY: Coordinating Council on Juvenile Justice and Delinquency Prevention. ACTION: Notice of meeting. SUMMARY: The Coordinating Council on Juvenile Justice and Delinquency...

  3. 77 FR 3453 - Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-01-24

    ... JUVENILE JUSTICE AND DELINQUENCY PREVENTION Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention AGENCY: Coordinating Council on Juvenile Justice and Delinquency Prevention. ACTION: Notice of meeting. SUMMARY: The Coordinating Council on Juvenile Justice and Delinquency...

  4. 78 FR 58288 - Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-09-23

    ... JUVENILE JUSTICE AND DELINQUENCY PREVENTION Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention AGENCY: Coordinating Council on Juvenile Justice and Delinquency Prevention. ACTION: Notice of meeting. SUMMARY: The Coordinating Council on Juvenile Justice and Delinquency...

  5. 75 FR 16177 - Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-03-31

    ... Coordinating Council on Juvenile Justice and Delinquency Prevention Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention AGENCY: Coordinating Council on Juvenile Justice and Delinquency Prevention. ACTION: Notice of meeting. SUMMARY: The Coordinating Council on Juvenile Justice...

  6. 77 FR 24687 - Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-04-25

    ... JUVENILE JUSTICE AND DELINQUENCY PREVENTION Meeting of the Coordinating Council on Juvenile Justice and Delinquency Prevention AGENCY: Coordinating Council on Juvenile Justice and Delinquency Prevention. ACTION: Notice of meeting. SUMMARY: The Coordinating Council on Juvenile Justice and Delinquency...

  7. Lateralization of the connections of the ovary to the celiac ganglia in juvenile rats.

    PubMed

    Morán, Carolina; Zarate, Fabiola; Morán, José Luis; Handal, Anabella; Domínguez, Roberto

    2009-05-21

    During the development of the female rat, a maturing process of the factors that regulate the functioning of the ovaries takes place, resulting in different responses according to the age of the animal. Studies show that peripheral innervation is one relevant factor involved.In the present study we analyzed the anatomical relationship between the neurons in the celiac-superior mesenteric ganglia (CSMG), and the right or left ovary in 24 or 28 days old female pre-pubertal rats. The participation of the superior ovarian nerve (SON) in the communication between the CSMG and the ovaries was analyzed in animals with unilateral section of the SON, previous to injecting true blue (TB) into the ovarian bursa. The animals were killed seven days after treatment. TB stained neurons were quantified at the superior mesenteric-celiac ganglia.The number of labeled neurons in the CSMG of rats treated at 28 days of age was significantly higher than those treated on day 24. At age 24 days, injecting TB into the right ovary resulted in neuron stains on both sides of the celiac ganglia; whereas, injecting the left side the stains were exclusively ipsilateral. Such asymmetry was not observed when the rats were treated at age of 28 days.In younger rats, sectioning the left SON resulted in significantly lower number of stained neurons in the left ganglia while sectioning the right SON did not modify the number of stained neurons. When sectioning of the SON was performed to 28 days old rats, no staining was observed.Present results show that the number and connectivity of post-ganglionic neurons of the CSMG connected to the ovary of juvenile female rats change as the animal mature; that the SON plays a role in this communication process as puberty approaches; and that this maturing process is different for the right or the left ovary.

  8. Lateralization of the connections of the ovary to the celiac ganglia in juvenile rats

    PubMed Central

    Morán, Carolina; Zarate, Fabiola; Morán, José Luis; Handal, Anabella; Domínguez, Roberto

    2009-01-01

    During the development of the female rat, a maturing process of the factors that regulate the functioning of the ovaries takes place, resulting in different responses according to the age of the animal. Studies show that peripheral innervation is one relevant factor involved. In the present study we analyzed the anatomical relationship between the neurons in the celiac-superior mesenteric ganglia (CSMG), and the right or left ovary in 24 or 28 days old female pre-pubertal rats. The participation of the superior ovarian nerve (SON) in the communication between the CSMG and the ovaries was analyzed in animals with unilateral section of the SON, previous to injecting true blue (TB) into the ovarian bursa. The animals were killed seven days after treatment. TB stained neurons were quantified at the superior mesenteric-celiac ganglia. The number of labeled neurons in the CSMG of rats treated at 28 days of age was significantly higher than those treated on day 24. At age 24 days, injecting TB into the right ovary resulted in neuron stains on both sides of the celiac ganglia; whereas, injecting the left side the stains were exclusively ipsilateral. Such asymmetry was not observed when the rats were treated at age of 28 days. In younger rats, sectioning the left SON resulted in significantly lower number of stained neurons in the left ganglia while sectioning the right SON did not modify the number of stained neurons. When sectioning of the SON was performed to 28 days old rats, no staining was observed. Present results show that the number and connectivity of post-ganglionic neurons of the CSMG connected to the ovary of juvenile female rats change as the animal mature; that the SON plays a role in this communication process as puberty approaches; and that this maturing process is different for the right or the left ovary. PMID:19460167

  9. De novo FUS P525L mutation in Juvenile amyotrophic lateral sclerosis with dysphonia and diplopia.

    PubMed

    Leblond, Claire S; Webber, Alina; Gan-Or, Ziv; Moore, Fraser; Dagher, Alain; Dion, Patrick A; Rouleau, Guy A

    2016-04-01

    Juvenile amyotrophic lateral sclerosis (jALS) is characterized by progressive upper and lower motor neuron degeneration leading to facial muscle spasticity, spastic dysarthria, and spastic gait with an early onset (before 25 years old). Unlike adult-onset amyotrophic lateral sclerosis (ALS), patients with jALS tend to have slower progression of motor neuron disease and prolonged survival to a normal life expectancy. Mutations in FUS gene have been reported in jALS,(1) including p.P525L mutation that has been consistently associated with early onset and aggressive presentation.(2) Here, we report a patient carrying p.P525L FUS mutation and experiencing an aggressive course of ALS presenting with dysphonia and diplopia. PMID:27123482

  10. Dendritic branching angles of pyramidal cells across layers of the juvenile rat somatosensory cortex.

    PubMed

    Leguey, Ignacio; Bielza, Concha; Larrañaga, Pedro; Kastanauskaite, Asta; Rojo, Concepción; Benavides-Piccione, Ruth; DeFelipe, Javier

    2016-09-01

    The characterization of the structural design of cortical microcircuits is essential for understanding how they contribute to function in both health and disease. Since pyramidal neurons represent the most abundant neuronal type and their dendritic spines constitute the major postsynaptic elements of cortical excitatory synapses, our understanding of the synaptic organization of the neocortex largely depends on the available knowledge regarding the structure of pyramidal cells. Previous studies have identified several apparently common rules in dendritic geometry. We study the dendritic branching angles of pyramidal cells across layers to further shed light on the principles that determine the geometric shapes of these cells. We find that the dendritic branching angles of pyramidal cells from layers II-VI of the juvenile rat somatosensory cortex suggest common design principles, despite the particular morphological and functional features that are characteristic of pyramidal cells in each cortical layer. J. Comp. Neurol. 524:2567-2576, 2016. © 2016 Wiley Periodicals, Inc.

  11. Sphingomyelin lipidosis (Niemann-Pick disease) in a juvenile raccoon (Procyon lotor).

    PubMed

    Vapniarsky, N; Wenger, D A; Scheenstra, D; Mete, A

    2013-01-01

    A wild caught juvenile male raccoon with neurological disease was humanely destroyed due to poor prognosis. Necropsy examination revealed hepatomegaly, splenomegaly and multicentric lymphadenomegaly with diffuse hepatic pallor and pulmonary consolidation with pinpoint pale subpleural foci. Microscopically, there was marked pale cytoplasmic swelling of the central and peripheral neurons as well as the glial cells in the brain, accompanied by multiorgan infiltration by abundant foamy macrophages. Ultrastructural investigation revealed accumulation of concentrically arranged lamellar material within lysosomes of the affected neurons, macrophages and endothelial cells. Biochemical enzymatic analysis detected sphingomyelinase deficiency and lysosomal storage disease consistent with sphingomyelin lipidosis (Niemann-Pick disease [NPD]) was diagnosed. This is the first report of NPD in a raccoon.

  12. Dendritic branching angles of pyramidal cells across layers of the juvenile rat somatosensory cortex.

    PubMed

    Leguey, Ignacio; Bielza, Concha; Larrañaga, Pedro; Kastanauskaite, Asta; Rojo, Concepción; Benavides-Piccione, Ruth; DeFelipe, Javier

    2016-09-01

    The characterization of the structural design of cortical microcircuits is essential for understanding how they contribute to function in both health and disease. Since pyramidal neurons represent the most abundant neuronal type and their dendritic spines constitute the major postsynaptic elements of cortical excitatory synapses, our understanding of the synaptic organization of the neocortex largely depends on the available knowledge regarding the structure of pyramidal cells. Previous studies have identified several apparently common rules in dendritic geometry. We study the dendritic branching angles of pyramidal cells across layers to further shed light on the principles that determine the geometric shapes of these cells. We find that the dendritic branching angles of pyramidal cells from layers II-VI of the juvenile rat somatosensory cortex suggest common design principles, despite the particular morphological and functional features that are characteristic of pyramidal cells in each cortical layer. J. Comp. Neurol. 524:2567-2576, 2016. © 2016 Wiley Periodicals, Inc. PMID:26850576

  13. Transporting mitochondria in neurons

    PubMed Central

    Course, Meredith M.; Wang, Xinnan

    2016-01-01

    Neurons demand vast and vacillating supplies of energy. As the key contributors of this energy, as well as primary pools of calcium and signaling molecules, mitochondria must be where the neuron needs them, when the neuron needs them. The unique architecture and length of neurons, however, make them a complex system for mitochondria to navigate. To add to this difficulty, mitochondria are synthesized mainly in the soma, but must be transported as far as the distant terminals of the neuron. Similarly, damaged mitochondria—which can cause oxidative stress to the neuron—must fuse with healthy mitochondria to repair the damage, return all the way back to the soma for disposal, or be eliminated at the terminals. Increasing evidence suggests that the improper distribution of mitochondria in neurons can lead to neurodegenerative and neuropsychiatric disorders. Here, we will discuss the machinery and regulatory systems used to properly distribute mitochondria in neurons, and how this knowledge has been leveraged to better understand neurological dysfunction. PMID:27508065

  14. How microglia kill neurons.

    PubMed

    Brown, Guy C; Vilalta, Anna

    2015-12-01

    Microglia are resident brain macrophages that become inflammatory activated in most brain pathologies. Microglia normally protect neurons, but may accidentally kill neurons when attempting to limit infections or damage, and this may be more common with degenerative disease as there was no significant selection pressure on the aged brain in the past. A number of mechanisms by which activated microglia kill neurons have been identified, including: (i) stimulation of the phagocyte NADPH oxidase (PHOX) to produce superoxide and derivative oxidants, (ii) expression of inducible nitric oxide synthase (iNOS) producing NO and derivative oxidants, (iii) release of glutamate and glutaminase, (iv) release of TNFα, (v) release of cathepsin B, (vi) phagocytosis of stressed neurons, and (vii) decreased release of nutritive BDNF and IGF-1. PHOX stimulation contributes to microglial activation, but is not directly neurotoxic unless NO is present. NO is normally neuroprotective, but can react with superoxide to produce neurotoxic peroxynitrite, or in the presence of hypoxia inhibit mitochondrial respiration. Glutamate can be released by glia or neurons, but is neurotoxic only if the neurons are depolarised, for example as a result of mitochondrial inhibition. TNFα is normally neuroprotective, but can become toxic if caspase-8 or NF-κB activation are inhibited. If the above mechanisms do not kill neurons, they may still stress the neurons sufficiently to make them susceptible to phagocytosis by activated microglia. We review here whether microglial killing of neurons is an artefact, makes evolutionary sense or contributes in common neuropathologies and by what mechanisms. This article is part of a Special Issue entitled SI: Neuroprotection.

  15. Repetitive mild traumatic brain injury induces ventriculomegaly and cortical thinning in juvenile rats.

    PubMed

    Goddeyne, Corey; Nichols, Joshua; Wu, Chen; Anderson, Trent

    2015-05-01

    Traumatic brain injury (TBI) most frequently occurs in pediatric patients and remains a leading cause of childhood death and disability. Mild TBI (mTBI) accounts for nearly 75% of all TBI cases, yet its neuropathophysiology is still poorly understood. While even a single mTBI injury can lead to persistent deficits, repeat injuries increase the severity and duration of both acute symptoms and long-term deficits. In this study, to model pediatric repetitive mTBI (rmTBI) we subjected unrestrained juvenile animals (postnatal day 20) to repeat weight-drop impacts. Animals were anesthetized and subjected to sham injury or rmTBI once per day for 5 days. Magnetic resonance imaging (MRI) performed 14 days after injury revealed marked cortical atrophy and ventriculomegaly in rmTBI animals. Specifically, beneath the impact zone the thickness of the cortex was reduced by up to 46% and the area of the ventricles increased by up to 970%. Immunostaining with the neuron-specific marker NeuN revealed an overall loss of neurons within the motor cortex but no change in neuronal density. Examination of intrinsic and synaptic properties of layer II/III pyramidal neurons revealed no significant difference between sham-injured and rmTBI animals at rest or under convulsant challenge with the potassium channel blocker 4-aminopyridine. Overall, our findings indicate that the neuropathological changes reported after pediatric rmTBI can be effectively modeled by repeat weight drop in juvenile animals. Developing a better understanding of how rmTBI alters the pediatric brain may help improve patient care and direct "return to game" decision making in adolescents.

  16. Repetitive mild traumatic brain injury induces ventriculomegaly and cortical thinning in juvenile rats

    PubMed Central

    Goddeyne, Corey; Nichols, Joshua; Wu, Chen

    2015-01-01

    Traumatic brain injury (TBI) most frequently occurs in pediatric patients and remains a leading cause of childhood death and disability. Mild TBI (mTBI) accounts for nearly 75% of all TBI cases, yet its neuropathophysiology is still poorly understood. While even a single mTBI injury can lead to persistent deficits, repeat injuries increase the severity and duration of both acute symptoms and long-term deficits. In this study, to model pediatric repetitive mTBI (rmTBI) we subjected unrestrained juvenile animals (postnatal day 20) to repeat weight-drop impacts. Animals were anesthetized and subjected to sham injury or rmTBI once per day for 5 days. Magnetic resonance imaging (MRI) performed 14 days after injury revealed marked cortical atrophy and ventriculomegaly in rmTBI animals. Specifically, beneath the impact zone the thickness of the cortex was reduced by up to 46% and the area of the ventricles increased by up to 970%. Immunostaining with the neuron-specific marker NeuN revealed an overall loss of neurons within the motor cortex but no change in neuronal density. Examination of intrinsic and synaptic properties of layer II/III pyramidal neurons revealed no significant difference between sham-injured and rmTBI animals at rest or under convulsant challenge with the potassium channel blocker 4-aminopyridine. Overall, our findings indicate that the neuropathological changes reported after pediatric rmTBI can be effectively modeled by repeat weight drop in juvenile animals. Developing a better understanding of how rmTBI alters the pediatric brain may help improve patient care and direct “return to game” decision making in adolescents. PMID:25695652

  17. Psychiatric and Medical Health Care Policies in Juvenile Detention Facilities

    ERIC Educational Resources Information Center

    Pajer, Kathleen A.; Kelleher, Kelly; Gupta, Ravindra A.; Rolls, Jennifer; Gardner, William

    2007-01-01

    A study aims to examine the existing health care policies in U.S. juvenile detention centres. The results conclude that juvenile detention facilities have many shortfalls in providing care for adolescents, particularly mental health care.

  18. Data Integration in the Evaluation of Juvenile Justice Education.

    ERIC Educational Resources Information Center

    Winokur, Kristin Parsons; Li, Spencer; McEntire, Ranee

    2002-01-01

    Discusses the evaluation of juvenile justice education through Florida's Juvenile Justice Educational Enhancement Program as it involves the integration of multiple data sources. Examines the methodological, political, and bureaucratic obstacles encountered in the development of this evaluation system. (SLD)

  19. SEASONAL VARIATION IN PLASMA SEX STEROID CONCENTRATION IN JUVENILE ALLIGATORS

    EPA Science Inventory

    Seasonal variation in plasma sex steroid concentrations is common in mature vertebrates, and is occasionally seen in juvenile animals. In this study, we examine the seasonal pattern of sex hormone concentration in juvenile American alligators (Alligator mississippiensis) and make...

  20. Corporal and capital punishment of juveniles.

    PubMed

    Frazier, H C

    1990-01-01

    There is a previously unobserved connection between corporal punishment of public school children and capital punishment of juveniles. Both are barometers of acceptable levels of violent punishment and their elimination is a hallmark of a maturing and decent society. Within a majority of the eighteen states where school authorities most frequently strike children are housed 25 of the nation's 28 juvenile death row inmates. On average, the homicide rates of these jurisdictions are two and a half times greater than those that have abolished both state-sanctioned corporal and capital punishment or limit death sentences to those age eighteen and older at the time of their crime(s). Most of the eighteen state abolitions of corporal punishment occurred in the 1980's. The US Supreme Court has ruled both corporal and capital punishment of juveniles constitutional. Additional state legislative abolition of both is anticipated in the 1990s.

  1. Immunization Coverage Among Juvenile Justice Detainees.

    PubMed

    Gaskin, Gregory L; Glanz, Jason M; Binswanger, Ingrid A; Anoshiravani, Arash

    2015-07-01

    This study sought to (1) quantify the baseline immunization coverage of adolescents entering the juvenile justice system and (2) assess the effect of detention-based care on immunization coverage in youth. A cross-sectional retrospective chart review was performed of 279 adolescents detained at a large juvenile detention facility. Only 3% of adolescents had received all study immunizations prior to detention. Before detention, immunization coverage was significantly lower than that for the general adolescent population for all vaccines except the first doses of hepatitis A and varicella-zoster virus vaccines. Subsequent to detention, most individual immunization coverage levels increased and were significantly higher than in the general adolescent population. The routine administration of immunizations in the juvenile justice setting can help detained youth achieve levels of immunization coverage similar to their nondetained peers.

  2. Juvenile Fibromyalgia: A Multidisciplinary Approach to Treatment.

    PubMed

    Tesher, Melissa S

    2015-06-01

    A 14-year-old boy presented with months of severe widespread musculoskeletal pain. He was profoundly fatigued and unable to attend school. Laboratory evaluation, including complete blood count, comprehensive metabolic panel, inflammatory markers, and thyroid function, was unrevealing. Physical examination was also normal except for multiple tender points. The patient was diagnosed with juvenile primary fibromyalgia syndrome and referred for multidisciplinary treatment including physical therapy, exercise, and counseling, and his daily functioning gradually improves. Juvenile fibromyalgia is a complex syndrome that often severely limits patients' activities and can impede normal adolescent development. Effective treatment requires an understanding of the biologic, psychologic, and social factors contributing to the perpetuation of chronic pain. The author reviews the diagnostic criteria, pathophysiology, and treatment of juvenile fibromyalgia. Medications, particularly antidepressants and anticonvulsants, can be useful adjuncts to therapy. However, multimodal pain management including intensive physical therapy, exercise, counseling, and sleep hygiene is most effective in treating fibromyalgia. PMID:26114368

  3. Corporal and capital punishment of juveniles.

    PubMed

    Frazier, H C

    1990-01-01

    There is a previously unobserved connection between corporal punishment of public school children and capital punishment of juveniles. Both are barometers of acceptable levels of violent punishment and their elimination is a hallmark of a maturing and decent society. Within a majority of the eighteen states where school authorities most frequently strike children are housed 25 of the nation's 28 juvenile death row inmates. On average, the homicide rates of these jurisdictions are two and a half times greater than those that have abolished both state-sanctioned corporal and capital punishment or limit death sentences to those age eighteen and older at the time of their crime(s). Most of the eighteen state abolitions of corporal punishment occurred in the 1980's. The US Supreme Court has ruled both corporal and capital punishment of juveniles constitutional. Additional state legislative abolition of both is anticipated in the 1990s. PMID:2122167

  4. Treatment with a clinically-relevant dose of methylphenidate alters NMDA receptor composition and synaptic plasticity in the juvenile rat prefrontal cortex.

    PubMed

    Urban, Kimberly R; Li, Yan-Chun; Gao, Wen-Jun

    2013-03-01

    Methylphenidate (Ritalin, MPH) is the most commonly prescribed psychoactive drug for children. Used to treat attention-deficit/hyperactivity disorder (ADHD) and for cognitive enhancement in healthy individuals, its cellular mechanisms of action and potential long-term effects are poorly understood. We recently reported that a clinically relevant (1 mg/kg i.p., single injection) dose of MPH significantly decreased neuronal excitability in the juvenile rat prefrontal cortical neurons. Here we further explore the actions of acute treatment with MPH on the level of NMDA receptor subunits and NMDA receptor-mediated short- and long-term synaptic plasticity in the juvenile rat prefrontal cortical neurons. We found that a single dose of MPH treatment (1 mg/kg, intraperitoneal) significantly decreased the surface and total protein levels of NMDA receptor subunits NR1 and NR2B, but not NR2A, in the juvenile prefrontal cortex. In addition, the amplitude, decay time and charge transfer of NMDA receptor-mediated EPSCs were significantly decreased whereas the amplitude and short-term depression of AMPA receptor-mediated EPSCs were significantly increased in the prefrontal neurons. Furthermore, MPH treatment also significantly increased the probability and magnitude of LTP induction, but had only a small effect on LTD induction in juvenile rat prefrontal cortical neurons. Our data thus present a novel mechanism of action of MPH, i.e., changes in glutamatergic receptor-mediated synaptic plasticity following early-life treatment. Furthermore, since a single dosage resulted in significant changes in NMDA receptors, off-label usage by healthy individuals, especially children and adolescents, may result in altered potential for plastic learning.

  5. EphA4 signaling in juveniles establishes topographic specificity of structural plasticity in the hippocampus.

    PubMed

    Galimberti, Ivan; Bednarek, Ewa; Donato, Flavio; Caroni, Pico

    2010-03-11

    The formation and loss of synapses is involved in learning and memory. Distinct subpopulations of permanent and plastic synapses coexist in the adult brain, but the principles and mechanisms underlying the establishment of these distinctions remain unclear. Here we show that in the hippocampus, terminal arborizations (TAs) with high plasticity properties are specified at juvenile stages, and account for most synapse turnover of adult mossy fibers. Out of 9-12 giant terminals along CA3, distinct subpopulations of granule neurons revealed by mouse reporter lines exhibit 0, 1, or >2 TAs. TA specification involves a topographic rule based on cell body position and EphA4 signaling. Upon disruption of EphA4 signaling or PSA-NCAM in juvenile circuits, single-TA mossy fibers establish >2 TAs, suggesting that intra-axonal competition influences plasticity site selection. Therefore, plastic synapse specification in juveniles defines sites of synaptic remodeling in the adult, and hippocampal circuit plasticity follows unexpected topographic principles.

  6. Vocal Experimentation in the Juvenile Songbird Requires a Basal Ganglia Circuit

    PubMed Central

    2005-01-01

    Songbirds learn their songs by trial-and-error experimentation, producing highly variable vocal output as juveniles. By comparing their own sounds to the song of a tutor, young songbirds gradually converge to a stable song that can be a remarkably good copy of the tutor song. Here we show that vocal variability in the learning songbird is induced by a basal-ganglia-related circuit, the output of which projects to the motor pathway via the lateral magnocellular nucleus of the nidopallium (LMAN). We found that pharmacological inactivation of LMAN dramatically reduced acoustic and sequence variability in the songs of juvenile zebra finches, doing so in a rapid and reversible manner. In addition, recordings from LMAN neurons projecting to the motor pathway revealed highly variable spiking activity across song renditions, showing that LMAN may act as a source of variability. Lastly, pharmacological blockade of synaptic inputs from LMAN to its target premotor area also reduced song variability. Our results establish that, in the juvenile songbird, the exploratory motor behavior required to learn a complex motor sequence is dependent on a dedicated neural circuit homologous to cortico-basal ganglia circuits in mammals. PMID:15826219

  7. Movies and juvenile delinquency: an overview.

    PubMed

    Snyder, S

    1991-01-01

    Film viewing may affect the juvenile delinquent through the processes known as social learning and instigation. Identification with the movie and its characters by the delinquent viewer is common, and studies have consistently demonstrated that films can affect delinquents, although in some cases the effects are small. Numerous examples of how films may serve as either the initiator or the final common pathway of delinquent acts are presented. However, prosocial aspects of films dealing with delinquency may exert a positive influence on the juvenile delinquent. Treatment implications of these observations are discussed from social learning and other perspectives.

  8. Electrophysiological changes in juvenile diabetics without retinopathy.

    PubMed

    Juen, S; Kieselbach, G F

    1990-03-01

    Several components of the electroretinogram were studied in 31 juvenile diabetics and 15 age-matched normal controls. The diabetic group consisted of 18 patients without retinopathy and 13 with mild background retinopathy. Oscillatory potentials were measured at low-stimulation energies. Significantly reduced amplitudes and component-specific delayed peak implicit times were found in both diabetic groups compared with the data from the controls. Similar results were obtained in the photopic and scotopic electroretinogram. From these findings, we suggest that retinal dysfunction is already present in juvenile diabetics without photographic evidence of retinopathy after a mean duration of diabetes of 7 years. PMID:2310337

  9. [HLA antigens in juvenile rheumatoid arthritis].

    PubMed

    Rumba, I V; Sochnev, A M; Kukaĭne, E M; Burshteĭn, A M; Benevolenskaia, L I

    1990-01-01

    Antigens of I class HLA system (locus A and B) were investigated in 67 patients of Latvian nationality suffering from juvenile rheumatoid arthritis (JRA). Associations of HLA antigens with juvenile rheumatoid arthritis partially coincided with the ones revealed earlier. Typing established an increased incidence of antigen B27 (p less than 0.01) and gaplotype A2, B40 (p less than 0.01). Antigen B15 possessed a protective action with respect to JRA. Interlocus combinations demonstrated a closer association with the disease than a single antigen. The authors also revealed markers of various clinico-anatomical variants of JRA.

  10. Treatment of large juvenile nasopharyngeal angiofibroma.

    PubMed

    Deschler, D G; Kaplan, M J; Boles, R

    1992-03-01

    The management of large juvenile nasopharyngeal angiofibromas with intracranial extension is controversial. We review our experience since 1980 with eighteen patients with juvenile nasopharyngeal angiofibroma. A diagnostic and treatment approach consisting of preoperative magnetic resonance imaging, embolization of feeding branches from the external carotid artery, and attempted complete resection was used in seven patients with intracranial disease since 1987. Serial magnetic resonance images were used for followup. Intracranial disease that was persistent or recurrent and demonstrated subsequent growth was irradiated (35 to 45 cGy). Extracranial tumor recurrences were reexcised. We advocate this approach as a safe and effective alternative to primary irradiation and its sequelae.

  11. Juvenile justice. A role for health professionals.

    PubMed

    Brookman, Monica

    2003-01-01

    The United States is the only nation in the world that continues to execute its youth. The use of the death penalty against those who committed crimes as children is an act contrary to American standards of decency and fairness, as well as international law. The adolescent brain has not fully developed before the age of 18 years of age. Thus children do not have the same emotional and mental capacity as adults. Although juveniles should be held accountable for their crimes, the United States must not impose this most extreme punishment. The medical profession must take a stand to stop the execution of juvenile offenders in the United States.

  12. Juvenile dermatomyositis in a Nigerian girl

    PubMed Central

    Adelowo, Olufemi; Nwankwo, Madu; Olaosebikan, Hakeem

    2014-01-01

    Juvenile dermatomyositis is an autoimmune connective tissue disease occurring in children less than 16 years old. It is part of a heterogeneous group of muscle diseases called idiopathic Iiflammatory myopathies. It had previously been reported in black Africans resident in UK. However, there is no documented case reported from Africa. The index sign of heliotrope rashes is often difficult to visualise in the black skin. An 11-year-old Nigerian girl presenting with clinical, laboratory and histopathological features of juvenile dermatomyositis is presented here. It is hoped that this case will heighten the index of suspicion of this condition among medical practitioners in Africa. PMID:24706700

  13. Best Practices in Juvenile Accountability: Overview. JAIBG Bulletin.

    ERIC Educational Resources Information Center

    Beyer, Marty

    This bulletin examines the Office of Juvenile Justice and Delinquency Prevention's Juvenile Accountability Incentive Block Grants (JAIBG) program, which asserts that juvenile offenders should be held accountable for their crimes as a matter of basic justice and to prevent and deter delinquency. It reviews the developmental perspective shaping…

  14. Resiliency Scales for Children and Adolescents: Profiles of Juvenile Offenders

    ERIC Educational Resources Information Center

    Mowder, Melissa H.; Cummings, Jack A.; McKinney, Robert

    2010-01-01

    An exploratory study of resiliency profiles of male and female juvenile offenders committed to a juvenile correctional facility was conducted. The goal of the present study was to examine juvenile offenders' positive characteristics (e.g., adaptability, optimism, self-efficacy, tolerance of differences). To assess positive characteristics and…

  15. Race, Legal Representation, and Juvenile Justice: Issues and Concerns

    ERIC Educational Resources Information Center

    Guevara, Lori; Spohn, Cassia; Herz, Denise

    2004-01-01

    The objective of this study was to examine the influence of type of counsel across race on juvenile court outcomes. Using data from a sample of juvenile court referrals from two midwestern juvenile courts, this study examined the interaction of race and type of counsel on disposition outcome. The results indicated that youth without an attorney…

  16. Conditions of Confinement: Juvenile Detention and Corrections Facilities. Research Summary.

    ERIC Educational Resources Information Center

    Parent, Dale G.; And Others

    The most comprehensive nationwide research ever conducted on the juvenile detention and corrections field was a study by the Office of Juvenile Justice and Delinquency Prevention (OJJDP) assessing conditions of confinement for juveniles and determining the extent to which those conditions conform to recognized national professional standards. The…

  17. Programa Shortstop: A Culturally Focused Juvenile Intervention for Hispanic Youth

    ERIC Educational Resources Information Center

    Cervantes, Richard C.; Ruan, Karen; Duenas, Norma

    2004-01-01

    Culturally sensitive juvenile delinquency and substance abuse interventions are relatively limited and unavailable to many first-time Hispanic juvenile offenders. The purpose of this study was to test the effectiveness of a culturally focused juvenile and substance abuse intervention program for first time Hispanic youth offenders. The intent of…

  18. Contagion and Repeat Offending among Urban Juvenile Delinquents

    ERIC Educational Resources Information Center

    Mennis, Jeremy; Harris, Philip

    2011-01-01

    This research investigates the role of repeat offending and spatial contagion in juvenile delinquency recidivism using a database of 7166 male juvenile offenders sent to community-based programs by the Family Court of Philadelphia. Results indicate evidence of repeat offending among juvenile delinquents, particularly for drug offenders. The…

  19. The World of Juvenile Justice According to the Numbers

    ERIC Educational Resources Information Center

    Rozalski, Michael; Deignan, Marilyn; Engel, Suzanne

    2008-01-01

    Intended to be an instructive, yet sobering, introduction to the complex and disturbing nature of the juvenile justice system, this article details the "numbers," including selected percentages, ratios, and dollar amounts, that are relevant to developing a better understanding of the juvenile justice system. General statistics about juvenile and…

  20. 28 CFR 0.57 - Criminal prosecutions against juveniles.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 28 Judicial Administration 1 2010-07-01 2010-07-01 false Criminal prosecutions against juveniles... JUSTICE Criminal Division § 0.57 Criminal prosecutions against juveniles. The Assistant Attorney General..., United States Code, relating to criminal proceedings against juveniles. The Assistant Attorney General...

  1. Neuromorphic Silicon Neuron Circuits

    PubMed Central

    Indiveri, Giacomo; Linares-Barranco, Bernabé; Hamilton, Tara Julia; van Schaik, André; Etienne-Cummings, Ralph; Delbruck, Tobi; Liu, Shih-Chii; Dudek, Piotr; Häfliger, Philipp; Renaud, Sylvie; Schemmel, Johannes; Cauwenberghs, Gert; Arthur, John; Hynna, Kai; Folowosele, Fopefolu; Saighi, Sylvain; Serrano-Gotarredona, Teresa; Wijekoon, Jayawan; Wang, Yingxue; Boahen, Kwabena

    2011-01-01

    Hardware implementations of spiking neurons can be extremely useful for a large variety of applications, ranging from high-speed modeling of large-scale neural systems to real-time behaving systems, to bidirectional brain–machine interfaces. The specific circuit solutions used to implement silicon neurons depend on the application requirements. In this paper we describe the most common building blocks and techniques used to implement these circuits, and present an overview of a wide range of neuromorphic silicon neurons, which implement different computational models, ranging from biophysically realistic and conductance-based Hodgkin–Huxley models to bi-dimensional generalized adaptive integrate and fire models. We compare the different design methodologies used for each silicon neuron design described, and demonstrate their features with experimental results, measured from a wide range of fabricated VLSI chips. PMID:21747754

  2. Neuronal ubiquitin homeostasis

    PubMed Central

    Hallengren, Jada; Chen, Ping-Chung; Wilson, Scott M.

    2013-01-01

    Neurons have highly specialized intracellular compartments that facilitate the development and activity of the nervous system. Ubiquitination is a post-translational modification that controls many aspects of neuronal function by regulating protein abundance. Disruption of this signaling pathway has been demonstrated in neurological disorders such as Parkinson’s disease, Amyotrophic Lateral Sclerosis and Angleman Syndrome. Since many neurological disorders exhibit ubiquitinated protein aggregates, the loss of neuronal ubiquitin homeostasis may be an important contributor of disease. This review discusses the mechanisms utilized by neurons to control the free pool of ubiquitin necessary for normal nervous system development and function as well as new roles of protein ubiquitination in regulating synaptic activity. PMID:23686613

  3. Juvenile rheumatoid arthritis: therapeutic perspectives.

    PubMed

    Chikanza, Ian C

    2002-01-01

    Juvenile rheumatoid arthritis (JRA) is the most common childhood chronic systemic autoimmune inflammatory disease. The therapeutic approach to JRA has, to date, been casual and based on extensions of clinical experiences gained in the management of adult rheumatoid arthritis (RA). The physiology of inflammation has been systemically studied and this has led to the identification of specific therapeutic targets and the development of novel approaches to the management of JRA. The classical treatments of the disease such as methotrexate, sodium aurothiomalate and sulfasalazine, are not always effective in controlling RA and JRA. This has necessitated the development of novel agents for treating RA, most of which are biological in nature and are targeted at specific sites of the inflammatory cascades. These biological therapeutic strategies in RA have proved successful and are being applied in the management of JRA. These developments have been facilitated by the advances in molecular biology which have heralded the advent of biodrugs (recombinant proteins) and gene therapy, in which specific genes can be introduced locally to enhance in vivo gene expression or suppress gene(s) of interest with a view to down-regulating inflammation. Some of these biodrugs, such as anti-tumor necrosis factor alpha (anti-TNFalpha), monoclonal antibodies (infliximab, adalimumab), TNF soluble receptor constructs (etanercept) and interleukin-1 receptor antagonist (IL-1Ra) have been tested and shown to be effective in RA. Etanercept has now been licensed for JRA. Clinical trials of infliximab in JRA are planned. Studies show that the clinical effects are transient, necessitating repeated treatments and the risk of vaccination effects. Anti-inflammatory cytokines such as IL-4, IL-10, transforming growth factor-beta and interferon-beta (IFN-beta) are undergoing clinical trials. Many of these agents have to be administered parenterally and production costs are very high; thus, there is a need

  4. The challenges of the first migration: movement and behaviour of juvenile vs. adult white storks with insights regarding juvenile mortality.

    PubMed

    Rotics, Shay; Kaatz, Michael; Resheff, Yehezkel S; Turjeman, Sondra Feldman; Zurell, Damaris; Sapir, Nir; Eggers, Ute; Flack, Andrea; Fiedler, Wolfgang; Jeltsch, Florian; Wikelski, Martin; Nathan, Ran

    2016-07-01

    Migration conveys an immense challenge, especially for juvenile birds coping with enduring and risky journeys shortly after fledging. Accordingly, juveniles exhibit considerably lower survival rates compared to adults, particularly during migration. Juvenile white storks (Ciconia ciconia), which are known to rely on adults during their first fall migration presumably for navigational purposes, also display much lower annual survival than adults. Using detailed GPS and body acceleration data, we examined the patterns and potential causes of age-related differences in fall migration properties of white storks by comparing first-year juveniles and adults. We compared juvenile and adult parameters of movement, behaviour and energy expenditure (estimated from overall dynamic body acceleration) and placed this in the context of the juveniles' lower survival rate. Juveniles used flapping flight vs. soaring flight 23% more than adults and were estimated to expend 14% more energy during flight. Juveniles did not compensate for their higher flight costs by increased refuelling or resting during migration. When juveniles and adults migrated together in the same flock, the juvenile flew mostly behind the adult and was left behind when they separated. Juveniles showed greater improvement in flight efficiency throughout migration compared to adults which appears crucial because juveniles exhibiting higher flight costs suffered increased mortality. Our findings demonstrate the conflict between the juveniles' inferior flight skills and their urge to keep up with mixed adult-juvenile flocks. We suggest that increased flight costs are an important proximate cause of juvenile mortality in white storks and likely in other soaring migrants and that natural selection is operating on juvenile variation in flight efficiency.

  5. The challenges of the first migration: movement and behaviour of juvenile vs. adult white storks with insights regarding juvenile mortality.

    PubMed

    Rotics, Shay; Kaatz, Michael; Resheff, Yehezkel S; Turjeman, Sondra Feldman; Zurell, Damaris; Sapir, Nir; Eggers, Ute; Flack, Andrea; Fiedler, Wolfgang; Jeltsch, Florian; Wikelski, Martin; Nathan, Ran

    2016-07-01

    Migration conveys an immense challenge, especially for juvenile birds coping with enduring and risky journeys shortly after fledging. Accordingly, juveniles exhibit considerably lower survival rates compared to adults, particularly during migration. Juvenile white storks (Ciconia ciconia), which are known to rely on adults during their first fall migration presumably for navigational purposes, also display much lower annual survival than adults. Using detailed GPS and body acceleration data, we examined the patterns and potential causes of age-related differences in fall migration properties of white storks by comparing first-year juveniles and adults. We compared juvenile and adult parameters of movement, behaviour and energy expenditure (estimated from overall dynamic body acceleration) and placed this in the context of the juveniles' lower survival rate. Juveniles used flapping flight vs. soaring flight 23% more than adults and were estimated to expend 14% more energy during flight. Juveniles did not compensate for their higher flight costs by increased refuelling or resting during migration. When juveniles and adults migrated together in the same flock, the juvenile flew mostly behind the adult and was left behind when they separated. Juveniles showed greater improvement in flight efficiency throughout migration compared to adults which appears crucial because juveniles exhibiting higher flight costs suffered increased mortality. Our findings demonstrate the conflict between the juveniles' inferior flight skills and their urge to keep up with mixed adult-juvenile flocks. We suggest that increased flight costs are an important proximate cause of juvenile mortality in white storks and likely in other soaring migrants and that natural selection is operating on juvenile variation in flight efficiency. PMID:27046512

  6. NeuronBank: A Tool for Cataloging Neuronal Circuitry

    PubMed Central

    Katz, Paul S.; Calin-Jageman, Robert; Dhawan, Akshaye; Frederick, Chad; Guo, Shuman; Dissanayaka, Rasanjalee; Hiremath, Naveen; Ma, Wenjun; Shen, Xiuyn; Wang, Hsui C.; Yang, Hong; Prasad, Sushil; Sunderraman, Rajshekhar; Zhu, Ying

    2010-01-01

    The basic unit of any nervous system is the neuron. Therefore, understanding the operation of nervous systems ultimately requires an inventory of their constituent neurons and synaptic connectivity, which form neural circuits. The presence of uniquely identifiable neurons or classes of neurons in many invertebrates has facilitated the construction of cellular-level connectivity diagrams that can be generalized across individuals within a species. Homologous neurons can also be recognized across species. Here we describe NeuronBank.org, a web-based tool that we are developing for cataloging, searching, and analyzing neuronal circuitry within and across species. Information from a single species is represented in an individual branch of NeuronBank. Users can search within a branch or perform queries across branches to look for similarities in neuronal circuits across species. The branches allow for an extensible ontology so that additional characteristics can be added as knowledge grows. Each entry in NeuronBank generates a unique accession ID, allowing it to be easily cited. There is also an automatic link to a Wiki page allowing an encyclopedic explanation of the entry. All of the 44 previously published neurons plus one previously unpublished neuron from the mollusc, Tritonia diomedea, have been entered into a branch of NeuronBank as have 4 previously published neurons from the mollusc, Melibe leonina. The ability to organize information about neuronal circuits will make this information more accessible, ultimately aiding research on these important models. PMID:20428500

  7. [Neurological alterations and intellectual deficits with sudden visual loss in a 7-year-old boy].

    PubMed

    Gotz-Wieckowska, A; Pawlak, M; Siwiec-Proscinska, J; Seget, M

    2013-05-01

    Neuronal ceroid-lipofuscinoses (NLC) are a clinically and genetically heterogeneous group of lysosomal storage diseases. The most common NCL is the juvenile type which begins between the ages of 4 and 10 years in most cases with sudden visual loss which correlates with maculopathy and leads to blindness within a few years of presentation. After several years neurological deterioration ensues and in most cases death occurs in the 3rd decade of life. As with other storage disorders NCL is an incurable disease. PMID:23224126

  8. Criminal Profiles of Violent Juvenile Sex and Violent Juvenile Non-Sex Offenders: An Explorative Longitudinal Study

    ERIC Educational Resources Information Center

    van Wijk, Anton Ph.; Mali, Bas R. F.; Bullens, Ruud A. R.; Vermeiren, Robert R.

    2007-01-01

    Few studies have longitudinally investigated the criminal profiles of violent juvenile sex and violent juvenile non-sex offenders. To make up for this lack, this study used police records of juveniles to determine the nature of the criminal profiles of violent sex offenders (n = 226) and violent non-sex offenders (n = 4,130). All offenders…

  9. Juvenile Residential Facility Census, 2010: Selected Findings. Juvenile Offenders and Victims: National Report Series. Bulletin NCJ 241134

    ERIC Educational Resources Information Center

    Hockenberry, Sarah; Sickmund, Melissa; Sladky, Anthony

    2013-01-01

    This bulletin is part of the "Juvenile Offenders and Victims National Report Series." The "National Report" offers a comprehensive statistical overview of the problems of juvenile crime, violence, and victimization and the response of the juvenile justice system. During each interim year, the bulletins in the "National…

  10. Juvenile penalty or leniency: Sentencing of juveniles in the criminal justice system.

    PubMed

    Jordan, Kareem L; McNeal, Brittani A

    2016-08-01

    The purpose of this study is to examine the impact of being juvenile on sentencing in the criminal justice system. More specifically, youth transferred to criminal court are compared to adults in terms of likelihood of incarceration, jail length, and prison length. In this study, 2 national data sets are merged. The juvenile sample includes 3,381 convicted offenders, and the adult sample is comprised of 6,529 convicted offenders. The final sample is 9,910 offenders across 36 U.S. counties. The key independent variable is juvenile status, and the dependent variables are incarceration, jail length, and prison length. Because of the multilevel nature of the data, hierarchical linear modeling is used across all models. Juveniles are punished less severely in the jail incarceration decision. However, when youth are actually sentenced to incarceration (either jail or prison), they are given longer confinement time than adults. (PsycINFO Database Record PMID:26974365

  11. Juvenile penalty or leniency: Sentencing of juveniles in the criminal justice system.

    PubMed

    Jordan, Kareem L; McNeal, Brittani A

    2016-08-01

    The purpose of this study is to examine the impact of being juvenile on sentencing in the criminal justice system. More specifically, youth transferred to criminal court are compared to adults in terms of likelihood of incarceration, jail length, and prison length. In this study, 2 national data sets are merged. The juvenile sample includes 3,381 convicted offenders, and the adult sample is comprised of 6,529 convicted offenders. The final sample is 9,910 offenders across 36 U.S. counties. The key independent variable is juvenile status, and the dependent variables are incarceration, jail length, and prison length. Because of the multilevel nature of the data, hierarchical linear modeling is used across all models. Juveniles are punished less severely in the jail incarceration decision. However, when youth are actually sentenced to incarceration (either jail or prison), they are given longer confinement time than adults. (PsycINFO Database Record

  12. Old Dogs Learning New Tricks: Neuroplasticity Beyond the Juvenile Period

    PubMed Central

    Lillard, Angeline S.; Erisir, Alev

    2014-01-01

    Twenty years ago, the prevalent view in psychology was that although learning and the formation of new memories are lifelong occurrences, the neural changes associated with these events were all in the existing receptors. No new neural hardware, from synapses to neurons, was thought to appear after a protracted period early in life. In the past 20 years, another view has supplanted this one, showing that although the juvenile period is especially suited to neuroplastic adaptation, there is hard neuroplastic change later in life as well. We review a selection of evidence for this view from both animal and human models, showing how it reflects three principles of neuroplasticity: 1) earlier and later experience-induced changes to neuroarchitecture differ in degree more so than in type; 2) the types of experiences that lead to neuroplastic change narrow with age; and 3) differences in the amenability of neural circuitry to change result from basic differences in neuroarchitecture and neuroenvironment in different phases of development. PMID:24648605

  13. Juvenile myoclonic epilepsy: A system disorder of the brain.

    PubMed

    Wolf, Peter; Yacubian, Elza Márcia Targas; Avanzini, Giuliano; Sander, Thomas; Schmitz, Bettina; Wandschneider, Britta; Koepp, Matthias

    2015-08-01

    The prevailing understanding of generalized epilepsy is shaped by the traditional definition that "the responsible neuronal discharge takes place, if not throughout the entire grey matter, then at least in the greater part of it and simultaneously on both sides". This view is no longer tenable since concurrent findings using multiple methods have accumulated to reveal the role of bilateral networks of distributed and selective cortical and subcortical structures in so-called generalized ictogenesis. Most of this research has been focused on juvenile myoclonic epilepsy (JME), which today is commonly considered the archetypical syndrome of the idiopathic generalized epilepsies. Based upon recent research in the fields of clinical epileptology, neuropsychology and psychiatry, clinical neurophysiology, neuroimaging and epilepsy genetics this article, for the first time, unites these new findings into a comprehensive nosological view. Genetically determined dysfunctions of important cognitive systems like visuomotor coordination and linguistic communication appear now as key mechanisms of seizure generation in JME. This review suggests a new paradigm to consider JME as a system disorder of the brain analogous to other neurological system disorders.

  14. Deinstitutionalization of Juvenile Offenders: A Selected Bibliography.

    ERIC Educational Resources Information Center

    National Center on Institutions and Alternatives, Washington, DC.

    This bibliography, compiled by the National Center on Institutions and Alternatives, encompasses a wide spectrum of documents related to juvenile deinstitutionalization. Although the bibliography is selective, it is a comprehensive listing of sources that deal with the negative effects of institutionalization, the movement toward…

  15. The Juvenile Justice System. Chapter 6.

    ERIC Educational Resources Information Center

    1996

    This collection of papers presented at a 1996 conference on children's mental health focuses on the juvenile justice system. Papers have the following titles and authors: (1) "Delinquency and Mental Illness: The Intersection of Problems and Systems" (Carolyn S. Breda); (2) "Assessing the Mental Health of Adolescents in the Mental Health and…

  16. Juvenile Delinquency--A Community Perspective.

    ERIC Educational Resources Information Center

    Sotomayor, Marta

    1979-01-01

    Discusses two situations dealing with juvenile delinquency: (1) prejudicial attitudes stemming from racism and how they are reflected in the service delivery infrastructure, service accessibility and utilization, and the development of more appropriate intervention strategies; and (2) the elements of small groups and families in a community…

  17. Predictors of juveniles' noncompliance with probation requirements.

    PubMed

    NeMoyer, Amanda; Goldstein, Naomi E S; McKitten, Rhonda L; Prelic, Ana; Ebbecke, Jenna; Foster, Erika; Burkard, Casey

    2014-12-01

    Probation is the most common disposition for adjudicated youth, but little is known about which specific requirements are commonly imposed on juveniles, the requirements with which juveniles most often fail to comply, and how certain youth characteristics and/or imposed requirements might relate to probation noncompliance. An investigation of 120 archived files of youth represented by an urban public defender's office identified 29 probation requirements imposed on youth and 18 requirements with which youth commonly failed to comply. Results revealed that 52% of youth failed to comply with at least one probation requirement; prior probation noncompliance and race were both significantly associated with noncompliance in the examined probation disposition. In addition, the probability of probation noncompliance was significantly higher when youth received either of two substance-related probation requirements: drug tests or drug and alcohol counseling. Such results may prompt further investigation of juvenile probation-related predictors, identify areas of need for clinical service provision to foster successful completion of probation requirements, and help identify areas of potential biases among juvenile court personnel. PMID:24933176

  18. Factors Involved in Juveniles' Decisions about Crime.

    ERIC Educational Resources Information Center

    Cimler, Edward; Beach, Lee Roy

    1981-01-01

    Investigated whether delinquency is the result of a rational decision. The Subjective Expected Utility (SEU) model from decision theory was used with male juvenile offenders (N=45) as the model of the decision process. Results showed that the SEU model predicted 62.7 percent of the subjects' decisions. (Author/RC)

  19. Juvenile Sex Offenders: Development and Correction.

    ERIC Educational Resources Information Center

    Ryan, Gail; And Others

    1987-01-01

    Three case histories elucidate a discussion of the developmental nature of the behaviors of juvenile male sexual offenders. The sexual assault cycle is defined in the stages of negative self-image, predicting rejection, isolation, fantasies, planning the offense, and committing the offense. Tools for treating the offender are outlined. (Author/JDD)

  20. Costs of Juvenile Violence: Policy Implications.

    ERIC Educational Resources Information Center

    Miller, Ted; Fisher, Deborah A.; Cohen, Mark A.

    2001-01-01

    Investigated the magnitude of juvenile violence in Pennsylvania in terms of victimization and perpetration. Used archival data on violent crimes in Pennsylvania during 1993 to develop cost estimates reflecting the costs incurred by society for both victims and perpetrators. Overall, violence against children and adolescents proved to be a much…

  1. Metamorphosis: How Missouri Rehabilitates Juvenile Offenders

    ERIC Educational Resources Information Center

    Dubin, Jennifer

    2012-01-01

    Juveniles convicted of serious offenses usually end up in large correctional facilities that focus on punishment--not rehabilitation. The state of Missouri, however, has found a better way to help end the cycle of crime: by creating a network of small facilities that provide therapy and educational opportunities, it has dramatically reduced…

  2. Phototaxis of larval and juvenile northern pike

    USGS Publications Warehouse

    Zigler, S.J.; Dewey, M.R.

    1995-01-01

    Age- Phi northern pike Esox lucius prefer vegetated habitats that are difficult to sample with standard towed gears. Light traps can be effective for sampling larval fishes in dense vegetation, given positive phototaxis of fish. We evaluated the phototactic response of young northern pike by comparing the catches of larvae and juveniles obtained with plexiglass traps deployed with a chemical light stick versus traps deployed without a light source (controls) in a laboratory raceway and in a vegetated pond. In the laboratory tests, catches of protolarvae and mesolarvae in lighted traps were 11-35 times greater than catches in control traps. The catches of juvenile northern pike in field and laboratory experiments were 3-15 times greater in lighted traps than in control traps, even though the maximum body width of the larger juveniles was similar to the width of the entrance slots of the traps (5 mm). Larval and juvenile northern pike were photopositive; thus, light traps should effectively sample age-0 northern pike for at least 6 weeks after hatching.

  3. Rehabilitation of the Personality of Juvenile Offenders

    ERIC Educational Resources Information Center

    Zaitsev, G. K.; Zaitsev, A. G.; Dmitriev, M. G.; Apal'kova, I. Iu.

    2009-01-01

    Russian youth has in recent years been increasingly involved in crime, narcotics addiction, and alcoholism, possibly due to a failure of socialization in childhood. Researchers are seeking the origins of this phenomenon and searching for ways to combat it through rehabilitation of juvenile offenders. The essential nature of social and pedagogical…

  4. The Visual Anatomy of the Juvenile Delinquent.

    ERIC Educational Resources Information Center

    Kaseno, Stanley L.

    1985-01-01

    A project offering comprehensive vision therapy evaluations and visual-perception testing to juvenile delinquents revealed a high percentage of undiagnosed and previously untreated visual perceptual problems. Treatment has resulted in marked reduction in recidivism and increases in reading skills. (CL)

  5. Youth for Justice. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Nessel, Paula A.

    Youth for Justice uses the power of active learning to teach youth practical information about the law while addressing the risks associated with being young in the United States today. This unique initiative is a law-related education (LRE) program supported by the United States Department of Justice's Office of Juvenile Justice and Delinquency…

  6. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis

    PubMed Central

    Nelson, Andrew D.; Fischer, Philip R.; Reed, Ann M.; Wylam, Mark E.

    2015-01-01

    We describe the development of rheumatoid factor-positive migratory polyarthritis in a 5-year-old male who had been administered bidaily oral mineral oil as a laxative since birth. Minor respiratory symptoms, radiographic and bronchoscopic findings were consistent with chronic lipoid pneumonia. We speculate that immune sensitization to mineral oil promoted the clinical syndrome of juvenile idiopathic arthritis. PMID:26171269

  7. Juvenile Arthritis: Discoveries Lead to Newer Treatments

    MedlinePlus

    ... clinical team leader at the Food and Drug Administration (FDA), says that children with juvenile arthritis and their parents have reason to be optimistic. In the last several years, new therapies have been developed by drug companies and approved by the FDA that moderate the ...

  8. Calcinosis in juvenile dermatomyositis mimicking cold abscess.

    PubMed

    Nagar, Rajendra P; Bharati, Joyita; Sheriff, Abraar; Priyadarshini, Praytusha; Chumber, Sunil; Kabra, S K

    2016-01-01

    We report a case of dystrophic calcification presenting as soft cystic swelling in a patient with juvenile dermatomyositis. A 15-year-old boy with lumbosacral cystic swelling, which was considered a cold abscess clinically, was evaluated for nonresponse to antitubercular therapy. The cystic swelling had liquefied calcium with a well circumscribed calcified wall on imaging, which was subsequently excised. PMID:27586213

  9. Diagnosis of juvenile angiofibroma by computed tomography.

    PubMed

    Weinstein, M A; Levine, H; Duchesneau, P M; Tucker, H M

    1978-03-01

    Computed tomography (CT) accurately localized juvenile angiofibromata in 3 patients. The expanded pterygopalatine fossa and canal were visualized by CT in all three cases. Because of the hemorrhagic tendency of these tumors, a noninvasive modality such as CT is especially valuable in planning therapy.

  10. Responding to Problematic Sexualized Behavior in Juveniles.

    ERIC Educational Resources Information Center

    Baker, Eugene A.

    Keeping children safe becomes a more complicated concern when many of the offenders are not adults, but other children. Data from the justice system reveals that juveniles account for about 20% of all forcible rapes and about 50% of child sexual abuse. Contrary to the media's depiction, the majority come from two-parent homes, have no prior…

  11. Iatrogenesis in the Juvenile Justice System.

    ERIC Educational Resources Information Center

    Miller, Michael O.; Gold, Martin

    1984-01-01

    Reviews the process by which the juvenile justice system seems to worsen the problem it intends to cure. Reports on a study which found that youth who are arrested and adjudicated subsequently become more deliquent than their peers who have committed a recent felonious act but were not caught. (KH)

  12. Behavioral science and the juvenile death penalty.

    PubMed

    Leong, G B; Eth, S

    1989-01-01

    Behavioral science data included in an amicus brief has been introduced into a recent Supreme Court decision (Thompson v. Oklahoma) involving the juvenile death penalty. However, a close examination of the data fails to provide support for either the pro- or antijuvenile death penalty position.

  13. Juvenile Drug Courts and Teen Substance Abuse

    ERIC Educational Resources Information Center

    Butts, Jeffrey A., Ed.; Roman, John, Ed.

    2004-01-01

    Juvenile justice officials across the United States are embracing a new method of dealing with adolescent substance abuse. Importing a popular innovation from adult courts, state and local governments have started hundreds of specialized drug courts to provide judicial supervision and coordinate substance abuse treatment for drug-involved…

  14. Predictors of Juvenile Delinquency and Violence.

    ERIC Educational Resources Information Center

    Daley, Christine E.; Onwuegbuzie, Anthony J.

    Violence among youth has reached epidemic proportions. Every five minutes a child is arrested for a violent crime. To understand this trend, this paper examines characteristics of adolescent males who come into contact with the juvenile justice system. The study focuses on drug and alcohol involvement, the relevance of education, sexual practices,…

  15. Program Performance Inventory: Six Juvenile Offender Programs.

    ERIC Educational Resources Information Center

    Thomalla, Terri Groff; Dougherty, Victoria J.

    This report describes the performance of 6 Connecticut juvenile justice alternative sanction programs in 14 qualitative areas: community reintegration; outcomes and evaluation; assessment methods; risk factors; escalation of criminal activity; family involvement; community involvement; work ethic and vocational training; education and life skills;…

  16. Language and Communication Difficulties in Juvenile Offenders

    ERIC Educational Resources Information Center

    Bryan, Karen; Freer, Jackie; Furlong, Cheryl

    2007-01-01

    Background: Studies of the prison population suggest that the numbers of prisoners with language and communication disorders is higher than that of the overall population. However, the prison population is heterogeneous and it is important to focus on specific areas of the population. This study focuses on juvenile offenders. Aims: The study aimed…

  17. Predictors of juveniles' noncompliance with probation requirements.

    PubMed

    NeMoyer, Amanda; Goldstein, Naomi E S; McKitten, Rhonda L; Prelic, Ana; Ebbecke, Jenna; Foster, Erika; Burkard, Casey

    2014-12-01

    Probation is the most common disposition for adjudicated youth, but little is known about which specific requirements are commonly imposed on juveniles, the requirements with which juveniles most often fail to comply, and how certain youth characteristics and/or imposed requirements might relate to probation noncompliance. An investigation of 120 archived files of youth represented by an urban public defender's office identified 29 probation requirements imposed on youth and 18 requirements with which youth commonly failed to comply. Results revealed that 52% of youth failed to comply with at least one probation requirement; prior probation noncompliance and race were both significantly associated with noncompliance in the examined probation disposition. In addition, the probability of probation noncompliance was significantly higher when youth received either of two substance-related probation requirements: drug tests or drug and alcohol counseling. Such results may prompt further investigation of juvenile probation-related predictors, identify areas of need for clinical service provision to foster successful completion of probation requirements, and help identify areas of potential biases among juvenile court personnel.

  18. Neuronal avalanches and learning

    NASA Astrophysics Data System (ADS)

    de Arcangelis, Lucilla

    2011-05-01

    Networks of living neurons represent one of the most fascinating systems of biology. If the physical and chemical mechanisms at the basis of the functioning of a single neuron are quite well understood, the collective behaviour of a system of many neurons is an extremely intriguing subject. Crucial ingredient of this complex behaviour is the plasticity property of the network, namely the capacity to adapt and evolve depending on the level of activity. This plastic ability is believed, nowadays, to be at the basis of learning and memory in real brains. Spontaneous neuronal activity has recently shown features in common to other complex systems. Experimental data have, in fact, shown that electrical information propagates in a cortex slice via an avalanche mode. These avalanches are characterized by a power law distribution for the size and duration, features found in other problems in the context of the physics of complex systems and successful models have been developed to describe their behaviour. In this contribution we discuss a statistical mechanical model for the complex activity in a neuronal network. The model implements the main physiological properties of living neurons and is able to reproduce recent experimental results. Then, we discuss the learning abilities of this neuronal network. Learning occurs via plastic adaptation of synaptic strengths by a non-uniform negative feedback mechanism. The system is able to learn all the tested rules, in particular the exclusive OR (XOR) and a random rule with three inputs. The learning dynamics exhibits universal features as function of the strength of plastic adaptation. Any rule could be learned provided that the plastic adaptation is sufficiently slow.

  19. Brain extracellular matrix retains connectivity in neuronal networks

    PubMed Central

    Bikbaev, Arthur; Frischknecht, Renato; Heine, Martin

    2015-01-01

    The formation and maintenance of connectivity are critically important for the processing and storage of information in neuronal networks. The brain extracellular matrix (ECM) appears during postnatal development and surrounds most neurons in the adult mammalian brain. Importantly, the removal of the ECM was shown to improve plasticity and post-traumatic recovery in the CNS, but little is known about the mechanisms. Here, we investigated the role of the ECM in the regulation of the network activity in dissociated hippocampal cultures grown on microelectrode arrays (MEAs). We found that enzymatic removal of the ECM in mature cultures led to transient enhancement of neuronal activity, but prevented disinhibition-induced hyperexcitability that was evident in age-matched control cultures with intact ECM. Furthermore, the ECM degradation followed by disinhibition strongly affected the network interaction so that it strongly resembled the juvenile pattern seen in naïve developing cultures. Taken together, our results demonstrate that the ECM plays an important role in retention of existing connectivity in mature neuronal networks that can be exerted through synaptic confinement of glutamate. On the other hand, removal of the ECM can play a permissive role in modification of connectivity and adaptive exploration of novel network architecture. PMID:26417723

  20. Brain extracellular matrix retains connectivity in neuronal networks.

    PubMed

    Bikbaev, Arthur; Frischknecht, Renato; Heine, Martin

    2015-09-29

    The formation and maintenance of connectivity are critically important for the processing and storage of information in neuronal networks. The brain extracellular matrix (ECM) appears during postnatal development and surrounds most neurons in the adult mammalian brain. Importantly, the removal of the ECM was shown to improve plasticity and post-traumatic recovery in the CNS, but little is known about the mechanisms. Here, we investigated the role of the ECM in the regulation of the network activity in dissociated hippocampal cultures grown on microelectrode arrays (MEAs). We found that enzymatic removal of the ECM in mature cultures led to transient enhancement of neuronal activity, but prevented disinhibition-induced hyperexcitability that was evident in age-matched control cultures with intact ECM. Furthermore, the ECM degradation followed by disinhibition strongly affected the network interaction so that it strongly resembled the juvenile pattern seen in naïve developing cultures. Taken together, our results demonstrate that the ECM plays an important role in retention of existing connectivity in mature neuronal networks that can be exerted through synaptic confinement of glutamate. On the other hand, removal of the ECM can play a permissive role in modification of connectivity and adaptive exploration of novel network architecture.

  1. Synapse-to-neuron ratio is inversely related to neuronal density in mature neuronal cultures.

    PubMed

    Cullen, D Kacy; Gilroy, Meghan E; Irons, Hillary R; Laplaca, Michelle C

    2010-11-01

    Synapse formation is a fundamental process in neurons that occurs throughout development, maturity, and aging. Although these stages contain disparate and fluctuating numbers of mature neurons, tactics employed by neuronal networks to modulate synapse number as a function of neuronal density are not well understood. The goal of this study was to utilize an in vitro model to assess the influence of cell density and neuronal maturity on synapse number and distribution. Specifically, cerebral cortical neurons were plated in planar culture at densities ranging from 10 to 5000 neurons/mm², and synapse number and distribution were evaluated via immunocytochemistry over 21 days in vitro (DIV). High-resolution confocal microscopy revealed an elaborate three-dimensional distribution of neurites and synapses across the heights of high-density neuronal networks by 21 DIV, which were up to 18 μm thick, demonstrating the complex degree of spatial interactions even in planar high-density cultures. At 7 DIV, the mean number of synapses per neuron was less than 5, and this did not vary as a function of neuronal density. However, by 21 DIV, the number of synapses per neuron had jumped 30- to 80-fold, and the synapse-to-neuron ratio was greatest at lower neuronal densities (< 500 neurons/mm²; mean approximately 400 synapses/neuron) compared to mid and higher neuronal densities (500-4500 neurons/mm²; mean of approximately 150 synapses/neuron) (p<0.05). These results suggest a relationship between neuronal density and synapse number that may have implications in the neurobiology of developing neuronal networks as well as processes of cell death and regeneration.

  2. Neuronal migration illuminated

    PubMed Central

    Trivedi, Niraj

    2011-01-01

    During vertebrate brain development, migration of neurons from the germinal zones to their final laminar positions is essential to establish functional neural circuits.1–3 Whereas key insights into neuronal migration initially came from landmark studies identifying the genes mutated in human cortical malformations,4 cell biology has recently greatly advanced our understanding of how cytoskeletal proteins and molecular motors drive the morphogenic cell movements that build the developing brain. This Commentary & View reviews recent studies examining the role of the molecular motors during neuronal migration and critically examines current models of acto-myosin function in the two-step neuronal migration cycle. Given the apparent emerging diversity of neuronal sub-type cytoskeletal organizations, we propose that two approaches must be taken to resolve differences between the current migration models: the mechanisms of radial and tangential migration must be compared, and the loci of tension generation, migration substrates and sites of adhesion dynamics must be precisely examined in an integrated manner. PMID:20935494

  3. Optical recording of neuronal spiking activity from unbiased populations of neurons with high spike detection efficiency and high temporal precision.

    PubMed

    Ranganathan, Gayathri N; Koester, Helmut J

    2010-09-01

    Activity in populations of neurons is essential for cortical function including signaling of information and signal transport. Previous methods have made advances in recording activity from many neurons but have both technical and analytical limitations. Here we present an optical method, dithered random-access functional calcium imaging, to record somatic calcium signals from up to 100 neurons, in vitro and in vivo. We further developed a maximum-likelihood deconvolution algorithm to detect spikes and precise spike timings from the recorded calcium fluorescence signals. Spike detection efficiency and spike timing detection was determined in acute slices of juvenile mice. The results indicate that the combination of the two methods detected precise spiking activity from unbiased and spatially distributed populations of neurons in acute slices with high efficiency of spike detection (>97%), low rate of false positives (0.0023 spikes/s), and high temporal precision. The results further indicate that there is only a small window of excitation intensities where high spike detection can be achieved consistently.

  4. Kappe neurons, a novel population of olfactory sensory neurons

    NASA Astrophysics Data System (ADS)

    Ahuja, Gaurav; Nia, Shahrzad Bozorg; Zapilko, Veronika; Shiriagin, Vladimir; Kowatschew, Daniel; Oka, Yuichiro; Korsching, Sigrun I.

    2014-02-01

    Perception of olfactory stimuli is mediated by distinct populations of olfactory sensory neurons, each with a characteristic set of morphological as well as functional parameters. Beyond two large populations of ciliated and microvillous neurons, a third population, crypt neurons, has been identified in teleost and cartilaginous fishes. We report here a novel, fourth olfactory sensory neuron population in zebrafish, which we named kappe neurons for their characteristic shape. Kappe neurons are identified by their Go-like immunoreactivity, and show a distinct spatial distribution within the olfactory epithelium, similar to, but significantly different from that of crypt neurons. Furthermore, kappe neurons project to a single identified target glomerulus within the olfactory bulb, mdg5 of the mediodorsal cluster, whereas crypt neurons are known to project exclusively to the mdg2 glomerulus. Kappe neurons are negative for established markers of ciliated, microvillous and crypt neurons, but appear to have microvilli. Kappe neurons constitute the fourth type of olfactory sensory neurons reported in teleost fishes and their existence suggests that encoding of olfactory stimuli may require a higher complexity than hitherto assumed already in the peripheral olfactory system.

  5. Imaging voltage in neurons

    PubMed Central

    Peterka, Darcy S.; Takahashi, Hiroto; Yuste, Rafael

    2011-01-01

    In the last decades, imaging membrane potential has become a fruitful approach to study neural circuits, especially in invertebrate preparations with large, resilient neurons. At the same time, particularly in mammalian preparations, voltage imaging methods suffer from poor signal to noise and secondary side effects, and they fall short of providing single-cell resolution when imaging of the activity of neuronal populations. As an introduction to these techniques, we briefly review different voltage imaging methods (including organic fluorophores, SHG chromophores, genetic indicators, hybrid, nanoparticles and intrinsic approaches), and illustrate some of their applications to neuronal biophysics and mammalian circuit analysis. We discuss their mechanisms of voltage sensitivity, from reorientation, electrochromic or electro-optical phenomena, to interaction among chromophores or membrane scattering, and highlight their advantages and shortcomings, commenting on the outlook for development of novel voltage imaging methods. PMID:21220095

  6. Repeated fluvoxamine treatment recovers juvenile stress-induced morphological changes and depressive-like behavior in rats.

    PubMed

    Lyttle, Kerise; Ohmura, Yu; Konno, Kohtarou; Yoshida, Takayuki; Izumi, Takeshi; Watanabe, Masahiko; Yoshioka, Mitsuhiro

    2015-08-01

    Human studies have suggested that early life stress such as child abuse could enhance susceptibility to depressive disorders. Moreover, the abnormalities of the prefrontal cortex have been associated with depression. Although clinical studies have implied the negative effects of early life stress on brain development, the causality and the detailed morphogenetic changes has not been clearly elucidated. In the present study, we determined the effect of juvenile stress exposure on the presentation of depressive-like behavior and the neural mechanisms involved using a rodent model. Rat pups were exposed to footshock stress during postnatal days 21-25 followed by repeated oral administration of fluvoxamine (0 or 10mg/kg/d × 14 days), which is a selective serotonin reuptake inhibitor. At the postadolescent stage forced swim test assessment of depressive-like behavior and Golgi-Cox staining of medial prefrontal cortex pyramidal neurons followed by morphological analyses were carried out. Post-adolescent behavioral and morphological studies identified the presentation of increased depressive-like behaviors and reduced spine densities and dendritic lengths of layer II/III pyramidal neuron in the infralimbic cortex, but not in the prelimbic cortex of rats exposed to juvenile stress. Repeated fluvoxamine treatment recovered the increased depressive-like behavior and reduced spine densities/dendritic lengths observed in rats exposed to footshock stress. Cortical thicknesses in the infralimbic cortex and prelimbic cortex were also reduced by juvenile stress, but these reductions were not recovered by fluvoxamine treatment. The results demonstrate cortical sensitivities to stress exposures during the juvenile stage which mediate behavioral impairments, and provide a clue to find therapeutics for early life stress-induced emotional dysfunctions.

  7. Movements of juvenile common ravens in an arid landscape

    USGS Publications Warehouse

    Webb, W.C.; Boarman, W.I.; Rotenberry, J.T.

    2009-01-01

    Movement patterns of juvenile birds are poorly understood, yet critically important ecological phenomena, especially for species with a prolonged juvenile period. We evaluated postfledging movements of juvenile common ravens (Corvus corax) in a western Mojave Desert landscape composed of a mosaic of natural and anthropogenic elements. Generally, ravens do not begin breeding until after their fourth year. We marked 2 annual cohorts of juvenile ravens and followed them from dispersal from their natal territory for up to 33 months. Movements of juvenile common ravens were similar for males and females. Conspecifics and confined livestock feeding operations represented important resources for juvenile ravens, and juveniles were rarely located in open desert. However, initial movements from the natal territory to the nearest communal point subsidy rather than the closest anthropogenic resource suggested juvenile dispersal was influenced by the combination of conspecifics and anthropogenic resources, rather than the distribution of those resources. Land managers concerned with growing raven populations should reduce access to concentrated anthropogenic resources such as landfills and dairies, which serve as important resources for juveniles. Because juvenile ravens rarely venture into open desert, reducing their numbers by lethal removal or other means is unlikely to lessen raven predation of desert tortoises (Gopherus agassizii).

  8. The impact of schools on juvenile substance initiation and use.

    PubMed

    Amuedo-Dorantes, Catalina; Mach, Traci; Clapp, John D

    2004-06-01

    We use data from the two rounds of the NLSY97 and the corresponding QED data to examine the effectiveness of school endowments and curricula in targeting juvenile use of tobacco, alcohol, and marijuana. Our results support the notion that schools matter in reducing juvenile involvement in substance use. Higher discretionary dollars per pupil are linked to reduced rates of juvenile initiation and repetitive use rates of cigarettes and marijuana. Additionally, school curricula, as indicated by the implementation of year round classes and some innovative and after-school programs--such as gifted and talented, attendance monitoring, homework hotline, international baccalaureate, extended-day, and mentoring, programs, affect both juvenile initiation to tobacco and alcohol use and juvenile repetitive use of tobacco and alcohol. In particular, we find that juvenile initiation to cigarette use is approximately between 2 percentage points and 3 percentage points lower among youths attending schools with gifted and talented and international baccalaureate programs. In addition, juvenile repetitive cigarette use is approximately 54%, 52%, and 48% lower among youths attending schools offering year round classes, international baccalaureate, and twenty-first century programs, respectively. Finally, juvenile initiation to alcohol use and juvenile repetitive use of alcohol are approximately 3% and 20% lower, respectively, among youths in schools offering gifted and talented programs. In sum, while these programs are not implemented to address substance use problems among the student body, we find that the implementation of these programs is often accompanied by a reduction in juvenile initiation and repetitive substance use.

  9. Exploring neuronal activity with photons

    NASA Astrophysics Data System (ADS)

    Bourdieu, Laurent; Léger, Jean-François

    2015-10-01

    The following sections are included: * Introduction * Information coding * Optical recordings of neuronal activity * Functional organization of the cortex at the level of a cortical column * Microarchitecture of a cortical column * Dynamics of neuronal populations * Outlook * Bibliography

  10. Juvenile rheumatoid arthritis: physical therapy and rehabilitation.

    PubMed

    Cakmak, Aysegul; Bolukbas, Nalan

    2005-02-01

    Juvenile arthritis is one of the most prevalent chronic diseases in the childhood period (ages 0 to 16 years). This disease was first defined in the first half of the 16th century. In the course of time, its differential diagnosis and characteristics have been determined, and it has been classified. Incidence and prevalence values are 10 to 20 in 100,000 and 56 to 113 in 100,000, respectively. Various factors are suggested for its underlying cause. Its denomination is also in dispute. Treatment of juvenile arthritis includes education, medical treatment, physical therapy, and occupational therapy. This article summarizes the objectives and methods of physical therapy and rehabilitation that are important parts of treatment.

  11. [Juvenile idiopathic arthritis: Definition and classification].

    PubMed

    Deslandre, C

    2016-04-01

    Juvenile idiopathic arthritis (JIA) is a group of diseases defined by the presence of arthritis of more than 6 weeks duration in patients aged less than 16 years and with unknown etiology. The international classification based on clinical and biological criteria define each type of JIA: systemic, oligoarticular, polyarticular with and without rheumatoid factor, enthesitis-related arthritis, and psoriatic arthritis. However, some discussions persist concerning systemic-onset juvenile idiopathic arthritis, whose clinical symptoms and pathogenic mechanisms are quite similar to those observed in autoinflammatory diseases, arthritis with antinuclear factors (poly- and oligoarticular) that could be considered as a homogenous group, and a family history of psoriasis that frequently led to unclassified arthritis. Better knowledge of the pathogenic mechanisms should improve the initial clinical classification with more homogeneous groups of patients and reduce the number of unclassified cases of arthritis. PMID:26968301

  12. The neuron classification problem

    PubMed Central

    Bota, Mihail; Swanson, Larry W.

    2007-01-01

    A systematic account of neuron cell types is a basic prerequisite for determining the vertebrate nervous system global wiring diagram. With comprehensive lineage and phylogenetic information unavailable, a general ontology based on structure-function taxonomy is proposed and implemented in a knowledge management system, and a prototype analysis of select regions (including retina, cerebellum, and hypothalamus) presented. The supporting Brain Architecture Knowledge Management System (BAMS) Neuron ontology is online and its user interface allows queries about terms and their definitions, classification criteria based on the original literature and “Petilla Convention” guidelines, hierarchies, and relations—with annotations documenting each ontology entry. Combined with three BAMS modules for neural regions, connections between regions and neuron types, and molecules, the Neuron ontology provides a general framework for physical descriptions and computational modeling of neural systems. The knowledge management system interacts with other web resources, is accessible in both XML and RDF/OWL, is extendible to the whole body, and awaits large-scale data population requiring community participation for timely implementation. PMID:17582506

  13. Nanoresolution radiology of neurons

    SciTech Connect

    Wu, H. R.; Chen, S. T.; Chu, Y. S.; Conley, R.; Bouet, N.; Chien, C. C.; Chen, H. H.; Lin, C. H.; Tung, H. T.; Chen, Y. S.; Margaritondo, G.; Je, J. H.; Hwu, Y.

    2012-05-29

    We report recent advances in hard-x-ray optics—including record spatial resolution—and in staining techniques that enable synchrotron microradiology to produce neurobiology images of quality comparable to electron and visible microscopy. In addition, microradiology offers excellent penetration and effective three-dimensional detection as required for many neuron studies. Our tests include tomographic reconstruction based on projection image sets.

  14. Nanoresolution radiology of neurons

    SciTech Connect

    Wu, H.R.; Chen, S.T.; Chu, Y.S.; Conley, R.; Bouet, N.; Chien, C.C.; Chen, H.H.; Lin, C.H.; Tung, H.T.; Chen, Y.S.; Margaritondo, G.; Je, J.H.; Hwu, Y.

    2013-04-08

    We report recent advances in hard-x-ray optics - including record spatial resolution - and in staining techniques that enable synchrotron microradiology to produce neurobiology images of quality comparable to electron and visible microscopy. In addition, microradiology offers excellent penetration and effective three-dimensional detection as required for many neuron studies. Our tests include tomographic reconstruction based on projection image sets.

  15. Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations(e–Pub ahead of print)

    PubMed Central

    Bäumer, D.; Hilton, D.; Paine, S.M.L.; Turner, M.R.; Lowe, J.; Talbot, K.; Ansorge, O.

    2010-01-01

    Background: Juvenile amyotrophic lateral sclerosis (ALS) with basophilic inclusions is a form of ALS characterized by protein deposits in motor neurons that are morphologically and tinctorially distinct from those of classic sporadic ALS. The nosologic position of this type of ALS in the molecular pathologic and genetic classification of ALS is unknown. Methods: We identified neuropathologically 4 patients with juvenile ALS with basophilic inclusions and tested the hypothesis that specific RNA binding protein pathology may define this type of ALS. Immunohistochemical findings prompted us to sequence the fused in sarcoma (FUS) gene. Results: Motor symptoms began between ages 17 and 22. Disease progression was rapid without dementia. No family history was identified. Basophilic inclusions were strongly positive for FUS protein but negative for TAR DNA binding protein 43 (TDP-43). Granular and compact FUS deposits were identified in glia and neuronal cytoplasm and nuclei. Ultrastructure of aggregates was in keeping with origin from fragmented rough endoplasmic reticulum. Sequencing of all 15 exons of the FUS gene in 3 patients revealed a novel deletion mutation (c.1554_1557delACAG) in 1 individual and the c.1574C>T (P525L) mutation in 2 others. Conclusion: Juvenile ALS with basophilic inclusions is a FUS proteinopathy and should be classified as ALS-FUS. The FUS c.1574C>T (P525L) and c.1554_1557delACAG mutations are associated with this distinct phenotype. The molecular genetic relationship with frontotemporal lobar degeneration with FUS pathology remains to be clarified. GLOSSARY ALS = amyotrophic lateral sclerosis; FTLD = frontotemporal lobar degeneration; FTLD-FUS = frontotemporal lobar degeneration with FUS pathology; NIFID = neuronal intermediate filament inclusion disease. PMID:20668261

  16. Serum ferritin in juvenile chronic polyarthritis.

    PubMed Central

    Craft, A W; Eastham, E J; Bell, J I; Brigham, K

    1977-01-01

    Six children with juvenile chronic polyarthritis were studied and their disease activity correlated with haematological values including serum ferritin. The latter is often raised above reference values, but even when within them appears to fluctuate significantly and correlates more closely with disease activity than any of the other parameters measured. We conclude that the serial measurement of serum ferritin may be a useful guide to the management of such children. PMID:879866

  17. Juvenile xanthogranuloma: a rare benign histiocytic disorder

    PubMed Central

    Kozicka, Dorota; Purzycka-Bohdan, Dorota; Biernat, Wojciech; Stawczyk, Marta; Nowicki, Roman

    2014-01-01

    Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder that typically affects children. The clinical presentation of this disease is characterized by single or, rarely, multiple yellow and brown skin nodules, most often found on the face and neck. Internal organ involvement has been sporadically observed in JXG and is associated with an increased risk of serious complications. We report two cases with a small and large nodular form of JXG. PMID:25097495

  18. Decreased fibrinolytic activity in juvenile chronic arthritis.

    PubMed

    Mussoni, L; Pintucci, G; Romano, G; De Benedetti, F; Massa, M; Martini, A

    1990-12-01

    The basal fibrinolytic activity in 17 children with active juvenile chronic arthritis (JCA) was investigated. It was found that patients with JCA, and particularly those with the systemic form, show decreased plasma fibrinolytic activity and a marked increase in plasminogen activator inhibitor. Additionally, it was found that patients with systemic JCA, but not those with the polyarticular or pauciarticular form, have increased circulating levels of tissue-type plasminogen activator, and endothelial cell protein, suggesting possible endothelial cell participation in systemic JCA.

  19. Tropical tree rings reveal preferential survival of fast-growing juveniles and increased juvenile growth rates over time.

    PubMed

    Rozendaal, Danaë M A; Brienen, Roel J W; Soliz-Gamboa, Claudia C; Zuidema, Pieter A

    2010-02-01

    Long-term juvenile growth patterns of tropical trees were studied to test two hypotheses: fast-growing juvenile trees have a higher chance of reaching the canopy ('juvenile selection effect'); and tree growth has increased over time ('historical growth increase'). Tree-ring analysis was applied to test these hypotheses for five tree species from three moist forest sites in Bolivia, using samples from 459 individuals. Basal area increment was calculated from ring widths, for trees < 30 cm in diameter. For three out of five species, a juvenile selection effect was found in rings formed by small juveniles. Thus, extant adult trees in these species have had higher juvenile growth rates than extant juvenile trees. By contrast, rings formed by somewhat larger juveniles in four species showed the opposite pattern: a historical growth increase. For most size classes of > 10 cm diameter none of the patterns was found. Fast juvenile growth may be essential to enable tropical trees to reach the forest canopy, especially for small juvenile trees in the dark forest understorey. The historical growth increase requires cautious interpretation, but may be partially attributable to CO(2) fertilization.

  20. Habitat selection by juvenile Mojave Desert tortoises

    USGS Publications Warehouse

    Todd, Brian D; Halstead, Brian J.; Chiquoine, Lindsay P.; Peaden, J. Mark; Buhlmann, Kurt A.; Tuberville, Tracey D.; Nafus, Melia G.

    2016-01-01

    Growing pressure to develop public lands for renewable energy production places several protected species at increased risk of habitat loss. One example is the Mojave desert tortoise (Gopherus agassizii), a species often at the center of conflicts over public land development. For this species and others on public lands, a better understanding of their habitat needs can help minimize negative impacts and facilitate protection or restoration of habitat. We used radio-telemetry to track 46 neonate and juvenile tortoises in the Eastern Mojave Desert, California, USA, to quantify habitat at tortoise locations and paired random points to assess habitat selection. Tortoise locations near burrows were more likely to be under canopy cover and had greater coverage of perennial plants (especially creosote [Larrea tridentata]), more coverage by washes, a greater number of small-mammal burrows, and fewer white bursage (Ambrosia dumosa) than random points. Active tortoise locations away from burrows were closer to washes and perennial plants than were random points. Our results can help planners locate juvenile tortoises and avoid impacts to habitat critical for this life stage. Additionally, our results provide targets for habitat protection and restoration and suggest that diverse and abundant small-mammal populations and the availability of creosote bush are vital for juvenile desert tortoises in the Eastern Mojave Desert.

  1. Juvenile xanthogranuloma: case report and review of the literature.

    PubMed

    Cypel, Tatiana Karine Simon; Zuker, Ronald Melvin

    2008-01-01

    The present case report describes a juvenile xanthogranuloma in a five-month-old girl. A circumscribed papule was located below the right nasal ala and above the right vermilion border. The lesion was histologically diagnosed as a juvenile xanthogranuloma after surgical resection of the mass. Juvenile xanthogranuloma is an uncommon diagnosis, with the head, neck and trunk being the most common sites. PMID:19721800

  2. Juvenile xanthogranuloma: Case report and review of the literature

    PubMed Central

    Cypel, Tatiana Karine Simon; Zuker, Ronald Melvin

    2008-01-01

    The present case report describes a juvenile xanthogranuloma in a five-month-old girl. A circumscribed papule was located below the right nasal ala and above the right vermilion border. The lesion was histologically diagnosed as a juvenile xanthogranuloma after surgical resection of the mass. Juvenile xanthogranuloma is an uncommon diagnosis, with the head, neck and trunk being the most common sites. PMID:19721800

  3. Juvenile ageism: unrecognized prejudice and discrimination against the young.

    PubMed

    Westman, J C

    1991-01-01

    Ageism is a form of prejudice and discrimination as virulent as racism and as pervasive as sexism. It has been described as it affects the elderly but has not been sufficiently recognized as it affects the young. Institutional juvenile ageism exists when social systems ignore the interests of children. Individual juvenile ageism exists when the developmental interests of a child are not respected. This article describes the dynamics, manifestations, and approaches to the amelioration of juvenile ageism.

  4. Giant Bilateral Juvenile Fibroadenoma of the Breast in Prepubescent Girl.

    PubMed

    Khan, Salma; Khan, Momna; Rafique, Sadia

    2015-10-01

    Juvenile fibroadenoma accounts for 4% of the total fibroadenomas. Giant juvenile fibroadenoma is found in only 0.5% of all fibroadenomas. The authors report a 10-year girl presenting with progressive enlargement of both breasts for one year. Based on clinical findings and Fine Needle Aspiration Cytology (FNAC), a diagnosis of bilateral giant juvenile fibroadenomas of breast was made. She underwent bilateral lumpectomy with breast conservation and made uneventful postoperative recovery. PMID:26522216

  5. Treatment of systemic-onset juvenile arthritis with canakinumab

    PubMed Central

    Peitz, Joachim; Horneff, Gerd

    2015-01-01

    Treatment of systemic-onset juvenile idiopathic arthritis is challenging, but the availability of cytokine antagonists targeting interleukin-1 and interleukin-6 have markedly advanced the therapeutic options. In this review, we focus on the current experience with canakinumab, an interleukin-1 monoclonal human antibody for the treatment of systemic-onset juvenile idiopathic arthritis and describe its efficacy and safety. Canakinumab is an important, safe, and valid drug in the treatment of systemic-onset juvenile idiopathic arthritis.

  6. Spatial distribution of neurons innervated by chandelier cells.

    PubMed

    Blazquez-Llorca, Lidia; Woodruff, Alan; Inan, Melis; Anderson, Stewart A; Yuste, Rafael; DeFelipe, Javier; Merchan-Perez, Angel

    2015-09-01

    Chandelier (or axo-axonic) cells are a distinct group of GABAergic interneurons that innervate the axon initial segments of pyramidal cells and are thus thought to have an important role in controlling the activity of cortical circuits. To examine the circuit connectivity of chandelier cells (ChCs), we made use of a genetic targeting strategy to label neocortical ChCs in upper layers of juvenile mouse neocortex. We filled individual ChCs with biocytin in living brain slices and reconstructed their axonal arbors from serial semi-thin sections. We also reconstructed the cell somata of pyramidal neurons that were located inside the ChC axonal trees and determined the percentage of pyramidal neurons whose axon initial segments were innervated by ChC terminals. We found that the total percentage of pyramidal neurons that were innervated by a single labeled ChC was 18-22 %. Sholl analysis showed that this percentage peaked at 22-35 % for distances between 30 and 60 µm from the ChC soma, decreasing to lower percentages with increasing distances. We also studied the three-dimensional spatial distribution of the innervated neurons inside the ChC axonal arbor using spatial statistical analysis tools. We found that innervated pyramidal neurons are not distributed at random, but show a clustered distribution, with pockets where almost all cells are innervated and other regions within the ChC axonal tree that receive little or no innervation. Thus, individual ChCs may exert a strong, widespread influence on their local pyramidal neighbors in a spatially heterogeneous fashion. PMID:25056931

  7. Eliminating the Competency Presumption in Juvenile Delinquency Cases.

    PubMed

    Katner, David R

    2015-01-01

    The legal presumption used in virtually all juvenile delinquency cases in the U.S. is that all juveniles are competent to stand trial. This Article calls for the elimination of that legal presumption, which is historically based on the Dusky v. United States decision and in the adult criminal justice system. The recent decisions of the U.S. Supreme Court recognize the developmental and organic brain differences between adults and juveniles. Current research demonstrates a higher frequency rate of incompetence based on intellectual deficiencies among children when compared with adults found to be not legally competent to stand trial. By eliminating the competency presumption for juveniles in both delinquency and adult criminal proceedings, the party seeking an adjudication would be responsible for establishing that the accused juvenile is in fact, competent to stand trial. Foreign jurisdictions in Europe, Asia, Africa, and South America have long required higher thresholds--at least fourteen years of age--for holding juveniles accountable for criminal misconduct, none of them presuming that juveniles are competent to go to trial. In the alternative, by expanding the factors currently in use for determination of juvenile competency by adding developmental immaturity and mental illness, juvenile justice systems could identify the reduction of recidivist offending as the primary systemic objective. PMID:26809160

  8. Natal homing in juvenile loggerhead turtles (Caretta caretta).

    PubMed

    Bowen, Brian W; Bass, Anna L; Chow, Shaio-Mei; Bostrom, Meredith; Bjorndal, Karen A; Bolten, Alan B; Okuyama, Toshinori; Bolker, Benjamin M; Epperly, Sheryan; Lacasella, Erin; Shaver, Donna; Dodd, Mark; Hopkins-Murphy, Sally R; Musick, John A; Swingle, Mark; Rankin-Baransky, Karen; Teas, Wendy; Witzell, Wayne N; Dutton, Peter H

    2004-12-01

    Juvenile loggerhead turtles (Caretta caretta) from West Atlantic nesting beaches occupy oceanic (pelagic) habitats in the eastern Atlantic and Mediterranean, whereas larger juvenile turtles occupy shallow (neritic) habitats along the continental coastline of North America. Hence the switch from oceanic to neritic stage can involve a trans-oceanic migration. Several researchers have suggested that at the end of the oceanic phase, juveniles are homing to feeding habitats in the vicinity of their natal rookery. To test the hypothesis of juvenile homing behaviour, we surveyed 10 juvenile feeding zones across the eastern USA with mitochondrial DNA control region sequences (N = 1437) and compared these samples to potential source (nesting) populations in the Atlantic Ocean and Mediterranean Sea (N = 465). The results indicated a shallow, but significant, population structure of neritic juveniles (PhiST = 0.0088, P = 0.016), and haplotype frequency differences were significantly correlated between coastal feeding populations and adjacent nesting populations (Mantel test R2 = 0.52, P = 0.001). Mixed stock analyses (using a Bayesian algorithm) indicated that juveniles occurred at elevated frequency in the vicinity of their natal rookery. Hence, all lines of evidence supported the hypothesis of juvenile homing in loggerhead turtles. While not as precise as the homing of breeding adults, this behaviour nonetheless places juvenile turtles in the vicinity of their natal nesting colonies. Some of the coastal hazards that affect declining nesting populations may also affect the next generation of turtles feeding in nearby habitats. PMID:15548292

  9. ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

    PubMed Central

    Sharma, Aarti; Lyashchenko, Alexander K.; Lu, Lei; Nasrabady, Sara Ebrahimi; Elmaleh, Margot; Mendelsohn, Monica; Nemes, Adriana; Tapia, Juan Carlos; Mentis, George Z.; Shneider, Neil A.

    2016-01-01

    Mutations in FUS cause amyotrophic lateral sclerosis (ALS), including some of the most aggressive, juvenile-onset forms of the disease. FUS loss-of-function and toxic gain-of-function mechanisms have been proposed to explain how mutant FUS leads to motor neuron degeneration, but neither has been firmly established in the pathogenesis of ALS. Here we characterize a series of transgenic FUS mouse lines that manifest progressive, mutant-dependent motor neuron degeneration preceded by early, structural and functional abnormalities at the neuromuscular junction. A novel, conditional FUS knockout mutant reveals that postnatal elimination of FUS has no effect on motor neuron survival or function. Moreover, endogenous FUS does not contribute to the onset of the ALS phenotype induced by mutant FUS. These findings demonstrate that FUS-dependent motor degeneration is not due to loss of FUS function, but to the gain of toxic properties conferred by ALS mutations. PMID:26842965

  10. 78 FR 42109 - Agency Information Collection Activities; Proposed Collection; Comments Requested: Juvenile...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-07-15

    ...: Juvenile Justice Reform and Reinvestment Initiative Stakeholder Survey Under OMB's Partnership Fund ACTION: 60 Day Notice. The Department of Justice (DOJ), Office of Justice Programs, Office of Juvenile...-3649, Office of Juvenile Justice and Delinquency Prevention, Office of Justice Programs,...

  11. 78 FR 40189 - Agency Information Collection Activities; Proposed Collection; Comments Requested: Juvenile...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-07-03

    ... of Juvenile Justice and Delinquency Prevention Agency Information Collection Activities; Proposed Collection; Comments Requested: Juvenile Residential Facility Census (Extension, Without Change, of a... Programs, Office of Juvenile Justice and Delinquency Prevention, will be submitting the...

  12. 78 FR 66383 - Agency Information Collection Activities; Proposed Collection; Comments Requested: Juvenile...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-11-05

    ... of Justice Programs Office of Juvenile Justice and Delinquency Prevention Agency Information Collection Activities; Proposed Collection; Comments Requested: Juvenile Justice Reform and Reinvestment... (DOJ), Office of Justice Programs, Office of Juvenile Justice and Delinquency Prevention, will...

  13. Neuronal synchrony: peculiarity and generality.

    PubMed

    Nowotny, Thomas; Huerta, Ramon; Rabinovich, Mikhail I

    2008-09-01

    Synchronization in neuronal systems is a new and intriguing application of dynamical systems theory. Why are neuronal systems different as a subject for synchronization? (1) Neurons in themselves are multidimensional nonlinear systems that are able to exhibit a wide variety of different activity patterns. Their "dynamical repertoire" includes regular or chaotic spiking, regular or chaotic bursting, multistability, and complex transient regimes. (2) Usually, neuronal oscillations are the result of the cooperative activity of many synaptically connected neurons (a neuronal circuit). Thus, it is necessary to consider synchronization between different neuronal circuits as well. (3) The synapses that implement the coupling between neurons are also dynamical elements and their intrinsic dynamics influences the process of synchronization or entrainment significantly. In this review we will focus on four new problems: (i) the synchronization in minimal neuronal networks with plastic synapses (synchronization with activity dependent coupling), (ii) synchronization of bursts that are generated by a group of nonsymmetrically coupled inhibitory neurons (heteroclinic synchronization), (iii) the coordination of activities of two coupled neuronal networks (partial synchronization of small composite structures), and (iv) coarse grained synchronization in larger systems (synchronization on a mesoscopic scale). PMID:19045493

  14. RNA Protein Interaction in Neurons

    PubMed Central

    Darnell, Robert B.

    2013-01-01

    Neurons have their own systems for regulating RNA. Several multigene families encode RNA binding proteins (RNABPs) that are uniquely expressed in neurons, including the well-known neuron-specific markers ELAV and NeuN, and the disease antigen NOVA. New technologies have emerged in recent years to assess the function of these proteins in vivo, and the answers are yielding insights into how and why neurons may regulate RNA in special ways—to increase cellular complexity, to spatially localize mRNA, and to regulate their expression in response to synaptic stimuli. The functions of such restricted neuronal proteins is likely to be complimented by more widely expressed RNABPs that may themselves have developed specialized functions in neurons, including Argonaute/miRNAs. Here we review what is known about such RNABPs, and explore the potential biologic and neurologic significance of neuronal RNA regulatory systems. PMID:23701460

  15. Add neurons, subtract anxiety

    PubMed Central

    Kheirbek, Mazen A.; Hen, René

    2014-01-01

    IN BRIEF To keep memories from becoming jumbled, the brain must encode the distinct features of events and situations in a way that allows them to be distinguished from one another—a process called pattern separation. Pattern separation enables us to distinguish dangerous situations from similar ones that pose no risk. People with defects in this ability may be prone to anxiety disorders. The process occurs in one of the two regions of the brain that generate neurons throughout life. These fledgling cells seem to be critical to pattern separation. Interventions that specifically boost the ranks of rookie neurons could provide new ways to regulate mood and possibly treat conditions such as post-traumatic stress disorder. PMID:24974712

  16. Single neuron modeling and data assimilation in BNST neurons

    NASA Astrophysics Data System (ADS)

    Farsian, Reza

    Neurons, although tiny in size, are vastly complicated systems, which are responsible for the most basic yet essential functions of any nervous system. Even the most simple models of single neurons are usually high dimensional, nonlinear, and contain many parameters and states which are unobservable in a typical neurophysiological experiment. One of the most fundamental problems in experimental neurophysiology is the estimation of these parameters and states, since knowing their values is essential in identification, model construction, and forward prediction of biological neurons. Common methods of parameter and state estimation do not perform well for neural models due to their high dimensionality and nonlinearity. In this dissertation, two alternative approaches for parameters and state estimation of biological neurons have been demonstrated: dynamical parameter estimation (DPE) and a Markov Chain Monte Carlo (MCMC) method. The first method uses elements of chaos control and synchronization theory for parameter and state estimation. MCMC is a statistical approach which uses a path integral formulation to evaluate a mean and an error bound for these unobserved parameters and states. These methods have been applied to biological system of neurons in Bed Nucleus of Stria Termialis neurons (BNST) of rats. State and parameters of neurons in both systems were estimated, and their value were used for recreating a realistic model and predicting the behavior of the neurons successfully. The knowledge of biological parameters can ultimately provide a better understanding of the internal dynamics of a neuron in order to build robust models of neuron networks.

  17. Comparison of the localization of tetrodotoxin between wild pufferfish Takifugu rubripes juveniles and hatchery-reared juveniles with tetrodotoxin administration.

    PubMed

    Okita, Kogen; Takatani, Tomohiro; Nakayasu, Junichi; Yamazaki, Hideki; Sakiyama, Kazutaka; Ikeda, Koichi; Arakawa, Osamu; Sakakura, Yoshitaka

    2013-09-01

    To reveal the accumulation profile of tetrodotoxin (TTX) in pufferfish Takifugu rubripes juveniles, we compared the localization of TTX in various tissues among wild juveniles and hatchery-reared juveniles with or without TTX administration using immunohistochemical technique with anti-TTX monoclonal antibody. Immuno-positive reaction was observed in hepatic tissue, basal cell of skin and olfactory, olfactory epithelium, optic nerve and brain (optic tectum, cerebellum, medulla oblongata) of wild juveniles (body length: BL, 4.7-9.4 cm). TTX was detected in the same tissues as wild juveniles and epithelial cell layer of intestine of hatchery-reared juveniles (BL, 5.0-5.3 cm) to which TTX was orally administrated. No positive reaction was observed from the tissues of hatchery-reared juveniles without TTX administration. These results suggest that orally administrated TTX to the non-toxic cultured juveniles is accumulated in the same manner of wild juveniles. In addition, our study revealed that pufferfish accumulates TTX in the central nervous system.

  18. Micropatterning neuronal networks.

    PubMed

    Hardelauf, Heike; Waide, Sarah; Sisnaiske, Julia; Jacob, Peter; Hausherr, Vanessa; Schöbel, Nicole; Janasek, Dirk; van Thriel, Christoph; West, Jonathan

    2014-07-01

    Spatially organised neuronal networks have wide reaching applications, including fundamental research, toxicology testing, pharmaceutical screening and the realisation of neuronal implant interfaces. Despite the large number of methods catalogued in the literature there remains the need to identify a method that delivers high pattern compliance, long-term stability and is widely accessible to neuroscientists. In this comparative study, aminated (polylysine/polyornithine and aminosilanes) and cytophobic (poly(ethylene glycol) (PEG) and methylated) material contrasts were evaluated. Backfilling plasma stencilled PEGylated substrates with polylysine does not produce good material contrasts, whereas polylysine patterned on methylated substrates becomes mobilised by agents in the cell culture media which results in rapid pattern decay. Aminosilanes, polylysine substitutes, are prone to hydrolysis and the chemistries prove challenging to master. Instead, the stable coupling between polylysine and PLL-g-PEG can be exploited: Microcontact printing polylysine onto a PLL-g-PEG coated glass substrate provides a simple means to produce microstructured networks of primary neurons that have superior pattern compliance during long term (>1 month) culture.

  19. Adolescent neglect, juvenile delinquency and the risk of recidivism.

    PubMed

    Ryan, Joseph P; Williams, Abigail B; Courtney, Mark E

    2013-03-01

    Victims of child abuse and neglect are at an increased risk of involvement with the juvenile justice and adult correctional systems. Yet, little is known about the continuation and trajectories of offending beyond initial contact with law enforcement. Neglect likely plays a critical role in continued offending as parental monitoring, parental rejection and family relationships are instrumental in explaining juvenile conduct problems. This study sought to determine whether neglect is associated with recidivism for moderate and high risk juvenile offenders in Washington State. Statewide risk assessments and administrative records for child welfare, juvenile justice, and adult corrections were analyzed. The sample was diverse (24 % female, 13 % African American, 8 % Hispanic, 5 % Native American) and included all moderate and high risk juvenile offenders screened by juvenile probation between 2004 and 2007 (n = 19,833). Official records from child protection were used to identify juvenile offenders with a history of child neglect and to identify juvenile offenders with an ongoing case of neglect. Event history models were developed to estimate the risk of subsequent offending. Adolescents with an ongoing case neglect were significantly more likely to continue offending as compared with youth with no official history of neglect. These findings remain even after controlling for a wide range of family, peer, academic, mental health, and substance abuse covariates. Interrupting trajectories of offending is a primary focus of juvenile justice. The findings of the current study indicate that ongoing dependency issues play a critical role in explaining the outcomes achieved for adolescents in juvenile justice settings. The implications for improved collaboration between child welfare and juvenile justice are discussed. PMID:23334336

  20. Curfew: An Answer to Juvenile Delinquency and Victimization? Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    LeBoeuf, Donni

    Many jurisdictions have implemented curfews in reaction to increased juvenile delinquency and other social trends. This bulletin explores developments in curfew ordinances, legal issues related to curfews, how community based jurisdictions have responded to these issues, and the elements of sound curfew programs as illustrated in seven…

  1. Community-Based Juvenile Reentry Services: The Effects of Service Dosage on Juvenile and Adult Recidivism

    ERIC Educational Resources Information Center

    Abrams, Laura S.; Terry, Diane; Franke, Todd M.

    2011-01-01

    In this study the authors examined the influence of length of participation in a community-based reentry program on the odds of reconviction in the juvenile and adult criminal justice systems. A structured telephone survey of reentry program alumni was conducted with 75 transition-age (18-25 year-old) young men. Binary logistic regression analysis…

  2. A Guide to Our Juvenile Delinquent System: The Family Court and the Juvenile Transgressor. [Volume II].

    ERIC Educational Resources Information Center

    Addelston, Lorraine W., Ed.

    A study of the criminal justice system in New York City led to the publication in December 1982 of "A Guide to Our Criminal Justice System." A portion of the guide dealt with the steps involved in the arrest to disposition of a juvenile. On July 1, 1983, the New York State Legislature's Act to "Recodify the Family Court Act" went into effect. The…

  3. Predictors of Support for Juvenile Sex Offender Registration: Educated Individuals Recognize the Flaws of Juvenile Registration

    ERIC Educational Resources Information Center

    Stevenson, Margaret C.; Smith, Amy C.; Sekely, Ady; Farnum, Katlyn S.

    2013-01-01

    We investigated demographic predictors of support for juvenile sex offender registration policies, including education level, gender, political orientation, and age. Participants were 168 individuals recruited from public places in a Midwest community (45% women; M age = 42). In line with hypotheses, as education level increased, support for…

  4. Juvenile Justice in Australia 2009-10. Juvenile Justice Series. Number 8

    ERIC Educational Resources Information Center

    Aalders, Rachel; Morgan, Kirsten

    2011-01-01

    In Australia, the state and territory governments are responsible for dealing with young people who are involved in crime. One major aspect of the juvenile justice system is the supervision of children and young people who have committed or are alleged to have committed an offence. This report presents information on the young people under…

  5. Consistent estimation of complete neuronal connectivity in large neuronal populations using sparse "shotgun" neuronal activity sampling.

    PubMed

    Mishchenko, Yuriy

    2016-10-01

    We investigate the properties of recently proposed "shotgun" sampling approach for the common inputs problem in the functional estimation of neuronal connectivity. We study the asymptotic correctness, the speed of convergence, and the data size requirements of such an approach. We show that the shotgun approach can be expected to allow the inference of complete connectivity matrix in large neuronal populations under some rather general conditions. However, we find that the posterior error of the shotgun connectivity estimator grows quickly with the size of unobserved neuronal populations, the square of average connectivity strength, and the square of observation sparseness. This implies that the shotgun connectivity estimation will require significantly larger amounts of neuronal activity data whenever the number of neurons in observed neuronal populations remains small. We present a numerical approach for solving the shotgun estimation problem in general settings and use it to demonstrate the shotgun connectivity inference in the examples of simulated synfire and weakly coupled cortical neuronal networks. PMID:27515518

  6. Consistent estimation of complete neuronal connectivity in large neuronal populations using sparse "shotgun" neuronal activity sampling.

    PubMed

    Mishchenko, Yuriy

    2016-10-01

    We investigate the properties of recently proposed "shotgun" sampling approach for the common inputs problem in the functional estimation of neuronal connectivity. We study the asymptotic correctness, the speed of convergence, and the data size requirements of such an approach. We show that the shotgun approach can be expected to allow the inference of complete connectivity matrix in large neuronal populations under some rather general conditions. However, we find that the posterior error of the shotgun connectivity estimator grows quickly with the size of unobserved neuronal populations, the square of average connectivity strength, and the square of observation sparseness. This implies that the shotgun connectivity estimation will require significantly larger amounts of neuronal activity data whenever the number of neurons in observed neuronal populations remains small. We present a numerical approach for solving the shotgun estimation problem in general settings and use it to demonstrate the shotgun connectivity inference in the examples of simulated synfire and weakly coupled cortical neuronal networks.

  7. Development of gonadotropin-releasing hormone (GnRH) neuron regulation in the female rat.

    PubMed

    Becú-Villalobos, D; Libertun, C

    1995-02-01

    1. After reaching its final destination the GnRH neuronal network develops under the influence of both excitatory and inhibitory inputs. 2. In the first 2 weeks of life, the immaturity of the GnRH neuronal system is reflected in sporadic unsynchronized bursts of the decapeptide, which determine the pattern of serum gonadotropin levels observed in female rats: high FSH levels and transient bursts of LH. The main inhibitory neuronal systems that operate in this period are the opioid and dopaminergic systems. A decrease in their inhibitory effectiveness may not be sufficient correctly to activate and synchronize the GnRH neuronal system. 3. There is a concomitant increase in excitatory inputs, mainly noradrenaline, excitatory amino acids, and NPY, which increase the synthesis and release of GnRH at the beginning of the juvenile period and participate in the coupling of GnRH neural activity to the ongoing rhythmic activity of a hypothalamic circadian oscillator. 4. The morphological changes of GnRH neurons which take place during the third and fourth weeks of life, and which are probably related to increasing estradiol levels, reflects the increasing complexity of the GnRH neuronal network, which establishes synaptic contacts to enable the expression of pulsatility and of the positive feedback of estradiol, both necessary components for the occurrence of puberty.

  8. [Explanation and forecast: relapse of juvenile offenders].

    PubMed

    Giebel, S M

    2006-01-01

    On the basis of n=82 juvenile offenders from a prison for juvenile offenders in Rheinland Pfalz the model of the logistic regression is compared with a procedure from the family of the neural nets in its efficiency to explain and predict "relapse" in form of a renewed imprisonment or prosecution /police search after dismissal. The group which can be examined is limited by the population of the prison for juvenile offenders and the explaining variables for "relapse" as "addicted to drugs" present non-metric scaling. For the explanation only probabilities for "relapse" can be indicated in this connection. By means of this probability it is possible to classify the individual case. The forecast is simulated by coincidental dividing of the data: the first part of the data is used for the explanation, the second for the forecast. With the comparison of the logistic regression with the neural nets, the superiority of neural nets in the explanation of "relapse" can be shown, since the neural nets are able to consider dependence between the explaining variables and according to that they offer a differentiated explanation. Their efficiency to predict "relapse" depends on the comparability of the distribution in the two coincidentally provided samples, the training data record for determining the explanation and the test case for the use of the explanation regarding the forecast. For optimal explanation and forecast neural nets are to be preferred to the logistic regression, since in the model with the better explanation also includes the potential for a usable better forecast. Moreover the model of the logistic regression is in fact a special case of the neural net, with a reduced complexity of the net.

  9. Colour preferences of juvenile turbot (Scophthalmus maximus).

    PubMed

    Li, Xian; Chi, Liang; Tian, Huiqin; Meng, Lingjie; Zheng, Jimeng; Gao, Xiaolong; Liu, Ying

    2016-03-15

    The background colour of aquaculture tanks is normally chosen based on practical experience and/or observations of fish behaviour and the growth rates achieved. However, some farmed species, including turbot, are sentient and can show a preference for a particular environment. In the current study, a self-referent colour preference device was developed and the self-referent colour preference of farmed fish investigated. In experiment 1, the background colour preference of juvenile turbot cultured under a grey background for >3months post-incubation was evaluated. Based on these results, in experiment 2, juvenile turbot were adapted to blue, pink, white, or black backgrounds for 50days and their preferences established. Meanwhile, the growth rates, feed intake, and metabolic rates (including oxygen consumption rate, and ammonia excretion rate) of the turbot were evaluated. The results showed that turbot farmed under a grey background, or after long-term white, blue, pink and black colour adaptation, always displayed a preference for a white background and a dislike for black, red, or brown backgrounds, although their body colour was greyish. Long-term adaptation influenced the frequency of juveniles selecting white, black, pink or blue backgrounds. They showed the highest growth rate, feed intake, and metabolic rates under blue and white backgrounds, and the lowest under a black background in accordance with their preferences shown in experiment 1. Although it is unclear how turbot determine their self-referent colour preferences over such a short period of time, these results indicate that dark colours are unsuitable for the aquaculture of turbot culture in terms of the welfare of the fish. PMID:26792527

  10. Parvalbumin+ Neurons and Npas1+ Neurons Are Distinct Neuron Classes in the Mouse External Globus Pallidus

    PubMed Central

    Hernández, Vivian M.; Hegeman, Daniel J.; Cui, Qiaoling; Kelver, Daniel A.; Fiske, Michael P.; Glajch, Kelly E.; Pitt, Jason E.; Huang, Tina Y.; Justice, Nicholas J.

    2015-01-01

    Compelling evidence suggests that pathological activity of the external globus pallidus (GPe), a nucleus in the basal ganglia, contributes to the motor symptoms of a variety of movement disorders such as Parkinson's disease. Recent studies have challenged the idea that the GPe comprises a single, homogenous population of neurons that serves as a simple relay in the indirect pathway. However, we still lack a full understanding of the diversity of the neurons that make up the GPe. Specifically, a more precise classification scheme is needed to better describe the fundamental biology and function of different GPe neuron classes. To this end, we generated a novel multicistronic BAC (bacterial artificial chromosome) transgenic mouse line under the regulatory elements of the Npas1 gene. Using a combinatorial transgenic and immunohistochemical approach, we discovered that parvalbumin-expressing neurons and Npas1-expressing neurons in the GPe represent two nonoverlapping cell classes, amounting to 55% and 27% of the total GPe neuron population, respectively. These two genetically identified cell classes projected primarily to the subthalamic nucleus and to the striatum, respectively. Additionally, parvalbumin-expressing neurons and Npas1-expressing neurons were distinct in their autonomous and driven firing characteristics, their expression of intrinsic ion conductances, and their responsiveness to chronic 6-hydroxydopamine lesion. In summary, our data argue that parvalbumin-expressing neurons and Npas1-expressing neurons are two distinct functional classes of GPe neurons. This work revises our understanding of the GPe, and provides the foundation for future studies of its function and dysfunction. SIGNIFICANCE STATEMENT Until recently, the heterogeneity of the constituent neurons within the external globus pallidus (GPe) was not fully appreciated. We addressed this knowledge gap by discovering two principal GPe neuron classes, which were identified by their nonoverlapping

  11. Metabolic reprogramming during neuronal differentiation.

    PubMed

    Agostini, M; Romeo, F; Inoue, S; Niklison-Chirou, M V; Elia, A J; Dinsdale, D; Morone, N; Knight, R A; Mak, T W; Melino, G

    2016-09-01

    Newly generated neurons pass through a series of well-defined developmental stages, which allow them to integrate into existing neuronal circuits. After exit from the cell cycle, postmitotic neurons undergo neuronal migration, axonal elongation, axon pruning, dendrite morphogenesis and synaptic maturation and plasticity. Lack of a global metabolic analysis during early cortical neuronal development led us to explore the role of cellular metabolism and mitochondrial biology during ex vivo differentiation of primary cortical neurons. Unexpectedly, we observed a huge increase in mitochondrial biogenesis. Changes in mitochondrial mass, morphology and function were correlated with the upregulation of the master regulators of mitochondrial biogenesis, TFAM and PGC-1α. Concomitant with mitochondrial biogenesis, we observed an increase in glucose metabolism during neuronal differentiation, which was linked to an increase in glucose uptake and enhanced GLUT3 mRNA expression and platelet isoform of phosphofructokinase 1 (PFKp) protein expression. In addition, glutamate-glutamine metabolism was also increased during the differentiation of cortical neurons. We identified PI3K-Akt-mTOR signalling as a critical regulator role of energy metabolism in neurons. Selective pharmacological inhibition of these metabolic pathways indicate existence of metabolic checkpoint that need to be satisfied in order to allow neuronal differentiation. PMID:27058317

  12. Metabolic reprogramming during neuronal differentiation

    PubMed Central

    Agostini, M; Romeo, F; Inoue, S; Niklison-Chirou, M V; Elia, A J; Dinsdale, D; Morone, N; Knight, R A; Mak, T W; Melino, G

    2016-01-01

    Newly generated neurons pass through a series of well-defined developmental stages, which allow them to integrate into existing neuronal circuits. After exit from the cell cycle, postmitotic neurons undergo neuronal migration, axonal elongation, axon pruning, dendrite morphogenesis and synaptic maturation and plasticity. Lack of a global metabolic analysis during early cortical neuronal development led us to explore the role of cellular metabolism and mitochondrial biology during ex vivo differentiation of primary cortical neurons. Unexpectedly, we observed a huge increase in mitochondrial biogenesis. Changes in mitochondrial mass, morphology and function were correlated with the upregulation of the master regulators of mitochondrial biogenesis, TFAM and PGC-1α. Concomitant with mitochondrial biogenesis, we observed an increase in glucose metabolism during neuronal differentiation, which was linked to an increase in glucose uptake and enhanced GLUT3 mRNA expression and platelet isoform of phosphofructokinase 1 (PFKp) protein expression. In addition, glutamate–glutamine metabolism was also increased during the differentiation of cortical neurons. We identified PI3K–Akt–mTOR signalling as a critical regulator role of energy metabolism in neurons. Selective pharmacological inhibition of these metabolic pathways indicate existence of metabolic checkpoint that need to be satisfied in order to allow neuronal differentiation. PMID:27058317

  13. Juvenile hyaline fibromatosis: a case report.

    PubMed

    Karaçal, Naci; Gülçelik, Nevzat; Yildiz, Kadriye; Mungan, Sevdegül; Kutlu, Necmettin

    2005-07-01

    Juvenile hyaline fibromatosis ( JHF ) is a rare autosomal recessive disease characterized by papulonodular skin lesions, gingival hyperplasia, joint contractures, and bone lesions. The skin lesions may consist of multiple large tumors, commonly on the scalp and around the neck, and small pearly, pink papules and plaques on the trunk, chin, ears, and around the nostrils. Here, we report a 2-year-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hyperplasia. The lesions were totally excised and clinicopathological diagnosis was JHF.

  14. Pneumatosis Intestinalis Associated with Juvenile Dermatomyositis

    PubMed Central

    Miyamae, Takako; Ishiguro, Naoko; Yonezawa, Maria; Tokushige, Katsutoshi; Yamanaka, Hisashi

    2016-01-01

    We herein report a case of pneumatosis intestinalis (PI), a condition characterized by the presence of gas within the wall of the digestive tract, associated with juvenile dermatomyositis (JDM). A 16-year-old girl, diagnosed with JDM at the age of 10, presented with abdominal pain and distention. She developed PI based on radiological findings that also included a dilated large intestine, extraluminal gas, and secondary diaphragmatic elevation. She was observed with medical therapy including bowel rest and hyperbaric oxygen therapy. However, she ultimately developed a strangulated obstruction 5 years after presentation with PI and large intestine resection and colostomy were performed emergently. PMID:27242944

  15. Habitat Suitability Index Models: Juvenile English sole

    USGS Publications Warehouse

    Toole, Christopher L.; Barnhart, Roger A.; Onuf, Christopher P.

    1987-01-01

    English sole (Parophrys vetulus) is one of the major commercial groundfish species caught along the Pacific coast. Landings in the United States and Canada averaged 4,947 t/yr between 1975 and 1984, placing it third in importance among flatfish caught by Pacific coast trawlers (Pacific Marine Fisheries Commission 1985). Juvenile English sole are also among the most abundant fishes in many bays and estuaries along the Pacific (Westrheim 1955; Sopher 1974; Ambrose 1976; Rogers 1985). The English sole is not an important recreational species.

  16. Decreased fibrinolytic activity in juvenile chronic arthritis.

    PubMed Central

    Mussoni, L; Pintucci, G; Romano, G; De Benedetti, F; Massa, M; Martini, A

    1990-01-01

    The basal fibrinolytic activity in 17 children with active juvenile chronic arthritis (JCA) was investigated. It was found that patients with JCA, and particularly those with the systemic form, show decreased plasma fibrinolytic activity and a marked increase in plasminogen activator inhibitor. Additionally, it was found that patients with systemic JCA, but not those with the polyarticular or pauciarticular form, have increased circulating levels of tissue-type plasminogen activator, and endothelial cell protein, suggesting possible endothelial cell participation in systemic JCA. PMID:2125408

  17. Pneumatosis Intestinalis Associated with Juvenile Dermatomyositis.

    PubMed

    Miyamae, Takako; Ishiguro, Naoko; Yonezawa, Maria; Tokushige, Katsutoshi; Yamanaka, Hisashi

    2016-01-01

    We herein report a case of pneumatosis intestinalis (PI), a condition characterized by the presence of gas within the wall of the digestive tract, associated with juvenile dermatomyositis (JDM). A 16-year-old girl, diagnosed with JDM at the age of 10, presented with abdominal pain and distention. She developed PI based on radiological findings that also included a dilated large intestine, extraluminal gas, and secondary diaphragmatic elevation. She was observed with medical therapy including bowel rest and hyperbaric oxygen therapy. However, she ultimately developed a strangulated obstruction 5 years after presentation with PI and large intestine resection and colostomy were performed emergently. PMID:27242944

  18. Surgical experience with juvenile nasopharyngeal angiofibroma.

    PubMed

    Maharaj, D; Fernandes, C M

    1989-04-01

    Our experience with 18 cases of juvenile nasopharyngeal angiofibroma (JNA) over a period of 9 years is discussed. All cases were managed surgically: 17 via a transpalatal approach and one case by a combined transpalatal, lateral rhinotomy, and transantral approach. In this series there were two recurrences following primary surgical procedures and these required a second procedure. There were no major complications and all 18 patients are alive. We believe that surgical excision should be the treatment of choice for JNA and that radiotherapy should be used adjunctively for cases wherein intracranial extension of the tumors prevents total excision.

  19. Idiopathic Juvenile Osteoporosis: A Case Report

    PubMed Central

    Tosun, Gül; Şen, Yaşar

    2015-01-01

    Idiopathic Juvenile Osteoporosis (IJO) is a very rare disease, self restrictive and shows marked, spontaneous improvement during adolescence. The major clinical features were pain with difficulty walking, growth retardation, oral and dental abnormalities with radiographically porous bone structure. A 13-year-old male referred to paediatric dentistry clinic for toothache. The observations made with extra-intraoral clinic examination that one revealed short and skinny stature, diffuse caries in deciduous teeth, abraded lower incisor, deep bite and dysmorphic appearance in permanent incisor. This report emphasizes the recognized features of IJO as well as describes facio-dental findings that could aid in the diagnosis and management of these patients. PMID:26436063

  20. Empirically Based Strategies for Preventing Juvenile Delinquency.

    PubMed

    Pardini, Dustin

    2016-04-01

    Juvenile crime is a serious public health problem that results in significant emotional and financial costs for victims and society. Using etiologic models as a guide, multiple interventions have been developed to target risk factors thought to perpetuate the emergence and persistence of delinquent behavior. Evidence suggests that the most effective interventions tend to have well-defined treatment protocols, focus on therapeutic approaches as opposed to external control techniques, and use multimodal cognitive-behavioral treatment strategies. Moving forward, there is a need to develop effective policies and procedures that promote the widespread adoption of evidence-based delinquency prevention practices across multiple settings. PMID:26980128

  1. Hypotrichosis with juvenile macular dystrophy: Portuguese case.

    PubMed

    Elfatoiki, Fatima Zahra; Cordoliani, Florance; Pascal Regane, Pascal; Afforitit-Demoge, Aude

    2016-01-01

    Hypotrichosis with juvenile macular dystrophy is a rare congenital disease mainly found in the Druze population of Northern Israel. This disorder is caused by the CDH3 mutation encoding P-cadherin, which is expressed in retinal pigment epithelium and hair follicles. An 11-year-old girl who was born to related Portuguese parents, had hypotrichosis since birth and macular dystrophy diagnosed at age 5. Fundus examination and fluorescein angiography revealed located macular pigmentary abnormalities. No molecular analysis was done. A fundus examination should be considered mandatory in the assessment of congenital hypotrichosis. PMID:27617529

  2. Gold nephropathy in juvenile rheumatoid arthritis.

    PubMed

    Husserl, F E; Shuler, S E

    1979-01-01

    A 2-year-old girl was treated with gold salts for juvenile rheumatoid arthritis. Treatment had to be discontinued when persistent proteinuria was detected. As this case report indicates, close monitoring of the urine is mandatory during treatment with gold salts to detect early signs of toxicity: hematuria followed by casts and then proteinuria as therapy is continued. Histologic examination with electron microscopy will help to differentiate the different forms of gold toxicity. When the findings are consistent with gold-induced renal involvement, therapy should be discontinued. The gold nephropathy usually resolves in time, with no permanent renal damage.

  3. Endosulfan affects GnRH cells in sexually differentiated juveniles of the perciform Cichlasoma dimerus.

    PubMed

    Piazza, Yanina; Pandolfi, Matías; Da Cuña, Rodrigo; Genovese, Griselda; Lo Nostro, Fabiana

    2015-06-01

    Endosulfan (ES) is an organochlorine pesticide widely used in agriculture despite its high toxicity towards non-target organisms such as fish. It has been demonstrated that ES can cause negative effects on aquatic animals, including disruption of hormonal systems. However, the alterations produced by this pesticide on the reproductive axis of fish prior to sexual maturity, as well as possible modes of action have hardly been studied. This study aimed at assessing the effect of waterborne exposure to the pesticide ES on the reproductive axis during sexual differentiation of juveniles of the South American freshwater cichlid fish Cichlasoma dimerus. No mortality was observed due to ES subchronic exposure (90 days post-fertilization). Exposure to ES did not affect body weight nor morphometric parameters, indicating that larvae nutritional state was not affected. Timing of sexual differentiation, gonadal morphology and sex ratio were likewise not altered by ES. However, ES acted as an endocrine disrupting chemical in this species as the morphometry of gonadotropin-releasing hormones (GnRH) producing cells was altered. Exposure to ES altered nuclear area, cell area and nucleus/cytoplasm ratio of GnRH II neurons, and cell and nuclear area and diameter of GnRH III neurons. Interestingly, in our previous study, exposure before sex differentiation (30 day exposure) caused no alteration to GnRH II and III, and did alter GnRH I and FSH cells. These alterations could lead to changes in circulating hormone levels, especially when fish are exposed for prolonged periods, ultimately impairing reproductive fitness. C. dimerus juveniles can be an interesting biological model to perform toxicological studies with the intent to assess early disruption endpoints in the reproductive axis during development. PMID:25800987

  4. Endosulfan affects GnRH cells in sexually differentiated juveniles of the perciform Cichlasoma dimerus.

    PubMed

    Piazza, Yanina; Pandolfi, Matías; Da Cuña, Rodrigo; Genovese, Griselda; Lo Nostro, Fabiana

    2015-06-01

    Endosulfan (ES) is an organochlorine pesticide widely used in agriculture despite its high toxicity towards non-target organisms such as fish. It has been demonstrated that ES can cause negative effects on aquatic animals, including disruption of hormonal systems. However, the alterations produced by this pesticide on the reproductive axis of fish prior to sexual maturity, as well as possible modes of action have hardly been studied. This study aimed at assessing the effect of waterborne exposure to the pesticide ES on the reproductive axis during sexual differentiation of juveniles of the South American freshwater cichlid fish Cichlasoma dimerus. No mortality was observed due to ES subchronic exposure (90 days post-fertilization). Exposure to ES did not affect body weight nor morphometric parameters, indicating that larvae nutritional state was not affected. Timing of sexual differentiation, gonadal morphology and sex ratio were likewise not altered by ES. However, ES acted as an endocrine disrupting chemical in this species as the morphometry of gonadotropin-releasing hormones (GnRH) producing cells was altered. Exposure to ES altered nuclear area, cell area and nucleus/cytoplasm ratio of GnRH II neurons, and cell and nuclear area and diameter of GnRH III neurons. Interestingly, in our previous study, exposure before sex differentiation (30 day exposure) caused no alteration to GnRH II and III, and did alter GnRH I and FSH cells. These alterations could lead to changes in circulating hormone levels, especially when fish are exposed for prolonged periods, ultimately impairing reproductive fitness. C. dimerus juveniles can be an interesting biological model to perform toxicological studies with the intent to assess early disruption endpoints in the reproductive axis during development.

  5. The Challenges in Providing Needed Transition Programming to Juvenile Offenders

    ERIC Educational Resources Information Center

    Platt, John S.; Bohac, Paul D.; Wade, Wanda

    2015-01-01

    The transition to and from juvenile justice settings is a complex and challenging process. Effectively preparing juvenile justice personnel to address the transition needs of incarcerated students is an essential aspect of reducing the negative effects of the school-to-prison pipeline. This article examines program and professional development…

  6. A Protocol for Reducing Juvenile Recidivism through Relapse Prevention.

    ERIC Educational Resources Information Center

    Roget, Nancy A.; Fisher, Gary L.; Johnson, Michael L.

    1998-01-01

    The connection between alcohol and other drug use and juvenile crime activities is well established. The source of this connection and specific interventions with juvenile offenders involved in alcohol and drug use is unclear. Reviews existing literature and presents an adolescent-specific relapse-prevention protocol that provides a structure for…

  7. The YouthARTS Development Project. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Clawson, Heather J.; Coolbaugh, Kathleen

    The arts enrich the culture and individual lives immeasurably, but what impact do arts-based programs have in preventing juvenile delinquency? To address this question, the YouthARTS Development Project, with the technical assistance of the Office of Juvenile Justice and Delinquency Prevention (OJJDP), brought together Federal agencies, national…

  8. 28 CFR 115.114 - Juveniles and youthful detainees.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 28 Judicial Administration 2 2014-07-01 2014-07-01 false Juveniles and youthful detainees. 115.114 Section 115.114 Judicial Administration DEPARTMENT OF JUSTICE (CONTINUED) PRISON RAPE ELIMINATION ACT NATIONAL STANDARDS Standards for Lockups Prevention Planning § 115.114 Juveniles and youthful...

  9. 28 CFR 115.114 - Juveniles and youthful detainees.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 28 Judicial Administration 2 2013-07-01 2013-07-01 false Juveniles and youthful detainees. 115.114 Section 115.114 Judicial Administration DEPARTMENT OF JUSTICE (CONTINUED) PRISON RAPE ELIMINATION ACT NATIONAL STANDARDS Standards for Lockups Prevention Planning § 115.114 Juveniles and youthful...

  10. 28 CFR 115.114 - Juveniles and youthful detainees.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 28 Judicial Administration 2 2012-07-01 2012-07-01 false Juveniles and youthful detainees. 115.114 Section 115.114 Judicial Administration DEPARTMENT OF JUSTICE (CONTINUED) PRISON RAPE ELIMINATION ACT NATIONAL STANDARDS Standards for Lockups Prevention Planning § 115.114 Juveniles and youthful...

  11. Chorioretinitis as a complication of pauciarticular juvenile rheumatoid arthritis.

    PubMed

    Thacker, Neepa M; Demer, Joseph L

    2005-01-01

    A girl with pauciarticular juvenile rheumatoid arthritis developed bilateral uveitis complicated by cataract and glaucoma. Sequential fundus photography documented development of extensive choroidal scarring and retinal pigment epithelial atrophy in the left macula. Vision was not impaired. This case suggests uveitis in juvenile rheumatoid arthritis can be associated with chorioretinitis.

  12. IDEA-Related Professional Development in Juvenile Corrections Schools

    ERIC Educational Resources Information Center

    Gagnon, Joseph Calvin; Steinberg, Mary Anne; Crockett, Jean; Murphy, Kristin M.; Gaddis, Justin

    2013-01-01

    Incarcerated youth are among the least academically and behaviorally competent students in the United States. In spite of juvenile justice reform efforts, including state and federal guarantees of appropriate education, educational services in juvenile corrections (JC) schools, especially for youth with disabilities, are lacking (Houchins,…

  13. Suicidal Ideation and Behaviors among Youths in Juvenile Detention

    ERIC Educational Resources Information Center

    Abram, Karen M.; Choe, Jeanne Y.; Washburn, Jason J.; Teplin, Linda A.; King, Devon C.; Dulcan, Mina K.

    2008-01-01

    An epidemiological study used data from the Northwestern Juvenile Project to examine the association between psychiatric diagnosis and suicide risk among newly detained youths in the US juvenile system. Results concluded that psychiatric disorders were associated with suicide attempts, and females were found at a higher risk than males.

  14. Manual of Standards for Juvenile Training Schools and Services.

    ERIC Educational Resources Information Center

    Commission on Accreditation for Corrections, Rockville, MD.

    This manual of standards for juvenile training schools and services contains 487 American Correctional Association standards for the accreditation of juvenile training schools (youth development centers, villages, correction centers, treatment centers, service centers, homes for boys and girls, camps, and ranches). Standards presented are…

  15. 28 CFR 0.57 - Criminal prosecutions against juveniles.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 28 Judicial Administration 1 2013-07-01 2013-07-01 false Criminal prosecutions against juveniles. 0.57 Section 0.57 Judicial Administration DEPARTMENT OF JUSTICE ORGANIZATION OF THE DEPARTMENT OF JUSTICE Criminal Division § 0.57 Criminal prosecutions against juveniles. The Assistant Attorney...

  16. Juvenile Probation Initiatives in California and Their Effects. Research Brief

    ERIC Educational Resources Information Center

    Turner, Susan; Fain, Terry

    2005-01-01

    Over the past ten years, probation departments across the state of California have undertaken five major initiatives aimed at juvenile offenders and at-risk youths. Although these initiatives were concomitant with reductions in juvenile arrests and other positive outcomes, we cannot definitively attribute such observed statewide trends to these…

  17. Trends in State Correction: Juveniles and the Violent Young Offender.

    ERIC Educational Resources Information Center

    Vinter, Robert D.

    1979-01-01

    Correlational analysis is used to examine associations between the 50 states' rates of juvenile and adult institutionalization and levels of four crime categories (total index, violent, property, and burglary). No associations are found between states' crime and juvenile institutionalization rates. Associations are found between states' crime and…

  18. Juveniles in Adult Prisons and Jails: A National Assessment. Monograph.

    ERIC Educational Resources Information Center

    Austin, James; Johnson, Kelly Dedel; Gregoriou, Maria

    The number of youth under 18 confined to adult prisons has more than doubled in the past decade. A nationwide study of juveniles in adult correctional facilities was undertaken to help policymakers form an effective response to this situation. The study determined the extent of juvenile confinement in facilities around the country. It also…

  19. Social Work and Juvenile Probation: Historical Tensions and Contemporary Convergences

    ERIC Educational Resources Information Center

    Peters, Clark M.

    2011-01-01

    Social work's weak presence in the field of corrections is peculiar, given that those involved in the criminal and juvenile justice systems are undeniably among the vulnerable and oppressed populations that the profession has traditionally served. The field of juvenile probation shares roots with the profession of social work but lacks a strong…

  20. Prosecution in the Juvenile Courts: Guidelines for the Future.

    ERIC Educational Resources Information Center

    Finkelstein, M. Marvin; And Others

    This monograph analyses the functions of the prosecutor in the juvenile system. The authors examined in detail the existing prosecution system in the Boston Juvenile Court and surveyed procedures in a number of other cities. The findings show a wide disparity in practice and in the quality of justice dispensed. For example, in the Boston sample…

  1. Family and Community Perceptions of Quality in Juvenile Justice Programs

    ERIC Educational Resources Information Center

    Selber, Katherine; Streeter, Calvin

    2004-01-01

    The conceptualization and empirical assessment of service quality in juvenile justice remains limited. There are few reports on programmatic attempts to assess satisfaction in juvenile justice programs or attempts to include what constitutes quality of service from multiple customer perspectives. This article describes a potential model, the Gap…

  2. Current Juvenile Corrections Professional Development Practices and Future Directions

    ERIC Educational Resources Information Center

    Gagnon, Joseph C.; Houchins, David E.; Murphy, Kristin M.

    2012-01-01

    Personnel in juvenile corrections (JC) work with students who have challenging academic, behavioral, and mental health needs. The complexity of the JC setting requires personnel to be highly skilled in effective practices to meet the demands of their job. Unfortunately, juvenile correctional personnel are neglected as an important link in the…

  3. Developing an AIDS Program in a Juvenile Detention Center.

    ERIC Educational Resources Information Center

    Gelber, Seymour

    1988-01-01

    Examines what is being done and what more must be done in terms of AIDS (Acquired Immune Deficiency Syndrome) testing, screening, counseling. Discusses education about AIDS for young people in juvenile detention centers, penal institutions, and residential rehabilitation programs. Dade County Juvenile Detention Center (Florida) exemplifies…

  4. Dating Violence and Girls in the Juvenile Justice System

    ERIC Educational Resources Information Center

    Kelly, Patricia J.; Cheng, An-Lin; Peralez-Dieckmann, Esther; Martinez, Elisabeth

    2009-01-01

    The purpose of this study is to explore the prevalence and associated behaviors of dating violence among a population of girls in the juvenile justice system. A sample of 590 girls from an urban juvenile justice system completed a questionnaire assessing attitudes and self-efficacy about and occurrence of dating violence. The analysis developed a…

  5. 8 CFR 236.3 - Detention and release of juveniles.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... parent-child relationship and/or the juvenile's rights and interests are adverse with those of the parent... parent; (ii) Legal guardian; or (iii) An adult relative (brother, sister, aunt, uncle, grandparent) who... to ensure the juvenile's safety or that of others. In cases where the parent, legal guardian,...

  6. 8 CFR 236.3 - Detention and release of juveniles.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... parent-child relationship and/or the juvenile's rights and interests are adverse with those of the parent... parent; (ii) Legal guardian; or (iii) An adult relative (brother, sister, aunt, uncle, grandparent) who... to ensure the juvenile's safety or that of others. In cases where the parent, legal guardian,...

  7. 8 CFR 236.3 - Detention and release of juveniles.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... parent-child relationship and/or the juvenile's rights and interests are adverse with those of the parent... parent; (ii) Legal guardian; or (iii) An adult relative (brother, sister, aunt, uncle, grandparent) who... to ensure the juvenile's safety or that of others. In cases where the parent, legal guardian,...

  8. 8 CFR 236.3 - Detention and release of juveniles.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... parent-child relationship and/or the juvenile's rights and interests are adverse with those of the parent... parent; (ii) Legal guardian; or (iii) An adult relative (brother, sister, aunt, uncle, grandparent) who... to ensure the juvenile's safety or that of others. In cases where the parent, legal guardian,...

  9. 8 CFR 236.3 - Detention and release of juveniles.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... parent-child relationship and/or the juvenile's rights and interests are adverse with those of the parent... parent; (ii) Legal guardian; or (iii) An adult relative (brother, sister, aunt, uncle, grandparent) who... to ensure the juvenile's safety or that of others. In cases where the parent, legal guardian,...

  10. Reintegration, Supervised Release, and Intensive Aftercare. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Altschuler, David M.; Armstrong, Troy L.; MacKenzie, Doris Layton

    Interest in the issue of aftercare for juvenile offenders continues to grow. Jurisdictions seek new ways to reintegrate youth being released from confinement back into their communities, while also ensuring for public safety. Juvenile justice policymakers and professionals are experimenting with aftercare and other reintegration models. After…

  11. Juvenile Crime and Criminal Justice: Resolving Border Disputes

    ERIC Educational Resources Information Center

    Fagan, Jeffrey

    2008-01-01

    Rising juvenile crime rates during the 1970s and 1980s spurred state legislatures across the country to exclude or transfer a significant share of offenders under the age of eighteen to the jurisdiction of the criminal court, essentially redrawing the boundary between the juvenile and adult justice systems. Jeffrey Fagan examines the legal…

  12. Effects of Juvenile Court Exposure on Crime in Young Adulthood

    ERIC Educational Resources Information Center

    Petitclerc, Amelie; Gatti, Uberto; Vitaro, Frank; Tremblay, Richard E.

    2013-01-01

    Background: The juvenile justice system's interventions are expected to help reduce recidivism. However, previous studies suggest that official processing in juvenile court fails to reduce adolescents' criminal behavior in the following year. Longer term effects have not yet been investigated with a rigorous method. This study used…

  13. Predictors of Sexual Aggression among Male Juvenile Offenders

    ERIC Educational Resources Information Center

    Yeater, Elizabeth A.; Lenberg, Kathryn L.; Bryan, Angela D.

    2012-01-01

    The purpose of this study was to conduct a longitudinal examination of predictors of sexual aggression among male juvenile offenders. Four hundred and four adolescent males between the ages of 14 and 17 years were recruited from juvenile probation offices to take part in a prospective study of substance use and sexual risk. At baseline,…

  14. Kids and Guns. 1999 National Report Series. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Department of Justice, Washington, DC. Office of Juvenile Justice and Delinquency Prevention.

    This report provides information about the use of guns by children and adolescents, with related information on juvenile homicides and suicides. The annual number of juveniles killed with a firearm increased substantially between 1987 and 1993 as occurrences of other types of homicide remained constant. Since 1980, one in four murders of juveniles…

  15. Trauma and Juvenile Delinquency: Theory, Research, and Interventions.

    ERIC Educational Resources Information Center

    Greenwald, Ricky, Ed.

    This book addresses the connection between childhood trauma and juvenile delinquency. It includes theoretical models of this relationship and examinations of its most important aspects, explorations of trauma-related assessment issues, and practical therapeutic interventions for use with juvenile delinquents. Chapters include: (1) "The Role of…

  16. The Juvenile Addiction Risk Rating: Development and Initial Psychometrics

    ERIC Educational Resources Information Center

    Powell, Michael; Newgent, Rebecca A.

    2016-01-01

    This article describes the development and psychometrics of the Juvenile Addiction Risk Rating. The Juvenile Addiction Risk Rating is a brief screening of addiction potential based on 10 risk factors predictive of youth alcohol and drug-related problems that assists examiners in more accurate treatment planning when self-report information is…

  17. The Content Validity of Juvenile Psychopathy: An Empirical Examination

    ERIC Educational Resources Information Center

    Lynam, Donald R.; Derefinko, Karen J.; Caspi, Avshalom; Loeber, Rolf; Stouthamer-Loeber, Magda

    2007-01-01

    This study examined the content validity of a juvenile psychopathy measure, the Childhood Psychopathy Scale (CPS; D. R. Lynam, 1997), based on a downward translation of an adult instrument, the Hare Psychopathy Checklist-Revised (PCL-R; R. D. Hare, 1991). The CPS was compared with two other indices of juvenile psychopathy: (a) an index derived…

  18. Use of the Adolescent SASSI in a Juvenile Correctional Setting

    ERIC Educational Resources Information Center

    Stein, L. A. R.; Lebeau-Craven, Rebecca; Martin, Rosemarie; Colby, Suzanne M.; Barnett, Nancy P.; Golembeske, Charles, Jr.; Penn, Joseph V.

    2005-01-01

    The Substance Abuse Subtle Screening Inventory-Adolescent (SASSI-A) is used in evaluation and treatment planning for incarcerated juveniles. Validity of the SASSI-A in a juvenile correctional facility was examined using archival data. Findings generally support the validity of SASSI-A substance use scales. However, there is concern regarding the…

  19. Assessing Juvenile Sex Offenders to Determine Adequate Levels of Supervision.

    ERIC Educational Resources Information Center

    Gerdes, Karen E.; And Others

    1995-01-01

    This study analyzed the internal consistency of four inventories used by Utah probation officers to determine adequate and efficacious supervision levels and placement for juvenile sex offenders. Three factors accounted for 41.2 percent of variance (custodian's and juvenile's attitude toward intervention, offense characteristics, and historical…

  20. The Community Assessment Center Concept. Juvenile Justice Bulletin.

    ERIC Educational Resources Information Center

    Oldenettel, Debra; Wordes, Madeline

    This bulletin is intended to inform juvenile justice practitioners and other youth service providers about the work of the Office of Juvenile Justice and Delinquency Prevention (OJJDP) in developing and demonstrating a Community Assessment Center (CAC) model, and to increase awareness about some of the challenges associated with its…

  1. Female Juvenile Offending: A Review of Characteristics and Contexts

    ERIC Educational Resources Information Center

    Mullis, Ronald L.; Cornille, Thomas A.; Mullis, Ann K.; Huber, Jessica

    2004-01-01

    We reviewed current literature on female juvenile offending including the scope and nature of offending by female adolescents and the risk and protective factors across ecological contexts. We suggested that female juvenile offending is best viewed within a multidimensional framework in which female adolescent developmental characteristics as well…

  2. Resiliency, Risk, and Substance Use among Hispanic Urban Juvenile Detainees.

    ERIC Educational Resources Information Center

    Taylor, Elizabeth R.; Karcher, Michael J.; Kelly, Patricia J.; Valescu, Smaranda

    2003-01-01

    A study of resiliency was conducted among 236 urban juvenile detainees. Findings reveal that resiliency processes related differently to risk and protective factors, differed among ethnic groups, and varied by age and gender among juvenile detainees. (Contains 48 references and 4 tables.) (Author)

  3. Psychiatric Disorders of Youth in Detention. Juvenile Justice Bulletin

    ERIC Educational Resources Information Center

    Teplin, Linda A.; Abram, Karen M.; McClelland, Gary M.; Mericle, Amy A.; Dulcan, Mina K.; Washburn, Jason J.

    2006-01-01

    This bulletin examines the prevalence of alcohol, drug, and mental disorders among youth at the Cook County (Illinois) Juvenile Temporary Detention Center, by gender, race/ethnicity, and age. Drawing on research conducted by the Northwestern Juvenile Project, this bulletin finds that nearly two-thirds of males and three-quarters of females studied…

  4. Report to Congress on Juvenile Violence Research. OJJDP Report.

    ERIC Educational Resources Information Center

    Office of Juvenile Justice and Delinquent Prevention (Dept. of Justice), Washington, DC.

    This report presents the collective results of studies funded under the Office of Juvenile Justice and Delinquency Prevention congressional directive. The studies confirm that young African-American males are disproportionately involved as offenders and victims of violence, that firearms play a large role in juvenile violence, and that gang…

  5. Police Role in Removing Juveniles from Adult Jails.

    ERIC Educational Resources Information Center

    Illinois Univ., Champaign. Community Research Center.

    The detention of juveniles in adult jails and lockups has long been a moral issue characterized by sporadic public concern and minimal action towards its resolution. Approximately 500,000 or more juveniles are held in jails each year. Rationales for keeping children in jails have included public safety, protection of children from themselves or…

  6. Juvenile Justice Reform: State Experiences. Criminal Justice Paper #4.

    ERIC Educational Resources Information Center

    Pierce, Robert; Yondorf, Barbara

    Community-based programs in the juvenile justice system present a promising alternative to the disappointing results achieved by large institutional facilities. A diverse group of states has found that intensive, individualized services provided in small, family-like residential settings or in the juvenile's own home yield comparable or reduced…

  7. Practitioner Views of Priorities, Policies, and Practices in Juvenile Justice

    ERIC Educational Resources Information Center

    Mears, Daniel P.; Shollenberger, Tracey L.; Willison, Janeen B.; Owens, Colleen E.; Butts, Jeffrey A.

    2010-01-01

    Dramatic changes in juvenile justice have occurred in recent decades. One result has been the emergence of new policies and practices, many of which remain largely unexamined. One avenue for gaining insight into whether such policies and practices are needed or effective, as well as into how the juvenile justice system might be improved, is to tap…

  8. Extinguishing All Hope: Life-without-Parole for Juveniles

    ERIC Educational Resources Information Center

    Butler, Frank

    2010-01-01

    Sentencing juveniles to life-without-parole (JLWOP) is a practice fraught with ethical dilemmas. Through in-depth interviews with 11 men living sentences of JLWOP, their narratives of their backgrounds and experiences as juveniles were studied. Common themes were identified, and 3 general categories of cases emerged from the narratives. Ethical…

  9. A Structural Equation Modeling Analysis of Influences on Juvenile Delinquency

    ERIC Educational Resources Information Center

    Barrett, David E.; Katsiyannis, Antonis; Zhang, Dalun; Zhang, Dake

    2014-01-01

    This study examined influences on delinquency and recidivism using structural equation modeling. The sample comprised 199,204 individuals: 99,602 youth whose cases had been processed by the South Carolina Department of Juvenile Justice and a matched control group of 99,602 youth without juvenile records. Structural equation modeling for the…

  10. Seasonal changes in neuron numbers in the hippocampal formation of a food-hoarding bird: the black-capped chickadee.

    PubMed

    Smulders, T V; Shiflett, M W; Sperling, A J; DeVoogd, T J

    2000-09-15

    The volume of the hippocampal formation (HF) in black-capped chickadees (Poecile atricapillus) varies across the seasons, in parallel with the seasonal cycle in food hoarding. In this study, we estimate cell density and total cell number in the HF across seasons in both juveniles and adults. We find that the seasonal variation in volume is due to an increase in the number of small and large cells (principally neurons) in the fall. Adults also have lower neuron densities than juveniles. Both juveniles and adults show an increase in cell density in the rostral part of the HF in August and a subsequent decrease toward October. This suggests that the net cell addition to the HF may already start in August. We discuss the implications of this early start with respect to the possibility that the seasonal change in HF volume is driven by the experience of food hoarding. We also speculate on the functional significance of the addition of neurons to the HF in the fall.

  11. Chronic Juvenile Stress Produces Corticolimbic Dendritic Architectural Remodeling and Modulates Emotional Behavior in Male and Female Rats

    PubMed Central

    Eiland, Lisa; Ramroop, Johnny; Hill, Matthew N.; Manley, Jasmine; McEwen, Bruce S.

    2011-01-01

    Nearly 12% of US children are exposed to intense adverse experiences. Research has demonstrated that these experiences can negatively impact adult health, often resulting in psychopathology. Less attention, however, is given to the impact of childhood adverse experiences on childhood health and wellbeing. Using a rodent model of chronic juvenile stress (restraint 6h daily from postnatal day 20–41), we report that chronic stress has significant immediate morbidities in both males and females during this developmental window. Specifically, we demonstrate that chronic juvenile stress produces depressive-like behavior and significant neuronal remodeling of brain regions likely involved in these behavioral alterations: the hippocampus, prefrontal cortex and amygdala. Chronically stressed males and females exhibit anhedonia, increased locomotion when exposed to novelty, and altered coping strategies when exposed to acute stress. Coincident with these behavioral changes, we report simplification of dendrites in the hippocampus and prefrontal cortex and concurrent hypertrophy of dendrites in the amygdala. Taken together, these results demonstrate that chronically stressed juveniles exhibit aberrant behavioral responses to acute challenges that occur in conjunction with stress-induced remodeling of brain regions intimately involved in regulating emotionality and stress reactivity. Further, the absence of sex differences in our reported stress responses, likely speaks to the decreased sensitivity of immature HPA regulating brain regions to sex hormones. PMID:21658845

  12. Chronic juvenile stress produces corticolimbic dendritic architectural remodeling and modulates emotional behavior in male and female rats.

    PubMed

    Eiland, Lisa; Ramroop, Johnny; Hill, Matthew N; Manley, Jasmine; McEwen, Bruce S

    2012-01-01

    Nearly 12% of US children are exposed to intense adverse experiences. Research has demonstrated that these experiences can negatively impact adult health, often resulting in psychopathology. Less attention, however, is given to the impact of childhood adverse experiences on childhood health and wellbeing. Using a rodent model of chronic juvenile stress (restraint 6 h daily from postnatal day 20 to 41), we report that chronic stress has significant immediate morbidities in both males and females during this developmental window. Specifically, we demonstrate that chronic juvenile stress produces depressive-like behavior and significant neuronal remodeling of brain regions likely involved in these behavioral alterations: the hippocampus, prefrontal cortex and amygdala. Chronically stressed males and females exhibit anhedonia, increased locomotion when exposed to novelty, and altered coping strategies when exposed to acute stress. Coincident with these behavioral changes, we report simplification of dendrites in the hippocampus and prefrontal cortex and concurrent hypertrophy of dendrites in the amygdala. Taken together, these results demonstrate that chronically stressed juveniles exhibit aberrant behavioral responses to acute challenges that occur in conjunction with stress-induced remodeling of brain regions intimately involved in regulating emotionality and stress reactivity. Further, the absence of sex differences in our reported stress responses, likely speaks to the decreased sensitivity of immature HPA regulating brain regions to sex hormones.

  13. Neonatal hypoxia-ischemia impairs juvenile recognition memory by disrupting the maturation of prefrontal-hippocampal networks.

    PubMed

    Domnick, Nina-Kristin; Gretenkord, Sabine; De Feo, Vito; Sedlacik, Jan; Brockmann, Marco D; Hanganu-Opatz, Ileana L

    2015-11-01

    High-prevalence/low-severity cognitive deficits represent the life-long burden of a perinatal hypoxic–ischemic (HI) insult. They have been proposed to result from dysmaturation of prelimbic-hippocampal networks, which account for mnemonic and executive performance. Already at neonatal age the communication within these networks is largely reduced after an early HI insult with mild/moderate structural outcome. However, the longlasting consequences of the neonatal network dysfunction remain unknown. Here,we combine MRI and electrophysiology in vivo with behavioral testing to assess the effects of an early HI insult on the structure and function of prelimbic-hippocampal networks and on related cognitive abilities of juvenile rats. Despite the absence of lesions over the prelimbic cortex (PL) and hippocampus (HP), juvenile rats experiencing an early HI have lower performance in item and temporal order recognition memory. These cognitive deficits do not result from delayed somatic development or increased locomotion or anxiety. More likely, abnormal activity patterns and interactions within prelimbic-hippocampal networks account for behavioral impairment. The early HI insult causes power reduction of the fast (12–48 Hz) network activity and diminishment of neuronal firing in the PL and HP. This weaker entrainment of local circuits at juvenile age emerges in the absence of sufficiently strong directed interactions within neonatal prelimbic-hippocampal networks. Similar developmental mechanisms may account for poorer academic achievements of HI-injured infants. PMID:26341391

  14. Estimating survival rates from banding of adult and juvenile birds

    USGS Publications Warehouse

    Johnson, D.H.

    1974-01-01

    The restrictive assumptions required by most available methods for estimating survival probabilities render them unsuitable for analyzing real banding data. A model is proposed which allows survival rates and recovery rates to vary with the calendar year, and also allows juveniles to have rates different from adults. In addition to survival rates and recovery rates, the differential vulnerability factors of juveniles relative to adults are estimated. Minimum values of the variances of the estimators are also given. The new procedure is applied to sets of duck and goose data in which reasonably large numbers of adult and juvenile birds were banded. The results are shown to be generally comparable to those procured by other methods, but, in addition, insight into the extent of annual variation is gained. Combining data from adults and juveniles also increases the effective sample size, since the juveniles are assumed to enter the adult age class after surviving their initial year.

  15. Neocortical neurogenesis and neuronal migration

    PubMed Central

    Tan, Xin; Shi, Song-Hai

    2012-01-01

    The neocortex, the evolutionarily newest part of the cerebral cortex, controls nearly all aspects of behavior, including perception, language and decision-making. It contains an immense number of neurons that can be broadly divided into two groups, excitatory neurons and inhibitory interneurons. These neurons are predominantly produced through extensive progenitor cell divisions during the embryonic stages. Moreover, they are not randomly dispersed, but spatially organized into horizontal layers that are essential for neocortex function. The formation of this laminar structure requires exquisite control of neuronal migration from their birthplace to their final destination. Extensive research over the past decade has greatly advanced our understanding of the production and migration of both excitatory neurons and inhibitory interneurons in the developing neocortex. In this review, we aim to give an overview on the molecular and cellular processes of neocortical neurogenesis and neuronal migration. PMID:24014417

  16. Interactions of neurons with topographic nano cues affect branching morphology mimicking neuron-neuron interactions.

    PubMed

    Baranes, Koby; Kollmar, Davida; Chejanovsky, Nathan; Sharoni, Amos; Shefi, Orit

    2012-08-01

    We study the effect of topographic nano-cues on neuronal growth-morphology using invertebrate neurons in culture. We use photolithography to fabricate substrates with repeatable line-pattern ridges of nano-scale heights of 10-150 nm. We plate leech neurons atop the patterned-substrates and compare their growth pattern to neurons plated atop non-patterned substrates. The model system allows us the analysis of single neurite-single ridge interactions. The use of high resolution electron microscopy reveals small filopodia processes that attach to the line-pattern ridges. These fine processes, that cannot be detected in light microscopy, add anchoring sites onto the side of the ridges, thus additional physical support. These interactions of the neuronal process dominantly affect the neuronal growth direction. We analyze the response of the entire neuronal branching tree to the patterned substrates and find significant effect on the growth patterns compared to non-patterned substrates. Moreover, interactions with the nano-cues trigger a growth strategy similarly to interactions with other neuronal cells, as reflected in their morphometric parameters. The number of branches and the number of neurites originating from the soma decrease following the interaction demonstrating a tendency to a more simplified neuronal branching tree. The effect of the nano-cues on the neuronal function deserves further investigation and will strengthen our understanding of the interplay between function and form.

  17. Microbes' roadmap to neurons.

    PubMed

    Kristensson, Krister

    2011-06-01

    The nervous system is protected by barriers that restrict the invasion of pathogens. Nevertheless, mechanisms have evolved by which microbes can pass these barriers, enter and exit neurons and target various regions of the nervous system. In the brain, immune responses to pathogens are generally not robust, so microbes can hide and survive or, conversely, cause severe uncontrolled infections. Depending on their sites of entry and the regions that they target, microbes can cause diverse nervous system dysfunctions and even influence host behaviour to their own advantage. This Review discusses routes by which microbes can reach the nervous system and cause persistent or life-threatening infections.

  18. Managing juvenile idiopathic arthritis-associated uveitis.

    PubMed

    Hawkins, Madeleine J; Dick, Andrew D; Lee, Richard J W; Ramanan, Athimalaipet V; Carreño, Ester; Guly, Catherine M; Ross, Adam H

    2016-01-01

    Bilateral chronic anterior uveitis is an extra-articular feature of juvenile idiopathic arthritis. Although figures vary, uveitis occurs in approximately 11%-13% of patients with this disease and is most commonly associated with the female gender, oligoarthritis, and presence of antinuclear antibodies. The disease has an insidious onset and is often asymptomatic. Managing patients with juvenile idiopathic arthritis-associated uveitis remains challenging as the disease may prove to be refractory to traditional treatment regimens. Stepwise immunomodulatory therapy is indicated, with new biologic drugs being used last in cases of refractory uveitis. Small scale studies and practice have provided the evidence to undertake randomized control trials to evaluate the efficacy, safety, and cost-effectiveness of anti-tumor necrosis factor-α therapies, such as infliximab and adalimumab. These have demonstrated promising results, with further data awaited from ongoing trials for adalimumab (as SYCAMORE and ADJUVITE trials). Lower grade evidence is supporting the use of newer biologics such as rituximab, daclizumab, tocilizumab, and abatacept in those cases refractory to anti-tumor necrosis factor-α therapy.

  19. Preferred temperatures of juvenile lake whitefish

    USGS Publications Warehouse

    Edsall, Thomas A.

    1999-01-01

    Lake whitefish (Coregonus clupeaformis) supported valuable commercial fisheries in all of the Great Lakes until the 1950s to 1960s when their populations collapsed due to overfishing, pollution, and predation by the exotic sea lamprey (Petromyzon marinus). Reduction of these population stresses has permitted significant recovery of the lake whitefish in the upper three Great Lakes since the 1980s, and limited but encouraging recovery is now apparent in Lakes Erie and Ontario. In the present study the thermal preferences of age-0 and age-1 lake whitefish were measured in the laboratory to provide a basis for determining thermal habitat use by juvenile lake whitefish and thermal niche overlap with exotic fishes that might prey on them. Final thermal preferenda of young lake whitefish varied inversely with fish size ranging from 16.8°C for fish averaging 1.9 g to 15.6°C for age-1 fish averaging 3.9 g. Final thermal preferenda were in agreement with the limited published information on temperature selection of juvenile lake whitefish in the laboratory and on thermal habitat use by wild, free-ranging populations in the Great Lakes.

  20. Primary radiation therapy for juvenile nasopharyngeal angiofibroma.

    PubMed

    Cummings, B J; Blend, R; Keane, T; Fitzpatrick, P; Beale, F; Clark, R; Garrett, P; Harwood, A; Payne, D; Rider, W

    1984-12-01

    Evidence is presented of the effectiveness and relative lack of serious toxicity of external beam megavoltage radiation therapy (RT) as primary treatment for juvenile nasopharyngeal angiofibroma. The importance of careful radiological evaluation of tumor extent prior to irradiation is stressed, and only moderate dose RT is required. Fifty-five patients have been treated by RT and followed for from 3 to 26 years. Forty-four of 55 patients (80%) had permanent tumor control following a single course of 3000 cGy to 3500 cGy over 3 weeks. Surgical resection or a second course of RT controlled the tumor in all 11 patients in whom regrowth occurred. Angiofibromas involute slowly after RT so that 50% of patients still had visible masses in the nasopharynx 12 months after treatment, but only 10% had any visible abnormality 36 months after RT. Retreatment was necessary only if symptoms recurred, and continued follow-up showed that most asymptomatic nasopharyngeal masses resolved completely. Acute and late toxicity rates were low. Two patients developed tumors in the head or neck following RT. There was no significant clinical impairment of growth or endocrine function. A single course of external beam megavoltage radiation to 3000 cGy in 3 weeks is an effective first treatment for patients with juvenile nasopharyngeal angiofibroma.

  1. Systemic-onset juvenile idiopathic arthritis.

    PubMed

    Cimaz, Rolando

    2016-09-01

    Systemic-onset juvenile idiopathic arthritis (SoJIA) is a systemic inflammatory disease which has up to now been classified as a category of juvenile idiopathic arthritis. However, in this context, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it may rather be part of the spectrum of autoinflammatory disorders. The disease is in fact unique with regard to the other JIA categories, in terms of clinical manifestations, prognosis, and response to conventional immunosuppressant therapies. It is characterized clinically by fever, lymphadenopathy, arthritis, rash, and serositis. IL-1 and IL-6 play a major role in the pathogenesis of SoJIA, and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. However, complications of SoJIA, including macrophage activation syndrome, limitations in functional outcome by arthritis and long-term damage from chronic inflammation continue to be a major issue in patients' care. Recent advances on the pathogenesis and treatment have revolutionized the care and prognosis of this potentially life-threatening pediatric condition.

  2. Juvenile Delinquency and Teenage Pregnancy: A Comparison of Ecological Risk Profiles among Midwestern White and Black Female Juvenile Offenders

    ERIC Educational Resources Information Center

    Khurana, Atika; Cooksey, Elizabeth C.; Gavazzi, Stephen M.

    2011-01-01

    The authors examined ecological risk factors associated with teen pregnancy with a sample of 1,190 court-involved female juvenile offenders between 11 and 18 years of age. Data were obtained from five Midwestern juvenile county courts using a recently developed youth risk assessment instrument called the global risk assessment device (GRAD). In…

  3. Characteristics and Patterns of At-Risk Juveniles and Factors That Contribute to Violence Committed by or against Juveniles.

    ERIC Educational Resources Information Center

    School Intervention Report, 1999

    1999-01-01

    Research into the characteristics of at-risk juveniles that contribute to violence and the patterns of behavior and neighborhood characteristics and patterns that are associated with youth violence or victimization are reviewed. The results of the studies reviewed cannot be generalized to the total population of juveniles, but the findings of…

  4. National Implications in Education and Juvenile Justice: Bridging the Gap between Court Ordered Juvenile Mentoring Programs and Secondary Educators

    ERIC Educational Resources Information Center

    Belshaw, Scott H.; Kritsonis, William Allan

    2006-01-01

    This article deals with effective strategies to show that educators and school administrators can utilize court-ordered mentors, for at-risk juveniles placed in their classrooms. In the late 1980s and 1990s there was a convergence of two major events in juvenile justice. These were the reduction of government funds to address serious social…

  5. The straintronic spin-neuron.

    PubMed

    Biswas, Ayan K; Atulasimha, Jayasimha; Bandyopadhyay, Supriyo

    2015-07-17

    In artificial neural networks, neurons are usually implemented with highly dissipative CMOS-based operational amplifiers. A more energy-efficient implementation is a 'spin-neuron' realized with a magneto-tunneling junction (MTJ) that is switched with a spin-polarized current (representing weighted sum of input currents) that either delivers a spin transfer torque or induces domain wall motion in the soft layer of the MTJ to mimic neuron firing. Here, we propose and analyze a different type of spin-neuron in which the soft layer of the MTJ is switched with mechanical strain generated by a voltage (representing weighted sum of input voltages) and term it straintronic spin-neuron. It dissipates orders of magnitude less energy in threshold operations than the traditional current-driven spin neuron at 0 K temperature and may even be faster. We have also studied the room-temperature firing behaviors of both types of spin neurons and find that thermal noise degrades the performance of both types, but the current-driven type is degraded much more than the straintronic type if both are optimized for maximum energy-efficiency. On the other hand, if both are designed to have the same level of thermal degradation, then the current-driven version will dissipate orders of magnitude more energy than the straintronic version. Thus, the straintronic spin-neuron is superior to current-driven spin neurons. PMID:26112081

  6. The straintronic spin-neuron.

    PubMed

    Biswas, Ayan K; Atulasimha, Jayasimha; Bandyopadhyay, Supriyo

    2015-07-17

    In artificial neural networks, neurons are usually implemented with highly dissipative CMOS-based operational amplifiers. A more energy-efficient implementation is a 'spin-neuron' realized with a magneto-tunneling junction (MTJ) that is switched with a spin-polarized current (representing weighted sum of input currents) that either delivers a spin transfer torque or induces domain wall motion in the soft layer of the MTJ to mimic neuron firing. Here, we propose and analyze a different type of spin-neuron in which the soft layer of the MTJ is switched with mechanical strain generated by a voltage (representing weighted sum of input voltages) and term it straintronic spin-neuron. It dissipates orders of magnitude less energy in threshold operations than the traditional current-driven spin neuron at 0 K temperature and may even be faster. We have also studied the room-temperature firing behaviors of both types of spin neurons and find that thermal noise degrades the performance of both types, but the current-driven type is degraded much more than the straintronic type if both are optimized for maximum energy-efficiency. On the other hand, if both are designed to have the same level of thermal degradation, then the current-driven version will dissipate orders of magnitude more energy than the straintronic version. Thus, the straintronic spin-neuron is superior to current-driven spin neurons.

  7. The biophysics of neuronal growth

    NASA Astrophysics Data System (ADS)

    Franze, Kristian; Guck, Jochen

    2010-09-01

    For a long time, neuroscience has focused on biochemical, molecular biological and electrophysiological aspects of neuronal physiology and pathology. However, there is a growing body of evidence indicating the importance of physical stimuli for neuronal growth and development. In this review we briefly summarize the historical background of neurobiophysics and give an overview over the current understanding of neuronal growth from a physics perspective. We show how biophysics has so far contributed to a better understanding of neuronal growth and discuss current inconsistencies. Finally, we speculate how biophysics may contribute to the successful treatment of lesions to the central nervous system, which have been considered incurable until very recently.

  8. Assessing Neuronal Bioenergetic Status

    PubMed Central

    Zeiger, Stephanie L.H.; Stankowski, Jeannette N.; McLaughlin, BethAnn

    2013-01-01

    Drug discovery and therapeutic development for disorders of the central nervous system (CNS) represents one of the largest unmet markets in modern medicine. We have increasingly recognized that the lack of stringent assessment of mitochondrial function during the discovery process has resulted in drug recalls, black box warnings, and an urgent need to understand the metabolic liability of small molecules in neural systems. Given that the brain is the most energetically demanding organ, even modest perturbations in neuronal energetic pathways have been shown to impact growth, signaling, connectivity, and the restorative capacity of the CNS. In this work, we describe several tools to assess metabolic activity of primary neuronal cultures and neural cell lines using an acute model of injury induced by oxygen glucose deprivation. Methods include the measurement of total ATP and NADH, enzymatic assessment of lactate production by anaerobic respiration, as well as viability assays. We also present a modified screening method for assessing aerobic respiration of immortalized cell lines using galactose challenge. PMID:21815069

  9. Assessing neuronal bioenergetic status.

    PubMed

    Zeiger, Stephanie L H; Stankowski, Jeannette N; McLaughlin, BethAnn

    2011-01-01

    Drug discovery and therapeutic development for disorders of the central nervous system (CNS) represents one of the largest unmet markets in modern medicine. We have increasingly recognized that the lack of stringent assessment of mitochondrial function during the discovery process has resulted in drug recalls, black box warnings, and an urgent need to understand the metabolic liability of small molecules in neural systems. Given that the brain is the most energetically demanding organ, even modest perturbations in neuronal energetic pathways have been shown to impact growth, signaling, connectivity, and the restorative capacity of the CNS. In this work, we describe several tools to assess metabolic activity of primary neuronal cultures and neural cell lines using an acute model of injury induced by oxygen glucose deprivation. Methods include the measurement of total ATP and NADH, enzymatic assessment of lactate production by anaerobic respiration, as well as viability assays. We also present a modified screening method for assessing aerobic respiration of immortalized cell lines using galactose challenge.

  10. Juvenile Correctional Workers' Perceptions of Suicide Risk Factors and Mental Health Issues of Incarcerated Juveniles

    PubMed Central

    Penn, Joseph V.; Esposito, Christianne; Stein, L. A. R.; Lacher-Katz, Molly; Spirito, Anthony

    2009-01-01

    Correctional staff knowledge, attitudes, and perceptions of incarcerated juveniles' mental health needs, including suicide prevention, have not been studied empirically. This study measured juvenile correctional officers' knowledge and attitudes regarding suicide risk factors and mental health and substance abuse issues through administration of the Mental Health Knowledge and Attitude Test (MHKAT) before and after a staff training on suicide prevention. Seventy-six participants completed the pre- and post-training MHKAT. They demonstrated significant improvement in knowledge of and attitudes toward mental health treatment of incarcerated youth as reflected by higher post-training MHKAT scores. Findings suggest that correctional staff are receptive to increasing knowledge of critical mental health issues. Studies of the retention and implementation of this new knowledge by direct care staff over time and the optimal type and frequency of new staff training and continuing education are indicated. PMID:19809578

  11. The Mentally Retarded and the Juvenile Court Project CAMIO, Volume 7.

    ERIC Educational Resources Information Center

    Haskins, Jimmy R.; Friel, Charles M.

    Studied was the relationship of the mentally retarded (MR) and the juvenile court through an attempt to determine the incidence of MR juveniles adjudicated by the juvenile court in one metropolitan county in Texas, to determine the attitude of juvenile probation officers toward the MR delinquent, and to determine the availability of community…

  12. From the Bench -- Juvenile Courts: How and Why They Have Changed.

    ERIC Educational Resources Information Center

    Van Nuys, Heather; Blitzman, Jay; Hibbler, William; Wakefield, Dana

    2000-01-01

    Offers four judges' perspectives on the various changes in the juvenile court system focusing on the increased violence among juveniles as having the greatest effect on the courts; includes issues such as juveniles being tried in adult courts, the need to improve juvenile courts, and the role of public interest. (CMK)

  13. Critical Factors in Mental Health Programming for Juveniles in Corrections Facilities

    ERIC Educational Resources Information Center

    Underwood, Lee A.; Phillips, Annie; von Dresner, Kara; Knight, Pamela D.

    2006-01-01

    Juveniles with mental health and other specialized needs are overrepresented in the juvenile justice system, and while juvenile corrections have not historically provided standardized and evidence-based mental health services for its incarcerated youth, the demand is evident. The reality is that juveniles with serious mental illness are committed…

  14. An Examination of the Educational Programs Held for Juvenile Delinquents in Turkey

    ERIC Educational Resources Information Center

    Ozdemir, Soner Mehmet

    2010-01-01

    The purpose of this study is to examine some educational programs given in prisons to socialization juvenile delinquents in Turkey, and to evaluate these educational programs in relation to the views and opinions of juveniles. The study was conducted in Ankara Juvenile and Youth Closed Prison (ACGKCIK), one of 3 juvenile prisons in Turkey. Data…

  15. Laser capture microdissection of gonads from juvenile zebrafish

    PubMed Central

    2009-01-01

    Background Investigating gonadal gene expression is important in attempting to elucidate the molecular mechanism of sex determination and differentiation in the model species zebrafish. However, the small size of juvenile zebrafish and correspondingly their gonads complicates this type of investigation. Furthermore, the lack of a genetic sex marker in juvenile zebrafish prevents pooling gonads from several individuals. The aim of this study was to establish a method to isolate the gonads from individual juvenile zebrafish allowing future investigations of gonadal gene expression during sex determination and differentiation. Methods The laser capture microdissection technique enables isolation of specific cells and tissues and thereby removes the noise of gene expression from other cells or tissues in the gene expression profile. A protocol developed for laser microdissection of human gonocytes was adjusted and optimised to isolate juvenile zebrafish gonads. Results The juvenile zebrafish gonad is not morphologically distinguishable when using dehydrated cryosections on membrane slides and a specific staining method is necessary to identify the gonads. The protocol setup in this study allows staining, identification, isolation and subsequent RNA purification and amplification of gonads from individual juvenile zebrafish thereby enabling gonadal gene expression profiling. Conclusion The study presents a protocol for isolation of individual juvenile zebrafish gonads, which will enable future investigations of gonadal gene expression during the critical period of sex differentiation. Furthermore, the presented staining method is applicable to other species as it is directed towards alkaline phosphatase that is expressed in gonocytes and embryonic stem cells, which is conserved among vertebrate species. PMID:19747405

  16. Few juvenile auditory perceptual skills correlate with adult performance.

    PubMed

    Sarro, Emma C; Sanes, Dan H

    2014-02-01

    Measures of human mental development suggest that behavioral skills displayed during early life can predict an individual's subsequent cognitive performance. Support for this draws from longitudinal studies that reveal compelling within-subject correlations during childhood. If this idea applies across the life span, then correlations in performance should persist into adulthood. Here, we address this prediction in juvenile and adult gerbils by evaluating within-subject measures of auditory learning and perception. Animals were trained and tested as juveniles on either an amplitude modulation (AM) or a frequency modulation (FM) detection task. Measures of learning and perception obtained from juveniles were then compared to similar measures obtained when each subject was tested in adulthood on either the same task or the untrained task. For animals trained and tested on the AM detection task as juveniles and adults, there was no correlation between juvenile and adult learning metrics, or perceptual sensitivity. For animals trained and tested on FM detection as juveniles, we observed a significant relationship to their adult performance. Juveniles that performed the best on FM detection were the poorest at AM detection, and the best at FM detection, when tested as adults. Thus, across-age correlations for sensory and cognitive measures, obtained during development and in adulthood, depend heavily on the specific type of developmental experience and the outcome measure.

  17. Synchronization by elastic neuronal latencies

    NASA Astrophysics Data System (ADS)

    Vardi, Roni; Timor, Reut; Marom, Shimon; Abeles, Moshe; Kanter, Ido

    2013-01-01

    Psychological and physiological considerations entail that formation and functionality of neuronal cell assemblies depend upon synchronized repeated activation such as zero-lag synchronization. Several mechanisms for the emergence of this phenomenon have been suggested, including the global network quantity, the greatest common divisor of neuronal circuit delay loops. However, they require strict biological prerequisites such as precisely matched delays and connectivity, and synchronization is represented as a stationary mode of activity instead of a transient phenomenon. Here we show that the unavoidable increase in neuronal response latency to ongoing stimulation serves as a nonuniform gradual stretching of neuronal circuit delay loops. This apparent nuisance is revealed to be an essential mechanism in various types of neuronal time controllers, where synchronization emerges as a transient phenomenon and without predefined precisely matched synaptic delays. These findings are described in an experimental procedure where conditioned stimulations were enforced on a circuit of neurons embedded within a large-scale network of cortical cells in vitro, and are corroborated and extended by simulations of circuits composed of Hodgkin-Huxley neurons with time-dependent latencies. These findings announce a cortical time scale for time controllers based on tens of microseconds stretching of neuronal circuit delay loops per spike. They call for a reexamination of the role of the temporal periodic mode in brain functionality using advanced in vitro and in vivo experiments.

  18. The straintronic spin-neuron

    NASA Astrophysics Data System (ADS)

    Biswas, Ayan K.; Atulasimha, Jayasimha; Bandyopadhyay, Supriyo

    2015-07-01

    In artificial neural networks, neurons are usually implemented with highly dissipative CMOS-based operational amplifiers. A more energy-efficient implementation is a ‘spin-neuron’ realized with a magneto-tunneling junction (MTJ) that is switched with a spin-polarized current (representing weighted sum of input currents) that either delivers a spin transfer torque or induces domain wall motion in the soft layer of the MTJ to mimic neuron firing. Here, we propose and analyze a different type of spin-neuron in which the soft layer of the MTJ is switched with mechanical strain generated by a voltage (representing weighted sum of input voltages) and term it straintronic spin-neuron. It dissipates orders of magnitude less energy in threshold operations than the traditional current-driven spin neuron at 0 K temperature and may even be faster. We have also studied the room-temperature firing behaviors of both types of spin neurons and find that thermal noise degrades the performance of both types, but the current-driven type is degraded much more than the straintronic type if both are optimized for maximum energy-efficiency. On the other hand, if both are designed to have the same level of thermal degradation, then the current-driven version will dissipate orders of magnitude more energy than the straintronic version. Thus, the straintronic spin-neuron is superior to current-driven spin neurons.

  19. Cryopreservation of adherent neuronal networks.

    PubMed

    Ma, Wu; O'Shaughnessy, Thomas; Chang, Eddie

    2006-07-31

    Neuronal networks have been widely used for neurophysiology, drug discovery and toxicity testing. An essential prerequisite for future widespread application of neuronal networks is the development of efficient cryopreservation protocols to facilitate their storage and transportation. Here is the first report on cryopreservation of mammalian adherent neuronal networks. Dissociated spinal cord cells were attached to a poly-d-lysine/laminin surface and allowed to form neuronal networks. Adherent neuronal networks were embedded in a thin film of collagen gel and loaded with trehalose prior to transfer to a freezing medium containing DMSO, FBS and culture medium. This was followed by a slow rate of cooling to -80 degrees C for 24 h and then storage for up to 2 months in liquid nitrogen at -196 degrees C. The three components: DMSO, collagen gel entrapment and trehalose loading combined provided the highest post-thaw viability, relative to individual or two component protocols. The post-thaw cells with this protocol demonstrated similar neuronal and astrocytic markers and morphological structure as those detected in unfrozen cells. Fluorescent dye FM1-43 staining revealed active recycling of synaptic vesicles upon depolarizing stimulation in the post-thaw neuronal networks. These results suggest that a combination of DMSO, collagen gel entrapment and trehalose loading can significantly improve conventional slow-cooling methods in cryopreservation of adherent neuronal networks.

  20. Immunogenetic Risk and Protective Factors for Juvenile Dermatomyositis in Caucasians

    PubMed Central

    Mamyrova, Gulnara; O’Hanlon, Terrance P.; Monroe, Jason B.; Carrick, Danielle Mercatante; Malley, James D.; Adams, Sharon; Reed, Ann M.; Shamim, Ejaz A.; James‐Newton, Laura; Miller, Frederick W.; Rider, Lisa G.

    2007-01-01

    Objective To define the relative importance of MHC Class II alleles and peptide binding motifs as risk and protective factors for juvenile dermatomyositis (DM) and to compare these to HLA associations in adult DM. Methods DRB1 and DQA1 typing was performed in 142 Caucasian patients with juvenile DM, and compared to HLA typing from 193 patients with adult DM and 797 race‐matched controls. Random Forests classification and multiple logistic regression assessed the relative importance of the HLA associations. Results The HLA DRB1*0301 allele was a primary risk factor (Odds Ratio [OR] 3.9), while DQA1*0301 (OR 2.8), DQA1*0501 (OR 2.1), and homozygosity of DQA1*0501 (OR 3.2) were additional risk factors for juvenile DM. These risk factors were not present in adult DM without defined autoantibodies. DQA1 *0201 (OR 0.37), *0101 (OR 0.38), and *0102 (OR 0.51) were identified as novel protective factors for juvenile DM, the latter two being shared with adult DM. The peptide binding motif DRB1 9EYSTS13 was a risk factor and DQA1 motifs F25, S26 and 45(V/A) W (R/K)47 were protective. Random Forests classification analysis revealed DRB1*0301 (Relative Importance [RI] 100%) had higher relative importance than DQA1*0301 (RI 57%), DQA1*0501 (RI 42%), or the peptide binding motifs among risk factors for juvenile DM. In a logistic regression model, DRB1*0301 and DQA*0201 were the strongest risk and protective factors, respectively, for juvenile DM. Conclusion DRB1*0301 has higher relative importance than DQA1*0501 as a risk factor for juvenile DM. DQA1*0301 has been identified as a new HLA risk factor for juvenile DM. Three DQA1 alleles are newly identified protective factors for juvenile DM. PMID:17133612