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dentatorubropallidoluysian atrophy (57) 57
humans (47) 47
clinical neurology (38) 38
adult (30) 30
female (30) 30
dentatorubral-pallidoluysian atrophy (28) 28
male (28) 28
middle aged (27) 27
expansion (24) 24
cag repeat (22) 22
drpla (22) 22
myoclonic epilepsies, progressive - genetics (16) 16
family (15) 15
neurosciences (15) 15
aged (14) 14
atrophy (14) 14
spinocerebellar ataxia (14) 14
adolescent (13) 13
nerve tissue proteins - genetics (13) 13
pedigree (12) 12
trinucleotide (12) 12
machado-joseph-disease (10) 10
myoclonus epilepsy (10) 10
child (9) 9
disease (9) 9
globus pallidus - pathology (9) 9
index medicus (9) 9
trinucleotide repeats (9) 9
ataxia (8) 8
genotype (8) 8
huntington's disease (8) 8
mutation (8) 8
myoclonic epilepsies, progressive - pathology (8) 8
phenotype (8) 8
trinucleotide repeat expansion (8) 8
alleles (7) 7
diagnosis, differential (7) 7
dominant cerebellar-ataxia (7) 7
features (7) 7
machado-joseph disease (7) 7
myoclonic epilepsies, progressive - complications (7) 7
red nucleus - pathology (7) 7
age of onset (6) 6
brain - pathology (6) 6
cerebellar ataxia (6) 6
dentatorubral‐pallidoluysian atrophy (6) 6
genetics & heredity (6) 6
myoclonic epilepsies, progressive - physiopathology (6) 6
neurology (6) 6
pathology (6) 6
spinocerebellar degenerations - genetics (6) 6
trinucleotide repeats - genetics (6) 6
brain (5) 5
cerebellar nuclei - pathology (5) 5
clinical-features (5) 5
dentate gyrus - pathology (5) 5
dna mutational analysis (5) 5
gene (5) 5
genes, dominant (5) 5
genetics (5) 5
haw-river-syndrome (5) 5
huntingtons-disease (5) 5
myoclonic epilepsies, progressive - diagnosis (5) 5
neurodegenerative diseases - genetics (5) 5
prevalence (5) 5
psychiatry (5) 5
trinucleotide repeat (5) 5
trinucleotide repeat expansion - genetics (5) 5
american (4) 4
cerebellar ataxia - genetics (4) 4
cerebellum - pathology (4) 4
degeneration (4) 4
dementia (4) 4
european continental ancestry group - genetics (4) 4
huntington disease - pathology (4) 4
locus (4) 4
machado-joseph disease - genetics (4) 4
magnetic resonance imaging (4) 4
medicine & public health (4) 4
neurodegenerative disease (4) 4
neurodegenerative diseases (4) 4
neurodegenerative diseases - pathology (4) 4
neurons - pathology (4) 4
nuclear proteins - genetics (4) 4
research (4) 4
spinocerebellar ataxias - genetics (4) 4
surgery (4) 4
aged, 80 and over (3) 3
brain stem - pathology (3) 3
cag repeats (3) 3
cag trinucleotide repeat (3) 3
cag-repeat (3) 3
cerebellar ataxia - epidemiology (3) 3
cerebellar-ataxia (3) 3
cerebellum (3) 3
chromosome aberrations - genetics (3) 3
chromosome disorders (3) 3
chromosomes, human, pair 12 (3) 3
dentate nucleus (3) 3
dentatorubro-pallidoluysian atrophy (3) 3
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1. Full Text Case report of optic atrophy in Dentatorubropallidoluysian Atrophy (DRPLA)
by Silver, Michael R and Sethi, Kapil D and Mehta, Shyamal H and Nichols, Fenwick T and Morgan, John C
BMC Neurology, ISSN 1471-2377, 12/2015, Volume 15, Issue 1, p. 260
Background: Dentatorubropallidoluysian atrophy (DRPLA) is a rare autosomal dominant neurodegenerative disease that is associated with numerous movement... 
Ataxia | Optic atrophy | Dystonia | Dentatorubropallidoluysian Atrophy (DRPLA) | African American | AMERICAN | SPINOCEREBELLAR ATAXIA | CEREBELLAR-ATAXIA | DISEASE | DENTATORUBRAL-PALLIDOLUYSIAN ATROPHY | CLINICAL NEUROLOGY | DEGENERATION | Optic Atrophy - etiology | Humans | Middle Aged | Leukoencephalopathies - etiology | Myoclonic Epilepsies, Progressive - complications | Ataxia - etiology | Male | Myoclonic Epilepsies, Progressive - genetics | Dystonia - etiology | Trinucleotide Repeats - genetics | Nerve Tissue Proteins - genetics | Complications and side effects | Care and treatment | Activities of daily living | Analysis | Nervous system | Degeneration | Research | Movement disorders | Risk factors
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2. Full Text A shared haplotype for dentatorubropallidoluysian atrophy (DRPLA) in Italian families testifies of the recent introduction of the mutation
by Veneziano, Liana and Mantuano, Elide and Catalli, Claudio and Gellera, Cinzia and Durr, Alexandra and Romano, Silvia and Spadaro, Maria and Frontali, Marina and Novelletto, Andrea
Journal of Human Genetics, ISSN 1434-5161, 2014, Volume 59, Issue 3, pp. 153 - 157
To clarify the population history of dentatorubropallidoluysian atrophy ( DRPLA) in Italy and to date back the introduction of the mutation, we reconstructed... 
gene flow | ATN1 | genetic dating | dentatorubropallidoluysian atrophy | DRPLA | haplotype/polyglutamine expansion diseases | haplotype | TRINUCLEOTIDE | ASIAN ORIGIN | SPINOCEREBELLAR ATAXIAS | CEREBELLAR-ATAXIA | GENETICS | polyglutamine expansion diseases | EXPANSION | GENETICS & HEREDITY | DENTATORUBRAL-PALLIDOLUYSIAN ATROPHY | REPEATS | Atrophy | Haplotypes - genetics | Neurodegenerative Diseases - pathology | Pedigree | Base Pairing - genetics | Humans | Recombination, Genetic - genetics | Female | Italy | Male | Neurodegenerative Diseases - genetics | Mutation - genetics
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3. Full Text A Severe Case of Dentatorubro-Pallidoluysian Atrophy (DRPLA) with Microcephaly, Very Early Onset of Seizures, and Cerebral White Matter Involvement
by Brunetti-Pierri, N and Wilfong, A. A and Hunter, J. V and Craigen, W. J
Neuropediatrics, ISSN 0174-304X, 2006, Volume 37, Issue 5, pp. 308 - 311
Abstract We report a severe case of Dentatorubro-pallidoluysian atrophy (DRPLA) presenting with microcephaly, developmental delay, severe epilepsy, and... 
Review Articles | White matter | Atrophin-1 | DRPLA | DENTATORUBROPALLIDOLUYSIAN ATROPHY | CAG REPEAT | white matter | TRINUCLEOTIDE | atrophin-1 | PEDIATRICS | PROTON | CLINICAL-FEATURES | JUVENILE-TYPE | CLINICAL NEUROLOGY | FAMILY | Microcephaly - complications | Humans | Infant, Newborn, Diseases | Magnetic Resonance Imaging - methods | Male | Myoclonic Epilepsies, Progressive - pathology | Review Literature as Topic | Microcephaly - pathology | Myoclonic Epilepsies, Progressive - complications | Seizures - etiology | Child | Longitudinal Studies | Infant, Newborn
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4. Full Text Huntington's disease-like disorders in Latin America and the Caribbean
by Walker, Ruth H and Gatto, Emilia M and Bustamante, M. Leonor and Bernal-Pacheco, Oscar and Cardoso, Francisco and Castilhos, Raphael M and Chana-Cuevas, Pedro and Cornejo-Olivas, Mario and Estrada-Bellmann, Ingrid and Jardim, Laura B and López-Castellanos, Ricardo and López-Contreras, Ricardo and Maia, Debora P and Mazzetti, Pilar and Miranda, Marcelo and Rodríguez-Violante, Mayela and Teive, Helio and Tumas, Vitor
Parkinsonism and Related Disorders, ISSN 1353-8020, 08/2018, Volume 53, pp. 10 - 20
Diseases with a choreic phenotype can be due to a variety of genetic etiologies. As testing for Huntington's disease (HD) becomes more available in previously... 
Genetics | HDL2 | Chorea-acanthocytosis | Spinocerebellar ataxia | Huntington disease | TRINUCLEOTIDE REPEAT EXPANSIONS | MCLEOD-SYNDROME | ATAXIA TYPE 10 | BRAZILIAN PATIENTS | CLINICAL NEUROLOGY | MOVEMENT-DISORDERS | GENETIC-CHARACTERISTICS | DENTATORUBROPALLIDOLUYSIAN ATROPHY DRPLA | DENTATORUBRAL-PALLIDOLUYSIAN ATROPHY
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5. Full Text Dentatorubral and pallidoluysian atrophy (DRPLA) Clinical and neuropathological findings in genetically confirmed north american and european pedigrees
by Becher, M. W and Rubinsztein, D. C and Leggo, J and Wagster, M. V and Stine, O. C and Ranen, N. G and Franz, M. L and Abbott, M. H and Sherr, M and Macmillan, J. C and Barron, L and Porteous, M and Harper, P. S and Ross, C. A
Movement Disorders, ISSN 0885-3185, 07/1997, Volume 12, Issue 4, pp. 519 - 530
Dentatorubral and pallidoluysian atrophy (DRPLA) is an autosomal dominant disorder that clinically overlaps with Huntington's disease (HD) and manifests... 
Huntington's disease | Dentate nucleus | Dentatorubral and pallidoluysian atrophy | CAG REPEAT | MACHADO-JOSEPH DISEASE | dentatorubral and pallidoluysian atrophy | TRINUCLEOTIDE REPEAT EXPANSION | dentate nucleus | MYOCLONUS EPILEPSY | NEURODEGENERATIVE DISEASES | CLINICAL NEUROLOGY | AGE-OF-ONSET | DENTATORUBROPALLIDOLUYSIAN ATROPHY | HUNTINGTONS-DISEASE GENE | LUYSIAN ATROPHY | HAW-RIVER-SYNDROME | Trinucleotide Repeats | United States | Humans | Middle Aged | Brain Diseases - genetics | Movement Disorders - diagnosis | Nerve Degeneration - genetics | Infant | Male | Brain Diseases - ethnology | Brain Diseases - physiopathology | Atrophy | Adult | Female | African Continental Ancestry Group - genetics | Child | Chromosomes, Human, Pair 12 - genetics | Globus Pallidus - pathology | Movement Disorders - ethnology | Diagnosis, Differential | European Continental Ancestry Group - genetics | Brain Diseases - diagnosis | Brain - physiopathology | United Kingdom | Phenotype | Movement Disorders - physiopathology | Pedigree | Brain - pathology | Movement Disorders - genetics | Dentate Gyrus - pathology | Red Nucleus - pathology
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6. Full Text Late onset ataxia phenotype in dentatorubro-pallidoluysian atrophy (DRPLA)
by Yabe, Ichiro and Sasaki, Hidenao and Kikuchi, Seiji and Nonaka, Michio and Moriwaka, Fumio and Tashiro, Kunio
Journal of Neurology, ISSN 0340-5354, 4/2002, Volume 249, Issue 4, pp. 432 - 436
We clinically and genetically studied three patients in a family with dentatorubro-pallidoluysian atrophy (DRPLA). The proband patient had 58/24 CAG repeat... 
Ataxia | Dentatorubro-pallidoluysian atrophy | Choreiform movements | Spinocerebellar ataxia | Dementia | Neurology | Neurosciences | Medicine & Public Health | Neuroradiology | dentatorubro-pallidoluysian atrophy | TRINUCLEOTIDE | CLINICAL-FEATURES | MYOCLONUS EPILEPSY | CLINICAL NEUROLOGY | FAMILY | choreiform movements | DENTATORUBROPALLIDOLUYSIAN ATROPHY | AMERICAN | ataxia | CHOREA | dementia | CAG-REPEAT EXPANSIONS | spinocerebellar ataxia | Ataxia - physiopathology | Diagnosis, Differential | Humans | Male | Ataxia - diagnosis | Cerebellum - pathology | Phenotype | Brain Stem - pathology | Pedigree | Myoclonic Epilepsies, Progressive - physiopathology | Female | Myoclonic Epilepsies, Progressive - genetics | Aged | Ataxia - genetics | Myoclonic Epilepsies, Progressive - diagnosis | Atrophy | Research | Risk factors
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7. Full Text 신경변성질환인 Dentatorubropallidoluysian Atrophy (DRPLA)에서의 각막병변 1예
by 이지은 and Lee Ji Eun and 박현준 and Park Hyeon Jun and 이종수 and Lee Jong Su and 이재혁 and Lee Jae Hyeog and 정대수 and Jeong Dae Su
대한안과학회지, ISSN 0378-6471, 2004, Volume 45, Issue 3, pp. 522 - 527
목적: Dentatorubropallioluysian atrophy (DRPLA)는 주로 상염색체우성으로 유전하는 드문 신경변성질환으로 각막내피세포의 변화를 동반하기에 증례 보고하고자 한다. 대상과 방법: 소뇌실조증 및 근간대성 경련을 보이며 자기공명영상상 소뇌 및 뇌교의 위축을 보여... 
Dentatorubropallidoluysian atrophy | DRPLA | Spinocerebellar degeneration | SCD | Specular microscopy | Corneal endothelium
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8. Full Text Is cerebral white matter involvement helpful in the diagnosis of dentatorubral-pallidoluysian atrophy?
by Yoon, Won Tae and Youn, Jinyoung and Cho, Jin Whan
Journal of Neurology, ISSN 0340-5354, 8/2012, Volume 259, Issue 8, pp. 1694 - 1697
Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disease clinically characterized by the presence of cerebellar ataxia... 
CAG trinucleotide repeats | Neurology | Neurosciences | Medicine & Public Health | Cerebral white matter involvement | Dentatorubral-pallidoluysian atrophy (DRPLA) | Neuroradiology | CAG REPEAT | EXPANSIONS | TRINUCLEOTIDE | ATAXIA | CLINICAL NEUROLOGY | FAMILY | DENTATORUBROPALLIDOLUYSIAN ATROPHY | MULTIPLE SYSTEM ATROPHY | DRPLA | ANTICIPATION | Nerve Fibers, Myelinated - pathology | Humans | Middle Aged | Adult | Female | Male | Myoclonic Epilepsies, Progressive - genetics | Myoclonic Epilepsies, Progressive - diagnosis | Cerebrum - pathology | Atrophy | Cerebellum | Dentatorubral-pallidoluysian atrophy | Neurodegenerative diseases | Brain stem | Cerebellar ataxia | Differentiation | Substantia alba
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9. Full Text Dentatorubropallidoluysian atrophy without involuntary movement or dementia—A case report
by Kasahata, Naoki and Iwasaki, Yasuo
Clinical Neurology and Neurosurgery, ISSN 0303-8467, 2010, Volume 112, Issue 8, pp. 722 - 725
Abstract Recently, discussions about the clinical features of dentatorubropallidoluysian atrophy (DRPLA), especially the existence of an ataxo-choreoathetoid... 
Neurology | Neurosurgery | Dentatorubropallidoluysian atrophy (DRPLA) | MJD/SCA3 (Machado-Joseph disease) | Genotype–phenotype dissociation | Involuntary movement | Dementia | Genotype-phenotype dissociation | SURGERY | ATAXIA | CLINICAL NEUROLOGY | FAMILY | FEATURES | DRPLA | GENE | MACHADO-JOSEPH-DISEASE | EXPANSION | DENTATORUBRAL-PALLIDOLUYSIAN ATROPHY | Diagnosis, Differential | Humans | Middle Aged | Male | Myoclonic Epilepsies, Progressive - pathology | Gait Ataxia - etiology | Machado-Joseph Disease - complications | Dementia - complications | Gait Ataxia - pathology | Myoclonic Epilepsies, Progressive - complications | Brain - pathology | Dyskinesias - complications | Machado-Joseph Disease - pathology | Genotype & phenotype | Ataxia | Medical imaging | Disease | Medical treatment
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10. Full Text Homozygosity for an allele carrying intermediate CAG repeats in the dentatorubral-pallidoluysian atrophy (DRPLA) gene results in spastic paraplegia
by Kurohara, K and Kuroda, Y and Maruyama, H and Kawakami, H and Yukitake, M and Matsui, M and Nakamura, S
Neurology, ISSN 0028-3878, 1997, Volume 48, Issue 4, pp. 1087 - 1090
We report a family with autosomal recessive spastic paraplegia. Patient 1 was a 37-year-old woman and patient 2 was her 35-year-old sister. They showed spastic... 
DENTATORUBROPALLIDOLUYSIAN ATROPHY | PHENOTYPIC VARIATION | MACHADO-JOSEPH DISEASE | TRINUCLEOTIDE | EXPANSION | K RT CLINICAL NEUROLOGY | MYOCLONUS EPILEPSY | CLINICAL NEUROLOGY | HAW-RIVER-SYNDROME | FEATURES | FAMILY | Electrophoresis, Polyacrylamide Gel | Humans | Brain Diseases - genetics | Globus Pallidus - physiology | Paraplegia - genetics | Red Nucleus - physiology | Genotype | Genes, Recessive | Atrophy | Homozygote | Dentate Gyrus - physiology | Pedigree | Alleles | Polymerase Chain Reaction | Repetitive Sequences, Nucleic Acid | Adult | Female | Dentate Gyrus - pathology | Globus Pallidus - pathology | Red Nucleus - pathology
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11. Full Text Analysis of SCA1, DRPLA, MJD, SCA2, and SCA6 CAG repeats in 48 portuguese ataxia families
by Silveira, I and Coutinho, P and Maciel, P and Gaspar, C and Hayes, S and Dias, A and Guimarães, J and Loureiro, L and Sequeiros, J and Rouleau, G.A
American Journal of Medical Genetics, ISSN 0148-7299, 03/1998, Volume 81, Issue 2, pp. 134 - 138
The spinocerebellar ataxias (SCAs) are clinically and genetically a heterogeneous group of neurodegenerative disorders. To date, eight different loci causing... 
dentatorubropallidoluysian atrophy and Machado‐Joseph disease | spinocerebellar ataxia | Dentatorubropallidoluysian atrophy and Machado- Joseph disease | Spinocerebellar ataxia | PALLIDOLUYSIAN ATROPHY DRPLA | GENE | MACHADO-JOSEPH-DISEASE | EXPANSION | GENETICS & HEREDITY | DOMINANT CEREBELLAR-ATAXIA | HUNTINGTONS-DISEASE | LOCUS | dentatorubropallidoluysian atrophy and Machado-Joseph disease | ONSET | TRINUCLEOTIDE REPEAT | Trinucleotide Repeats | Ataxin-1 | Humans | Middle Aged | Family Health | Male | Spinocerebellar Degenerations - genetics | Nerve Tissue Proteins - genetics | Portugal | Proteins - genetics | Ataxins | Genes, Dominant | Adolescent | Age of Onset | Alleles | Adult | Female | Aged | Chromosomes, Human | Mutation | Nuclear Proteins - genetics | Calcium Channels - genetics | Machado-Joseph Disease - genetics
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12. Full Text Huntington's disease and other choreas
by Quinn, Niall and Schrag, Anette
Journal of Neurology, ISSN 0340-5354, 1998, Volume 245, Issue 11, pp. 709 - 716
Chorea can have many causes, some hereditary and many sporadic in nature. The archetypal hereditary cause of chorea is Huntington's disease (HD). However, this... 
Dentatorubro-pallidoluysian atrophy (DRPLA) | Benign hereditary chorea | Huntington's disease | Chorea | Neuroacanthocytosis | PHENOTYPIC VARIATION | neuroacanthocytosis | EXPANDED CAG REPEAT | SENILE CHOREA | CLINICAL NEUROLOGY | FAMILY | DENTATORUBROPALLIDOLUYSIAN ATROPHY | HD GENE | EXPANSION | chorea | dentatorubro-pallidoluysian atrophy (DRPLA) | INTRANUCLEAR INCLUSIONS | DENTATORUBRAL-PALLIDOLUYSIAN ATROPHY | benign hereditary chorea | Diagnosis, Differential | Vascular Diseases - complications | Humans | Huntington Disease - pathology | Pregnancy Complications | Autoimmune Diseases - complications | Chorea - pathology | Pregnancy | Huntington Disease - genetics | Female | Huntington Disease - diagnosis | Chorea - diagnosis | Chorea - genetics
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13. Full Text Head tremor in dentatorubral‐pallidoluysian atrophy
by Ohizumi, H and Okuma, Y and Fukae, J and Fujishima, K and Goto, K and Mizuno, Y
Acta Neurologica Scandinavica, ISSN 0001-6314, 11/2002, Volume 106, Issue 5, pp. 319 - 321
Dentatorubral‐pallidoluysian atrophy (DRPLA) is a rare autosomal‐dominant neurodegenerative disorder characterized by variable combination of clinical... 
essential tremor | head tremor | dystonia | dentatorubral‐pallidoluysian atrophy (DRPLA) | Dystonia | Dentatorubral-pallidoluysian atrophy (DRPLA) | Essential tremor | Head tremor | dentatorubral-pallidoluysian atrophy (DRPLA) | DENTATORUBROPALLIDOLUYSIAN ATROPHY | CAG REPEAT | DRPLA | EXPANSION | CLINICAL NEUROLOGY | FAMILY | Humans | Myoclonic Epilepsies, Progressive - complications | Tremor - genetics | Myoclonic Epilepsies, Progressive - physiopathology | Female | Myoclonic Epilepsies, Progressive - genetics | Aged | Electromyography | Head - physiopathology | Tremor - etiology | Tremor - physiopathology
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14. Full Text Hereditary dentatorubral–pallidoluysian atrophy
by Oyanagi, Shinsaku
Neuropathology, ISSN 0919-6544, 09/2000, Volume 20, Issue s1, pp. 42 - 46
Pathology and associated clinical symptoms of hereditary dentatorubral–pallidoluysian atrophy (H‐DRPLA) which was established as a new inherited... 
choreoathetosis | hereditary dentatorubral– pallidoluysian atrophy (H‐DRPLA) | myoclonus epilepsy | CAG repeat disease | autosomal dominant inheritance | Autosomal dominant inheritance | Choreoathetosis | Hereditary dentatorubral-pallidoluysian atrophy (H-DRPLA) | Myoclonus epilepsy | CAG REPEAT | hereditary dentatorubral-pallidoluysian atrophy (H-DRPLA) | INCLUSIONS | TRINUCLEOTIDE | PATHOLOGY | CLINICAL NEUROLOGY | DENTATORUBROPALLIDOLUYSIAN ATROPHY | DRPLA | EXPANSION | BRAIN | Subthalamic Nucleus - physiopathology | Humans | Nerve Degeneration - physiopathology | Nerve Degeneration - genetics | Cerebellar Nuclei - pathology | Myoclonic Epilepsies, Progressive - pathology | Nerve Degeneration - pathology | Subthalamic Nucleus - pathology | Cerebellar Nuclei - physiopathology | Red Nucleus - physiopathology | Myoclonic Epilepsies, Progressive - genetics | Globus Pallidus - physiopathology | Globus Pallidus - pathology | Red Nucleus - pathology
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15. Full Text Juvenile myoclonic epilepsy is not associated with the DRPLA gene in a European population
by Yapijakis, Christos and Gatzonis, Stergios and Youroukos, Sotirios and Kollia, Vasiliki and Karachristianou, Stella and Anagnostouli, Maria
In Vivo, ISSN 0258-851X, 11/2014, Volume 28, Issue 6, pp. 1193 - 1196
Background: Juvenile myoclonic epilepsy (JME), is an early-onset inherited generalized epilepsy which displays genetic heterogeneity, with at least 10 known... 
Trinucleotide repeat | Greek population | DRPLA | Juvenile myoclonic epilepsy | Dentato-rubro-pallidoluysian atrophy | MEDICINE, RESEARCH & EXPERIMENTAL | CAG REPEATS | FAMILY | DENTATORUBROPALLIDOLUYSIAN ATROPHY | SEIZURES | dentato-rubro-pallido-luysian atrophy | IDIOPATHIC GENERALIZED EPILEPSY | EXPANSION | trinucleotide repeat | SUSCEPTIBILITY LOCUS | MUTATIONS | DENTATORUBRAL-PALLIDOLUYSIAN ATROPHY | EPISODIC ATAXIA | Trinucleotide Repeats | European Continental Ancestry Group - genetics | Genetic Predisposition to Disease | Humans | Middle Aged | Male | Nerve Tissue Proteins - genetics | Case-Control Studies | Myoclonic Epilepsy, Juvenile - genetics | Polymorphism, Genetic | Young Adult | Adolescent | Adult | Female | Myoclonic Epilepsy, Juvenile - epidemiology
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16. Full Text Clinical and genetic characteristics of non‐Asian dentatorubral‐pallidoluysian atrophy: A systematic review
by Wardle, Mark and Morris, Huw R and Robertson, Neil P
Movement Disorders, ISSN 0885-3185, 08/2009, Volume 24, Issue 11, pp. 1636 - 1640
Dentatorubral‐pallidoluysian atrophy (DRPLA) is an inherited neurodegenerative disorder regarded as found almost exclusively among the Japanese. We have... 
spinocerebellar ataxia | CAG repeat | myoclonic epilepsy | dentatorubral‐pallidoluysian atrophy | Myoclonic epilepsy | Dentatorubral-pallidoluysian atrophy | Spinocerebellar ataxia | DANISH FAMILY | DRPLA GENE | ATAXIA | CLINICAL NEUROLOGY | FEATURES | TRINUCLEOTIDE CAG REPEAT | DENTATORUBROPALLIDOLUYSIAN ATROPHY | ORIGIN | JUVENILE | EXPANSION | dentatorubral-pallidoluysian atrophy | HUNTINGTONS-DISEASE | Trinucleotide Repeats | Diagnosis, Differential | European Continental Ancestry Group - genetics | Humans | Middle Aged | Myoclonic Epilepsies, Progressive - ethnology | African Americans - genetics | Child, Preschool | Infant | Male | Nerve Tissue Proteins - genetics | Young Adult | Phenotype | Adolescent | Age of Onset | Adult | Female | Myoclonic Epilepsies, Progressive - genetics | Aged | Anticipation, Genetic | Child
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17. Full Text Dentatorubral pallidoluysian atrophy in South Wales
by Wardle, M and Majounie, E and Williams, N M and Rosser, A E and Morris, H R and Robertson, N P
Journal of Neurology, Neurosurgery & Psychiatry, ISSN 0022-3050, 07/2008, Volume 79, Issue 7, pp. 804 - 807
Background:Dentatorubral pallidoluysian atrophy (DRPLA) is a rare, autosomal dominant, clinically heterogeneous neurodegenerative disorder characterised... 
SURGERY | FREQUENCIES | ALLELES | PSYCHIATRY | EXPANSIONS | ITALIAN FAMILIES | CLINICAL NEUROLOGY | DENTATORUBROPALLIDOLUYSIAN ATROPHY | ORIGIN | DRPLA | GENES | SPINOCEREBELLAR ATAXIA PATIENTS | CAG-REPEAT | European Continental Ancestry Group - genetics | Prevalence | Wales - epidemiology | Humans | Middle Aged | Myoclonic Epilepsies, Progressive - ethnology | European Continental Ancestry Group - statistics & numerical data | Male | Nerve Tissue Proteins - genetics | Case-Control Studies | Founder Effect | Pedigree | Myoclonic Epilepsies, Progressive - complications | Aged, 80 and over | Adult | Female | Myoclonic Epilepsies, Progressive - genetics | Polymorphism, Genetic - genetics | Aged | Genetic markers | Nervous system | Development and progression | Whites | Degeneration | Genetic aspects | Research
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18. Full Text Atrophy of the cerebellum and brainstem in dentatorubral pallidoluysian atrophy: Influence of CAG repeat size on MRI findings
by Koide, R and Onodera, O and Ikeuchi, T and Kondo, R and Tanaka, H and Tokiguchi, S and Tomoda, A and Miike, T and Isa, F and Beppu, H and Shimizu, N and Watanabe, Y and Horikawa, Y and Shimohata, T and Hirota, K and Ishikawa, A and Tsuji, Shoji
Neurology, ISSN 0028-3878, 1997, Volume 49, Issue 6, pp. 1605 - 1612
To elucidate how the size of the expanded CAG repeat of the gene for dentatorubral pallidoluysian atrophy (DRPLA) and other factors affect the atrophy of the... 
DENTATORUBROPALLIDOLUYSIAN ATROPHY | DRPLA | TRINUCLEOTIDE | EXPANSION | HUNTINGTONS-DISEASE | CLINICAL NEUROLOGY | FAMILY | Repetitive Sequences, Nucleic Acid - genetics | Neurodegenerative Diseases - pathology | Humans | Middle Aged | Neurodegenerative Diseases - diagnosis | Male | Neurodegenerative Diseases - genetics | Nerve Tissue Proteins - genetics | Cerebellum - pathology | Atrophy | Magnetic Resonance Imaging | Aging - physiology | Brain Stem - pathology | Adolescent | Adult | Female | Aged | Longitudinal Studies
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