Ontology type: schema:ScholarlyArticle Open Access: True
2014-12
AUTHORSPtolemaios G Sarrigiannis, Nigel Hoggard, Daniel Aeschlimann, David S Sanders, Richard A Grünewald, Zoe C Unwin, Marios Hadjivassiliou
ABSTRACTBACKGROUND: Cortical myoclonus with ataxia has only rarely been reported in association with Coeliac Disease (CD). Such reports also suggested that it is unresponsive to gluten-free diet. We present detailed electro-clinical characteristics of a new syndrome of progressive cortical hyperexcitability with ataxia and refractory CD. At our gluten/neurology clinic we have assessed and regularly follow up over 600 patients with neurological manifestations due to gluten sensitivity. We have identified 9 patients with this syndrome. RESULTS: All 9 patients (6 male, 3 female) experienced asymmetrical irregular myoclonus involving one or more limbs and sometimes face. This was often stimulus sensitive and became more widespread over time. Three patients had a history of Jacksonian march and five had at least one secondarily generalised seizure. Electrophysiology showed evidence of cortical myoclonus. Three had a phenotype of epilepsia partialis continua at onset. There was clinical, imaging and/or pathological evidence of cerebellar involvement in all cases. All patients adhered to a strict gluten-free diet with elimination of gluten-related antibodies in most. However, there was still evidence of enteropathy in all, suggestive of refractory celiac disease. Two died from enteropathy-associated lymphoma and one from status epilepticus. Five patients were treated with mycophenolate and one in addition with rituximab and IV immunoglobulins. Their ataxia and enteropathy improved but myoclonus remained the most disabling feature of their illness. CONCLUSIONS: This syndrome may well be the commonest neurological manifestation of refractory CD. The clinical involvement, apart from ataxia, covers the whole clinical spectrum of cortical myoclonus. More... »
PAGES11
http://scigraph.springernature.com/pub.10.1186/2053-8871-1-11
DOIhttp://dx.doi.org/10.1186/2053-8871-1-11
DIMENSIONShttps://app.dimensions.ai/details/publication/pub.1010244213
PUBMEDhttps://www.ncbi.nlm.nih.gov/pubmed/26331035
JSON-LD is the canonical representation for SciGraph data.
TIP: You can open this SciGraph record using an external JSON-LD service: JSON-LD Playground Google SDTT
[
{
"@context": "https://springernature.github.io/scigraph/jsonld/sgcontext.json",
"about": [
{
"id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/1109",
"inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/",
"name": "Neurosciences",
"type": "DefinedTerm"
},
{
"id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/11",
"inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/",
"name": "Medical and Health Sciences",
"type": "DefinedTerm"
}
],
"author": [
{
"affiliation": {
"alternateName": "Royal Hallamshire Hospital",
"id": "https://www.grid.ac/institutes/grid.416126.6",
"name": [
"Departments of Neurophysiology, Royal Hallamshire Hospital, S10 2JF, Sheffield, UK"
],
"type": "Organization"
},
"familyName": "Sarrigiannis",
"givenName": "Ptolemaios G",
"id": "sg:person.01132771412.11",
"sameAs": [
"https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01132771412.11"
],
"type": "Person"
},
{
"affiliation": {
"alternateName": "Royal Hallamshire Hospital",
"id": "https://www.grid.ac/institutes/grid.416126.6",
"name": [
"Neuroradiology, Royal Hallamshire Hospital, S10 2JF, Sheffield, UK"
],
"type": "Organization"
},
"familyName": "Hoggard",
"givenName": "Nigel",
"id": "sg:person.0610025364.39",
"sameAs": [
"https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0610025364.39"
],
"type": "Person"
},
{
"affiliation": {
"alternateName": "Cardiff University",
"id": "https://www.grid.ac/institutes/grid.5600.3",
"name": [
"College of Biomedical and Life Sciences, Cardiff University, CF14 4XY, Cardiff, UK"
],
"type": "Organization"
},
"familyName": "Aeschlimann",
"givenName": "Daniel",
"id": "sg:person.01007061523.54",
"sameAs": [
"https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01007061523.54"
],
"type": "Person"
},
{
"affiliation": {
"alternateName": "Royal Hallamshire Hospital",
"id": "https://www.grid.ac/institutes/grid.416126.6",
"name": [
"Gastroenterology, Royal Hallamshire Hospital, S10 2JF, Sheffield, UK"
],
"type": "Organization"
},
"familyName": "Sanders",
"givenName": "David S",
"id": "sg:person.016071637312.37",
"sameAs": [
"https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.016071637312.37"
],
"type": "Person"
},
{
"affiliation": {
"alternateName": "Royal Hallamshire Hospital",
"id": "https://www.grid.ac/institutes/grid.416126.6",
"name": [
"Neurology, Royal Hallamshire Hospital, S10 2JF, Sheffield, UK"
],
"type": "Organization"
},
"familyName": "Gr\u00fcnewald",
"givenName": "Richard A",
"id": "sg:person.01130424341.44",
"sameAs": [
"https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01130424341.44"
],
"type": "Person"
},
{
"affiliation": {
"alternateName": "Royal Hallamshire Hospital",
"id": "https://www.grid.ac/institutes/grid.416126.6",
"name": [
"Departments of Neurophysiology, Royal Hallamshire Hospital, S10 2JF, Sheffield, UK"
],
"type": "Organization"
},
"familyName": "Unwin",
"givenName": "Zoe C",
"id": "sg:person.01162355373.58",
"sameAs": [
"https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01162355373.58"
],
"type": "Person"
},
{
"affiliation": {
"alternateName": "Royal Hallamshire Hospital",
"id": "https://www.grid.ac/institutes/grid.416126.6",
"name": [
"Neurology, Royal Hallamshire Hospital, S10 2JF, Sheffield, UK"
],
"type": "Organization"
},
"familyName": "Hadjivassiliou",
"givenName": "Marios",
"id": "sg:person.01354423242.83",
"sameAs": [
"https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01354423242.83"
],
"type": "Person"
}
],
"citation": [
{
"id": "https://doi.org/10.1093/brain/awg050",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1000547915"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1002/mds.22576",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1006600012"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1002/mds.22576",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1006600012"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1016/j.clinph.2008.03.016",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1009057249"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1371/journal.pone.0009698",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1012024760"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1093/brain/119.2.393",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1017611115"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1002/mds.870010306",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1023768017"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1002/mus.20234",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1024117043"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1001/archneur.1977.00500170020003",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1029142705"
],
"type": "CreativeWork"
},
{
"id": "sg:pub.10.1186/1741-7015-10-13",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1035374109",
"https://doi.org/10.1186/1741-7015-10-13"
],
"type": "CreativeWork"
},
{
"id": "sg:pub.10.1007/bf00314464",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1039767984",
"https://doi.org/10.1007/bf00314464"
],
"type": "CreativeWork"
},
{
"id": "sg:pub.10.1007/bf00314464",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1039767984",
"https://doi.org/10.1007/bf00314464"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1111/j.1600-0404.2010.01356.x",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1043103838"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1016/j.bpg.2005.02.001",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1043465266"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1016/j.bpg.2005.02.001",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1043465266"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1016/j.seizure.2012.04.003",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1045936543"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1053/j.gastro.2008.09.069",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1047014122"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1016/0022-510x(82)90076-4",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1050043968"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1016/0022-510x(82)90076-4",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1050043968"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1093/brain/108.1.193",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1059437751"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1093/brain/118.5.1087",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1059439521"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1093/brain/89.4.683",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1059443877"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1212/wnl.30.3.245",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1064363370"
],
"type": "CreativeWork"
},
{
"id": "https://doi.org/10.1212/wnl.54.6.1350",
"sameAs": [
"https://app.dimensions.ai/details/publication/pub.1064378519"
],
"type": "CreativeWork"
}
],
"datePublished": "2014-12",
"datePublishedReg": "2014-12-01",
"description": "BACKGROUND: Cortical myoclonus with ataxia has only rarely been reported in association with Coeliac Disease (CD). Such reports also suggested that it is unresponsive to gluten-free diet. We present detailed electro-clinical characteristics of a new syndrome of progressive cortical hyperexcitability with ataxia and refractory CD. At our gluten/neurology clinic we have assessed and regularly follow up over 600 patients with neurological manifestations due to gluten sensitivity. We have identified 9 patients with this syndrome.\nRESULTS: All 9 patients (6 male, 3 female) experienced asymmetrical irregular myoclonus involving one or more limbs and sometimes face. This was often stimulus sensitive and became more widespread over time. Three patients had a history of Jacksonian march and five had at least one secondarily generalised seizure. Electrophysiology showed evidence of cortical myoclonus. Three had a phenotype of epilepsia partialis continua at onset. There was clinical, imaging and/or pathological evidence of cerebellar involvement in all cases. All patients adhered to a strict gluten-free diet with elimination of gluten-related antibodies in most. However, there was still evidence of enteropathy in all, suggestive of refractory celiac disease. Two died from enteropathy-associated lymphoma and one from status epilepticus. Five patients were treated with mycophenolate and one in addition with rituximab and IV immunoglobulins. Their ataxia and enteropathy improved but myoclonus remained the most disabling feature of their illness.\nCONCLUSIONS: This syndrome may well be the commonest neurological manifestation of refractory CD. The clinical involvement, apart from ataxia, covers the whole clinical spectrum of cortical myoclonus.",
"genre": "research_article",
"id": "sg:pub.10.1186/2053-8871-1-11",
"inLanguage": [
"en"
],
"isAccessibleForFree": true,
"isPartOf": [
{
"id": "sg:journal.1051116",
"issn": [
"2053-8871"
],
"name": "Cerebellum & Ataxias",
"type": "Periodical"
},
{
"issueNumber": "1",
"type": "PublicationIssue"
},
{
"type": "PublicationVolume",
"volumeNumber": "1"
}
],
"name": "Myoclonus ataxia and refractory coeliac disease",
"pagination": "11",
"productId": [
{
"name": "readcube_id",
"type": "PropertyValue",
"value": [
"30ce0685ef9e5da726930e16a8cd42576a24b6016bc12e8f65f2a0c9a0796db8"
]
},
{
"name": "pubmed_id",
"type": "PropertyValue",
"value": [
"26331035"
]
},
{
"name": "nlm_unique_id",
"type": "PropertyValue",
"value": [
"101648460"
]
},
{
"name": "doi",
"type": "PropertyValue",
"value": [
"10.1186/2053-8871-1-11"
]
},
{
"name": "dimensions_id",
"type": "PropertyValue",
"value": [
"pub.1010244213"
]
}
],
"sameAs": [
"https://doi.org/10.1186/2053-8871-1-11",
"https://app.dimensions.ai/details/publication/pub.1010244213"
],
"sdDataset": "articles",
"sdDatePublished": "2019-04-10T23:23",
"sdLicense": "https://scigraph.springernature.com/explorer/license/",
"sdPublisher": {
"name": "Springer Nature - SN SciGraph project",
"type": "Organization"
},
"sdSource": "s3://com-uberresearch-data-dimensions-target-20181106-alternative/cleanup/v134/2549eaecd7973599484d7c17b260dba0a4ecb94b/merge/v9/a6c9fde33151104705d4d7ff012ea9563521a3ce/jats-lookup/v90/0000000001_0000000264/records_8693_00000510.jsonl",
"type": "ScholarlyArticle",
"url": "http://link.springer.com/10.1186%2F2053-8871-1-11"
}
]
Download the RDF metadata as: json-ld nt turtle xml License info
JSON-LD is a popular format for linked data which is fully compatible with JSON.
curl -H 'Accept: application/ld+json' 'https://scigraph.springernature.com/pub.10.1186/2053-8871-1-11'
N-Triples is a line-based linked data format ideal for batch operations.
curl -H 'Accept: application/n-triples' 'https://scigraph.springernature.com/pub.10.1186/2053-8871-1-11'
Turtle is a human-readable linked data format.
curl -H 'Accept: text/turtle' 'https://scigraph.springernature.com/pub.10.1186/2053-8871-1-11'
RDF/XML is a standard XML format for linked data.
curl -H 'Accept: application/rdf+xml' 'https://scigraph.springernature.com/pub.10.1186/2053-8871-1-11'
This table displays all metadata directly associated to this object as RDF triples.
178 TRIPLES
21 PREDICATES
49 URIs
21 LITERALS
9 BLANK NODES