{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,27]],"date-time":"2023-10-27T07:42:55Z","timestamp":1698392575473},"reference-count":11,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2016,2,4]],"date-time":"2016-02-04T00:00:00Z","timestamp":1454544000000},"content-version":"vor","delay-in-days":7704,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Neuroimaging"],"published-print":{"date-parts":[[1995,1]]},"abstract":"<jats:p>Late\u2010onset GM, gangliosidosis, a rare inherited neuronal storage disease, is characterized by a vanety of clinical manifestations. The common clinical picture comprises neuromuscular, spinocerebellar, extrapyramidal, cognitive, and psychiatric abnormalities. Details of the extent of muscle involvement have never been reported. Eight patients with this syndrome were evaluated for the existence and extent of motor neuron disease using routine electrodiagnosis and systematic evaluation of skeletal musculature by computed tomography. Motor neuron disease was present in each and every patient regardless of the clmical manifestations and to a degree beyond that suspected on neurological examination. Muscle imaging disclosed a diffuse wasting and fatty replacement of muscles with predilection of pelvic and thigh muscles, and especially the quadriceps group. It seems that progressive motor disability in this syndrome IS mainly due to motor neuron disease, as manifested by muscle atrophy, which can be easily demonstrated by muscle computed tomography.<\/jats:p>","DOI":"10.1111\/jon1995514","type":"journal-article","created":{"date-parts":[[2016,2,8]],"date-time":"2016-02-08T10:33:14Z","timestamp":1454927594000},"page":"4-8","source":"Crossref","is-referenced-by-count":1,"title":["Muscle Computed Tomography Features of Motor Neuron Disease in Late\u2010Onset GM<sub>2<\/sub> Gangliosidosis"],"prefix":"10.1111","volume":"5","author":[{"given":"Jonathan Y.","family":"Streifler","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Herzlia","family":"Hadar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natan","family":"Gadoth","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2016,2,4]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320240123"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410160105"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1192\/bjp.155.3.410"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.43.10.2055"},{"key":"e_1_2_1_6_1","first-page":"1087","article-title":"Fatty replacement of lower paraspinal muscles normal and neuromuscular disorders","volume":"4","author":"Hadar H","year":"1983","journal-title":"AJNR"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1976.00500020048008"},{"key":"e_1_2_1_8_1","first-page":"611","article-title":"An amyotrophic lateral sclerosis-like syndrome with hexosaminidase-A deficiency a new type of GM2 gangliosidosis","volume":"29","author":"Yaffe MG","year":"1979","journal-title":"Neurology"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410110103"},{"key":"e_1_2_1_10_1","unstructured":"ABBaker Clinical neurology vol 3. Hagerstown MD: Harper & Row 1978; 40: 37-41"},{"key":"e_1_2_1_11_1","doi-asserted-by":"crossref","unstructured":"JALBuckle ALBaert Clinical and radiological aspects of myopathies. Berlin: Springer 1982","DOI":"10.1007\/978-3-662-02354-9"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/116.1.217"}],"container-title":["Journal of Neuroimaging"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjon1995514","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjon1995514","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jon1995514","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,26]],"date-time":"2023-10-26T19:00:03Z","timestamp":1698346803000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jon1995514"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1995,1]]},"references-count":11,"journal-issue":{"issue":"1","published-print":{"date-parts":[[1995,1]]}},"alternative-id":["10.1111\/jon1995514"],"URL":"https:\/\/doi.org\/10.1111\/jon1995514","archive":["Portico"],"relation":{},"ISSN":["1051-2284","1552-6569"],"issn-type":[{"value":"1051-2284","type":"print"},{"value":"1552-6569","type":"electronic"}],"subject":[],"published":{"date-parts":[[1995,1]]}}}