{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,3,27]],"date-time":"2024-03-27T11:13:12Z","timestamp":1711537992076},"reference-count":45,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2020,8,10]],"date-time":"2020-08-10T00:00:00Z","timestamp":1597017600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>In this study, we aimed to investigate the progression of peripheral nervous system involvement in xeroderma pigmentosum group A (XP\u2010A).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We performed nerve conduction studies in 17 genetically confirmed XP\u2010A patients and conducted follow\u2010ups. Of these patients we also analyzed gray matter volume (GMV) using brain MRI and assessed the severity score of clinical and skin manifestation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We found significant reduction in the motor and sensory nerve action potential amplitude and mild reduction in conduction velocity. These findings were predominant in sensory nerves and the lower limbs, were observed since early childhood, and gradually deteriorated with age.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The electrophysiological characteristics of XP\u2010A patients are consistent with length\u2010dependent axonal polyneuropathy and there is progressive deterioration from early childhood.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27028","type":"journal-article","created":{"date-parts":[[2020,7,22]],"date-time":"2020-07-22T06:07:05Z","timestamp":1595398025000},"page":"534-540","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Progressive length\u2010dependent polyneuropathy in xeroderma pigmentosum group A"],"prefix":"10.1002","volume":"62","author":[{"given":"Yukio","family":"Tsuji","sequence":"first","affiliation":[{"name":"Division of Neurology Kobe University Graduate School of Medicine  Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takehiro","family":"Ueda","sequence":"additional","affiliation":[{"name":"Division of Neurology Kobe University Graduate School of Medicine  Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kenji","family":"Sekiguchi","sequence":"additional","affiliation":[{"name":"Division of Neurology Kobe University Graduate School of Medicine  Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masahiro","family":"Nishiyama","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Kobe University Graduate School of Medicine  Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fumio","family":"Kanda","sequence":"additional","affiliation":[{"name":"Kusunoki clinic  Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chikako","family":"Nishigori","sequence":"additional","affiliation":[{"name":"Division of Dermatology Kobe University Graduate School of Medicine  Kobe Hyogo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatsushi","family":"Toda","sequence":"additional","affiliation":[{"name":"Department of Neurology The University of Tokyo Graduate School of Medicine  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Riki","family":"Matsumoto","sequence":"additional","affiliation":[{"name":"Division of Neurology Kobe University Graduate School of Medicine  Kobe Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,8,10]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-6-70"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-80-2-221"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mrfmmm.2006.06.010"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.72.1.219"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2010.083022"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(94)70270-5"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0625.2008.00763.x"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/114.3.1335"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awn153"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1993.tb00154.x"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/php.13052"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroscience.2006.10.025"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.55.10.1442"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1994.01690020057009"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1987.01660260111026"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdermsci.2012.10.008"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.13907"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awn126"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2017.08.3238"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/exd.13082"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26499"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1529-8027.2010.00269.x"},{"key":"e_1_2_9_24_1","first-page":"143","article-title":"Xeroderma pigmentosum with disorders of the central nervous system; 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