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The patient had a <jats:styled-content style=\"fixed-case\">HLA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">B<\/jats:styled-content>51 serotype, a recent history of uveitis and had suffered paraparesis, sensory and autonomic disturbance, frontal signs and tremor. <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content> brain and spine MRI study revealed a longitudinally extensive thoracic cord (<jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>h) lesion, but no apparent intracranial abnormalities. The lesion extended ventrally from <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>h4 to <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>h9, exhibiting low intensity on <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>1\u2010weighted images, high intensity on <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>2\u2010weighted and fluid\u2010attenuated inversion recovery images and gadolinium enhancement. The patient's upper and lower motor neuron signs and sensory disturbance worsened and he died 16 months after admission. At autopsy, the spinal cord and brain exhibited characteristic histopathological features of both <jats:styled-content style=\"fixed-case\">NBD<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">ALS<\/jats:styled-content>, including chronic destruction of the ventral thoracic white and gray matter, perivascular lymphocytic infiltration, binucleated neurons, lower and upper motor neuron degeneration, <jats:styled-content style=\"fixed-case\">B<\/jats:styled-content>unina bodies and skein\u2010like inclusions. Although incidental coexistence of these rare disorders could occur in an individual, this case raises the possibility of a pathomechanistic association between <jats:styled-content style=\"fixed-case\">NBD<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">ALS<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/neup.12074","type":"journal-article","created":{"date-parts":[[2013,10,12]],"date-time":"2013-10-12T01:20:18Z","timestamp":1381540818000},"page":"185-189","source":"Crossref","is-referenced-by-count":5,"title":["Coexistence of amyotrophic lateral sclerosis with neuro\u2010<scp>B<\/scp>eh\u00e7et's disease presenting as a longitudinally extensive spinal cord lesion: clinicopathologic features of an autopsied patient"],"prefix":"10.1111","volume":"34","author":[{"given":"Tomoe","family":"Sato","sequence":"first","affiliation":[{"name":"Department of Neurology Niigata City General Hospital  Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haruka","family":"Ouchi","sequence":"additional","affiliation":[{"name":"Department of Neurology Niigata City General Hospital  Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junsuke","family":"Shimbo","sequence":"additional","affiliation":[{"name":"Department of Neurology Niigata City General Hospital  Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aki","family":"Sato","sequence":"additional","affiliation":[{"name":"Department of Neurology Niigata City General Hospital  Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Motoyoshi","family":"Yamazaki","sequence":"additional","affiliation":[{"name":"Department of Neurology Niigata City General Hospital  Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hideki","family":"Hashidate","sequence":"additional","affiliation":[{"name":"Department of Pathology Niigata City General Hospital  Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuichi","family":"Igarashi","sequence":"additional","affiliation":[{"name":"Department of Neurology Niigata City General Hospital  Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akiyoshi","family":"Kakita","sequence":"additional","affiliation":[{"name":"Department of Pathology Brain Research Institute University of Niigata  Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,9,30]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/122.11.2171"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/122.11.2183"},{"key":"e_1_2_4_4_1","first-page":"1015","article-title":"CNS involvement in neuro\u2010Beh\u00e7et's syndrome: an MR study","volume":"20","author":"Ko\u00e7er N","year":"1999","journal-title":"Am J Neuroradiol"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2007.01754.x"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(09)70015-8"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.5692\/clinicalneurol.48.48"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.49.2175"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.1179\/2045772311Y.0000000050"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(11)70015-1"},{"key":"e_1_2_4_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-011-1923-y"},{"key":"e_1_2_4_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10048-008-0164-y"},{"key":"e_1_2_4_13_1","first-page":"1078","article-title":"Criteria for diagnosis of Beh\u00e7et's disease","volume":"335","author":"International Study Group for Beh\u00e7et's Disease","year":"1990","journal-title":"Lancet"},{"key":"e_1_2_4_14_1","first-page":"96","article-title":"El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. 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