{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T12:31:23Z","timestamp":1789821083588,"version":"4.0.1"},"reference-count":80,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2015,1,27]],"date-time":"2015-01-27T00:00:00Z","timestamp":1422316800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Dr. Senckenbergische Stiftung (Frankfurt\/Main, Germany)"},{"name":"Deutsche Huntington Hilfe e.V."},{"name":"Huntington-Selbsthilfe Nordrhein-Westfalen e.V"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Pathology"],"published-print":{"date-parts":[[2015,11]]},"abstract":"Abstract<\/jats:title>\n \n Huntington's disease (\n HD<\/jats:styled-content>\n ), an autosomal dominantly inherited polyglutamine or\n CAG<\/jats:styled-content>\n repeat disease along with somatomotor, oculomotor, psychiatric and cognitive symptoms, presents clinically with impairments of elementary and complex visual functions as well as altered visual\u2010evoked potentials (\n VEPs<\/jats:styled-content>\n ). Previous volumetric and pathoanatomical post\u2010mortem investigations pointed to an involvement of Brodmann's primary visual area 17 (\n BA<\/jats:styled-content>\n 17) in\n HD<\/jats:styled-content>\n . Because the involvement of\n BA<\/jats:styled-content>\n 17 could be interpreted as an early onset brain neurodegeneration, we further characterized this potential primary cortical site of\n HD<\/jats:styled-content>\n \u2010related neurodegeneration neuropathologically and performed an unbiased estimation of the absolute nerve cell number in thick gallocyanin\u2010stained frontoparallel tissue sections through the striate area of seven control individuals and seven\n HD<\/jats:styled-content>\n patients using\n C<\/jats:styled-content>\n avalieri's principle for volume and the optical disector for nerve and glial cell density estimations. This investigation showed a reduction of the estimated absolute nerve cell number of\n BA<\/jats:styled-content>\n 17 in the\n HD<\/jats:styled-content>\n patients (71\u2009044\u2009037\u2009\u00b1\u200912\u2009740\u2009515 nerve cells) of 32% in comparison with the control individuals (104\u2009075\u2009067\u2009\u00b1\u20099\u2009424\u2009491 nerve cells) (\n M<\/jats:styled-content>\n ann\u2013\n W<\/jats:styled-content>\n hitney\n \n U<\/jats:italic>\n <\/jats:styled-content>\n \u2010test;\n P<\/jats:italic>\n \u2009<\u20090.001). Additional pathoanatomical studies showed that nerve cell loss was most prominent in the outer pyramidal layer\n III<\/jats:styled-content>\n , the inner granular layers\n IVa<\/jats:styled-content>\n and\n IVc<\/jats:styled-content>\n as well as in the multiform layer\n VI<\/jats:styled-content>\n of\n BA<\/jats:styled-content>\n 17 of the\n HD<\/jats:styled-content>\n patients. Our neuropathological results in\n BA<\/jats:styled-content>\n 17 confirm and extend previous post\u2010mortem, biochemical and\n in vivo<\/jats:italic>\n neuroradiological\n HD<\/jats:styled-content>\n findings and offer suitable explanations for the elementary and complex visual dysfunctions, as well as for the altered\n VEP<\/jats:styled-content>\n observed in\n HD<\/jats:styled-content>\n patients.\n <\/jats:p>","DOI":"10.1111\/bpa.12237","type":"journal-article","created":{"date-parts":[[2014,12,12]],"date-time":"2014-12-12T03:13:31Z","timestamp":1418354011000},"page":"701-711","source":"Crossref","is-referenced-by-count":28,"title":["Huntington's Disease (HD): Neurodegeneration of Brodmann's Primary Visual Area 17 (BA17)"],"prefix":"10.1111","volume":"25","author":[{"given":"Udo","family":"R\u00fcb","sequence":"first","affiliation":[{"name":"Dr. Senckenbergisches Chronomedizinisches Institut Goethe\u2010University Frankfurt\/Main 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