{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,2]],"date-time":"2026-08-02T22:14:45Z","timestamp":1785708885266,"version":"3.56.0"},"reference-count":45,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,12,19]],"date-time":"2017-12-19T00:00:00Z","timestamp":1513641600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100004319","name":"Pfizer","doi-asserted-by":"publisher","award":["WI201057"],"award-info":[{"award-number":["WI201057"]}],"id":[{"id":"10.13039\/100004319","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain and Behavior"],"published-print":{"date-parts":[[2018,1]]},"abstract":"Abstract<\/jats:title>Background<\/jats:title>Familial transthyretin amyloidosis is a life\u2010threatening disease presenting with sensorimotor and autonomic polyneuropathy. Delayed diagnosis has a detrimental effect on treatment and prognosis. To facilitate diagnosis, we analyzed data patterns of patients with transthyretin familial amyloid polyneuropathy (TTR<\/jats:styled-content>\u2010FAP<\/jats:styled-content>) and compared them to polyneuropathies of different etiology for clinical and electrophysiological discriminators.<\/jats:p><\/jats:sec>Methods<\/jats:title>Twenty\u2010four patients with TTR<\/jats:styled-content>\u2010FAP<\/jats:styled-content> and 48 patients with diabetic polyneuropathy (dPNP<\/jats:styled-content>) were investigated (neurological impairment score NIS<\/jats:styled-content>; neurological disability score NDS<\/jats:styled-content>) in a cross\u2010sectional design. Both groups were matched for gender and presence of pain. Quantitative sensory testing (QST<\/jats:styled-content>), sympathetic skin response (SSR<\/jats:styled-content>), heart rate variability (HRV<\/jats:styled-content>), and nerve conduction studies (NCV<\/jats:styled-content>) were performed. Both groups were compared using univariate analysis. In a stepwise discriminant analysis, discriminators between both neuropathies were identified. These discriminators were validated comparing TTR<\/jats:styled-content>\u2010FAP<\/jats:styled-content> patients with a cohort of patients with chemotherapy\u2010induced polyneuropathy (CIN<\/jats:styled-content>) and chronic inflammatory demyelinating neuropathy (CIDP<\/jats:styled-content>).<\/jats:p><\/jats:sec>Results<\/jats:title>TTR<\/jats:styled-content>\u2010FAP<\/jats:styled-content> patients scored higher in NDS<\/jats:styled-content> and NIS<\/jats:styled-content> and had impaired cold detection (CDT<\/jats:styled-content>, p<\/jats:italic>\u00a0=\u00a0.024), cold\u2013warm discrimination (TSL<\/jats:styled-content>, p<\/jats:italic>\u00a0=\u00a0.019) and mechanical hyperalgesia (MPT<\/jats:styled-content>, p<\/jats:italic>\u00a0=\u00a0.029) at the hands, SSR<\/jats:styled-content> (upper limb, p<\/jats:italic>\u00a0=\u00a0.022) HRV<\/jats:styled-content> and ulnar and sural NCS<\/jats:styled-content> (all p<\/jats:italic>\u00a0<\u00a0.05) were more affected in TTR<\/jats:styled-content>\u2010FAP<\/jats:styled-content>. Ulnar nerve sensory NCV<\/jats:styled-content>, CDT<\/jats:styled-content>, and the MPT<\/jats:styled-content> but not the other parameters discriminated TTR<\/jats:styled-content>\u2010FAP<\/jats:styled-content> from dPNP<\/jats:styled-content> (82% of cases), from CIN<\/jats:styled-content> (86.7%) and from CIDP<\/jats:styled-content> (68%; only ulnar sNCV<\/jats:styled-content>).<\/jats:p><\/jats:sec>Conclusion<\/jats:title>Low ulnar SNCV<\/jats:styled-content>, impaired cold perception, and mechanical hyperalgesia at the hands seem to characterize TTR<\/jats:styled-content>\u2010FAP<\/jats:styled-content> and might help to differentiate from other polyneuropathies.<\/jats:p><\/jats:sec>","DOI":"10.1002\/brb3.889","type":"journal-article","created":{"date-parts":[[2017,12,19]],"date-time":"2017-12-19T07:56:42Z","timestamp":1513670202000},"source":"Crossref","is-referenced-by-count":22,"title":["Transthyretin familial amyloid polyneuropathy (TTR<\/scp>\u2010FAP<\/scp>): Parameters for early diagnosis"],"prefix":"10.1002","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8155-6497","authenticated-orcid":false,"given":"Fabiola","family":"Escolano\u2010Lozano","sequence":"first","affiliation":[{"name":"Department of Neurology University Medical Center of the Johannes Gutenberg University Mainz Mainz Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Paula","family":"Barreiros","sequence":"additional","affiliation":[{"name":"German Association for Liver Transplantation Mainz Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Birklein","sequence":"additional","affiliation":[{"name":"Department of Neurology University Medical Center of the Johannes Gutenberg University Mainz Mainz Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Geber","sequence":"additional","affiliation":[{"name":"Department of Neurology University Medical Center of the Johannes Gutenberg University Mainz Mainz Germany"},{"name":"Red Cross Pain Center Mainz Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,12,19]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000001870"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000000289"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-31"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/75.3.408"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.282.2.153"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/124.12.2439"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880110106"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410190503"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.45.6.1115"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000149522.32823.EA"},{"key":"e_1_2_8_12_1","first-page":"95","article-title":"The natural history of diabetic autonomic neuropathy","volume":"49","author":"Ewing D. 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