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We have observed a subset of patients who instead presented initially with urinary retention requiring catheterisation. At presentation, these patients had only subtle neurological signs that would not fulfil the diagnostic criteria of MSA; however, the anal sphincter electromyography (EMG) was abnormal and they reported bowel and sexual dysfunction, suggesting localisation at the level of the sacral spinal cord. They subsequently developed classical neurological signs, meeting the diagnostic criteria for probable MSA. One patient was confirmed to have MSA at autopsy. We postulate that in a subset of patients with MSA, the disease begins in the sacral spinal cord and then spreads to other regions resulting in the classical signs of MSA. The transmissibility of alpha-synuclein has been demonstrated in animal models and the spread of pathology from sacral cord to other regions of the central nervous system is therefore plausible. Patients presenting with urinary retention and mild neurological features would be an ideal group for experimental trials evaluating neuroprotection in MSA<\/jats:p>","DOI":"10.1007\/s00415-019-09597-2","type":"journal-article","created":{"date-parts":[[2019,12,5]],"date-time":"2019-12-05T07:14:36Z","timestamp":1575530076000},"page":"659-664","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":44,"title":["Early presentation of urinary retention in multiple system atrophy: can the disease begin in the sacral spinal cord?"],"prefix":"10.1007","volume":"267","author":[{"given":"Jalesh N.","family":"Panicker","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1513-8136","authenticated-orcid":false,"given":"Sara","family":"Simeoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuo","family":"Miki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amit","family":"Batla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Iodice","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janice L.","family":"Holton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryuji","family":"Sakakibara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas T.","family":"Warner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2019,11,12]]},"reference":[{"key":"9597_CR1","doi-asserted-by":"publisher","first-page":"670","DOI":"10.1212\/01.wnl.0000324625.00404.15","volume":"71","author":"S Gilman","year":"2008","unstructured":"Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ, Wood NW, Colosimo C, Durr A, Fowler CJ, Kaufmann H, Klockgether T, Lees A, Poewe W, Quinn N, Revesz T, Robertson D, Sandroni P, Seppi K, Vidailhet M (2008) Second consensus statement on the diagnosis of multiple system atrophy. 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