{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T13:44:49Z","timestamp":1790775889884,"version":"4.1.0"},"reference-count":64,"publisher":"Cambridge University Press (CUP)","issue":"2","license":[{"start":{"date-parts":[[2017,2,24]],"date-time":"2017-02-24T00:00:00Z","timestamp":1487894400000},"content-version":"unspecified","delay-in-days":5778,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Can. j. neurol. sci."],"published-print":{"date-parts":[[2001,5]]},"abstract":"<jats:title>ABSTRACT:<\/jats:title><jats:sec id=\"S0317167100052811_abs1\"><jats:title>Background:<\/jats:title><jats:p>The aims of the present study were: i) to measure levels of the dopamine metabolite homovanillic acid (HVA), the serotonin metabolite 5-hydroxindoleacetic acid (5HIAA) and precursor tryptophan, as well as the noradrenaline metabolite 3-methoxy-4-hydroxyphenylethylene glycol (MHPG) and thiamine in the cerebrospinal fluid (CSF) of patients with Friedreich's ataxia (FA), olivopontocerebellar atrophy (OPCA), and the autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSAC), as compared with sex- and age-matched control subjects.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167100052811_abs2\"><jats:title>Patients and methods:<\/jats:title><jats:p>CSF amine related compound levels and thiamine results were compared in 40 FA, 44 OPCAand nine ARSAC patients with those of 94 sex- and age-matched subjects. Neuroimaging (CT scans and single photon emission computed tomographies i.e. SPECT) were carried out in all patients and controls. Genetic studies were conducted on OPCApatients. CSF amine related compounds were measured by high performance liquid chromatography, whereas CSF thiamine levels were measured by a microbiological method.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167100052811_abs3\"><jats:title>Results:<\/jats:title><jats:p>FA patients had significantly lower CSF HVA, 5HIAA and thiamine values than control patients and a trend for lower MHPG levels. In OPCA patients, CSF HVA, MHPG and thiamine values were markedly lower whereas CSF 5HIAA values showed only a trend towards lower levels; in ARSAC patients only thiamine and HVA CSF values were lower than those in control subjects.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167100052811_abs4\"><jats:title>Conclusion:<\/jats:title><jats:p>After presenting the relationships between neurochemical findings on one side, the degree of ataxia, the degree of cerebellar atrophy and the SPECT findings on the other, the authors concluded that replacement and neuroprotective clinical trials in these patients would have to include two or three drugs because the neurotransmitter deficiencies are multiple.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s0317167100052811","type":"journal-article","created":{"date-parts":[[2017,3,3]],"date-time":"2017-03-03T07:05:32Z","timestamp":1488524732000},"page":"134-140","source":"Crossref","is-referenced-by-count":19,"title":["Biogenic Amine Metabolites and Thiamine in Cerebrospinal Fluid in Heredo-Degenerative Ataxias"],"prefix":"10.1017","volume":"28","author":[{"given":"M.I.","family":"Botez","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.N.","family":"Young","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2017,2,24]]},"reference":[{"key":"S0317167100052811_ref010","doi-asserted-by":"publisher","DOI":"10.1007\/BF00180841"},{"key":"S0317167100052811_ref004","doi-asserted-by":"crossref","first-page":"2129","DOI":"10.1523\/JNEUROSCI.07-07-02129.1987","article-title":"Depletion of dopamine in the caudate nucleus but not in nucleus accumbens impairs reaction-time performance in rats","volume":"7","author":"Amalric","year":"1987","journal-title":"J Neurosci"},{"key":"S0317167100052811_ref001","doi-asserted-by":"publisher","DOI":"10.1620\/tjem.150.435"},{"key":"S0317167100052811_ref058","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1995.00540340074015"},{"key":"S0317167100052811_ref002","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.39.5.749-b"},{"key":"S0317167100052811_ref045","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(92)90721-I"},{"key":"S0317167100052811_ref014","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.28.7.691"},{"key":"S0317167100052811_ref017","first-page":"333","article-title":"Effects of anticonvulsant treatment and low levels of folate and thiamine on amine metabolites in cerebrospinal fluid","volume":"114","author":"Botez","year":"1991","journal-title":"Brain"},{"key":"S0317167100052811_ref037","first-page":"39","volume-title":"The Olivopontocerebellar Atrophies","author":"Huang","year":"1984"},{"key":"S0317167100052811_ref027","first-page":"173","article-title":"Cerebello-cerebral diaschisis","volume":"40","author":"Botez","year":"1990","journal-title":"Neurology"},{"key":"S0317167100052811_ref029","doi-asserted-by":"publisher","DOI":"10.1161\/01.STR.23.4.511"},{"key":"S0317167100052811_ref008","first-page":"367","article-title":"Amantadine and ketamine-induced improvement of motor coordination in lurcher mutant mice","volume":"5","author":"Lalonde","year":"1993","journal-title":"Restor Neurol Neurosci"},{"key":"S0317167100052811_ref024","doi-asserted-by":"publisher","DOI":"10.1148\/116.1.85"},{"key":"S0317167100052811_ref034","first-page":"59","article-title":"Metabolism of thiamin and its phosphoric esters in different regions of the nervous system: a new approach","volume":"4","author":"Rindi","year":"1982","journal-title":"Acta Vitaminol Enzymol"},{"key":"S0317167100052811_ref030","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1111\/j.1600-0404.1993.tb05507.x","article-title":"Reduced contralateral hemispheric flow measured by SPECT in cerebellar lesions: crossed cerebral diaschisis","volume":"87","author":"Sonmezoglu","year":"1993","journal-title":"Acta Neurol Scand"},{"key":"S0317167100052811_ref009","doi-asserted-by":"publisher","DOI":"10.1126\/science.715450"},{"key":"S0317167100052811_ref062","doi-asserted-by":"publisher","DOI":"10.1016\/0014-2999(89)90385-3"},{"key":"S0317167100052811_ref039","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100022873"},{"key":"S0317167100052811_ref003","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.51.7.914"},{"key":"S0317167100052811_ref018","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100027785"},{"key":"S0317167100052811_ref007","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(93)90279-V"},{"key":"S0317167100052811_ref032","first-page":"117","volume-title":"Clinical Vitaminology: Methods and Interpretation","author":"Baker","year":"1968"},{"key":"S0317167100052811_ref040","first-page":"311","volume-title":"Serotonin, the Cerebellum and Ataxia","author":"Trouillas","year":"1992"},{"key":"S0317167100052811_ref054","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(77)90757-0"},{"key":"S0317167100052811_ref052","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(84)91240-X"},{"key":"S0317167100052811_ref013","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.55.2.136"},{"key":"S0317167100052811_ref016","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.45.8.731"},{"key":"S0317167100052811_ref019","doi-asserted-by":"publisher","DOI":"10.1159\/000116959"},{"key":"S0317167100052811_ref021","first-page":"37","volume-title":"Molecular Genetics of Neurological and Neuromuscular Disease: Advances in Neurology","author":"Harding","year":"1988"},{"key":"S0317167100052811_ref005","doi-asserted-by":"publisher","DOI":"10.1152\/jn.1987.58.5.1100"},{"key":"S0317167100052811_ref023","first-page":"399","volume-title":"Handbook of Cerebellar Disease","author":"Bouchard","year":"1992"},{"key":"S0317167100052811_ref026","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(87)90207-2"},{"key":"S0317167100052811_ref047","doi-asserted-by":"publisher","DOI":"10.1016\/0022-3956(86)90002-6"},{"key":"S0317167100052811_ref028","doi-asserted-by":"publisher","DOI":"10.1159\/000116705"},{"key":"S0317167100052811_ref031","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100040956"},{"key":"S0317167100052811_ref033","first-page":"205","article-title":"Blood thiamine and blood folate levels. A comparative study in control, alcoholic and folatedeficient subjects","volume":"51","author":"Bachevalier","year":"1981","journal-title":"Int J Vitam Nutr Res"},{"key":"S0317167100052811_ref022","first-page":"774","article-title":"Confirmation of the SCA-2 locus as an alternative locus for dominantly inherited spinocerebellar ataxias and refinement of the candidate region","volume":"54","author":"Lopes-Cendes","year":"1994","journal-title":"Am J Hum Genet"},{"key":"S0317167100052811_ref035","first-page":"112","article-title":"Amperometric determination of 3-methoxy-4-hydroxyphenyl-ethyleneglycol in human cerebrospinal fluid","volume":"222","author":"Anderson","year":"1981"},{"key":"S0317167100052811_ref041","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.42.8.1573"},{"key":"S0317167100052811_ref043","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(91)90398-D"},{"key":"S0317167100052811_ref044","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(90)91065-O"},{"key":"S0317167100052811_ref046","doi-asserted-by":"publisher","DOI":"10.1016\/0891-0618(96)00115-9"},{"key":"S0317167100052811_ref048","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1984.tb02813.x"},{"key":"S0317167100052811_ref049","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(89)90504-0"},{"key":"S0317167100052811_ref050","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(84)90518-3"},{"key":"S0317167100052811_ref006","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(91)90669-M"},{"key":"S0317167100052811_ref051","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(84)91239-3"},{"key":"S0317167100052811_ref053","first-page":"1081","article-title":"Ultrastructural features of early brain stem lesions of thiamine-deficient rats","volume":"52","author":"Robertson","year":"1968","journal-title":"Am J Pathol"},{"key":"S0317167100052811_ref055","first-page":"112","article-title":"L-5-hydroxytryptophan in the cerebellar syndrome treatment","volume":"35","author":"Rascol","year":"1981","journal-title":"Biomedicine"},{"key":"S0317167100052811_ref056","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1988.00520350055016"},{"key":"S0317167100052811_ref057","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1995.00540290037015"},{"key":"S0317167100052811_ref015","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(85)91243-0"},{"key":"S0317167100052811_ref059","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(05)65674-7"},{"key":"S0317167100052811_ref060","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100031863"},{"key":"S0317167100052811_ref020","first-page":"75","article-title":"Sur un sympt\u00f4me caract\u00e9ristique d\u2019une l\u00e9sion des noyaux centraux moteurs: la rigidit\u00e9 musculaire latente","volume":"63","author":"Noica","year":"1935","journal-title":"Rev Neurol"},{"key":"S0317167100052811_ref011","doi-asserted-by":"publisher","DOI":"10.1016\/0278-2626(91)90001-O"},{"key":"S0317167100052811_ref061","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.61.3.259"},{"key":"S0317167100052811_ref064","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.12-11-04427.1992"},{"key":"S0317167100052811_ref063","doi-asserted-by":"publisher","DOI":"10.1016\/0014-2999(89)90384-1"},{"key":"S0317167100052811_ref012","first-page":"33","volume-title":"Advances in Neurology","author":"Oertel","year":"1993"},{"key":"S0317167100052811_ref038","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/94.2.359"},{"key":"S0317167100052811_ref036","doi-asserted-by":"publisher","DOI":"10.1016\/S0378-4347(00)81553-4"},{"key":"S0317167100052811_ref025","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.126.3.593"},{"key":"S0317167100052811_ref042","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.490290411"}],"container-title":["Canadian Journal of Neurological Sciences \/ Journal Canadien des Sciences Neurologiques"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S0317167100052811","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,5,8]],"date-time":"2019-05-08T00:27:09Z","timestamp":1557275229000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S0317167100052811\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2001,5]]},"references-count":64,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2001,5]]}},"alternative-id":["S0317167100052811"],"URL":"https:\/\/doi.org\/10.1017\/s0317167100052811","relation":{},"ISSN":["0317-1671","2057-0155"],"issn-type":[{"value":"0317-1671","type":"print"},{"value":"2057-0155","type":"electronic"}],"subject":[],"published":{"date-parts":[[2001,5]]}}}