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The clinical picture was consistent with a possible diagnosis of Alzheimer\u2019s Disease associated with parkinsonian symptoms or with a Parkinson Plus syndrome. After a complete investigation, she was found to have a high plasma level of homocysteine due to homozygous methylene-tetrahydrofolate reductase (MTHFR) gene C665 T polymorphism, cognitive and motor impairment were associated with a severe cortical atrophy and mild subcortical vascular disease. PET neuroimaging excluded a significant amyloid load. Clinically, she showed improvement of the movement disorder and functional status after folate integration plus levodopa and memantine administration. We concluded for a primary degenerative dementia with movement impairment associated with persistent hyperhomocysteinemia. We hypothesized that neurodegeneration is driven by mechanisms linked to homocysteine metabolism possibly associated with tauopathy.<\/jats:p>","DOI":"10.1177\/0891988720988910","type":"journal-article","created":{"date-parts":[[2021,1,19]],"date-time":"2021-01-19T04:30:13Z","timestamp":1611030613000},"page":"57-61","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":3,"title":["Hyperhomocysteinemia and Dementia Associated With Severe Cortical Atrophy, but No Amyloid Burden"],"prefix":"10.1177","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0879-2076","authenticated-orcid":false,"given":"Alessandro","family":"Reffo","sequence":"first","affiliation":[{"name":"Department of Medicine (DIMED), Geriatrics Division, University of Padova, Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carlo","family":"Gabelli","sequence":"additional","affiliation":[{"name":"Regionale Brain Aging Center, Department of Medicine (DIMED), University of Padova, Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2021,1,19]]},"reference":[{"key":"bibr1-0891988720988910","unstructured":"Alzheimer\u2019s Disease International. 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