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Most often neurological signs and symptoms predominate and hypogonadism remains undetected and untreated, while in other cases, a thorough evaluation of a hypogonadal male reveals previously unrecognized ataxia, movement disorder, muscle weakness, tremor, or seizures, leading to a syndromic diagnosis. Androgen deficiency in patients with neurological diseases may aggravate muscle weakness and fatigue and predispose patients to osteoporosis and obesity. The purpose of this mini review is to provide a current understanding of the clinical, biochemical, histologic, and genetic features of syndromes in which male hypogonadism and neurological dysfunction may coexist and may be encountered by the clinical endocrinologist.<\/jats:p>","DOI":"10.1210\/clinem\/dgac421","type":"journal-article","created":{"date-parts":[[2022,8,1]],"date-time":"2022-08-01T11:18:59Z","timestamp":1659352739000},"page":"e3974-e3989","source":"Crossref","is-referenced-by-count":4,"title":["Hypogonadism in Males With Genetic Neurodevelopmental Syndromes"],"prefix":"10.1210","volume":"107","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3721-7388","authenticated-orcid":false,"given":"Stephen J","family":"Winters","sequence":"first","affiliation":[{"name":"Division of Endocrinology, Metabolism & Diabetes, University of Louisville , Louisville, KY , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2022,8,1]]},"reference":[{"issue":"2","key":"2022092808390954000_CIT0001","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1038\/nrneurol.2017.175","article-title":"Adulthood leukodystrophies","volume":"14","author":"K\u00f6hler","year":"2018","journal-title":"Nat Rev Neurol."},{"key":"2022092808390954000_CIT0002","doi-asserted-by":"crossref","first-page":"102665","DOI":"10.1016\/j.msard.2020.102665","article-title":"MRI signs of CNS demyelinating diseases","volume":"47","author":"Etemadifar","year":"2021","journal-title":"Mult Scler Relat Disord."},{"issue":"2","key":"2022092808390954000_CIT0003","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1016\/j.gene.2015.08.029","article-title":"MMACHC gene mutation in familial hypogonadism with neurological symptoms","volume":"574","author":"Shi","year":"2015","journal-title":"Gene."},{"issue":"4","key":"2022092808390954000_CIT0004","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1210\/endrev\/bnaa013","article-title":"The changing face of adrenoleukodystrophy","volume":"41","author":"Zhu","year":"2020","journal-title":"Endocr Rev."},{"issue":"4","key":"2022092808390954000_CIT0005","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1023\/A:1022535930009","article-title":"X-linked adrenoleukodystrophy: genes, mutations, and phenotypes","volume":"24","author":"Smith","year":"1999","journal-title":"Neurochem Res."},{"issue":"10","key":"2022092808390954000_CIT0006","doi-asserted-by":"crossref","first-page":"606","DOI":"10.1038\/nrendo.2016.90","article-title":"Adrenoleukodystrophy\u2014neuroendocrine pathogenesis and redefinition of natural history","volume":"12","author":"Kemp","year":"2016","journal-title":"Nat Rev Endocrinol."},{"issue":"4","key":"2022092808390954000_CIT0007","doi-asserted-by":"crossref","first-page":"528","DOI":"10.1016\/j.jpeds.2004.10.067","article-title":"Adrenal insufficiency in asymptomatic adrenoleukodystrophy patients identified by very long-chain fatty acid screening","volume":"146","author":"Dubey","year":"2005","journal-title":"J Pediatr."},{"issue":"6","key":"2022092808390954000_CIT0008","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1507\/endocrj.EJ19-0576","article-title":"A 29-year-old patient with adrenoleukodystrophy presenting with Addison\u2019s disease","volume":"67","author":"Tanaka","year":"2020","journal-title":"Endocr J."},{"issue":"3","key":"2022092808390954000_CIT0009","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1111\/cen.12159","article-title":"Screening for X-linked adrenoleukodystrophy among adult men with Addison\u2019s disease","volume":"79","author":"Horn","year":"2013","journal-title":"Clin Endocrinol (Oxf)."},{"issue":"1","key":"2022092808390954000_CIT0010","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1210\/jcem-66-1-33","article-title":"Monocytes stimulate cortisol production by cultured human adrenocortical cells","volume":"66","author":"Whitcomb","year":"1988","journal-title":"J Clin Endocrinol Metab."},{"issue":"2","key":"2022092808390954000_CIT0011","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/j.ecl.2016.01.003","article-title":"Endocrine dysfunction in X-linked adrenoleukodystrophy","volume":"45","author":"Burtman","year":"2016","journal-title":"Endocrinol Metab Clin North Am."},{"issue":"12","key":"2022092808390954000_CIT0012","doi-asserted-by":"crossref","first-page":"1107","DOI":"10.1212\/WNL.27.12.1107","article-title":"Adrenomyeloneuropathy: a probable variant of adrenoleukodystrophy. 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