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An unusual cause of adult onset cerebellar ataxia with hypogonadism

We report an unusual case of sporadic adult onset cerebellar ataxia with hypogonadism. A 40-year-old unmarried man presented with progressive ataxia and dysarthria along with complaints of non-development of secondary sexual characteristics and erectile dysfunction. There were complaints of intermit...

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Autores principales: Menon, Ramshekhar N., Sanghani, Nirav, Javali, Mahendra, Jain, Neeraj, Shah, Arun B.
Formato: Texto
Lenguaje:English
Publicado: Medknow Publications 2009
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2811977/
https://www.ncbi.nlm.nih.gov/pubmed/20151008
http://dx.doi.org/10.4103/0972-2327.48852
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author Menon, Ramshekhar N.
Sanghani, Nirav
Javali, Mahendra
Jain, Neeraj
Shah, Arun B.
author_facet Menon, Ramshekhar N.
Sanghani, Nirav
Javali, Mahendra
Jain, Neeraj
Shah, Arun B.
author_sort Menon, Ramshekhar N.
collection PubMed
description We report an unusual case of sporadic adult onset cerebellar ataxia with hypogonadism. A 40-year-old unmarried man presented with progressive ataxia and dysarthria along with complaints of non-development of secondary sexual characteristics and erectile dysfunction. There were complaints of intermittent diarrhea. Clinical examination revealed a pan-cerebellar syndrome with features of hypoandrogenism. No eye movement abnormalities were evident. There were signs of malabsorption. Investigations confirmed the presence of auto-antibodies found in celiac disease, and a duodenal biopsy confirmed the same. Hypoandrogenism was postulated to be due to hypergonadotropic hypogonadism which has been mentioned in a few patients of celiac disease. However, the pattern seen in our patient was of a hypogonadotropic hypogonadism. This is probably secondary to an autoimmune hypophysitis seen in some patients in the absence of other clinical manifestations. Autoantibody testing should be a diagnostic necessity in any adult with a sporadic cerebellar ataxia.
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spelling pubmed-28119772010-02-11 An unusual cause of adult onset cerebellar ataxia with hypogonadism Menon, Ramshekhar N. Sanghani, Nirav Javali, Mahendra Jain, Neeraj Shah, Arun B. Ann Indian Acad Neurol Case Report We report an unusual case of sporadic adult onset cerebellar ataxia with hypogonadism. A 40-year-old unmarried man presented with progressive ataxia and dysarthria along with complaints of non-development of secondary sexual characteristics and erectile dysfunction. There were complaints of intermittent diarrhea. Clinical examination revealed a pan-cerebellar syndrome with features of hypoandrogenism. No eye movement abnormalities were evident. There were signs of malabsorption. Investigations confirmed the presence of auto-antibodies found in celiac disease, and a duodenal biopsy confirmed the same. Hypoandrogenism was postulated to be due to hypergonadotropic hypogonadism which has been mentioned in a few patients of celiac disease. However, the pattern seen in our patient was of a hypogonadotropic hypogonadism. This is probably secondary to an autoimmune hypophysitis seen in some patients in the absence of other clinical manifestations. Autoantibody testing should be a diagnostic necessity in any adult with a sporadic cerebellar ataxia. Medknow Publications 2009 /pmc/articles/PMC2811977/ /pubmed/20151008 http://dx.doi.org/10.4103/0972-2327.48852 Text en © Annals of Indian Academy of Neurology http://creativecommons.org/licenses/by/2.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Menon, Ramshekhar N.
Sanghani, Nirav
Javali, Mahendra
Jain, Neeraj
Shah, Arun B.
An unusual cause of adult onset cerebellar ataxia with hypogonadism
title An unusual cause of adult onset cerebellar ataxia with hypogonadism
title_full An unusual cause of adult onset cerebellar ataxia with hypogonadism
title_fullStr An unusual cause of adult onset cerebellar ataxia with hypogonadism
title_full_unstemmed An unusual cause of adult onset cerebellar ataxia with hypogonadism
title_short An unusual cause of adult onset cerebellar ataxia with hypogonadism
title_sort unusual cause of adult onset cerebellar ataxia with hypogonadism
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2811977/
https://www.ncbi.nlm.nih.gov/pubmed/20151008
http://dx.doi.org/10.4103/0972-2327.48852
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