Literature DB >> 32089651

Fragile X- associated Neuropsychiatric Disorders: A Case Report.

Maria Melinda Tan1,2,3, Jeanne Barbara Dy1,2,4, Maria Jimena Salcedo-Arellano1,5, Flora Tassone1,6, Randi J Hagerman1,5.   

Abstract

Mutations in the Fragile X Mental Retardation 1 (FMR1) gene create a spectrum of developmental disorders in children in addition to neurodegenerative problems in older populations. Two types of mutations are recognized in the FMR1 gene. The full mutation (>200 CGG repeats) in the FMR1 gene leads to Fragile X Syndrome which is the most common inherited cause of intellectual disability and autism, while the premutation (55 to 200 CGG repeats) identified among carriers leads to a range of problems linked to elevated levels of the FMR1 mRNA leading to mRNA toxicity and occasionally mildly deficient FMRP levels. Two disorders among premutation carriers have been recognized namely: the Fragile X-associated Primary Ovarian Insufficiency (FXPOI) and Fragile X-associated Tremor/Ataxia Syndrome (FXTAS). Recently, in order to recognize a group of associated disorders commonly found in premutation carriers and extensively reported in co-morbidities studies, a new distinctive name was proposed: Fragile X-associated Neuropsychiatric Disorders (FXAND). This paper will present a case report of a female premutation carrier who has encountered predominantly psychiatric problems, but also chronic pain and sleep disturbances consistent with FXAND.

Entities:  

Keywords:  FMR1; FXAND; Fragile X-associated Neuropsychiatric Disorders; anxiety; depression; premutation carrier

Year:  2019        PMID: 32089651      PMCID: PMC7034938          DOI: 10.2217/fnl-2018-0040

Source DB:  PubMed          Journal:  Future Neurol        ISSN: 1479-6708


  39 in total

1.  Decreased functional brain response to emotional arousal and increased psychiatric symptomology in FMR1 premutation carriers.

Authors:  Stephanie S G Brown; Heather C Whalley; Peter C Kind; Andrew C Stanfield
Journal:  Psychiatry Res Neuroimaging       Date:  2019-01-29       Impact factor: 2.376

2.  Autism spectrum disorders and attention-deficit/hyperactivity disorder in boys with the fragile X premutation.

Authors:  Faraz Farzin; Hazel Perry; David Hessl; Danuta Loesch; Jonathan Cohen; Susan Bacalman; Louise Gane; Flora Tassone; Paul Hagerman; Randi Hagerman
Journal:  J Dev Behav Pediatr       Date:  2006-04       Impact factor: 2.225

3.  Fibromyalgia in fragile X mental retardation 1 gene premutation carriers.

Authors:  Maureen A Leehey; Wendi Legg; Flora Tassone; Randi Hagerman
Journal:  Rheumatology (Oxford)       Date:  2011-09-16       Impact factor: 7.580

Review 4.  Premature ovarian failure in the fragile X syndrome.

Authors:  S L Sherman
Journal:  Am J Med Genet       Date:  2000

5.  Towards an Understanding of Neuropsychiatric Manifestations in Fragile X Premutation Carriers.

Authors:  Aaron D Besterman; Scott A Wilke; Tua-Elisabeth Mulligan; Stephen C Allison; Randi Hagerman; Andreea L Seritan; James A Bourgeois
Journal:  Future Neurol       Date:  2014-03

6.  Fragile-X-associated tremor/ataxia syndrome (FXTAS) in females with the FMR1 premutation.

Authors:  R J Hagerman; B R Leavitt; F Farzin; S Jacquemont; C M Greco; J A Brunberg; F Tassone; D Hessl; S W Harris; L Zhang; T Jardini; L W Gane; J Ferranti; L Ruiz; M A Leehey; J Grigsby; P J Hagerman
Journal:  Am J Hum Genet       Date:  2004-04-02       Impact factor: 11.025

7.  Penetrance of the fragile X-associated tremor/ataxia syndrome in a premutation carrier population.

Authors:  Sébastien Jacquemont; Randi J Hagerman; Maureen A Leehey; Deborah A Hall; Richard A Levine; James A Brunberg; Lin Zhang; Tristan Jardini; Louise W Gane; Susan W Harris; Kristin Herman; James Grigsby; Claudia M Greco; Elizabeth Berry-Kravis; Flora Tassone; Paul J Hagerman
Journal:  JAMA       Date:  2004-01-28       Impact factor: 56.272

8.  Molecular Advances Leading to Treatment Implications for Fragile X Premutation Carriers.

Authors:  Jonathan Polussa; Andrea Schneider; Randi Hagerman
Journal:  Brain Disord Ther       Date:  2014

9.  FMR1 CGG allele size and prevalence ascertained through newborn screening in the United States.

Authors:  Flora Tassone; Ka Pou Iong; Tzu-Han Tong; Joyce Lo; Louise W Gane; Elizabeth Berry-Kravis; Danh Nguyen; Lisa Y Mu; Jennifer Laffin; Don B Bailey; Randi J Hagerman
Journal:  Genome Med       Date:  2012-12-21       Impact factor: 11.117

10.  Translation of Expanded CGG Repeats into FMRpolyG Is Pathogenic and May Contribute to Fragile X Tremor Ataxia Syndrome.

Authors:  Chantal Sellier; Ronald A M Buijsen; Fang He; Sam Natla; Laura Jung; Philippe Tropel; Angeline Gaucherot; Hugues Jacobs; Hamid Meziane; Alexandre Vincent; Marie-France Champy; Tania Sorg; Guillaume Pavlovic; Marie Wattenhofer-Donze; Marie-Christine Birling; Mustapha Oulad-Abdelghani; Pascal Eberling; Frank Ruffenach; Mathilde Joint; Mathieu Anheim; Veronica Martinez-Cerdeno; Flora Tassone; Rob Willemsen; Renate K Hukema; Stéphane Viville; Cecile Martinat; Peter K Todd; Nicolas Charlet-Berguerand
Journal:  Neuron       Date:  2017-01-05       Impact factor: 18.688

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  2 in total

1.  Psychosis and Catatonia in Fragile X Syndrome.

Authors:  Mitra Keshtkarjahromi; Karishma Palvadi; Aayush Shah; Kendall R Dempsey; Silvina Tonarelli
Journal:  Cureus       Date:  2021-01-21

Review 2.  Fragile X Premutation: Medications, Therapy and Lifestyle Advice.

Authors:  Deepika Kour Sodhi; Randi Hagerman
Journal:  Pharmgenomics Pers Med       Date:  2021-12-29
  2 in total

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