Literature DB >> 18695063

Clinical and neuropathologic findings in a woman with the FMR1 premutation and multiple sclerosis.

Claudia M Greco1, Flora Tassone, Dolores Garcia-Arocena, Nicole Tartaglia, Sarah M Coffey, Timothy K Vartanian, James A Brunberg, Paul J Hagerman, Randi J Hagerman.   

Abstract

BACKGROUND: Multiple sclerosis (MS) and fragile X-associated tremor/ataxia syndrome (FXTAS) have overlapping clinical signs and symptoms.
OBJECTIVES: To present a case with evidence of both MS and FXTAS and to discuss the relationship of both disorders.
DESIGN: Case report.
SETTING: Fragile X Research and Treatment Center at the University of California, Davis, Medical Center. Patient Woman with the FMR1 premutation who died of MS at the age of 52 years. MAIN OUTCOME MEASURES: Magnetic resonance imaging, physical examination, and neuropathologic examination results.
RESULTS: Magnetic resonance imaging, physical examination, and autopsy neuropathologic examination revealed diagnostic features of MS and FXTAS.
CONCLUSION: The molecular mechanism of RNA toxicity, including the elevation of alphaB-crystallin levels observed in FXTAS, may lead to enhanced predisposition to autoimmune diseases.

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Year:  2008        PMID: 18695063      PMCID: PMC3081275          DOI: 10.1001/archneur.65.8.1114

Source DB:  PubMed          Journal:  Arch Neurol        ISSN: 0003-9942


  12 in total

1.  Expression of small heat shock proteins and intermediate filaments in the human optic nerve head astrocytes exposed to elevated hydrostatic pressure in vitro.

Authors:  M Salvador-Silva; C S Ricard; O A Agapova; P Yang; M R Hernandez
Journal:  J Neurosci Res       Date:  2001-10-01       Impact factor: 4.164

2.  Axonal damage in acute multiple sclerosis lesions.

Authors:  B Ferguson; M K Matyszak; M M Esiri; V H Perry
Journal:  Brain       Date:  1997-03       Impact factor: 13.501

3.  Initial diagnoses given to persons with the fragile X associated tremor/ataxia syndrome (FXTAS).

Authors:  D A Hall; E Berry-Kravis; S Jacquemont; C D Rice; J Cogswell; L Zhang; R J Hagerman; P J Hagerman; M A Leehey
Journal:  Neurology       Date:  2005-07-26       Impact factor: 9.910

4.  Induction of inclusion formation and disruption of lamin A/C structure by premutation CGG-repeat RNA in human cultured neural cells.

Authors:  Dolores Garcia Arocena; Christine K Iwahashi; Nelly Won; Alexandra Beilina; Anna L Ludwig; Flora Tassone; Philip H Schwartz; Paul J Hagerman
Journal:  Hum Mol Genet       Date:  2005-10-20       Impact factor: 6.150

5.  The small heat-shock protein alpha B-crystallin as candidate autoantigen in multiple sclerosis.

Authors:  J M van Noort; A C van Sechel; J J Bajramovic; M el Ouagmiri; C H Polman; H Lassmann; R Ravid
Journal:  Nature       Date:  1995-06-29       Impact factor: 49.962

6.  Expanded clinical phenotype of women with the FMR1 premutation.

Authors:  Sarah M Coffey; Kylee Cook; Nicole Tartaglia; Flora Tassone; Danh V Nguyen; Ruiqin Pan; Hannah E Bronsky; Jennifer Yuhas; Mariya Borodyanskaya; Jim Grigsby; Melanie Doerflinger; Paul J Hagerman; Randi J Hagerman
Journal:  Am J Med Genet A       Date:  2008-04-15       Impact factor: 2.802

Review 7.  The fragile-X premutation: a maturing perspective.

Authors:  Paul J Hagerman; Randi J Hagerman
Journal:  Am J Hum Genet       Date:  2004-03-29       Impact factor: 11.025

8.  Intermediate filament interactions can be altered by HSP27 and alphaB-crystallin.

Authors:  M D Perng; L Cairns; P van den IJssel; A Prescott; A M Hutcheson; R A Quinlan
Journal:  J Cell Sci       Date:  1999-07       Impact factor: 5.285

Review 9.  Diagnostic criteria for multiple sclerosis: 2005 revisions to the "McDonald Criteria".

Authors:  Chris H Polman; Stephen C Reingold; Gilles Edan; Massimo Filippi; Hans-Peter Hartung; Ludwig Kappos; Fred D Lublin; Luanne M Metz; Henry F McFarland; Paul W O'Connor; Magnhild Sandberg-Wollheim; Alan J Thompson; Brian G Weinshenker; Jerry S Wolinsky
Journal:  Ann Neurol       Date:  2005-12       Impact factor: 10.422

10.  Protective and therapeutic role for alphaB-crystallin in autoimmune demyelination.

Authors:  Shalina S Ousman; Beren H Tomooka; Johannes M van Noort; Eric F Wawrousek; Kevin C O'Connor; David A Hafler; Raymond A Sobel; William H Robinson; Lawrence Steinman
Journal:  Nature       Date:  2007-06-13       Impact factor: 49.962

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  35 in total

1.  Immune mediated disorders in women with a fragile X expansion and FXTAS.

Authors:  Isha Jalnapurkar; Nuva Rafika; Flora Tassone; Randi Hagerman
Journal:  Am J Med Genet A       Date:  2014-11-14       Impact factor: 2.802

2.  Mouse models of the fragile x premutation and the fragile X associated tremor/ataxia syndrome.

Authors:  Michael R Hunsaker; Gloria Arque; Robert F Berman; Rob Willemsen; Renate K Hukema
Journal:  Results Probl Cell Differ       Date:  2012

3.  Microglial cell activation and senescence are characteristic of the pathology FXTAS.

Authors:  Verónica Martínez Cerdeño; Tiffany Hong; Sarwat Amina; Mirna Lechpammer; Jeanelle Ariza; Flora Tassone; Stephen C Noctor; Paul Hagerman; Randi Hagerman
Journal:  Mov Disord       Date:  2018-12-10       Impact factor: 10.338

4.  Dysregulated iron metabolism in the choroid plexus in fragile X-associated tremor/ataxia syndrome.

Authors:  Jeanelle Ariza; Craig Steward; Flora Rueckert; Matt Widdison; Robert Coffman; Atiyeh Afjei; Stephen C Noctor; Randi Hagerman; Paul Hagerman; Verónica Martínez-Cerdeño
Journal:  Brain Res       Date:  2014-12-09       Impact factor: 3.252

5.  Induced expression of expanded CGG RNA causes mitochondrial dysfunction in vivo.

Authors:  Renate K Hukema; Ronald A M Buijsen; Chris Raske; Lies Anne Severijnen; Ingeborg Nieuwenhuizen-Bakker; Michelle Minneboo; Alex Maas; Rini de Crom; Johan M Kros; Paul J Hagerman; Robert F Berman; Rob Willemsen
Journal:  Cell Cycle       Date:  2014       Impact factor: 4.534

Review 6.  Fragile X: a family of disorders.

Authors:  Weerasak Chonchaiya; Andrea Schneider; Randi J Hagerman
Journal:  Adv Pediatr       Date:  2009

7.  Immune-mediated disorders among women carriers of fragile X premutation alleles.

Authors:  Tri Indah Winarni; Weerasak Chonchaiya; Tanjung Ayu Sumekar; Paul Ashwood; Guadalupe Mendoza Morales; Flora Tassone; Danh V Nguyen; Sultana M H Faradz; Judy Van de Water; Kylee Cook; Alyssa Hamlin; Yi Mu; Paul J Hagerman; Randi J Hagerman
Journal:  Am J Med Genet A       Date:  2012-08-17       Impact factor: 2.802

8.  Fragile X and autism: Intertwined at the molecular level leading to targeted treatments.

Authors:  Randi Hagerman; Gry Hoem; Paul Hagerman
Journal:  Mol Autism       Date:  2010-09-21       Impact factor: 7.509

9.  Autoimmune disease in mothers with the FMR1 premutation is associated with seizures in their children with fragile X syndrome.

Authors:  Weerasak Chonchaiya; Flora Tassone; Paul Ashwood; David Hessl; Andrea Schneider; Luis Campos; Danh V Nguyen; Randi J Hagerman
Journal:  Hum Genet       Date:  2010-09-01       Impact factor: 4.132

Review 10.  Fragile X-associated tremor/ataxia syndrome - features, mechanisms and management.

Authors:  Randi J Hagerman; Paul Hagerman
Journal:  Nat Rev Neurol       Date:  2016-06-24       Impact factor: 42.937

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