Literature DB >> 24120273

Cerebral involvement in spinal and bulbar muscular atrophy (Kennedy's disease): a pilot study of PET.

Tzu-Hsien Lai1, Ren-Shyan Liu, Bang-Hung Yang, Po-Shan Wang, Kon-Ping Lin, Yi-Chung Lee, Bing-Wen Soong.   

Abstract

OBJECTIVE: To investigate possible cerebral involvement in patients with spinal and bulbar muscular atrophy (SBMA) by (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET).
DESIGN: Ten patients with molecularly-confirmed SBMA and 5 age- and gender-matched healthy controls were recruited for brain FDG-PET studies. The data were analyzed and compared using the statistical parametric mapping (SPM) method.
RESULTS: Glucose hypometabolism in frontal areas of the cerebrum was found in patients with SBMA. However, no significant correlation with clinical variables, such as CAG repeat length, age at onset, or serum testosterone levels, was noted.
CONCLUSIONS: The perturbation of cerebral glucose metabolism in patients with SBMA argues against SBMA being a pure lower motor and sensory neuron syndrome. Mutations in the androgen receptor gene might have a more widespread effect in the cerebrum than previously recognized.
© 2013. Published by Elsevier B.V. All rights reserved.

Entities:  

Keywords:  Androgen receptor; Kennedy's disease; Motor neuron disease; Polyglutamine disease; Positron emission tomography; Spinal and bulbar muscular atrophy

Mesh:

Substances:

Year:  2013        PMID: 24120273     DOI: 10.1016/j.jns.2013.09.016

Source DB:  PubMed          Journal:  J Neurol Sci        ISSN: 0022-510X            Impact factor:   3.181


  13 in total

Review 1.  Onset Manifestations of Spinal and Bulbar Muscular Atrophy (Kennedy's Disease).

Authors:  Josef Finsterer; Gianni Soraru
Journal:  J Mol Neurosci       Date:  2015-10-19       Impact factor: 3.444

2.  Theory of mind, empathy and neuropsychological functioning in X-linked spinal and bulbar muscular atrophy: a controlled study of 20 patients.

Authors:  Elisa Di Rosa; Gianni Sorarù; Johann Roland Kleinbub; Vincenzo Calvo; Antonino Vallesi; Giorgia Querin; Sonia Marcato; Irene Grasso; Arianna Palmieri
Journal:  J Neurol       Date:  2014-11-20       Impact factor: 4.849

3.  Combined brain and spinal FDG PET allows differentiation between ALS and ALS mimics.

Authors:  Donatienne Van Weehaeghe; Martijn Devrome; Michel Koole; Koen Van Laere; Georg Schramm; Joke De Vocht; Wies Deckers; Kristof Baete; Philip Van Damme
Journal:  Eur J Nucl Med Mol Imaging       Date:  2020-04-20       Impact factor: 9.236

Review 4.  Kennedy's disease (spinal and bulbar muscular atrophy): a clinically oriented review of a rare disease.

Authors:  Marianthi Breza; Georgios Koutsis
Journal:  J Neurol       Date:  2018-07-13       Impact factor: 4.849

5.  Multimodal structural MRI in the diagnosis of motor neuron diseases.

Authors:  Pilar M Ferraro; Federica Agosta; Nilo Riva; Massimiliano Copetti; Edoardo Gioele Spinelli; Yuri Falzone; Gianni Sorarù; Giancarlo Comi; Adriano Chiò; Massimo Filippi
Journal:  Neuroimage Clin       Date:  2017-08-02       Impact factor: 4.881

6.  Regional glucose hypometabolic spread within the primary motor cortex is associated with amyotrophic lateral sclerosis disease progression: A fluoro-deoxyglucose positron emission tomography study.

Authors:  Hironobu Endo; Kenji Sekiguchi; Takehiro Ueda; Hisatomo Kowa; Fumio Kanda; Tatsushi Toda
Journal:  eNeurologicalSci       Date:  2017-01-07

Review 7.  Beyond motor neurons: expanding the clinical spectrum in Kennedy's disease.

Authors:  Raquel Manzano; Gianni Sorarú; Christopher Grunseich; Pietro Fratta; Emanuela Zuccaro; Maria Pennuto; Carlo Rinaldi
Journal:  J Neurol Neurosurg Psychiatry       Date:  2018-01-20       Impact factor: 10.154

8.  The French national protocol for Kennedy's disease (SBMA): consensus diagnostic and management recommendations.

Authors:  Pierre-François Pradat; Emilien Bernard; Philippe Corcia; Philippe Couratier; Christel Jublanc; Giorgia Querin; Capucine Morélot Panzini; François Salachas; Christophe Vial; Karim Wahbi; Peter Bede; Claude Desnuelle
Journal:  Orphanet J Rare Dis       Date:  2020-04-10       Impact factor: 4.123

9.  Unimpaired Neuropsychological Performance and Enhanced Memory Recall in Patients with Sbma: A Large Sample Comparative Study.

Authors:  S Marcato; J R Kleinbub; G Querin; E Pick; I Martinelli; C Bertolin; S Cipolletta; E Pegoraro; G Sorarù; A Palmieri
Journal:  Sci Rep       Date:  2018-09-11       Impact factor: 4.379

Review 10.  Biomarkers of Spinal and Bulbar Muscle Atrophy (SBMA): A Comprehensive Review.

Authors:  Giorgia Querin; Peter Bede; Veronique Marchand-Pauvert; Pierre-Francois Pradat
Journal:  Front Neurol       Date:  2018-10-10       Impact factor: 4.003

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