Literature DB >> 34085109

Acetylcholinesterase inhibitor responsive myasthenia in a Filipino male with X-linked recessive spinal and bulbar muscular atrophy.

Roland Dominic G Jamora1, Mario B Prado2, Carlos L Chua2,3.   

Abstract

We report a 51-year-old male diagnosed with X-linked recessive spinal and bulbar muscular atrophy (SBMA) by genetic testing who presented with 30 years history of progressive proximal and bulbar weakness responsive to cholinesterase inhibitor. Although the anti-acetylcholine receptor antibody (anti-AChR Ab) was negative, the myasthenic state was confirmed by decremental response in repetitive nerve stimulation and increased jitter frequency and blocking in single fiber-electromyography. While myasthenia gravis and SBMA may co-exist independently in an individual having the signs and symptoms of both conditions, the absence of anti-AChR Ab may imply that myasthenia can be an exaggerated activity-induced fatigue or weakness from the latter.

Entities:  

Keywords:  Activity dependent conduction block; Activity-induced weakness; CAG repeat; Myasthenia; Spinal and bulbar muscular atrophy

Year:  2021        PMID: 34085109     DOI: 10.1007/s10072-021-05358-0

Source DB:  PubMed          Journal:  Neurol Sci        ISSN: 1590-1874            Impact factor:   3.307


  10 in total

1.  X-linked spinal and bulbar muscular atrophy with myasthenic symptoms.

Authors:  M Yamada; A Inaba; T Shiojiri
Journal:  J Neurol Sci       Date:  1997-03-10       Impact factor: 3.181

2.  Myasthenic symptoms in a patient with Kennedy's disease.

Authors:  Zorica Stevic; Stojan Peric; Sanja Pavlovic; Ivana Basta; Dragana Lavrnic
Journal:  Acta Neurol Belg       Date:  2013-02-27       Impact factor: 2.396

3.  Activity-dependent conduction block in multifocal motor neuropathy.

Authors:  R Kaji; H Bostock; N Kohara; N Murase; J Kimura; H Shibasaki
Journal:  Brain       Date:  2000-08       Impact factor: 13.501

Review 4.  Myasthenia gravis: recommendations for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America.

Authors:  A Jaretzki; R J Barohn; R M Ernstoff; H J Kaminski; J C Keesey; A S Penn; D B Sanders
Journal:  Neurology       Date:  2000-07-12       Impact factor: 9.910

5.  Ocular myasthenia gravis associated with x-linked recessive spinal and bulbar muscular atrophy.

Authors:  Cavit Boz; Ersan Kalay; Nilufer Sahin; Sibel Velioglu; Mehmet Ozmenoglu; Ahmet Karagüzel
Journal:  J Clin Neuromuscul Dis       Date:  2004-03

6.  Polymorphic CAG repeat length in the androgen receptor gene and association with neurodegeneration in a heterozygous female carrier of Kennedy's disease.

Authors:  Karen J Greenland; Jonathan Beilin; Julian Castro; Paul N Varghese; Jeffrey D Zajac
Journal:  J Neurol       Date:  2004-01       Impact factor: 4.849

Review 7.  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

8.  Prominent fatigue in spinal muscular atrophy and spinal and bulbar muscular atrophy: evidence of activity-dependent conduction block.

Authors:  Yu-ichi Noto; Sonoko Misawa; Masahiro Mori; Naoki Kawaguchi; Kazuaki Kanai; Kazumoto Shibuya; Sagiri Isose; Saiko Nasu; Yukari Sekiguchi; Minako Beppu; Shigeki Ohmori; Masanori Nakagawa; Satoshi Kuwabara
Journal:  Clin Neurophysiol       Date:  2013-04-30       Impact factor: 3.708

Review 9.  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

Review 10.  Molecular Mechanisms and Therapeutics for SBMA/Kennedy's Disease.

Authors:  Frederick J Arnold; Diane E Merry
Journal:  Neurotherapeutics       Date:  2019-10       Impact factor: 7.620

  10 in total

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