Perioral and tongue fasciculations in Kennedy’s disease

We report the case of a 54-year-old right-handed man who presented with a 2-year history of progressive upper-limb weakness with mild dysarthria and prominent involuntary perioral abnormal movements that were characterized as fasciculations. Electromyography disclosed motor neuron disease. The diagn...

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Bibliographic Details
Published in:Neurological sciences 2018-04, Vol.39 (4), p.777-779
Main Authors: Pedroso, José Luiz, Vale, Thiago Cardoso, Barsottini, Orlando G., Oliveira, Acary S. B., Espay, Alberto J.
Format: Article
Language:English
Subjects:
Androgen receptors
Atrophy
Diagnosis
Electromyography
Genetic screening
Gynecomastia
Handedness
Letter to the Editor
Medicine
Medicine & Public Health
Motor neuron diseases
Neurology
Neuroradiology
Neurosurgery
Polyglutamine
Polymerase chain reaction
Psychiatry
Tongue
Trinucleotide repeats
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Summary:We report the case of a 54-year-old right-handed man who presented with a 2-year history of progressive upper-limb weakness with mild dysarthria and prominent involuntary perioral abnormal movements that were characterized as fasciculations. Electromyography disclosed motor neuron disease. The diagnosis of Kennedy’s disease was established by polymerase chain reaction. Perioral abnormal movements and fasciculations may represent important clinical clues to the diagnosis of Kennedy’s disease, particularly when associated with proximal muscle atrophy and gynecomastia. In suspected cases, genetic testing for elevated CAG repeats in the androgen receptor Xq12 gene is warranted.
ISSN:1590-1874
1590-3478
DOI:10.1007/s10072-017-3170-8