Miri, S.

Rohani, M.

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RESIDENT& FELLOWSECTIONSection EditorMitchell S.V. Elkind,MD, MSMohammad Rohani, MDShahnaz Miri, MDCorrespondence toDr. Rohani:mohammadroohani@gmail.comDownload teaching slides:Neurology.orgSupplemental dataat Neurology.orgTeaching Video NeuroImages:Hand tremor, tongue and perioralfasciculation in a patient with Kennedy diseaseA 54-year-old man presented with a 10-year historyof progressive gait difficulty, muscle weakness, andfatigue. Clinical examinations revealed mild nasalspeech; postural hand tremor; absence of deep tendonreflexes; atrophy and fasciculation in tongue, facial(perioral), and limb muscles; and gynecomastia (videoon the Neurology® Web site at Neurology.org). Nerveconduction studies had normal results; however, nee-dle EMG demonstrated a neurogenic pattern withspontaneous activity (fibrillation, positive sharp wave,and fasciculation) in cranial and limb muscles.Genetic testing confirmed (CAG expansion of theandrogen receptor gene on X chromosome) the diag-nosis of spinobulbar muscular atrophy or Kennedydisease, an X-linked adult-onset degenerative disorderof motor neurons.1,2AUTHOR CONTRIBUTIONSDr. Mohammad Rohani: acquisition of data, drafting and revising themanuscript. Dr. Shahnaz Miri: drafting and revising the manuscript.STUDY FUNDINGNo targeted funding reported.DISCLOSUREThe authors report no disclosures relevant to the manuscript. Go toNeurology.org for full disclosures.REFERENCES1. Finsterer J. Bulbar and spinal muscular atrophy (Kennedy’sdisease): a review. Eur J Neurol 2009;16:556–561.2. Harding AE, Thomas PK, Baraitser M, Bradbury PG,Morgan-Hughes JA, Ponsford JR. X-linked recessivebulbospinal neuronopathy: a report of ten cases. J NeurolNeurosurg Psychiatry 1982;45:1012–1019.From the Department of Neurology (M.R.), Iran University of Medical Sciences, Tehran, Iran; and the Department of Neurology (S.M.), SUNYDownstate Medical Center, Brooklyn, NY.e76 © 2015 American Academy of Neurologyª 2015 American Academy of Neurology. Unauthorized reproduction of this article is prohibited.DOI 10.1212/WNL.00000000000013402015;84;e76 Neurology Mohammad Rohani and Shahnaz Miripatient with Kennedy disease: Hand tremor, tongue and perioral fasciculation in aImagesTeaching Video NeuroThis information is current as of March 9, 2015ServicesUpdated Information & http://n.neurology.org/content/84/10/e76.fullincluding high resolution figures, can be found at:Supplementary Material 340.DC2http://n.neurology.org/content/suppl/2015/03/07/WNL.0000000000001 340.DC1http://n.neurology.org/content/suppl/2015/03/07/WNL.0000000000001Supplementary material can be found at: References http://n.neurology.org/content/84/10/e76.full#ref-list-1This article cites 2 articles, 1 of which you can access for free at: Subspecialty Collections http://n.neurology.org/cgi/collection/trinucleotide_repeat_diseasesTrinucleotide repeat diseases http://n.neurology.org/cgi/collection/tremorTremor http://n.neurology.org/cgi/collection/other_educationOther Education http://n.neurology.org/cgi/collection/anterior_nerve_cell_diseaseAnterior nerve cell diseasefollowing collection(s): This article, along with others on similar topics, appears in the  Permissions & Licensing http://www.neurology.org/about/about_the_journal#permissionsits entirety can be found online at:Information about reproducing this article in parts (figures,tables) or in  Reprints http://n.neurology.org/subscribers/advertiseInformation about ordering reprints can be found online:rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.1951, it is now a weekly with 48 issues per year. Copyright © 2015 American Academy of Neurology. All ® is the official journal of the American Academy of Neurology. Published continuously sinceNeurology 

Teaching Video NeuroImages: Hand tremor, tongue and perioral fasciculation in a patient with Kennedy disease

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Teaching Video NeuroImages: Hand tremor, tongue and perioral fasciculation in a patient with Kennedy disease

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