Research Article| Volume 27, ISSUE 5, P642-643, September 2013

Pantothenate Kinase-Associated Neurodegeneration Causing Paradoxical Vocal Fold Motion

  • Sassan Rafizadeh
    Department of Head and Neck Surgery, University of California, Los Angeles, California

    University of Toledo - College of Medicine, Toledo, Ohio
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  • Jennifer L. Long
    Address correspondence and reprint requests to Jennifer L. Long, Department of Head and Neck Surgery, 200 Medical Plaza, Suite 550, Los Angeles, CA 90095-1624.
    Department of Head and Neck Surgery, University of California, Los Angeles, California
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      We report a patient with paradoxical vocal fold motion (PVFM) due to pantothenate kinase-associated neurodegeneration, a rare neurodegenerative disease and a subclass of neurodegeneration with brain iron accumulation disorders. PVFM is marked by normal vocal fold anatomy and physiology with intermittent adduction during the respiratory cycle. Many etiologies have been reported and include laryngeal hypersensitivity such as asthma and gastroesophageal reflux, functional disorders, and neurologic disorders such as focal respiratory dystonia. This case highlights the occasional association of PVFM with underlying neurologic disorders, especially those that disrupt autonomic functioning.

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        • Maschka D.A.
        • Bauman N.M.
        • McCray Jr., P.B.
        • Hoffman H.T.
        • Karnell M.P.
        • Smith R.J.
        A classification scheme for paradoxical vocal cord motion.
        Laryngoscope. 1997; 107: 1429-1435
        • McNeill A.
        • Birchall D.
        • Hayflick S.J.
        • et al.
        T2* and FSE MRI distinguishes four subtypes of neurodegeneration with brain iron accumulation.
        Neurology. 2008; 70: 1614-1619
        • Kurian M.A.
        • McNeill A.
        • Lin J.P.
        • Maher E.R.
        Childhood disorders of neurodegeneration with brain iron accumulation (NBIA).
        Dev Med Child Neurol. 2011; 53: 394-404
        • Hortnagel K.
        • Prokisch H.
        • Meitinger T.
        An isoform of hPANK2, deficient in pantothenate kinase-associated neurodegeneration, localizes to mitochondria.
        Hum Mol Genet. 2003; 12: 321-327
        • Brin M.F.
        • Blitzer A.
        • Velickovic M.
        Movement disorders of the larynx.
        in: Blitzer A. Brin M.F. Ramig L.O. Neurologic disorders of the larynx. Thieme, New York, NY2009
        • Forrest L.A.
        • Husein T.
        • Husein O.
        Paradoxical vocal cord motion: classification and treatment.
        Laryngoscope. 2012; 122: 844-853
        • Altman K.W.
        • Mirza N.
        • Ruiz C.
        • Sataloff R.T.
        Paradoxical vocal fold motion: presentation and treatment options.
        J Voice. 2000; 14: 99-103
        • Vetrugno R.
        • Liguori R.
        • Cortelli P.
        • et al.
        Sleep-related stridor due to dystonic vocal cord motion and neurogenic tachypnea/tachycardia in multiple system atrophy.
        Mov Disord. 2007; 22: 673-678
        • Worley G.
        • Witsell D.L.
        • Hulka G.F.
        Laryngeal dystonia causing inspiratory stridor in children with cerebral palsy.
        Laryngoscope. 2003; 113: 2192-2195
        • Murry T.
        • Sapienza C.
        The role of voice therapy in the management of paradoxical vocal fold motion, chronic cough, and laryngospasm.
        Otolaryngol Clin North Am. 2010; 43: 73-83
        • Grillone G.A.
        • Blitzer A.
        • Brin M.F.
        • Annino Jr., D.J.
        • Saint-Hilaire M.H.
        Treatment of adductor laryngeal breathing dystonia with botulinum toxin type A.
        Laryngoscope. 1994; 104: 30-32
        • Cukier-Blaj S.
        • Bewley A.
        • Aviv J.E.
        • Murry T.
        Paradoxical vocal fold motion: a sensory-motor laryngeal disorder.
        Laryngoscope. 2008; 118: 367-370