Successful botulinum toxin treatment of dysphagia in a spinal muscular atrophy type 2 patient


      Prominent dysphagia is seen among patients with spinal muscular atrophy (SMA) type 2, especially at the late stage of their disease progression. Nasogastric tube feeding and gastrostomy are commonly utilized to maintain their nutritional status. However, choosing a treatment strategy to maintain appropriate nutritional status is often complicated by multiple factors, such as physical conditions and social aspects. We report a 21-year-old man with SMA type 2 who has been suffering from severe dysphagia. The findings at video-fluoroscopic swallow study (VSS) were consistent with a diagnosis of cricopharyngeal dysphagia. His dysphagia was successfully treated with percutaneous injection of botulinum toxin A (BTA) into the cricopharyngeal muscle. Our result demonstrates that administration of BTA is one of the effective treatment choices for dysphagia in SMA patients.


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        • Zerres K.
        • Rudnik-Schoneborn S.
        Natural history in proximal spinal muscular atrophy. Clinical analysis of 445 patients and suggestions for a modification of existing classifications.
        Arch Neurol. 1995; : 518-523
        • Gary Y.M.
        • Jeffrey P.S.
        Botulinum toxin treatment for cricopharyngeal dysfunction.
        Dysphagia. 2001; : 161-167
        • Ming-Jang C.
        • Yeun-chung C.
        • Tzu-Yu H.
        Prolonged effect of toxin injection in the treatment of cricopharyngeal dysphagia: case report and literature review.
        Dysphagia. 2004; : 52-57
        • Willing T.N.
        • Paulus J.
        • Lacau Saint Guily J.
        • Beon C.
        • Navarro J.
        Swallowing problems in neuromuscular disorders.
        Arch Phys Med Rehabil. 1994; : 1175-1181
        • Tilton A.H.
        • Miller M.D.
        • Khoshoo V.
        Nutrition and swallowing in pediatric neuromuscular patients.
        Semin Pediatr Neurol. 1998; : 106-115
        • Schneider I.
        • Thumfart W.F.
        • Pototschnig C.
        • Eckel H.E.
        Treatment of dysfunction of the cricopharyngeal muscle with botulinum A toxin: introduction of a new, noninvasive method.
        Ann Otol Rhinol Laryngol. 1994; : 31-35
        • Restivo D.A.
        • Giuffrida S.
        • Ragona R.M.
        • Falsaperla R.
        Successful botulinum toxin treatment of dyphasia in a young child with nemaline myopathy.
        Dysphagia. 2001; : 228-229
        • Haapaniemi J.J.
        • Laurikainen E.A.
        • Pulkkinen J.
        • Marttila R.J.
        Botulinum toxin in the treatment of cricopharyngeal dysphagia.
        Dysphagia. 2001; : 171-175
        • Moerman M.B.
        Cricopharyngeal botox injection: indications and technique.
        Curr Opin Otolaryngol Head Neck Surg. 2006; : 431-436
        • Yeh T.C.
        • Yeung C.Y.
        • Sheu J.C.
        • Lee H.C.
        • Lin S.P.
        • Hsu C.H.
        • et al.
        Percutaneous–endoscopic–gastrostomy in children: 15 cases experience.
        Acta Paediatr Taiwan. 2003; : 135-139