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Botulinum toxin treatment improves dysphagia in patients with oculopharyngeal muscular dystrophy and sporadic inclusion body myositis

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Abstract

Objective

Dysphagia can be troublesome in sporadic inclusion body myositis (sIBM) and oculopharyngeal muscular dystrophy (OPMD), but no established treatment exists. Cricopharyngeal muscle botulinum toxin injection has at case level been reported to be effective. We evaluated safety and efficacy of botulinum toxin injections in the cricopharyngeal muscle in patients with dysphagia due to sIBM or OPMD.

Methods

Participants were included from our outpatient clinic. Cricopharyngeal constriction was confirmed by laryngoscopy. After EMG confirmation of needle placement in the cricopharyngeal muscle, botulinum toxin A was injected in awake patients. An individualized dose of 5–10 units of botulinum toxin A was applied initially and titrated up a maximum of 3 times. Outcome measures were change in dysphagia questionnaire, timed cold-water swallow test and subjective dysphagia status (worse, unchanged, improved). Due to the need for individualized dosing and a limited number of available patients, an uncontrolled, un-blinded design was used.

Results

Thirteen patients, 3 with OPMD, received at least 1 injection. In the dysphagia questionnaire, all but 2 subjects, none with subjective worsening, improved (p < 0.001). Subjectively, seven felt an improvement, 4 no change and 2 a worsening. No overall change was seen the timed cold-water swallow test. No serious adverse events were observed.

Conclusion

Botulinum toxin injection of the cricopharyngeal muscle in patients with OPMD and sIBM had a beneficial effect on dysphagia in most of the treated patients. Two of 13 patients experienced a temporary worsening not reflected in dysphagia score. Limitations are the un-blinded and un-randomized design and subjective assessments methods.

Prospective trial registration

EudraCT-number: 2014-002210-23.

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Funding

The study was not funded.

Author information

Authors and Affiliations

  1. Department of Neurology, Copenhagen Neuromuscular Center and Department of Neurology, Rigshospitalet and Copenhagen University, Copenhagen, Denmark

    N. Witting & J. Vissing

  2. Department of Neurology, Bispebjerg Hospital, Copenhagen, Denmark

    D. Daugaard & H. Biernat

  3. Section Bispebjerg, Foniatric Section, Department of Oto-Rhino-Laryngology, Rigshospitalet and Copenhagen University, Copenhagen, Denmark

    S. Prytz

  4. Department of Rheumatology, Rigshospitalet and Copenhagen University, Copenhagen, Denmark

    L. P. Diederichsen

  5. Department of Rheumatology, Odense University Hospital, Odense, Denmark

    L. P. Diederichsen

Authors
  1. N. Witting
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  2. D. Daugaard
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  3. S. Prytz
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  4. H. Biernat
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  5. L. P. Diederichsen
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  6. J. Vissing
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Contributions

NW: planned and executed the study, carried out the statistical analysis and is the corresponding author, HB, LPD, SP and DD contributed importantly to collection and data and preparation of manuscript, JV partipated in conception and planning of the study besides preparation of the manuscript.

Corresponding author

Correspondence to N. Witting.

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Conflicts of interest

None of the authors have financial or non-financial interests to disclose.

Ethical approval

The authours state that the study adhere to the COPE guidelines.

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Witting, N., Daugaard, D., Prytz, S. et al. Botulinum toxin treatment improves dysphagia in patients with oculopharyngeal muscular dystrophy and sporadic inclusion body myositis. J Neurol 269, 4154–4160 (2022). https://doi.org/10.1007/s00415-022-11028-8

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  • DOI: https://doi.org/10.1007/s00415-022-11028-8

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