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Progressive dysphagia without dysarthria
  1. Benjamin R Wakerley1,
  2. Katherine Warburton1,
  3. Puneet Plaha2,
  4. Stana Bojanic2,
  5. Matthew Jackson1,
  6. Martin R Turner3
  1. 1 Department of Neurology, John Radcliffe Hospital, Oxford, UK
  2. 2 Department of Neurosurgery, John Radcliffe Hospital, Oxford, UK
  3. 3 Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK
  1. Correspondence to Dr Martin Turner, Department of Neurology, John Radcliffe Hospital, West Wing Level 3, Oxford, OX3 9DU, UK; martin.turner{at}ndcn.ox.ac.uk

https://doi.org/10.1136/practneurol-2012-000441

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    A 50-year-old woman was referred with a 6-month history of progressive dysphagia. On examination, there was no dysarthria. Her voice was initially ‘wet’ but improved with coughing and throat clearing. Palatal movement, pharyngeal sensation and tongue appearance were normal. Laryngoscopy was normal. MRI of the brain showed a type 1 Chiari malformation with brainstem compression (figure 1). Following surgical decompression, her symptoms resolved completely.

    Figure 1
    Figure 1

    Sagittal T1-weighted MRI demonstrating a Chiari type I defect of the skull base with cerebellar tonsillar descent, and presumed compression of the medullary swallowing centre.

    Neurogenic dysphagia is a well-recognised symptom of Chiari malformation,1 but is rare as a presenting or isolated feature. We considered motor neurone disease but marked dysphagia without dysarthria would be exceptional. Structural abnormalities of the pharynx and oesophagus, either intrinsic (eg, stricture, diverticulum) or extrinsic (eg, retropharyngeal mass, thyroid goitre) can present as isolated progressive dysphagia,2 as can other neuromuscular causes (box 1). This case shows the importance of considering a structural neurological cause in patients with progressive dysphagia.

    Box 1

    Neuromuscular causes of progressive dysphagia

    Degenerative disorders

    • Bulbar-onset motor neurone disease (or frontotemporal dementia with motor neurone disease)

    • Kennedy's disease (X-linked spinobulbar muscular atrophy)

    • Progressive supranuclear palsy

    • Parkinson's disease

    • Huntington's disease

    • Inclusion body myositis

    • Oculopharyngeal muscular dystrophy

    • Myotonic dystrophy

    • Multiple system atrophy

    Immune/Inflammatory

    • Myasthenia gravis

    • Lambert–Eaton myasthenic syndrome (rare)

    • Dermatomyositis

    • Sarcoidosis

    • Miller Fisher syndrome

    Structural/Neoplastic

    • Base of skull/retropharyngeal/brainstem tumours

    • Carcinomatous meningitis

    • Chiari malformation (with or without syringobulbia)

    Metabolic

    • Wilson's disease

    Infective

    • Wound botulism

    • Listeria sp. rhombencephalitis

    References

    1. ↵
      1. Nathadwarawala KM,
      2. Richards CA,
      3. Lawrie B,
      4. et al
      . Recurrent aspiration due to Arnold–Chiari type I malformation. BMJ 1992;304:565–6.
      OpenUrlFREE Full Text
    2. ↵
      1. Buchholz DW
      . Neurogenic dysphagia: what is the cause when the cause is not obvious? Dysphagia 1994;9:245–55.
      OpenUrlCrossRefPubMed

    Footnotes

    • Contributors BRW: acquisition and interpretation of data. MRT: study concept and design, interpretation of data. All authors: critical revision of the manuscript.

    • Competing interests None.

    • Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Tom Hughes, Cardiff, UK.

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