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Jaw clonus and opercular syndrome in ALS: a rare and interesting finding

  • Alvee Saluja
  • Rajesh Kumar Singh
  • Deepa Dash
  • Rohit Bhatia
  • Manjari Tripathi
Letter to the Editor
  • 18 Downloads

Dear Editor,

Jaw clonus has been reported rarely in amyotrophic lateral sclerosis (ALS) due to corticobulbar dysfunction. Opercular syndrome has been described commonly after stroke but is rarely described in other conditions. We report a young patient who was diagnosed as bulbar onset ALS and has rare finding of jaw clonus as well as opercular syndrome.

35 year old male developed nasal twang in his voice 3 years back which progressed to extent that he was unable to produce vocal sounds within 2 year of illness. He also developed pseudobulbar affect within one year of onset of illness. He started having slippage of slippers from the feet one and a half years into the illness. Gradually, he developed difficulty in buttoning and unbuttoning his shirt followed by difficulty in raising the arms overhead. The patient also noticed progressive thinning of all four limbs with fasciculations all over the body for last 2 years. He also had difficulty in swallowing and frequent cough during...

Notes

Compliance with ethical standards

Conflict of interest

The author declares that there is no competing interest.

Informed consent

Informed consent was obtained.

Research involving human participants and or animals

For this type of study (case report) formal consent is not required.

Supplementary material

Video showing jaw clonus and restricted extra ocular movements on command (MP4 19808 KB)

References

  1. 1.
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    Weller M, Poremba M, Dichgans J (1990) Opercular syndrome without opercular lesions: Foix-Chavany-Marie syndrome in progressive supranuclear motor system degeneration. Eur Arch Psychiatry Neurol Sci 239:370–372CrossRefPubMedGoogle Scholar
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    Lapresle J, Salisachs P (1976) Loss of voluntary control and retention of automatic reflex in certain muscles innervated by cranial nerves in 2 cases of amyotrophic lateral sclerosis. Rev Neurol 132:157–161PubMedGoogle Scholar
  5. 5.
    Hudson AJ (1981) Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review. Brain 104:217–247CrossRefGoogle Scholar

Copyright information

© Belgian Neurological Society 2018

Authors and Affiliations

  • Alvee Saluja
    • 1
  • Rajesh Kumar Singh
    • 1
  • Deepa Dash
    • 1
  • Rohit Bhatia
    • 1
  • Manjari Tripathi
    • 1
  1. 1.Department of Neurology, C.N. CentreAll India Institute of Medical SciencesNew DelhiIndia

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