A 77-year-old woman diagnosed with a pontine cavernoma developed progressive difficulty swallowing. A videofluoroscopic swallowing study showed low-frequency rhythmic contractions of the soft palate and upper larynx (video at Neurology.org). Brain MRI revealed hypertrophy in the right inferior olivary nucleus (figure).
*The pontine cavernoma in the coronal slice. The arrows highlight a hyperintense signal and volumetric growth of the right inferior olivary nucleus. The bottom figure emphasizes the contrast between the hypertrophic right olivary nucleus and the unaffected contralateral nucleus.
A chronic cavernoma induces deafferentation of the olivary nuclei, leading to progressive neuronal vacuolization and hypertrophy. The deafferented olivary neurons spontaneously organize into synchronously oscillating clusters, which produces the common clinical sign: a low-frequency tremor of the muscles derived from the brachial arch, pharynx, larynx, diaphragm, eyelids, and face.1 In 1886, Spencer2 defined this clinical sign as pharyngeal and laryngeal “nystagmus.”
AUTHOR CONTRIBUTIONS
Darío A. Yacovino: oversaw neurologic examination and treatment of patient, analyzed data, critical revision of manuscript. John B. Finlay: analyzed data, wrote and revised manuscript.
STUDY FUNDING
No targeted funding reported.
DISCLOSURE
The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.
Footnotes
Download teaching slides: Neurology.org
Supplemental data at Neurology.org
- © 2017 American Academy of Neurology
Letters: Rapid online correspondence
- Temporal window of MRI changes in secondary hypertrophic olivary degeneration
- The interval between the cavernous lesion and the palato-paryngeal tremor
- Pharyngeal nystagmus and oculopalatal tremor
- Pharyngeal myorhythmia
REQUIREMENTS
You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
You May Also be Interested in
Hastening the Diagnosis of Amyotrophic Lateral Sclerosis
Dr. Brian Callaghan and Dr. Kellen Quigg