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1539 - Palato‐Pharyngo‐Laryngeal Myoclonus: A Unique Cause of Persistent Dysphagia After Hemorrhagic Stroke

Friday, February 28, 2025

5:00 PM - 6:30 PM MST

Presenting Author(s)

TM

Tejaswi Makkapati, MD

Resident Physician
HMH/JFK Johnson Rehabilitation Institute
Edison, New Jersey, United States

Co-Author(s)

DM

Daniel McBride, MD

Fellow Physician
HMH/JFK Johnson Rehabilitation Institute
Edison, New Jersey, United States
MM

Meredith Mcloughlin, n/a

Speech Language Pathologist
HMH/JFK Johnson Rehabilitation Institute
Edison, New Jersey, United States
RK

Rachel Komosinsky, n/a

Speech Language Pathologist
HMH/JFK Johnson Rehabilitation Institute
Edison, New Jersey, United States
KH

Kristen A. Harris, MD

Attending Physician
JFK Johnson Rehabilitation Institute
Edison, New Jersey, United States

Case Diagnosis: Palato-pharyngo-laryngeal myoclonus in a 54 yo woman

Case Description: 54 yo woman with deficits from left cerebellar AVM rupture with bilateral posterior fossa hemorrhage s/p AVM resection, tracheostomy and PEG who presented with persistent dysphagia during rehabilitation. She was decannulated during her IRF admission however struggled with mucosal clearance and swallowing and was recannulated. MBS demonstrated aspiration with larynx tremors and secretion pooling and NPO status with tube feeding was continued. MRI revealed brainstem signal alteration in addition to posterior fossa changes. Patient underwent a laryngoscopy with ENT which revealed a notable tremor. The treatment team felt that myoclonus contributed significantly to the patient’s dysphagia. Medical management was initiated.

Discussions: Palato-pharyngo-laryngeal myoclonus is classically a result of a Guillain-Mollaret triangle lesion in the medulla which causes hypertrophic olivary degeneration, removing inhibitory signals to the inferior olives from the dentate nucleus. As the inferior olives have an intrinsic rhythmic capability, an injury can result in myoclonus. These contraction abnormalities are divided into essential palatal myoclonus (EPM) or symptomatic palatal myoclonus (SPM) . EPM causes an audible click from the Eustacian tube moving as the tensor veli palatini rhythmically contracts. In SPM, it is the levator veli palatini that is affected. SPM commonly occurs after stroke and is likely the variant observed in this patient. The brainstem signal seen on MRI may be reflective of olivary hypertrophy. While imaging changes appear within months of injury, palatal myoclonus can appear up to 49 months later. Treatment options for dysphagia associated with palato-pharyngo-laryngeal myoclonus are limited to case reports.

Conclusions:
We describe a unique case of palato-pharyngo-laryngeal myoclonus and its treatment. Recognizing this type of myoclonus is important as it may cause persistent aspiration and warrant medical intervention.