Palato-pharyngo-laryngeal myoclonus with recurrent retrograde feeding tube migration after cerebellar hemorrhagic stroke: a case report and review of hypertrophic olivary degeneration

Abstract

Background: Palato-pharyngo-laryngeal myoclonus, a variant of palatal myoclonus, is characterized by involuntary rhythmic movements of palatal, pharyngeal, and laryngeal muscles. Symptomatic palatal myoclonus is classically associated with hypertrophic olivary degeneration on MRI imaging due to a lesion in the triangle of Guillain-Mollaret. Case presentation: We report a case of palato-pharyngo-laryngeal myoclonus in a patient post-cerebellar hemorrhagic stroke who presented with recurrent retrograde migration of his gastrojejunostomy feeding tubes. Treatment with either divalproex sodium or gabapentin resulted in a significant decrease in his gastrointestinal symptoms and no further episodes of gastrojejunostomy tube migration. Conclusions: This case study indicates that the movement disorder associated with hypertrophic olivary degeneration may involve the gastrointestinal system. Anticonvulsants, such as gabapentin and divalproex sodium, may reduce the severity of gastrointestinal symptoms in cases associated with hypertrophic olivary degeneration. The anatomy of the Guillain-Mollaret triangle and the pathophysiology of hypertrophic olivary degeneration are reviewed.

Palato-Pharyngo-Laryngeal Rhythmic Myoclonus in Neuro-Behcet Syndrome: A Case Report

“…It is reported that symptomatic palato-pharyngo-laryngeal rhythmic myoclonus in NBS presents anywhere between 1 month and 8 years after the initial lesion. 8…”

“…It is reported that symptomatic palato-pharyngolaryngeal rhythmic myoclonus in NBS presents anywhere between 1 month and 8 years after the initial lesion. 8 Palato-pharyngo-laryngeal rhythmic myoclonus is a unique otolaryngologic presentation of Neuro-Behcet's disease with several laryngeal complications. First, the synchronous myoclonus of the vocal folds, in combination with presumed multifocal CNS lesions, resulted in significant dysarthria with dysphonia.…”

“…Symptomatic palato-pharyngo-laryngeal myoclonus is due to a lesion in the denato-rubro-olivary pathway, also known as the Guillain-Mollaret triangle, which results in a transneuronal hypertrophic olivary degeneration (HOD). 8 Most lesions to this pathway occur due to infarction, though hemorrhage, demyelination, trauma, encephalitis, tumors, syphilis and other degenerative diseases. 5 It is theorized this lesion removes inhibitory input from the dentate nucleus to the inferior olives.…”

Palato‐Pharyngo‐Laryngeal Myoclonus with Facial Involvement After an Ischemic Stroke

“…Palatal myoclonus can rarely be associated with synchronous myoclonus of other branchial arch muscles, the diaphragm, facial muscles, or even the extremities 2 . One specific variant is termed palato‐pharyngo‐laryngeal myoclonus, which can result in dysphagia, dysphonia, and dysarthria 4 …”

“…2 One specific variant is termed palato-pharyngo-laryngeal myoclonus, which can result in dysphagia, dysphonia, and dysarthria. 4 A 56-year-old woman awoke from sleep with new right-sided weakness and dizziness. Magnetic resonance imaging (MRI) of the brain showed restricted diffusion in the right superomedial cerebellum consistent with an acute infarct (Fig.…”