A 77 year old female with progressive difficulty

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A 77 year old female with progressive difficulty swallowing Teaching Neuro. Images Neurology Resident

A 77 year old female with progressive difficulty swallowing Teaching Neuro. Images Neurology Resident and Fellow Section © 2017 American Academy of Neurology

VIGNETTE • 77 year-old female diagnosed with a pontine cavernoma 3 years prior •

VIGNETTE • 77 year-old female diagnosed with a pontine cavernoma 3 years prior • Developed progressive difficulty swallowing • Conducted videofluoroscopic swallowing study and brain MRI © 2017 American Academy of Neurology Finlay, et al.

IMAGING © 2017 American Academy of Neurology Finlay, et al.

IMAGING © 2017 American Academy of Neurology Finlay, et al.

IMAGING * © 2017 American Academy of Neurology Finlay, et al.

IMAGING * © 2017 American Academy of Neurology Finlay, et al.

Olivary Enlargement and Pharyngeal Nystagmus • Videofluoroscopic swallowing study showed low frequency rhythmic contractions

Olivary Enlargement and Pharyngeal Nystagmus • Videofluoroscopic swallowing study showed low frequency rhythmic contractions of the soft palate and upper larynx • Critical analysis of brain MRI revealed hypertrophy in the right inferior olivary nucleus • Chronic cavernoma induces deafferentation of the olivary nuclei, leading to progressive neuronal vacuolization and hypertrophy. • 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, Spencer defined this clinical sign as pharyngeal and laryngeal “nystagmus” (2). © 2017 American Academy of Neurology Finlay, et al.