Objective: We present a case report of a 59 year old with a diagnosis of clinically possible progressive supranuclear palsy (PSP) and clinical finding of oculo-palatal-pharyngeal tremor.

Background: Palatal tremor has been described in case reports with atypical variants involving extra-palatal manifestations. Symptomatic palatal tremor occurs with lesions within the Guillain Mollaret triangle which can result in hypertrophic olivary degeneration (HOD). There are case reports of palatal tremor and HOD seen in neurodegenerative conditions including PSP.

Method: A 59 year old lady presented with a history from 2022 of postural instability, emotional blunting and dysphonia with features on examination of slowing of vertical saccades, gait instability and Parkinsonism.  Investigations revealed bilateral HOD on MRI brain imaging.  Clinical signs include oculo-palatal-pharyngeal tremor with time synchronised jerks of the neck and nasal muscles.

Results: Differentials considered for her presentation included paraneoplastic, autoimmune (IgLON5), genetic or neurodegenerative processes such as Alexander’s disease, progressive ataxia with palatal tremor or atypical Parkinsonian disorders. Follow up MRI imaging showed no identifiable vascular, ischaemic, neoplastic or inflammatory cause for MRI findings of HOD.  Blood tests and cerebrospinal fluid studies were negative for infectious or antibody mediated processes including Whipple’s serology.  Genetic panels for adult-onset neurodegenerative disorders and movement disorders were negative. Pending electrophysiology assessment with EEG and EMG will also be presented. 

A diagnosis of clinically possible PSP was made given the ocular motor dysfunction with slowing of vertical saccades, the postural instability and Parkinsonism. Other supportive features included poor levodopa response, cognitive dysfunction and dysphagia.  Symptomatic palatal tremor and HOD on MRI brain has rarely been described in neurodegenerative conditions including PSP.

Conclusion: This case highlights the clinical phenomenology of oculo-palatal-pharyngeal tremor with time synchronised, brief, involuntary contractions of the soft palate and other derivatives of the branchial arches, in combination with the characteristic finding of bilateral hypertrophic olivary degeneration on MRI. Palatal tremor can rarely be seen in neurodegenerative conditions and we present an atypical variant of oculo-pharyngeal-palatal tremor in presumed PSP.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/an-interesting-case-of-oculo-palatal-pharyngeal-tremor/