Objective: Present rare cause of symptomatic palatal tremor and ataxia after fourth ventricle cyst removal with unusual respiratory muscle involvement.

Background: Symptomatic palatal tremor (SPT) with olivary hypertrophy and ataxia is an uncommon and non-specific syndrome caused by a brainstem structural lesion of any kind within the dentato-rubro-olivary pathway. Fourth ventricle tumors are a rare cause, and involvement of respiratory muscles is extremely rare.

Methods: Case report and literature review.

Results: A 59 year old woman was referred for “upper body vibrations.” This developed after removal of a massive 4th ventricle epidermoid cyst. Pre-operative symptoms included gait ataxia without focal brainstem signs, though the tumor was significantly compressing the dorsal brainstem. There were no surgical complication. Three months after surgery, she noted a change in respiratory patterns, and laryngologists noted that she had continuous pulsation of the laryngeal muscles. Repeat MRI scans revealed only olivary hypertrophy without focal brainstem lesions. Since that time she also developed oscillopsia, impaired but stable balance, and speech alteration. Examination revealed rotary nystagmus on far lateral gaze, more to the right, saccadic vertical eye movements, 1-2 Hz lip pulsations that were synchronous with midline palatal tremor. In addition there were additional synchronous lower facial, laryngeal, abdominal and diaphragm contractions. She had mild midline more than limb ataxia, worse on the left, and mild gait ataxia. Review of the literature showed only four other cases with SPT and diaphragmatic and laryngeal involvement, and two case reports with fourth ventricle (specifically epidermoid) cysts were identified. Medications have not been able to successfully control her movements, and treatments for bothersome respirations are being pursued.

Conclusions: SPT appears to be a particular risk with fourth ventricle epidermoid cyst removal, given this third reported case. Prominent respiratory involvement of SPT is unusual, suggesting functional modulation of descending pathways.

Submitted for American Academy of Neurology meeting 2016, no notification of acceptance at this time.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/symptomatic-palatal-and-respiratory-tremor-with-ataxia-following-fourth-ventricle-epidermoid-cyst-removal/