Objective:

To review vestibular and ocular motor (VOM) examination findings in three patients with progressive imbalance and dizziness who were found to have immune-mediated balance disorders.

Background:

An increasing amount of immune-mediated balance disorders are being diagnosed, but detailed VOM examination findings are not well documented in the literature. 

Design/Methods:

We provide three video cases, including videooculography and video head impulse testing (HIT), that highlight examination findings in patients with immune mediated balance disorders. 

Results:

(1) A 43-year-old man presented with progressive weight loss, diarrhea, and dizziness. His initial VOM examination revealed spontaneous upbeat torsional nystagmus (top pole of eyes toward left ear), gaze evoked nystagmus, and skew deviation. These findings localized to the brainstem semicircular canal (spontaneous nystagmus) and utricle (skew deviation) pathways. He was found to have anti-dipeptidyl-peptidase-like protein-6 (DPPX) encephalitis. (2) A 63-year-old man presented with progressive imbalance and slurred speech. His VOM examination revealed downbeat nystagmus, gaze evoked nystagmus, saccadic smooth pursuit, and bilaterally abnormal HIT. These findings localized to the flocculus/paraflocculus and the peripheral vestibular apparatus. He was found to have anti-kelch-like protein-11 encephalitis. (3) A 48-year-old woman presented with subacute dizziness. Her VOM examination revealed downbeat nystagmus, gaze evoked nystagmus, and saccadic smooth pursuit. Supine roll testing revealed an apogeotropic nystagmus. These findings localized to the flocculus/paraflocculus and nodulus/uvula. She was found to have anti-septin-5 cerebellar ataxia. 

Conclusions:

The patient’s history alongside VOM examination can aid in diagnosis. (1) GI symptoms and brainstem VOM findings are suggestive of anti-DPPX. (2) A prolonged course in a male patient with progressive peripheral and cerebellar vestibular findings is suggestive of anti-Kelch. (3) The subacute onset of VOM signs suggests an immune mediated cerebellar ataxia. Lastly, knowledge of the patient’s VOM findings can guide symptomatic treatments, including medications for nystagmus or prism for skew deviation.