A 5-year-old typically developing boy presented with a 4-week history of moving his head to follow objects due to inability to move his eyes side to side. His neurologic examination was normal except for this inability to voluntarily move his eyes horizontally, consistent with oculomotor apraxia (Video 1). MRI of the brain showed pontine mass suggestive of diffuse high-grade glioma (DIPG) (Figure). The patient underwent radiotherapy, and a ventriculoperitoneal shunt was placed for hydrocephalus.
Video 1
Oculomotor apraxia with inability to move the eyes horizontally. The patient is shown turning his head to the object to compensate.Download Supplementary Video 1 via http://dx.doi.org/10.1212/207376_Video_1
MRI of the brain T1-weighted image (sagittal view) showing a mass centered in the pons (arrow) with significant expansion and mild extension to the midbrain as well as a posterior exophytic component obliterating the 4th ventricle leading to early hydrocephalus (A). T2-weighted image showing high signal intensity of the tumor (B).
In pediatric patients, oculomotor apraxia may be seen in ataxia with oculomotor apraxia, Cogan syndrome, Joubert syndrome, and ataxia telangiectasia. In our case, the brainstem tumor disrupted the structural connectivity between the frontal eye fields and oculomotor network including the pons, the superior colliculus, and caudate nucleus leading to oculomotor apraxia.1
DIPG is an aggressive pediatric tumor with a median survival of 9–12 months. It classically presents with cranial nerve palsies, long tract signs, and ataxia.2
Author Contributions
F. Thabet: drafting/revision of the manuscript for content, including medical writing for content. Mohammed Sawahreh: drafting/revision of the manuscript for content, including medical writing for content. D. Thaher: major role in the acquisition of data. F.A. Maadid: major role in the acquisition of data.
Study Funding
The authors report no targeted funding.
Disclosure
The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Submitted and externally peer reviewed. The handling editor was Resident & Fellow Section Deputy Editor Ariel Lyons-Warren, MD, PhD.
Teaching slides links.lww.com/WNL/C786
- Received December 13, 2022.
- Accepted in final form March 20, 2023.
- © 2023 American Academy of Neurology
Letters: Rapid online correspondence
- Reader Response: Teaching Video NeuroImage: Oculomotor Apraxia as the Only Presentation of Diffuse Intrinsic Pontine Glioma
- Reader Response: Teaching Video NeuroImage: Oculomotor Apraxia as the Only Presentation of Diffuse Intrinsic Pontine Glioma
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