Ping‐Pong Gaze in Sporadic Creutzfeldt‐Jakob Disease

A 56‐year‐old woman was admitted to hospital with dizziness and unstable gait for 18 days. Medical history showed that she had dyscalculia for ⁓1 month. Cranial magnetic resonance imaging revealed high signal change on diffusion‐weighted MRI in the right frontal, parietal, and temporal lobes and bilateral caudate nucleus, with ribbon signs in the cortex, particularly in the right cerebral hemisphere (Fig. 1A). Electroencephalogram showed three‐phase waves in the background of slow waves. Real‐time quaking‐induced conversion assay was positive for cerebrospinal fluid. She had no family history of similar conditions. Using the sporadic Creutzfeldt‐Jakob disease (s‐CJD) criteria, ¹ a diagnosis of “probable s‐CJD” was made. Her consciousness gradually worsened during hospitalization. On Day 8, akinetic mutism with hemifield ping‐pong gaze (PPG) ² developed (Video 1, Fig. 1B‐D), with spontaneous and continuous slow conjugate eye movements back and forth between the midline (ie, central gaze position) and left lateral gaze position; the cycles lasted 3–4 s without pauses between cycles. Pupillary responses to light were normal consistent with normal brainstem function. She was discharged after 11 days in hospital and died after 7 days.

Figure 1.

Magnetic resonance imaging (MRI) findings and sequence of Ping‐Pong Gaze. MRI showing high signal intensity in the right frontal lobe, right parietal lobe, right temporal lobe, and bilateral caudate nucleus on diffusion‐weighted imaging, with zonal signs in the cortex (A). Eyes in the orthotropic primary position (B), which deviated conjugated to the left involuntarily (C‐D).

VIDEO 1.

Ping‐pong Gaze.

PPG has localizing value and indicates hemispheric damage. PPG asymmetry predicts lesion laterality. In this case, the right hemisphere was affected more severely on the diffusion‐weighted MRI, resulting in left PPG in both eyes. Previous studies have documented oculomotor disturbances in patients with Creutzfeldt‐Jakob disease. However, the terminology employed in these reports, such as “ophthalmoplegia,” “erratic eye movements,” “fluttering ocular movements,” or “difficulty with conjugate eye movements,” limited the ability to provide further comment. ³ This case presents the first instance of PPG gaze observed in a patient with sporadic CJD, thereby adding to the existing understanding of the significance of oculomotor disorders in CJD.

Author Roles

(1) Research Project: A. Conception, B. Organization, C. Execution; (2) Data Collection: A. Design, B. Execution, C. Review and Critique; (3) Manuscript: A. Writing of the first draft, B. Review and Critique.

G.X.: 1A, 2B, 3B.

C.X.: 1A, 2B, 3B.

Y.Y.: 1B, 3A.

T.H.: 1B, 3A.

X.S.: 1B, 3A.

Disclosures

Ethical Compliance Statement Section: This case report was approved by the ethics committees of the Affiliated Hospital of Southwest Medical University, and the informed consent was obtained. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines.

Funding Sources and Conflicts of Interest: YY has received a grant from the Doctoral Research Initiation Fund of Affiliated Hospital of Southwest Medical University and a grant from the Luzhou Science and Technology Plan Project. No conflict of interest. TH has no conflict of interest to report. XSL has no conflict of interest to report. CX has no conflict of interest to report. GXY has no conflict of interest to report.

Financial Disclosures for the Previous 12 Months: The authors declare that there are no additional disclosures to report.

Additional Contributions

We thank the patient's husband for granting permission to publish this information.