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Related Experiment Video

Updated: Jan 14, 2026

Using Eye-tracking to Assess the Relative Importance of Visual and Vestibular Input to Subcortical Motion Processing in the Roll Plane
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Hyperactive Otolith-Ocular Reflex During Heat Tilt (Rabbit's Eye Sign) In Anti-Ma2 Rhombencephalitis.

João Lemos1,2, Sara Matos3, Ana Inês Martins3,4

  • 1Neurology Department, Coimbra University Hospital Centre, Praceta Professor Mota Pint, 3000-075, Coimbra, Portugal. merrin72@hotmail.com.

Cerebellum (London, England)
|October 27, 2025
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Summary
This summary is machine-generated.

Hyperactive otolith-ocular reflex (OOR) in humans, a vestigial reflex, can manifest pathologically. This case suggests cerebellar inhibition failure in anti-Ma2-associated rhombencephalitis, releasing the OOR.

Keywords:
Anti-Ma2 antibodiesOcular counter-rollOcular tilt reactionOtolith-ocular reflexSkew deviationSupranuclear gaze palsy

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Area of Science:

  • Neuroscience
  • Ophthalmology
  • Neurology

Background:

  • The otolith-ocular reflex (OOR) stabilizes gaze during head tilt but is typically inhibited in humans.
  • Pathological conditions can release this vestigial reflex, leading to abnormal eye movements.

Purpose of the Study:

  • To describe a unique case of hyperactive OOR in a patient with anti-Ma2-associated rhombencephalitis.
  • To investigate the potential link between cerebellar inhibition failure and OOR release.

Main Methods:

  • Clinical case presentation and neurological examination.
  • Neuroimaging (Brain MRI) and serological testing for anti-Ma2 antibodies.
  • Assessment of ocular torsion and eye movement abnormalities during head tilt.

Main Results:

  • A 38-year-old male presented with vertical diplopia, difficulty looking down, and marked ocular counter-roll (OCR) and hyperdeviation during head tilts.
  • High titers of anti-Ma2 antibodies were detected, associated with a mediastinal seminoma.
  • Brain MRI revealed mild midbrain atrophy; anti-inflammatory treatment was ineffective.

Conclusions:

  • Hyperactive OOR, observed as the "rabbit's eye sign" in this patient, is a rare but significant finding in humans.
  • This suggests a failure of cerebellar inhibitory control over brainstem OOR pathways.
  • The presence of hyperactive OOR warrants investigation for underlying cerebellar or brainstem pathology, potentially linked to paraneoplastic syndromes.