Supranuclear Gaze Palsy
Supranuclear Gaze Palsy
A supranuclear gaze palsy results from pathology located above the nuclei of the nerves supplying the extraocular muscles. Voluntary gaze is impaired while the integrity of the oculomotor nuclei and infranuclear connections may be demonstrated by the preservation of:
Vestibulo-ocular reflexes (VOR): overcoming the ophthalmoplegia, at least in the early stages (e.g., the supranuclear gaze palsy in the vertical plane in progressive supranuclear palsy);
Oculocephalic reflex (doll’s head, doll’s eye maneuver); Bell’s phenomenon.
Supranuclear gaze palsies may be:
- Horizontal:
Hemisphere (frontal) lesion: eyes deviated to the side of the lesion, or in the case of an irritative (e.g., epileptic) focus away from the side of the lesion
Paramedian pontine reticular formation: eyes deviated to contralateral side.
- Vertical:
Brainstem compression/distortion
Dorsal upper midbrain (e.g., rostral interstitial nucleus of the median longitudinal fasciculus; pineal lesion causing Parinaud’s syndrome)
Skew deviation
Recognized causes of supranuclear gaze palsy include:
Progressive supranuclear palsy (PSP; Steele-RichardsonOlszewski syndrome)
Creutzfeldt-Jakob disease Corticobasal degeneration
Progressive subcortical gliosis of Neumann Adult-onset Niemann-Pick disease Gaucher’s disease.
References
Lees AJ. The Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). In: Marsden CD, Fahn S (eds.). MovementDisorders 2. London: Butterworth, 1987: 272-287
Cross References
Gaze palsy; Parinaud’s syndrome; Parkinsonism; Prevost’s sign; Skew deviation; Vestibulo-ocular reflexes
