Parinaud's syndrome

Signs and symptoms

Parinaud's syndrome is characterized by paralysis of upwards gaze, with downward gaze usually preserved; this vertical palsy is supranuclear, so the doll's head maneuver may elevate the eyes initially, but eventually all upward gaze mechanisms fail. In extreme form, conjugate down gaze in the primary position, or the setting-sun sign, is observed, most commonly in patients with hydrocephalus. Pseudo-Argyll Robertson pupils occur, with accommodative paresis, mid-dilated pupils, and light-near dissociation.

Convergence-retraction nystagmus appears on attempted upward gaze, easily elicited by following down-going stripes on an optokinetic drum. Eyelid retraction (Collier's sign) is present. The syndrome is commonly associated with bilateral papilledema and less commonly with spasm of accommodation on attempted upward gaze, pseudoabducens palsy, slower abduction than adduction during horizontal saccades, see-saw nystagmus, skew deviation, oculomotor nerve palsy, trochlear nerve palsy, and internuclear ophthalmoplegia.

Causes

Parinaud's syndrome results from direct or compressive injury to the dorsal midbrain, specifically compression or ischemic damage of the mesencephalic tectum including the superior colliculus, adjacent oculomotor nucleus, and Edinger-Westphal nuclei. Classically, it is associated with three groups: young patients with pineal or midbrain tumors causing hydrocephalus; women in their 20s–30s with multiple sclerosis; and older patients following upper brainstem stroke. Other causes include hydrocephalus, midbrain hemorrhage, cerebral arteriovenous malformation, trauma, brainstem toxoplasmosis, neoplasms, and giant aneurysms of the posterior fossa. Vertical supranuclear ophthalmoplegia has also been associated with metabolic disorders such as Niemann-Pick disease, Wilson's disease, kernicterus, and barbiturate overdose.

Did You Know?

• Treatment is primarily directed toward the etiology; visually significant upgaze palsy can be relieved with bilateral inferior rectus recessions.
• Retraction nystagmus and convergence movement usually improve after bilateral inferior rectus recessions.
• Eye findings generally improve slowly over months, especially with resolution of the causative factor; rapid resolution after normalization of intracranial pressure via ventriculoperitoneal shunt has been reported.