Lid retraction:(Collier’s sign)
See saw nystagmus.
Pineal region tumors.
A dilated suprapineal recess can compress the mesencephalic tectum, due to hydrocephalus.
Stroke or brainstem hemorrhage in the upper part.
Guillain Barré syndrome
Gradual benign loss of upgaze in senescence.
Sylvian aqueduct syndrome
Aberrant regeneration of the third nerve
Limited upgaze in elderly patients
Progressive supranuclear palsy
Classically, it has been associated with three major groups:
Pineal region tumor: Pinealoma (intracranial germinomas) are the most common lesion producing this syndrome).
Women in their 20s-30s with multiple sclerosis.
A 26 year old Pakistani lady with first presentation of a demyelinating event, presenting as Parinaud’s syndrome. A video demonstrates a convergence-retraction nystagmus on upgaze and failure of accommodation, and her brain imaging confirms a corresponding pre-tectal contrast enhancing T2 hyperintense lesion suggestive of demyelination 4).
Older patients following stroke of the upper brainstem However, any other compression, ischemia or damage to this region can produce these phenomena: obstructive hydrocephalus, midbrain hemorrhage, cerebral arteriovenous malformation, trauma and brainstem toxoplasmosis infection. Neoplasms and giant aneurysms of the posterior fossa have also been associated with the midbrain syndrome.
Vertical supranuclear ophthalmoplegia has also been associated with metabolic disorders, such as Niemann-Pick disease, Wilson’s disease, kernicterus, and barbiturate overdose.
Isolated Oculomotor Nerve Nucleus Infarct 5).
Unilateral vascular ischemic lesion 6).
A case of longstanding, undiagnosed spontaneous intracranial hypotension (SIH) with an acute presentation of Parinaud’s syndrome, in whom serial imaging demonstrated development of a midbrain mass. The patient was ultimately diagnosed with tumefactive venous infarction secondary to SIH. However, this patient underwent a brainstem biopsy, which in retrospect may have been avoidable. This case demonstrates the imaging features of tumefactive venous infarction in SIH and highlights the risk of misinterpretation as a neoplasm with potentially catastrophic consequences 7).
Miller Fisher syndrome 9).
Parinaud’s Syndrome is a cluster of abnormalities of eye movement and pupil dysfunction, characterized by:
Paralysis of upgaze: Downward gaze is usually preserved. This vertical palsy is supranuclear, so doll’s head maneuver should elevate the eyes, but eventually all upward gaze mechanisms fail.
Pseudo-Argyll Robertson pupils: Accommodative paresis ensues, and pupils become mid-dilated and show light-near dissociation.
Convergence-Retraction nystagmus: Attempts at upward gaze often produce this phenomenon. On fast up-gaze, the eyes pull in and the globes retract. The easiest way to bring out this reaction is to ask the patient to follow down-going stripes on an optokinetic drum.
Eyelid retraction (Collier’s sign) Conjugate down gaze in the primary position: “setting-sun sign”. Neurosurgeons will often see this sign most commonly in patients with failed ventriculoperitoneal shunts. It is also commonly associated with bilateral papilledema. It has less commonly been associated with spasm of accommodation on attempted upward gaze, pseudoabducens palsy (also known as thalamic esotropia) or slower movements of the abducting eye than the adducting eye during horizontal saccades, see-saw nystagmus and associated ocular motility deficits including skew deviation, oculomotor nerve palsy, trochlear nerve palsy and internuclear ophthalmoplegia.