Parinaud's syndrome
Parinaud's syndrome is a constellation of neurological signs indicating injury to the dorsal midbrain, more specifically compression of the vertical gaze center at the rostral interstitial nucleus of medial longitudinal fasciculus (riMLF).
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It is a group of abnormalities of eye movement and pupil dysfunction.
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It is a group of abnormalities of eye movement and pupil dysfunction
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It is named for Henri Parinaud, considered the father of French ophthalmology.
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named for Henri Parinaud [ 6 ] [ 7 ] (1844–1905), considered to be the father of French ophthalmology
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It results from injury, either direct or compressive, to the dorsal midbrain.
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Parinaud's syndrome results from injury, either direct or compressive, to the dorsal midbrain
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It involves compression or ischemic damage of the mesencephalic tectum including the superior colliculus, oculomotor and Edinger-Westphal nuclei.
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compression or ischemic damage of the mesencephalic tectum, including the superior colliculus adjacent oculomotor (origin of cranial nerve III) and Edinger-Westphal nuclei, causing dysfunction to the motor function of the eye
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Paralysis of upwards gaze with downward gaze usually preserved; the palsy is supranuclear.
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Paralysis of upwards gaze: Downward gaze is usually preserved. This vertical palsy is supranuclear
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In extreme form, conjugate down gaze in primary position known as the setting-sun sign is observed.
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In the extreme form, conjugate down gaze in the primary position, or the "setting-sun sign" is observed
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Pseudo-Argyll Robertson pupils with accommodative paresis, mid-dilated pupils, and light-near dissociation.
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Pseudo- Argyll Robertson pupils : Accommodative paresis ensues, and pupils become mid-dilated and show light-near dissociation
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Convergence-retraction nystagmus where attempts at upward gaze cause eyes to pull in and globes to retract.
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Convergence-retraction nystagmus : Attempts at upward gaze often produce this phenomenon. On fast up-gaze, the eyes pull in and the globes retract
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Eyelid retraction known as Collier's sign.
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Eyelid retraction ( Collier's sign )
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Commonly associated with bilateral papilledema.
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It is also commonly associated with bilateral papilledema
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Less commonly associated with spasm of accommodation on attempted upward gaze, pseudoabducens palsy, see-saw nystagmus, and other ocular motility deficits.
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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
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Diagnosis can be made via physical exam, particularly deficits of relevant cranial nerves.
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Diagnosis can be made via combination of physical exam, particularly deficits of the relevant cranial nerves
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Confirmation can be made via imaging such as CT scan or MRI.
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Confirmation can be made via imaging, such as CT scan or MRI
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Treatment is primarily directed towards the etiology of the dorsal midbrain syndrome.
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Treatment is primarily directed towards etiology of the dorsal midbrain syndrome
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A thorough workup including neuroimaging is essential to rule out anatomic lesions or other causes.
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A thorough workup, including neuroimaging is essential to rule out anatomic lesions or other causes of this syndrome
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Visually significant upgaze palsy can be relieved with bilateral inferior rectus recessions.
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Visually significant upgaze palsy can be relieved with bilateral inferior rectus recessions
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Retraction nystagmus and convergence movement are usually improved with bilateral inferior rectus recessions as well.
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Retraction nystagmus and convergence movement are usually improved with this procedure as well
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Classically associated with brain tumors in the pineal gland or midbrain in young patients, causing hydrocephalus.
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Young patients with brain tumors in the pineal gland or midbrain , causing hydrocephalus
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Associated with multiple sclerosis in women in their 20s-30s.
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Women in their 20s-30s with multiple sclerosis
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Associated with stroke of the upper brainstem in older patients.
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Older patients following stroke of the upper brainstem
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Other causes include hydrocephalus, midbrain hemorrhage, cerebral arteriovenous malformation, trauma, and brainstem toxoplasmosis infection.
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hydrocephalus , midbrain hemorrhage, cerebral arteriovenous malformation , trauma and brainstem toxoplasmosis infection
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Neoplasms and giant aneurysms of the posterior fossa have also been associated.
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Neoplasms and giant aneurysms of the posterior fossa have also been associated with the midbrain syndrome
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Associated with metabolic disorders such as Niemann-Pick disease, Wilson's disease, kernicterus, and barbiturate overdose.
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Vertical supranuclear ophthalmoplegia has also been associated with metabolic disorders, such as Niemann-Pick disease , Wilson's disease , kernicterus , and barbiturate overdose
The eye findings generally improve slowly over months, especially with resolution of the causative factor; continued resolution after the first 3–6 months of onset is uncommon, though rapid resolution after normalization of intracranial pressure following ventriculoperitoneal shunt placement has been reported.
| Parinaud's syndrome | |
|---|---|
| Other names | Dorsal midbrain syndrome, vertical gaze palsy, upward gaze palsy, sunset sign,[1] setting-sun sign,[2] sun-setting sign,[3] sunsetting sign,[4] sunset eye sign,[5] setting-sun phenomenon[5] |
| Specialty | Neurology |
Parinaud's syndrome is a constellation of neurological signs indicating injury to the dorsal midbrain. More specifically, compression of the vertical gaze center at the rostral interstitial nucleus of medial longitudinal fasciculus (riMLF).
It is a group of abnormalities of eye movement and pupil dysfunction and is named for Henri Parinaud[6][7] (1844–1905), considered to be the father of French ophthalmology.
Parinaud's syndrome is a cluster of abnormalities of eye movement and pupil dysfunction, characterized by:
- Paralysis of upwards gaze: 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. In the extreme form, conjugate down gaze in the primary position, or the "setting-sun sign" is observed. Neurosurgeons see this sign most commonly in patients with hydrocephalus.[8]
- 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.[9]
- Eyelid retraction (Collier's sign)
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.
Parinaud's syndrome results from injury, either direct or compressive, to the dorsal midbrain. Specifically, compression or ischemic damage of the mesencephalic tectum, including the superior colliculus adjacent oculomotor (origin of cranial nerve III) and Edinger-Westphal nuclei, causing dysfunction to the motor function of the eye.
Classically, it has been associated with three major groups:
- Young patients with brain tumors in the pineal gland or midbrain, causing hydrocephalus
- Women in their 20s-30s with multiple sclerosis
- Older patients following stroke of the upper brainstem
However, any other compression, ischemia or damage to this region can produce these phenomena: 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.
Diagnosis can be made via combination of physical exam, particularly deficits of the relevant cranial nerves. Confirmation can be made via imaging, such as CT scan or MRI.
Treatment is primarily directed towards etiology of the dorsal midbrain syndrome. A thorough workup, including neuroimaging is essential to rule out anatomic lesions or other causes of this syndrome. Visually significant upgaze palsy can be relieved with bilateral inferior rectus recessions. Retraction nystagmus and convergence movement are usually improved with this procedure as well.
The eye findings of Parinaud's syndrome generally improve slowly over months, especially with resolution of the causative factor; continued resolution after the first 3–6 months of onset is uncommon. However, rapid resolution after normalization of intracranial pressure following placement of a ventriculoperitoneal shunt has been reported.
- ↑ Larner AJ (2001). A Dictionary of Neurological Signs: Clinical Neurosemiology. Springer Science & Business Media. p. 202. ISBN 978-1-4020-0042-3.
- ↑ Biglan AW (January 1984). "Setting Sun Sign in Infants". American Orthoptic Journal. 34 (1): 114–116. doi:10.1080/0065955X.1984.11981637.
- ↑ Lee EQ, Schiff D, Wen PY (28 September 2011). Neurologic Complications of Cancer Therapy. Demos Medical Publishing. p. 383. ISBN 978-1-61705-019-0.
- ↑ Waterston T, Helms P, Ward-Platt M (6 July 2016). Paediatrics: A Core Text on Child Health, Second Edition. CRC Press. p. 149. ISBN 978-1-138-03131-9.
- 1 2 Gaillard F. "Sunset eye sign". Radiopaedia. Archived from the original on 19 August 2019. Retrieved 5 January 2020.
- ↑ synd/1906 at Whonamedit?
- ↑ Parinaud H (1883). "Paralysie des mouvements associés des yeux". Archives de neurologie (in French). 5. Paris: 145–172.
- ↑ "Neuro-Ophthalmic Examination". Archived from the original on 1 September 2009. Retrieved 14 December 2008.
- ↑ "Convergence-retraction nystagmus". www.aao.org. Archived from the original on 14 September 2016. Retrieved 17 March 2020.
- Aguilar-Rebolledo F, Zárate-Moysén A, Quintana-Roldán G (1998). "Parinaud's syndrome in children". Rev. Invest. Clin. (in Spanish). 50 (3): 217–20. PMID 9763886.
- Waga S, Okada M, Yamamoto Y (1979). "Reversibility of Parinaud syndrome in thalamic hemorrhage". Neurology. 29 (3): 407–9. doi:10.1212/wnl.29.3.407. PMID 571990. S2CID 42247406.