Parinaud's syndrome is an inability to move the eyes up and down. It is caused by compression of the vertical gaze center at the

rostral interstitial nucleus of medial longitudinal fasciculus The rostral interstitial nucleus of medial longitudinal fasciculus (riMLF) is a portion of the medial longitudinal fasciculus which controls vertical gaze. They project to the vestibular nuclei The vestibular nuclei (VN) are the cranial nucle ...

(riMLF). The eyes lose the ability to move upward and down. It is a group of abnormalities of

eye movement Eye movement includes the voluntary or involuntary movement of the eyes. Eye movements are used by a number of organisms (e.g. primates, rodents, flies, birds, fish, cats, crabs, octopus) to fixate, inspect and track visual objects of interest ...

and

pupil The pupil is a black hole located in the center of the Iris (anatomy), iris of the Human eye, eye that allows light to strike the retina.Cassin, B. and Solomon, S. (1990) ''Dictionary of Eye Terminology''. Gainesville, Florida: Triad Publishing ...

dysfunction. It is caused by

lesion A lesion is any damage or abnormal change in the tissue of an organism, usually caused by disease or trauma. ''Lesion'' is derived from the Latin "injury". Lesions may occur in plants as well as animals. Types There is no designated classif ...

s of the upper

brain stem The brainstem (or brain stem) is the posterior stalk-like part of the brain that connects the cerebrum with the spinal cord. In the human brain the brainstem is composed of the midbrain, the pons, and the medulla oblongata. The midbrain is co ...

and is named for Henri Parinaud (1844–1905), considered to be the father of French

ophthalmology Ophthalmology ( ) is a surgical subspecialty within medicine that deals with the diagnosis and treatment of eye disorders. An ophthalmologist is a physician who undergoes subspecialty training in medical and surgical eye care. Following a medic ...

Signs and symptoms

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. * Pseudo- Argyll Robertson pupils: Accommodative paresis ensues, and pupils become mid-dilated and show light-near dissociation. * Convergence-retraction

nystagmus Nystagmus is a condition of involuntary (or voluntary, in some cases) eye movement. Infants can be born with it but more commonly acquire it in infancy or later in life. In many cases it may result in reduced or limited vision. Due to the invol ...

: 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 An optokinetic drum—also called catford drum—is a rotating instrument to test vision in which individuals are seated facing the wall of the drum. The interior surface of the drum is normally striped; thus, as the drum rotates, the subject's eye ...

. *

Eyelid An eyelid is a thin fold of skin that covers and protects an eye. The levator palpebrae superioris muscle retracts the eyelid, exposing the cornea to the outside, giving vision. This can be either voluntarily or involuntarily. The human eye ...

retraction ( Collier's sign) * Conjugate down gaze in the primary position: "setting-sun sign". Neurosurgeons see this sign most commonly in patients with failed hydrocephalus shunts. It is also commonly associated with bilateral

papilledema Papilledema or papilloedema is optic disc swelling that is caused by increased intracranial pressure due to any cause. The swelling is usually bilateral and can occur over a period of hours to weeks. Unilateral presentation is extremely rare. In ...

. It has less commonly been associated with spasm of accommodation on attempted upward gaze, pseudoabducens palsy (also known as thalamic

esotropia Esotropia is a form of strabismus in which one or both eyes turns inward. The condition can be constantly present, or occur intermittently, and can give the affected individual a "cross-eyed" appearance. It is the opposite of exotropia and usual ...

) or slower movements of the abducting eye than the adducting eye during horizontal saccades, see-saw

and associated ocular motility deficits including skew deviation, oculomotor nerve palsy,

trochlear nerve The trochlear nerve (), ( lit. ''pulley-like'' nerve) also known as the fourth cranial nerve, cranial nerve IV, or CN IV, is a cranial nerve that innervates just one muscle: the superior oblique muscle of the eye, which operates through the pu ...

palsy and

internuclear ophthalmoplegia Internuclear ophthalmoplegia (INO) is a disorder of conjugate lateral gaze in which the affected eye shows impairment of adduction. When an attempt is made to gaze contralaterally (relative to the affected eye), the affected eye adducts minimally ...

Causes

400px, Cross section of midbrain. The area affected in Parinaud's syndrome is indicated by the striped region. Parinaud's syndrome results from injury, either direct or compressive, to the dorsal

midbrain The midbrain or mesencephalon is the forward-most portion of the brainstem and is associated with vision, hearing, motor control, sleep and wakefulness, arousal (alertness), and temperature regulation. The name comes from the Greek ''mesos'', " ...

. Specifically, compression or ischemic damage of the

mesencephalic The midbrain or mesencephalon is the forward-most portion of the brainstem and is associated with vision, hearing, motor control, sleep and wakefulness, arousal (alertness), and temperature regulation. The name comes from the Greek ''mesos'', " ...

tectum, including the superior colliculus adjacent

oculomotor The oculomotor nerve, also known as the third cranial nerve, cranial nerve III, or simply CN III, is a cranial nerve that enters the orbit through the superior orbital fissure and innervates extraocular muscles that enable most movements of ...

(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

pinealoma A pinealoma is a tumor of the pineal gland, a part of the brain that produces melatonin. If a pinealoma destroys the cells of the pineal gland in a child, it can cause precocious puberty. Signs and symptoms The pineal gland produces the hormone me ...

( intracranial germinomas) are the most common lesion producing this syndrome. * 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: obstructive

hydrocephalus Hydrocephalus is a condition in which an accumulation of cerebrospinal fluid (CSF) occurs within the brain. This typically causes increased pressure inside the skull. Older people may have headaches, double vision, poor balance, urinary inc ...

, midbrain hemorrhage,

cerebral arteriovenous malformation A cerebral arteriovenous malformation (cerebral AVM, CAVM, cAVM) is an abnormal connection between the arteries and veins in the brain—specifically, an arteriovenous malformation in the cerebrum. Signs and symptoms The most frequently observed ...

, trauma and brainstem

toxoplasmosis Toxoplasmosis is a parasitic disease caused by '' Toxoplasma gondii'', an apicomplexan. Infections with toxoplasmosis are associated with a variety of neuropsychiatric and behavioral conditions. Occasionally, people may have a few weeks or mont ...

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 Wilson's disease is a genetic disorder in which excess copper builds up in the body. Symptoms are typically related to the brain and liver. Liver-related symptoms include vomiting, weakness, fluid build up in the abdomen, swelling of the legs, ...

kernicterus Kernicterus is a bilirubin-induced brain dysfunction. The term was coined in 1904 by Christian Georg Schmorl. Bilirubin is a naturally occurring substance in the body of humans and many other animals, but it is neurotoxic when its concentratio ...

, and barbiturate overdose.

Diagnosis

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

Treatment is primarily directed towards etiology of the dorsal midbrain syndrome. A thorough workup, including

neuroimaging Neuroimaging is the use of quantitative (computational) techniques to study the structure and function of the central nervous system, developed as an objective way of scientifically studying the healthy human brain in a non-invasive manner. Incr ...

is essential to rule out anatomic lesions or other causes of this syndrome. Visually significant upgaze palsy can be relieved with bilateral

inferior rectus Inferior may refer to: * Inferiority complex * An anatomical term of location * Inferior angle of the scapula, in the human skeleton * ''Inferior'' (book), by Angela Saini * ''The Inferior'', a 2007 novel by Peadar Ó Guilín See also *Junior (di ...

recessions. Retraction nystagmus and convergence movement are usually improved with this procedure as well.

Prognosis

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.

References

External links

{{Lesions of spinal cord and brainstem Medical signs Disorders of ocular muscles, binocular movement, accommodation and refraction Syndromes affecting the nervous system