Patients suffering from Parinaud syndrome show vertical gaze palsy and other neurological deficits triggered by lesions of nerve nuclei located in the dorsal midbrain.
Presentation
The most common symptom of PS is the inability to look upwards. However, while patients are unable to move their eyes upwards, downward movements are only disturbed in severe cases of PS. Impairment of upward eye movement can result in a permanent downward gaze.
Patients suspicious for PS should undergo neurologic examinations to identify the precise location of neuronal damage. In this context, passive eye movements may be completely preserved, i.e., it should be possible to provoke full vertical eye movements by rotating the patient's head while they are focusing on an immobile object (doll’s eye maneuver). This test proves that gaze palsy is indeed caused by functional impairment of brain structures superior to motor neurons; the latter are working fine.
If patients try to force upward eye movements, they often provoke a pathologic nystagmus that results from simultaneous, uncontrolled contraction of several ocular muscles. Eyes converge in an attempt to look upwards, retract and repeat this operation. This kind of nystagmus can most easily be stimulated with an optokinetic drum turned downwards.
Horizontal eye movements are not commonly affected in PS. However, it has been described that lateral eye movements occur slower than medial eye movements. Therefore, there may be an incongruence between both eyes during horizontal movements.
Furthermore, patients show pupillary anomalies corresponding to pseudo-Argyll Robertson effects. Pupils are widened and do not contract upon stimulation with light, but they do narrow to focus on a near object. Accommodation reflexes may be altered. Such symptoms may be more pronounced after attempts to look upwards.
Additionally, PS patients may maintain their eyelids abnormally retracted.
In summary, leading symptoms for PS are:
- Vertical gaze palsy
- Setting-sun sign
- Convergence retraction nystagmus
- Light-near dissociation
- Collier's sign
An ophthalmologic examination may reveal a swollen head of the optic nerve.
Of note, additional symptoms may be present if further cerebral structures are affected by tumors, increased intracranial pressure, infection, inflammation or any other underlying disease. Eye movements not typically seen in PS may be disturbed due to oculomotor nerve palsy, trochlear nerve palsy or abducens nerve palsy, for example.
Entire Body System
- Cat Scratch
Parinaud syndrome Endocrinology An often unilateral eye problem accompanied by lymphadenopathy, which may be linked to infections–eg, tularemia and cat-scratch disease. See Cat-scratch disease, Tularemia. [medical-dictionary.thefreedictionary.com]
The authors report a case of Parinaud syndrome (conjunctivitis with pre-auricular satellite adenitis) caused by Bartonella henselae, the etiologic agent of Cat Scratch Disease. [ncbi.nlm.nih.gov]
The importance of a complete examination of the eyes and of skin testing patients who have preauricular adenopathy with cat-scratch-disease antigen is emphasized. [pediatrics.aappublications.org]
Rabbit and cat-scratch fever, the most common causes of Parinaud syndrome, can be avoided by not touching ticks, squirrels, or wild rabbits. Related wiseGEEK Articles [wisegeek.com]
Avoid being scratched by a cat, even a healthy cat. You can avoid tularemia by not having contact with wild rabbits, squirrels, or ticks. Cat scratch disease; Oculoglandular syndrome Bowling B, Conjunctiva. In: Bowling B, ed. [medlineplus.gov]
- Weakness
Facial nerve nucleus causing weakness of the facial muscles on the ipsilateral side. This is due to involvement of the corticobulbar tract. [lecturio.com]
The neurologic examination revealed a bilateral paralysis of upward gaze and a questionable weakness of convergence. The pupils constricted upon convergence but not in response to light. [lksom.temple.edu]
When protruded, the tongue points to the side of the lesion (i.e., the weak side). See Figure 1 3-7. Figure 14-1. [vertigoexercises.us]
[…] light uncontrollable, jerky eye movements, sometimes called convergence retraction nystagmus retraction of your eyelids blurry vision Depending on the underlying cause, you may notice other symptoms not related to your eyes, such as: slurred speech weak [healthline.com]
EXAM SYSTEMIC : Ataxia, sluggish speech, tremors of the right hand and weakness of lower extremities. OCULAR: Best corrected visual acuities: 20/25 OD and 20/25 OS. [eyerounds.org]
- Movement Disorder
Eye Movement Disorders When you look at an object, you're using several muscles to move both eyes to focus on it. If you have a problem with the muscles, the eyes don't work properly. There are many kinds of eye movement disorders. [icdlist.com]
Identifier: Daroff_02-1 Title: Parinaud's Syndrome or Dorsal Midbrain Syndrome Subject: Parinaud Syndrome; Dorsal Midbrain Syndrome; Eye Movement Disorders; Convergence Retraction Nystagmus Descrsiption: Also known as the pretectal, or sylvian aqueduct [kaltura.com]
Variantes de point d'accès Ocular Motility Disorder [Nom commun] Eye Movement Disorders [Nom commun] Eye Movement Disorder [Nom commun] Eye Motility Disorders [Nom commun] Eye Motility Disorder [Nom commun] Troubles de l'appareil moteur de l'oeil [Nom [idref.fr]
Identifier Daroff_02-1 Title Parinaud's Syndrome or Dorsal Midbrain Syndrome Subject Parinaud Syndrome; Dorsal Midbrain Syndrome; Eye Movement Disorders; Convergence Retraction Nystagmus Description Also known as the pretectal, or sylvian aqueduct syndromes [collections.lib.utah.edu]
- Pallor
Compressive or infiltrative optic neuropathies may present with unilateral or bilateral vision loss (depending on the extent of the lesion), reduced visual acuity, color vision loss, afferent pupillary defect, and optic nerve pallor. [ajnr.org]
- Malaise
Typical symptoms include regional lymphadenopathy, fever, malaise, and fatigue, possibly with more severe complications such as splenomegaly, granulomatous hepatitis, and encephalopathy. [dial.uclouvain.be]
Gastrointestinal
- Nausea
The vestibular nuclei causing vertigo, nausea, and vomiting. The cochlear nuclei resulting in the loss of hearing sensation. The cerebellar tracts causing ipsilateral ataxic gait. [lecturio.com]
His symptoms have been associated with episodes of nausea and he has complained of a headache. Physical exam is notable for an upward gaze palsy and a pupillary light-near dissociation. An MRI of the brain demonstrates a pinealoma. [step2.medbullets.com]
[…] of Intracranial Aneurysms 226 Venous Angiomas Developmental Venous Anomaly 227 Capillary Angiomas and Telangiectasia 228 Presenting Symptoms 229 Outcomes 230 Moyamoya Syndrome 231 Outcomes 232 Nursing Care for Vascular Brain Lesions 233 Management of Nausea [books.google.de]
Cardiovascular
- Thrombosis
Developed lower limb deep venous thrombosis 6 months prior to presentation. FH significant for blood coagulopathies in multiple family members. EXAM SYSTEMIC : Ataxia, sluggish speech, tremors of the right hand and weakness of lower extremities. [eyerounds.org]
James Paget first proposed the idea of venous thrombosis causing upper extremity pain and swelling, and Leopold von Schrötter later linked the clinical syndrome to thrombosis of the axillary and subclavian veins. [context.reverso.net]
Thrombosis of the proximal part of the subclavian artery (left) results in retrograde blood flow through the ipsilateral vertebral artery and into the left subclavian artery. [vertigoexercises.us]
The most important concern for the neuroradiologist is to exclude sinus thrombosis. [ajnr.org]
Musculoskeletal
- Muscle Spasm
spasms To diagnose Parinaud syndrome, your doctor will likely start by giving you a thorough eye exam to test your eyes’ movement abilities. [healthline.com]
Skin
- Erythema
First" = ATF = Anterior Talofibular ligament "RICE Right Now" = Rest, Ice, Compression, Elevation, Range of motion exercises, Nsaids "JONES" Criteria: Joint (migratory polyarthritis), O looks like a heart (endocarditis), Nodules in skin (subcutaneous), Erythema [quizlet.com]
Eyes
- Diplopia
43-year-old male referred for evaluation of binocular diplopia February 21, 2005 Chief Complaint: 43-year-old male referred for evaluation of binocular diplopia. [eyerounds.org]
The commonest presenting symptoms were diplopia (67.5%) and blurred vision (25%) followed by visual field defect (12.5%), ataxia (7.5%) and manifest squint (7.5%). [ncbi.nlm.nih.gov]
[…] midbrain syndrome often associated with bilateral superior oblique palsy and both these condition lead to oscillopsia, diplopia, and head posture abnormality. [syndromespedia.com]
Snapshot A 5-year-old boy is brought to his pediatriatrician for diplopia. His mother noticed he is unable to look up at her. His symptoms have been associated with episodes of nausea and he has complained of a headache. [step2.medbullets.com]
- Vertical Gaze Palsy
The commonest presenting signs were vertical gaze palsy (100%), convergence-retraction nystagmus (87.5%) and light-near dissociation (65.0%). [ncbi.nlm.nih.gov]
Vertical gaze palsy may thus result from lesions to the medial longitudinal fasciculus, the posterior commissure or the midbrain itself. [symptoma.com]
The signs of dorsal midbrain syndrome seen in this patient were light near dissociation, convergence retraction nystagmus, and vertical gaze palsy. [eyerounds.org]
From Wikipedia, the free encyclopedia Parinaud's syndrome Other names Dorsal midbrain syndrome, vertical gaze palsy, upward gaze palzy, sunset sign,[1] setting-sun sign,[2] sun-setting sign,[3] sunsetting sign,[4] sunset eye sign,[5] setting-sun phenomenon [en.wikipedia.org]
This case report further supports current limited literature that suggests that thalamic lesions may also manifest as vertical gaze palsies. [sciencedomain.org]
- Strabismus
Wong AM, Colpa L, Chandrakumar M (2011) Ability of an upright-supine test to differentiate skew deviation from other vertical strabismus causes. [link.springer.com]
H50.8 Other specified strabismus H50.89 Other specified strabismus H50.9 Unspecified strabismus H51 Other disorders of binocular movement H51.0 Palsy (spasm) of conjugate gaze H51.1 Convergence insufficiency and excess H51.11 Convergence insufficiency [icd10data.com]
Two common ones are Strabismus - a disorder in which the two eyes don't line up in the same direction. This results in "crossed eyes" or "walleye." [icdlist.com]
Decompensated strabismus may be diagnosed if there is a history of strabismus or other clinical signs of strabismus. [dizziness-and-balance.com]
He investigated ocular movements and strabismus, Parinaud’s oculo-glandular syndrome (often caused by Bartonella henselae, responsible for cat scratch disease or tularaemia). His studies of lachrymal infections are well known. [jnnp.bmj.com]
- Esotropia
Postoperatively the torsion was significantly corrected, and the patient had a moderate residual esotropia in primary gaze. [eyerounds.org]
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 [en.wikipedia.org]
อาการมักมี bilateral papilledema ร่วมด้วย บางครั้งอาจพบ(ไม่บ่อย) ว่ามี -spasm of accommodation เมื่อผู้ป่วยพยายามกลอกตามองบน -pseudoabducens palsy (also known as thalamic esotropia) or slower movements of the abducting eye than the adducting eye during [neurologycoffecup.wordpress.com]
- Blurred Vision
The commonest presenting symptoms were diplopia (67.5%) and blurred vision (25%) followed by visual field defect (12.5%), ataxia (7.5%) and manifest squint (7.5%). [ncbi.nlm.nih.gov]
vision at near Oscillopsia Accompanying neurological symptoms TREATMENT Treatment is primarily directed towards the etiology of dorsal midbrain syndrome. [eyerounds.org]
Symptoms at presentation (Table 1 ) included headache or vomiting in 5 patients, blurred vision or “bumping into objects” in 2 patients, and hearing loss with vertigo in 1 patient. [healio.com]
Results: A 15 years old male presented with morning headaches, blurred vision, bilateral papilledema, failure of upward gaze, bilateral tinnitus and decreased hearing acuity. [oncogen.org]
Psychiatrical
- Suggestibility
It showed a well-defined solid lesion in right upper lobe suggestive of a lung carcinoma [Figure 4]. Considering the age and built of the patient a CT-guided biopsy from the lung lesion was done; biopsy was suggestive of adenocarcinoma. [annalsofian.org]
CIS consisting of unilateral optic neuritis, incomplete transverse myelitis, internuclear ophthalmoplegia, trigeminal neuralgia, and paroxysmal attacks are particularly suggestive of MS. [reviewofophthalmology.com]
There is a suggestion of convergence and retraction movements when the patient attempts to make voluntary upward saccades. [opt.indiana.edu]
This case report further supports current limited literature that suggests that thalamic lesions may also manifest as vertical gaze palsies. [sciencedomain.org]
CT examination indicated posttraumatic lesions in the dorsal midbrain and in the cavity of third ventricle, suggestive of acute hemorrhages. [foliamedica.bg]
Neurologic
- Nystagmus
Retraction nystagmus and convergence movement are usually improved with this procedure as well. [en.wikipedia.org]
The commonest presenting signs were vertical gaze palsy (100%), convergence-retraction nystagmus (87.5%) and light-near dissociation (65.0%). [ncbi.nlm.nih.gov]
The signs of dorsal midbrain syndrome seen in this patient were light near dissociation, convergence retraction nystagmus, and vertical gaze palsy. [eyerounds.org]
Supplementary material Online Resource 1 Converge-retraction nystagmus on attempting an upward gaze (MPG 2190 kb) References 1. [link.springer.com]
; In addition you might see a very unusual movement called convergence retraction, and even though it's called nystagmus, it really isn't rhythmic, so it's really a "nystagmoid" movement and not a "nystagmus". [collections.lib.utah.edu]
- Paresis
The authors report a patient presenting with a 1-week history of paresis of upward gaze as his initial manifestation of demyelinating disease. [ncbi.nlm.nih.gov]
; For an explanation of CRN/upgaze paresis in the Parinaud's syndrome, see video "Parinaud's in a man with glioblastoma multiforme of the pineal gland." [collections.lib.utah.edu]
In the study of motor paralysis of the eye one seems to assume that the lesion is always peripheral…But, more often, the lesion is central…] He referred to Louis Foville’s (1799−1878) case of facial paralysis and conjugate gaze palsy to the left and paresis [jnnp.bmj.com]
[…] of convergence Spasm/paresis of accomodation Pseudoabducens palsy (thalamic esotropia) Associated Ocular Motility Deficits Skew deviation Third nerve palsy Internuclear ophthalmoplegia See-saw nystagmus SYMPTOMS Difficulty looking up Diplopia Blurred [eyerounds.org]
Secondary Components Convergence of spasm or paresis Accommodation of spasm or paresis Thalamic esotropia or pseudoabducens palsy Associated Ocular Motility Deficits Skew divergence Nerve palsy at third position Internuclear ophthalmoplegia See-saw nystagmus [syndromespedia.com]
- Headache
Case reports A 3-year-old boy presented with headache, disturbance of consciousness, and Parinaud’s syndrome. Magnetic resonance (MR) imaging revealed a pineal mass lesion, and total resection of the tumor was achieved. [neurocirugia.com]
His symptoms have been associated with episodes of nausea and he has complained of a headache. Physical exam is notable for an upward gaze palsy and a pupillary light-near dissociation. An MRI of the brain demonstrates a pinealoma. [step2.medbullets.com]
Symptoms at presentation (Table 1 ) included headache or vomiting in 5 patients, blurred vision or “bumping into objects” in 2 patients, and hearing loss with vertigo in 1 patient. [healio.com]
- Convergence Nystagmus
The clinical features are: a loss of vertical gaze nystagmus on attempted convergence pseudo-Argyll-Robertson pupils: large pupils with sluggish reaction to light Bilateral papilloedema is common. [gpnotebook.co.uk]
: Nystagmus provoqués par le regard en haut et la convergence. In: Les nystagmus. Masson, Paris 1973 Google Scholar [10] Hatcher M.A., Klintworth G.K. : The sylvian aqueduct syndrome. Arch. [link.springer.com]
During the crisis of intracranial hypertension, all of them developed upward gaze palsy and variable abnormalities of the convergence mechanism such as paralysis, spasm, and convergence nystagmus. [ncbi.nlm.nih.gov]
The main clinical manifestations were that of a severe restriction in upward gaze, and convergence-retraction nystagmus. [sciencedomain.org]
All presented with limited upward voluntary gaze and convergence nystagmus with attempted upward voluntary gaze. [n.neurology.org]
- Oscillopsia
He also complained about oscillopsia that was more pronounced in upgaze. The family members noticed an anomalous chin up position subsequent to the CVA. [eyerounds.org]
[…] vertical and diplopia. 1,2 Alternatively, Parinaud Syndrome is also known as dorsal midbrain syndrome. [2] Discussion Parinaud Syndrome or dorsal midbrain syndrome often associated with bilateral superior oblique palsy and both these condition lead to oscillopsia [syndromespedia.com]
Symptoms Parinaud Syndrome has following symptoms Looking upward direction is difficult Double vision or Diplopia: Horizontal or vertical two images formation of a single object Near vision become blurred Oscillopsia, in which patient experience unclear [healthmaza.com]
Patients may complain of difficulty looking up, blurred near vision, diplopia, oscillopsia, and associated neurological symptoms. Diplopia is present in 65 percent of patients. [ncbi.nlm.nih.gov]
Workup
Clinical and ophthalmologic examination allow for diagnosis of PS. In order to establish a therapeutic scheme, the underlying disease needs to be identified. Age, gender and a possible medical history of demyelinating diseases such as multiple sclerosis may give important hints as to the most likely cause of PS in this specific case.
Diagnostic imaging, particularly magnetic resonance imaging and computed tomography scans, are applied to identify space-occupying masses in the pineal gland and the midbrain. These same techniques allow for an evaluation of the ventricular system. Findings may point at an increased intracranial pressure due to hydrocephalus. Disseminated foci of inflammation and reactive gliosis, also visible in magnetic resonance imaging, are characteristic of multiple sclerosis. Older patients should undergo the aforementioned scans immediately since they may have sustained cerebral infarction and the time window for stroke treatment is very narrow.
Microbiology
- Bartonella Henselae
The authors report a case of Parinaud syndrome (conjunctivitis with pre-auricular satellite adenitis) caused by Bartonella henselae, the etiologic agent of Cat Scratch Disease. [ncbi.nlm.nih.gov]
henselae, principally affecting children under 15 years of age. [dial.uclouvain.be]
The most common bacterial cause is Bartonella henselae, which is called cat-scratch disease due to contraction from exposure to cats and their fleas. [visualdx.com]
He investigated ocular movements and strabismus, Parinaud’s oculo-glandular syndrome (often caused by Bartonella henselae, responsible for cat scratch disease or tularaemia). His studies of lachrymal infections are well known. [jnnp.bmj.com]
Treatment
Treatment largely depends on the underlying disease. If it is curable, neurologic deficits affecting eye movements should resolve within a few months time. Only in non-curable cases, e.g., when a patient suffers from developmental defects that cannot be treated, surgical interventions can be considered. Vertical gaze palsy in PS is caused by paresis of the superior rectus muscle and that, in turn, means that the inferior rectus muscle is missing its antagonist. Thus, the recession of the latter may help to correct a constant downward gaze. This same procedure is usually applied to correct convergence-retraction nystagmus.
Prognosis
Prognosis largely depends on the underlying disease. If it can be treated, neurologic deficits usually diminish over the course of several months. In the case of increased intracranial pressure due to hydrocephalus, abrupt relief may be associated with rapid resolution of neurological symptoms.
Etiology
Any disease that may damage the superior colliculus of the tectum, the posterior commissure, and the rostral interstitial nucleus of medial longitudinal fasciculus, which are the main parts of the cerebrum that affect vertical eye movement, may possibly cause PS.
Older studies almost exclusively name tumors of the pineal gland as triggers of PS [1] [3] [4] [5] [6] [7]. These space-occupying masses presumably compress the posterior commissure and as they are rarely located in the median plane, they generally cause unilateral symptoms. Of note, intrinsic tumors of the midbrain or neoplasms of any other adjacent tissue may lead to similar neurologic deficits. Depending on the precise location of such neoplasias, other symptoms may, however, dominate the clinical picture. Because in the ventral direction, the posterior commissure is delimited by the third ventricle, increasing pressures due to hydrocephalus may also damage this structure and cause PS.
Cerebral ischemia, infection, and inflammation affecting any of the aforementioned anatomical structures are other possible causes of PS. With regards to infectious diseases, brain stem toxoplasmosis, for instance, may trigger this disease.
In some cases, degenerative alterations that may occur in the course of multiple sclerosis, other demyelinating disorders or progressive supranuclear palsy may account for the neurological deficits observed in PS patients. Metabolic disorders such as congenital Niemann-Pick disease and Wilson's disease as well as acquired kernicterus may cause PS. This condition has also been related to barbiturate abuse [8]. Rarely, hemorrhages and developmental defects manifesting as vascular malformations have been reported as triggers of PS.
Epidemiology
PS patients most commonly belong to one of three major risk groups.
On the one hand, there are those patients that suffer from neoplasms affecting their pineal gland or midbrain. With regards to the pineal gland, germinomas are most frequently detected, but astrocytoma and pineocytoma may also develop. These space-occupying masses compress cerebral structures in close proximity that are involved in eye movement. The corresponding patients are usually adolescents, sometimes prepubertal children.
On the other hand, there are young women previously diagnosed with multiple sclerosis. Here, demyelination damages neuronal structures and impairs their function.
Finally, PS is often diagnosed in older patients that recently suffered a cerebral infarction.
Pathophysiology
The most common symptoms of PS are vertical gaze palsy, blunt pupillary reflexes as well as nystagmus, but other ocular and neurologic symptoms may be present [8] [9] [10]. To comprehend the pathophysiology of this disease, the neuroanatomical basis for the above-mentioned movements and reflexes needs to be understood.
The medial longitudinal fasciculus comprises axons of cranial nerves III, IV and V (oculomotor nerve, trochlear nerve, and abducens nerve, respectively). These nerves are essential for a wide variety of eye movements, e.g., for vertical gaze control and many fibers cross the posterior commissure. The medial longitudinal fasciculus ascends to the interstitial nuclei of Cajal in the midbrain and the oculomotor nuclei [8] [10] [11]. Here, signals are transduced to the cortex. Vertical gaze palsy may thus result from lesions to the medial longitudinal fasciculus, the posterior commissure or the midbrain itself.
The neuronal chain that controls the pupillary reflex consists of retinal neurons, pretectal nuclei, the Erdinger-Westphal nuclei in the rostral midbrain and finally the ciliary ganglions from which the iris sphincter muscle is innervated. Both the pretectal nuclei as well as the Erdinger-Westphal nuclei may directly be affected in PS. The Erdinger-Westphal nuclei are also involved in the accommodation reflex and therefore, a blunt pupillary reflex may be observed.
As can be seen, eye movements largely depend on midbrain structures, adjacent nuclei as well as on axons that ascend to and descend from these nuclei. It has been stated that lesions of the medial longitudinal fasciculus and the posterior commissure most frequently account for PS [10].
Prevention
No preventive measures can be recommended.
Summary
Parinaud syndrome (PS) is named in honor of Henri Parinaud, a French ophthalmologist, and neurologist who practiced in the second half of the 19th century. His two areas of expertise combine perfectly to understand this disease, which is characterized by the patient's inability to perform certain eye movements, most notably to control their gaze in the vertical direction [1] [2]. These are neurological deficits that manifest because of damage to the dorsal midbrain, which is why PS is also designated dorsal midbrain syndrome.
Eye movements are principally controlled by the superior colliculus of the tectum, which is located in the dorsal part of the midbrain. In patients suffering from PS, this and possibly adjacent structures, e.g, the pretectal area, the posterior commissure and the rostral interstitial nucleus of medial longitudinal fasciculus, are compromised by any underlying disease. In this context, a neoplasm in close proximity that compresses the midbrain, ischemia due to cerebral infarction or infectious and inflammatory processes may trigger PS. Tumors of the pineal gland or midbrain structures are the most common causes of PS.
Because the above-mentioned structures are not only involved in vertical gaze control, PS patients typically show additional symptoms. Their pupillary reflex is disturbed and a pathological nystagmus, as well as unilateral or bilateral eyelid retraction, may be present.
Patient Information
The medical term Parinaud syndrome (PS) refers to an eye disease comprising the inability to gaze upwards, a blunted pupillary reflex and nystagmus, i.e., uncontrolled, rhythmic eye movement. The disease is also called dorsal midbrain syndrome because damage to neuronal structures located in this part of the brain account for the aforementioned symptoms.
Causes
Any disease compromising neuronal structures involved in eye movement control may trigger PS. Most frequently, tumors of the midbrain itself or of the pineal gland, a hormonal gland in close proximity to the midbrain, cause PS by compressing the neighboring tissue. Such tumors are often diagnosed in young people. A second, also very common cause of PS is an increased intracranial pressure, a hydrocephalus. This condition will affect other cerebral structures as well. The latter also applies to degenerative diseases like multiple sclerosis, infection, and inflammation of brain structures. Older people showing signs of PS may have sustained a stroke.
Symptoms
PS patients are unable to look upwards, but usually, don't have any problems to gaze downwards. If they strain to look up, contraction of lower eye muscles may lead to the aforementioned nystagmus. The pupils of PS patients are often widened and don't contract upon light exposure. They do, however, narrow to focus on near objects.
More generalized conditions may trigger additional symptoms.
Diagnosis
Diagnosis of PS is based on clinical and ophthalmological examination. However, further diagnostic measures have to be applied to identify the cause of PS. Most commonly, imaging techniques such as magnetic resonance imaging and computed tomography scans are applied to this end. They may help to identify tumors, obstructed cerebral ventricle and tissue inflammation.
Treatment
The underlying disease needs to be treated. If this is possible, neurologic deficits usually resolve over the course of time. In some severe cases with a persistent downward gaze, surgery may be considered.
References
- Keane JR. The pretectal syndrome: 206 patients. Neurology. 1990; 40(4):684-690.
- Slyman JF, Kline LB. Dorsal midbrain syndrome in multiple sclerosis. Neurology. 1981; 31(2):196-198.
- Kortman K, Bradley WG. Supratentorial neoplasms. In: Stark DD, Bradley WG, eds. Magnetic resonance imaging. St. Louis: Mosby; 1988:375-424.
- Berman SA, Hayman LA, Hinck VC. Cerebral vascular territories: anatomic-functional correlation with axial and coronal images. In: Latchaw RE, ed. MR and CT imaging of the head, neck and spine. Vol 2. St. Louis: Mosby; 1991:45-62.
- Atlas S. lntraaxial brain tumors. In: Atlas S, ed. Magnetic resonance imaging of the brain and spine. New York: Raven; 1991:223-326.
- Williams AL. Tumors. In: Williams AL, Haughton VM, eds. Cranial computed tomography: a comprehensive text. St. Louis: Mosby; 1985:148-239.
- Barkovich AJ, Edwards MSB. Brain tumors of childhood. In: Barkovich AJ, ed. Pediatric neuroimaging. New York: Raven; 1990:149-203.
- Leigh RJ, Zee DS. Contemporary neurology series: the neurology of eye movements. Vol 23. Philadelphia: Davis; 1991:439-444.
- Costantino A, Black SE, Carr T, Nicholson RL, Noseworthy JH. Dorsal midbrain syndrome in multiple sclerosis with magnetic resonance imaging correlation. Can J Neurol Sci. 1986; 13(1):62-65.
- Buttner-Ennever JA, Buttner U, Cohen B, Baumgartner G. Vertical glaze paralysis and the rostral interstitial nucleus of the medial longitudinal fasciculus. Brain. 1982; 105(Pt 1):125-149.
- Lavin PJM. Conjugate and disconjugate eye movements. In: Walsh TJ, ed. Neuro-ophthalmoiogy: clinical signs and symptoms. Philadelphia: Lea & Febiger; 1985:412-421.