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Pertemuan Ilmiah Tahunan Perdami ke-40

DORSAL MIDBRAIN SYNDROME


By
Rusti Hanindya Sari
NEURO-OPHTHALMOLOGY UNIT
NATIONAL EYE CENTRE CICENDO EYE HOSPITAL
2015

Ophthalmoplegia is an obscure problem for ophthalmologist, and


other associated specialties. Ophthalmoplegia makes many physicians to
refer the patients to neurolosits and/or ophthalmologist for neuroophthalmic evaluation. It has different etiologies some of which may be
very harmful and need urgent intervention. The responsibility of the
ophthalmologist is to provide symptomatic relief, determine the source of
the problem, and to arrange for appropriate consultation with other clinical
disciplines.1,2
The study of eye movement is a source of valuable information to
clinicians. To ophthalmologist, abnormalities of ocular motility are
frequently the clue to the localization of a disease process. Eye movement
is also being used to identify and test new therapies for arrange of genetic
and degenerative disorder.3
The effects of focal disease in the nervous systems, especially
abnormal eye movement can aid the ophthalmologist in topological
diagnosis.4 In this paper, we will discuss about the effect of focal disease
at the level of the midbrain.

ANATOMY
The midbrain represent the uppermost portion of the brainstem,
containing numerous important nuclei and white matter tract, most of
which are involved in motor control, as well as auditory and visual
pathways.5
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(A).

(B).

Figure 1. The brainstem The Midbrain. (A) Dorsal view, (B).


Ventral view

The interior contents of the midbrain can be anatomically segmented


in a dorsal to ventral fashion, including tectum, tegmentum, and basis. The
tectum is the most dorsal aspect of the midbrain and includes the
quadrigeminal plate as well as additional gray matter and fiber tracts
residing dorsal to the cerebral aqueduct. The centrally located tegmentum
represents the largest portion of midbrain realestate and contains cranial
nerve nuclei in addition to gray matter and fiber tracts. The remaining
ventral midbrain is termed the basis and is comprised of cerebral
peduncles, substantia nigra, crus cerebri, corticobulbar fibers.5,6

Figure 2. Midbrain Lateral view

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The external midbrain displays distinct landmarks for easy


identification. The cerebral peduncles lie along the ventral aspect of the
midbrain, just superior to the pons. In addition, the quadrigemnial plate
includes the superior and inferior colliculi, which are noted as two paired
eminences along the dorsal surface of the midbrain. The oculomotor nerve
(cranial nerve III) can be identified along the ventral midbrain within the
groove formed between the cerebral peduncles. The trochlear nerve
(cranial nerve IV) emerges from the dorsal midbrain near the inferior
colliculus.5,6
The tegmentum of the pons contain the neural machinery that
ultimately controls horizontal conjugate eye movements. The most
important structure is the abduscens nucleus, which controls conjugate
movements of both the ipsilateral lateral rectus and the contralateral
medial rectus muscles, and so may be regarded as the horizontal gaze
center. Axons of abducens internuclear neurons across the midline to
ascend in the medial longitudinal fasciculus to contact contralateral medial
rectus motorneurons in the oculomotor nucleus.7
The ocular motorneurons concerned with vertical and torsional eye
movement lie in the oculomotor nucleus and trochlear nucleus. Vertical
and torsional saccades are generated in the rostal interstitial nucleus of
the medial longitudinal fasciculus (riMLF), a wing shape structure that lies
dorsomedial to rednucleus, rostal to interstitial of Cajal (INC), and caudal
to the posterior branch branch of the thalamo-subthalamic paramedian
artery. Each riMLF contains neuron that burst for upward and downward
eye movement, but for the torsional quick phase in only one direction.
Burst neurons for clockwise movements (right eye extorts, left eye intorts)
lie in the right riMLF; those for counterclockwise movements lie in the left
riMLF.7
A critical structure for the vertical gaze holding (the nueral integrator)
is the INC. The INS receives inputs from the vestibular nuclei, y-group,
and axon collaterals from burst neuron in riMLF. In addition, the INC may
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house inhibitory burst neurons for vertical and torsional saccades. The
INC projects to vertical motorneurons in the oculomotor and trochlear
subnuclei on the contralateral side of the brainstem via posterior
commissure. The INC also contains neurons that project to motor neurons
of the neck and trunck muscles, and appears to coordinate combines eyehead movements in torsional and vertical planes.7

Figure 3. Midbrain anatomy at the level of superior colliculus.


Posterior commissure contains axons from INC projecting to
contralateral CN III, CN IV, and INC. It also contains axons from the
nucleus of the posterior commissure projecting the contralateral ri MLF
and INC, which may be important for upgaze; and to the M group of
neurons, which may be important for coordination of vertical eye and lid
movements.7
The midbrain is important in control of vertical eye movements,
especially saccades and gaze holding. Lesion within the midbrain may
result in distinct syndromes, but given the close proximity of these vital
nuclei and fiber tracts, these distinct syndromes often result in overlapping
symptomatology. Disturbance of vertical eye movements from midbrain
lesion usually caused by damage to one or more of three main structure:
the posterior commisure, the rostal interstitial nucleus of the medial
longitudinal fasciculus (riMLF), and the interstitial nucleus of Cajal.

DORSAL MIDBRAIN SYNDROME


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Lesion of the posterior commisure cause a syndrome characterized


by loss of upward gaze and a number of other associated findings. This
condition is known by a variety of names: dorsal midbrain syndrome,
parinaunds syndrome, pretectal syndrome, and the sylvian aqueduct
syndrome. Unilateral midrain lesions can also create the same ocular
motor syndrome, but probably by interrupting the afferent and efferent
connections of the posterior commisure.8
Limited upgaze is the distinguishing symptom, as the vertical gaze
center lies in close vicinity to the superior colliculus, with some of the main
nuclei being the interstitial nucleus of Cajal and rostal interstitial nucleus of
MLF. Interestingly, downward gaze is often preserved. This is in distinction
to progressive supranuclear palsy, which also presents with a vertical
gaze palsy, but one which preferentially affects downward gaze. The
reasons for this difference are not entirely clear, but it has been suggested
that the pathway for downward gaze are directly medially out of the rostal
interstitial nucleus of the MLF, while those the upward gaze are directly
laterally and decussate in the posterior commisure, making them more
susceptible to external mass effect. With dorsal midbrain syndrome,
patients may have downgaze at rest, known as Setting Sun sign. 5
The dorsal midbrain syndrome is also characterized by disturbance
of horizontal eye movements, especially vergence. In some patients,
convergence is paralyzed, whereas in others it is excessive, resulting in
convergence spasm. During horizontal saccades, the abducting eye may
move more slowly than its adducting fellow eye. This finding called pseudo
abducens palsy and may reflect an excess of convergence tone.5,8

Table1. Features of the dorsal midbrain syndrome


Limitaion of upward eye movements:
1.

Saccades
Smooth pursuit
Vestibulo-ocular reflex

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Bells phenomenon
Disturbances of downward eye movements:
Downward gaze preference (setting sun sign)
2.

Downbeating nystagmus
Downward saccades and smooth pursuit may be
impaired, but vestibular movements are relatively
preserved
Disturbances of vergence eye movements:
Convergence-retraction nystagmus (Koeber-SalusElschnig syndrome)

3.

Paralysis of convergence
Spasm of convergence
Paeralysis if divergence
A or V pattern exotropia
Pseudo-abducens palsy

4.

Lid retraction (Colliers sign); occasionally ptosis

5.

Pupillary abnormalities (Light-near dissociation)

6.

Fixation instability (square-wave jerks)

7.

Skew deviation

Source: Walsh and Hoyts. Clinical Neuro-Ophthalmology: The Essentials.8

Convergence retraction nystagmus may occur in patients with


disease of midbrain. This disorder presumably results from damage to the
posterior commisure, because it can be produce by experimental lesion
restricted to this structure.8
Eye abnormalities occur in patients with dorsal midbrain lesions. The
most common is eyelid retraction (Colliers tucked lid sign), but ptosis may
occasionally occur.8
The impulses from the optic tract synapse in the pretectal area and
then travel via the posterior commisure to both Edinger-Westphal nuclei in
the posterior commisure. Patients with lesion of the midbrain in the region
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of posterior commisure has pupillary size and reactivity are commonly


abnormal. The pupils are usually large and react better to an
accommodative stimulus than to light (light-near dissociation). 8

Table 2. Etiology of disorders vertical gaze


Tumor
1.

Classically, pineal germioma or teratoma in an adolescent male;


also pineocytoma, glioma, metastasis
Hydrocephalus

2.

Usually aqueductal stenosis leading to dilation of the 3rd ventricle


and aqueduct or enlargement of the suprapineal recess with
pressure on the posterior commisure
Vascular

3.

Mid brain or thalamic haemorrhage or infarction; subdural


hematoma
Metabolic

4.

Lipid storage disease: Niemann-pick variants, Gauchers disease,


Tay-Sachs disease, Wilsons disease, kernicterus

5.

Drug-induced
Barbiturates, carbamazepine, neuroleptic agents
Degenerative

6.

Progressive supranuclear palsy, Huntingtons disease, diffuse


Lewy body disease, miscellaneous degenerations
Miscellaneous

7.

Multiple sclerosis, Whopples disease, hypoxia, encephalitis,


syphilis, aneurysm, neurosurgical procedure, tuberculoma, trauma

Source: Walsh and Hoyts. Clinical Neuro-Ophthalmology: The Essentials.8

A variety of disease processes can affect the region of the posterior


commisure. Pine tumors produce the dorsal midbrain syndrome either by
direct pressure on the posterior commisure or by causing obstructive
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hydrocephalus. Hydrocephalus may produce this syndrome by enlarging


the aqueduct and 3rd ventricle or suprapineal recess, thus stretching or
compressing the posterior commisure.8
CASE REPORT
A 70 years old man came to Neuroophthalmology unit in Cicendo
Eye Hospital with blurred vision on both eyes. Blurred became worse in
one year, accompanied with headache without any nausea or vomiting.
Patient has a history of hypertension, but there are no diabetes mellitus
and never have been examined for dyslipidemia. No history of ocular
movement pain, redness eye, trauma, and surgery.
General examination revealed hypertension grade II. Ocular
examination showed best corrected visual acuity on the right eye was 0.1
and on the left eye was 0.08. The intraocular pressure was within normal
limit.

Extraocular

examination

motility

examination

of

both

eyes

demonstrated restricted with convergence on up gaze movement. In


primary position showed setting sun sign with eyelid retraction. The
slitlamp biomicroscopy was within normal limit in both eye, except
decrease of pupillary reaction to light and hazy lens. Direct funduscopy on
the right and left eye was within normal limit.

Figure 4. Setting Sun: sign in pimary position with retraction of


palpebra superior. Upgaze limitation of upgaze movement
on both eye.
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Source:.Neuroophthalmologi Unit National Eye Center Cicendo Eye


Hospital

Patients was able to read all of ischihara color plates with each eye.
Contrast examination was 1,25% for both eyes. Amsler grid testing
revealed no scotoma or metamorphopsia on both eyes.
This patient showed evidence of dorsal midbrain syndrome. The
ocular examination showed vertical gaze palsy with limitation and
convergence of upward eye movement, Setting Sun sign at rest, Lid
retraction, and light-near dissociation.
The clinical diagnosis for this patient was dorsal midbrain syndrome,
bilateral immature senile cataract, and hypertension. The patient was
planned to do MRI examination and consult to internal department for the
underlying disease.

CONCLUSION
Patients with eye movement disorder should undergo proper work up
in order to search for the underlyying mechanism. An understanding the
functions and relationships between these different midbrain structures
allows better correlation between regions of pathologic involvement and
patient symptomatology.

REFERENCE
1.

Afshinmajd S, Ghasemi H. Clinical Evaluation, Prevalence and


Etiologic Factors in Patients with Ophthalmoplegia. Iranian Journal of
Ophthalmology. Iran: 2011; p.48-54.

2.

Hoyt WF. Clinical neuro-ophthalmology-a practical guide. Berlin:


Springer Verlag Heidelberg; 2007. p.155-160

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3.

Leigh RJ, Zee DS. The Neurology of Eye Movements. Fourth edition.
Chapter 1: A Survey of Eye Movements: Characteristics and
Teleology. Oxford; 2006. p. 3-19

4.

Leigh RJ, Zee DS. The Neurology of Eye Movements. Fourth edition.
Chapter 12: Diagnosis of Central Disorders of Ocular Motility. Oxford;
2006. p. 599-718

5.

Ruchalski K, Mathout GM. A Medley of Midbrain Maladies: A Brief


Review of Midbrain Anatomy and Syndromology for Radiologists.
Hindawi Publishing Cooperation Radiologi Research and Practice.
2012.

6.

Afifi AK, Bergman RA. Functional Neuroanatomy Text and Atlas.


McGraw-Hill Companies. 1998

7.

Leigh RJ, Zee DS. The Neurology of Eye Movements. Fourth edition.
Chapter 6: Synthesis of the Command for Conjugate Eye
Movements. Oxford; 2006. p. 261-314

8.

Hoyt WF. Clinical neuro-ophthalmology: The Essentials. Second


Edition. Lippincott Williams & Wilkins. 2008. p.344-376

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