Fourth Cranial Nerve (Trochlear Nerve) Palsy

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Fourth cranial nerve (trochlear nerve) palsy

Author: Andrew G Lee, MD
Section Editor: Paul W Brazis, MD
Deputy Editor: Janet L Wilterdink, MD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Jun 2021. | This topic last updated: Oct 01, 2019.
INTRODUCTION
Paralytic strabismus refers to nonalignment of the eyes and is caused by weakness or paralysis of
one or more of the extraocular muscles. Dysfunction of the fourth cranial nerve (trochlear nerve),
which innervates the superior oblique muscle, is one cause of paralytic strabismus and can result
from lesions anywhere along its path between the fourth nerve nucleus in the midbrain and the
superior oblique muscle within the orbit. These lesions can be congenital or acquired.
The manifestations and diagnosis of fourth nerve palsy, also known as superior oblique paralysis and
trochlear nerve palsy, are reviewed here. Palsies of the third and sixth cranial nerves are discussed
separately. (See "Third cranial nerve (oculomotor nerve) palsy in children" and "Sixth cranial nerve
(abducens nerve) palsy".)
ANATOMY
The fourth cranial nerve (trochlear nerve) has the longest intracranial course; it is the only cranial
nerve that has a dorsal exit from the brainstem ( figure 1). It begins in the midbrain at the level of
the inferior colliculus as fascicles extending from the fourth nerve nuclei. The trochlear nerves cross
after they exit dorsally in the anterior medullary velum. The fourth nerve passes between the superior
cerebellar artery and the posterior cerebral artery and runs in the subarachnoid space. It travels
within the lateral wall of the cavernous sinus and enters the orbit via the superior orbital fissure to
innervate the superior oblique muscle [1-10].
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The fourth cranial nerve is a purely efferent or pure motor nerve and it innervates a single muscle, the
superior oblique. The primary action of the superior oblique muscle is intorsion of the eye in the
primary position; the secondary action is depression of the eye in the adducted position; a tertiary
action of the muscle is abduction (especially in the abducted position) ( table 1).
The long course of the fourth cranial nerve renders it particularly prone to injury from blunt head
trauma or compression from changes in intracranial pressure, brain tumors, or swelling anywhere
along its course.
CLINICAL MANIFESTATIONS
Patients may present with fourth cranial nerve palsy at any age and in either gender. Many case
series report a male predominance, presumably because of the greater incidence of trauma in young
males [11,12].
Fourth cranial nerve palsy — Individuals with fourth nerve palsies may complain of binocular (ie,
present with both eyes open) vertical diplopia and/or subjective tilting of objects (torsional diplopia).
Because the superior oblique also depresses the eye in the adducted position, objects viewed in
primary position, and especially in down-gaze, may appear double (eg, when going down a flight of
stairs). More mildly affected individuals may complain of blurred vision, difficulty focusing, and
dizziness rather than overt diplopia.
The patient may adopt a conscious or unconscious head tilt. This is because torsional and vertical
diplopia often improve with head tilting to the side opposite the paralyzed muscle ( picture 1). As an
example, vertical and torsional diplopia in a patient with left fourth nerve palsy improve with the head
tilted toward the right. Old photographs may show the long-standing compensatory contralateral head
tilt in congenital or long-standing palsies. Additionally, some patients may subconsciously close one
eye when reading.
On examination, an ipsilateral hypertropia (deviation upward) and excyclotorsion (rotation outward) of

the involved eye is present because the superior oblique muscle intorts and depresses the eye. The
upward deviation is greater when gaze is in the direction of action of the weak muscle (downgaze and
contralateral horizontal gaze). The deviation is also greater in ipsilateral head tilt. Thus, a left fourth
nerve palsy causes a left hypertropia, which is greater in right gaze and with the head tilted to the left.
These characteristics form the basis for the diagnosis. (See 'Unilateral fourth nerve palsy' below.)
Patients with only a small angle deviation from a unilateral superior oblique palsy can present with
little or no primary position hypertropia and only head tilt test and gaze tests upon examination (ie, the
three-step test). In this setting, it is important to consider the possibility of masked bilateral superior
oblique palsies (the primary position deviation is obscured because the bilateral fourth nerve palsies
cancel each other out). These examinations are described in detail below. (See 'Clinical evaluation
and diagnosis' below.)
The direction and degree of subjective torsion can be measured objectively with a double Maddox
rod. The affected eye is usually extorted because the superior oblique muscle is an intorter of the
eye. (See 'Measurement of torsion' below.)
Associated clinical features — Fourth nerve palsies may be isolated or accompanied by other

contributory neurologic abnormalities. Nonisolated palsies may be divided into four syndromes based
upon the topographic anatomy of the fourth nerve ( table 2) [13]:
● A midbrain nuclear or fascicular lesion (eg, tumor, stroke, demyelination) is typically

accompanied by other brainstem symptoms, such as hemisensory loss, hemiparesis, a central
Horner syndrome, or other brainstem cranial neuropathies.
● Lesions of the subarachnoid space (eg, meningitis) are typically associated with other signs and
symptoms including headache, stiff neck, and other cranial nerve abnormalities. Focal lesions
(aneurysms, schwannomas) may produce isolated fourth nerve palsies.
● Cavernous sinus lesions are usually associated with other localizing signs (eg, third, fifth, or sixth
nerve dysfunction, or Horner syndrome).
● Orbital apex lesions often involve third, fifth, and sixth cranial nerves as well as the optic nerve.
Orbital involvement may also produce such signs as proptosis, chemosis, and orbital or
conjunctival edema.
The fourth nerve exits from the dorsum of the brainstem. Thus, neurologic signs of cerebellar disease
may be present if a tumor in that region is causing the fourth nerve palsy.
CLINICAL EVALUATION AND DIAGNOSIS
Fourth cranial nerve palsy should be suspected in patients with binocular vertical diplopia and/or
subjective tilting of objects (torsional diplopia). The diagnosis can be confirmed using a three-step
clinical evaluation as described below.
Unilateral fourth nerve palsy — During gross ductional testing in various directions of gaze, the
ocular movements can appear to be full despite weakness of the superior oblique muscle. In some
cases, however, the affected eye has reduced infraduction with adduction. The Parks-Bielschowsky
three-step test is performed to make the diagnosis of fourth nerve palsy ( picture 1 and
algorithm 1) [14,15]:
● Step 1 – Which is the higher (hypertropic) eye ( picture 1)? (See panel B.)
The determination of the more hypertropic eye narrows the paretic possibilities to four
extraocular muscles (the ipsilateral superior oblique or inferior rectus or the contralateral inferior
oblique or superior rectus) as in panel B ( picture 1). As an example, if the left eye is higher (a
left hypertropia), the muscles that are potentially involved include the depressors of the left eye,
the left superior oblique, and the left inferior rectus; or the elevators of the right eye, the right
inferior oblique, and the right superior rectus.
● Step 2 – Is the hypertropia worse in right or left gaze?
The determination of which horizontal gaze worsens the hypertropia narrows the possible
muscles involved from four to two because only two muscles act in right gaze and two in left
gaze. The hypertropia is worse in contralateral gaze because that movement is controlled by the
paretic muscle, as in panel C ( picture 1). Thus, worsening of the hypertropia in right gaze in a
patient with left hypertropia implicates either the left superior oblique or the right superior rectus.
● Step 3 – Is the hypertropia worse in right or left head tilt?
The determination of which head tilt worsens the hypertropia (Bielschowsky head tilt test)
identifies the involved muscle, as in panel D ( picture 1). Hyperdeviation is worse in ipsilateral
head tilt in a fourth nerve palsy because the intorsion ability of the ipsilateral superior oblique is
weak and is compensated for by the other ipsilateral intorter (the superior rectus). Activation of
the superior rectus causes elevation of the eye and increases the hypertropia. The deviation
improves in contralateral head tilt, the position that typically is adopted by the patient to reduce
diplopia. Thus, left hypertropia that worsens with right gaze and left head tilt is consistent with a
left superior oblique (or fourth nerve) palsy.
Inconsistencies in these findings suggest that there is not an isolated fourth nerve palsy and that
other diagnoses should be considered ( algorithm 1). (See 'Differential diagnosis' below and
"Overview of diplopia".)
The best time to diagnose a fourth nerve palsy is early in its course. If the palsy does not resolve,
there is a tendency for the deviation to become more "comitant." This means the hypertropia that was
once limited largely to one field of gaze will creep into the opposite field due to the contraction, and
thus overaction, of the antagonistic muscle [16].
Some authors advocate for a "fourth step" to ensure that the typical excyclotorsion of a weak superior
oblique muscle is present on the affected side using double Maddox rod or fundus photography
(demonstrating the ipsilateral excyclotorsion). The upright-supine test is an alternative proposed
fourth step to differentiate skew deviation from other causes of vertical strabismus. (See 'Ocular tilt
reaction and skew deviation' below.)
The exam can be limited in noncooperative patients. Therefore, if head tilt is present and the three-
step test is unable to be performed, some ophthalmologists patch one eye and look for resolution of
tilt [17].
Bilateral fourth nerve palsies — Bilateral fourth nerve palsies are suggested by the following
features ( picture 2):
● Alternating hypertropia on horizontal gaze or head tilt (eg, right hypertropia in left gaze and left
hypertropia in right gaze).
● Positive head tilt test to either shoulder (double Bielschowsky head tilt test).
● Large degree of excyclotorsion (>10 degrees).
● V-pattern esotropia (ie, a difference of 15 prism diopters or more in esotropia between upward
and downward gaze) caused by a decrease in the abducting effect of the superior oblique(s) in
depression and overaction of the inferior oblique muscle(s).
● Underaction of both superior oblique muscles and/or overaction of both inferior oblique muscles.
● A relatively small hypertropia in primary position (the two eyes have a vertical deviation and tend
to cancel each other out).
These clinical features should be used in combination to diagnose bilateral fourth palsy. Individual
tests (eg, alternating hypertropia, head tilt) are insufficiently sensitive to be used in isolation [18].
Measurement of torsion — Torsion is measured objectively with a double Maddox rod. It is

important to measure torsion because significant torsion (>5 to 8 degrees) may require torsion
surgery (eg, the Harada-Ito procedure). In addition, the torsion may be a limiting factor for fusion even
if the vertical and horizontal deviations are corrected surgically or with prism.
DIFFERENTIAL DIAGNOSIS
Other conditions can present with similar findings and should be considered, particularly when the
patient does not meet criteria for fourth nerve palsy. (See 'Unilateral fourth nerve palsy' above.)
Restrictive vertical strabismus — Restrictive vertical strabismus can be produced by orbital floor

fracture, orbital tumors, orbital pseudotumor, and Graves ophthalmopathy. Patients with these
conditions usually have additional orbital signs, such as proptosis, chemosis, and conjunctival
injection. Forced ductions (passive rotation of the eyes with ophthalmic forceps) typically confirm the
restrictive nature of the ophthalmoplegia. (See "Clinical features and diagnosis of Graves' orbitopathy
(ophthalmopathy)".)
Other paretic vertical strabismus — Paretic vertical strabismus can be produced by a partial third
nerve palsy that affects the inferior rectus, superior rectus, or inferior oblique. Other features of third
nerve palsy (eg, ptosis, deficits in adduction, elevation, and/or depression) demonstrated on
examination help to differentiate third from fourth nerve palsy. (See "Third cranial nerve (oculomotor
nerve) palsy in children" and "Third cranial nerve (oculomotor nerve) palsy in adults".)
Myasthenia gravis — Myasthenia gravis can mimic any painless, pupil-spared, nonproptotic

ophthalmoplegia [19]. Other signs of myasthenia gravis, such as ptosis, variability, and fatigue, are
usually present. (See "Ocular myasthenia gravis", section on 'Clinical features'.)
Ocular tilt reaction and skew deviation — Ocular tilt reaction and skew deviation are vertical
misalignments caused by disruption of vestibulo-ocular connections. Similar to fourth nerve palsy,
skew deviation presents with vertical misalignment, cyclotorsion, and head tilt [20,21]. In skew
deviation, the pattern of vertical misalignment does not follow a set pattern with gaze direction or
head tilt, but may mimic a fourth nerve palsy. However, as measured by double Maddox rod, the
laterality and direction of torsion in skew deviation (bilateral rather than unilateral torsion or
incyclotorsion rather than excyclotorsion) are often inconsistent with superior oblique paralysis. In the
uncooperative patient, a photograph of the fundus can be taken to demonstrate the torsion; in skew
deviation, the fundus of the hypertropic eye is typically incyclotorted, while in fourth nerve palsy the
fundus is excyclotorted.
In addition, ocular torsion and vertical misalignment are position dependent in skew deviation
(decreasing with position change from upright to supine), whereas they are relatively unaffected by
position in fourth nerve palsy [22,23]. A positive upright-supine test (vertical strabismus decreases by
≥50 percent with position change from upright to supine) suggests skew deviation with high specificity
in one series [23].
Finally, skew deviation typically is accompanied by other posterior fossa signs (eg, other cranial
neuropathies, hemisensory loss, or hemiparesis) and more widespread brainstem disease. (See
"Supranuclear disorders of gaze in children", section on 'Skew deviation'.)
Thyroid ophthalmopathy — Thyroid ophthalmopathy is more commonly seen in adults. (See

"Clinical features and diagnosis of Graves' orbitopathy (ophthalmopathy)".)
ETIOLOGIC EVALUATION
Causes of isolated fourth nerve palsy — Most isolated fourth nerve palsies are congenital, even
those presenting in adulthood. The most common causes of acquired lesions are trauma and
microvascular disease [11,24-26].
Congenital — In most series, the most common causes of fourth nerve palsies in both children
and adults are congenital (33 to 50 percent) [11,24-28]. Differentiating acquired fourth nerve palsy
from decompensation of a congenital fourth nerve palsy is important because patients with isolated
congenital palsies do not typically require further neurologic evaluation or imaging.
Patients with congenital fourth nerve palsy are often able to compensate for the double vision with a
head tilt that minimizes symptoms ( picture 1). Over time, many patients with congenital fourth
nerve palsy have increasing difficulty controlling the symptoms and may present with diplopia. They
may insist that this is a new problem, although old photos frequently demonstrate a long-standing
head tilt [14].
A peculiar feature of congenital fourth nerve palsy is that the amount of hypertropia in the primary
position is very large (deviations as high as 20 or 30 prism diopters). A true acquired fourth nerve
palsy of recent onset will have a hypertropia that measures prism diopters in the mid-teens in the
primary position at the most [29].
The following additional features may be present in patients with congenital fourth nerve palsies:
● Lack of complaints of cyclotropia (subjective image tilting), despite objective fundus

excyclotorsion.
● Ability to fuse relatively large vertical deviation of the eyes (ie, large vertical fusional amplitudes
measuring greater than 6 to 8 prism diopters). In some cases of congenital fourth nerve palsy,
the amount of hypertropia may be as high as 20 or 30 prism diopters.
● Facial asymmetry (hypoplasia of the face on the side of head turn) in long-standing or congenital
palsies.
The amount of hypertropia may not match the degree of head tilt in fourth nerve palsy for a number of
reasons:
● The head tilt generally compensates for the torsion, but not necessarily the vertical deviation, and
some patients may even tilt in the opposite direction to better suppress or ignore the second
image.
● Variable amounts of sensory cyclofusion exist in patients, which can obscure or negate the
torsion that is seen objectively.
● Some patients tilt their heads to create disparity in the image rather than relieve the deviation
(and thus can even be tilting their head in the wrong direction).
While familial fourth nerve palsy is rare, congenital fourth nerve palsy has been reported in multiple
families [30,31]. The pedigree analysis of five affected members in one family indicated an autosomal
dominant inheritance pattern [31].
While magnetic resonance imaging (MRI) is not required to evaluate patients with congenital fourth
nerve palsy, corroborative findings may include absent or atrophic superior oblique muscle;
comparison with the unaffected side may be required as the findings may be subtle [32].
In one series of isolated fourth nerve palsy, the degree and direction of horizontal eye deviation
differed between congenital and microvascular causes; in congenital lesions, exotropia (outward
deviation) was most common, occurring in 86 percent, while in microvascular lesions, esotropia and
horizontal orthophoria occurred in 29 and 34 percent, respectively [27]. The clinical utility of this
observation is uncertain.
Traumatic — Traumatic fourth nerve palsies, in contrast to traumatic third and sixth nerve palsies,
may occur with a relatively mild blow to the head (ie, not associated with loss of consciousness or a
skull fracture) [33,34].
Bilateral involvement occurs in a significant minority of patients with traumatic fourth nerve palsy. In
one series of 270 patients with superior oblique paralysis, one-fourth of cases caused by trauma had
bilateral involvement [24]. Careful examination for bilateral fourth nerve palsies is necessary; the
palsies may be asymmetric. It is unfortunate for a surgeon to have corrected a fourth nerve palsy by
weakening the ipsilateral inferior oblique muscle, only to find a contralateral fourth nerve palsy now
uncovered and obvious to the patient [26,35,36]. (See 'Bilateral fourth nerve palsies' above.)
In most cases, MRI is unremarkable, implicating an injury to the nerve itself; however, in some
patients, a dorsal midbrain contusion or hemorrhage is documented [37].
Some traumatic fourth nerve palsies improve with time, but most do not.
Microvascular disease — While more commonly affecting the third and sixth cranial nerves,
microvascular disease can involve the fourth cranial nerve as well [27,38-41]. In one series,
microvascular disease related to hypertension and/or diabetes was believed to underlie 24 percent of
cases [11].
Such patients are typically older with microvascular risk factors (hypertension, diabetes) and usually
present with an abrupt onset of symptoms [39,42]. MRI may show an infarction in the tegmentum of
the midbrain, affecting the fascicle of the fourth nerve. While microvascular infarction of the fourth
nerve presumably also occurs, these are not demonstrable on MRI and the diagnosis is presumptive
rather than confirmed.
Idiopathic — A minority of patients with trochlear nerve palsy have no etiologic diagnosis after
evaluation (3 of 75 patients or 4 percent in one of the largest more recent series) [11]. Idiopathic
fourth nerve palsies, like idiopathic third and sixth nerve palsies, often present with mild discomfort or
pain over the eyebrow of the affected eye that stops shortly after the diplopia starts [2,3,24,43,44].
When asked to point to the pain, patients usually indicate an area just above the brow on the side of
the muscle weakness. Rarely does the pain require medication.
The idiopathic cases may resolve in a few weeks, and some authors defer additional neuroradiologic
testing until two months have passed without improvement as long as the other cranial nerves are
normal [5,45].
Other — Less common causes of isolated fourth nerve palsies include tumors of the brainstem or
along the course of the fourth nerve (eg, schwannoma [46-50], cavernous hemangioma [40],
metastasis [51]), intracranial or meningeal infections [52-54], zoster ophthalmicus [11,12,16], pituitary
macroadenoma [55,56], intracavernous or superior cerebellar artery aneurysm [57,58], and
perimesencephalic subarachnoid hemorrhage [59].
Rarely, a fourth nerve palsy can be due to raised intracranial pressure [60,61]. It is important to look
carefully at the optic nerve head to be sure that there is no papilledema.
Causes of nonisolated fourth nerve palsy — These are categorized according the anatomic
location of involvement as listed in the table ( table 2) [13].
Radiologic evaluation — Individuals who have nonisolated fourth nerve palsies with neurologic
signs or symptoms that localize to the brainstem, subarachnoid space, cavernous sinus, or orbit
should undergo neuroimaging to best evaluate the localized area ( table 2). Patients with a
neurologically isolated fourth nerve palsy that seems clearly congenital, traumatic, or ischemic do not
require neuroimaging; however, corroborative findings on neuroimaging are sometimes seen for
these conditions. (See 'Congenital' above and 'Traumatic' above and 'Microvascular disease' above.)
MRI is superior to computed tomography (CT) scan for the evaluation of a nonisolated fourth nerve
palsy. MRI is more sensitive in the detection of intracranial lesions in the posterior fossa and is not
susceptible to the bone artifacts that are seen with CT scanning. CT scanning should be used in
patients who require evaluation of bone or acute bleeding, or who cannot undergo an MRI.
In addition to detecting causative lesions in the midbrain, cavernous sinus, and orbit, MRI may
demonstrate enhancing nerve lesions in the subarachnoid space that may represent schwannoma or
focal inflammation [48,49].
Additional evaluation — Lumbar puncture may be warranted in patients who have normal imaging
studies but are suspected of having a meningitic (subarachnoid space) process. (See 'Associated
clinical features' above.)
Evaluation for mimics (eg, myasthenia gravis, thyroid eye disease, skew deviation) may be warranted
depending on the clinical presentation, but typically, an isolated fourth nerve palsy is a relatively
straightforward clinical diagnosis. (See 'Differential diagnosis' above.)
TREATMENT AND PROGNOSIS
Treatment of the underlying etiology in patients with acquired nontraumatic fourth nerve palsy (eg,
brain tumor, increased intracranial pressure) may alleviate the diplopia.
Symptomatic patients with isolated palsies that are congenital or traumatic should be considered for
treatment. The goal of treatment is to maximize visual function, including ocular alignment.
Microvascular lesions are likely to improve and resolve over weeks to months [12,27]. Patients with
traumatic fourth nerve palsy, depending upon the severity of the injury to the nerve, may improve over
time and often are observed for several months before being considered for treatment. If the palsy
does not resolve, the tendency is for it to become comitant, making it suitable for prism therapy.
Comitant deviations are those in which the distance between the double images is equal in the
various gaze directions and symptoms are only slightly affected by the direction of gaze.
Prism therapy may be employed for small, comitant, long-standing deviations [28]. A temporary
press-on (Fresnel) prism of sufficient power to align the eyes is placed on the spectacle lens.
Permanent prisms can be ground into the spectacle lens if the patient is happy with the result and the
deviation fails to improve spontaneously. Strabismus surgery may be helpful in patients who fail prism
therapy.
Patching one eye will alleviate binocular diplopia and is useful for patients who are being observed or
who defer prism or surgical therapy. Patching should be employed with caution and with the
assistance of an ophthalmology consultant in children in the amblyopic age range. (See "Amblyopia in
children: Classification, screening, and evaluation", section on 'Definition'.)
Additional therapies may alleviate the symptoms of traumatic fourth nerve palsy while it recovers its
function (ie, botulinum toxin injection in the inferior oblique muscle) [62]. Galantamine has been
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proposed in traumatic trochlear nerve palsy, but anecdotal reports of efficacy require independent
confirmation [63].
SUMMARY AND RECOMMENDATIONS
● The fourth cranial nerve (trochlear nerve) supplies the superior oblique muscle. The actions of
the superior oblique muscle include intorsion, depression, and abduction ( table 1). (See
'Anatomy' above.)
● Clinical manifestations of fourth nerve palsy include vertical diplopia, torsional diplopia, extorsion,
head tilt (to avoid diplopia), and ipsilateral hypertropia (elevation of the affected eye). (See
'Clinical manifestations' above.)
● Fourth nerve palsies can be classified as isolated or nonisolated (ie, accompanied by other
neurologic abnormalities). Nonisolated palsies produce symptoms according to the topographic
anatomy of the fourth nerve ( table 2). (See 'Associated clinical features' above.)
● Unilateral fourth nerve palsy is diagnosed using the three-step test ( picture 1 and
algorithm 1). (See 'Unilateral fourth nerve palsy' above.)
● Bilateral fourth nerve palsies are suggested by alternating hypertropia on horizontal gaze,
positive head tilt test to either shoulder, excyclotorsion >10 degrees, V-pattern esotropia,
underaction of both superior oblique muscles and/or overaction of both inferior oblique muscles,
and a relatively small hypertropia in primary position. (See 'Bilateral fourth nerve palsies' above.)
● The differential diagnosis of fourth nerve palsy includes childhood strabismus, myasthenia gravis,
ocular tilt reaction and skew deviation, and thyroid ophthalmopathy. These can usually be
distinguished on careful examination as described. (See 'Differential diagnosis' above.)
● The most common causes of fourth nerve palsies are congenital, traumatic, and microvascular.
(See 'Causes of isolated fourth nerve palsy' above.)
● Patients with isolated fourth nerve palsy with a clearly defined etiology that is congenital,
traumatic, or microvascular do not require further imaging, although corroborative findings may
be identified in such cases.
Individuals with nonisolated fourth nerve palsies (neurologic signs or symptoms that localize to
the brainstem, subarachnoid space, cavernous sinus, or orbit) should undergo brain imaging,
usually with contrast-enhanced magnetic resonance imaging (MRI) to best evaluate the localized
area ( table 2). (See 'Radiologic evaluation' above.)
● Lumbar puncture may be warranted in patients who have normal imaging studies but are
suspected of having subarachnoid space lesions. (See 'Additional evaluation' above.)
● Treatment for fourth nerve palsy is directed at the underlying etiology. The goal of other therapies
is to maximize visual function, including ocular alignment. (See 'Treatment and prognosis'
above.)
ACKNOWLEDGMENT
The editorial staff at UpToDate would like to acknowledge Claudia Prospero Ponce, MD, and Aroucha
Vickers, MD, who contributed to an earlier version of this topic review.
Use of UpToDate is subject to the Subscription and License Agreement.
REFERENCES
1. Berlit P. Isolated and combined pareses of cranial nerves III, IV and VI. A retrospective study of
412 patients. J Neurol Sci 1991; 103:10.
2. Burger LJ, Kalvin NH, Smith JL. Acquired lesions of the fourth cranial nerve. Brain 1970; 93:567.
3. Coppeto JM, Lessell S. Cryptogenic unilateral paralysis of the superior oblique muscle. Arch
Ophthalmol 1978; 96:275.
4. Harley RD. Paralytic strabismus in children. Etiologic incidence and management of the third,
fourth, and sixth nerve palsies. Ophthalmology 1980; 87:24.
5. Keane JR. Fourth nerve palsy: historical review and study of 215 inpatients. Neurology 1993;
43:2439.
6. Khawam E, Scott AB, Jampolsky A. Acquired superior oblique palsy. Diagnosis and
management. Arch Ophthalmol 1967; 77:761.
7. Richards BW, Jones FR Jr, Younge BR. Causes and prognosis in 4,278 cases of paralysis of the
oculomotor, trochlear, and abducens cranial nerves. Am J Ophthalmol 1992; 113:489.
8. RUCKER CW. Paralysis of the third, fourth and sixth cranial nerves. Am J Ophthalmol 1958;
46:787.
9. Rucker CW. The causes of paralysis of the third, fourth and sixth cranial nerves. Am J
10. Rush JA, Younge BR. Paralysis of cranial nerves III, IV, and VI. Cause and prognosis in 1,000
cases. Arch Ophthalmol 1981; 99:76.
11. Dosunmu EO, Hatt SR, Leske DA, et al. Incidence and Etiology of Presumed Fourth Cranial
Nerve Palsy: A Population-based Study. Am J Ophthalmol 2018; 185:110.
12. Khaier A, Dawson E, Lee J. Clinical course and characteristics of acute presentation of fourth
nerve paresis. J Pediatr Ophthalmol Strabismus 2012; 49:366.
13. Gold DR, Shin RK, Galetta S. Pearls and oy-sters: central fourth nerve palsies. Neurology 2012;
79:e193.
14. Brazis PW. Palsies of the trochlear nerve: diagnosis and localization--recent concepts. Mayo Clin
Proc 1993; 68:501.
15. Bixenman WW. Diagnosis of superior oblique palsy. J Clin Neuroophthalmol 1981; 1:199.
16. Ellis FD, Helveston EM. Superior oblique palsy: diagnosis and classification. Int Ophthalmol Clin
1976; 16:127.
17. Koller HP, Olitsky SE, O'Hara M, Nelson LB. Diagnosis and Treatment of Fourth Nerve Palsy. J
Pediatr Ophthalmol Strabismus 2016; 53:70.
18. Muthusamy B, Irsch K, Peggy Chang HY, Guyton DL. The sensitivity of the bielschowsky head-
tilt test in diagnosing acquired bilateral superior oblique paresis. Am J Ophthalmol 2014;
157:901.
19. Rush JA, Shafrin F. Ocular myasthenia presenting as superior oblique weakness. J Clin
Neuroophthalmol 1982; 2:125.
20. Donahue SP, Lavin PJ, Hamed LM. Tonic ocular tilt reaction simulating a superior oblique palsy:
diagnostic confusion with the 3-step test. Arch Ophthalmol 1999; 117:347.
21. Ohashi T, Fukushima K, Chin S, et al. Ocular tilt reaction with vertical eye movement palsy
caused by localized unilateral midbrain lesion. J Neuroophthalmol 1998; 18:40.
22. Parulekar MV, Dai S, Buncic JR, Wong AM. Head position-dependent changes in ocular torsion
and vertical misalignment in skew deviation. Arch Ophthalmol 2008; 126:899.
23. Wong AM, Colpa L, Chandrakumar M. Ability of an upright-supine test to differentiate skew
deviation from other vertical strabismus causes. Arch Ophthalmol 2011; 129:1570.
24. von Noorden GK, Murray E, Wong SY. Superior oblique paralysis. A review of 270 cases. Arch
Ophthalmol 1986; 104:1771.
25. Holmes JM, Mutyala S, Maus TL, et al. Pediatric third, fourth, and sixth nerve palsies: a
population-based study. Am J Ophthalmol 1999; 127:388.
26. Helveston EM, Mora JS, Lipsky SN, et al. Surgical treatment of superior oblique palsy. Trans Am
Ophthalmol Soc 1996; 94:315.
27. Hata M, Miyamoto K, Nakagawa S, et al. Horizontal deviation as diagnostic and prognostic
values in isolated fourth nerve palsy. Br J Ophthalmol 2013; 97:180.
28. Tamhankar MA, Ying GS, Volpe NJ. Success of prisms in the management of diplopia due to
fourth nerve palsy. J Neuroophthalmol 2011; 31:206.
29. Mittelman D, Folk ER. The evaluation and treatment of superior oblique muscle palsy. Trans
Sect Ophthalmol Am Acad Ophthalmol Otolaryngol 1976; 81:893.
30. Astle WF, Rosenbaum AL. Familial congenital fourth cranial nerve palsy. Arch Ophthalmol 1985;
103:532.
31. Botelho PJ, Giangiacomo JG. Autosomal-dominant inheritance of congenital superior oblique
palsy. Ophthalmology 1996; 103:1508.
32. Kim JH, Hwang JM. Imaging of Cranial Nerves III, IV, VI in Congenital Cranial Dysinnervation
Disorders. Korean J Ophthalmol 2017; 31:183.
33. Sydnor CF, Seaber JH, Buckley EG. Traumatic superior oblique palsies. Ophthalmology 1982;
89:134.
34. Dhaliwal A, West AL, Trobe JD, Musch DC. Third, fourth, and sixth cranial nerve palsies
following closed head injury. J Neuroophthalmol 2006; 26:4.
35. Kushner BJ. The diagnosis and treatment of bilateral masked superior oblique palsy. Am J
36. Lee J, Flynn JT. Bilateral superior oblique palsies. Br J Ophthalmol 1985; 69:508.
37. Sudhakar P, Bapuraj JR. CT demonstration of dorsal midbrain hemorrhage in traumatic fourth
cranial nerve palsy. J Neuroophthalmol 2010; 30:59.
38. Evliyaoglu F, Karadag R, Burakgazi AZ. Ocular neuropathy in peripheral neuropathies. Muscle
Nerve 2012; 46:681.
39. Abkur TM. Trochlear nerve palsy. Pract Neurol 2017; 17:474.
40. Jeong SH, Kim SH, Lee SH, et al. Central Trochlear Palsy: Report of Two Patients With
Ipsilesional Palsy and Review of the Literature. J Neuroophthalmol 2016; 36:377.
41. Tamhankar MA, Biousse V, Ying GS, et al. Isolated third, fourth, and sixth cranial nerve palsies
from presumed microvascular versus other causes: a prospective study. Ophthalmology 2013;
120:2264.
42. Lee SH, Park SW, Kim BC, et al. Isolated trochlear palsy due to midbrain stroke. Clin Neurol
Neurosurg 2010; 112:68.
43. Younge BR, Sutula F. Analysis of trochlear nerve palsies. Diagnosis, etiology, and treatment.
Mayo Clin Proc 1977; 52:11.
44. Beard CM, Benson RC Jr, Kelalis PP, Elveback LR. Incidence of malignant testicular tumors in
the population of Rochester, Minnesota, 1935 through 1974. Mayo Clin Proc 1977; 52:8.
45. Bagolini B, Campos EC, Chiesi C. Plagiocephaly causing superior oblique deficiency and ocular
torticollis. A new clinical entity. Arch Ophthalmol 1982; 100:1093.
46. Jindal G, Gilbert A, Bhadelia R, Torun N. An unusual case of binocular oblique diplopia in an 82-
year-old man. Digit J Ophthalmol 2015; 21:1.
47. Torun N, Laviv Y, Jazi KK, et al. Schwannoma of the trochlear nerve-an illustrated case series
and a systematic review of management. Neurosurg Rev 2018; 41:699.
48. Elflein HM, Thömke F, Müller-Forell W, Pitz S. Trochlear palsies caused by isolated trochlear
schwannomas. Strabismus 2010; 18:83.
49. Elmalem VI, Younge BR, Biousse V, et al. Clinical course and prognosis of trochlear nerve
schwannomas. Ophthalmology 2009; 116:2011.
50. Younes WM, Hermann EJ, Krauss JK. Cisternal trochlear nerve schwannoma: improvement of
diplopia after subtotal tumour excision. Br J Neurosurg 2012; 26:107.
51. Mielke C, Alexander MS, Anand N. Isolated bilateral trochlear nerve palsy as the first clinical
sign of a metastatic [correction of metastasic] bronchial carcinoma. Am J Ophthalmol 2001;
132:593.
52. Müller D, Neubauer BA, Waltz S, Stephani U. Neuroborreliosis and isolated trochlear palsy. Eur
J Paediatr Neurol 1998; 2:275.
53. Carter N, Miller NR. Fourth nerve palsy caused by Ehrlichia chaffeensis. J Neuroophthalmol
1997; 17:47.
54. García-Zamora M, Sánchez-Tocino H, Villanueva-Gómez A, et al. Isolated Fourth Nerve Palsy in
Tuberculous Meningitis. Neuroophthalmology 2016; 40:40.
55. Petermann SH, Newman NJ. Pituitary macroadenoma manifesting as an isolated fourth nerve
palsy. Am J Ophthalmol 1999; 127:235.
56. Muthukrishnan J, Bharadwaj K, Singh Y. Isolated fourth cranial nerve palsy due to pituitary
macroadenoma. Med J Armed Forces India 2016; 72:S67.
57. Agostinis C, Caverni L, Moschini L, et al. Paralysis of fourth cranial nerve due to superior-
cerebellar artery aneurysm. Neurology 1992; 42:457.
58. Maurice-Williams RS, Harvey PK. Isolated palsy of the fourth cranial nerve caused by an
intracavernous aneurysm. J Neurol Neurosurg Psychiatry 1989; 52:679.
59. Adachi K, Hironaka K, Suzuki H, Oharazawa H. Isolated trochlear nerve palsy with
perimesencephalic subarachnoid haemorrhage. BMJ Case Rep 2012; 2012.
60. Halpern JI, Gordon WH Jr. Trochlear nerve palsy as a false localizing sign. Ann Ophthalmol
1981; 13:53.
61. Mantopoulos D, Hunter DG, Cestari DM. Isolated bilateral fourth cranial nerve palsies as the
presenting sign of hydrocephalus. Case Rep Ophthalmol 2011; 2:211.
62. Talebnejad MR, Tahamtan M, Nowroozzadeh MH. Botulinum Toxin Injection for Treatment of
Acute Traumatic Superior Oblique Muscle Palsy. J Ophthalmic Vis Res 2015; 10:263.
63. Tokarz-Sawińska E, Lachowicz E, Gosławski W. The use of galantamine in the treatment of post-
traumatic oculomotor and trochlear nerve palsy. Klin Oczna 2013; 115:275.
Topic 6268 Version 16.0
GRAPHICS
Origin and distribution of the trochlear nerve
Anatomic schematic showing the course of the fourth cranial nerve.

Graphic 117252 Version 1.0
Actions of extraocular muscles

Nerve and musclePrimary action Secondary action Tertiary action
Cranial nerve III
Superior rectus Elevation (maximal on lateral gaze) Intorsion Adduction
Inferior rectus Depression (maximal on lateral gaze)Extorsion Adduction
Medial rectus Adduction None None
Inferior oblique Excyclotorsion Elevation (maximal on medial gaze) Abduction
Cranial nerve IV
Superior oblique Incyclotorsion Depression (maximal on medial gaze)Abduction
Cranial nerve VI
Lateral rectus Abduction None None
Congenital superior oblique palsy of the left eye
(A) A typical abnormal head position is shown. The head is tilted to the right, away
from the side of the palsied muscle. The chin is tilted downward slightly. In this head
posture, the child fuses and avoids diplopia.
(B) There is a small left hypertropia (upward deviation) in primary gaze when the
head is nearly straightened; this is the first step in the "three-step test".
(C) The left hypertropia is worse in gaze to the right; this is the second step in the
"three-step test".
(D) A large left hypertropia is seen on forced (by the mother's hand) left head tilt; this
is the third step in the "three-step test".
Reproduced with permission from: Tasman W, Jaeger E. The Wills Eye Hospital
Atlas of Clinical Ophthalmology, 2nd ed, Lippincott Williams & Wilkins, Philadelphia
2001. Copyright ©2001 Lippincott Williams & Wilkins.
Etiology of fourth nerve palsy

Common associated clinical
Anatomic localization Specific cause
features
Aplasia of the nucleus
Vascular lesions (eg, brainstem arteriovenous
Hemisensory loss,
malformations)
Midbrain hemiparesis, central Horner
Demyelination (multiple sclerosis)
(nuclear/fascicular) syndrome, other brainstem
Brainstem hemorrhage, ischemia, or infarction
cranial neuropathies.
Trauma (including surgical)
Neoplasm (eg, glioma, metastasis)
Aneurysms (eg, superior cerebellar artery)
Increased intracranial pressure and hydrocephalus Headache, stiff neck, and
Infections (eg, mastoiditis, encephalitis meningitis) other cranial nerve
Postlumbar puncture or spinal anesthesia abnormalities. Focal lesions
Subarachnoid space Trauma (aneurysms,
Neoplasm (eg, carcinomatous meningitis, cerebellar schwannomas) may
hemangioblastoma, ependymoma, meningioma, produce isolated fourth
metastasis, neurilemmoma, pineal tumors, or trochlear nerve palsies.
nerve sheath tumors)
Neoplasm (eg, meningioma, metastasis)
Infection (eg, syphilis, tuberculosis, herpes zoster)
Third, fifth, or sixth nerve
Inflammation (eg, sarcoid, granulomatosis with
Cavernous sinus dysfunction, or Horner
polyangiitis [Wegener], or the Tolosa-Hunt syndrome)
syndrome.
Vascular lesions (eg, carotid-cavernous fistulas, internal
carotid artery aneurysm)
Neoplasm (eg, hemangioma, metastasis) Third, fifth, and sixth cranial
nerves as well as the optic
Infection (orbital cellulitis)
nerve. Orbital involvement
Orbital Infiltration (eg, sarcoid) may also produce such
signs as proptosis,
Inflammation (eg, orbital inflammatory pseudotumor)
chemosis, and orbital or
Trauma conjunctival edema.
Parks-Bielschowsky three-step test
The Parks-Bielschowsky three-step test is performed to make the diagnosis of a unilateral fourth nerve
palsy. Refer to UpToDate topics on fourth cranial nerve palsy and diplopia for other aspects of evaluation
and diagnosis.
Bilateral superior oblique palsy
A patient with a bilateral fourth nerve palsy demonstrates a reversing

hypertropia (left hypertropia in right gaze and a right hypertropia in left gaze in
the middle panels right and left). The primary position deviation is not readily
apparent, because of the bilateral palsy (middle row, center panel). There is
underaction of both superior oblique muscles (right lower and left lower panels).
There is overaction of the inferior oblique muscle bilaterally (right upper and left
upper panels).
Reproduced with permission from: Tasman W, Jaeger E. The Wills Eye Hospital
Atlas of Clinical Ophthalmology, 2nd ed, Lippincott Williams & Wilkins,
Philadelphia 2001. Copyright ©2001 Lippincott Williams & Wilkins.
Contributor Disclosures
Andrew G Lee, MD Consultant/Advisory Boards: Bayer [Intracranial idiopathic hypotension]. Paul W Brazis,
MD Nothing to disclose Janet L Wilterdink, MD Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must conform to
UpToDate standards of evidence.
Conflict of interest policy

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On examination, an ipsilateral hypertropia (deviation upward) and excyclotorsion (rotation outward) of

Associated clinical features — Fourth nerve palsies may be isolated or accompanied by other

● A midbrain nuclear or fascicular lesion (eg, tumor, stroke, demyelination) is typically

CLINICAL EVALUATION AND DIAGNOSIS

● Step 2 – Is the hypertropia worse in right or left gaze?

● Step 3 – Is the hypertropia worse in right or left head tilt?

● Large degree of excyclotorsion (>10 degrees).

Measurement of torsion — Torsion is measured objectively with a double Maddox rod. It is

Restrictive vertical strabismus — Restrictive vertical strabismus can be produced by orbital floor

Myasthenia gravis — Myasthenia gravis can mimic any painless, pupil-spared, nonproptotic

Thyroid ophthalmopathy — Thyroid ophthalmopathy is more commonly seen in adults. (See

● Lack of complaints of cyclotropia (subjective image tilting), despite objective fundus

TREATMENT AND PROGNOSIS

SUMMARY AND RECOMMENDATIONS

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Anatomic schematic showing the course of the fourth cranial nerve.

Actions of extraocular muscles

Congenital superior oblique palsy of the left eye

Etiology of fourth nerve palsy

Parks-Bielschowsky three-step test

Bilateral superior oblique palsy

A patient with a bilateral fourth nerve palsy demonstrates a reversing

Conflict of interest policy

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