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Fourth Nerve Palsies

What Is the Topographic Anatomy of the

Fourth Nerve?

The fourth nerve nucleus is located in the midbrain beneath the inferior colliculus. The fourth nerve is the only cranial nerve that exits dorsally from the brainstem, it has the longest intracranial course, and it crosses in the anterior medullary velum. It passes between the superior cerebellar artery and the posterior cerebellar artery, runs in the subarachnoid space, travels within the lateral wall of the cavernous sinus, and enters the orbit via the superior orbital fissure to innervate the superior oblique muscle.

What Are the Clinical Features of Fourth

Nerve Palsies?

Fourth cranial nerve palsies may cause the following (von Noorden, 1986):

1.Incomitant hypertropia is demonstrated with the three-step maneuver. The hypertropia increases on head tilt toward the paralyzed side (positive Bielschowsky’s test). Usually the unaffected eye is fixating and the hypertropia occurs in the involved eye. Hypotropia may occur in the normal eye if the affected eye is fixating. The hypertropia is usually most prominent in the field of gaze of the involved superior oblique muscle, especially in cases of acute or recent onset. The hypertropia may also be most prominent in the field of gaze of the ipsilateral overacting inferior oblique muscle in subacute or chronic cases. In palsies of longer duration, the hypertropia may be relatively equal in the various gaze positions (spread of comitance).

2.Duction testing may variably reveal underaction of the ipsilateral superior oblique muscle, overaction of the ipsilateral inferior oblique muscle, or overaction of the contralateral superior oblique muscle.

281

282 Clinical Pathways in Neuro-Ophthalmology, second edition

3.Pseudo-overaction of the superior oblique in the uninvolved eye may occur with spread of comitance. Secondary contracture of the superior rectus muscle in the involved eye may cause hypertropia involving the entire lower field of gaze. In a patient with a superior oblique muscle paralysis who habitually fixates with the paretic eye and in whom overaction of the ipsilateral inferior oblique muscle has developed, less than the normal amount of innervation will be required when the patient looks up and to the contralateral side. Because the innervation flowing to the opposite superior rectus is ‘‘determined’’ by the overacting ipsilateral inferior oblique (Hering’s law), the opposite superior rectus muscle will seem paretic (inhibitional palsy of the contralateral antagonist). In these cases, the head tilt test will correctly determine which of the two eyes is paretic.

4.Excyclotropia due to loss of incyclotorsion function of the superior oblique muscle. This torsion may be evident on fundus exam and can be measured using double Maddox rod testing. The excyclotropia is usually symptomatic in acquired cases but is often asymptomatic in congenital cases.

5.An anomalous head tilt eliminates the hypertropia or less commonly the cyclotropia. This head tilt is present in approximately 70% of patients and is usually away from the involved side but may be paradoxical (toward the involved side) in about 3%.

It is important to differentiate patients with decompensation of a congenital fourth nerve palsy (FNP) from those with an acquired FNP. In patients with congenital FNPs:

1.

Old photos may show a long-standing head tilt.

2.

Patients usually are noted to have cyclotropia on examination but often do

 

not complain of cyclotropia (subjective image tilting) as do some patients with

 

acquired FNPs.

3.Large vertical fusional amplitudes (> 8 prism diopters) in primary gaze are characteristic of congenital cases.

4.Facial asymmetry (hypoplasia on side of head turn) suggests a congenital lesion.

Bilateral FNPs are suggested by the following:

1.A right hypertropia in left gaze and left hypertropia in right gaze (a reversing hypertropia).

2.A positive Bielschowsky test on tilt to either shoulder (‘‘double Bielschowsky test’’).

3.Large excyclotropia (> 10 degrees).

4.V-pattern esotropia (15 prism diopters or more difference in esotropia between upward and downward gaze). The V pattern is caused by a decrease of the abducting effect of the superior oblique(s) in depression and secondary overaction of the abducting effect of the inferior oblique muscle(s).

5.Underaction of both superior oblique muscles and=or overaction of both inferior oblique muscles on duction testing.

6.In general, bilateral FNPs tend to have a smaller hypertropia in primary position than do unilateral FNPs.

The criteria for the diagnosis of FNPs are listed in Table 12–1. FNP may be categorized as either isolated or nonisolated. For diagnostic classification based on topographic localization, nonisolated FNP may be grouped into the following four syndromes:

1.Midbrain (nucleus=fascicle syndrome) FNP

2.Subarachnoid space FNP

3.Cavernous sinus FNP

Fourth Nerve Palsies

283

Table 12–1. Criteria for the Diagnosis of Fourth Nerve Palsy

Binocular vertical and=or torsional diplopia or misalignment

Ipsilateral hyperdeviation in primary position, worsened by contralateral gaze and ipsilateral head tilt (the three-step test)

Variable ipsilateral excyclotorsion

Anomalous compensatory head or face position

Weakness of the involved superior oblique muscle on ductions

4. Orbital FNP

Nonisolated FNP (type 1; see below), with findings that localize to the brainstem, subarachnoid space, cavernous sinus, or orbit, should undergo a directed neuroimaging study (Berlit, 1991; Brazis, 1993; Burde, 1992; Celli, 1992; Elliot, 1991; Kim, 1992; Richards, 1992; Vanooteghem, 1992).

Table 12–2 outlines the clinical features of FNP by location of the responsible lesion. Table 12–3 lists the etiologies for an FNP based on clinical topographic localization.

Is the FNP Due to a Midbrain Lesion?

A midbrain (i.e., nuclear=fascicular) FNP is defined by the ‘‘company it keeps’’; other brainstem signs usually present, including hemisensory loss, hemiparesis, a central Horner’s syndrome, or other brainstem cranial neuropathies (e.g., third nerve palsy). The differential diagnosis includes midbrain ischemia, hemorrhage, demyelination, and neoplasm. Neuroimaging (preferably magnetic resonance imaging [MRI]) should be directed to the midbrain (class II–III, level B).

Is the FNP the Result of a Subarachnoid

Space Lesion?

Lesions of the subarachnoid space are rarely associated with an isolated FNP. Patients with subarachnoid space lesions usually have associated signs and symptoms including headache, stiff neck, and other cranial neuropathies. Neuroimaging (MRI) should be directed to the brainstem and subarachnoid space. Computed tomography (CT) imaging should be considered in cases of acute trauma, to evaluate bone lesions, or in the evaluation of acute vascular processes (e.g., subarachnoid hemorrhage). Lumbar puncture following negative neuroimaging should be considered in these cases (class II–III, level B).

Is the FNP Due to a Cavernous Sinus Lesion?

Cavernous sinus lesions are usually associated with other cranial nerve signs (e.g., third, fifth, or sixth nerve paresis) or a Horner’s syndrome. Neuroimaging (preferably MRI) should be directed to the cavernous sinus (class II–III, level B).

284 Clinical Pathways in Neuro-Ophthalmology, second edition

Table 12–2. The Localization of Trochlear Nerve Lesions

Structure Involved

Clinical Manifestation

A: Lesions affecting the trochlear nucleus and=or fascicles (superior oblique palsy contralateral to lesions)

Nucleus=fascicles alone

Isolated trochlear palsy (rare)

Pretectal region

Vertical gaze palsy (dorsal midbrain

 

syndrome)

Superior cerebellar peduncle

Dysmetria on side of lesion

Descending sympathetic fibers

Horner’s syndrome on side of lesion

Medial longitudinal fasciculus (MLF)

Ipsilateral paresis of adduction with

 

nystagmus of contralateral

 

abducting eye

Brachium of superior colliculus

Contralateral relative afferent pupillary

 

defect (RAPD) without visual

 

impairment

Anterior medullary velum

Bilateral trochlear nerve palsies

B: Lesions affecting the trochlear nerve within the subarachnoid space (superior oblique palsy usually ipsilateral to lesion unless mesencephalon compressed)

Trochlear nerve alone

Isolated trochlear palsy

Superior cerebellar peduncle

Ipsilateral dysmetria

Cerebral peduncle

Contralateral hemiparesis

C: Lesions affecting the trochlear nerve within the cavernous sinus and=or superior orbital fissure

Trochlear nerve alone

Isolated trochlear palsy (rare)

Cranial nerves III, VI, sympathetic

Ophthalmoplegia, pupil small, large, or

 

spared, ptosis

Cranial nerve V (ophthalmic division)

Facial=retro-orbital pain; sensory loss

 

(forehead)

Increased venous pressure

Proptosis; chemosis

D: Lesions affecting the trochlear nerve within the orbit

 

Trochlear nerve, trochlea, superior oblique

Superior oblique palsy

muscle or tendon

 

Mechanical restriction of superior oblique

Brown’s superior oblique tendon sheath

tendon

syndrome

Other ocular motor nerves=extraocular

Ophthalmoplegia, ptosis, restricted ocular

muscles

movements

Optic nerve

Visual loss; optic disc swelling=atrophy

Mass effect

Proptosis (occasionally enophthalmos),

 

chemosis, eyelid swelling, etc.

Source: Modified from Brazis, 2001, with permission from Lippincott Williams & Wilkins.

Is the FNP Caused by an Orbital Lesion?

Orbital lesions usually produce signs such as proptosis, chemosis, and orbital or conjunctival edema. Neuroimaging (preferably MRI) should be directed to the orbit (class II–III, level B).

We define six types of FNP, as shown in Table 12–4.

Fourth Nerve Palsies

285

Table 12–3. Etiologies for a Fourth Nerve Palsy Based on Clinical Topographic Localization

Midbrain (nuclear=fascicular) (Elliot, 1991; Tho¨mke, 2000) Aplasia of the nucleus

Arteriovenous malformation (Gonyea, 1990; Kim, 1992) Demyelination (Jacobson, 1999)

Hemorrhage (Galetta, 1998; Kim, 1993; Mon, 1996; Mu¨ ri, 1995; Tachibana, 1990; Tho¨mke, 1999)

Ischemia=infarction (Kim, 1993; Tho¨mke, 1999; Ulrich, 1998) Tumor (e.g., glioma) (Barr, 1997; Landolfi, 1998; Mielke, 2001) Trauma (including surgical)

Sarcoidosis (Leiba, 1996)

Arachnoid cyst of quadrigeminal cistern (Ohtsuka, 1998) Subarachnoid space

Aneurysm (e.g., superior cerebellar artery) (Agostinis, 1992; Collins, 1992) Hydrocephalus

Infections (mastoiditis, meningitis) (Carter, 1997; Ferreira, 1997; Sadun, 1999) Wegener’s granulomatosis (Newman, 1995)

Sarcoidosis (Frohman, 2001)

Superficial siderosis of central nervous system (CNS) (Hashimoto, 1996; Shinmei, 1997) Post–lumbar puncture or spinal anesthesia

Pseudotumor cerebri (Lee, 1995; Patton, 2000; Speer, 1999)

Trauma, including surgical (Baker, 1991; Hara, 2001; Hoya, 2000; Jacobson 1995; Lepore, 1995; Sabates, 1991)

Neoplasm

Carcinomatous meningitis Cerebellar hemangioblastoma Ependymoma

Meningioma

Metastasis

Neurolemmoma=schwannoma (Feinberg, 1999; Gentry, 1991; Santoreneos, 1997) Pineal tumors

Trochlear nerve sheath tumors Fisher’s syndrome (Tanaka, 1998) Churg-Strauss syndrome (Vitali, 1996)

Cavernous sinus

Neoplasm (e.g., meningioma, pituitary adenoma) (Eisenberg, 1999; Keane, 1996; Petermann, 1999)

Infectious: herpes zoster (Chang-Godinich, 1997), mucormycosis (Keane, 1996) Inflammation: Tolosa-Hunt syndrome, Wegener’s granulomatosis (Herman, 1999)

Internal carotid artery aneurysm (Arruga, 1991; FitzSimon, 1995; Hahn, 2000; Keane, 1996; Shimo-oku, 1998)

Dural carotid-cavernous sinus fistula (Tsai, 2000) Superior ophthalmic vein thrombosis (Polito, 1996) Foramen ovale electrode placement (Herrendorf, 1997)

Balloon test occlusion of cervical internal carotid artery (Lopes, 1998)

(continued)

286 Clinical Pathways in Neuro-Ophthalmology, second edition

Table 12–3. (continued)

Orbit Neoplasm Infection Infiltration

Waldenstro¨m’s macroglobulinemia Inflammation (orbital pseudotumor) Progressive systemic sclerosis Trauma (orbital floor fracture)

Other

Migraine (Wong, 1996)

Congenital (Botelho, 1996; Holmes, 1999)

Congenital unmasked by botulinum toxin therapy for cervical torticollis) (Varrato, 2000)

Cephalic tetanus (Orwitz, 1997)

Table 12–4. Definitions of the Six Types of Fourth Nerve Palsy (FNP)

Type 1: nonisolated

FNPs are considered nonisolated in the presence of the following features: Other neurologic or neuro-ophthalmologic signs

Evidence to suggest myasthenia gravis such as ptosis, or fatigability of the motility deficit Evidence for systemic inflammatory disorders such as giant cell arteritis

Type 2: traumatic

Isolated, unilateral, or bilateral FNPs that have a clearly established temporal relationship to previous head trauma and do not progress are considered traumatic in origin; patients have no other neurologic deficits other than those associated with the initial

traumatic event Type 3: congenital

Congenital FNPs may show the following:

Large vertical fusional amplitudes (greater than 8 prism diopters) Facial asymmetry or sternocleidomastoid muscle hypertrophy

Long-standing anomalous head position that may be present in old photographs Type 4: vasculopathic

Vasculopathic FNPs occur in patients older than 50 years of age with or without known hypertension or diabetes, or in younger patients with known vasculopathic risk factors

Type 5: nonvasculopathic

Patients without vasculopathic risk factors and not classified as any of the above types are classified nonvasculopathic FNP

Type 6: progressive or unresolved

FNPs that worsen after the acute stage (greater than 1 week) as defined by a significant increase in the measured ocular vertical deviation are considered to be progressive, and patients without improvement in the measured ocular vertical deviation after 6 to 8 weeks are considered unresolved

Fourth Nerve Palsies

287

Is the FNP Due to Trauma?

At least 23 retrospective studies of traumatic (type 2) FNP have recommended that isolated, traumatic, unilateral, or bilateral FNP do not require additional neuroimaging or further evaluation (Baker, 1991; Berlit, 1991; Burde, 1992; Hoya, 2000; Richards, 1992; Sabates, 1991). FNP after mild head trauma and out of proportion to the deficit have been observed in association with an underlying asymptomatic basal intracranial tumor in at least three reports (Jacobson, 1988; Miller, 1989; Neetens, 1981). Neetens reported three such cases, but two cases had other neuro-ophthalmologic signs as well (Neetens, 1981). Although uncommon, neuroimaging may be warranted in patients with FNP after minimal or trivial head trauma to exclude a mass lesion (class III, level C).

Is the FNP Congenital?

Clearly congenital unilateral or bilateral FNP (type 3) are not associated with intracranial lesions in isolation and therefore do not require further diagnostic evaluation such as neuroimaging studies (Burde, 1992; Robb, 1990; von Noorden, 1986, 1994) (class III–IV, level C).

Is the FNP Vasculopathic?

Vasculopathic FNP (type 4) do not require any initial neuroimaging studies, and observation for improvement over the next 6 to 8 weeks is recommended (Burde, 1992). Patients with vasculopathic FNP (type 4), often resolve spontaneously within 4 to 6 months. Rush reported a recovery rate for FNP of 53.5% in 172 nonselected cases, and a higher recovery rate of 71% in 166 patients with diabetes mellitus, hypertension, or atherosclerosis (Rush, 1981). Another report by Ksiazek et al described improvement in 90% of 39 patients with microvascular and idiopathic FNP within 6 months (Ksiazek, 1988). Vasculopathic FNP usually improves within a few months (Burde, 1992; Rush, 1981; von Noorden, 1986), and patients with progressive or unresolved FNP, or with new neurologic signs or symptoms, should have neuroimaging (class II–III, level B) (Agostinis, 1992; Arruga, 1991; Burde, 1992; Gentry, 1991; Miller, 1989; Rush, 1981). Patients with spontaneously resolving palsies do not require any further neuroimaging (class II–III, level B). It is recommended that elderly patients who present with headache, scalp tenderness, jaw claudication, or visual loss undergo an appropriate evaluation for giant cell arteritis, including an erythrocyte sedimentation rate and a temporal artery biopsy (Burde, 1992; Miller, 1989; Reich, 1990) (class III–IV, level B). There is insufficient evidence to recommend evaluation for giant cell arteritis in every patient with motility suggesting an isolated FNP (class IV, level U).

What Is the Evaluation of Nonvasculopathic

FNP?

Nonvasculopathic FNP (type 5) may be observed for improvement over the next 6 to 8 weeks (class III, level B). Patients with resolution of symptoms and signs do not require

288 Clinical Pathways in Neuro-Ophthalmology, second edition

further evaluation (class III, level B). Patients with progression or lack of resolution should undergo neuroimaging (preferably MRI). Myasthenia gravis may mimic FNP, and patients with variable or fatigable motility findings and=or ptosis should be evaluated for myasthenia gravis (see Chapter 15) (Burde, 1992; Miller, 1989) (class III–IV, level B).

Testing for vasculopathic risk factors in type 4 or type 5 FNP should be considered, even in the absence of a history of previous diabetes or hypertension. Green et al reported an isolated third nerve palsy as the initial clinical manifestation of diabetes in almost half of 25 patients (Green, 1964). Shrader and Schlezinger reported that almost 50% of diabetic sixth nerve palsies were the presenting clinical manifestation of the disease (Shrader, 1960). The results of these studies concerning vasculopathic third and sixth nerve palsies may well be applicable to vasculopathic FNP (class III, level C).

Table 12–5 summarizes the etiologies of FNP in 11 large retrospective series (Ellis, 1976; Harley, 1980; Keane, 1993; Ksiazek, 1988; Mittleman, 1976; Richards, 1992; Rucker, 1956, 1958, 1966; von Noorden, 1986; Wright, 1977). Traumatic FNP occurred in 35%, idiopathic FNP in 34%, vasculopathic FNP in 16%, neoplasm was reported in 3%, aneurysm in 0.5%, and a wide variety of miscellaneous conditions including myasthenia gravis, infections, thyroid disease, and inflammation in 11% of patients.

Younger patients, or those without vasculopathic risk factors (type 5), may require initial neuroimaging, but the data suggest that observation for spontaneous improvement may be sufficient (class III, level C). Isolated, idiopathic FNPs very rarely have been found to have an underlying etiology after prolonged follow-up, and most resolve spontaneously within several weeks to months (Coppeto, 1978; Ksiazek, 1988; Nemet, 1980). Two retrospective case series with follow-up greater than 6 months described the prognosis of isolated, idiopathic FNP. Coppeto et al reported that 12 of 15 cases had resolved by 4 months after a mean follow-up of 5.5 years (Coppeto, 1978). Nemet et al described 13 cases, with a follow-up ranging from 4 to 7 years, and all had resolved by 10 weeks (Nemet, 1980). None of the patients in either series developed new neurologic

Table 12–5. Etiologies for Acquired Isolated Fourth Nerve Palsy

Author

Cases

Trauma

Tumor

Vascular

Aneurysm

Unknown

Other

 

 

 

 

 

 

 

 

Rucker, 1956

40

12

1

8

1

15

3

Rucker, 1958

67

24

3

24

0

9

7

Rucker, 1966

84

23

7

13

0

28

13

Mittleman, 1976

64

22

42

Ellis, 1976

104

32

0

1

63

8

Wright, 1977

23

9

0

8

0

3

3

Harley, 1980

18

5

0

0

0

12

1

Richards, 1992

578

169

28

103

5

186

87

von Noorden, 1986

141

73

0

62

6*

Ksiazek, 1988

88

24

2

39

23

Keane, 1993

81

64

0

8

9

TOTAL

1288

457

41

203

7

443

137

 

100%

35%

3%

16%

0.5%

34%

11%

*‘‘Other’’ in this study included tumor, vascular, and myasthenia gravis.

 

 

 

Fourth Nerve Palsies

289

Table 12–6. ‘‘Isolated’’ Fourth Nerve Palsy Due to Intracranial Lesion

 

 

 

 

 

Author

Cases

Pathology

Other Neurologic Signs

 

 

 

 

 

Suzuki, 1962

4

Pinealomas

Yes

 

Rucker, 1956

2

Frontal lobe glioma

Unknown

 

 

 

Aneurysm of circle of Willis

Unknown

 

Rucker, 1958

3

Primary brain tumor (1)

Unknown

 

 

 

Metastatic (2)

Unknown

 

Wise, 1965

1

Persistent trigeminal artery

Headache

 

Rucker, 1966

7

Midbrain gliomas (2)

Unknown

 

 

 

Meningioma (1)

Unknown

 

 

 

Primary brain tumors (3)

Unknown

 

Khawam, 1967

1

‘‘Brain tumor’’

Unknown

 

Burger, 1970

8

Cerebellopontine angle (CPA)

Yes

 

 

 

tumors (4)

 

 

 

 

Cerebellar tumor (1)

Yes

 

 

 

Nasopharyngeal cancer (1)

Yes

 

 

 

Metastatic lung cancer (1)

Yes

 

 

 

Aneurysm (1)

Yes

 

Robert, 1973

2

Pituitary tumors

Yes

 

Ellis, 1976

1

‘‘Intracranial aneurysm’’

Unknown

 

King, 1976

1

Schwannoma

No

 

Scully, 1976

1

Medulloblastoma

Yes

 

Younge, 1977

4

Gliomas (2)

Unknown

 

 

 

Metastatic breast cancer (1)

Unknown

 

 

 

Metastatic ovarian cancer (1)

Unknown

 

Wray, 1977

2

Pituitary tumors

Yes

 

Coppeto, 1978

3

Ependymoma (1)

Yes

 

 

 

Medulloblastoma (1)

Yes

 

 

 

Acoustic neuroma (1)

Yes

 

Boggan, 1979

1

Schwannoma

Yes

 

Rush, 1981

10

Meningiomas (2)

Unknown

 

 

 

Primary brain tumor (1)

Unknown

 

 

 

Metastatic tumors (4)

Unknown

 

 

 

Intracavernous aneurysm (1)

Yes

 

 

 

Basilar aneurysm (1)

Unknown

 

 

 

Aneurysm=subarachnoid

Yes

 

 

 

hemorrhage (1)

 

 

Ho, 1981

1

Schwannoma

No

 

Neetens, 1981

3

Skull base tumors

Yes

 

Krohel, 1982

1

Juvenile pilocytic astrocytoma

Yes

 

Leunda, 1982

1

Schwannoma

Yes

 

McKinna, 1983

3

Aneurysms

Unknown

 

Reinecke, 1986

1

Reported in Krohel, 1982

Yes

 

Jacobson, 1988

1

Vascular malformation

Yes

 

Slavin, 1987

1

Cavernous meningioma

*

 

Yamamoto, 1987

1

Schwannoma

Headache

 

 

 

 

(continued)

290

Clinical Pathways in Neuro-Ophthalmology, second edition

 

Table 12–6. (continued)

 

 

 

 

 

 

 

Ksiazek, 1988

2

‘‘Compressive etiologies’’

Unknown

Maurice-Williams, 1989

1

Intracavernous aneurysm

Headache

Gonyea, 1990

1

Brainstem arteriovenous

Headache

 

 

 

malformation (AVM)

 

Arruga, 1991

1

Intracavernous aneurysm

No

Agostinis, 1992

1

Superior cerebellar aneurysm

Headache

Collins, 1992

1

Superior cerebellar aneurysm

Headache

Richards, 1992

14

Meningioma (7)

Unknown

 

 

 

Metastatic (1)

Unknown

 

 

 

Glioma (4)

Unknown

 

 

 

Acoustic neuroma (1)

Unknown

 

 

 

Other primary (1)

Unknown

Kim, 1993

1

Brainstem stroke

Yes

Mon, 1996

1

Midbrain hemorrhage

No

Galetta, 1998

1

Midbrain hemorrhage

No

Petermann, 1999

1

Pituitary tumor

Headache

Feinhers, 1999

6

Trochlear schwannoma

No

Tho¨mke, 1999

3

Brainstem lacunes (2)

No

 

 

 

Hemorrhage (1)

No

Mielke, 2001

1

Metastatic bronchial cancer

No

*Patient developed progression of deviation after 2 years.

disease over an extensive follow-up period. Although type 5 patients who improve may not require neuroimaging, the clinical certainty of such a recommendation is not sufficiently strong in our opinion to obviate the need for neuroimaging in these nonvasculopathic patients (class III, level U). However, neuroimaging should be considered for patients who do not improvement in 2 months (class III, level C). Some reports have described aneurysm as an extremely rare cause for isolated FNP (Agostinis, 1992; Arruga, 1991; Collins, 1992; Richards, 1992; Rucker, 1956; Rush, 1981), and cerebral angiography is not recommended unless an aneurysm is suggested by other neuroimaging studies (class III, level B). Agostinis et al and Collins et al reported isolated FNP due to superior cerebellar aneurysms, but both patients described headaches (Agostinis, 1992; Collins, 1992). In these cases, neuroimaging studies confirmed the presence of the aneurysm before angiography. There are insufficient data to make a comment on the usefulness of MR angiography in FNP (class III–IV, level U).

Although MR scans are generally felt to be a more sensitive and specific than CT in the evaluation of cranial neuropathies, no conclusive evidence demonstrates an increased yield from performing an MR scan rather than a CT scan for the specific evaluation of FNP. Richards et al reported an etiologic diagnosis in 69 of 144 (48%) FNP using MRI and in 289 of 684 (42%) cases using CT. These authors felt that ‘‘multiplanar CT may be a sufficient noninvasive study, especially when clinical suspicion is high . . .

[or] in patients with other neurologic findings’’ (Richards, 1992). Nevertheless, we believe that MRI is the study of choice for patients with FNP (class II–III, level B).

A number of cases have been reported in the literature documenting intracranial lesions in patients with FNP. Table 12–6 summarizes 86 cases of ‘‘isolated’’ FNP due to

Fourth Nerve Palsies

291

Figure 12–1. Evaluation of fourth nerve palsy (FNP).

an intracranial lesion. Of these 86 patients, only five (5.8%) did not have other neurologic signs or symptoms and thus would be considered truly isolated by our criteria. One developed other neurologic signs after a short follow-up period, and in the remaining four patients persistence or progression of symptoms would have eventually resulted in a neuroimaging study. Of the remaining 81 patients, six had headache or

292 Clinical Pathways in Neuro-Ophthalmology, second edition

pain (7%), 31 had other neurologic signs (38%), and the clinical information was insufficient to determine if the FNP was truly isolated in 44 patients (54%). Keane reported intracranial tumor as an etiology in 12 of 95 unilateral cases, but all 12 (100%) had other neuro-ophthalmic signs, and none of 81 isolated FNP later reported by Keane had an intracranial tumor (Keane, 1993). This would suggest that the yield for evaluation of an isolated FNP is low (class III, level C).

All patients with progressive FNP (type 6) should undergo neuroimaging (preferably MRI). Lumbar puncture should be considered if neuroimaging is normal or if there are signs or symptoms of meningeal irritation (class III, level C).

An approach to FNP is outlined in Figure 12–1.

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