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Hannah Song, O.D.
Jesse Brown VA resident under Thomas Stelmack, O.D.
Abstract:
A patient, SW, presents to the Veterans Administration with Parinaud’s upgaze palsy with skew
deviation. Atypical in presentation, Parinaud’s syndrome and skew deviation is further explored
with displays of the SW’s photos and/or video.
I.
Case History
SW, 46 yo white female, presents with chief complaint of constant horizontal and vertical
diplopia. Ocular history was unremarkable prior to the event of subarachnoid hemorrhage.
Medical history is remarkable for hypothyroidism, hypertension, alcohol abuse, subarachnoid
hemorrhage 3/26/08 from a basilar artery aneurysm, coiling procedure 3/26/08, post op
complication of deep vein thrombosis in right lower extremity, and inferior vena cava filter
4/3/08. Medications include Omeprazole 20 mg/day, Lisinopril 10 mg/day, and Synthroid
0.088 mg/day. Medical allergies include tetanus toxoid and penicillin.
II.
Pertinent findings
SW initially presents to the Jesse Brown VA with ataxia on walking, losing her balance
on her right side. Best corrected visual acuities is 20/25 OD and 20/25 OS. Pupils have a
sluggish response, however no afferent pupillary defect. Color vision (Ishihara) is normal OD
and OS. SW is unable to converge, however, light-near dissociation is noted. In primary
gaze, SW presents with left hypertropia. She fixates with OD and is able to adduct under full
effort. OS is capable of depression but difficulty when fixating with OD. Doll’s Head shows
both eyes elevate with doll’s head maneuver and intact. Cover test at distance shows 18
prism diopters of alternating exotropia and 20 prism diopters of alternating hypertropia.
Cover test at near presents with 18 prism diopters of constant right exotropia and 20 prism
diopters of constant left hypertropia. Results for Maddox rod testing in nine positions of
gaze with red lens over OD are as follow:
25 BI
20 BD OS
25 BI
20 BD OS
12BI
20 BD OS
14 BI
20 BD OS
18 BI
20 BD OS
12 BI
20 BD OS
16 BI
20 BD OS
30 BI
20 BD OS
12 BI
20 BD OS
Cranial nerve testing results are:
I:
intact
III:
paresis
IV, VI:
intact, torsion noted
VII:
intact
VIII:
intact
IX:
intact
X:
intact
XI:
intact
XII:
intact
Slit lamp examination is remarkable for mild OD ptosis with a MRD1=3 mm and MRD2=6
mm. Intraocular pressures is unremarkable. Dilated fundus examination presents with
extorsion of the right eye and intorsion of the left eye, otherwise unremarkable. MRA head
and neck, CT head, CXR, MRI brain are stable comparing to 4/08 findings.
III.
Differential diagnosis
Differential diagnosis for SW includes Superior oblique palsy, CN III palsy with pupil
sparing OD, skew deviation, and Parinaud’s syndrome. Differential diagnosis for light-near
dissociation include Argyll Robertson pupil, Adie’s Tonic pupil, Amaurotic pupil, Abberent
regeneration of the IIIrd nerve, and Dorsal Midbrain syndrome. Differential diagnosis for
Skew deviation, from Table one in “skew deviation revisited” by Brodsky, Donahue, et al.
include: superior oblique palsy, inferior oblique palsy, third nerve palsy, superior division 3rd
nerve palsy, ocular neuromyotonia, myasthenia gravis, systemic botulism, Lamber-Eaton
Syndrome, thyroid eye disease, monocular elevation deficiency, congenital fibrosis
syndrome, acquired brown syndrome, congenital brown syndrome, chronic progressive
external ophthalmoplegia, orbital fibrous bands, and extraocular muscle aplasia.
IV.
Diagnosis and discussion
Parinaud’s syndrome, also known as dorsal midbrain syndrome, may present with
bilateral ocular findings such as upgaze palsy, light near dissociation, eyelid retraction, and
convergence retraction nystagmus.5 Skew deviation is atypical vertical deviation caused by a
disturbance to the midbrain.3 SW did present with upgaze palsy and light near dissociation,
however sluggish response to direct light. Superior oblique palsy can mimic a comitant skew
deviation with presentations of head tilt toward paretic side.2 Key difference between the two
is by observing the retinal photographs. Skew deviation with ocular tilt will show intorsion
of the higher eye and extorsion of the lower eye, whereas superior oblique presents with
extorsion of the higher eye.2 SW presents in DFE with extorsion of OD (right hypo) and
intorsion of OS (left hyper). Diagnosis for CN III palsy with pupil sparing was eliminated
after extraocular muscles were observed to be grossly full with full effort and both eyes
elevated with doll’s head maneuver.
V.
Treatment, management
Prior to seeing SW, she was given Fresnel prism of +10 D BI OD and +20 D BD OS
using a plano carrier lens. SW states that she could only wear the Fresnel prisms for a max of
one hour due to decrease in clarity and onset of headache. Du Toit, Ramke, and Brian began
a study of nine participants to determine the tolerated amount of induced prism in plano
spectacles. They found that the majority could not wear spectacles with the highest prism
power of 1 pd BU, 2 pd BO, and 2 pd BI for eight hours.4 After discussion with neuroophthalmologist, it is believed that SW has a good prognosis and symptoms of diplopia will
improve in time. No extraocular muscle surgery was explored at this time. At her follow-up
in August, SW was observed to be stable with little improvement in her horizontal and
vertical deviations. Instead of wearing the Fresnel prism, monocular occlusion provided SW
the best relief from diplopia. She is scheduled to return to the clinic September 4, 2008. We
will then determine whether surgical intervention is necessary or continue with monocular
occlusion.
VI.
Conclusion
Although rarely seen, key point for comitant skew deviation is the extorsion of lower eye
and intorsion of the higher eye observed with double Maddox testing and/or fundus photo.
Parinaud’s syndrome observed in SW was due to the basilar aneurysm, which lead to
subarachnoid hemorrhage. Once her medical condition is stable, there is a chance that the
amount of horizontal and vertical deviation can change in time. An exact time period is not
known, however a time frame of weeks to four and a half months is given.6 If the deviation
is significant and the patient is ready, extraocular muscle surgery can be further explored.
VII. Bibliography
1. Ajtai B, Fine EJ, and Lincoff N. Pupil-sparing, painless compression of the
oculomotor nerve by expanding basilar artery aneurysm. Archives of Neurology
2004; 61: 1448-1450.
2. Brodsky MC, Donahue SP, Vaphiades M, and Brandt T. Skew Deviation revisited.
Survey of Ophthalmology 2006; 51 (2): 105-128.
3. Cassin B and Rubin M. Dictionary of Eye Terminology, 4th Ed. Gainesville, Triad
Communications, 2001, pp 40, 244.
4. Du Toit R, Ramke J, Brian G. Tolerance to prism induced by readymade spectacles:
setting and using a standard. Optometry and Vision Science, 2007; 84 (11): 10531059.
5. Kunimoto DY, Kanitkar KD, Makar MS (eds). The Wills Eye Manual. Philadelphia,
Lippincott Williams & Wilkins, 2004, pp 128-129, 204-207.
6. Miller NR. Late improvement in upward gaze in a patient with hydrocephalus related
Parinaud dorsal midbrain syndrome. British Journal of Ophthalmology 2006; 90 (1):
123.
7. Spector RH. Vertical diplopia. Survey of Ophthalmology 1993; 38 (1): 31-62.