Graefe’s Arch Clin Exp Ophthalmol
(2007) 245: 321–323 CASE REPORT
DOI 10.1007/s00417-005-0235-9
Flemming Staubach
Christina Pieh
Relative afferent pupillary defect with normal
Philip Maier
Wolf A. Lagrèze
vision and vertical strabismus—implications
for pupillary pathway anatomy
Received: 24 August 2005
Abstract Background: A relative There was a right-sided strabismus
Revised: 26 October 2005 afferent pupillary defect (RAPD) is sursoadductorius. Conclusions: A
Accepted: 4 December 2005 usually a sign of visual dysfunction. pathologic RAPD with normal vision
Published online: 22 March 2006 Here, an unusual case of an RAPD can be caused by tumour compression
# Springer-Verlag 2006 combined with vertical strabismus but of the contralateral pretectal nucleus
normal vision is described. Implica- or its afferent or efferent fibres. As an
tions for pupillary pathway anatomy implication for pupillary pathway
are discussed. Methods: A 12-year- anatomy, our case suggests that there
F. Staubach . C. Pieh . P. Maier . old girl with chronic headache was is equal distribution between crossing
W. A. Lagrèze shown to have a midbrain tumour. and non-crossing intercalated neu-
University Eye Hospital, She presented to us with intermittent rons. An associated strabismus can
Freiburg, Germany diplopia. Examination included visual show a non-paralytic pattern.
F. Staubach (*) acuity, visual fields, pupillary func-
Universitaets-Augenklinik, tion, anterior and posterior segments, Keywords Pupillary reflex .
Killianstr. 5, and strabismus evaluation with the Strabismus . Brain stem neoplasms .
79106 Freiburg, Germany tangent screen. Results: There was a Afferent pathways . Trochlear nerve
e-mail: flemming.staubach@uniklinik-
freiburg.de right-sided pathologic RAPD but no diseases . Pupil disorders . Ocular
Tel.: +49-761-2704047 afferent visual impairment. Isocoria motility disorders . Visual fields .
Fax: +49-761-2704114 was present in light and darkness. Strabismus . Midbrain tumors
Introduction the dorsal midbrain region, on the left more than on the
right side (Fig. 1a).
A relative afferent pupillary defect (RAPD) is usually a Biopsy revealed a pilocytic astrocytoma. After place-
sign of asymmetric prechiasmal or chiasmal visual dysfunc- ment of a ventriculo-peritoneal shunt the headache dis-
tion. Optic tract and, rarely, retrogeniculate lesions may appeared, but vertical and torsional diplopia at left gaze
also cause an RAPD [10]. Since pupillary fibres exit the occurred. We examined the patient for the first time 1 year
pregeniculate visual pathway toward the pretectum, a le- later. She reported that diplopia was unchanged. There
sion in this region may cause afferent pupillary dysfunction was isocoria in light and darkness (Fig. 2a, top row). The
without visual impairment. We present a case of RAPD swinging flashlight test revealed a right-sided RAPD
with normal vision associated with strabismus sursoad- (Fig. 2a, bottom row) of 0.6 log units as quantified with
ductorius which to our knowledge has not been reported neutral density filters.
before. Visual acuity, visual fields, colour vision and visually
evoked potentials were normal. Dissociated strabismic angles
as measured with the tangent screen showed excyclotropia
Case report that increased at upward gaze and right-sided hypertropia
that increased at left and upward gaze (strabismus
A 12-year-old girl presented with chronic headache to her sursoadductorius, Fig. 2b). Random dot stereopsis was
general practitioner. MRI showed a tumour compressing present in primary position and monocular duction ranges
322
Fig. 1 a Axial T1 weighted
contrast enhanced MRI scan
depicting a pilocytic astrocyto-
ma (asterisk) behind the mid-
brain. Note that there is more
compression of the left than of
the right side. b Schematic
drawing of the pupillary light
reflex pathway. The afferent part
is drawn according to the ori-
entation of the MRI scan. For
better legibility, the efferent part
is not, but continues the sche-
matic direction instead. The box
encloses the hypothetic sites of
the lesion: the left-sided pretec-
tal nucleus, its afferent fibres, or
its efferent fibres
A B
were full. In particular there was no restriction in vertical visual impairment. Eight such cases have been published
gaze or any other sign of Parinaud’s dorsal midbrain between 1984 and 2004 [4], only three of which included
syndrome. The same findings were present 4 and 12 months explicit quantification of the RAPD [5, 6, 8]. All eight
later. cases were caused by a tumour or haemorrhage in the
dorsal midbrain. The RAPD was, as here, always contra-
lateral to the lesion.
Discussion Figure 1b illustrates the current concept of the pupillary
pathway. Afferent pupillary dysfunction without afferent
We present a case of an RAPD without afferent visual dys- visual dysfunction suggests that the lesion affects afferent
function. An RAPD can be physiologic up to 0.3 log units pupillary fibres that no longer travel adjacent to visual
[7]. 0.6 log, as in our case, is pathologic. Quantification of fibres.
an RAPD as >0.3 log is therefore important to demonstrate Because these pupillary fibres do not leave the visual
that an RAPD is pathologic, especially in the absence of pathway before the chiasm [10], the presence of the RAPD
Fig. 2 a Flash and infrared
photography of the pupils. Note
the isocoria in light and dark-
ness (top row) and the right-
sided RAPD demonstrated with
the swinging flashlight test
(bottom row). b Strabismic an-
gles (horizontal, vertical, rota-
tional) at ten directions of gaze
measured with the Harms tan-
gent screen under dark red glass. A
C convergent deviation, + hy-
pertropia right eye, Ex excyclo-
tropia. Torsional deviations
B 0 +4 C1 +1 C2 0 Upward gaze
were only measured in the mid- 20 degrees
Ex 4
vertical line
C2 +7 C2 +4 C1 +1 C3 0 Horiz. Vert.
Ex 3 Torsional
C5 +2 C2 +0.5 C3 0 Downward gaze
20 degrees
0
Far left gaze Left gaze Right gaze
30 degrees 20 degrees 20 degrees
323
suggests asymmetric chiasmal distribution. Indeed, more Edinger-Westphal nucleus cross [9]. However, if this was
pupillary fibres cross in the chiasm than do not cross [9], so in our patient, one would expect anisocoria with a larger
hence the contralaterality of the RAPD. These pupillary pupil contralaterally. We did not find such anisocoria. We
fibres may to some degree originate in melanopsin gan- conclude that at least in our patient there seems to be fairly
glion cells [1]. It has been proposed that a considerable equal distribution of the intercalated fibres, provided the
proportion of the pupillary fibres from the temporal retina concept of Fig. 1b is correct here.
cross in the chiasm [9]; this might explain the surprising Associated motility disorders as reported in three other
magnitude of the RAPD. cases [5, 6, 8] were ipsilateral or bilateral trochlear nerve
In view of the large tumour, the exact site of the lesion is palsies or Parinaud’s dorsal midbrain syndrome. Our case
unclear. However, our findings suggest one or a combina- was associated with contralateral strabismus sursoadduc-
tion of the following left-sided pupillary pathway struc- torius. This strabismus pattern resembles classic trochlear
tures (Fig. 1b): nerve palsy but differs in that angles do not increase at
downward gaze but are constant or even increase from
(1) the fibres, which run toward the pretectal nucleus. These
downward to upward gaze. Strabismus sursoadductorius is
are located in the brachium of the superior colliculus.
also named inferior oblique overaction, a widespread, most-
(2) the pretectal nucleus itself or
ly latent congenital condition [2]. Thus, our patient may
(3) the intercalated fibres from the pretectal nucleus to the
have had an unrelated primary inferior oblique overaction
Edinger-Westphal nucleus.
not caused but decompensated by the tumour. However, in
More proximal lesions (e.g. optic tract) would entail vi- view of the tumour size a trochlear nerve palsy, even with a
sual field defects, more distal lesions (e.g. Edinger-Westphal non-classic pattern, may be an alternative explanation, es-
nucleus) anisocoria. pecially because trochlear nerve palsies can lose their in-
In addition to the pupillary pathway of Fig. 1b, there is crease in angles at downward gaze due to adaptive changes
input from the visual cortex [10]. However, it appears un- [3].
likely that a lesion of these fibres from the cortex causes the In case of an RAPD with normal visual function, we
RAPD, as in a study by Wilhelm et al. retrogeniculate le- recommend quantification of the RAPD and search for eye
sions did not cause an RAPD if located farther than 18 mm movement disorders. An associated strabismus sursoad-
from the lateral geniculate nucleus [10]. ductorius may either represent an unrelated decompensated
From the literature, there is evidence that more than 50% primary inferior oblique overaction or an old trochlear
of the intercalated fibres from the pretectal nucleus to the nerve palsy.
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