Taiwan Journal of Ophthalmology 3 (2013) 75e77

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Taiwan Journal of Ophthalmology

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Case report

Foville’s syndrome with ipsilateral internuclear ophthalmoplegia due

to spontaneous pontine hemorrhage
Hui-Chen Cheng a, b, May-Yung Yen a, b, An-Guor Wang a, b, *
a
Department of Ophthalmology, School of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC
b
Department of Ophthalmology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC

a r t i c l e i n f o a b s t r a c t

Article history: A 30-year-old male presented to the emergency department with a complaint of acute headache with
Received 31 August 2012 pain in the right eye and simultaneous weakness and numbness on his left side. Results of ophthalmic
Received in revised form examinations showed limited abduction and adduction only in the right eye, while the vertical move-
4 October 2012
ment was normal. Mild ptosis with concurrent miosis oculus dexter was also found. A neurological
Accepted 28 November 2012
Available online 25 December 2012
examination revealed left hemiplegia, left hemiparesthesia, left-side sensory ataxia, right facial palsy,
and right facial analgesia. Computer tomography revealed a 2.4-cm high-density hemorrhage in the
bilateral dorsal aspect of pons, more at the right side. Foville’s syndrome with ipsilateral internuclear
Keywords:
Defoville’s syndrome
ophthalmoplegia was diagnosed and the patient received supportive treatment. The patient later com-
Foville’s syndrome plained of left hearing loss and the brainstem auditory evoked potential study suggested a peripheral
internuclear ophthalmoplegia lesion. Follow-up magnetic resonance imaging studies at the 3rd and 10th months showed old pontine
pontine hemorrhage hemorrhage with right hypertrophic olivary degeneration (HOD). We reported a rare case of Foville’s
syndrome with ipsilateral internuclear ophthalmoplegia due to spontaneous pontine hemorrhage. The
clinical manifestations correlated well with anatomical involvement. The sequela of HOD after potine
hemorrhage should be monitored for the possible late-onset movement disorder.
Copyright Ó 2012, The Ophthalmologic Society of Taiwan. Published by Elsevier Taiwan LLC. All rights
reserved.

1. Introduction side. He denied any history of systemic disease, trauma, or alcohol

Foville’s syndrome was first described by Achille-Louis François
Foville (1831e1887), a French anatomist and psychiatrist, who is
also called Defoville.1
3 It is characterized by ipsilateral sixth nerve
palsy, facial palsy, facial hypoesthesia, peripheral deafness, Horner’s
syndrome, contralateral hemiparesis, ataxia, pain, and thermal
hypoesthesia, with lesions in the pontine tegmentum.4 The aim of
this study was to report a rare case of Foville’s syndrome with
ipsilateral internuclear ophthalmoplegia due to spontaneous
pontine hemorrhage.
2. Case report
A 30-year-old well-nourished male presented to the emergency
department with a complaint of acute headache with pain in the
right eye and simultaneous weakness and numbness on his left
* Corresponding author. Department of Ophthalmology, Taipei Veterans General
Hospital, 201, Section 2, Shih-Pai Road, Taipei 112, Taiwan, ROC.
E-mail address: agwang@vghtpe.gov.tw (A.-G. Wang).
consumption. Results of ophthalmic examinations showed that his
best-corrected visual acuity was 6/10 in the right eye (oculus dexter
or OD) and 6/6 in the left eye (oculus sinister). Limited abduction
and adduction was noted only in the right eye, and the vertical
movement was normal. Mild ptosis with concurrent miosis OD was
also found (Fig. 1). Slit-lamp examination showed normal anterior
segment in both eyes (oculus unitas or OU) with prompt pupillary
reaction without relative afferent pupillary defect. An ophthal-
moscopy revealed clear disc margin and normal retina OU. Visual
field (Humphrey central 30-2, SITA Fast) was within the normal
limit OU with low-test reliability. A neurological examination
revealed left hemiplegia, left hemiparesthesia, left-side sensory
ataxia, right facial palsy, and right facial analgesia. The blood
pressure was 149/96 mmHg. A computer tomography scan showed
a 2.4-cm high-density hemorrhage in the bilateral dorsal aspect of
pons, more at the right side. According to the clinical manifesta-
tions and neuroimaging findings, Foville’s syndrome with ipsilat-
eral internuclear ophthalmoplegia resulting from the dorsal
pontine hemorrhage was diagnosed. The patient was admitted and
received supportive treatment. Serial laboratory tests including
complete blood count, electrolyte, prothrombin time, activated

2211-5056/$ e see front matter Copyright Ó 2012, The Ophthalmologic Society of Taiwan. Published by Elsevier Taiwan LLC. All rights reserved.
http://dx.doi.org/10.1016/j.tjo.2012.12.001
76 H.-C. Cheng et al. / Taiwan Journal of Ophthalmology 3 (2013) 75e77

Fig. 1. Gross appearance and extraocular muscle motility in the patient with Foville’s syndrome associated with ipsilateral internuclear ophthalmoplegia. Limited abduction and
adduction of the right eye (oculus dexter or OD) was noted. Mild ptosis with concurrent miosis OD was also found.

partial thrombin time, protein C, protein S, urine catecholamine,
antinuclear antibody, antiphospholipid antibody, cardiolipin anti-
body, and thyroid function were conducted and their results were
within the normal limit except for mildly elevated erythrocyte
sedimentation rate (87 mm/hour). The patient later complained of
left hearing loss. The pure tone audiometry showed left high tone
loss and the brainstem auditory evoked potential study suggested
a peripheral lesion.
Magnetic resonance imaging (MRI) 1 month later revealed a 3-
cm hemorrhage involving bilateral dorsal aspects of the pons,
mainly on the right side. No other significant vascular lesions can be
identified on an MR angiogram (Fig. 2). Ophthalmic examinations
at the 2nd month showed residual limitation of abduction and
adduction OD. Follow-up MRI studies at the 3rd and 10th months
showed old pontine hemorrhage with right hypertrophic olivary
degeneration (HOD). The patient underwent a rehabilitation
program during the follow-up period of 22 months.
3. Discussion
Foville’s syndrome, also known as Defoville’s syndrome, was
first described by the French anatomist and psychiatrist Achille-
Louis François Foville (1831e1887) in 1858.1e3 It is characterized
by ipsilateral sixth nerve palsy, facial palsy, facial hypoesthesia,
peripheral deafness, Horner’s syndrome and contralateral hemi-
paresis, ataxia, pain, and thermal hypoesthesia. The syndrome

Fig. 2. Magnetic resonance imaging (MRI) study 1 month later and associated anatomical location in the patient with Foville’s syndrome associated with ipsilateral internuclear
ophthalmoplegia. (A and B) T1-weighted MRI showed hemorrhage involving bilateral dorsal aspects of the pons, mainly on the right side. (C) MR angiogram revealed no significant
vascular lesions. (D) Schematic drawing depicted associated anatomical location and the affected area. CN5 ¼ trigeminal tract and nucleus; CN6 ¼ abducens nucleus and fascicle;
CN7 ¼ facial nerve nucleus and fascicle; CTT ¼ central tegmental tract; MLF ¼ medial longitudinal fasciculus; SON ¼ superior olivary nucleus; TB ¼ trapezoid body.
 H.-C. Cheng et al. / Taiwan Journal of Ophthalmology 3 (2013) 75e77
suggested a lesion in lower pontine tegmentum.4 It has been re-
ported in association with pontine infarction, hemorrhage, tuber-
culoma, and cerebellar tumors.5e8 Another clinical variant of
Foville’s syndrome, superior pons type of Foville’s syndrome, had
also been proposed and reported in association with the upper
pons.9,10 The clinical features of this variant included ipsilateral
cerebellar ataxia, Horner’s syndrome, paresis of conjugate gaze and
contralateral hemiparesis, facial palsy, pain, and thermal hypo-
esthesia. It usually occurs in association with the upper pons and is
caused by aneurysm of basilar artery.9,10
In our patient, limited abduction and adduction in the right
eye, with all directions of extraocular muscle motility preserved
in the left eye, suggested involvement of the right abducens
fascicle and medial longitudinal fasciculus, which were different
from the classical Foville’s syndrome. The patient also presented
with right Horner’s syndrome, right facial palsy, right facial
analgesia, left hemiplegia, and left hemiparesthesia, which rep-
resented the involved areas of right sympathetic fibers, facial
nerve fascicle, trigeminal tract, uncrossed corticospinal tract, and
uncrossed spinothalamic tract. In classic Foville’s syndrome,
ipsilateral peripheral deafness due to the involvement of the
superior olivary nucleus was noted.4 However, contralateral
peripheral deafness was noted in our patient, which might be
associated with affected crossed fibers in trapezoid body or
lateral lemniscus (Fig. 2).11
Ipsilateral HOD was noted in our patient during follow-up. HOD
is a form of trans-synaptic degeneration often caused by lesions in
the contralateral dentate nucleus or superior cerebellar peduncle
and ipsilateral central tegmental tract. The associated clinical
features were palatal myoclonus, cyclic jerk of the soft palate, and
ocular myoclonus.12 In this patient, pontine hemorrhage with
central tegmental tract involvement may result in HOD. Although
he did not have the fine movement disorders previously described
77
during follow-up, he should be monitored for the possible HOD-
associated movement disorders in the future.
In conclusion, we reported a rare case of Foville’s syndrome with
ipsilateral internuclear ophthalmoplegia due to spontaneous
pontine hemorrhage. The clinical manifestations were well corre-
lated with anatomical involvement. The sequela of HOD after
potine hemorrhage should be monitored for the possible late-onset
movement disorder.
References
1. Brogna C, Fiengo L, Ture U. Achille Louis Foville’s atlas of brain anatomy and the
Defoville syndrome. Neurosurgery 2012;70:1265e73. discussion 1273.
2. Foville A. Note sur une paralysie peu connue de certains muscles de l’oeil, et sa
liaison avec quelques points de l’anatomie et la physiologie de la protubĕrance
annulaire. Bull Soc Anat Paris 1858;33:393e414.
3. Silverman IE, Liu GT, Volpe NJ, Galetta SL. The crossed paralyses. The original
brain-stem syndromes of Millard-Gubler, Foville, Weber, and Raymond-Cestan.
Arch Neurol 1995;52:635e8.
4. Wong AMF. Eye movement disorder. New York: Oxford University Press; 2008.
5. Ahmed N, Riazn A, Shuaib A, Siddiqi Z. Foville’s syndrome masquerading as
Wernicke’s encephalopathy. N Z Med J 2006;119. U1928.
6. Sato K, Nitta E. [Pontine hemorrhage presenting with Foville syndrome and
transient contralateral hyperhidrosis]. Rinsho Shinkeigaku 2000;40:271e3
[Article in Japanese].
7. Bedi HK, Devpura JC, Bomb BS. Clinical tuberculoma of pons presenting as
Foville’s syndrome. J Indian Med Assoc 1973;61:184e5.
8. Levene LJ. Benign cerebellar tumor presenting a modified Foville syndrome.
J Am Geriatr Soc 1958;6:346e51.
9. Takase M, Saeki N, Oka N, Satoh A, Otaki M, Yamaura A. [Superior Foville
syndrome after clipping of basilar bifurcation aneurysmdcase report (author’s
transl)]. No Shinkei Geka 1981;9:343e7 [Article in Japanese].
10. Nakaso K, Nakayasu H, Isoe K, Nakashima K, Takahashi K. [A case of dissecting
aneurysm of the basilar artery presented as superior pons type of Foville’s
syndrome]. Rinsho Shinkeigaku 1995;35:1040e3 [Article in Japanese].
11. Doyle KJ, Fowler C, Starr A. Audiologic findings in unilateral deafness resulting
from contralateral pontine infarct. Otolaryngol Head Neck Surg 1996;114:482e6.
12. Asal N, Yılmaz O, Turan A, Yig it H, Duymuş M, Tekin E. Hypertrophic olivary
degeneration after pontine hemorrhage. Neuroradiology 2012;54:413e5.