Malta Medical Journal, 35 (1): Pages (2023)

MMJ http://mmsjournals.org/index.php/mmj

CASE REPORT

One-and-a-half syndrome: Its presentation, causes and

neuroanatomy
Matthew Azzopardi, David Agius

B ACKGROUND
One-and-a-half syndrome involves a combination of an ipsilateral horizontal
gaze palsy and an ipsilateral internuclear ophthalmoplegia. This condition is
easily missed due to its presentation, but can be the first sign of serious
disease. We aim to increase awareness of this syndrome’s presentation and
give an insight into the intricate neuroanatomical connections that are
affected in it.

C ASE P RESENTATION
We present a case of a 39-year-old previously healthy female who presented
with a one-week history of diplopia and non-vertiginous dizziness. On
examination, a left horizontal gaze palsy with deficits in left abduction and
right adduction was noted, accompanied by left adduction weakness and
right horizontal disconjugate jerk nystagmus in abduction. A diagnosis of
OAHS was made, and she was admitted for further tests. An MRI of her brain
revealed multiple hyperintensities throughout, along with an enhancing
lesion in keeping with active disease. A diagnosis of Multiple Sclerosis was
made and she was given a five-day course of methylprednisolone, with which
her vision, and ultimately her gait, improved. She was discharged with
outpatient follow-up, to further discuss treatment options for her new
Matthew Azzopardi* MD (Melit.)
diagnosis.
Royal London Hospital
C ONCLUSION Barts Health NHS Trust
London, UK
Diplopia and vertigo are symptoms that should prompt careful clinical
[email protected]
examination with proper attention to ocular motility testing, and
subsequent referral to neurology if required. Unnecessary delays in David Agius MD(Melit.) MRCS FEBO
diagnosis and management could ultimately be detrimental to the FRCOphth
patient, and being aware of uncommon presentations would go a long Ophthalmologist and Lead Researcher
Faculty of Medicine and Surgery
way in enhancing patient safety.
Department of Surgery
Ophthalmology Outpatients
Mater Dei Hospital
Msida, Malta

*Corresponding Author

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Malta Medical Journal Volume 35 Issue 01 2023 124

 I NTRODUCTION horizontal disconjugate jerk nystagmus in
abduction. Convergence of the eyes was intact, and
One-and-a-half syndrome (OAHS), first described in no other significant findings were noted on
1967,1 presents with a combination of an ipsilateral examination.
horizontal gaze palsy (the ‘one’) and an ipsilateral
In view of her left lateral gaze palsy and left sided
internuclear ophthalmoplegia (INO) (the ‘half’).
internuclear ophthalmoplegia, a diagnosis of OAHS
Here we present a case of this syndrome which was made. The on-call neurology team assessed her
presented to Ophthalmic Casualty in Malta, in order and admitted her for further investigations, to
to increase awareness of this syndrome’s delineate the underlying cause.
presentation and give an insight into the intricate
Her blood investigations were all within normal
neuroanatomical connections that are affected in
limits, and a CT scan of the brain demonstrated no
this syndrome.
acute intracranial pathology. However, an MRI scan
C ASE of her brain revealed a midline symmetrical
hyperintensity underneath the floor of the fourth
A 39-year-old previously healthy female presented
ventricle, along with multiple hyperintensities in
to ophthalmic casualty with a one-week history of
both the cerebrum and cerebellum in a
diplopia and non-vertiginous dizziness. These were
periventricular and subcortical distribution. An
interfering with her daily life due to multiple falls, as
enhancing lesion in the left periventricular white
well as with her job as a lifeguard due to a decreased
matter was also noted, in keeping with an active
ability to differentiate between different areas of
plaque. These lesions were highly suggestive of
the beach. She denied having other
demyelinating plaques, and a diagnosis of Multiple
symptomatology such as headaches or nausea. She
Sclerosis was made. She was given a five-day course
was prescribed prochlorperazine by her general
of methylprednisolone, with which her vision
practitioner, but her symptoms only got worse and
improved and she started mobilizing independently.
she decided to go to the Emergency Department.
Alternating eye patching was also done to help with
Her past medical history included occasional
her vision. Once improved she was discharged with
sinusitis and myopia, for which she uses glasses. Her
outpatient follow-up, to further discuss treatment
only past surgery was a lower segment caesarean
options for her new diagnosis of multiple sclerosis.
section. She denied any smoking, drug use or excess
alcohol intake, and was on no regular treatment. D ISCUSSION
Her family history was unremarkable, and she was
OAHS is a syndrome which manifests only in
only allergic to seafood.
horizontal gaze. Three main structures are involved
On examination, her aided visual acuity was 6/18 in in horizontal gaze, namely the paramedian pontine
each eye, which improved to 6/12 in either eye reticular formation (PPRF), the internuclear fibres of
using pinhole. Pupils were equal and reactive to the medial longitudinal fasciculus (MLF) and the
light, and no relative afferent pupillary defect was abducens nuclei in the lower pons. Upon initiating
present. On attempted left gaze, there was a left horizontal gaze, an excitatory stimulus is sent from
horizontal gaze palsy (ie deficits in left abduction the PPRF to the ipsilateral abducens nucleus.2 In
and right adduction) whilst on attempted right gaze, turn, this trigger both the motor fibres of the
there was a left adduction weakness and right

Malta Medical Journal Volume 35 Issue 01 2023 125

ipsilateral lateral rectus and also the contralateral gaze palsy. This occurs because of the integral role
medial rectus via the interneuronal fibres of the the internuclear fibres of the MLF play in conjugate
contralateral MLF, which ascend and terminate in horizontal eye movements. Because of this damage,
the oculomotor nuclear complex.3 the ipsilateral eye would be unable to adduct, and
on abducting the contralateral eye a horizontal jerk
Since OAHS is purely a combination of ipsilateral
nystagmus is observed, giving rise to INO.3
horizontal gaze palsy and ipsilateral INO, a
combination of lesions giving rise to these two In fact, amongst the lesions found on MRI in our
components would need to be present. Firstly, with patient, a midline symmetrical hyperintensity
regards to the horizontal gaze palsy component, underneath the floor of the fourth ventricle was
there are four theoretically possible lesion noted, corresponding to the location of the
locations: colliculus facialis and the abducens nucleus (Figure
1).5 This lesion was probably the cause of the
1. Damage to the ipsilateral abducens nucleus and
horizontal gaze palsy component of her OAHS.
ipsilateral PPRF
The commonest visual symptoms reported in OAHS
2. Damage to the ipsilateral PPRF only
include diplopia, blurred vision and oscillopsia.3
3. Damage to the ipsilateral abducens nucleus However, it often presents with non-visual
only symptoms which would arise either as a
4. Two separate lesions damaging the ipsilateral consequence of the underlying pathology or of the
abducens nerve root fibre and the contralateral visual disturbance itself, such as nausea, vertigo,
MLF3 and unsteadiness.3 This highlights the importance of
not dismissing non-specific symptoms and signs
Furthermore, the pattern of horizontal gaze palsy
without a proper, thorough examination.
would also depend on the exact location of the
lesion. In situations where ipsilateral PPRF damage Many different disease pathologies can give rise to
is the culprit, both saccadic and pursuit eye the pontine lesions behind OAHS. In our patient,
movements are lost in addition to the horizontal OAHS was the first presenting feature of multiple
gaze palsy. However, if the PPRF lesion is rostral to sclerosis (MS). When one considers ocular
the abducens nucleus, vestibular reflexic horizontal presenting features of MS, optic neuritis
eye movements are preserved, contrasting with immediately comes to mind. However, this is not
PPRF lesions located at the level of the abducens the only possible ocular presenting feature, and in
nucleus, in which these reflexes are also lost. All of fact there are a number of other cases reported in
these voluntary and reflexic eye movements are which MS presented with OAHS.3 Most OAHS cases
also lost if the horizontal gaze palsy is due to are due to vascular causes, such as ischaemic or
ipsilateral abducens nucleus damage.3-4 haemorrhagic brainstem infarction, basilar artery
aneurysms and arteriovenous malformations. Other
Secondly, the ipsilateral INO component of OAHS
causes such as brainstem malignancies, infiltrative
occurs due to lesions involving the ipsilateral MLF,
lesions, and infections have also been reported.6
in combination with those causing the horizontal

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Figure 1: Transverse section of the Magnetic Resonance Imaging scan of our patient’s brain showing the
midline symmetrical hyperintensity underneath the floor of the fourth ventricle corresponding
with the abducens nucleus within the colliculus facialis (A)

Treatment of this syndrome is usually aimed at the C ONCLUSION

underlying cause, as in our case. However
symptomatic treatment modalities for diplopia, One-and-a-half syndrome is one of the lesser known
oscillopsia, blurred vision and other symptoms can ocular manifestations of common diseases such as
be employed, such as eye patching or prism use. multiple sclerosis and stroke. Diplopia and vertigo
Botulinum toxin injections in the lateral rectus are symptoms that should prompt careful clinical
muscle and surgical extraocular muscle recession examination including attention to ocular motility
have also been used with good effect.7-8 testing, with subsequent referral to neurology if
required. In many of the pathologies that cause this
Ever since this syndrome was first reported, similar
syndrome, unnecessary delays in diagnosis and
syndromes have been described. For example,
management could be detrimental to the patient.
eight-and-a-half syndrome refers to one-and-a-half
Proper examination and early involvement of the
syndrome with ipsilateral facial nerve palsy,9 whilst
relevant specialities could go a long way in helping
sixteen-and-a-half syndrome refers to eight-and-a-
ensure the best possible outcome for our patients.
half syndrome with ipsilateral hearing loss (VIII
cranial nerve).10

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 R EFERENCES
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2. Cohen, B. and A. Komatsuzaki, Eye movements induced
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evidence for integration in oculomotor pathways. Exp supranuclear and internuclear ocular motility disorders.
Neurol, 1972. 36(1): p. 101-17. Eye (Lond), 1997. 11 ( Pt 3): p. 377-80.

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20 cases and review of the literature. Neurology, 1983. p. 918-9.
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9. Eggenberger, E., Eight-and-a-half syndrome: one-and-a-
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case report with the "one-and-a-half" syndrome without Neuroophthalmol, 1998. 18(2): p. 114-6.
pyramidal tract deficit. Arq Neuropsiquiatr, 1995. 53(3-
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