One and a half syndrome
   HOME

TheInfoList



OR:

The one and a half
syndrome A syndrome is a set of medical signs and symptoms which are correlated with each other and often associated with a particular disease or disorder. The word derives from the Greek language, Greek σύνδρομον, meaning "concurrence". When a sy ...
is a rare weakness in eye movement affecting both eyes, in which one cannot move laterally at all, and the other can move only in outward direction. More formally, it is characterized by "''a conjugate horizontal
gaze palsy Conjugate gaze palsies are neurological disorders affecting the ability to move both eyes in the same direction. These palsies can affect gaze in a horizontal, upward, or downward direction. These entities overlap with ophthalmoparesis and opht ...
in one direction and an internuclear ophthalmoplegia in the other''".
Nystagmus Nystagmus is a condition of involuntary (or voluntary, in some cases) eye movement. Infants can be born with it but more commonly acquire it in infancy or later in life. In many cases it may result in reduced or limited vision. Due to the invol ...
is also present when the eye on the opposite side of the lesion is abducted. Convergence is classically spared as cranial nerve III (oculomotor nerve) and its nucleus is spared bilaterally.


Causes

Causes of the one and a half syndrome include pontine haemorrhage,
ischemia Ischemia or ischaemia is a restriction in blood supply to any tissue, muscle group, or organ of the body, causing a shortage of oxygen that is needed for cellular metabolism (to keep tissue alive). Ischemia is generally caused by problems w ...
, tumors, infective mass lesions such as tuberculomas, demyelinating conditions like
multiple sclerosis Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This ...
,
Arteriovenous malformation Arteriovenous malformation is an abnormal connection between arteries and veins, bypassing the capillary system. This vascular anomaly is widely known because of its occurrence in the central nervous system (usually cerebral AVM), but can app ...
, Basilar artery aneurysms and Trauma.


Anatomy

The syndrome usually results from single unilateral lesion of the
paramedian pontine reticular formation The paramedian pontine reticular formation, also known as PPRF or paraabducens nucleus, is part of the pontine reticular formation, a brain region without clearly defined borders in the center of the pons. It is involved in the coordination of eye ...
and the ipsilateral
medial longitudinal fasciculus The medial longitudinal fasciculus (MLF) is an area of crossed over tracts, on each side of the brainstem. These bundles of axons are situated near the midline of the brainstem. They are made up of both ascending and descending fibers that ...
. An alternative anatomical cause is a lesion of the abducens nucleus (VI) on one side (resulting in a failure of abduction of the ipsilateral eye and adduction of the contralateral eye = conjugate gaze palsy towards affected side), with interruption of the ipsilateral
medial longitudinal fasciculus The medial longitudinal fasciculus (MLF) is an area of crossed over tracts, on each side of the brainstem. These bundles of axons are situated near the midline of the brainstem. They are made up of both ascending and descending fibers that ...
after it has crossed the midline from its site of origin in the contralateral abducens (VI) nucleus (resulting in a failure of adduction of the ipsilateral eye).


Treatment

There have been cases of improvement in extra-ocular movement with
botulinum toxin Botulinum toxin, or botulinum neurotoxin (BoNT), is a neurotoxic protein produced by the bacterium ''Clostridium botulinum'' and related species. It prevents the release of the neurotransmitter acetylcholine from axon endings at the neurom ...
injection. Cases related to
multiple sclerosis Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This ...
sometimes subside with adequate treatment.


See also

* Internuclear ophthalmoplegia


References

{{Lesions of spinal cord and brain Disorders of ocular muscles, binocular movement, accommodation and refraction Syndromes