Ophthalmoplegia

Definition
Ophthalmoplegia is a paralysis or weakness of one or more of the muscles that
control eye movement. The condition can be caused by any of several neurologic
disorders. It may be myopathic, meaning that the muscles controlling eye movement
are directly involved, or neurogenic, meaning that the nerve pathways controlling eye
muscles are affected. Diseases associated with ophthalmoplegia are ocular myopathy,
which affects muscles, and internuclear ophthalmoplegia, a disorder caused by
multiple sclerosis, a disease which affects nerves.
Description
Because the eyes do not move together in ophthalmoplegia, patients may complain of
double vision. Double vision is especially troublesome if the ophthalmoplegia comes
on suddenly or affects each eye differently. Because ophthalmoplegia is caused by
another, underlying disease, it is often associated with other neurologic symptoms,
including limb weakness, lack of coordination, and numbness.
Causes and symptoms
Ocular myopathy is also known as mitochondrial encephalomyelopathy with
ophthalmoplegia or progressive external ophthalmoplegia. Because it is so often
associated with diseases affecting many levels of the neurologic system, it is often
referred to as ophthalmoplegia plus. The main feature is progressive limitation of
eye movements, usually with drooping of the eyelids (ptosis). !tosis may occur
years before other symptoms of ophthalmoplegia. Because both eyes are e"ually
involved and because ability to move the eyes lessens gradually over the course of
years, double vision is rare. On examination, the eyelids may appear thin. This disease
usually begins in childhood or adolescence but may start later.
#hen ophthalmoplegia is caused by muscle degeneration $myopathic%, muscle
biopsy, in which a small piece of muscle is surgically removed and examined
microscopically, will find characteristic abnormal muscle fibers called ragged red
fibers. In this form of ophthalmoplegia, the patient may experience weakness of the
face, the muscles involved in swallowing, the neck, or the limbs.
!rogressive external ophthalmoplegia is sometimes associated with specific
neurologic syndromes. These syndromes include familial forms of spastic paraplegia,
spinocerebellar disorders, or sensorimotor peripheral neuropathy. &earns'(ayre
syndrome causes ophthalmoplegia along with loss of pigment in the retina, the light'
sensitive membrane lining the eye. In addition, the disease may cause heart block that
must be corrected with a pacemaker, increased protein in the cerebrospinal fluid, and
a progressively disabling lack of muscular coordination $cerebellar syndrome%.
(ymptoms of the disease appear before age )*.
(ome of the progressive external ophthalmoplegia syndromes are unusual in that
inheritance is controlled by D+, in the mitochondria. The mitochondria are rod'
shaped structures within a cell that convert food to usable energy. -ost inherited
diseases are passed on by D+, in the cell nucleus, the core that contains the
hereditary material. -itochondrial inheritance tends to be passed on by the mother.
Other forms of progressive external ophthalmoplegia are not inherited but occur
sporadically with no clear family history. It is not known why some forms are
neurogenic and others are myopathic. In the forms inherited through mitochondrial
D+,, it is not known which gene product is affected.
Internuclear ophthalmoplegia in multiple sclerosis is caused by damage to a bundle of
fibers in the brainstem called the medial longitudinal fasciculus. In this syndrome, the
eye on the same side as the damaged medial longitudinal fasciculus is unable to look
outward $that is, the left eye cannot look left%. The other eye exhibits .erking
movements $nystagmus% when the patient tries to look left. Internuclear
ophthalmoplegia may be seen rarely without multiple sclerosis in patients with certain
types of cancer or with /hiari type II malformation.
0ye movement disorders and ophthalmoplegia can also be seen with progressive
supranuclear palsy, thyroid disease, diabetes mellitus, brainstem tumors, migraine,
basilar artery stroke, pituitary stroke, myasthenia gravis, muscular dystrophy, and the
1isher variant of 2uillain'Barr3 syndrome. , tumor or aneurysm in the cavernous
sinus, located behind the eyes, can cause painful ophthalmoplegia. !ainful
ophthalmoplegia can also be caused by an inflammatory process in the same area,
called Tolosa'4unt syndrome.
Diagnosis
The patient5s medical and family history and the examination findings will usually
help differentiate the various syndromes associated with ophthalmoplegia. In addition,
each syndrome is associated with characteristic features, such as nystagmus or ptosis.
,ll patients with progressive external ophthalmoplegia should have a muscle biopsy
to look for ragged red fibers or changes suggesting muscular dystrophy. , sample
should be sent for analysis of mitochondrial D+,. 0lectromyogram $0-2%,
measurement of electrical activity in the muscle, helps diagnose myopathy.
/omputed tomography scan $/T scan% or magnetic resonance imaging $-6I% scans of
the brain may be needed to rule out brain tumor, stroke, aneurysm, or multiple
sclerosis. #hen multiple sclerosis is suspected, evoked potential testing of nerve
response may also be helpful. ,nalysis of cerebrospinal fluid may show changes
characteristic of multiple sclerosis or &earns'(ayre syndrome. Other tests that may be
helpful in &earns'(ayre include electrocardiogram $measuring electrical activity of
the heart muscles%, retinal examination, and a hearing test $audiogram%. 1or possible
myasthenia gravis, the Tensilon $edrophonium% test should be done. Tests should also
be done to measure activity of the cell'surface receptors for acetylcholine, a chemical
that helps pass electrical impulses along nerve cells in the muscles. Thyroid disease
and diabetes mellitus should be excluded by appropriate blood work.
Treatment
There are no specific cures for ocular myopathy or progressive external
ophthalmoplegia. 7itamin 0 therapy has been used to treat &earns'(ayre syndrome.
/oen8yme 9 $ubi"uinone%, a naturally occurring substance similar to vitamin &, is
widely used to treat other forms of progressive external ophthalmoplegia, but the
degree of success varies. (pecific treatments are available for multiple sclerosis,
myasthenia gravis, diabetes mellitus, and thyroid disease. (ymptoms of
ophthalmoplegia can be relieved by mechanical treatment. (urgical procedures can
lift drooping eyelids or a patch over one eye can be used to relieve double vision.
Because there is no blink response, a surgically lifted eyelid exposes the cornea of the
eye so that it may become dry or be scratched. These complications must be avoided
by using artificial tears and wearing eyepatches at night. In &earns'(ayre syndrome, a
pacemaker may be needed.
Prognosis
The prognosis of progressive external ophthalmoplegia depends on the associated
neurological problems: in particular, whether there is severe limb weakness or
cerebellar symptoms that may be mild or disabling. ,s with most chronic neurologic
diseases, mortality increases with disability. !rogressive external ophthalmoplegia
itself is not a life'threatening condition. &earns'(ayre syndrome is disabling, probably
shortens the life span, and few if any patients have children. Overall life expectancy
for multiple sclerosis patients is seven years less than normal: death rates are higher
for women than for men.
Prevention
There is no way to prevent ophthalmoplegia.
Resources
Organiations
,merican ,cademy of +eurology. );<; -ontreal ,ve., (t. !aul, -+ **))=. $=)>%
=?*')?@;. httpABBwww.aan.com.
!ey terms
Cere"ellar C Involving the cerebellum, which controls walking, balance, and
coordination.
Cere"rospinal fluid C 1luid bathing the brain and spinal cord.
#eart "lock C , problem with electrical conduction in the heart muscle that may
lead to irregular heart beat and re"uire a pacemaker for treatment.
$itochondria C (pherical or rod shaped parts of the cell. -itochondria contain
genetic material $D+, and 6+,% and are responsible for converting food to energy.
ophthalmoplegia BophDthalDmoDpleDgiaB $of'thalEmo'pleF.ah% paralysis of the eye
muscles.ophthalmopleFgic
e%ternal ophthalmoplegia paralysis of the external ocular muscles.
internal ophthalmoplegia paralysis of the iris and ciliary apparatus.
nuclear ophthalmoplegia that due to a lesion of nuclei of motor nerves of the eye.
Parinaud&s ophthalmoplegia paralysis of con.ugate upward movement of the eyes
without paralysis of convergence, associated with midbrain lesions.
partial ophthalmoplegia that affecting some of the eye muscles.
progressive e%ternal ophthalmoplegia gradual paralysis affecting the extraocular
muscles, and sometimes also the orbicularis oculi, leading to ptosis and progressive
total ocular paresis.
total ophthalmoplegia paralysis of all the eye muscles, both intraocular and
extraocular.
ophDthalDmoDpleDgia $ f'th l m 'pl . , p'%
n.
!aralysis of one or more of the muscles of the eye.
oph'thal mo'ple gic adj.
ophthalmoplegia
GofthalHmIplJH.JDIK
0tymologyA 2k, ophthalmos L plege, stroke
an abnormal condition characteri8ed by paralysis of the motor nerves of the eye.
Bilateral ophthalmoplegia of rapid onset is associated with acute myasthenia gravis,
acute thiamine deficiency, botulism, and acute inflammatory cranial polyneuropathy.
These diseases are potentially very destructive and re"uire prompt attention. In some
patients with myopathic ophthalmoplegia, structural abnormalities and biochemical
disorders may be evident in limb muscles. Ophthalmoplegia is also associated with
ocular dystrophy.
ophthalmoplegia Gof'thalEmo'pleF.ahK
paralysis of the eye muscles. ad.., adj ophthalmopleFgic.
ophthalmoplegia e%ter(na paralysis of the extraocular muscles.
ophthalmoplegia inter(na paralysis of the iris and ciliary apparatus.
nuclear ophthalmoplegia that due to a lesion of nuclei of motor nerves of eye.
Parinaud&s ophthalmoplegia !arinaud syndrome.
partial ophthalmoplegia that affecting some of the eye muscles.
progressive ophthalmoplegia gradual paralysis of all the eye muscles.
total ophthalmoplegia paralysis of all the eye muscles, both intraocular and
extraocular.
ophthalmoplegia
paralysis of the eye muscles.
ophthalmoplegia e%terna
paralysis of the extraocular muscles.
ophthalmoplegia interna
paralysis of the iris and ciliary apparatus.
nuclear ophthalmoplegia
that due to a lesion of nuclei of motor nerves of the eye.
partial ophthalmoplegia
that affecting some of the eye muscles.
progressive ophthalmoplegia
gradual paralysis of all the eye muscles.
total ophthalmoplegia
paralysis of all the eye muscles, both intraocular and extraocular.
ophthalmoplegia
!aralysis of the ocular muscles. )%ternal ophthalmoplegia refers to paralysis of one
or more extraocular muscles. If the levator palpebrae muscle is also involved, the
condition is usually referred to as ocular myopathy. *nternal ophthalmoplegia
refers to a paralysis of the muscles of the iris and the ciliary muscle. Total
ophthalmoplegia refers to a paralysis of all the muscles in the eye, which results in
ptosis, immobility of the eye and pupil, and loss of accommodation. See 2raves5
disease: paralysis of the third nerve.
chronic progressive e%ternal ophthalmoplegia (CP)O) , rare disorder
characteri8ed by a progressive, bilateral ptosis and a loss of ocular motility. It is
associated with mutation of mitochondrial D+,, which results in abnormalities in
highly oxidative tissues such as the muscles and the brain. The initial sign is bilateral
ptosis, followed later by strabismus. , related mitochondrial myopathy is the !earns+
,ayre syndrome in which there is /!0O, a type of retinitis pigmentosa characteri8ed
by coarse pigment clumping which principally affects the central retina, and cardiac
conduction defects. This syndrome presents before age >; years. See myasthenia
gravis.
internuclear ophthalmoplegia (*-O) ,n eye movement disorder resulting from a
lesion in the medial longitudinal fasciculus, which disrupts the coordination between
the oculomotor nucleus and the abducens nucleus. It is characteri8ed by a limited
adduction by the eye on the same side of the body as the lesion, and a .erky,
hori8ontal nystagmus and overshoot by the other eye on abduction, when moving the
eyes towards the side of the body opposite to that of the lesion. /onvergence is
usually intact, unless the lesion is widespread. 7ertical ga8e gives rise to nystagmus
and oscillopsia. The condition is associated with multiple sclerosis, vascular disease,
tumour of the brainstem or encephalitis. See ocular dysmetria: supranuclear ga8e
palsy: 5one and one half5 syndrome.
ophDthalDmoDpleDgiDa $of'thal5mM'plJ5.J'N%,
!aralysis of one or more of the ocular muscles.
Gophthalmo' L 2. plg, strokeK
ophDthalDmoDpleDgiDa $of'thal5mM'plJ5.J'N%
!aralysis of one or more of the ocular muscles.
Gophthalmo- L 2. plg, strokeK
ophDthalDmoDpleDgiDa $of'thal5mM'plJ5.J'N%
!aralysis of one or more of the ocular muscles.
Gophthalmo- L 2. plg, stroke