Ophthalmoplegic syndromes are conditions in which there is complete weakness or paralysis of one or more of the muscles controlling eye movement.
An ophthalmoplegic (off-thal-mo-PLEE-jik) syndrome * refers to a condition in which there is complete weakness or paralysis * of one or more of the muscles controlling eye movement. (“Ophtho” means eye and “plegia” means paralysis.)
The control of eye movements is complex and involves muscles, nerves, and interconnecting pathways. Three pairs of muscles, known as the extraocular muscles, are involved in the movement of each eye: the superior and inferior oblique, superior and inferior recti, and lateral and medial recti. The extraocular muscles are responsible for three types of eye movement: horizontal (the pupil directed toward or away from the nose), vertical (the pupil directed up or down), and torsional (rotational movement of the eyes). The three nerves that originate from the brain stem *
The most common symptom is diplopia (de-PLO-pee-ah), or double vision * . Diplopia can involve one or both eyes, can be painful or painless, can come on suddenly or gradually, and can be congenital (present at birth) or acquired. Other symptoms, depending on the type of opthalmoplegic syndrome encountered, include blurred vision, a drooping of the eyelids, difficulty controlling eye movement, a general weakness of the skeletal muscles, and difficulty swallowing.
Congenital ophthalmoplegia is a rare nonprogressive syndrome due to fibrosis (increased fibrous tissue) of one or several of the muscles supplied by the third nerve, leading to restriction of upward gaze.
Acquired ophthalmoplegic syndromes can be classified by the part of the eye movement control apparatus that is involved.
In Graves’ disease, in which there is too much functioning of the thyroid gland * , the extraocular muscles become bulky due to fat and connective tissue * deposits leading to a “staring look” with restriction of eye movements. Orbital fractures, direct trauma * to the eye, or tumors * may lead to muscle entrapment and ophthalmoplegia.
Progressive external ophthalmoplegia develops in late childhood or adolescence with paralysis of the extraocular muscles, ptosis (droopy eyelids), hearing loss, cataracts * , neuropathy, ataxia (lack of coordination), and sexual dysfunction.
Kearns-Sayre syndrome starts in the first or second decade with ophthalmoplegia, retinal degeneration, ataxia, deafness, muscle weakness (face and swallowing), and heart rhythm abnormalities. These disorders are due to DNA mutation and are either inherited or occur sporadically.
The typical disorder is myasthenia gravis (MY-as-theen-a GRAV-us), which is characterized by ptosis and extraocular, skeletal, and respiratory * muscle weakness that typically worsens with activity and improves with rest. It occurs at any age from childhood to adulthood and is due to an immune * attack on acetylcholine (a chemical involved in neuromuscular transmission) receptors * .
Another disorder is botulism, in which the botulinum toxin from certain bacteria leads to pupil dilatation and ophthalmoplegia.
A tumor, aneurysm, or clot located behind the eye can lead to painful, puffy, and paralyzed eyes. Tolosa-Hunt syndrome is an inflammatory condition of the same region that causes painful ophthalmoplegia. Various infections or infiltration of cancer cells can affect the cranial nerves, resulting in ophthalmoplegia. In the brainstem, strokes * , tumors, and multiple sclerosis * can all result in this condition.
Ophthalmoplegic migraine is a type of headache that causes transient paralysis of the extraocular muscles. In diabetes * , due to interruption of blood supply, painful paralysis of one or more muscles supplied by that cranial nerve occurs.
In internuclear ophthalmoplegia, the MLF is affected by multiple sclerosis, tumor, or stroke, leading to a disturbance in conjugate (paired or parallel) eye movements.
Progressive supranuclear palsy is a rare neurodegenerative disorder affecting certain parts of the brain that exert control over the nuclei involved in eye movement, resulting in restricted upgaze, gait imbalance, dementia * , and symptoms similar to Parkinson's disease * . Neurodegenerative diseases such as Huntington disease * , Wilson's disease (due to impaired copper metabolism), and Wernicke's disease (due to thiamine deficiency from chronic alcohol use) impair the upward gaze.
Injury to various parts of the cerebral cortex * due to tumors, strokes, and trauma may result in ophthalmoplegia.
Antipsychotic drugs (medications used to treat severe mental disorders) such as phenothiazines, which are antidepressants used for treating depression, and anticonvulsants (medications used to prevent or stop seizures) such as phenytoin can result in ophthalmoplegia. Thiamine and vitamin E deficiencies have been identified as the cause of this disorder as well.
Diagnosis is made by a neurologist, a physician specializing in diagnosing and treating diseases of the nervous system; an ophthalmologist, a physician specializing in diseases of the eye; or by a neurologist who specializes in eye disorders (neuro-ophthalmologist). Diagnosis is made by a combination of thorough history taking, physical examination, and laboratory tests.
Eyes are examined for protrusion, redness, ptosis, and tenderness. Then, eye movements are examined separately in each eye and together in both eyes. A red glass or prism in front of each eye helps to pinpoint the eye and the muscle that is responsible for the false image leading to diplopia. Pupils are examined with respect to their reaction to light. A dilated eye exam is done to examine other structures, such as the retina. Finally, a thorough neurological * exam is conducted that includes systematic tests of how well various parts of the nervous system are functioning. A general physical exam may also be performed.
Laboratory testing includes a complete blood count, blood chemistry profile, and thyroid testing. Based on the history and physical exam, one or more of the following tests may be performed:
Specific treatment depends on the syndrome. Myasthenia gravis is treated with corticosteroids (kor-tih-ko-STIRoyds), which are chemical substances made by the adrenal glands that have several functions in the body, including maintaining blood pressure during stress and controlling inflammation; diabetes with insulin * or medications; and multiple sclerosis with immunosuppressants (im-yoono-su-PRES-ants), which are substances that weaken the body's immune system. Medical professionals have tried Vitamin E to treat Kearns-Sayre syndrome and Coenzyme Q in progressive external ophthalmoplegia, but there is no definitive treatment for these. A pacemaker * may be needed in Kearns-Sayre syndrome. All syndromes benefit from symptomatic treatment for alleviating diplopia. The basic principle is to eliminate the abnormal image from the eye with the weak muscle by using an eye patch or special glasses with prisms, or by having surgery for ptosis.
See also Dementia • Diabetes • Eye Disorders: Overview • Huntington Disease • Muscular Dystrophy • Myasthenia Gravis (MG) • Parkinson's Disease • Stroke • Tumor • Thyroid Disease
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Merck Manual: Consumer Version. “Vision, Double (Diplopia).” https://www.merckmanuals.com/home/eye-disorders/symptoms-of-eye-disorders/vision,-double (accessed July 29, 2016).
National Organization for Rare Disorders. “Kearns Sayre Syndrome.” https://rarediseases.org/rare-diseases/kearns-sayre-syndrome/ (accessed March 3, 2016).
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American Academy of Ophthalmology. 655 Beach St., San Francisco, CA 94109. Telephone: 415-561-8500. Website: http://www.aao.org (accessed March 3, 2016).
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National Organization for Rare Disorders. 55 Kenosia Ave., Danbury, CT 06810. Toll-free: 800–999–6673. Website: http://www.rarediseases.org (accessed March 3, 2016).
* syndrome is a group or pattern of symptoms or signs that occur together.
* paralysis (pah-RAHL-uh-sis) is the loss or impairment of the ability to move some part of the body.
* brain stem is the part of the brain that connects to the spinal cord. The brain stem controls the basic functions of life, such as breathing and blood pressure.
* double vision is a vision problem in which a person sees two images of a single object.
* thyroid gland (THY-roid GLAND) is located in the lower part of the front of the neck. The thyroid produces hormones that regulate the body's metabolism (meh-TABo-liz-um), the processes the body uses to produce energy, grow, and maintain body tissues.
* connective tissue helps hold the body together. It is found in skin, joints, and bones.
* trauma refers to a wound or injury, whether psychological or physical. Psychological trauma refers to an emotional shock that leads to lasting psychological damage.
* tumors (TOO-morz) are abnormal growths of body tissue that have no known cause or physiologic purpose. Tumors may or may not be cancerous.
* cataracts (KAH-tuh-rakts) are areas of cloudiness of the lens of the eye that can interfere with vision.
* respiratory (RES-pi-ra-tor-ee) refers to the breathing passages and lungs.
* immune (ih-MYOON) means resistant to or not susceptible to a disease.
* receptors are cell structures that form a chemical bond with specific substances, such as neurotransmitters. This leads to a specific effect.
* strokes are brain-damaging events usually caused by interference with blood flow to the brain.
* multiple sclerosis (skluh-RO-sis), or MS, is an inflammatory disease of the nervous system that disrupts communication between the brain and other parts of the body.
* diabetes (dye-uh-BEE-teez) is a condition in which the body's pancreas does not produce enough insulin or the body cannot use the insulin it makes effectively, resulting in increased levels of sugar in the blood.
* dementia (dih-MEN-sha) is a loss of mental abilities, including memory, understanding, and judgment.
* Parkinson's disease is a disorder of the nervous system that causes shaking, rigid muscles, slow movements, and poor balance.
* Huntington disease is a genetic condition that leads to involuntary twitching or jerking of the muscles in the face, arms, and legs, along with a gradual loss of mental abilities.
* cerebral cortex (suh-REE-brul KOR-teks) is the part of the brain that controls functions such as conscious thought, listening, and speaking.
* neurological (nur-a-LAH-je-kal) refers to the nervous system, which includes the brain, spinal cord, and the nerves that control the senses, movement, and organ functions throughout the body.
* computed tomography (kom-PYOO-ted toe-MAH-gruh-fee), or CT, is a technique in which a machine takes many x-rays of the body to create a threedimensional picture.
* magnetic resonance imaging (or MRI) uses magnetic waves, instead of x-rays, to scan the body and produce detailed pictures of the body's structures.
* biopsy (BI-op-see) is a test in which a small sample of skin or other body tissue is removed and examined for signs of disease.
* DNA or deoxyribonucleic acid (dee-OX-see-ry-bo-nyoo-KLAY-ik AH-sid) is the specialized chemical substance that contains the genetic code necessary to build and maintain the structures and functions of living organisms.
* insulin is a hormone, or chemical produced in the body, that is crucial in controlling the level of glucose (sugar) in the blood and in helping the body use glucose to produce energy.
* pacemaker is a device whose function is to send electrical signals that control the heartbeat.