Ophthalmoplegia (Weak or Paralyzed Eye Muscles)

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What is Ophthalmoplegia (Weak Eye Muscles)?

Ophthalmoplegia refers to the weakness or paralysis of the eye muscles. The condition can affect one or more of the six muscles that keep the eye in place and control its movement. This can lead to eye movement abnormalities.

If you are diagnosed with ophthalmoplegia, you are likely under observation for any shifts in muscle weakness or development of a genetic syndrome. The degree of muscle control you have may reduce over time.

If you experience the following, it is essential to contact your ophthalmologist:

  • Blurred vision
  • Double vision
  • ‘Floaters’ in your vision
  • A sudden headache
  • Dizziness

Different Types of Ophthalmoplegia

An ophthalmoplegia diagnosis may be confusing to patients. Doctors often use various lengthy words before or after ‘ophthalmoplegia.’ These words and phrases indicate different characteristics of the condition.

Such phrases may inform you:

  • Which particular eye muscles are being affected (internal vs. external ophthalmoplegia)
  • What area of the brain is having issues sending signals to the eye muscles (supranuclear vs. internuclear ophthalmoplegia)
  • Whether the ophthalmoplegia is chronic and/or progressive (chronic ophthalmoplegia or progressive ophthalmoplegia) 
  • Whether the condition affects one or both eyes (unilateral vs. bilateral ophthalmoplegia) 

If you receive an ophthalmoplegia diagnosis, the name of your condition may include one or more of these words or terms. Your eye doctor will explain what your case means and answer any questions you have about it.

Ophthalmoplegia cases may be split into two main groups based on whether it affects the external eye muscles (externalophthalmoplegia) or the internal eye muscles (internal ophthalmoplegia.

Internal Ophthalmoplegia

Internal ophthalmoplegia refers to paralysis or weakness in one of three internal muscles that help the eye focus and control pupil size.

This type of opthalmoplegia may affect any of the following three internal muscles of the eye:

  • Ciliary muscle. A ring of muscle that affects the shape of the lens. This enables the eye to focus on nearby or far away objects
  • Dilator pupillae. A muscle that largens the size of the pupil to let in more light
  • Sphincter pupillae. A muscle that shrinks the size of the pupil to allow in less light

External Ophthalmoplegia

External ophthalmoplegia is a weakness or paralysis in one of six muscles that work together to create eye movements. These muscles enable the eye to move up, down, side to side, and roll in a circle. 

This type of ophthalmol may affect any of the following six extraocular muscles:

  • Superior rectus. A muscle fixed to the top of the eye that moves the eye upward
  • Inferior rectus. A muscle attached to the base of the eye that moves it downward
  • Medial rectus. A muscle fixed to the inside of the eye that moves it toward the nose
  • Lateral rectus. A muscle attached to the exterior of the eye that moves it towards the ear
  • Superior oblique. A muscle that rotates the eye downward and inward
  • Inferior oblique. A muscle that rotates the eye upward and outward

Chronic Progressive External Ophthalmoplegia

A genetic mutation causes chronic progressive external ophthalmoplegia (CPEO). Symptoms usually start between the ages of 18 and 40.

This type of opthalmoplegia may affect one eye (unilateral) or both eyes (bilateral.) 

Those with CPEO may experience:

  • Weakness in the eyelids
  • Ptosis
  • Problems swallowing (dysphagia)
  • General weakness in the leg, arm, or neck muscles, especially following exercise

CPEO may result from different gene mutations. Inheritance patterns differ based on those mutations. In some cases, CPEO may develop as part of another condition.

For example, CPEO may occur with two rare neuromuscular disorders. These are ataxia neuropathy spectrum or Kearns-Sayre syndrome. Patients with either may experience other symptoms associated with those conditions.

Internuclear Ophthalmoplegia

Internuclear ophthalmoplegia is typically caused by a lesion in the brain and may be associated with MS or stroke. The term internuclear refers to the area of the brain affected. 

It is an area that works as a pathway between the nuclei. These are two clusters of nerve cells in the brain. Around 23 percent of MS patients have internuclear ophthalmoplegia.

Other conditions that may result in internuclear ophthalmoplegia include:

  • Infections such as HIV or syphilis
  • Trauma or tumors like glioma or medulloblastoma

Internuclear ophthalmoplegia results from a lesion on the brain called the medial longitudinal fasciculus (MLF.) The MLF is a tract of fiber located in the brain that transfers information between the brain and the eyes. The data transmitted by the MLF helps muscles to order eye movements.

In internuclear ophthalmoplegia, the affected eye has difficulty adducting (turning towards the nose). The fellow eye abducts (turns out towards the ear), but with nystagmus. Nystagmus is a vision problem in which the eyes create repetitive, uncontrolled movements. 

The divergence of the eyes during internuclear ophthalmoplegia leads to horizontal diplopia. If the right eye is affected, the individual will have double vision when looking to the left.

This type of ophthalmoplegia may develop in one eye (unilateral internuclear ophthalmoplegia) or both eyes (bilateral internuclear ophthalmoplegia).

Supranuclear Ophthalmoplegia

Most people with supranuclear ophthalmoplegia suffer from progressive supranuclear palsy (PSP.) This is a rare brain condition that leads to muscle weakness, walking issues, and other symptoms.

The term supranuclear refers to the brain area that is affected, which is an area above the nuclei. This type of opthalmoplegia may also be called progressive supranuclear ophthalmoplegia as the condition is progressive. This means that the disease gets worse over time, and there is no cure.

What Causes Ophthalmoplegia?

Ophthalmoplegia can be congenital, which means present at birth. Or it can develop later in life.

The condition is typically caused by a disruption of the messages transferred from the brain to the eyes. 

Internuclear ophthalmoplegia typically results from multiple sclerosis, infarction, or trauma. External ophthalmoplegia is typically caused by genetic mutations and may be associated with underlying conditions such Kearns-Sayre syndrome.

Other common causes of Ophthalmoplegia include:

  • Migraines
  • Thyroid disease
  • Stroke
  • Brain injury
  • Brain tumor
  • Infection

Signs & Symptoms of Ophthalmoplegia

People affected by ophthalmoplegia may experience double or blurred vision. They may also have an inability to position the eyes in sync.

Some people may also have a challenging time moving both eyes in every direction. Many will also experience drooping eyelids. This is known as ptosis.

If ophthalmoplegia links with a systemic disorder, other symptoms may include problems swallowing and general muscle weakness.

Risk Factors of Ophthalmoplegia

Ophthalmoplegia is slightly more common in people with diabetes. Men with diabetes over the age of 45 who have had type 2 diabetes for more than ten years were identified as a higher risk category for having ophthalmoplegia.

People with a condition that changes their muscle control, like multiple sclerosis or Graves’ disease, are also more at risk than others.

Generally, there are no lifestyle choices that increase the risk of developing ophthalmoplegia. However, maintaining a healthy vascular system by following healthy lifestyle practices will reduce your risk of stroke and associated vision problems.

How is Ophthalmoplegia Diagnosed?

Ophthalmoplegia is diagnosed with a physical examination to assess eye movements. Then an MRI or CT scan may be required to look at the eye close up.

Blood tests may be needed to determine whether ophthalmoplegia is caused by another condition, like thyroid disease. You may need to visit an eye specialist or neurologist for these tests.

Treatment Options for Ophthalmoplegia

Treatment for opthalmoplegia depends on the type, symptoms, and underlying cause of the condition.

Children born with ophthalmoplegia usually learn to compensate and may not be aware of their vision issues. Adults may receive special glasses or wear an eye patch to assist with double vision and help reach normal vision.

In some cases, treatments of migraines can result in improved outcomes for individuals with ophthalmoplegia. 

Ophthalmoplegia is usually a symptom of another syndrome or disease. But regular visits to the eye doctor may help with early detection. 

Even if you have normal vision, you should visit an eye specialist every two years or so. The eye possesses delicate muscles that rely on a healthy nervous and vascular system to function correctly.

People that have isolated ophthalmoplegia usually have a standard life expectancy. However, the underlying cause of the condition affects the overall prognosis. 

Genetic and mitochondrial diseases, genetic syndromes, or a history of vascular disease or stroke can affect someone’s outlook significantly. It is essential to discover, address, and treat the underlying cause of your ophthalmoplegia for the best outcome.

Resources
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Al Kahtani, Eman S et al. “Assessment of the prevalence and risk factors of ophthalmoplegia among diabetic patients in a large national diabetes registry cohort.” BMC ophthalmology vol. 16 118. 22 Jul. 2016, doi:10.1186/s12886-016-0272-7, https://pubmed.ncbi.nlm.nih.gov/27449153/ 

Chronic progressive external ophthalmoplegia, Genetic and Rare Diseases Information Center, https://rarediseases.info.nih.gov/diseases/4503/chronic-progressive-external-ophthalmoplegia 

Progressive external ophthalmoplegia, MedlinePlus, September 2020, https://medlineplus.gov/genetics/condition/progressive-external-ophthalmoplegia/ 

Al Kahtani, Eman S et al. “Assessment of the prevalence and risk factors of ophthalmoplegia among diabetic patients in a large national diabetes registry cohort.” BMC ophthalmology vol. 16 118. 22 Jul. 2016, doi:10.1186/s12886-016-0272-7, https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4957375/

Virgo, Jonathan D, and Gordon T Plant. “Internuclear ophthalmoplegia.” Practical neurology vol. 17,2 (2017): 149-153. doi:10.1136/practneurol-2016-001428, https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4957375/ 

Carlow, Thomas J. “Oculomotor ophthalmoplegic migraine: is it really migraine?.” Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society vol. 22,3 (2002): 215-21. doi:10.1097/00041327-200209000-00006, https://pubmed.ncbi.nlm.nih.gov/12352586/

Reddel, S W et al. “Chronic ophthalmoplegia with anti-GQ1b antibody.” Neurology vol. 54,4 (2000): 1000-2. doi:10.1212/wnl.54.4.1000, https://pubmed.ncbi.nlm.nih.gov/10691005/

Yu-Wai-Man, Cynthia et al. “Extraocular muscle atrophy and central nervous system involvement in chronic progressive external ophthalmoplegia.” PloS one vol. 8,9 e75048. 27 Sep. 2013, doi:10.1371/journal.pone.0075048, https://pubmed.ncbi.nlm.nih.gov/24086434/

Nyce, Maxwell Q et al. “Resolved External Ophthalmoplegia and Hearing Loss in Wernicke's Encephalopathy With Thiamine Replacement.” Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society, https://pubmed.ncbi.nlm.nih.gov/32833862/

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