Ophthalmoplegia Progressiva is a rare neurological disease that affects the muscles controlling eye movement. The spelling of the word may seem daunting, but it can be broken down using the International Phonetic Alphabet (IPA) as follows: / ɒfθælmɒpˈlɛdʒɪə prəˈɡresɪvə/. The first syllable is pronounced like "off" with the letter "h" and the second syllable sounds like "tham." The last two syllables form the sound of "progressive." Although challenging to spell, knowing how to say it can facilitate conversations with healthcare professionals.
Ophthalmoplegia progressiva, also known as chronic progressive external ophthalmoplegia (CPEO), is a rare genetic disorder characterized by the progressive weakening and paralysis of the muscles responsible for eye movement. It is a subtype of ophthalmoplegia, which refers to a general paralysis or weakness of the eye muscles.
The condition typically manifests in early adulthood and slowly worsens over time. Individuals with ophthalmoplegia progressiva may experience symptoms such as droopy eyelids (ptosis), difficulty moving the eyes in certain directions (ophthalmoplegia), and a decreased ability to control eye movement. These eye movement abnormalities can result in double vision (diplopia) or a restricted field of vision, making it challenging for affected individuals to focus properly on objects.
Ophthalmoplegia progressiva is commonly caused by mitochondrial DNA mutations, which affect the energy-producing structures within cells (mitochondria). These mutations disrupt the normal functioning of muscle cells, leading to the muscle weakness and gradual decline in eye muscle control. In some cases, other symptoms may be present, including muscle weakness in other parts of the body (such as facial muscles or limb muscles), heart abnormalities, or impairment of other organ systems.
Currently, there is no cure for ophthalmoplegia progressiva. However, treatment approaches aim to manage the symptoms and improve quality of life. Treatment options may include corrective eyewear, prisms or eye patches to alleviate diplopia, physical therapy to strengthen weakened muscles, or in severe cases, surgery to address eyelid drooping or correct misalignment of the eyes.
Progressive upper bulbar palsy, due to degeneration of the nuclei of the motor nerves of the eye.
A practical medical dictionary. By Stedman, Thomas Lathrop. Published 1920.
The term "ophthalmoplegia progressiva" has its etymology rooted in Greek and Latin.
1. Ophthalmoplegia:
- "Ophthalmos" (ὀφθαλμός) means "eye" in Greek.
- "Plegia" (πληγία) means "paralysis" or "inability to move" also in Greek.
The combination of these two words gives us "Ophthalmoplegia", which refers to paralysis or weakness of the extraocular muscles that control eye movement.
2. Progressiva:
- "Progressiva" comes from the Latin word "progressus", which means "progress" or "advancement".
The term "progressiva" is added to indicate that the condition of ophthalmoplegia worsens or progresses over time.