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Amaurosis – Causes, Symptoms and Treatment

Amaurosis refers to complete blindness in one or both eyes without any visible structural change to the eyeball. It can occur transiently or permanently.

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Things worth knowing about "Amaurosis"

Amaurosis refers to complete blindness in one or both eyes without any visible structural change to the eyeball. It can occur transiently or permanently.

What is Amaurosis?

Amaurosis (from Greek amauros meaning dark or dim) is a term used to describe complete or near-complete loss of vision in one or both eyes that occurs without an apparent external abnormality of the eye itself. The eyeball typically looks normal on the outside, yet vision is severely impaired or entirely absent. Amaurosis can be sudden and temporary (transient) or permanent, depending on its underlying cause.

Causes

Amaurosis can result from a wide range of conditions affecting the retina, optic nerve, or the visual processing centers in the brain:

Vascular causes: Central retinal artery occlusion, transient ischemic attacks affecting the eye (amaurosis fugax)
Neurological causes: Optic neuropathy, stroke affecting the visual cortex
Hereditary causes: Leber congenital amaurosis (LCA), a rare inherited retinal dystrophy
Toxic causes: Poisoning (e.g., methanol), adverse drug reactions
Inflammatory causes: Optic neuritis, often associated with multiple sclerosis
Tumor-related causes: Compression of the optic nerve by a tumor or elevated intracranial pressure

Symptoms

The primary symptom of amaurosis is the complete or substantial loss of vision. Depending on the underlying cause, additional symptoms may accompany the vision loss:

Sudden, painless loss of vision (typical in vascular causes)
Blurred or hazy vision preceding complete blindness
Pain in or behind the eye (e.g., in optic neuritis)
Flashing lights or visual disturbances (photopsias)
In amaurosis fugax: a brief curtain-like darkening of the visual field that resolves within minutes

Diagnosis

Diagnosing amaurosis requires a thorough ophthalmological and neurological evaluation:

Ophthalmoscopy: Examination of the fundus to assess the retina and optic disc
Visual evoked potentials (VEP): Measurement of signal conduction along the optic nerve
Fluorescein angiography: Imaging of retinal blood vessels
Magnetic resonance imaging (MRI): Assessment of the optic nerve and visual pathways in the brain
Electroretinogram (ERG): Evaluation of retinal electrical activity, especially for hereditary dystrophies
Laboratory tests and Doppler ultrasound: Used when a vascular cause is suspected

Treatment

Treatment of amaurosis depends on the underlying cause:

Vascular amaurosis: Emergency interventions (e.g., thrombolysis, ocular massage, reduction of intraocular pressure) and management of cardiovascular risk factors
Optic neuritis: High-dose corticosteroid therapy to reduce inflammation
Leber congenital amaurosis: Gene therapy (e.g., Voretigene Neparvovec/Luxturna for RPE65 mutations) is approved for certain patients
Toxic amaurosis: Detoxification and discontinuation of the causative substance
Tumor-related compression: Surgical removal or radiation therapy to relieve pressure on the optic nerve

Amaurosis fugax is considered a medical emergency, as it is often a warning sign of an impending stroke. Immediate neurological evaluation is essential.

References

Grehn, F. - Augenheilkunde. 32nd edition. Springer Medizin Verlag, 2019.
Hayreh, S.S. - Acute Retinal Arterial Occlusive Disorders. Progress in Retinal and Eye Research, 2011. PubMed PMID: 21620997.
Weleber, R.G. et al. - Leber Congenital Amaurosis. GeneReviews, NCBI Bookshelf, 2022. https://www.ncbi.nlm.nih.gov/books/NBK1298/

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