Palak Kodan(1), B. Optom

Roshni Sengupta(2), Assistant Professor(2)

GD Goenka University, Gurugram, Haryana, India

 

Anton Syndrome, also known as Anton–Babinski Syndrome (ABS), is a rare neurological condition that arises due to damage to the visual cortex. The hallmark feature of this syndrome is cortical blindness accompanied by a complete denial of visual loss. To compensate for their lack of vision, the brain generates false visual experiences or memories, a phenomenon termed confabulation, which the patient presents as real without any awareness of fabrication them. (1)

Cortical Blindness (CB) refers to loss of vision in the absence of any ophthalmic abnormality, characterised by preserved pupillary light reflexes. It results from bilateral damage to the striate cortex located in the occipital lobes and is classified under cerebral blindness, where visual loss occurs due to injury to visual pathways posterior to the lateral geniculate nuclei. (2)

Feature Cortical Blindness Anton Syndrome
Definition Loss of vision due to bilateral occipital lobe damage with awareness of visual loss. A subtype of cortical blindness characterised by denial of visual loss.
Cause Stroke, hypoxic injury, trauma, infection, or metabolic causes affecting the occipital cortex. Same causes as cortical blindness with additional impairment of insight.
Vision Status Complete or partial vision loss. Complete or partial vision loss.
Awareness of Blindness Typically aware of visual impairment. No awareness; patient insists vision is intact.
Confabulation Absent. Present; fabricated visual descriptions.
Neural Pathology Damage limited to bilateral occipital cortex. Occipital damage with disconnection involving insight-related pathways.
Associated Signs Visual field defects, disorientation, hallucinations (rare). Unsafe ambulation, confabulation, inability to navigate surroundings.

Table 1: Author-constructed Comparison Chart: Cortical Blindness vs Anton Syndrome

Blindness vs Belief

Some individuals with cortical blindness demonstrate an ability to respond accurately during forced-choice visual tasks despite denying visual perception. This phenomenon, termed “blindsight,” represents unconscious visual processing occurring without perceptual awareness. (3) Patients with ABS continue to deny their blindness even when repeatedly colliding with objects or describing environments and people that do not exist. (4)

Unconscious processing of facial attributes has also been documented. Individuals with prosopagnosia, despite being unable to recognise faces consciously, may demonstrate altered autonomic responses when exposed to familiar versus unfamiliar faces. (3)

The “Why” Behind the Denial: Anosognosia

Visual anosognosia, or denial of blindness, is a defining feature of ABS and commonly results from bilateral occipital strokes. (5) While ABS is not typically associated with global cognitive impairment, the posterior cerebral artery supplies regions involved in memory, recognition, and visual processing.

Damage extending to these areas, such as the fusiform gyrus, can result in deficits in executive functioning and memory. (6) Furthermore, ABS cases involving blindness from damage to pre-cortical visual pathways are often complicated by coexisting prefrontal lesions, leading to behavioral and cognitive impairments. (7) Notably, the primary motor and sensory pathways often remain unaffected despite significant brain damage. (6)

Seeing Beyond Denial: Diagnosis and Prognosis

ABS results from injury, typically bilateral ischemic stroke, to the occipital lobes (visual cortex), while preserving the anterior visual pathways. (8) Cerebrovascular disease is the primary cause, though any condition leading to cortical blindness can induce ABS. Prognosis is contingent on the underlying cause, with recovery being less likely after occipital infarction. (9) Management, therefore, focuses on secondary prevention and rehabilitation. While there is no specific cure, a multidisciplinary approach including medical, rehabilitative, and psychological support is crucial for improving the affected individual’s quality of life and helping them navigate daily challenges. Regular follow-up is essential

Conclusion

ABS is an uncommon disorder characterised by the coexistence of cortical blindness and a denial of visual impairment. Individuals affected by this condition inadvertently generate visual confabulations, typically resulting from bilateral damage to the occipital lobes, which complicates the processes of diagnosis, recovery, and management.

References

  1. Das, J. M., & Naqvi, I. A. (2023). Anton syndrome. StatPearls. StatPearls Publishing.
  2. Island, T. (2023). StatPearls Publishing; PMID.
  3. Solcà, M., et al. (2015). Facial blindsight. Frontiers in Human Neuroscience, 9, 522.
  4. Ziaul, Y. H., et al. (2024). Anton–Babinski syndrome. Cureus, 16(3).
  5. Zukić, S., et al. (2014). Anton’s syndrome due to bilateral occipital stroke. Case Reports in Neurological Medicine.
  6. Chaudhry, F. B., et al. (2019). Anton’s syndrome. BMJ Case Reports, 12(12).
  7. Michel, M., et al. (2024). Visual anosognosia. Trends in Cognitive Sciences.
  8. Mankoo, D. (2023). Anton syndrome with bilateral occipital infarct. Radiology Case Reports, 18(12).
  9. Maddula, M., et al. (2009). Anton’s syndrome due to cerebrovascular disease. Journal of Medical Case Reports, 3(1).

 

About the Author

 
 

Palak Kodan

B. Optom Student; Assistant Professor
GD Goenka University, Gurugram, Haryana, India

 

 

Roshni Sengupta

B. Optom Student; Assistant Professor
GD Goenka University, Gurugram, Haryana, India