|Year : 2018 | Volume
| Issue : 3 | Page : 175-178
Bilateral cortical blindness with Anton–Babinski syndrome in an elderly Nigerian woman: Challenges for tertiary prevention
Obehi Aituaje Akoria1, Francis Ikechukwu Enebe2
1 Geriatrics Unit, Department of Medicine, University of Benin Teaching Hospital, Benin City, Nigeria
2 Department of Medicine, University of Benin Teaching Hospital, Benin City, Nigeria
|Date of Web Publication||4-Oct-2018|
Dr. Obehi Aituaje Akoria
Geriatrics Unit, Department of Medicine, University of Benin Teaching Hospital, P. O. Box 5212, Benin City
Anton–Babinski syndrome (Anton's syndrome) is well described in the scientific literature even though it is a rare neurological condition. Most publications have highlighted the anatomy, neurophysiology, and pathology of visual anosognosia, which is the hallmark of the syndrome. We are not aware of any published report of cortical blindness with Anton's syndrome from Africa. We report a catastrophic complication of severe hypertension in an elderly Nigerian woman who was on follow-up for stroke, chronic heart failure, diabetes mellitus, and glaucoma. She developed bilateral cortical blindness with Anton's syndrome as a complication of severe hypertension, following 3 weeks of missed medications. This report highlights some challenges of tertiary prevention in this elderly woman without health insurance, who before becoming blind, had been largely dependent on family members' goodwill for her medical care.
Keywords: Anton's syndrome, cortical blindness, elderly, Nigeria, tertiary prevention
|How to cite this article:|
Akoria OA, Enebe FI. Bilateral cortical blindness with Anton–Babinski syndrome in an elderly Nigerian woman: Challenges for tertiary prevention. Sahel Med J 2018;21:175-8
|How to cite this URL:|
Akoria OA, Enebe FI. Bilateral cortical blindness with Anton–Babinski syndrome in an elderly Nigerian woman: Challenges for tertiary prevention. Sahel Med J [serial online] 2018 [cited 2018 Dec 14];21:175-8. Available from: http://www.smjonline.org/text.asp?2018/21/3/175/242744
| Introduction|| |
Anton's syndrome is bilateral cortical blindness with visual anosognosia and visual confabulation. The first published description of someone who was blind but denied it was that of a nobleman reported by Montaigne in the 16th century. Gabriel Anton's description of the syndrome was published in 1899 with descriptions of three patients who had visual, hearing, and motor impairments, respectively, but who denied these deficits,, Joseph François Babinski in 1914 named this phenomenon of blind hemiplegic patients who were unaware of their blindness, “anosognosia.” Although the syndrome has been named after Anton and Babinski, it had earlier been described by Wernicke in 1874 and by Westphal in 1882.
Clinical diagnosis of Anton's syndrome is based on five criteria: (i) failure to blink in response to threat, (ii) loss of light and dark visual impulses, (iii) preservation of pupillary and accommodation reflexes, (iv) normal fundi, and (v) preserved extraocular movements. The absence of global cognitive deficits was central in Anton's original description of the syndrome.
Hypoperfusion of the occipital cortex is the usual cause of cortical blindness. This commonly results from ischemic vascular injury but could also result from hemorrhage and various other causes, including advanced glaucoma. Cerebrovascular disease is the most common underlying etiology that is reported in association with Anton's syndrome.
Damage to the visual association cortex (Brodmann area 18 and 19) and the primary visual cortex (Brodmann area 17) is responsible for cortical blindness with Anton's syndrome. The former is believed to be responsible for patients' unawareness of their visual impairment. Other authors suggest that parietal rather than occipital injury may be responsible for the disconnection that results in visual anosognosia. Recovery of visual function occurs in some cases and may be total or partial. Complete recovery has been reported in association with hypertensive encephalopathy, cerebral hypoperfusion, and following treatment with amantadine.
We present an elderly Nigerian woman (OP) with previous stroke, chronic stable cardiac failure, diabetes mellitus, and glaucoma, who defaulted in adherence to antihypertensives and suffered a catastrophic complication of severe hypertension – bilateral cortical blindness, with Anton's syndrome. Written informed consent to publish this case report was obtained from the patient's adult daughter.
| Case Report|| |
OP is a 64-year-old right-handed female Nigerian, a petty trader with primary school level of education, who had been on follow-up at the University of Benin Teaching Hospital's Geriatric Outpatient Clinic for stable chronic heart failure (New York Heart Association Class I) and stroke, both complications of hypertension. She also has type 2 diabetes mellitus and glaucoma and had been referred to the ophthalmologist.
At a routine clinic visit recently, her blood pressure which was previously controlled was 240/140 mmHg in both supine and erect positions; random blood glucose was normal. Her adherence to prescribed medications in the 3 weeks preceding this clinic visit had been poor due to lack of funds to purchase medicines. When she and her accompanying relative were informed about the dangers of her severely elevated blood pressure and offered hospital admission, they declined despite concerted persuasion. They, however, agreed to purchase a single dose of OP's usual oral antihypertensives (all that they could afford), which OP took before their leaving the clinic.
Three days later, OP presented at the medical emergency room with a 4-h history of inability to walk and expressive dysphasia. The accompanying relatives also gave a history of blindness in both eyes. There was no alteration in her mental status. General, chest and abdominal examinations were normal. Blood pressure was 170/100 mmHg with a laterally displaced and heaving apex beat. Glasgow Coma Score was 14 and both optic discs were blurred. There was left-sided hemiparesis, with hyperreflexia (from a stroke 8 years prior) and new onset right hemineglect. She denied being blind despite giving wrong answers to questions like “what is the color of my shirt?”
A subsequent review by the ophthalmologist confirmed cortical blindness. Her comprehensive geriatric assessment required further input from the cardiologist, geriatrician, medical social worker, neurologist, occupational therapist, and physical therapist.
Cranial computerized tomography scan revealed acute bilateral occipital infarcts with extensions into the parietal and temporal lobes [Figure 1] and [Figure 2].
|Figure 1: Axial contrast-enhanced brain computed tomography scan showing (a) old lacunar infarct of the left basal ganglia and (b and c) extensive infarcts involving right parieto-occipital and left occipital lobes|
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|Figure 2: Axial contrast-enhanced brain computed tomography scan showing (a) extensive left occipital lobe infarct and (b) right temporal lobe infarct|
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Baseline geriatric assessments revealed a frail woman (Fried's score 4/5), with functional impairment (modified Barthel score of 40%) and malnutrition (Mini Nutritional Assessment score 7/14). She was not depressed: Geriatric Depression Scale score (short form) was 1/15.
She was treated with 0.9% saline infusion and intravenous mannitol initially; soluble aspirin was added at 150 mg orally daily after the brain computed tomography excluded cerebral hemorrhage. She also had other medications for blood pressure, diabetes, and heart disease. By the 4th day of admission, OP and her relatives requested for discharge, but this was declined until a family meeting (with OP's children and members of the multidisciplinary geriatric team) was held to discuss strategies for OP's rehabilitation and continuing care (tertiary prevention).
| Discussion|| |
OP requires focused, multidisciplinary care with family participation. Broader social and societal interventions will also be required to mitigate the myriad complications that could arise from her recent blindness.
Anton's syndrome occurring in association with bilateral cortical blindness presents special challenges because of the risks posed to patients and others when patients who are in denial of their visual impairments engage in activities that could cause themselves or others harm.
A patient living in Nigeria, especially one who is elderly, frail and blind but insists that she sees and vividly (but incorrectly) describes what she sees could be labeled as having psychiatric disease. Worse still, she may be accused of witchcraft, with dire personal consequences. In either case, OP may be stigmatized, and this would come with further negative health consequences.
Immediate fallouts of OP's visual loss would include enforced immobility, which if unchecked could result in worsening frailty and sarcopenia, worsening functional status, worsening malnutrition, as well as incontinence, decubitus ulcers, and depression, to name a few.
OP will require nutritional, occupational, and physical rehabilitation, in addition to her routine medical care. She will also require more family support than she got before her recent blindness – emotionally, financially, physically, and psychologically. These have implications for OP and her caregivers, including the risk of caregiver burden and elder mistreatment.
Judging by her medical and social history and the current economic, infrastructural, and health-care climate in Nigeria, OP's visual and functional impairment may result in disability: inability to perform basic and instrumental activities of daily living, for a start. Depending on how her visual and functional impairments are perceived by family and society and depending on what environmental modifications are possible or not, OP may also experience handicap. However, according to the World Health Organization, anatomical, physical, or psychological impairment needs not result in disability or in handicap.
In the ideal world, OP would be supported to maintain an independent, functional status in spite of her visual impairment and comorbidities. However, in Nigeria, the likes of OP pay out of pocket for their entire health care, and social support is practically nonexistent. The support that OP got from her family before her becoming blind with Anton's syndrome was less than desirable. We can only hope that our efforts to institute appropriate tertiary preventative interventions (in spite of many limitations) will yield the desired result of a worthwhile quality of life for OP, despite her visual and functional impairment.
Adherence to pharmacological and other interventions (secondary prevention) would have been less costly for OP and her family. The saying “an ounce of prevention is worth a pound of cure” is certainly apt in this case.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]