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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 4  |  Issue : 3  |  Page : 109-112

A middle adulthood arabic female suffering from Anton–Babinski syndrome


1 Department of Critical Care, King Saud Medical City, Riyadh, KSA
2 Department of Critical Care, King Saud Medical City, Riyadh, KSA; Department of Anesthesia, Faculty of Medicine, Tanta University, Tanta, Egypt
3 Department of Critical Care, King Saud Medical City, Riyadh, KSA; Department of Critical Care, Keck Medical School, USC, Los Angeles, CA, USA

Date of Submission23-Jun-2020
Date of Decision03-Aug-2020
Date of Acceptance14-Aug-2020
Date of Web Publication30-Sep-2020

Correspondence Address:
Waleed Tharwat Aletreby
Department of Critical Care, King Saud Medical City, Riyadh
KSA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sccj.sccj_26_20

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  Abstract 


Anton–Babinski (AB) syndrome is a form of cortical blindness in which patients are unaware of their blindness despite the definite signs of vision loss. Moreover, patients insist on their ability to see, repeatedly denying their vision loss. This denial of vision loss is a form of visual anosognosia and a hallmark of this syndrome. Although rare, AB syndrome has been well described in literature; however, to our knowledge, there have been no published case reports of patients with AB syndrome in the Middle East. This could be because of the rarity of the diseases or simply because of underdiagnosis of the condition. Here, we report the case of an Arabic female with AB syndrome in order to highlight the importance of physical examination skills in intensive care unit settings where sophisticated observation technology abounds. This case demonstrates how a thorough clinical history and neurological examination are essential in detecting this critical diagnosis early in the course of disease.

Keywords: Anton babinski, blindness, case report


How to cite this article:
Mohammed Noor AS, Alshaya R, Alharthy AM, Madi AF, Aletreby WT, Karakitsos D. A middle adulthood arabic female suffering from Anton–Babinski syndrome. Saudi Crit Care J 2020;4:109-12

How to cite this URL:
Mohammed Noor AS, Alshaya R, Alharthy AM, Madi AF, Aletreby WT, Karakitsos D. A middle adulthood arabic female suffering from Anton–Babinski syndrome. Saudi Crit Care J [serial online] 2020 [cited 2020 Oct 31];4:109-12. Available from: https://www.sccj-sa.org/text.asp?2020/4/3/109/296836




  Introduction Top


Visual anosognosia is a rare complication of cortical blindness in which patients are unaware of their visual deficits.[1] This lack of awareness has been attributed to damage to the visual association cortex (Brodmann areas 18 and 19) and the primary visual cortex (Brodmann area 17)[2],[3],[4] in the presence of a normal anterior visual pathway. Patients with Anton–Babinski (AB) syndrome exhibit visual anosognosia and confabulation, or the fabrication of memories of events or experiences that never took place. These patients, therefore, deny their vision loss using various explanations, such as poor lighting in a room, in the setting of obvious vision loss, and cortical blindness.[5]

In this report, we present the case of a 48-year-old female who developed AB syndrome after a posterior circulation stroke.


  Case Report Top


A 48-year-old Arab female with a past medical history of diabetes mellitus, hypertension, and hypothyroidism presented to the emergency department with recent-onset respiratory distress, mild hypoxia, and a decreased level of consciousness (Glasgow Coma Scale: 10/15). She was diagnosed with a community-acquired pneumonia and intubated, sedated, mechanically ventilated, and admitted to the intensive care unit. Notably, brain computed tomography (CT) imaging performed at admission revealed bilateral occipital lobe infarcts [Figure 1]. Forty-eight hours after admission, the patient was in a stable condition and was successfully weaned from mechanical ventilation and extubated. At this point, a thorough neurological examination revealed mild right-sided weakness, gait ataxia, and a notable lack of eye contact. A subsequent cranial nerve examination and medical interview revealed visual anosognosia, as the patient was found to be completely unaware of her vision loss. She was then transferred to the neurocritical care unit for further monitoring.
Figure 1: Brain computed tomography imaging at admission. Low attenuation is noted in the bilateral cerebellar hemispheres, centrum semiovale, and parietal deep periventricular areas. Furthermore, multiple tiny hypodensities are seen in both basal ganglia and thalami, as well as on the right side of the brainstem

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Subsequent brain magnetic resonance imaging revealed acute bilateral occipital infarcts and a left thalamic infarction with mild hemorrhage [Figure 2]. Magnetic resonance angiography (MRA) depicted a filling defect at the V4 segment of the right vertebral artery and bilateral periventricular microangiopathic changes [Figure 3]. She received appropriate stroke treatment and was discharged to home in an awake state, with neither residual limb motor weakness nor sensory involvement; however, she did experience persistent cortical blindness and was diagnosed with AB syndrome. Informed written consent was signed by the patient's next of kin to use her de-identified data in this case report.
Figure 2: Magnetic resonance imaging of the brain. Acute bilateral occipital and left thalamic infarction with some hemorrhagic components

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Figure 3: Magnetic resonance angiography. Filling defect at the V4 segment of the right vertebral artery (orange arrow)

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  Discussion Top


In this report, we have presented the case of a middle-aged Arabic female who experienced a posterior circulation stroke leading to cortical blindness and AB syndrome. The first historical description of AB syndrome likely originated from the writings of Seneca, a Roman philosopher, author, and mentor to Emperor Nero. In his 50th moral letter to Lacillus (63 CE), he described the case of Harpaste, his wife's slave, who became acutely blind and adamantly denied her illness, arguing irrationally about darkness in the room.[6] Monaknow, Rossolimo, and F. Muller [7] later described the phenomenon of cortical blindness,[8],[9],[10] and Gabriel Anton (1858–1933) and Francois Babinski (1857–1932) highlighted the occurrence of anosognosia in patients with preserved cognition. Babinski described anosognosia in hemiplegic patients,[11] and Anton delved into the psychology of the disease to develop a neuropathological theory.[12] He attributed patient unawareness of their deficits to external factors.[13],[14],[15]

Since then, multiple theories have been proposed to explain the pathophysiology of various forms of anosognosia, all of which share a common concept, called the “disconnection” phenomenon.[16] In this framework, AB syndrome involves damage to the association pathways that run between the visual cortex and the conscious awareness system, which is located in the parietal lobe and monitors all information received from the senses. This system connects with regions in the frontal lobe that integrate information in order to perform complex cognitive tasks. Damage to these association pathways is believed to cause the lack of awareness of visual deficits in AB syndrome.

The disconnection phenomenon also entails disconnection of the visual area from the language area, which leads to confabulation, as patients are not able to accurately describe visual stimuli. Finally, the disconnection phenomenon also involves overactivity of secondary visual pathways located in the superior colliculus, pulvinar, and temporoparietal regions.[17] This system usually sends impulses to the visual association cortex (the visual monitor). However, if there is an interruption of impulse transmission via the main pathway, these secondary visual pathways will gain predominance and result in confabulation. Therefore, the neuropsychological mechanism of this disease is explained by a defective visual monitor, leading to incorrect interpretation of images and the presence of false-positive feedback.

AB syndrome is diagnosed by excluding diseases of the anterior visual pathway, which necessitates a complete neuroophthalmological examination. The diagnosis requires five criteria, including loss of all visual sensation including light and dark perception, preservation of light and accommodation pupillary reflexes, loss of the menace reflex, a normal fundoscopic examination, and preservation of ocular movement.

An ischemic stroke in the posterior circulation is the most common cause of this syndrome. Anatomical variations in the posterior circulation are common and are usually asymptomatic; however, they may raise stroke risk and should be considered a potential determinant of stroke etiology. The posterior cerebral arteries (PCAs) cover two vascular territories through superficial and deep proximal branches. Superficial branches feed the occipital lobe and variable parts of the medial temporal and parietal lobes, while deep PCA branches feed the paramedian midbrain and the medial and posterolateral thalamus.[18] Posterior circulation strokes are one of the most commonly missed diagnoses in patients presenting with a variety of nonspecific symptoms. For example, in 407 patients with posterior circulation strokes in a New England Medical Center Posterior Circulation Registry study, the most common presenting symptoms included dizziness (47%), unilateral limb weakness (41%), dysarthria (31%), headache (28), and nausea and vomiting (27%). The most frequently observed signs were unilateral limb weakness (38%), gait ataxia (31%), unilateral limb ataxia (30%), dysarthria (28%), and nystagmus (24%). Most of these findings were nonspecific and presented in isolation.[19],[20]

Other rare causes of AB syndrome include mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes;[21] preeclampsia;[1] obstetric hemorrhages;[22] hypertensive encephalopathy;[23] angiographic procedures;[24] trauma;[25] adrenoleukodystrophy;[26] tuberculosis;[27] and hepatic encephalopathy.[28]

Regarding the prognosis, recovery of visual function has occurred in conditions causing AB syndrome, such as hypertensive encephalopathy and cortical hypoperfusion. In these conditions, correction of the causative factor may lead to resolution of symptoms. Other factors that can impact the outcome include age of the patient, duration, initial recovery time, and medical history.


  Conclusion Top


AB syndrome is a disease featuring a unique neuropsychiatric presentation of difficulty in establishing awareness of blindness, with evidence of occipital lobe damage. It is often overlooked because of its complexity and rarity. Emphasis should be made on proper ophthalmological evaluation, requesting proper investigation including CT brain and MRA, in addition to early involvement of stroke team, as early diagnosis and management can affect the outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given consent for images and other clinical information to be reported in the journal. The guardian understands that the patient's name and initial will not be published, and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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