Atypical Presentations of Progressive Multifocal Leukoencephalopathy (PML): Aseptic Meningitis and Anton's Syndrome

Burns, Bruce T.; Katner, Harold; Couch, D.A.; Bhutta, Tayyab; Stephens, Jeffrey W.

doi:10.1089/apc.1997.11.71

Cited by 3 publications

(1 citation statement)

References 6 publications

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“…Anton syndrome has been associated with bilateral or non‐dominant parieto‐occipital lesions that spare pathways anterior to the visual primary cortex [4–6]. Anton syndrome has been most frequently described in the context of ischemic lesions, but has also been reported in other conditions [7–9].…”

Section: Discussionmentioning

confidence: 99%

Anton‐Babinski syndrome in a child with early‐stage adrenoleukodystrophy

Trifiletti

Syed

Hayes-Rosen

et al. 2007

Euro J of Neurology

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Section: Discussionmentioning

confidence: 99%

Anton‐Babinski syndrome in a child with early‐stage adrenoleukodystrophy

Trifiletti

Syed

Hayes-Rosen

et al. 2007

Euro J of Neurology

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Insights into Anton Syndrome: When the brain denies blindness

Atallah,

Badary,

Almealawy

et al. 2024

Journal of Clinical Neuroscience

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Anton syndrome as a result of MS exacerbation

2017

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isual anosognosia is the lack of awareness and denial of one's blindness. It is often referred to as Anton syndrome (AS), after one of the physicians who described the phenomenon of lacking insight into one's sensory deficit. There are limited numbers of case reports documenting the condition and many refer to the phenomenon in association with bilateral occipital infarcts. We describe a patient who developed visual anosognosia as a result of multiple sclerosis (MS). Case report A 24-year-old left-handed woman presented with right-sided hemiparesis and homonymous hemianopia. Head CT showed a hypodensity spanning the left temporo-parieto-occipital white matter. Brain MRI demonstrated a subcortical lesion with high T2/fluid-attenuated inversion recovery (FLAIR) signal, diffusion restriction, areas of apparent diffusion coefficient (ADC) dropout, no contrast enhancement, and mild mass effect on the lateral ventricle (figure, A). Serologic investigation (erythrocyte sedimentation rate, C-reactive protein, SS-A/B, antineutrophil cytoplasmic antibodies, antinuclear antibodies, angiotensin-converting enzyme, rheumatoid factor, aquaporin-4 antibodies, HIV, hepatitis A, B, and C, JC virus, cytomegalovirus, Epstein-Barr virus, human T-cell lymphotropic virus) was unremarkable. CSF glucose, protein, and cell counts were normal. CSF immunoglobulin G was elevated (.6 mg/dL) and isoelectric focusing reported presence of oligoclonal bands (.2 unique bands) in CSF with absence in serum. The patient was treated with IV methylprednisolone due to the high suspicion of a demyelinating process. She made gradual, incomplete recovery. Follow-up MRI scans performed over the course of 1 year began to show small, asymptomatic juxtacortical and periventricular lesions in addition to one infratentorial pontine lesion (figure, B-D). The initial left-sided tumefactive lesion remained stable with ex vacuo dilation of the left lateral horn adjacent to gliosis in the left hemisphere. Sixteen months after the first attack, the patient presented with global aphasia, left-sided sensorimotor deficiency, and cortical blindness. Brain MRI showed a new right-sided parieto-occipital white matter lesion with T2/FLAIR hyperintensity, restricted diffusion, dropout on ADC, and no contrast enhancement (figure, E). MRI of the total spine did not show any additional lesions. Laboratory studies were again unrevealing. The patient met the revised MacDonald criteria for MS with her 2 attacks and numerous T2 lesions throughout time and space. 1 Due to the aggressive nature of the patient's tumefactive relapsing-remitting MS, she was treated with methylprednisolone and plasmapheresis, and promptly started on alemtuzumab. All her symptoms improved over months except for cortical blindness. Despite being unable to detect light or blink to threat, the patient insisted that her vision was intact and confabulated the presence and placement of objects when asked about her surroundings. Her vision recovered over 2 years; first she gained insight that she was blind, then w...

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Atypical Presentations of Progressive Multifocal Leukoencephalopathy (PML): Aseptic Meningitis and Anton's Syndrome

Cited by 3 publications

References 6 publications

Anton‐Babinski syndrome in a child with early‐stage adrenoleukodystrophy

Anton‐Babinski syndrome in a child with early‐stage adrenoleukodystrophy

Insights into Anton Syndrome: When the brain denies blindness

Anton syndrome as a result of MS exacerbation

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