Anton syndrome as a result of MS exacerbation

Abstract

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...

Anton syndrome as a result of MS exacerbation

“…Preoperative evaluation for epilepsy surgery: Process improvement Nitin K. Sethi, MD: I read with interest the results of the Drees et al 1 study and agree with the process improvements highlighted to reduce the time for epilepsy surgery (ES) evaluation. My personal experience working in a level IV comprehensive epilepsy center has been as follows.…”

“…Nitin K. Sethi, MD: I read with interest the case report by Kim et al 1 detailing Anton syndrome (AS) in a patient with multiple sclerosis. Apart from visual anosognosia, confabulation is an important symptom of AS.…”

“…Author Responds: Nina H. Kim, MD: We thank Dr. Sethi for his interest in our case report. 1 Cortical blindness can be ascertained by the examinations that Dr. Sethi highlights. It is also important that the patient is able to speak (and confabulate) to determine the diagnosis.…”

“…This analysis was performed in an effort to demonstrate that the MS specialist, often undervalued, contributes substantially to the financial well-being of the institution with which he or she is associated. 1 These data provide a framework for physicians providing MS care to negotiate with hospital administrators to provide adequate resources for their programs similar to that provided for cancer care and neurosurgery in light of their well-recognized financial contributions.…”

“…Authors Respond: Cornelia Drees, MD; Stefan Sillau, PhD; Mesha-Gay Brown, MD; Aviva Abosch, MD, PhD: We agree with Dr. Sethi's observations regarding our article. 1 An epilepsy provider who manages the patient was crucial for the surgical workup, but he or she may not be aware of which tests have been accomplished until the next office visit. In our institution, a nurse navigator was key to a streamlined processing of patients, actively facilitating testing, and keeping track of what tests had been scheduled and performed.…”