Case Report: SMART ANTON: Anton-Babinski Syndrome in Stroke-Like Migraine Attacks (SMART) After Radiation Therapy: Two Rare Syndromes, One Case

Abstract

Introduction: We describe the case of a 57-years-old patient who presented an Anton-Babinski syndrome in the context of a stroke-like migraine attack after radiation therapy (SMART).

Case report: The patient was brought to the emergency room following a sudden loss of vision in the context of a pre-existing left-sided hemianopia after excision of a right occipital astrocytoma followed by radio-chemotherapy 35 years prior to his admission in our services. At admittance, he also presented hyperthermia, hypertension, and a GCS of 7. The MRI showed a leptomeningeal enhancement in the left temporal, parietal, and occipital lobes. After exclusion of other differential diagnoses, we diagnosed a cortical blindness in the context of a SMART syndrome affecting the left hemisphere. While the symptoms improved under corticosteroid therapy, the patient successively presented an Anton-Babinski syndrome, a Riddoch syndrome and a visual associative agnosia before finally regaining his usual sight.

Discussion: This is, to our knowledge, the first report of an Anton-Babinski syndrome in the context of a SMART syndrome. A dual etiology is mandatory for cortical blindness in SMART syndrome since the latter affects only one hemisphere. A SMART syndrome affecting the contralateral hemisphere in respect to the radiation site seems to be uncommon, which makes this case even more exceptional.

Keywords: Anton-Babinski syndrome; Riddoch syndrome; SMART syndrome; cortical blindness; dual etiology; visual associative agnosia.

Figure 1

Side to side comparison MRI flair day 1 (A), day 6 (B), day 14 (C) and day 45 (D). The follow-up MRIs shows a gradual reduction of the initially displayed leptomeningeal enhancement in the left temporal, parietal and occipital lobes. The substance defect after astrocytoma excision appears as a liquid-filled black cyst in the lower part of the right hemisphere (left-sided in the pictures).

Figure 2

(A) Visual perception recovery during hospitalization (right hemifield); (B) Visualization of the treatment with corticosteroids: Steroid pulse therapy with 1 g intravenous methylprednisolone during the first 3 days (striped pattern) followed by oral therapy with prednisolone 100mg gradually reduced over 9 weeks.