Dr. Sonali Gupta, G14389, Dr. Zia Chaudhuri, Dr. Kishore Govekar, Dr. Ajay Sharma
We report a 12-year female patient who presented with throbbing, right-sided headache and sudden diminution of visual acuity (VA) in both eyes (BE). The VA at presentation was ‘perception of light present’ with ‘projection of rays inaccurate’ that eventually progressed within a week to absent light perception BE with evidence of visual hallucinations. MR Angiography (MRA) showed Circle of Willis and bilateral posterior cerebral artery (PCA) vasculopathy with abnormal small lenticulostriate vessels, consistent with MMD. Visually evoked response (VER) was bilaterally absent. The vessels became progressively stenotic over a period of 2 years with no VA recovery. Steno-occlusive lesions in MMD usually begin from the terminal portion of internal carotid artery (ICA) or proximal portion of middle or anterior cerebral arteries. Bilateral cortical blindness suggesting early involvement of posterior circulation, presenting as the first feature of MMD in a child, makes this case unique.