Transient Focal Neurological Episodes with Subsequent Catastrophic Intracerebral Hemorrhage Due To Cortical Superficial Siderosis and Amyloid Angiopathy (P1.233)

A 74 year-old, right handed man was admitted after experiencing a 15 minute episode of expressive aphasia, and recent complaints of intermittent left hand, arm, leg and face dysaesthesias. Recent outpatient neurological workup for dysaesthesias included a non-contrast MRI that demonstrated abnormal susceptibility in the right frontal and parietal sulci compatible with areas of old subarachnoid hemorrhage. He had a history of back injuries due to several motorcycle accidents. He was normotensive with a normal neurologic exam on admission. MRI brain demonstrated a punctuate area of restricted diffusion in the left sylvian fissure representing an acute stroke. He was discharged on aspirin and clopidogrel after a negative stroke work up as per recent guidelines. After two days, he was brought unresponsive with agonal breathing to the emergency department after sudden onset of headache with vomiting. CT head revealed an acute right temporal lobe intraparenchymal hemorrhage and a right subdural hemorrhage. A 2.4 cm leftward midline shift, uncal and subfalcine herniations and hydrocephalus was noted. He underwent an emergent right craniectomy and external ventricular drain placement. He failed to improve and his family opted for palliative care; he subsequently expired. A specimen from the right temporal lobe was consistent with scattered amyloid plaques and amyloid angiopathy, consistent with a definitive diagnosis of cerebral amyloid angiopathy (CAA). Transient focal neurologic episodes (TFNE) that occur in CAA have been reported to be associated with cortical superficial siderosis (cSS) on MRI. The presence of cSS has also been associated with an increased risk of future symptomatic lobar ICH. Our patient had evidence of cSS on brain MRI, however it was presumed to be from prior traumatic subarachnoid hemorrhages. Our case represents a catastrophic outcome by treating an acute ischemic stroke with anti-platelet agents in the setting of TFNE with cSS.