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The main clinical manifestations of Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) are migraine with aura, ischemic strokes, and progressive cognitive decline. Intracerebral hemorrhage (ICH) has been described in CADASIL, but is not widely recognized. Here we report a case with CADASIL that presented with fatal ICH. A 57-year-old right-handed man of Pakistani descent with history of genetically confirmed CADASIL, hypertension, and mood disorder presented to the emergency department via Emergency Medical Services (EMSs) after he was found down. Initial neurological examination showed a Glasgow Coma Scale (GCS) of 7 (E2, V1, M4), left gaze deviation, pinpoint pupils, and left hemiplegia. His medications included antihypertensive agents and aspirin. He was intubated in the emergency department due to inability to protect his airway. Computed tomographic scan of the head revealed acute hemorrhage in the right pons (ICH score 2) with extension into the right cerebral peduncle, as well as enlargement of the third and lateral ventricles suggesting early obstructive hydrocephalus that required an external ventricular drain placement. He had no improvement of his clinical status, and eventually extubation and comfort care were pursued. He died 6 days after presentation. CADASIL vasculopathy, cerebral microbleeds, hypertension, and antithrombotic agents are factors that could be related to ICH in patients with CADASIL. This case highlights the importance of adequate blood pressure control, magnetic resonance imaging assessment of cerebral microbleed, and careful discussion of the risk and benefits of antiplatelet agents when evaluating and treating patients with CADASIL.

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INTRODUCTION: Takayasu arteritis is a large-vessel vasculitis that may cause oculo-cerebral ischemia. We report a patient with visual loss as initial manifestation, and discuss transcranial Doppler (TCD) findings before and after surgical revascularization. CASE DESCRIPTION: A 19-year-old female developed episodes of transient positional right vision loss, progressing to permanent right vision loss and bright light-induced left amaurosis. Examination demonstrated subclavian bruits, palpable epigastrium aortic pulsation, faint radial pulses, right retinal pallor, arteriolar narrowing, and bilateral boxcarring. Head and neck computed tomography angiogram demonstrated left subclavian origin and right common carotid artery occlusion, and severe innominate and left common carotid artery stenosis. TCD demonstrated right ophthalmic artery flow reversal and nonpulsatile waveforms with dampened spectra in the right anterior circulation. Corticosteroids, methotrexate, infliximab, and dual-antiplatelet therapy were initiated. Eleven weeks later, the patient underwent ascending aortic aneurysm repair and bilateral carotid artery bypass with an aortic graft. Pathology was consistent with chronic active Takayasu arteritis. Two weeks postoperatively, left eye visual symptoms resolved; right visual loss persisted. Postoperative TCD showed marked improvement in cerebral perfusion. CONCLUSIONS: Retinal ischemia in young women should prompt emergent inflammatory and systemic vascular evaluation. In our subject, prolonged right retinal ischemia had dismal prognosis despite carotid-aortic revascularization, whereas left retinal boxcarring reversed. Surgical revascularization is recommended for severely symptomatic oculo-cerebrovascular disease, once inflammation is better controlled with immunosuppressive therapy. TCD documented the presence and monitored the subsequent resolution of blood flow changes causing retinal and brain hypoxia.

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BACKGROUND: Adults with cryptogenic stroke often undergo transesophageal echocardiogram (TEE) because this is the gold-standard for evaluation of potential proximal sources of emboli. The risks of performing this invasive test must be weighed against its clinical efficacy and limitations, determined by the rate of positive findings, and the impact on clinical outcome for the patient. OBJECTIVE: To critically appraise current evidence regarding the rate of positive TEE findings in cryptogenic stroke patients, and analyze the implications of these findings on management decisions and outcomes. METHODS: The objective was addressed through the development of a structured, critically appraised topic. We incorporated a clinical scenario, background information, a structured question, literature search strategy, critical appraisal, results, evidence summary, commentary, and bottom line conclusions. Participants included consultant and resident neurologists, a medical librarian, and vascular neurology and cardiology content experts. RESULTS: TEE commonly identifies abnormalities, including aorta atherosclerosis in 51%, patent foramen ovale in 43%, and atrial septal aneurysm in 13% of patients. Findings such as left-sided chamber thrombus or intracardiac tumor that definitively warrant a change in management by guideline-supported use of anticoagulation are less common, occurring in ∼3% of patients. CONCLUSIONS: TEE identifies potential causal sources of embolus in patients with cryptogenic stroke that leads to changes in management and outcomes at least 3% of the time. Other findings, particularly aorta atherosclerosis, are identified much more commonly but the causal link to stroke is uncertain, thus changes in management in these cases is variable and data describing resulting outcomes are lacking.

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INTRODUCTION: Major surgery in the past 14 days is a relative contraindication to treatment of acute stroke with intravenous (IV) alteplase. The 2016 American Heart Association/American Stroke Association scientific statement discussing inclusion and exclusion criteria for alteplase in acute stroke allows for provider judgment citing a lack of evidence to support surgery as an absolute contraindication. CASE REPORT: A 59-year-old woman presented with acute left hemiparesis, dysarthria, and acute respiratory failure. National Institutes of Health Stroke Scale was 17. Two days prior she underwent abdominoplasty and liposuction. Computed tomography angiogram of the head demonstrated acute occlusion of right M2, and computed tomography angiogram of the chest was positive for bilateral pulmonary emboli. After urgent consultation with the plastic surgery and neurosurgery teams, the patient was treated with IV alteplase followed by cerebral mechanical thrombectomy. This was complicated by surgical-site hemorrhage in the abdomen requiring operative intervention. Abdominal hemorrhage was controlled and the patient showed remarkable neurological recovery, later being discharged with no residual neurological deficits. CONCLUSIONS: Interdisciplinary collaboration can successfully inform the decision to treat acute ischemic stroke with IV alteplase in the setting of recent major surgery.

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Ramucirumab is an antiangiogenesis agent targeting the vascular endothelial growth factor receptor-2 (VEGFR-2), approved to treat advanced gastric and colon cancer. In clinical trials, it was shown to cause a small increase in arterial thromboembolism compared to placebo, including cerebral and myocardial ischemia, which was not statistically significant. Detailed case reports are lacking and we here present one of the first case reports of stroke secondary to ramucirumab-induced in situ thrombosis.