• Ellis S van Etten, M Edip Gurol, Jeroen van der Grond, Joost Haan, Anand Viswanathan, Kristin M Schwab, Alison M Ayres, Ale Algra, Jonathan Rosand, Mark A van Buchem, Gisela M Terwindt, Steven M Greenberg, and Marieke J H Wermer.
• From the Departments of Neurology (E.S.v.E., J.H., G.M.T., M.J.H.W.), Radiology (J.v.d.G., M.A.v.B.), and Clinical Epidemiology (A.A.), Leiden University Medical Center; Department of Neurology (J.H.), Alrijne Hospital; Department of Neurology and Neurosurgery (A.A.), Brain Center Rudolf Magnus and Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, the Netherlands; Hemorrhagic Stroke Research Program (M.E.G., A.V., K.M.S., A.M.A., S.M.G.), Department of Neurology, Massachusetts General Hospital Stroke Research Center; and Division of Neurocritical Care and Emergency Neurology (J.R.), Massachusetts General Hospital, Harvard Medical School, Boston. e.s.van_etten@lumc.nl.
• Neurology. 2016 Oct 4; 87 (14): 1482-1487.

ObjectiveTo determine whether hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D), a monogenetic disease model for the sporadic variant of amyloid angiopathy (sCAA), has a comparable recurrent intracerebral hemorrhage (ICH) risk and mortality after a first symptomatic ICH.MethodsWe included patients with HCHWA-D from the Leiden University Medical Center and patients with sCAA from the Massachusetts General Hospital in a cohort study. Baseline characteristics, hemorrhage recurrence, and short- and long-term mortality were compared. Hazard ratios (HRs) adjusted for age and sex were calculated with Cox regression analyses.ResultsWe included 58 patients with HCHWA-D and 316 patients with sCAA. Patients with HCHWA-D had fewer cardiovascular risk factors (≥1 risk factor 24% vs 70% in sCAA) and were younger at the time of presenting hemorrhage (mean age 54 vs 72 years in sCAA). Eight patients (14%) with HCHWA-D and 46 patients (15%) with sCAA died before 90 days. During a mean follow-up time of 5 ± 4 years (total 1,550 person-years), the incidence rate of recurrent ICH in patients with HCHWA-D was 20.9 vs 8.9 per 100 person-years in sCAA. Patients with HCHWA-D had a long-term mortality of 8.2 vs 8.4 per 100 person-years in patients with sCAA. After adjustments, patients with HCHWA-D had a higher risk of recurrent ICH (HR 2.8; 95% confidence interval 1.6-4.9; p < 0.001) and a higher long-term mortality (HR 2.8; 95% confidence interval 1.5-5.2; p = 0.001).ConclusionsPatients with HCHWA-D have worse long-term prognosis after a first ICH than patients with sCAA. The absence of cardiovascular risk factors in most patients with HCHWA-D suggests that vascular amyloid is responsible for the recurrent hemorrhages. HCHWA-D is therefore a pure form of cerebral amyloid angiopathy with an accelerated clinical course and provides a good model to study the pathophysiology and future therapeutic interventions of amyloid-related hemorrhages.© 2016 American Academy of Neurology.

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