Flemming, Kelly D.

Background and Purpose— Moyamoya disease is an uncommon, cerebrovascular occlusive disease of unknown pathogenesis. Previously described Moyamoya cohorts include predominantly Asian populations or ethnically diverse North American cohorts. To gain further insight into the pathogenesis of moyamoya, we examined clinical characteristics of a primarily white, Midwestern US population Methods— Retrospective analysis of patients with angiographically confirmed moyamoya disease evaluated at our institution was performed. Prevalence of comorbidities, cerebrovascular risk factors, and autoimmune diseases were compared with the general population. Results— Ninety-four patients with moyamoya were evaluated; 72.3% were female. Ethnic composition was primarily white (85%). A significantly higher prevalence of autoimmune disease was seen, particularly type 1 diabetes mellitus (8.5% versus 0.4% in the general population) and thyroid disease (17.0% versus 8.0% in the institutional general patient population). Hyperlipidemia was also increased (27.7% versus 16.3% in the general population). Conclusions— This study of a unique, primarily white, Midwestern population of moyamoya patients demonstrates a significantly higher prevalence of autoimmune disease than in the general population. This supports a possible autoimmune component to the pathogenesis of moyamoya disease.

Object The goal of this study was to determine the risk of using antithrombotic agents in patients with established intracerebral cavernous malformations (ICMs). Methods From a previously described cohort of 292 patients with radiographically defined ICMs, 40 required an antithrombotic after the ICM was diagnosed. Patients underwent follow-up to determine the incidence of hemorrhage. Results The mean age of these 40 patients was 62.4 years; there were 21 male and 19 female patients. Five (12.5%) of the 40 patients initially presented with hemorrhage and 4 (10%) had multiple ICMs. Of these patients, 32 were placed on an antiplatelet agent alone, 6 on an anticoagulant alone, and 2 were placed on both. In patients necessitating any antithrombotic agent, 1 patient developed a prospective hemorrhage over the 258 person-years of follow-up (prospective hemorrhage rate 0.41% per person-year). Conclusions Antithrombotics likely do not precipitate hemorrhage in patients with known ICMs. However, caution should be exercised in the use of antithrombotics in patients with ICMs at high risk for hemorrhage. The risks and benefits of antithrombotics in each situation should be carefully weighed against the natural history of ICM.

Background and Purpose— Postpartum angiopathy (PPA), a rare cause of stroke in the puerperium, is heralded by severe headaches within 1–2 weeks after delivery. Angiography demonstrates segmental vasoconstriction that often resolves spontaneously. PPA is generally regarded as benign. We aimed to define clinical presentations, radiological findings, and outcomes of patients with PPA. Methods— We retrospectively reviewed patients from 3 centers with acute neurological symptoms and angiography showing vasoconstriction in the postpartum period. Patients without neuroimaging and with diagnoses of cerebral venous sinus thrombosis and aneurysmal hemorrhage were excluded. Patient characteristics, clinical symptoms, neuroimaging findings, and clinical condition at hospital discharge were collected. Results— Eighteen patients (mean age, 31 years; range, 15–41) were identified. Median gestation was 38 weeks. Twelve (67%) had a history of prior uneventful pregnancy. Neurological symptoms began on median day 5 postpartum and included headache (n=16, 89%), focal deficit (n=9, 50%), visual disturbance (n=8, 44%), encephalopathy (n=6, 33%), and seizure (n=5, 28%), often in combination. Brain imaging was abnormal in most (n=13, 72%). The most common abnormalities were intracranial hemorrhage (n=7, 39%), vasogenic edema (n=6, 35%), and infarction (n=6, 35%). Clinical outcomes were markedly variable with full recovery seen in 9 (50%), death after a fulminant course in 4 (22%), and residual deficits in 5 (28%). Conclusions— In contrast to prior reports, this group of patients with PPA had a higher proportion of nonbenign outcomes. Most patients who undergo neuroimaging have parenchymal abnormalities, which are most often stroke (hemorrhagic or ischemic) or reversible vasogenic edema.

The risk of hemorrhage from an intracerebral cavernous malformation has been estimated at 2%–4% per year. In patients with multiple cavernous malformations, typically there are 1 or 2 dominant lesions that result in symptoms. This report highlights an unusual case of recurrent hemorrhage from de novo cavernous malformations. This 35-year-old man had a generalized seizure in 2007. Magnetic resonance imaging performed at the time showed multiple hemorrhagic lesions suggestive of cavernous malformations. Two years later, the patient had clinical symptoms referable to a midbrain hemorrhage. This lesion was not present on 2007 standard and gradient echo images. One year later, the patient had another clinical hemorrhage at the cervical medullary junction. This lesion was also not present on earlier imaging. Genetic testing was negative for the known familial types of cavernous malformation. A lesion was biopsied to ensure correct diagnosis, and the results were pathologically consistent with a cavernous malformation. The patient had a fourth clinical hemorrhage in 2011 from a separate lesion. All hemorrhage symptoms were mild, and he returned to normal functioning and work after each hemorrhage. This case highlights several unusual features of the known natural history of intracerebral cavernous malformations. In this case, resection of the hemorrhagic lesion would not have altered future hemorrhage risk since each new hemorrhage was from a de novo lesion.