Perfusion-weighted MRI in cerebral amyloid angiopathy-related transient focal neurological episodes.

INTRODUCTION: Sporadic cerebral amyloid angiopathy (CAA) is a common age-related cerebral small vessel disease characterized by progressive ß-amyloid deposition in the walls of small cortical arteries, arterioles, and capillaries in the cerebral cortex and overlying leptomeninges. CAA-related transient focal neurological episodes (CAA-TFNEs) represent a challenging clinical feature interesting from a pathophysiological point of view. CASE REPORT: Here we present two cases of CAA-TFNEs in which we performed functional imaging with perfusion-weighted imaging MR and brain 18 F-FDG PET. In both cases, we found a topographic relationship between the involved cortical areas and the clinical expression of CAA-TFNEs. Cortical superficial siderosis in the first case and a convexity subarachnoid hemorrhage in the second case were found in the contralateral rolandic area corresponding to the clinical symptoms. The same areas showed a reduction of rCBV and rCBF on perfusion-weighted MR and were also associated in one case with hypometabolism on 18 F-FDG PET. DISCUSSION: These new findings strengthen the hypothesis that CAA involves the superficial leptomeningeal arteries but also the short penetrating arterioles reaching different depths in the cortex generating hypoperfusion and altered vascular reactivity and consequently reduced neuronal activity. CONCLUSION: Understanding CAA-TFNEs is pivotal because they carry a very high risk of subsequent lobar intracerebral hemorrhage but are frequently misdiagnosed as TIAs and treated with antithrombotics enhancing the bleeding risk associated with CAA.

Reversible cerebral vasoconstriction syndrome and dissection in the setting of COVID-19 infection.

The current COVID-19 pandemic has recently brought to attention the myriad of neuro- logic sequelae associated with Coronavirus infection including the predilection for stroke, particularly in young patients. Reversible cerebral vasoconstriction syndrome (RCVS) is a well-described clinical syndrome leading to vasoconstriction in the intracra- nial vessels, and has been associated with convexity subarachnoid hemorrhage and oc- casionally cervical artery dissection. It is usually reported in the context of a trigger such as medications, recreational drugs, or the postpartum state; however, it has not been described in COVID-19 infection. We report a case of both cervical vertebral ar- tery dissection as well as convexity subarachnoid hemorrhage due to RCVS, in a pa- tient with COVID-19 infection and no other triggers.

Convexity Subarachnoid Hemorrhage Soon after Starting a Direct Oral Anticoagulant in 2 Patients with Acute Infarction.

Convexity subarachnoid hemorrhage (cSAH) is typically due to head trauma, but it rarely occurs subsequent to acute ischemic stroke. Direct oral anticoagulants (DOACs) have favorable bleeding profiles as compared with warfarin, and, to our knowledge, no DOAC has been regarded as a causative agent for cSAH. Here, we reported 2 patients with cSAH apparently caused by starting DOAC therapy. No hemorrhage had been evident just prior to treatment initiation, but cSAH occurred so soon after DOAC therapy began. Each of our patients had occlusion or severe stenosis of a major artery due to emboligenic disease, and cSAH occurred in the territory of the affected artery. Reperfusion and dynamic changes in perfusion pressure due may trigger cSAH. Clinicians should remain alert for cSAH when starting DOAC for treatment of embolic ischemic stroke during the acute phase.

Subarachnoid Hemorrhage with Progressive Cerebral Steno-Occlusive Disease: Report of 2 Cases.

Nontraumatic nonaneurysmal subarachnoid hemorrhage (SAH) is a rare condition. Among them, SAH with cerebral steno-occlusive disease is quite rare. Moreover there has been no report of SAH patient who had been diagnosed with steno-occlusive disease since before. We here report 2 cases of nontraumatic nonaneurysmal convexity SAH who originally had progressive cerebral steno-occlusive disease. Case 1, a woman in her 40s who had diagnosed left internal carotid artery (ICA) stenosis 6 years before complained of headache. She was diagnosed SAH and progressive ICA stenosis, then performed revascularization. Case 2, a woman in her 40s who had diagnosed right ICA stenosis 7 months before complained of headache. She was diagnosed with SAH and ICA occlusion. These 2 cases suggested that progressive cerebral steno-occlusive disease lead to SAH due to collapse of their fragile pial anastomoses.

The Spectrum of Nontraumatic Convexity Subarachnoid Hemorrhage.

BACKGROUND: Nontraumatic convexity subarachnoid hemorrhage (cSAH) is a nonaneurysmal variant that is associated with diverse etiologies. METHODS: With IRB approval, we retrospectively reviewed consecutive nontraumatic cSAH from July 1, 2006 to July 1, 2016. Data were abstracted on demographics, medical history, neuroimaging, etiology, and clinical presentation. RESULTS: We identified 94 cases of cSAH. The cases were classified according to the following etiologies: reversible cerebral vasoconstriction syndrome (RCVS) 17 (18%), cerebral amyloid angiopathy (CAA) 15 (16%), posterior reversible encephalopathy syndrome 16 (17%), cerebral venous thrombosis 10 (11%), large artery occlusion 7 (7%), endocarditis 6 (6%), and cryptogenic 25 (27%). Early rebleeding occurred in 9 (10%) patients. Time from initial imaging to CT rebleeding was 40 hours (range, 5-74). CAA was associated with the highest mean age at 75.8 and RCVS the lowest at 47.6 years (P< .0001). Among patients with RCVS, initial vascular imaging was negative in 6 (35%), and repeat imaging documented vasoconstriction at a mean delay of 5 days (range, 3-16). CONCLUSION: There were significant differences among the subgroups in cSAH, with CAA presenting as older men with transient neurological deficits, and RCVS presenting as younger women with thunderclap headache. Rebleeding was seen in 10% of cSAH patients. One-third of RCVS patients with cSAH required repeat vascular imaging to diagnose vasoconstriction.

Incidence of Convexal Subarachnoid Hemorrhage in the Elderly: The Mayo Clinic Study of Aging.

OBJECTIVES: Nontraumatic convexal subarachnoid hemorrhages in the elderly can be a manifestation of cerebral amyloid angiopathy associated with a high risk of future intracerebral hemorrhage. The incidence in the elderly population is unknown. Our objectives were to: 1) determine the incidence of convexal subarachnoid hemorrhage in a population-based study, and, 2) to compare apopolipoprotein-E genotype and amyloid positron emission tomographic (PET) imaging for those with versus without hemorrhage. METHODS: Between 11/29/2004 and 3/11/2017, 4462 individuals without hemorrhage at baseline participated in the population-based Mayo Clinic Study of Aging. We used the Rochester Epidemiology Project medical records-linkage system to identify intracerebral hemorrhages. Records and images were reviewed to identify convexal subarachnoid hemorrhage. Neuroimaging characteristics, demographics, medications, and apopolipoprotein-E genotype were recorded. RESULTS: Four cases were identified. The incidence of convexal subarachnoid hemorrhage was 14.1 per 100,000 person years. Three occurred in women, median age, 79 (range: 71-84). One patient had coexisting cerebral microbleeds. Two participants developed a subsequent lobar intracerebral hemorrhage at a median of 4.75 years after convexal subarachnoid hemorrhage. The apopolipoprotein-E -allele combinations of the 4 were: 3/3, 3/3, 2/2, and 2/3. On Pittsburgh Compound B-PET imaging, median standardized uptake value ratio with convexal subarachnoid hemorrhage was 1.86 (range: 1.38-2.34). CONCLUSIONS: Convexal subarachnoid hemorrhage is rare in the older population, occurring with an incidence of about 14 per 100,000 person years. Yet, when present, it may be associated with a high risk of future intracerebral hemorrhage.

Cortical superficial siderosis and acute convexity subarachnoid hemorrhage in cerebral amyloid angiopathy.

BACKGROUND AND PURPOSE: Acute convexity subarachnoid hemorrhage (cSAH) and cortical superficial siderosis (cSS) are neuroimaging markers of cerebral amyloid angiopathy (CAA) that may arise through similar mechanisms. The prevalence of cSS in patients with CAA presenting with acute cSAH versus lobar intracerebral hemorrhage (ICH) was compared and the physiopathology of cSS was explored by examining neuroimaging associations. METHODS: Data from 116 consecutive patients with probable CAA (mean age, 77.4 ± 7.3 years) presenting with acute cSAH (n = 45) or acute lobar ICH (n = 71) were retrospectively analyzed. Magnetic resonance imaging scans were analyzed for cSS and other imaging markers. The two groups' clinical and imaging data were compared and the associations between cSAH and cSS were explored. RESULTS: Patients with cSAH presented mostly with transient focal neurological episodes. The prevalence of cSS was higher amongst cSAH patients than amongst ICH patients (88.9% vs. 57.7%; P < 0.001). In multivariable logistic regression analysis, focal [odds ratio (OR) 6.73; 95% confidence interval (CI) 1.75-25.81; P = 0.005] and disseminated (OR 11.68; 95% CI 3.55-38.35; P < 0.001) cSS were independently associated with acute cSAH, whereas older age (OR 0.93; 95% CI 0.87-0.99; P = 0.025) and chronic lobar ICH count (OR 0.45; 95% CI 0.25-0.80; P = 0.007) were associated with acute lobar ICH. CONCLUSIONS: Amongst patients with CAA, cSS is independently associated with acute cSAH. These findings suggest that cSAH may be involved in the pathogenesis of the cSS observed in CAA. Longitudinal studies are warranted to assess this potential causal relationship.

Acute Convexity Subarachnoid Hemorrhage Related to Cerebral Amyloid Angiopathy: Clinicoradiological Features and Outcome.

BACKGROUND: The specificities of acute convexity subarachnoid hemorrhage (cSAH) related to cerebral amyloid angiopathy (CAA) and its evolution are not well known. We aimed to describe the clinicoradiological pattern, the magnetic resonance imaging (MRI) evolution, and the risk of recurrent bleeding in such patients. METHODS: Among consecutive patients with an acute nontraumatic cSAH, subjects with available MRI who meet the modified Boston criteria for probable CAA were included. Review of medical records, MRI findings, and follow-up data was performed. RESULTS: Twenty-three patients (14 women; mean age ± standard deviation: 75.9 ± 7.3 years) were included. cSAH was revealed by transient focal neurological episodes (TFNEs) in 18 of 23 (78.3%) patients. In all patients, acute cSAH appeared as a sulcal fluid-attenuated inversion recovery hyperintensity and GRE T2 hypointensity. Cortical superficial siderosis and cortical microbleeds, respectively, were observed in 21 (91.3%) and 20 (86.9%) patients. Twenty patients (87%) had available follow-up data with a mean duration of 29.8 ± 20.2 months. Recurrent TFNEs occurred in 40% of patients. Acute cSAH evolved into cortical superficial siderosis in all patients. New subarachnoid bleedings defined by recurrent acute cSAH (n = 8) or extension of siderosis (n = 14) were detected in 83.3% of the patients. Lobar intracerebral hemorrhage (ICH) occurred in 7 patients (35%). CONCLUSION: CAA-related cSAH has a specific pattern defined by a high prevalence of TFNEs and cortical superficial siderosis, with a high risk of recurrent bleeding, either cSAH or lobar ICH. The systematic evolution from cSAH to focal cortical superficial siderosis reveals data on siderosis physiopathology.

Nontraumatic convexity subarachnoid hemorrhage: different etiologies and outcomes.

BACKGROUND: Nontraumatic convexity subarachnoid hemorrhage (cSAH) is a rarely reported condition with multiple etiologies. We report the clinical presentation, imaging findings, etiologies, and long-term outcomes of a case series of cSAH. METHODS: We retrospectively analyzed consecutive cases of cSAH, admitted at a Stroke Unit of a tertiary hospital (January 2006 to March 2012). Recorded variables were demographics, clinical presentation, complementary investigation, etiology, and outcome. RESULTS: We included 15 patients (9 men, median age of 65 years), 7% of the 210 nontraumatic SAH patients in this period. The most common clinical manifestation was a focal neurologic deficit. Predominant location of the cSAH was frontal. In 5 cases, there was a clinical significant internal carotid artery (ICA) atheromatous stenosis, ipsilateral to cSAH. Two patients had a possible cerebral amyloid angiopathy (CAA) at presentation. There were 2 cases of reversible cerebral vasoconstriction syndrome, 1 cerebral venous thrombosis, 2 dural fistulae, and 3 undetermined. Short-term outcomes were good in most patients. At follow-up (24.3 months), 2 of the patients with undetermined etiology had a lobar hematoma conferring a severe disability, and the diagnosis of CAA was made. There were no other relevant events or added disability in the other patients. CONCLUSIONS: Significant ICA atherosclerotic stenosis was the most frequent cause of cSAH in our series, reinforcing that cSAH should prompt vascular imagiological evaluation including cervical vessels. Outcomes in cSAH seem to be related to etiology. Patients with undetermined etiology should be followed up because cSAH may be the first manifestation of CAA.

Spontaneous Non-Aneurysmal Convexity Subarachnoid Hemorrhage: A Scoping Review of Different Etiologies beyond Cerebral Amyloid Angiopathy.

Spontaneous convexity subarachnoid hemorrhage (cSAH) is a vascular disease different from aneurysmal SAH in neuroimaging pattern, causes, and prognosis. Several causes might be considered in individual patients, with a limited value of the patient's age for discriminating among these causes. Cerebral amyloid angiopathy (CAA) is the most prevalent cause in people > 60 years, but reversible cerebral vasoconstriction syndrome (RCVS) has to be considered in young people. CAA gained attention in the last years, but the most known manifestation of cSAH in this context is constituted by transient focal neurological episodes (TFNEs). CAA might have an inflammatory side (CAA-related inflammation), whose diagnosis is relevant due to the efficacy of immunosuppression in resolving essudation. Other causes are hemodynamic stenosis or occlusion in extracranial and intracranial arteries, infective endocarditis (with or without intracranial infectious aneurysms), primary central nervous system angiitis, cerebral venous thrombosis, and rarer diseases. The diagnostic work-up is fundamental for an etiological diagnosis and includes neuroimaging techniques, nuclear medicine techniques, and lumbar puncture. The correct diagnosis is the first step for choosing the most effective and appropriate treatment.

Cerebral venous sinus thrombosis presenting with subarachnoid hemorrhage: a series of 11 cases.

BACKGROUND AND PURPOSE: Cerebral venous sinus thrombosis (CVST) is a rare cerebrovascular disease with a wide spectrum of clinical manifestations. The aim of this study is to assess the presence of subarachnoid hemorrhage (SAH) in the context of CVST and to present its epidemiological, clinical and radiological/laboratory aspects. METHODS: Between October 2012 and October 2019, 43 patients with CVST were reviewed. Eleven (25.58%) cases presented SAH. RESULTS: A quarter of the patients evaluated with CVST had SAH. There were 9 women (81.82%) and 2 men, with a mean age at presentation of 37.7 years (range 28-49). The most common risk factor was the use of oral contraceptives and the most prevalent symptom was headache. Both sinuses, the superior sagittal sinus and the transverse sinus, were the most affected. There was no isolated involvement of the cortical vein. The SAH was limited to some sulci of cerebral convexity in 8 cases (72.73%). In one case, the location of SAH was in the cerebellum and in two cases in the Sylvian fissure. In two cases, there was an evolution to venous infarction; and in three cases, intraparenchymal hemorrhage was present. Seven patients (63.64%) improved considerably with anticoagulation after 6 months of treatment. CONCLUSIONS: This series found that 25.58% of patients with CVST had SAH. It is the highest incidence described in the literature so far. Findings of SAH located in the cerebral convexities, without affecting the base cisterns, should always lead to the suspicion of CVST.

Acute ischemic stroke and convexity subarachnoid hemorrhage in large vessel atherosclerotic stenosis: Case series and review of the literature.

Atraumatic convexity subarachnoid hemorrhage (cSAH) is a rare non-aneurysmal SAH, commonly due to ipsilateral internal carotid artery (ICA) stenosis. It is unusual for the cSAH to occur contralaterally to the infarct. We report two cases of acute ischemic stroke associated with contralateral and ipsilateral cSAH that had different presentations.

CT-Visible Convexity Subarachnoid Hemorrhage Predicts Early Recurrence of Lobar Hemorrhage.

Background and Purpose: Convexity subarachnoid hemorrhage (cSAH) may predict an increased recurrence risk in cerebral amyloid angiopathy (CAA)-related intracerebral hemorrhage (ICH) survivors. We aimed to investigate whether cSAH detected on CT was related to early recurrence in patients with ICH related to CAA. Methods: We analyzed data from consecutive lobar ICH patients diagnosed as probable or possible CAA according to the Boston criteria using the method of cohort study. Demographic and clinical data, ICH recurrence at discharge and within 90 days were collected. The association between cSAH detected on CT and early recurrent ICH was analyzed using multivariable logistic regression. Results: A total of 197 cases (74 [66-80] years) were included. cSAH was observed on the baseline CT of 91 patients (46.2%). A total of 5.1% (10/197) and 9.5% (17/179) of patients experienced ICH recurrence within 2 weeks and 90 days, respectively. The presence of cSAH was related to recurrence within 2 weeks (OR = 5.705, 95%CI 1.070-30.412, P = 0.041) after adjusting for hypertension, previous symptomatic ICH and anticoagulant use. The presence of cSAH was related to recurrence within 90 days (OR 5.473, 95%CI 1.425-21.028, P = 0.013) after adjusting for hypertension, previous symptomatic ICH and intraventricular hemorrhage. The similar results were obtained in other models using different methods to select adjusting variables. Conclusion: In patients with lobar ICH related to CAA, 5.1% and 9.5% of them experienced ICH recurrence within 2 weeks and 90 days, respectively. CT-visible cSAH was detected in 46.2% of patients and indicates an increased risk for early recurrent ICH.

[Subdural hematoma with reversible cerebral vasoconstriction syndrome: a case report].

A 71-year-old man had persistent cervical pain secondary to thunderclap headache during sleep. MRI conducted the next morning revealed subdural hematoma and convexity subdural hemorrhage on the right occipital region, and the patient was hospitalized. MRA showed vascular narrowing in the bilateral PCA. Follow-up MRA on day 8 of admission showed aggravated vascular narrowing of PCA, indicative of reversible cerebral vasoconstriction syndrome (RCVS). The patient was treated with a calcium-channel antagonist. Post-discharge MRA showed improvement of PCA narrowing, and the diagnosis of RCVS was confirmed.

The First Case Report of Community-Acquired Infective Endocarditis Due to Sequence Type 1223 Staphylococcus argenteus Complicated with Convexity Subarachnoid Hemorrhage.

Staphylococcus argenteus is a new species classified as part of the Staphylococcus aureus-related complex in 2015 and has been recognized to be as pathogenic as S. aureus. We describe the first case of endocarditis caused by S. argenteus. A 51-year-old man presented with chief complaints of fever and headaches. On admission, he showed a slight decrease in consciousness level (Glasgow Coma Scale, E4V4M6). Careful physical examination and imaging revealed a systolic heart murmur, Janeway lesions, and complicating convexity subarachnoid hemorrhage (cSAH) of the left frontal lobe. Ceftriaxone 4 g/day was administered immediately after blood cultures were obtained. The next day, all blood cultures grew Gram-positive cocci, identified as Staphylococcus aureus or Staphylococcus argenteus by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS). However, the version of MALDI-TOF MS used could not distinguish these bacteria. Although we could not find valvular vegetation, he was clinically diagnosed with infective endocarditis according to the modified Duke's criteria. Meropenem 6 g/day and linezolid 1.2 g/day were started to cover S. aureus and methicillin-resistant S. aureus. Finally, ampicillin was selected based on drug susceptibility, and the patient was treated for 8 weeks and recovered without permanent damage. The isolated strain formed white colonies on blood agar plates, characteristic of S. argenteus, and differs from golden colony-forming S. aureus. Genetic analysis revealed the isolated strain as S. argenteus (sequence type 1223). Although distinguishing S. argenteus from S. aureus using routine conventional laboratory tests is difficult, the updated library version of MALDI-TOF MS is useful in identifying S. argenteus. Interestingly, all published cases of infection caused by ST1223 have been reported in Japan. Therefore, the trend of infections from the ST1223 strain should be carefully monitored, particularly in Japan. Further investigation is needed to clarify the epidemiology and clinical characteristics of S. argenteus infection, as there are few studies regarding this pathogen.

Multifocal Atraumatic Convexity Subarachnoid Hemorrhage.

Background Multifocal convexity subarachnoid hemorrhage (cSAH) has generally been described in the setting of traumatic brain injury, however, it has also been reported in the absence of trauma in conditions such as with reversible cerebral vasoconstriction syndrome. We describe the clinical and radiographic characteristics of multifocal cSAH in an academic center. Methods We analyzed our single-center retrospective database of nontraumatic convexity subarachnoid hemorrhage from January 2015-January 2018. Convexity subarachnoid hemorrhage was defined as blood in one or more cortical sulci in the absence of trauma; patients with blood in the cisterns or Sylvian fissure were excluded. Multifocal location was defined as at least two distinct foci of hemorrhage occurring in two or more lobes. Clinical and neuroimaging data were collected. Results Out of 70 total patients with convexity subarachnoid hemorrhage, 13 cases were of multifocal convexity subarachnoid hemorrhage, occurring in 18.6% of all cases. The mean age was 58 years (SD = 14.7). Eleven patients were female. Seven patients had reversible cerebral vasoconstriction syndrome (RCVS)/posterior reversible encephalopathy syndrome (PRES), two had cerebral amyloid angiopathy (CAA), three had intrinsic coagulopathy, and one patient had endocarditis as the etiology of multifocal cSAH. Headache was the most common complaint, in eight (61.5%) patients. Conclusion Multifocal cSAH occurs in approximately 18.6% of all cSAH and can occur in the absence of trauma. In our larger cohort of all cSAH, CAA was the most common cause; however, multifocal cSAH is more commonly caused by RCVS/PRES spectrum. Clinicians should be aware that multifocal cSAH can occur in the absence of trauma, and may be a harbinger of RCVS/PRES, particularly in young patients with thunderclap headaches.

Intracerebral Hemorrhage and Reversible Cerebral Vasoconstriction Syndrome in a Patient With COVID-19.

Neurological manifestations, such as encephalopathy, intracranial neuropathy, headache, and cognitive decline, are often presented in patients with COVID-19 infection. Since the onset of the pandemic, acute ischemic stroke associated with a hypercoagulable state caused by COVID-19 is increasingly being reported. Hemorrhagic stroke is also reported via poorly understood mechanisms. We report one of the first-ever cases of intraparenchymal hemorrhage, subarachnoid hemorrhage secondary to reversible cerebral vasoconstriction syndrome in a patient with COVID-19 infection.

Convexity subarachnoid hemorrhage presented with loss of consciousness.

When patients present with transient loss of consciousness without headache and head computed tomography is performed, clinicians should pay attention to cortical high densities as convexity subarachnoid hemorrhage can be a differential diagnosis.

Patterns, Predictors, and Outcomes of Postprocedure Delayed Hemorrhage Following Flow Diversion for Intracranial Aneurysm Treatment.

OBJECTIVE: To evaluate patterns, predictors, and outcomes of postprocedure delayed hemorrhage (PPDH) following flow diversion therapy for intracranial aneurysm treatment. METHODS: From 2012 to 2016, 50 patients with 52 aneurysms were treated with the Pipeline embolization device. Device placement was performed as a standalone therapy or with adjunctive coil embolization. Patients underwent dual antiplatelet therapy for 6 months after treatment. Medical comorbidities; aneurysm traits; and treatment factors, including platelet function testing, were studied. Statistical analysis was performed using cross-tabulation. RESULTS: Six PPDHs (12%) occurred 2-16 days (mean 6.8 days) after Pipeline placement, manifesting as 1 of 2 distinct patterns: convexity subarachnoid hemorrhage (cSAH) (n = 4) or lobar intraparenchymal hemorrhage (IPH) (n = 2). All PPDHs occurred ipsilateral to the device; 1 IPH occurred ipsilateral but in a different arterial territory. PPDH occurred in both treated anterior communicating artery aneurysms. Cases of PPDH demonstrated on average lower P2Y12 reaction unit values at the time of treatment. Platelet function testing at the time of hemorrhage was consistently hypertherapeutic. Patients with cSAH had only minimal worsening of modified Rankin Scale score at the time of discharge, whereas the 2 patients with IPH experienced significant deterioration. CONCLUSIONS: PPDH is a poorly understood complication following flow diversion therapy that can result in significant morbidity. In our experience, nonaneurysmal cSAH does not result in poor clinical outcomes, whereas IPH leads to long-term deficits or death. As previously suggested, there appears to be a correlation between low P2Y12 reaction unit values and PPDH.