• CNS vasculitis
• IgG4-related
• dilating arteriopathy
• disease
• dolichoectasia
• eosinophilic granulomatosis with polyangiitis
• mycotic aneurysm

• Female
• Humans
• Middle Aged
• Acute Disease
• Brain/diagnostic imaging
• Diagnosis, Differential
• Headache/etiology
• Magnetic Resonance Imaging
• Subarachnoid Hemorrhage/diagnostic imaging
• Subarachnoid Hemorrhage/etiology
• Takotsubo Cardiomyopathy/complications
• Takotsubo Cardiomyopathy/diagnosis
• Takotsubo Cardiomyopathy/diagnostic imaging
• Tomography, X-Ray Computed
• Vasospasm, Intracranial/complications
• Vasospasm, Intracranial/diagnosis
• Vasospasm, Intracranial/diagnostic imaging
• Syndrome

• cerebral amyloid angiopathy
• dementia
• intracranial hemorrhage
• seizure
• subarachnoid hemorrhage
• superficial siderosis

• Female
• Humans
• Cerebral Hemorrhage/complications
• Siderosis
• Retrospective Studies
• Incidence
• Magnetic Resonance Imaging
• Cerebral Amyloid Angiopathy/complications
• Cerebral Amyloid Angiopathy/epidemiology
• Risk Factors
• Seizures/etiology
• Seizures/complications
• Inflammation/complications

• Cerebral venous sinus thrombosis
• Convexity subarachnoid hemorrhage
• Intraparenchymal hemorrhage
• Magnetic resonance imaging
• Venous infarction

Nontraumatic convexal subarachnoid hemorrhage (cSAH) is a rare type of atypical subarachnoid hemorrhage. It mainly presents as a focal and transient neurological deficit with similar manifestations as transient ischemic attack.

We report a case of a 64-year-old man who visited the hospital with paroxysmal left-sided numbness and weakness is presented in this study. Computed tomography examination indicated a high-density image of the right frontal-parietal sulcus. Digital subtraction angiography showed severe stenosis at the right anterior cerebral artery A2-A3 junction (stenosis rate approximately 70%).

The findings of this case indicate that anterior cerebral artery stenosis may lead to the occurrence of cSAH.

• Nontraumatic convexal subarachnoid hemorrhage
• Subarachnoid hemorrhage
• Transient ischemic attack
• Artery atherosclerosis stenosis
• Case report

• atherosclerosis
• convexity
• intracranial
• subarachnoid haemorrhage

To investigate the frequency, time-course and predictors of intracerebral haemorrhage (ICH), recurrent convexity subarachnoid haemorrhage (cSAH), and ischemic stroke after cSAH associated with cerebral amyloid angiopathy (CAA).

We performed a systematic review and international individual patient-data pooled analysis in patients with cSAH associated with probable or possible CAA diagnosed on baseline MRI using the modified Boston criteria. We used Cox proportional hazards models with a frailty term to account for between-cohort differences.

We included 190 patients (mean age 74.5 years; 45.3% female) from 13 centers with 385 patient-years of follow-up (median 1.4 years). The risks of each outcome (per patient-year) were: ICH 13.2% (95% CI 9.9–17.4); recurrent cSAH 11.1% (95% CI 7.9–15.2); combined ICH, cSAH, or both 21.4% (95% CI 16.7–26.9), ischemic stroke 5.1% (95% CI 3.1–8) and death 8.3% (95% CI 5.6–11.8). In multivariable models, there is evidence that patients with probable CAA (compared to possible CAA) had a higher risk of ICH (HR 8.45, 95% CI 1.13–75.5, p = 0.02) and cSAH (HR 3.66, 95% CI 0.84–15.9, p = 0.08) but not ischemic stroke (HR 0.56, 95% CI 0.17–1.82, p = 0.33) or mortality (HR 0.54, 95% CI 0.16–1.78, p = 0.31).

Patients with cSAH associated with probable or possible CAA have high risk of future ICH and recurrent cSAH. Convexity SAH associated with probable (vs possible) CAA is associated with increased risk of ICH, and cSAH but not ischemic stroke. Our data provide precise risk estimates for key vascular events after cSAH associated with CAA which can inform management decisions.

The online version contains supplementary material available at 10.1007/s00415-021-10706-3.

• Cerebral amyloid angiopathy
• Intracerebral haemorrhage
• Ischemic stroke
• Non-traumatic convexity/convexal/cortical subarachnoid haemorrhage
• Stroke

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.

• cerebral amyloid angiopathy
• convexity subarachnoid hemorrhage
• mri- magnetic resonance imaging
• reversible cerebral vasoconstriction syndrome
• subarachnoid hemorrhage

• Angiographic vasospasm
• Cerebral aneurysm
• Delayed cerebral ischemia
• Stroke
• Subarachnoid hemorrhage

Convexal subarachnoid hemorrhage (cSAH) is characterized by isolated bleeding in one or a few adjacent sulci and has diverse etiologies and symptoms. Acute ischemic stroke co-occurring with cSAH has been infrequently reported. Nearly all cases of cSAH have been described to occur on the side with acute ischemic stroke, and it is unusual for cSAH to occur on the opposite side of the infarct territory.

Our report presents two cases of acute ischemic stroke associated with contralateral cSAH. The first patient had left atherosclerotic internal carotid artery (ICA) occlusion with developing right parietal cSAH. The other patient developed left parietal cSAH in the setting of right ICA occlusion caused by cardiogenic embolism with acute right cerebral hemisphere infarction. Both patients remained clinically stable with good prognosis after antithrombotic treatment.

Our report suggest that cSAH may simultaneously occur on the opposite side of an infarction. Although there is no consensus on the etiology and treatment of this rare phenomenon, cSAH did not lead to a poor outcome in our patients.

• Acute ischemic stroke
• Cardioembolic stroke
• Case report
• Convexal subarachnoid hemorrhage
• Internal carotid artery occlusion

• Aetiology
• Angiopatía amiloide
• Cerebral amyloid angiopathy
• Cortical subarachnoid haemorrhage
• Etiología
• Hemorragia subaracnoidea atraumática
• Hemorragia subaracnoidea cortical
• Ictus isquémico
• Ischaemic stroke
• Non-traumatic subarachnoid haemorrhage
• Prognosis
• Pronóstico

• Aged
• Brain Ischemia
• Humans
• Prognosis
• Prospective Studies
• Retrospective Studies
• Stroke
• Subarachnoid Hemorrhage/diagnosis

• cerebral amyloid angiopathy
• intracranial haemorrhage
• magnetic resonance imaging
• stroke

Patient: Female, 37

Final Diagnosis: Lupus vasculitis

Symptoms: Back pain • headache • paralysis • sensory loss

Medication: Cyclophosphamide • prednisone • methylprednisolone

Clinical Procedure: CT/MRI thoracic spine • lumbar puncture • plasmapheresis • kidney biopsy

Specialty: Neurology

• Embolic stroke
• atrial fibrillation
• cSAH
• cortical sulci

Report a case of cortical subarachnoid hemorrhage (cSAH) and discuss its management.

A 66-year-old woman presents with acute onset left arm numbness and weakness. Initial head CT shows small hyperdensity in sulci typical for cSAH. Extensive workup with MRI, lumbar puncture and blood tests is performed. No signs of infection, vascular malformations, thrombosis or cancer are found. At outpatient follow-up, she is diagnosed with cSAH secondary to amyloid angiopathy. She is treated with gabapentin.

Diagnosis of cSAH is challenging given its subtle findings, and management is empiric as there are only a few case series in literature.

• cerebrovascular disease
• headache
• imaging
• neuroimaging
• seizures
• transient ischemic attack

To compare the clinical evolution of perimesencephalic subarachnoid hemorrhage and non-perimesencephalic subarachnoid hemorrhage.

The study was conducted retrospectively in a tertiary hospital center in the north region of Portugal. Included patients had no identifiable cause for subarachnoid hemorrhage. Several epidemiologic, clinical and imaging aspects were statistically analyzed, taking into account the differences in perimesencephalic subarachnoid hemorrhage and non-perimesencephalic subarachnoid hemorrhage.

Sixty-two patients met the inclusion criteria (46.8% - perimesencephalic subarachnoid hemorrhage; 53.2% - non-perimesencephalic subarachnoid hemorrhage). Demographic and clinical background characteristics were similar in both groups. Complications were more frequent in patients with non-perimesencephalic subarachnoid hemorrhage - 84.8% of the patients had at least one complication versus 48.3% in perimesencephalic subarachnoid hemorrhage. Vasospasm, infection and hydrocephaly were the most common complications (each was detected more frequently in the non-perimesencephalic subarachnoid hemorrhage group than in perimesencephalic subarachnoid hemorrhage group). Two patients died, both had a non-perimesencephalic subarachnoid hemorrhage. The median inpatient time was longer in the non-perimesencephalic subarachnoid hemorrhage group (21 versus 14 days). No incidents of rebleeding were reported during the follow-up period (mean time of 15 ± 10.3 months).

Perimesencephalic subarachnoid hemorrhage and non-perimesencephalic subarachnoid hemorrhage are two different entities that have different clinical outcomes, namely in terms of complication rate and median inpatient time. The management of these patients should respect this difference to improve treatment and optimize health care resources.

The risk of future symptomatic intracerebral haemorrhage (sICH) remains uncertain in patients with acute convexity subarachnoid haemorrhage (cSAH) associated with suspected cerebral amyloid angiopathy (CAA). We assessed the risk of future sICH in patients presenting to our comprehensive stroke service with acute non-traumatic cSAH due to suspected CAA, between 2011 and 2016. We conducted a systematic search and pooled analysis including our cohort and other published studies including similar cohorts. Our hospital cohort included 20 patients (mean age 69 years; 60% male); 12 (60%) had probable CAA, and 6 (30%) had possible CAA according to the modified Boston criteria; two did not meet CAA criteria because of age <55 years, but were judged likely to be due to CAA. Fourteen patients (70%) had cortical superficial siderosis; 12 (60%) had cerebral microbleeds. Over a mean follow-up period of 19 months, 2 patients (9%) suffered sICH, both with probable CAA (annual sICH risk for probable CAA 8%). In a pooled analysis including our cohort and eight other studies (n = 172), the overall sICH rate per patient-year was 16% (95% CI 11–24%). In those with probable CAA (n = 104), the sICH rate per patient-year was 19% (95% CI 13–27%), compared to 7% (95% CI 3–15%) for those without probable CAA (n = 72). Patients with acute cSAH associated with suspected CAA are at high risk of future sICH (16% per patient-year); probable CAA might carry the highest risk.

• Cerebral amyloid angiopathy
• Intracerebral haemorrhage
• Non-traumatic convexity/convexial/cortical subarachnoid haemorrhage
• Stroke
• Superficial siderosis

Multicentric Castleman’s disease (MCD) is characterized by a systemic lymphoproliferative disorder affecting systemic lymph nodes. Cerebrovascular involvements have rarely been reported, and to our knowledge, cerebral angiitis causing subarachnoid hemorrhage (SAH) in patients with Multicentric Castleman’s disease (MCD) has not been previously described.

We identified two cases of MCD with SAH who were receiving immunosuppressive therapy with low dose prednisolone. Both patients presented with sudden-onset headache and were diagnosed with cortical SAH in the sulci by a computed tomography scan. Digital subtraction angiography showed segmental stenosis in the peripheral area of the middle cerebral artery. In both cases, cerebral angiitis causing SAH induced by a systemic inflammatory condition and elevated levels of interleukin (IL) -6 were suspected and resolved over a period of several months.

Our cases highlight the clinical diversity of the potential causes of cerebral angiitis and expand the association of MCD and cortical SAH; however, cortical SAH patients have a more favorable outcome than aneurysmal SAH patients.

• Castleman’s disease
• Cerebral angiitis
• Interleukin-6
• Subarachnoid hemorrhage

• Cortical subarachnoid hemorrhage
• Headache
• Isolated cortical venous thrombosis

• Apolipoprotein E
• Apolipoproteína E
• Beta-amyloid peptide
• Cheiro-oral symptoms
• Hemorragia subaracnoidea
• Hemosiderosis meníngea
• Meningeal haemosiderosis
• Microbleeds
• Microsangrados
• Péptido beta-amiloide
• Subarachnoid haemorrhage
• Síntomas queiroorales

• Aneurysm
• Computed tomography angiography
• Digital subtraction angiography
• Nonaneurysmal subarachnoid hemorrhage
• Perimesencephalic subarachnoid hemorrhage
• Quadrigeminal perimesencephalic subarachnoid hemorrhage

• apoe
• biomarkers
• cerebral amyloid angiopathy
• lobar hemorrhage
• subarachnoid hemorrhage

• Convexity subarachnoid hemorrhage
• Extracranial vaso-occlusive disease
• Ischemic stroke

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset headache with focal neurologic deficit and prolonged but reversible multifocal narrowing of the distal cerebral arteries. Stroke, either hemorrhagic or ischemic, is a relatively frequent presentation in RCVS, but progressive manifestations of subarachnoid hemorrhage, intracerebral hemorrhage, cerebral infarction in a patient is seldom described. We report a rare case of a 56-year-old woman with reversible cerebral vasoconstriction syndrome consecutively presenting as cortical subarachnoid hemorrhage, intracerebral hemorrhage, and cerebral infarction. When she complained of severe headache with subtle cortical subarachnoid hemorrhage, her angiography was non-specific. But, computed tomographic angiography showed typical angiographic features of this syndrome after four days. Day 12, she suffered mental deterioration and hemiplegia due to contralateral intracerebral hematoma, and she was surgically treated. For recurrent attacks of headache, medical management with calcium channel blockers has been instituted. Normalized angiographic features were documented after 8 weeks. Reversible cerebral vasoconstriction syndrome should be considered as differential diagnosis of non-aneurysmal subarachnoid hemorrhage, and repeated angiography is recommended for the diagnosis of this under-recognized syndrome.

• Angiography
• Cerebral infarction
• Intracerebral hemorrhage
• Reversible cerebral vasoconstriction syndrome
• Subarachnoid hemorrhage

• Fusiform aneurysm
• Multiple aneurysms
• Subarachnoid hemorrhage
• Systemic lupus erythematous

There are many potential causes of sudden and severe headache (thunderclap headache), the most important of which is aneurysmal subarachnoid haemorrhage. Published academic reviews report a wide range of causes. We sought to create a definitive list of causes, other than aneurysmal subarachnoid haemorrhage, using a systematic review.

Systematic Review of EMBASE and MEDLINE databases using pre-defined search criteria up to September 2009. We extracted data from any original research paper or case report describing a case of someone presenting with a sudden and severe headache, and summarized the published causes.

Our search identified over 21,000 titles, of which 1224 articles were scrutinized in full. 213 articles described 2345 people with sudden and severe headache, and we identified 6 English language academic review articles. A total of 119 causes were identified, of which 46 (38%) were not mentioned in published academic review articles. Using capture-recapture analysis, we estimate that our search was 98% complete. There is only one population-based estimate of the incidence of sudden and severe headache at 43 cases per 100,000. In cohort studies, the most common causes identified were primary headaches or headaches of uncertain cause. Vasoconstriction syndromes are commonly mentioned in case reports or case series. The most common cause not mentioned in academic reviews was pneumocephalus. 70 non-English language articles were identified but these did not contain additional causes.

There are over 100 different published causes of sudden and severe headache, other than aneurysmal subarachnoid haemorrhage. We have now made a definitive list of causes for future reference which we intend to maintain. There is a need for an up to date population based description of cause of sudden and severe headache as the modern epidemiology of thunderclap headache may require updating in the light of research on cerebral vasoconstriction syndromes.

Reversible cerebral vasoconstriction syndrome (RCVS) refers to a number of disorders characterized by severe and sudden-onset (“thunderclap”) headaches and angiographic features of reversible, segmental, multifocal vasoconstriction of cerebral arteries. Although RCVS generally resolves without significant sequelae, a rare and possibly underrecognized hemorrhagic presentation has a worse potential course. We report three cases of hemorrhagic RCVS and review the literature. Three females (42, 54, and 33 years old, resp.) presented with severe headache, neurological deficits, and dramatic intracerebral hemorrhage (ICH). Patient 1 presented comatose with a 9 × 4 × 6.6 cm left deep intraparenchymal hemorrhage (IPH) and 1 cm midline shift. She underwent emergent surgical intervention. Patient 2 had a 3.3 × 1.5 cm left superior frontal IPH that enlarged to 4 × 2.5 cm within 12 hours with worsening headache and neurological deficits. She was successfully managed nonoperatively. Patient 3, after uncomplicated pregnancy and delivery, presented with a 1.5 cm left superior parietal IPH on postpartum day 7. Two days later, she acutely developed right hemiplegia. Repeat CT demonstrated a new 3.3 × 1.7 cm left frontal IPH. She was also successfully managed nonoperatively. Many diverse conditions are grouped within the category of RCVS. Dramatic ICH remains a rare and possibly underrecognized presenting feature. Prompt diagnosis and management are essential for obtaining the best outcome.

• Convexity subarachnoid hemorrhage
• acute ischemic stroke
• amyloid angiopathy
• atherosclerotic carotid artery stenosis or occlusion
• cortical subarachnoid haemorrhage
• hyperdense sulcus

• amyloid
• headache
• subarachnoid hemorrhage
• thunderclap

• Aged
• Anticoagulants/adverse effects
• Cerebral Amyloid Angiopathy/complications
• Cerebrovascular Disorders/complications
• Diagnostic Imaging/methods
• Female
• Headache/etiology
• Humans
• Intracranial Hypertension/complications
• Male
• Meninges/blood supply
• Middle Aged
• Rupture, Spontaneous
• Seizures/etiology
• Subarachnoid Hemorrhage/complications
• Subarachnoid Hemorrhage/diagnosis
• Subarachnoid Hemorrhage/epidemiology
• Subarachnoid Hemorrhage/etiology
• Symptom Assessment