Abstract only Objective: We sought to determine the prevalence, radiological characteristics, and clinical outcomes of intracerebral hemorrhage (ICH) of undetermined etiology. Methods: Systematic ...review and meta-analysis of studies involving patients with spontaneous ICH was conducted to assess the prevalence and clinical-radiological characteristics of undetermined ICH. Additionally, we assessed the rates for ICH secondary to hypertensive arteriopathy (HTN-A) and cerebral amyloid angiopathy (CAA). Subgroup analyses were performed based on the use of a) etiology-oriented ICH classification, b) detailed neuroimaging, and c) Boston criteria among CAA-ICH. Results: 24 studies were included (n=15,828; mean age: 64.8 years, males: 60.8%). The pooled prevalence of HTN-A ICH, undetermined ICH and CAA-ICH were 50% (95%CI: 43-58%), 18% (95%CI: 13-23%), and 12% (95%CI: 7-17%; p<0.001 between subgroups). The volume of ICH was largest in CAA-ICH 24.7mL (95%CI: 19.7-29.8mL), followed by HTN-A ICH 16.2mL (95%CI: 10.9-21.5mL) and undetermined ICH 15.4mL (95%CI: 6.2-24.5mL). Among patients with undetermined ICH, the rates of short-term mortality and intraventricular hemorrhage were 33% (95%CI: 25-42%) and 38% (95%CI: 28-48%), respectively. Subgroup analysis demonstrated a higher rate of undetermined ICH among studies that did not use an etiology-oriented classification (22%; 95%CI: 15-29%). No difference was observed between studies based on the completion of detailed neuroimaging to assess the rates of undetermined ICH (p=0.62). Conclusions: The etiology of spontaneous ICH remains undetermined among one in five patients in studies using etiology-oriented classification and among one in four patients in studies that avoid using etiology-oriented classification. The short-term mortality in undetermined ICH is high despite the relatively small ICH volume. Our findings suggest the use of etiology-oriented classification to approach ICH patients (Figure).
Background and purpose
In recent years, the use of coiling has gained increased popularity for the treatment of intracranial aneurysms, and stroke physicians are confronted with rare pathologies ...associated with this relatively new and evolving treatment method, such as embolization of pieces of the polymeric filaments from the coils and a subsequent inflammatory response. In particular, white matter enhancing lesions are a rare complication after aneurysm endovascular therapy (EVT), suggesting a foreign body reaction to shedding of hydrophilic coating from the endovascular devices into the blood stream. The description of such a case aims to raise the clinicians' awareness of the symptomatic delayed and recurring inflammatory changes that may occur after endovascular aneurysmal treatment with the use of coiling devices.
Case description
A 64‐year‐old woman underwent coiling of a ruptured right posterior communicating artery aneurysm. She was asymptomatic after EVT. One year later, she presented with headache, acoustic hallucinations, paresthesias and left arm weakness. Brain magnetic resonance imaging (MRI) revealed multiple enhancing white matter lesions in the right hemisphere. She was treated with pulse intravenous methylprednisolone, followed by oral prednisolone; all clinical symptoms resolved and imaging findings improved substantially. Two years after tapering the steroids, follow‐up symptoms recurred and repeat brain MRI revealed new enhancing white matter lesions.
Discussion and conclusions
There is an increasing number of similar reports of enhancing white matter lesions after coiling of intracranial aneurysms, with the incidence estimated to be between 0.5% and 2.3% in different cohort studies. Close monitoring for the appearance of new neurologic symptoms that could suggest delayed brain reactivity should be recommended.
Clinicians should consider delayed brain reactivity after coiling of cerebral aneurysms as part of the differential diagnosis of vasogenic edema confined to one vascular territory in patients with relevant history. Another characteristic neuroimaging finding may be low‐signal spots in susceptibility‐weighted magnetic resonance imaging, indicating the presence of microembolic paramagnetic materials from the coiling. Although steroid pulse therapy typically results in significant clinical and neuroimaging improvement, corticosteroid tapering may be associated with relapse of clinical symptoms and recurrent enhancing brain lesions and patients should be monitored closely for the appearance of new neurologic symptoms.
Several risk factors of symptomatic intracerebral hemorrhage (SICH) following intravenous thrombolysis for acute ischaemic stroke have been established. However, potential predictors of good ...functional outcome post-SICH have been less studied.
Patient data registered in the Safe Implementation of Treatment in Stroke-International Stroke Thrombolysis Register (SITS-ISTR) from 2005 to 2021 were used. Acute ischaemic stroke patients who developed post intravenous thrombolysis SICH according to the SITS Monitoring Study definition were analyzed to identify predictors of functional outcomes.
A total of 1679 patients with reported SICH were included, out of which only 2.8% achieved good functional outcome (modified Rankin Scale scores of 0-2), whilst 80.9% died at 3 months. Higher baseline National Institutes of Health Stroke Scale (NIHSS) score and 24-h ΔNIHSS score were independently associated with a lower likelihood of achieving both good and excellent functional outcomes at 3 months. Baseline NIHSS and hematoma location (presence of both SICHs, defined as remote and local SICH concurrently; n = 478) were predictors of early mortality within 24 h. Independent predictors of 3-month mortality were age, baseline NIHSS, 24-h ΔNIHSS, admission serum glucose values and hematoma location (both SICHs). Age, baseline NIHSS score, 24-h ΔNIHSS, hyperlipidemia, prior stroke/transient ischaemic attack, antiplatelet treatment, diastolic blood pressure at admission, glucose values on admission and SICH location (both SICHs) were associated with reduced disability at 3 months (≥1-point reduction across all modified Rankin Scale scores). Patients with remote SICH (n = 219) and local SICH (n = 964) had comparable clinical outcomes, both before and after propensity score matching.
Symptomatic intracerebral hemorrhage presents an alarmingly high prevalence of adverse clinical outcomes, with no difference in clinical outcomes between remote and local SICH.
Background
Differences have been noted in the clinical presentation and mutational spectrum of CADASIL among various geographical areas. The aim of the present study was to investigate the mode of ...clinical presentation and genetic mutations reported in Greece.
Methods
After a systematic literature search, we performed a pooled analysis of all published CADASIL cases from Greece.
Results
We identified 14 studies that reported data from 14 families comprising 54 patients. Migraine with aura was reported in 39%, ischemic cerebrovascular diseases in 68%, behavioral‐psychiatric symptoms in 47% and cognitive decline in 60% of the patients. The mean (±SD) age of onset for migraine with aura, ischemic cerebrovascular diseases, behavioral‐psychiatric symptoms and cognitive decline was 26.2 ± 8.7, 49.3 ± 14.6, 47.9 ± 9.4 and 42.9 ± 10.3, respectively; the mean age at disease onset and death was 34.6 ± 12.1 and 60.2 ± 11.2 years. With respect to reported mutations, mutations in exon 4 were the most frequently reported (61.5% of all families), with the R169C mutation being the most common (30.8% of all families and 50% of exon 4 mutations), followed by R182C mutation (15.4% of all families and 25% of exon 4 mutations).
Conclusions
The clinical presentation of CADASIL in Greece is in accordance with the phenotype encountered in Caucasian populations, but differs from the Asian phenotype, which is characterized by a lower prevalence of migraine and psychiatric symptoms. The genotype of Greek CADASIL pedigrees is similar to that of British pedigrees, exhibiting a high prevalence of exon 4 mutations, but differs from Italian and Asian populations, where mutations in exon 11 are frequently encountered.
The clinical presentation of CADASIL in Greece is in accordance with the phenotype encountered in Caucasian populations: migraine with aura was reported in 39%, ischemic cerebrovascular diseases in 68%, behavioral‐psychiatric symptoms in 47% and cognitive decline in 60% of the patients.
Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism and causes neurological manifestations because of excessive accumulation of phenylalanine (PHE). It can also affect ...adult patients who discontinue their treatment, even if they had been under adequate metabolic control during childhood. For that reason, it is recommended that PKU treatment should be continued throughout life and target PHE levels for adult patients should range between 120 and 600 μmol/L.
The authors present an adult patient with PKU who discontinued treatment and developed cognitive dysfunction because of high blood levels of PHE. Brain magnetic resonance imaging (MRI) of the patient was characteristic for PKU, presenting periventricular and callosal white matter hyperintensities in T2 and fluid-attenuated inversion recovery sequences, which were additionally associated with true restriction in diffusion-weighted imaging sequence, a far less recognized PKU neuroimaging feature.
Cognitive dysfunction and psychiatric disorders can be present in adult patients with PKU who discontinue treatment and have poor PHE metabolic control. The presence of white matter hyperintensities in T2 and fluid-attenuated inversion recovery MRI-sequences is a well-described neuroimaging feature of PKU, but diffusion-weighted imaging sequence may also be reliable in detecting brain lesions in patients with PKU. PKU lesions should be considered in the differential diagnosis of true diffusion restriction in brain MRI of patients with PKU history or those who might have escaped newborn screening diagnosis but present neurocognitive dysfunction. Appropriate treatment for the management of PKU should be initiated for the reversal of the clinical and neuroimaging findings.
Perioperative neck hematoma (PNH) requiring re-intervention is an important complication after carotid endarterectomy (CEA). There are limited data regarding the potential risk factors associated ...with PNH. The aim of this prospective, multicenter study was to document the rate of PNH in symptomatic carotid artery stenosis (sCAS) patients treated with CEA within the first 14 days of cerebrovascular symptom onset and to identify possible predictors of this complication.
Patients with non-disabling (mRS ≤ 2) acute ischemic stroke or transient ischemic attack due to sCAS (≥70%) underwent CEA at three stroke-centers during a seven-year period. PNH requiring surgical re-intervention or transfusion during a 30-day follow-up period was determined by the attending surgeon but was also confirmed by an independent neurologist.
A total of 280 patients with sCAS underwent CEA within 14 days of ictus. PNH occurred in 10 cases (3.6%; 95%CI: 1.4%–5.8%). Pretreatment with therapeutic anticoagulation (TA) and history of atrial fibrillation were more prevalent in patients with PNH (20% vs. 3.1%, p= 0.047 & 30% vs. 8.2%, p = 0.05 respectively). Elapsed time between symptom onset and carotid surgery, pretreatment with dual antiplatelets, intravenous thrombolysis or prophylactic anticoagulation were not related to PNH in univariable analyses. Pretreatment with TA was independently associated with higher likelihood of PNH OR: 10.69, 95%CI (1.74–65.72), p = 0.011 in multivariate logistic regression models adjusting for potential confounders.
PNH is uncommon in patients with sCAS that are operated during the first 14 days of ictus. Pretreatment with TA appears to be associated with higher risk of PNH.
•PNH is rare in patients undergoing CEA during the first 14 days of symptom onset.•PNH is not related to increased risk of cardiovascular or poor functional outcomes.•Treatment with therapeutic anticoagulation might be associated with a higher risk of PNH.•The role of anticoagulants in PNH after CEA needs further evaluation.
PDF