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1.
Front Immunol ; 15: 1381472, 2024.
Article En | MEDLINE | ID: mdl-38726008

X-linked lymphoproliferative disease (XLP) is a rare genetic disorder characterized by immune dysregulation. The three most common clinical phenotypes are EBV-associated infectious mononucleosis (FIM), abnormal gammaglobulinemia, and lymphoma. We present a rare case of XLP1 with neurovasculitis, which is non-EBV-related and involves multiple systems, a condition rarely seen in children. The patient initially presented with an unsteady gait, which progressively evolved into language and consciousness disorders. Additionally, CT scans revealed multiple nodules in the lungs. Subsequent genetic testing and brain tissue biopsy confirmed the diagnosis: XLP1-related cerebral vasculitis and cerebral hemorrhage. Tragically, during the diagnostic process, the child experienced a sudden cerebral hemorrhage and herniation, ultimately resulting in fatality. This case offers a comprehensive insight into XLP1-related cerebral vasculitis and cerebral hemorrhage, underscoring the significance of early diagnosis and prompt treatment, while also imparting valuable clinical experience and lessons to the medical community.


Cerebral Hemorrhage , Lymphoproliferative Disorders , Vasculitis, Central Nervous System , Humans , Vasculitis, Central Nervous System/diagnosis , Vasculitis, Central Nervous System/etiology , Male , Cerebral Hemorrhage/etiology , Cerebral Hemorrhage/diagnosis , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/complications , Lymphoproliferative Disorders/genetics , Fatal Outcome
2.
Sci Rep ; 14(1): 10008, 2024 05 01.
Article En | MEDLINE | ID: mdl-38693282

Historically, investigators have not differentiated between patients with and without hemorrhagic transformation (HT) in large core ischemic stroke at risk for life-threatening mass effect (LTME) from cerebral edema. Our objective was to determine whether LTME occurs faster in those with HT compared to those without. We conducted a two-center retrospective study of patients with ≥ 1/2 MCA territory infarct between 2006 and 2021. We tested the association of time-to-LTME and HT subtype (parenchymal, petechial) using Cox regression, controlling for age, mean arterial pressure, glucose, tissue plasminogen activator, mechanical thrombectomy, National Institute of Health Stroke Scale, antiplatelets, anticoagulation, temperature, and stroke side. Secondary and exploratory outcomes included mass effect-related death, all-cause death, disposition, and decompressive hemicraniectomy. Of 840 patients, 358 (42.6%) had no HT, 403 (48.0%) patients had petechial HT, and 79 (9.4%) patients had parenchymal HT. LTME occurred in 317 (37.7%) and 100 (11.9%) had mass effect-related deaths. Parenchymal (HR 8.24, 95% CI 5.46-12.42, p < 0.01) and petechial HT (HR 2.47, 95% CI 1.92-3.17, p < 0.01) were significantly associated with time-to-LTME and mass effect-related death. Understanding different risk factors and sequelae of mass effect with and without HT is critical for informed clinical decisions.


Hospitalization , Infarction, Middle Cerebral Artery , Humans , Female , Male , Aged , Retrospective Studies , Middle Aged , Infarction, Middle Cerebral Artery/complications , Cerebral Hemorrhage/etiology , Cerebral Hemorrhage/mortality , Cerebral Hemorrhage/complications , Brain Edema/etiology , Risk Factors , Ischemic Stroke/mortality
3.
J Int Med Res ; 52(4): 3000605241237680, 2024 Apr.
Article En | MEDLINE | ID: mdl-38606755

Intraventricular haemorrhage (IVH) is a severe and acute type of stroke with a complex pathophysiology and is a therapeutic challenge. This case report described a man in his early 50's diagnosed with IVH by computed tomography (CT). Although bilateral extraventricular drainage (EVD) was undertaken, a postoperative CT scan showed that while the left catheter was correctly positioned, the right catheter had been wrongly inserted into the cisterna ambiens. The procedure was equivalent to simultaneous EVD combined with cisternostomy. As a consequence, the haematoma was rapidly removed, the risk of infection and long-term hydrocephalus was reduced, and prognosis was improved. Large case-control studies or prospective studies are needed to evaluate the safety and effectiveness of this treatment modality.


Cerebral Hemorrhage , Hydrocephalus , Male , Humans , Zolpidem/therapeutic use , Treatment Outcome , Cerebral Hemorrhage/diagnostic imaging , Cerebral Hemorrhage/etiology , Cerebral Hemorrhage/surgery , Hydrocephalus/surgery , Drainage/methods , Catheters/adverse effects
4.
J Am Heart Assoc ; 13(7): e034032, 2024 Apr 02.
Article En | MEDLINE | ID: mdl-38533990

BACKGROUND: Intracerebral hemorrhage (ICH) is a major cause of maternal morbidity, but its pathophysiology is poorly characterized. We investigated characteristics of pregnancy-associated ICH (P-ICH), compared with ICH in similar aged nonpregnant adults of both sexes. METHODS AND RESULTS: We performed a retrospective analysis of 134 adults aged 18 to 44 years admitted to our center with nontraumatic ICH from January 1, 2012, to December 31, 2021. We compared ICH characteristics among 3 groups: those with P-ICH (pregnant or within 12 months of end of pregnancy); nonpregnant women; and men. We categorized ICH pathogenesis according to a modified scheme, SMASH-UP (structural, medications, amyloid angiopathy, systemic, hypertension, undetermined, posterior reversible encephalopathy syndrome/reversible cerebral vasoconstriction syndrome), and calculated odds ratios and 95% CIs for primary (spontaneous small-vessel) ICH versus secondary ICH (structural lesions or coagulopathy related), using nonpregnant women as the reference. We also compared specific ICH pathogenesis by SMASH-UP criteria and functional outcomes between groups. Of 134 young adults with nontraumatic ICH, 25 (19%) had P-ICH, of which 60% occurred postpartum. Those with P-ICH had higher odds of primary ICH compared with nonpregnant women (adjusted odds ratio, 4.5 [95% CI, 1.4-14.7]). The odds of primary ICH did not differ between men and nonpregnant women. SMASH-UP pathogenesis for ICH differed significantly between groups (P<0.001). While the in-hospital mortality rate was lowest in the P-ICH group (4%) compared with nonpregnant women (13%) and men (24%), 1 in 4 patients with P-ICH were bedbound and dependent at the time of discharge. CONCLUSIONS: In our cohort of young adults with ICH, 1 in 5 was pregnancy related. P-ICH differed in pathogenesis compared with non-pregnancy-related ICH in young adults, suggesting unique pathophysiology.


Hypertension , Posterior Leukoencephalopathy Syndrome , Pregnancy Complications , Male , Pregnancy , Humans , Female , Young Adult , Retrospective Studies , Posterior Leukoencephalopathy Syndrome/complications , Cerebral Hemorrhage/etiology , Hypertension/complications
5.
Article Ru | MEDLINE | ID: mdl-38465814

Cerebral amyloid angiopathy (CAA) is a progressive disease characterized by the deposition of ß-amyloid in the walls of blood vessels in the brain, which leads to their damage and disruption of normal blood flow. Morphologically, CAA is characterized by both isolated lesions (microhemorrhages with the appearance of cortical superficial siderosis, lacunar infarctions) and widespread changes (hyperintensity of the deep and periventricular white matter, expansion of the perivascular spaces) of cortical and subcortical localization. CAA is considered a major cause of cognitive impairment and intracerebral microbleeds, especially in patients with Alzheimer's disease. The review presents modern ideas about the etiology, pathogenesis, clinical manifestations of CAA, and also outlines the provisions of the Boston principles of CAA, revised in 2022. Understanding the features of pathogenetic methods of CAA is crucial for adjusting the accuracy of diagnosis and developing treatment methods to preserve and prolong cognitive health.


Alzheimer Disease , Cerebral Amyloid Angiopathy , Humans , Cerebral Amyloid Angiopathy/diagnosis , Cerebral Amyloid Angiopathy/diagnostic imaging , Brain/pathology , Alzheimer Disease/pathology , Amyloid beta-Peptides/metabolism , Cerebral Hemorrhage/diagnostic imaging , Cerebral Hemorrhage/etiology , Magnetic Resonance Imaging
6.
J R Coll Physicians Edinb ; 54(1): 48-54, 2024 Mar.
Article En | MEDLINE | ID: mdl-38462907

The ventriculoperitoneal shunt (VP shunt) is one of the most common neurosurgical procedures performed in daily practice. Various complications following a VP shunt are as follows: post-procedure shunt-related infections, shunt block, shunt displacement or exteriorisation and haemorrhage associated with it. Delayed intraventricular haemorrhage is a relatively uncommon complication following the aforementioned procedure. Here we present an atypical case of a 72-year-old male who presented with subarachnoid haemorrhage with hydrocephalus and underwent a VP shunt, following which the patient had early intraventricular haemorrhage (eIVH) with an unfortunate outcome. Here, we propose pathophysiology and risk factors for eIVH.


Hydrocephalus , Ventriculoperitoneal Shunt , Male , Humans , Aged , Ventriculoperitoneal Shunt/adverse effects , Ventriculoperitoneal Shunt/methods , Cerebral Hemorrhage/etiology , Hydrocephalus/surgery , Hydrocephalus/etiology , Neurosurgical Procedures , Risk Factors , Retrospective Studies
7.
CNS Neurosci Ther ; 30(3): e14588, 2024 03.
Article En | MEDLINE | ID: mdl-38475869

AIMS: With the progress of thrombectomy technology, the vascular recanalization rate of patients with stroke has been continuously improved, but the proportion of futile recanalization (FR) is still quite a few. The long-term prognosis and survival of patients with FR and its influencing factors remain unclear. METHODS: Consecutive patients who received endovascular treatment (EVT) for ischemic stroke were enrolled between 2013 and 2021 from a single-center prospectively registry study. We evaluated the long-term outcome of these patients by Kaplan-Meier survival analysis, and the multivariable logistic regression curve was performed to analyze influencing factors. RESULTS: Among 458 patients with FR, 56.4% of patients survived at 1 year, and 50.4% at 2 years. In the multivariate regression analysis, age, premorbid modified Rankin Scale (mRS), National Institutes of Health Stroke Scale (NIHSS), posterior circulation infarct, general anesthesia, symptomatic intracerebral hemorrhage (sICH), and decompressive craniectomy were found to be related to unfavorable outcomes in long-term. Age, premorbid mRS, NIHSS, general anesthesia, and sICH were predictors of long-term mortality. CONCLUSIONS: Futile recanalization accounts for a large proportion of stroke patients after thrombectomy. This study on the long-term prognosis of such patients is beneficial to the formulation of treatment plans and the prediction of therapeutic effects.


Brain Ischemia , Endovascular Procedures , Stroke , Humans , Treatment Outcome , Stroke/therapy , Prognosis , Thrombectomy , Cerebral Hemorrhage/etiology , Reperfusion , Brain Ischemia/therapy , Retrospective Studies
8.
Rinsho Shinkeigaku ; 64(3): 185-189, 2024 Mar 22.
Article Ja | MEDLINE | ID: mdl-38382934

A 71-year-old male who suffered from Hoehn and Yahr stage III Parkinson's disease with bradykinesia, rigidity and a 5-6-Hz tremor at rest in the right extremities was admitted to our hospital due to the sudden onset of vertigo. Right cerebellar hemorrhage was confirmed by CT. The patient's resting tremor in the right extremities disappeared immediately following the cerebellar hemorrhage. Six days later, MRI showed Wallerian degeneration in the cerebello-rubro-thalamic tract. Approximately 5 months later, a 2-3-Hz Holmes' tremor gradually appeared in the right upper extremity. This tremor was improved by increasing L-dopa doses. Case reports of the disappearance of Parkinson's resting tremor and subsequent emergence of Holmes' tremor due to cerebellar lesion are rare. Furthermore, the Wallerian degeneration of the cerebello-rubro-thalamic tract identified on MRI between tremors of the different frequencies is very rare. We hypothesize that the cause of the tremor frequency change was simultaneous damage to the nigro-striatal network and the cerebello-thalamo-cerebral network.


Parkinson Disease , Tremor , Male , Humans , Aged , Tremor/etiology , Parkinson Disease/complications , Parkinson Disease/pathology , Wallerian Degeneration/pathology , Thalamus/diagnostic imaging , Cerebral Hemorrhage/diagnostic imaging , Cerebral Hemorrhage/etiology
9.
J Neurol ; 271(5): 2631-2638, 2024 May.
Article En | MEDLINE | ID: mdl-38355868

BACKGROUND AND PURPOSE: In patients with acute ischemic stroke (AIS) treated with endovascular therapy (EVT), the association of pre-existing cerebral small vessel disease (cSVD) with symptomatic intracerebral hemorrhage (sICH) remains controversial. We tested the hypothesis that the presence of cerebral microbleeds (CMBs) and their burden would be associated with sICH after EVT of AIS. METHODS: We conducted a retrospective study combining cohorts of patients that underwent EVT between January 1st 2015 and January 1st 2020. CMB presence, burden, and other cSVD markers were assessed on a pre-treatment MRI, evaluated independently by two observers. Primary outcome was the occurrence of sICH. RESULTS: 445 patients with pretreatment MRI were included, of which 70 (15.7%) demonstrated CMBs on baseline MRI. sICH occurred in 36 (7.6%) of all patients. Univariate analysis did not demonstrate an association between CMB and the occurrence of sICH (7.5% in CMB+ group vs 8.6% in CMB group, p = 0.805). In multivariable models, CMBs' presence was not significantly associated with increased odds for sICH (-aOR- 1.19; 95% CI [0.43-3.27], p = 0.73). Only ASPECTs (aOR 0.71 per point increase; 95% CI [0.60-0.85], p < 0.001) and collaterals status (aOR 0.22 for adequate versus poor collaterals; 95% CI [0.06-0.93], p 0.019) were independently associated with sICH. CONCLUSION: CMB presence and burden is not associated with increased occurrence of sICH after EVT. This result incites not to exclude patients with CMBs from EVT. The risk of sICH after EVT in patients with more than10 CMBs will require further investigation. REGISTRATION: Registration-URL: http://www. CLINICALTRIALS: gov ; Unique identifier: NCT01062698.


Cerebral Hemorrhage , Ischemic Stroke , Thrombectomy , Humans , Male , Female , Aged , Cerebral Hemorrhage/diagnostic imaging , Cerebral Hemorrhage/etiology , Cerebral Hemorrhage/epidemiology , Ischemic Stroke/diagnostic imaging , Middle Aged , Retrospective Studies , Thrombectomy/adverse effects , Aged, 80 and over , Endovascular Procedures/adverse effects , Magnetic Resonance Imaging , Cerebral Small Vessel Diseases/diagnostic imaging , Cerebral Small Vessel Diseases/epidemiology , Cerebral Small Vessel Diseases/complications
10.
JAMA Netw Open ; 7(2): e2355368, 2024 Feb 05.
Article En | MEDLINE | ID: mdl-38363572

Importance: Intracerebral hemorrhage (ICH) is a serious complication of brain arteriovenous malformation (AVM). Apolipoprotein E (APOE) ε4 is a well-known genetic risk factor for ICH among persons without AVM, and cerebral amyloid angiopathy is a vasculopathy frequently observed in APOE ε4 carriers that may increase the risk of ICH. Objective: To assess whether APOE ε4 is associated with a higher risk of ICH in patients with a known AVM. Design, Setting, and Participants: This cross-sectional study including 412 participants was conducted in 2 stages (discovery and replication) using individual-level data from the UK Biobank (released March 2012 and last updated October 2023) and the All of Us Research Program (commenced on May 6, 2018, with its latest update provided in October 2023). The occurrence of AVM and ICH was ascertained at the time of enrollment using validated International Classification of Diseases, Ninth Revision and Tenth Revision, codes. Genotypic data on the APOE variants rs429358 and rs7412 were used to ascertain the ε status. Main Outcomes and Measures: For each study, the association between APOE ε4 variants and ICH risk was assessed among patients with a known AVM by using multivariable logistic regression. Results: The discovery phase included 253 UK Biobank participants with known AVM (mean [SD] age, 56.6 [8.0] years, 119 [47.0%] female), of whom 63 (24.9%) sustained an ICH. In the multivariable analysis of 240 participants of European ancestry, APOE ε4 was associated with a higher risk of ICH (odds ratio, 4.58; 95% CI, 2.13-10.34; P < .001). The replication phase included 159 participants with known AVM enrolled in All of Us (mean [SD] age, 57.1 [15.9] years; 106 [66.7%] female), of whom 29 (18.2%) sustained an ICH. In multivariable analysis of 101 participants of European ancestry, APOE ε4 was associated with higher risk of ICH (odds ratio, 4.52; 95% CI, 1.18-19.38; P = .03). Conclusions and Relevance: The results of this cross-sectional study of patients from the UK Biobank and All of Us suggest that information on APOE ε4 status may help identify patients with brain AVM who are at particularly high risk of ICH and that cerebral amyloid angiopathy should be evaluated as a possible mediating mechanism of the observed association.


Apolipoprotein E4 , Cerebral Hemorrhage , Intracranial Arteriovenous Malformations , Female , Humans , Male , Middle Aged , Apolipoprotein E4/genetics , Brain/blood supply , Cerebral Amyloid Angiopathy/complications , Cerebral Hemorrhage/etiology , Cerebral Hemorrhage/genetics , Cross-Sectional Studies , Intracranial Arteriovenous Malformations/complications
12.
J Stroke Cerebrovasc Dis ; 33(4): 107629, 2024 Apr.
Article En | MEDLINE | ID: mdl-38325675

OBJECTIVES: Our goal was to quantify the independent association of brain microbleeds with future intracranial hemorrhage (ICrH). Microbleed findings on brain magnetic resonance imaging (MRI) may identify distinctive risk factors for ICrH which could inform the anticoagulant therapy decision for atrial fibrillation (AF) patients. Our study design includes patients with MRIs for numerous reasons, not limited to evaluation of stroke. MATERIALS AND METHODS: The source population was all patients with AF from a nationwide Swedish health care register. Case patients had an ICrH between 2006 and 2013 and ≥1 brain MRI for an unrelated condition before the ICrH. Each case was matched to four controls who had a brain MRI without a subsequent ICrH. The MRIs were re-reviewed by neuroradiologists. Associations between MRI findings and subsequent ICrH were assessed using logistic regression, adjusting for comorbidities and antithrombotic medications. RESULTS: A total of 78 cases and 312 matched controls were identified; 29 cases and 79 controls had MRI sequences suitable for analysis of microbleeds. Patients with ≥10 microbleeds had a markedly increased risk of ICrH (adjusted odds ratio 14.56; 95 % confidence interval: 2.86-74.16, p < 0.001). All patients with ≥10 microbleeds had microbleeds in the lobar region and ≥10 lobar microbleeds was associated with intracerebral hemorrhages, univariable OR 8.54 (2.01-36.33), p = 0.004. CONCLUSIONS: Leveraging a nationwide database with brain imaging obtained prior to ICrH, we identified a strong association between ≥10 microbleeds on brain MRI and subsequent ICrH among AF patients. Lobar brain regions were involved whenever there were ≥10 microbleeds. Brain MRIs may help optimize the anticoagulation decision in selected AF patients.


Atrial Fibrillation , Stroke , Humans , Atrial Fibrillation/complications , Atrial Fibrillation/diagnostic imaging , Atrial Fibrillation/drug therapy , Case-Control Studies , Sweden/epidemiology , Intracranial Hemorrhages/etiology , Intracranial Hemorrhages/complications , Brain/pathology , Stroke/epidemiology , Cerebral Hemorrhage/etiology , Cerebral Hemorrhage/complications , Magnetic Resonance Imaging/adverse effects , Risk Factors
13.
World Neurosurg ; 184: e774-e783, 2024 Apr.
Article En | MEDLINE | ID: mdl-38354769

OBJECTIVE: This study aims to investigate independent factors associated with 30-day mortality in patients with acute spontaneous intracerebral hemorrhage (SICH) before treatment. METHODS: A retrospective analysis was performed on medical records of patients hospitalized with acute SICH between 2019 and 2021. Data included personal history, hospital stay duration, symptom onset, chief complaint, underlying diseases, medication, and alcohol/smoking habits. Physical examination records comprised baseline blood pressure, Glasgow Coma Scale assessment, and pupil reaction evaluation. Diagnostic imaging, specifically computed tomography brain scans, was examined for hemorrhage details. Multivariable logistic analysis was utilized for data analysis. RESULTS: Among 663 cases, 185 (27.9%) experienced mortality. Risk factors for mortality included chronic kidney disease, ischemic heart disease, loss of follow-up in hypertension clinic, and pontine hemorrhage. Conversely, motor response (m), reactive pupils, and basal cistern persistence significantly decreased the risk of mortality in multivariable analysis. Receiver operating characteristic analysis identified a m score of 5 as the cutoff for predicting survival. CONCLUSIONS: Chronic kidney disease, ischemic heart disease, loss of hypertension follow-up, m, reactive pupils, pontine hemorrhage, and basal cistern persistence were independent variables associated with the 30-day mortality rate in SICH patients before treatment initiation. A m, pupil reaction, and basal cistern persistence serve as predictive tools for assessing mortality in SICH before treatment.


Hypertension , Myocardial Ischemia , Renal Insufficiency, Chronic , Humans , Retrospective Studies , Hospital Mortality , Cerebral Hemorrhage/etiology , Risk Factors , Hypertension/complications , Renal Insufficiency, Chronic/complications
14.
J Neurosurg Pediatr ; 33(4): 315-322, 2024 Apr 01.
Article En | MEDLINE | ID: mdl-38181511

OBJECTIVE: Cerebral cavernous malformations (CCMs) are the second most common vascular anomaly affecting the CNS in children. Although stereotactic radiosurgery (SRS) has been proposed as an alternative to microsurgery in the management of selected cases in adults, there is a paucity of studies focusing on pediatric patients. The aim of this study was to present the outcomes and associated risks of SRS in this subgroup of patients. METHODS: This retrospective multicenter study included pediatric patients treated with single-session SRS for CCMs. The annual hemorrhage rate (AHR) was calculated before and after SRS in hemorrhagic lesions. The Engel classification was used to describe post-SRS epileptic control. Adverse radiation effects (AREs) and the occurrence of new neurological deficits were recorded. RESULTS: The study included 50 patients (median age 15.1 [IQR 5.6] years) harboring 62 CCMs. Forty-two (84%) and 22 (44%) patients had a history of hemorrhage or epilepsy prior to SRS, respectively. The AHR from diagnosis to SRS excluding the first hemorrhage was 7.19 per 100 CCM-years, dropping to 3.15 per 100 CCM-years after treatment. The cumulative risk of first hemorrhage after SRS was 7.4% (95% CI 0%-14.3%) at 5 years and 23.6% (95% CI 0%-42.2%) at 10 years. Eight hemorrhagic events involving 6 CCMs in 6 patients were recorded in the post-SRS follow-up period; 4 patients presented with transient symptoms and 4 with permanent symptoms. Of the 22 patients with pre-SRS seizures, 11 were seizure free at the last follow-up (Engel class I), 6 experienced improvement (Engel class II or III), 5 had no improvement (Engel class IVA or IVB), and 1 experienced worsening (Engel class IVC). Radiographic AREs were documented in 14.5% (9/62) of CCMs, with 4 being symptomatic. CONCLUSIONS: Single-session SRS reduces the CCM hemorrhage rate in the pediatric population and provides adequate seizure control.


Epilepsy , Hemangioma, Cavernous, Central Nervous System , Intracranial Arteriovenous Malformations , Radiosurgery , Adult , Child , Humans , Adolescent , Treatment Outcome , Radiosurgery/adverse effects , Hemangioma, Cavernous, Central Nervous System/diagnostic imaging , Hemangioma, Cavernous, Central Nervous System/surgery , Seizures/surgery , Epilepsy/surgery , Cerebral Hemorrhage/etiology , Retrospective Studies , Intracranial Arteriovenous Malformations/diagnostic imaging , Intracranial Arteriovenous Malformations/radiotherapy , Intracranial Arteriovenous Malformations/surgery , Follow-Up Studies
15.
J Neurol ; 271(5): 2423-2433, 2024 May.
Article En | MEDLINE | ID: mdl-38217707

BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common monogenic form of stroke and is characterised by early onset stroke and dementia. Most strokes are lacunar ischaemic strokes, but intracerebral haemorrhage (ICH) has also been reported, although there are limited published data on its frequency and characteristics. METHODS: A retrospective review of a prospectively recruited CADASIL register from the British National Referral clinic was performed to identify acute ICH cases and their characteristics. In addition, a systematic review of ICH in CADASIL was performed. MEDLINE (Pubmed), Embase, and Web of Science were searched for articles published from inception until 31/05/2023. RESULTS: Ten cases of ICH were identified from the National clinic register of 516 symptomatic patients, giving an estimated point prevalence of 1.9%. An additional 119 cases were identified from the systematic review, comprising 129 cases and 142 ICH events in total. Including all identified cases, the mean age at onset of ICH was 56.6 ± 15.7 (SD) years, and 74 (57.4%) were male. ICH was the first manifestation of the disease in 32 patients (38.1%), and ICH recurrence occurred in 16 (12.4%). Most ICHs were subcortical, with the thalamus, 58 (40.8%), and basal ganglia, 34 (23.9%), being the commonest sites. Anticoagulation, but not antiplatelet agents, was associated with an increased risk of ICH (20.0% vs. 1.9%, p = 0.006). CONCLUSIONS: ICH is a relatively rare manifestation of CADASIL, occurring in about 2% of symptomatic cases. Most of the haemorrhages occurred in the subcortical regions.


CADASIL , Cerebral Hemorrhage , Humans , CADASIL/epidemiology , CADASIL/complications , CADASIL/diagnostic imaging , Cerebral Hemorrhage/epidemiology , Cerebral Hemorrhage/diagnostic imaging , Cerebral Hemorrhage/etiology , Middle Aged , Male , Prevalence , Risk Factors , Female , Aged , Adult , Retrospective Studies
17.
J Neonatal Perinatal Med ; 17(1): 111-121, 2024.
Article En | MEDLINE | ID: mdl-38189714

BACKGROUND: To find the obstetrical and delivery associated risk factors of antenatal and postnatal grade III intraventricular hemorrhage (IVH) or periventricular hemorrhagic infarction (PVHI) in preterm neonates. METHODS: A retrospective study of obstetric and delivery associated risk factors included neonates (<35 gestational weeks) with severe IVH/PVHI (n = 120) and a prospectively collected control group (n = 50). The children were divided into: (1) antenatal onset group (n = 27) with insult visible on cerebral ultrasonography within the first 12 hours of birth or periventricular cystic changes visible in PVHI within the first 3 days; (2) neonatal onset group (n = 70) with insult diagnosed after initial normal findings or I-II grade IVH, and (3) unknown time-onset group (n = 23) with insult visible at > 12 h of age. RESULTS: The mothers of the antenatal onset group had significantly more bacterial infections before delivery compared to the neonatal onset group: 20/27 (74.1%) versus 23/69 (33.3%), (odds ratio (OR) 5.7 [95% confidence interval 2.1-16]; p = 0.0008) or compared to the control group (11/50 (22%); OR 11 [2.8-42]; p = 0.0005). Placental histology revealed chorioamnionitis more often in the antenatal compared to the neonatal onset group (14/21 (66.7%) versus 16/42 (38.1%), respectively; OR 3.7 [1.18-11]; p = 0.025). Neonates with neonatal development of severe IVH/PVHI had significantly more complications during delivery or intensive care. CONCLUSIONS: Bacterial infection during pregnancy is an important risk factor for development of antenatal onset severe IVH or PVHI. In neonates born to mothers with severe bacterial infection during pregnancy, cerebral ultrasonography is indicated for early detection of severe IVH or PVHI.


Bacterial Infections , Infant, Newborn, Diseases , Infant, Premature, Diseases , Infant, Newborn , Child , Female , Humans , Pregnancy , Retrospective Studies , Gestational Age , Placenta/pathology , Cerebral Hemorrhage/diagnostic imaging , Cerebral Hemorrhage/epidemiology , Cerebral Hemorrhage/etiology , Infarction/complications , Infarction/pathology , Infant, Premature, Diseases/diagnostic imaging , Infant, Premature, Diseases/epidemiology , Infant, Premature, Diseases/etiology
18.
Radiographics ; 44(2): e230117, 2024 Feb.
Article En | MEDLINE | ID: mdl-38206831

Perinatal venous infarcts are underrecognized clinically and at imaging. Neonates may be susceptible to venous infarcts because of hypercoagulable state, compressibility of the dural sinuses and superficial veins due to patent sutures, immature cerebral venous drainage pathways, and drastic physiologic changes of the brain circulation in the perinatal period. About 43% of cases of pediatric cerebral sinovenous thrombosis occur in the neonatal period. Venous infarcts can be recognized by ischemia or hemorrhage that does not respect an arterial territory. Knowledge of venous drainage pathways and territories can help radiologists recognize characteristic venous infarct patterns. Intraventricular hemorrhage in a term neonate with thalamocaudate hemorrhage should raise concern for internal cerebral vein thrombosis. A striato-hippocampal pattern of hemorrhage indicates basal vein of Rosenthal thrombosis. Choroid plexus hemorrhage may be due to obstruction of choroidal veins that drain the internal cerebral vein or basal vein of Rosenthal. Fan-shaped deep medullary venous congestion or thrombosis is due to impaired venous drainage into the subependymal veins, most commonly caused by germinal matrix hemorrhage in the premature infant and impeded flow in the deep venous system in the term infant. Subpial hemorrhage, an underrecognized hemorrhage stroke type, is often observed in the superficial temporal region, and its cause is probably multifactorial. The treatment of cerebral sinovenous thrombosis is anticoagulation, which should be considered even in the presence of intracranial hemorrhage. ©RSNA, 2024 Test Your Knowledge questions in the supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article.


Cerebral Veins , Intracranial Thrombosis , Stroke , Thrombosis , Infant, Newborn , Infant , Humans , Child , Cerebral Hemorrhage/etiology , Cerebral Veins/diagnostic imaging , Neuroimaging , Infarction/complications
19.
BMJ Case Rep ; 17(1)2024 Jan 09.
Article En | MEDLINE | ID: mdl-38199667

A woman in her 20s, who was pregnant, underwent a routine antenatal ultrasound revealing organised intraventricular haemorrhage and a vascular area in the left thalamic region. Fetal MRI raised suspicion of arteriovenous malformation (AVM). The baby was delivered at 36 weeks via C-section, and initial brain MRI suggested a possible neoplastic lesion.Cerebral angiography confirmed a large AVM involving the left choroid plexus with arterial feeders from the left anterior and posterior choroidal arteries and a large venous varix draining into the vein of Galen. Both arterial feeders were successfully embolised with Onyx and glue, achieving complete occlusion.Choroid plexus AVMs are rare and often lead to intraventricular or intraparenchymal haemorrhage. Surgical treatment has shown success, but endovascular management is effective, especially for small, deep-seated AVMs, offering a better prognosis.


Arteriovenous Malformations , Choroid Plexus , Female , Pregnancy , Infant , Humans , Choroid Plexus/diagnostic imaging , Arteriovenous Malformations/diagnostic imaging , Arteriovenous Malformations/therapy , Fetus , Prenatal Care , Cerebral Hemorrhage/diagnostic imaging , Cerebral Hemorrhage/etiology
20.
Acta Neurochir (Wien) ; 166(1): 17, 2024 Jan 17.
Article En | MEDLINE | ID: mdl-38231317

PURPOSE: In aneurysmal intracerebral hemorrhage (aICH), our review showed the lack of the patient's individual (i) timeline panels and (ii) serial brain CT/MRI slice panels through the aICH evacuation and neurointensive care until the final brain tissue outcome. METHODS: Our retrospective cohort consists of 54 consecutive aICH patients from a defined population who acutely underwent the clipping of a middle cerebral artery bifurcation saccular aneurysm (Mbif sIA) with the aICH evacuation at Kuopio University Hospital (KUH) from 2010 to 2019. We constructed the patient's individual timeline panels since the emergency call and serial brain CT/MRI slice panels through the aICH evacuation and neurointensive care until the final brain tissue outcome. The patients were indicated by numbers (1.-54.) in the pseudonymized panels, tables, results, and discussion. RESULTS: The aICH volumes on KUH admission (median 46 cm3) plotted against the time from the emergency call to the evacuation (median 8 hours) associated significantly with the rebleeds (n=25) and the deaths (n=12). The serial CT/MRI slice panels illustrated the aICHs, intraventricular hemorrhages (aIVHs), residuals after the aICH evacuations, perihematomal edema (PHE), delayed cerebral injury (DCI), and in the 42 survivors, the clinical outcome (mRS) and the brain tissue outcome. CONCLUSIONS: Regarding aICH evacuations, serial brain CT/MRI panels present more information than words, figures, and graphs. Re-bleeds associated with larger aICH volumes and worse outcomes. Swift logistics until the sIA occlusion with aICH evacuation is required, also in duty hours and weekends. Intraoperative CT is needed to illustrate the degree of aICH evacuation. PHE may evoke uncontrollable intracranial pressure (ICP) in spite of the acute aICH volume reduction.


Aneurysm , Middle Cerebral Artery , Humans , Brain , Cerebral Hemorrhage/diagnostic imaging , Cerebral Hemorrhage/etiology , Cerebral Hemorrhage/surgery , Disease Progression , Hematoma , Magnetic Resonance Imaging , Retrospective Studies , Tomography, X-Ray Computed
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