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BACKGROUND: Intracranial vessel tortuosity is a key component of dolichoectasia and has been associated with atherosclerosis and adverse neurologic outcomes. However, the evaluation of tortuosity is mainly a descriptive assessment. PURPOSE: To compare the performance of three automated tortuosity metrics (angle metric [AM], distance metric [DM], and distance-to-axis metric [DTA]) for detection of dolichoectasia and presence of segment-specific plaques. STUDY TYPE: Observational, cross-sectional metric assessment. POPULATION: 1899 adults from the general population; mean age = 76 years, female = 59%, and black = 29%. FIELD STRENGTH/SEQUENCE: 3-T, three-dimensional (3D) time-of-flight MRA and 3D vessel wall MRI. ASSESSMENT: Tortuosity metrics and mean luminal area were quantified for designated segments of the internal carotid artery, middle cerebral artery, anterior cerebral artery, posterior cerebral artery, vertebral artery, and entire length of basilar artery (BA). Qualitative interpretations of BA dolichoectasia were assessed based on Smoker's visual criteria. STATISTICAL TESTS: Descriptive statistics (2-sample t-tests, Pearson chi-square tests) for group comparisons. Receiver operating characteristics area under the curve (AUC) for detection of BA dolichoectasia or segment-specific plaque. Model inputs included 1) tortuosity metrics, 2) mean luminal area, and 3) demographics (age, race, and sex). RESULTS: Qualitative dolichoectasia was identified in 336 (18%) participants, and atherosclerotic plaques were detected in 192 (10%) participants. AM-, DM-, and DTA-calculated tortuosity were good individual discriminators of basilar dolichoectasia (AUCs: 0.76, 0.74, and 0.75, respectively), with model performance improving with the mean lumen area: (AUCs: 0.88, 0.87, and 0.87, respectively). Combined characteristics (tortuosity and mean luminal area) identified plaques with better performance in the anterior (AUCs ranging from 0.66 to 0.78) than posterior (AUCs ranging from 0.54 to 0.65) circulation, with all models improving by the addition of demographics (AUCs ranging from 0.62 to 0.84). DATA CONCLUSION: Quantitative vessel tortuosity metrics yield good diagnostic accuracy for the detection of dolichoectasia. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY STAGE: 2.
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Aterosclerosis , Placa Aterosclerótica , Insuficiencia Vertebrobasilar , Adulto , Humanos , Femenino , Anciano , Arteria Basilar , Imagen por Resonancia Magnética , Aterosclerosis/diagnóstico por imagen , Angiografía por Resonancia Magnética/métodosRESUMEN
BACKGROUND: Dolichoectasia is a rare arterial condition characterized by the dilatation, tortuosity, and elongation of cerebral blood vessels. The vertebrobasilar artery and internal carotid artery are the common sites of dolichoectasia. However, dolichoectasia of the branch arteries, such as the ophthalmic artery (OA), is extremely rare. To the best of our knowledge, this is the first case of ophthalmic dolichoectasia that was successfully treated with endovascular internal coil trapping. CASE PRESENTATION: A 54-year-old female patient presented with transient left ophthalmalgia and visual disturbance. Magnetic resonance imaging revealed a dilated and elongated left OA compressing the optic nerve at the entrance of the optic canal. However, a previous image that was taken 17 years back revealed that the OA was normal, which suggested the change in dolichoectasia was acquired. Cerebral angiography showed that the dilated and tortuous OA was running from the ophthalmic segment of the left internal carotid artery into the orbit. The symptoms could have been attributed to the direct compression of the dolichoectatic OA in the optic canal. A sufficient anastomosis between the central retinal artery and the middle meningeal artery was identified on external carotid angiography with balloon occlusion of the internal carotid artery. Endovascular treatment with internal trapping of the OA was performed due to ophthalmic symptom progression. Internal coil trapping of the OA was performed at the short segment between the OA bifurcation and the entrance of the optic canal. As expected, the central retinal artery was supplied via the middle meningeal artery after the treatment. The transient visual disturbance was immediately resolved. Ophthalmalgia worsened temporarily after the treatment. However, it completely resolved after several days of oral corticosteroid therapy. Postoperative angiography showed that the origin of the OA was occluded and that the OA in the optic canal was shrunk. The flow of the central retinal arteries via the middle meningeal artery was preserved. CONCLUSIONS: OA dolichoectasia is rare, and its pathogenesis and long-term visual prognosis are still unknown. However, endovascular therapy can improve symptom by releasing the pressure site in the optic canal.
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Procedimientos Endovasculares , Arteria Oftálmica , Femenino , Humanos , Persona de Mediana Edad , Arteria Oftálmica/diagnóstico por imagen , Arteria Oftálmica/cirugía , Arteria Carótida Interna/diagnóstico por imagen , Arteria Carótida Interna/cirugía , Angiografía Cerebral , Imagen por Resonancia Magnética , Dilatación PatológicaRESUMEN
OBJECTIVES: Intracranial arterial dolichoectasia (IADE) is characterized by the dilation, elongation, and tortuosity of intracranial arteries. We aimed to investigate the association between variations of the Circle of Willis (COW) and IADE in the general population, as well as estimate the genetic correlation between COW variations and IADE. METHODS: A total of 981 individuals from a population-based cohort were included. Brain magnetic resonance angiography was performed to assess COW variants and measure the diameters of intracranial arteries. IADE was defined as a total intracranial volume-adjusted diameter ≥ 2 standard deviations. Logistic regression models were used to analyze the association between COW variations and IADE. The heritability and genetic correlation were estimated using genome-wide complex trait analysis (GCTA) based on single nucleotide polymorphism (SNP) array data. RESULTS: The prevalence of IADE was 6.2â¯%. Hypoplastic/absent A1 segments were associated with an increase in contralateral ICA diameter (ß ± SE, 0.279 ± 0.049; p = 0.001) and a decrease in ipsilateral ICA diameter (ß ± SE, -0.300 ± 0.050; p = 0.001). Fetal-type posterior cerebral artery (FTP) was associated with a larger ICA diameter (ß ± SE, 0.326 ± 0.048; p = 0.001) and a smaller BA diameter (ß ± SE, -0.662 ± 0.043; p = 0.001). FTP revealed a positive genetic correlation with ICA dilation (rG = 0.259 ± 0.175; p = 0.0009) and a negative genetic correlation with BA dilation (rG = -0.192 ± 0.153, p = 0.015). CONCLUSIONS: There was an association between COW variations and larger intracranial arterial diameters in the general population. Genetic factors may play a role in the development of intracranial arterial dilation and the formation of COW variants.
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Círculo Arterial Cerebral , Predisposición Genética a la Enfermedad , Angiografía por Resonancia Magnética , Fenotipo , Polimorfismo de Nucleótido Simple , Humanos , Círculo Arterial Cerebral/anomalías , Círculo Arterial Cerebral/diagnóstico por imagen , Femenino , Masculino , Persona de Mediana Edad , Adulto , Factores de Riesgo , Prevalencia , Anciano , Angiografía Cerebral , Estudio de Asociación del Genoma Completo , Dilatación Patológica , Medición de Riesgo , Herencia , Estudios de Asociación Genética , Herencia MultifactorialRESUMEN
INTRODUCTION: Brain arterial diseases, including atherosclerosis, vasculitis, and dissections, are major contributors to cerebrovascular morbidity and mortality worldwide. These diseases not only increase the risk of stroke but also play a significant role in neurodegeneration and dementia. Clear and unambiguous terminology and classification of brain arterial disease phenotypes is crucial for research and clinical practice. MATERIAL AND METHODS: This review aims to summarize and harmonize the terminology used for brain large and small arterial phenotypes based on pathology studies and relate them to imaging phenotypes used in medical research and clinical practice. CONCLUSIONS AND RESULTS: Arteriosclerosis refers to hardening of the arteries but does not specify the underlying etiology. Specific terms such as atherosclerosis, calcification, or non-atherosclerotic fibroplasia are preferred. Atherosclerosis is defined pathologically by an atheroma. Other brain arterial pathologies occur and should be distinguished from atherosclerosis given therapeutic implications. On brain imaging, intracranial arterial luminal stenosis is usually attributed to atherosclerosis in the presence of atherosclerotic risk factors but advanced high-resolution arterial wall imaging has the potential to more accurately identify the underlying pathology. Regarding small vessel disease, arteriosclerosis is ambiguous and arteriolosclerosis is often used to denote the involvement of arterioles rather than arteries. Lipohyalinosis is sometimes used synonymously with arteriolosclerosis, but less accurately describes this common small vessel thickening which uncommonly shows lipid. Specific measures of small vessel wall thickness, the relationship to the lumen as well as changes in the layer composition might convey objective, measurable data regarding the status of brain small vessels.
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Arterias Cerebrales , Fenotipo , Humanos , Angiografía Cerebral , Arterias Cerebrales/diagnóstico por imagen , Arterias Cerebrales/patología , Enfermedades de los Pequeños Vasos Cerebrales/diagnóstico por imagen , Arteriosclerosis Intracraneal/diagnóstico por imagen , Valor Predictivo de las Pruebas , Pronóstico , Factores de Riesgo , Terminología como AsuntoRESUMEN
BACKGROUND: Tortuosity of intracranial arteries is rare and usually mistaken for arteriovenous malformations. In the world literature, the term «pure arterial malformations¼ is used to refer to this disease. OBJECTIVE: To summarize the experience of the Burdenko Neurosurgery Center on diagnosis of intracranial artery tortuosity, management and treatment of these patients, as well as to review appropriate literature data. MATERIAL AND METHODS: Tortuosity of intracranial arteries was detected in 11 patients (8 women and 3 men) aged 7-48 years who underwent outpatient or inpatient examination and treatment at the Burdenko Neurosurgery Center between 2009 and 2022. We analyzed angiographic, clinical and follow-up data of these patients, as well as appropriate literature data. RESULTS: According to angiography data, all patients had moderate dilatation, elongation and tortuosity of intracranial arteries without signs of arteriovenous shunting. The most common finding was tortuosity of several segments of internal carotid artery (5 cases). Lesion of PCA, PComA, MCA and ACA was less common. In 7 cases, the walls of the deformed vessels had calcified zones. In two cases, there were saccular aneurysms in the walls of the tortuous vessels. In one case, tortuosity was combined with kinking of the left subclavian artery, in another one - tortuosity of C1 segment of the right ICA. No patient had specific clinical manifestations. The follow-up period was 1-10 years in 7 patients. There were no changes in structure of tortuosity or appearance of new aneurysms. CONCLUSION: Tortuosity of intracranial arteries is an extremely rare disease with the highest incidence in young women. This abnormality has no specific clinical manifestations and does not require surgical or conservative treatment. Tortuosity of intracranial arteries should be differentiated from arterial dolichoectasia, fusiform aneurysms and AVMs.
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Aneurisma Intracraneal , Femenino , Humanos , Masculino , Arteria Carótida Interna , Angiografía Cerebral , Aneurisma Intracraneal/cirugíaRESUMEN
INTRODUCTION: Limited cross-sectional or case-control studies have identified the relationship between basilar artery (BA) curvature and posterior circulation infarction (PCI). This study aimed to identify the influence of BA curvature severity on the risk of PCI occurrence in patients without vertebrobasilar stenosis through a prospective cohort study. METHODS: In this study, we enrolled 171 patients with BA dolichosis but without vertebrobasilar stenosis. The BA geometric parameters were evaluated on MRA. The primary outcome was the occurrence of PCI, mainly referring to cerebellar and/or brainstem infarction. Cox proportional hazard models were used to detect possible predictors of PCI. RESULTS: Among them, 134 (78.4%) patients were diagnosed with BA curvature, including 124 with moderate curvature and 10 with prominent curvature. The defined PCI occurrence was observed in 32 (18.7%) patients with a median follow-up time of 45.6 months. Cox proportional hazard analysis showed that BA prominent curvature (HR = 6.09; 95% CI: 1.36-27.28; P = 0.018) significantly increased the risk of PCI occurrence, and bending length (BL) was also significantly associated with PCI occurrence, with the adjusted HR per 1-mm increase of BL of 1.09 (95% CI: 1.01-1.18; P = 0.040). In the subgroup analysis stratified by age, BA prominent curvature was highly associated with PCI occurrence in patients aged > 61 years (HR = 11.76; 95% CI: 1.21-113.90; P = 0.033). Additionally, good antiplatelet therapy adherence could significantly reduce the risk of PCI occurrence. CONCLUSION: BA curvature may increase the risk of PCI occurrence, especially in elderly patients with prominent curvature. Improving adherence to antiplatelet therapy can help reduce the risk of PCI occurrence.
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Infartos del Tronco Encefálico , Insuficiencia Vertebrobasilar , Anciano , Humanos , Persona de Mediana Edad , Arteria Basilar/diagnóstico por imagen , Estudios Prospectivos , Constricción Patológica , Estudios Transversales , Inhibidores de Agregación Plaquetaria/uso terapéutico , Insuficiencia Vertebrobasilar/complicaciones , Insuficiencia Vertebrobasilar/diagnóstico por imagen , Insuficiencia Vertebrobasilar/epidemiología , Infartos del Tronco Encefálico/complicaciones , Infartos del Tronco Encefálico/diagnóstico por imagen , Infartos del Tronco Encefálico/epidemiologíaRESUMEN
Previous studies have indicated that the small cerebellopontine angle (CPA) cistern plays a role in the pathogenesis of trigeminal neuralgia (TN), but they are likely not involved in TN associated with vertebrobasilar artery (VBA) compression because of its rarity. Forty-four patients with VBA-associated TN and 44 age-, sex-, and hypertension-matched TN patients without VBA compression (non-VBA-associated) were included. All patients underwent high-resolution MRI. The CPA cistern volumes were measured bilaterally. The presence of vertebrobasilar dolichoectasia (VBD) and laterality of the vertebrobasilar junction (VBJ) were observed. The CPA cistern volume on the affected side was smaller than the unaffected side (714.4 ± 372.8 vs 890.2 ± 462.2 mm3, p < 0.001) in non-VBA-associated TN patients, while VBA-associated TN patients show a larger CPA cistern on the affected side than the unffected side (1107.0 ± 500.5 vs 845.3 ± 314.8 mm3, p < 0.001). The prevalence of VBD was higher in patients with VBA-associated TN than in matched non-VBA-associated TN patients (90.9% vs 4.5%, p < 0.001). A positive correlation between the laterality of VBJ and the affected side was found in the VBA-associated TN group (p < 0.0001). Large CPA cistern may be a neuroradiological feature of VBA-associated TN, and most of the VBA-associated TN is accompanied by VBD. The presence of VBD and the lateral shift of VBJ may expand the CPA cistern by squeezing the surrounding tissue on the affected side and also increase the chance of VBA compression on the trigeminal nerve, resulting in the genesis of VBA-associated TN.
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Hipertensión , Neuralgia del Trigémino , Humanos , Neuralgia del Trigémino/diagnóstico por imagen , Neuralgia del Trigémino/cirugía , Ángulo Pontocerebeloso/diagnóstico por imagen , Ángulo Pontocerebeloso/cirugía , Nervio Trigémino , Lateralidad FuncionalRESUMEN
Percutaneous balloon compression (PBC) of Gasserian ganglion has been popularly used to treat trigeminal neuralgia (TN), one of the most painful syndromes in human experience. Vertebrobasilar dolichoectasia (VBD) is a rare cause of TN and remains challenging to treat. To our knowledge, no study has reported the therapeutic outcome of PBC for VBD-related TN (VBD-TN). In this retrospective study, we collected and analyzed the medical records of all patients undergoing PBC procedure for VBD-TN under the guidance of CT plus three-dimensional reconstruction at the Pain Management Center of Beijing Tiantan Hospital from January 2017 to December 2022. All 23 patients (15 men and 8 women) had a substantial pain relief as modified Barrow Neurological Institute (BNI) I-IIIb immediately after procedure. The follow-up duration ranged from 2 to 63 months, and at the last follow-up visit, only 3 patients (13%) relapsed (BNI IV-V). The cumulative recurrence-free survival was 95%, 87%, and 74% within 1, 3, and 5 years, respectively. Patients' reported satisfactory rate was 100% as Likert scale 4-5 throughout the whole follow-up period, with no severe complications occurring. Our data revealed promising efficacy and safety of PBC procedure for treatment of VBD-TN, thus suggesting a valuable option for pain control in these rare cases of TN. However, there has been no supporting evidence that PBC treatment is a preferred option to other treatments.
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Neuralgia del Trigémino , Insuficiencia Vertebrobasilar , Masculino , Humanos , Femenino , Neuralgia del Trigémino/etiología , Neuralgia del Trigémino/cirugía , Estudios Retrospectivos , Insuficiencia Vertebrobasilar/complicaciones , Insuficiencia Vertebrobasilar/cirugía , Tomografía Computarizada por Rayos X , Dolor , Resultado del TratamientoRESUMEN
OBJECTIVE: The aim of this study was to describe the efficacy, clinical outcomes, and complications of open cerebrovascular surgery, endovascular surgery, and conservative management of dolichoectatic vertebrobasilar aneurysms (DVBAs). METHODS: Relevant articles were retrieved from PubMed, Scopus, Web of Science, and Cochrane databases according to PRISMA guidelines. A meta-analysis was conducted for clinical presentation, treatment protocols, and clinical outcomes-good (improved or stable clinical status) or poor (deteriorated clinical status or death)-and mortality rates. RESULTS: The 9 identified articles described 41 cases (27.5%) of open cerebrovascular surgery, 61 endovascular procedures (40.9%), and 47 cases (31.5%) of conservative management for DVBAs. The total cohort had a good outcome rate of 51.9% (95% CI 28.3%-74.6%), a poor outcome rate of 45.5% (95% CI 23.0%-70.1%), and a mortality rate of 22.3% (95% CI 11.8%-38.0%). The treatment groups had comparable good clinical outcome rates (open cerebrovascular surgery group: 24.7% [95% CI 2.9%-78.2%]; endovascular surgery group: 69.0% [95% CI 28.7%-92.5%]; conservative management group: 57.7% [95% CI 13.0%-92.5%]; p = 0.19) and poor outcome rates (open vascular surgery group: 75.3% [95% CI 21.8%-97.1%]; endovascular surgery group: 27.2% [95% CI 5.6%-0.70.2%]; conservative management group: 39.9% [95% CI 9.1%-81.6%]; p = 0.15). The treatment groups also had comparable mortality rates (open vascular surgery group: 39.5% [95% CI 11.4%-76.8%]; endovascular surgery group: 15.8% [95% CI 4.4%-43.0%]; conservative management group: 19.2% [95% CI 6.8%-43.5%]; p = 0.23). CONCLUSIONS: The current study of DVBAs illustrated poor outcomes and high mortality rates regardless of the treatment modality. The subgroup analysis showed heterogeneity among the subgroups and advice for personalized management.
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Procedimientos Endovasculares , Aneurisma Intracraneal , Humanos , Aneurisma Intracraneal/cirugía , Resultado del Tratamiento , Procedimientos Endovasculares/métodosRESUMEN
OBJECTIVE: To explore and analyze the clinical efficacy of a stepwise decompression technique in the treatment of trigeminal neuralgia (TN) caused by vertebrobasilar dolichoectasia (VBD). METHODS: A total of 918 patients with TN admitted to our hospital from June 2015 to May 2020 were divided into the VBD group (n = 61) and the conventional group (n = 857). The VBD group underwent stepwise decompression, while the conventional group received traditional microvascular decompression (MVD) surgery. Patients associated with the VBD were divided into direct compression (n = 14) and indirect compression group (n = 47) who had compression by other vessels in the presence of the VBD. Thereafter, the clinical data, intraoperative findings, efficacy, and complications were analyzed. RESULTS: The curative ratio, efficacy, and recurrence rate for the VBD and conventional group were 83.6% and 89.6%, 93.4% and 95.3%, and 8.2% and 5.3%, respectively. The curative ratio, efficacy, and recurrence rate for the direct compression and indirect compression group were 85.7% and 83.0%, 92.9% and 93.6%, and 7.1% and 8.5%, separately. There were no significant differences in the curative ratio, efficacy, recurrence rate, and the incidence of complications between the two series (P > 0.05). CONCLUSION: For TN caused by VBD, stepwise decompression not only reduces the direct compression of the trigeminal nerve by VBA but also allows identification and decompression of the actual vessels responsible for the compression. It has a good curative ratio, efficacy, and long-term pain relief rate, and it does not significantly increase the incidences of complications after surgery.
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Cirugía para Descompresión Microvascular , Neuralgia del Trigémino , Insuficiencia Vertebrobasilar , Humanos , Neuralgia del Trigémino/cirugía , Neuralgia del Trigémino/complicaciones , Insuficiencia Vertebrobasilar/complicaciones , Insuficiencia Vertebrobasilar/diagnóstico por imagen , Insuficiencia Vertebrobasilar/cirugía , Nervio Trigémino/cirugía , Cirugía para Descompresión Microvascular/efectos adversos , Resultado del Tratamiento , Descompresión/efectos adversos , Estudios RetrospectivosRESUMEN
INTRODUCTION: Hypersomnia is a condition in which a person has trouble staying awake during the day. There are several potential causes of it, including sleep apnea and sleep disorders. CASE PRESENTATION: A 43 year old male was referred to our practice with complaints of hypersomnia, snoring, slurred speech and sleep apnea for more diagnostic and therapeutic interventions. His brain MRI was significant for a vascular loop compression on medulla oblongata. The patient underwent microvascular decompression surgery subsequently and showed improvement in all of his symptoms. CONCLUSION: One of the rare causes of sleep apnea is medulla oblongata compression by a vascular loop. Vertebrobasilar dolichoectasia may cause this phenomenon probably and should be reviewed in imaging examinations more precisely. Microvascular decompression by using a synthetic Teflon patch may be helpful in management of these patients.
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Trastornos de Somnolencia Excesiva , Cirugía para Descompresión Microvascular , Síndromes de la Apnea del Sueño , Masculino , Humanos , Adulto , Arteria Vertebral/diagnóstico por imagen , Arteria Vertebral/cirugía , Cirugía para Descompresión Microvascular/métodos , Bulbo Raquídeo/diagnóstico por imagen , Bulbo Raquídeo/cirugía , Síndromes de la Apnea del Sueño/cirugía , Trastornos de Somnolencia Excesiva/cirugíaRESUMEN
INTRODUCTION: Dolichoectatic vessels can cause cranial nerve dysfunction by either direct compression or ischemia. Abducens nerve palsy due to neurovascular compression by elongated, enlarged, tortuous or dilated arteries is an uncommon but important cause. AIM: To highlight neurovascular compression as a cause of abducens nerve palsy and discuss various diagnostic techniques. METHODS: Manuscripts were identified using the National Institutes of Health PubMed literature search system. Search terms included abducens nerve palsy, neurovascular compression, dolichoectasia and arterial compression. Inclusion criteria required that the articles were written in English. RESULTS: The literature search identified 21 case reports where abducens nerve palsy was due to vascular compression. Out of these 18 patients were male and the mean age was 54 years. Eight patients had unilateral right abducens nerve involvement; eleven patients had unilateral left nerve involvement and two patients had bilateral involvement. The arteries causing the compression were basilar, vertebral and anterior inferior cerebellar arteries. A compressed abducens nerve is not usually clearly detected on CT (Computed Tomography) or MRI (Magnetic Resonance Imaging). MRA (Magnetic Resonance Angiography), Heavy T2- WI (weighted imaging), CISS (constructive interference in steady state) and FIESTA (Fast Imaging Employing Steady-state Acquisition) are essential to demonstrate vascular compression of the abducens nerve. The various treatment options included controlling hypertension, glasses with prisms, muscle resection and microvascular decompression.
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Enfermedades del Nervio Abducens , Insuficiencia Vertebrobasilar , Humanos , Masculino , Persona de Mediana Edad , Femenino , Enfermedades del Nervio Abducens/diagnóstico , Enfermedades del Nervio Abducens/etiología , Enfermedades del Nervio Abducens/terapia , Insuficiencia Vertebrobasilar/complicaciones , Insuficiencia Vertebrobasilar/diagnóstico por imagen , Insuficiencia Vertebrobasilar/terapia , Nervio Abducens , Arteria Basilar/diagnóstico por imagen , Arteria Basilar/patología , Imagen por Resonancia Magnética/métodosRESUMEN
Our objective was to investigate brain structure, cerebral vasculature, and cognitive function in a cohort of patients with late-onset Pompe disease, with particular reference to the differences from those with the classic infantile phenotype, where extensive white-matter abnormalities (WMA) and impaired cognition on long-term enzyme treatment are reported in a subset of patients. Brain imaging (T1, T2, T2 fluid-attenuated inversion recovery, susceptibility-weighted images, and magnetic resonance angiography-time of flight) was combined with extensive cognitive testing of general intelligence (Wechsler IQ Test, Montreal Cognitive Assessment [MoCA]) and specific neuropsychological domains (verbal fluency, cognitive flexibility, attention, memory, and visuospatial abilities). We included 19 patients with late-onset Pompe disease (age range 11-56 years). Two patients showed mild punctate WMA within normal range for age, with a Fazekas score (FS) of 1 to 2. Magnetic resonance angiography revealed a slight vertebrobasilar dolichoectasia in two patients yet did not show any aneurysms or vascular dissections. Most patients had age-adjusted scores within the normal range for the Wechsler index scores (verbal comprehension, perceptual reasoning, working memory, and processing speed) and combined total intelligence (IQ) score (median 101, interquartile range 91-111; one patient had a below-average score for total IQ) as well as for the specific domains verbal fluency, attention, and memory. A subset of patients performed suboptimally on the Rey Complex Figure Test (9/14 patients) or cube-copying/clock-drawing test of the MoCA (8/10 patients). We therefore concluded that our study showed no brain abnormalities, other than minor microvascular lesions considered within normal range for age, nor general cognitive impairment in late-onset Pompe patients. These findings are in sharp contrast with the widespread WMA and cognitive problems found in some classic infantile patients.
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Enfermedad del Almacenamiento de Glucógeno Tipo II , Encéfalo/patología , Cognición , Enfermedad del Almacenamiento de Glucógeno Tipo II/patología , Humanos , Pruebas de Inteligencia , Imagen por Resonancia Magnética , Pruebas NeuropsicológicasRESUMEN
BACKGROUND: Although inflammation is found to be related to arteriopathy pathogenesis, it is yet to be determined the distinct correlations of specific inflammatory biomarker types contributing to different cerebral large vessel diseases. We aimed to investigate the association between multiple inflammatory biomarkers and cerebral atherosclerosis and dolichoectasia in a community-based sample. METHODS: A total of 960 participants of the Shunyi study were included. A panel of 14 circulatory inflammatory biomarkers was assessed and then grouped in three sets as systemic, endothelial-related, and media-related inflammation, based on underlying different inflammatory cascades. Intracranial atherosclerotic stenosis (ICAS), dolichoectasia estimated by magnetic resonance angiography, and carotid plaques estimated by ultrasound were also performed. RESULTS: Endothelial-related inflammatory group was related to the presence of ICAS (R2 = 0.215, p = 0.024) and carotid plaques (R2 = 0.342, p = 0.013). Backward stepwise elimination showed that E-selectin was prominent (ß = 0.67, 95% CI: 0.54-0.85, p = 0.001; ß = 0.79, 95% CI: 0.68-0.93, p = 0.005). Systemic inflammatory group was associated with an increased basilar artery diameter (R2 = 0.051, p < 0.001), and backward stepwise elimination showed that IL-6 was prominent (ß = 0.07, 95% CI: 0.03-0.11, p < 0.001). CONCLUSION: Different types of inflammatory biomarkers were associated with atherosclerosis and dolichoectasia, respectively, implying dissimilar inflammatory processes. Further confirming of their distinct anti-inflammatory roles as potential therapeutic targets is warrant.
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Aterosclerosis , Arteriosclerosis Intracraneal , Aterosclerosis/complicaciones , Aterosclerosis/diagnóstico por imagen , Aterosclerosis/patología , Arteria Basilar , Biomarcadores , Humanos , Inflamación/complicaciones , Inflamación/diagnóstico por imagen , Inflamación/patología , Arteriosclerosis Intracraneal/complicaciones , Arteriosclerosis Intracraneal/diagnóstico por imagenRESUMEN
INTRODUCTION: Dolichoectasia is a form of brain large artery disease associated with a high risk of mortality and morbidity. Progressive enlargement of arterial size is a predictor of mortality, but there are no specific treatments for arresting or slowing down dilatation. Additionally, dilated brain arteries can cause flow stagnation, which can trigger thrombosis and cause stroke. Pathology and genetic studies indicate a possible role for increased matrix metalloproteinase activation in arterial dilatation and thus in the pathophysiology of dolichoectasia. Therefore, therapeutic interventions aimed at slowing down arterial dilatation and preventing thrombosis could hypothetically play a role in treating patients with dolichoectasia. METHODS: We present four patients with dolichoectasia that exemplify therapeutic challenges worth discussing in the context of the current literature. Two patients were treated off-label with doxycycline (based on its antiMMP properties) and with apixaban, one patient was put on warfarin and later switched to aspirin, and the fourth patient underwent endovascular treatment. RESULTS: We report four cases, all men 50 years or older. Of the two patients treated with doxycycline, we noted a slowdown of the basilar artery (BA) growth, but the BA continued to grow in the other patient. Of the two patients who received apixaban, none had a subsequent stroke in 5 and 4 years of follow-up, respectively. One patient was admitted with a fatal BA thrombosis and rupture, and pathological examination of the brain arteries demonstrated advanced arterial wall degeneration but no atherosclerosis. DISCUSSION: These cases exemplify the challenges of treating people with dolichoectasia and highlight the need for better evidence regarding the best possible treatment for this population.
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Enfermedades Arteriales Intracraneales , Accidente Cerebrovascular , Insuficiencia Vertebrobasilar , Arteria Basilar , Dilatación Patológica/complicaciones , Doxiciclina , Humanos , Enfermedades Arteriales Intracraneales/complicaciones , Enfermedades Arteriales Intracraneales/patología , Masculino , Accidente Cerebrovascular/complicaciones , Insuficiencia Vertebrobasilar/complicaciones , Insuficiencia Vertebrobasilar/diagnóstico por imagen , Insuficiencia Vertebrobasilar/tratamiento farmacológicoRESUMEN
Trigeminal neuralgia (TN) due to vertebrobasilar dolichoectasia (VBD) is a rare disease that can be challenging to treat. The objectives of this study are to investigate the characteristics of patients with TN due to VBD and to analyze the efficacy of microvascular decompression (MVD) by the interposition method for treatment of the condition. From 2010 until 2020, the data of 30 patients with TN due to VBD who were treated with MVD by the interposition method were analyzed retrospectively. The characteristics of the patients were compared with those of patients with non-VBD TN (n = 815). Kaplan-Meier survival analysis was performed to determine pain-free survival. The 30 patients (21 males, 9 females; mean age, 63.03 years) accounted for 3.55% of all patients with TN during the study period. In 30 patients, the offending vessel was the basilar artery (BA) in 1 patient, the vertebral artery (VA) in 6 patients, the VA plus the superior cerebellar artery (SCA) in 6 patients, the VA plus the anterior inferior cerebellar artery (AICA) in 12 patients, and the VA + SCA + AICA in 5 patients. Compared to non-VBD TN patients, those with TN due to VBD were significantly more likely to be male, to have TN of the left side, and to have hypertension (all P < 0.001). Mean age at surgery (P = 0.057) and symptom duration (P = 0.308) were comparable between the two groups. All 30 patients had immediate relief of facial pain after MVD and could stop medication. There were no postoperative complications. Over mean follow-up of 76.67 months, 3 patients had recurrence. The mean duration of pain-free survival was 70.77 months. In conclusions, TN due to VBD appears to be more likely in males, in those with hypertension, and to involve the left side. The interposition method performed by experienced and skilled neurosurgeons is a safe and effective treatment for TN due to VBD. Further studies are needed to analyze the associated long-term results and the pain recurrence rate among this special population.
Asunto(s)
Hipertensión , Cirugía para Descompresión Microvascular , Neuralgia del Trigémino , Insuficiencia Vertebrobasilar , Femenino , Humanos , Hipertensión/complicaciones , Masculino , Cirugía para Descompresión Microvascular/métodos , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del Tratamiento , Neuralgia del Trigémino/etiología , Neuralgia del Trigémino/cirugía , Insuficiencia Vertebrobasilar/complicaciones , Insuficiencia Vertebrobasilar/cirugíaRESUMEN
BACKGROUND: Giant dolichoectatic basilar trunk aneurysms have an unfavorable natural history and are associated with high morbidity, but their neurosurgical treatment is complex and challenging. METHODS: Flow reversal reconstruction with fourth-generation bypass and proximal vertebral artery clip occlusion is performed via orbitozygomatic craniotomy with the Kawase approach under rapid ventricular pacing. CONCLUSION: Fourth-generation bypass is an innovative, technically challenging, and clinically effective tool in the treatment armamentarium for giant dolichoectatic basilar trunk aneurysms.
Asunto(s)
Arteria Basilar , Aneurisma Intracraneal , Humanos , Arteria Basilar/diagnóstico por imagen , Arteria Basilar/cirugía , Aneurisma Intracraneal/diagnóstico por imagen , Aneurisma Intracraneal/cirugía , Arteria Vertebral/cirugía , Microcirugia , Procedimientos NeuroquirúrgicosRESUMEN
OBJECTIVE: This study investigates the differences and changing trend of posterior circulation blood perfusion between different levels of vertebrobasilar dolichoectasia(VBD) patients. The relationship between the deviation of the basilar artery(BA) in different directions and the location of pontine infarction are also investigated. METHODS: A cohort of 106 patients(74 males and 32 females) who satisfied the diagnostic criteria for VBD were recruited for this study and classified according to the bifurcation height and the deviation position of the BA, as well as the measured blood perfusion value of the pontine, which includes rCBF, rCBV, MTT, and TTP. RESULTS: Out of the 106 patients, 19 cases were classified as Level 1, 74 cases were classified as Level 2, and 13 cases were classified as Level 3. The different levels between the VBD groups were statistically significant (P<0.05, P<0.01), and it was found that as the level increases, rCBF and rCBV gradually decreased, while MTT and TTP gradually increased. The statistic results of different perfusion parameters were also significant, when pairwise comparisons between Level 1 and Level 3, and Level 2 and Level 3 were performed. However, when comparing Level 1 and Level 2, only the TTP showed significant result. Among 106 patients, 22 cases had brainstem infarction, 13 cases had left brainstem infarction, 8 cases had right brainstem infarction, and 1 case had brainstem infarction on both sides. Brainstem infarction generally occurs on the opposite side of the direction of BA deviation(P<0.05). Regardless of the BA was deviated to the left or right, perfusion analysis showed that there was significant difference in blood perfusion on both sides of the pontine when BA is deviated(P<0.05, P<0.01). The rCBF and rCBV on the contralateral side of deviation were lower than those on the same side, and the MTT and TTP were longer than those on the same side. There were 37 cases with vertebral artery dominance(VAD), 16 cases with left VAD, and 21 cases with right VAD. Statistical analysis showed that BA was more likely to deflect to the opposite side of the dominant artery(P<0.05), and compared with non-VAD, there was no significant difference in pontine blood perfusion (p>0.05). CONCLUSION: As VBD level increases, rCBF and rCBV will gradually decreases while MTT and TTP showed sign of increasing. The location of brainstem infarction is opposite to the direction of the BA deviation, and BA is more likely to deviate to the opposite side of the dominant artery.
Asunto(s)
Infartos del Tronco Encefálico , Insuficiencia Vertebrobasilar , Arteria Basilar/diagnóstico por imagen , Infartos del Tronco Encefálico/diagnóstico por imagen , Femenino , Humanos , Masculino , Perfusión , Arteria Vertebral/diagnóstico por imagen , Insuficiencia Vertebrobasilar/diagnóstico por imagenRESUMEN
INTRODUCTION: The abnormalities in the vertebrobasilar arterial system such as enlarged, tortuous or dilated arteries and aneurysms can generate pressure and distortion of brain structures especially in the medulla and pons. This brainstem compression by abnormal arteries can lead to various sleep disorders such as sleep related hypoventilation, central sleep apnea, obstructive sleep apnea, and positional sleep apnea. AIM: To highlight brainstem compression due to vascular abnormalities as a cause of sleep disordered breathing. METHODS: Manuscripts were identified using the National Institutes of Health PubMed literature search system. Search terms included sleep disorders, vertebrobasilar dolichoectasia, tortuous vertebral arteries, vertebrobasilar aneurysms, vascular compression and brainstem compression. Inclusion criteria required that the articles were written in English, highlighted both a sleep disorder and a vascular abnormality. RESULTS: The literature search resulted in 10 case reports where vascular abnormalities were leading to sleep disorders. Out of these: four patients had central sleep apnea; three had sleep related hypoventilation; one had positional central sleep apnea; one had positional central apnea along with obstructive sleep apnea; and one had obstructive sleep apnea. The various vascular lesions identified were: megadolichobasilar artery; dolichoectatic vertebral artery; fusiform vertebrobasilar aneurysm; tortuous and elongated vertebral arteries; calcified vertebral artery and medullary telangiectasia. CONCLUSIONS: Arterial compression of the respiratory centers in the pons and medulla can lead to central sleep apnea. Obstructive sleep apnea can also result from vascular compression of the medulla. The proposed mechanism is compression of nucleus ambiguus which controls pharyngeal tone. Pressure on cranial nerves IX and X can lead to pharyngeal dysfunction leading to occlusive apnea.
Asunto(s)
Aneurisma , Apnea Central del Sueño , Apnea Obstructiva del Sueño , Insuficiencia Vertebrobasilar , Humanos , Hipoventilación/complicaciones , Apnea Central del Sueño/complicaciones , Insuficiencia Vertebrobasilar/complicaciones , Insuficiencia Vertebrobasilar/diagnóstico por imagenRESUMEN
OBJECTIVE: To systematize the data on diagnosis and treatment of non-saccular vertebrobasilar aneurysms. MATERIAL AND METHODS: We have analyzed modern and fundamental studies in the PubMed database for the period 1969-2020. The following keywords were used: non-saccular aneurysm, dolichoectasia, vertebrobasilar pool. Fifty-nine studies including 1 meta-analysis, 9 case reports, 24 reviews and 25 original articles were selected. RESULTS: Analysis of literature data indicates various unresolved problems associated with difficult diagnosis and choice of treatment that results high rates of disability and mortality. CONCLUSION: Data on anatomical features of non-saccular aneurysms, such as presence or absence of perforating arteries within the affected arterial segment, analysis of electrophysiological changes following brainstem compression by aneurysm can serve as factors that will improve treatment outcomes in these patients.