Intracranial vasculopathy: an important organ damage in young adult patients with late-onset Pompe disease.

BACKGROUND: Late-onset Pompe disease (LOPD) is mainly characterized by progressive limb-girdle muscle weakness and respiratory impairment, whereas stroke and cerebrovascular abnormalities have been insufficiently studied in LOPD. This study aimed to evaluate the frequency and pattern of intracranial artery and brain parenchyma abnormalities in LOPD patients. RESULTS: Neuroimaging data from 30 Chinese adult LOPD patients were collected from our center. Seven patients (7/30) had acute cerebral infarction or hemorrhage. Brain magnetic resonance angiography (MRA) or computed tomography angiography (CTA) revealed artery abnormalities in 23 patients (23/30). Dilative arteriopathy was found in 19 patients (19/30), with vertebrobasilar dolichoectasia found in 17 patients and dilatation of the anterior circulation arteries found in 8 patients. The maximum diameter of the basilar artery was correlated with disease duration (p < 0.05). In addition, aneurysms (7/30) and fenestrations (3/30) were discovered. There were 14 patients with arterial stenosis (14/30), and both anterior and posterior circulation involvement occurred in 9 patients (9/14). Stenosis and dilative arteriopathy simultaneously occurred in 10 patients (10/30). White matter hyperintensities were present in 13 patients (13/28). Microbleeds, predominantly located in the cerebellum and brainstem, were detected in 7 patients (7/22) via susceptibility-weighted imaging. CONCLUSIONS: Intracranial vasculopathy involving both large arteries and small vessels is an important organ damage in LOPD patients. LOPD should be considered a key differential diagnosis in young adults with cryptogenic stroke, and a series of imaging evaluations of the brain and intracranial blood vessels is recommended as a routine workup in adult LOPD patients.

Left Hemifacial Spasms Due to Left Vertebrobasilar Dolichoectasia.

Hemifacial spasm (HFS) arises from involuntary, recurrent, irregular tonic-clonic-like contractions of the muscles innervated by the facial nerve. Typically, compression of the facial nerve root exit on the same side is attributed to either a vascular loop or a mass located in the cerebellopontine (CP) angle. Dolichoectasia, alternatively termed dilated arteriopathy, is characterized by arterial dilatation, elongation, and tortuosity. Here, we present a case involving vertebrobasilar dolichoectasia (VBD) as the cause of HFS, alongside relevant imaging findings.

Conservative treatment in hemifacial spasm due to vertebrobasilar dolichoectasia.

Primary or secondary hemifacial spasm (HFS) can be caused by a variety of conditions, one of which is caused by neurovascular contact with a vertebrobasilar dolichoectasia (VBD). Microvascular decompression (MVD) had been known for the treatment of neurovascular contact that gives best outcome, however there were still limitations which surgery cannot be performed. In that case, conservative treatment plays essential role. Our case reported A 69-year-old man with chief complaint right HFS for four years that getting better with conservative treatment (blood pressure management and clonazepam oral).

Validation of bedside manual versus automated measurements of brain arterial diameters from MR angiography.

BACKGROUND AND PURPOSE: Brain arterial luminal diameters are reliably measured with automated imaging software. Nonautomated imaging software alternatives such as a Picture Archiving Communication System are more common bedside tools used for manual measurement. This study is aimed at validating manual measurements against automated methods. METHODS: We randomly selected 600 participants from the Northern Manhattan Study (NOMAS) and 260 participants from the Atahualpa Project studied with 1.5 Tesla MR angiography. Using the Radiant measuring tool, three independent readers (general practitioner, neurology resident, and vascular neurologist) measured manually the diameter of arterial brain vessels. The same vessels were also measured by LKEB Automated Vessel Analysis (LAVA). We calculated the intraclass correlation coefficient (ICC) of each rater's diameters versus those obtained with LAVA. RESULTS: The ICC between diameters obtained by the general practitioner or the neurology resident compared to LAVA was excellent for both internal carotid arteries (ICA) and Basilar Arteries (BA) (ICC > .80 in all comparisons) in NOMAS. In the Atahualpa Project, ICC between diameters obtained by a vascular neurologist and LAVA was good for both ICA and BA (ICC > .60 in all comparisons). The ICCs for the measurements of the remaining arteries were moderate to poor. CONCLUSION: Results suggest that manual measurements of ICA and BA diameters, but not MCA or ACA, are valid and could be used to identify dilated brain arteries at the bedside and for eventual selection of patients with dolichoectasia into clinical trials.

[Pathological tortuosity of intracranial arteries (pure arterial malformation) - diagnosis and management tactics]. / Patologicheskaya izvitost' intrakranial'nykh arterii (arterial'naya mal'formatsiya) ­ diagnostika i taktika vedeniya.

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.

Dolichoectasia of the ophthalmic artery: a case report on the treatment strategy in endovascular therapy and literature review.

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.

Pathology-based brain arterial disease phenotypes and their radiographic correlates.

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.

Glossopharyngeal neuralgia due to vertebrobasilar dolichoectasia: a case report.

Glossopharyngeal neuralgia due to vertebrobasilar dolichoectasia is a rare form of neuropathic pain, and presents diagnostic and therapeutic challenges. Clinical presentation: A 67-year-old man presented with severe burning pain in the left oral cavity, with no explanatory findings during dental and ear, nose, and throat evaluations. Temporomandibular joint examination revealed tenderness, and panoramic radiographs showed a noncontributory periapical radiolucency. Magnetic resonance imaging/magnetic resonance angiography revealed abnormally tortuous vertebral arteries compressing the glossopharyngeal nerves and the brainstem. Topical lidocaine reduced pain, confirming glossopharyngeal neuralgia. Carbamazepine was initially ineffective, but at 200 mg pain reduced from 90 to 20 on the visual analog scale. The patient requested and underwent microvascular decompression surgery, which eliminated his pain. Conclusion: When the vertebral artery compresses the glossopharyngeal nerve, the pain is more intense, attributed to its thicker vascular structure. Local anesthetic testing aids in identifying glossopharyngeal neuralgia. Dental practitioners must be skilled in diagnostics and possess anatomical knowledge for accurate evaluation and referral of throat and ear pain.

Vertebrobasilar dolichoectasia to trigeminal neuralgia: case report.

Vertebrobasilar dolichoectasia (VBD) is a dilated arterial disease with a diameter ˃4.5 mm. Trigeminal neuralgia (TN) is chronic neuropathic pain. Through the diagnosis and treatment of this case we believe that there is a significant risk associated with the endovascular treatment of VBD. The development of post-operative complications caused some functional impairment to the patient, but the improvement in TN symptoms with this endovascular treatment was unexpected. This treatment procedure was considered to be possibly related to the alteration of the tortuous path of the vessels, changing their course, allowing the displacement of vascular compression in the trigeminal root entry zone, and possibly also altering the hemodynamics of the posterior circulation, improving the progression of ischemia and hypoxia-induced demyelination of the trigeminal nerve. Due to the low incidence of this disease, there are not enough large sample studies for systematic statistical analysis.

Relationships between intracranial arterial dolichoectasia and small vessel disease in patients with ischaemic stroke: a systematic review and meta-analysis.

BACKGROUND: Intracranial arterial dolichoectasia (IADE) is a common arterial finding of dilation, elongation, or both, affecting large intracranial vessels, and associated with vascular risk factors, including hypertension. Associations of IADE with neuroimaging cerebral small vessel disease (CSVD) may be relevant for diagnosis and prognosis in patients with stroke. The study aimed to conduct an updated systematic review and meta-analysis of observational studies to investigate the relationships of IADE with well-defined CSVD markers in patients with ischaemic stroke. METHODS: We systematically searched PubMed, Embase, and Scopus for studies on IADE in ischaemic stroke patients with fulfilling predefined inclusion criteria. We pooled data to conduct a meta-analysis to compare the prevalence of SVD markers between patients with and without IADE groups using risk ratios (RRs) and 95% confidence intervals (CIs). RESULTS: From 157 retrieved abstracts, we included six studies from seven publications comprising 6102 patients with ischaemic stroke. The mean age of patients was 52.8 years, and 3691 (60.5%) were male. IADE was diagnosed in 11.4% (95% CI 8.9-13.9) (761) of included patients; 51.8% (3160) had hypertension. Compared to patients without IADE, individuals diagnosed with IADE had a significantly increased prevalence of lacune (RR 1.67, 95% CI 1.36-2.06, P < 0.01, I2 = 0.00%), cerebral microbleeds (CMBs) (RR 2.56, 95% CI 1.53-4.28, P < 0.01, I2 = 84.95%) and white matter hyperintensities (WMHs) (RR 2.17, 95% CI 1.84-2.56, P < 0.01, I2 = 0.00%). CONCLUSIONS: In patients with ischaemic stroke, IADE is associated with a higher prevalence of CSVD markers, including lacunes, CMBs, and WMHs. Further studies are needed to clarify the mechanisms underlying these associations and their potential relevance for the understanding, diagnosis, and treatment of CSVD.

Detection of Dolichoectasia and Atherosclerosis by Automated MRA Tortuosity Metrics in a Population-Based Study.

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.

Modified Tongqiao Huoxuetang Down-regulates PI3K/Akt Pathway to Treat Basilar Artery Dolichoectasia / 中国实验方剂学杂志

ObjectiveTo establish a mouse model of basilar artery dolichoectasia （BAD） and explore the mechanism of modified Tongqiao Huoxuetang （JTQHX） in regulating BAD via phosphatidylinositol 3-kinase （PI3K）/protein kinase B （Akt） pathway. MethodSixty C57/BL6 female mice were randomized into sham operation （injected with 10 U·mL-1 inactivate elastase）， model， atorvastatin calcium tablets （2.6 mg·kg·d-1）， and low- and high-dose （crude drug 3.4， 17 g·kg-1·d-1， respectively） JTQHX groups. The mouse model of BAD was established by injection with 10 U·mL-1 elastase. After 14 days of modeling， the sham operation group and model group were administrated with equal volumes of pure water by gavage， and other groups with corresponding drugs for 2 months. The levels of interleukin-6 （IL-6） and calpain （LpA） in the serum were measured by enzyme-linked immunosorbent assay （ELISA）. Verhoeff 's Van Gieson （EVG） staining was employed to observe the pathological changes of blood vessels. Terminal-deoxynucleotidyl transferase mediated nick end labeling （TUNEL） was employed to examine the apoptosis rate of vascular smooth muscle cells （VSMCs）. Image Pro Plus was used to observe and calculate the curvature index， elongation length， percentage increase in vessel diameter， and curvature angle of the basilar artery vessels in mice. Western blot was employed to determine the expression levels of PI3K and Akt in the vascular tissue. ResultCompared with the sham operation group， the model group showed lowered IL-6 level （P<0.01）， no significant change in LpA level， increased apoptosis of VSMCs （P<0.01）， and increased curvature index， elongation length， percentage increase in vessel diameter， and curvature angle （P<0.01）. Furthermore， the modeling up-regulated the protein levels of PI3K and Akt in blood vessels （P<0.01） and aggravated the destruction of the inner elastic layer， atrophy of the muscular layer， and hyaline changes in the connective tissue of the medial membrane of the basilar artery wall. Compared with the model group， 2 months of treatment with JTQHX elevated the IL-6 level （P<0.01）， reduced the apoptosis of VSMCs （P<0.01）， decreased the curvature index， elongation length， percentage increase in vessel diameter， and curvature angle （P<0.05， P<0.01）， and down-regulated the protein levels of PI3K and Akt in blood vessels （P<0.01）. In addition， the treatment alleviated the destruction of the inner elastic layer， atrophy of the muscular layer， and hyaline changes in the connective tissue of the medial membrane of the basilar artery wall. ConclusionJTQHX inhibits the elongation， expansion， and curvature of basilar artery vessels and alleviates the pathological changes by reducing the apoptosis of VSMCs and down-regulating the expression of PI3K/Akt pathway.

Comorbidity Mechanism of Vertebrobasilar Dolichoectasia and Cerebral Small Vessel Disease Based on Collateral Disease Theory / 中国实验方剂学杂志

The comorbidities of vertebrobasilar dolichoectasia（VBD） and cerebral small vessel disease（CSVD） increase the poor prognosis of patients，and elucidating the mechanism underlying their comorbidities helps to explore effective treatment strategies. Therefore，based on the collateral disease theory and combining with the pathogenesis and research progress of traditional Chinese and Western medicine on the understanding of the two，this study proposes that both the disease locations are in the brain collaterals and the pathogenesis is deficiency in foundation and excess in symptoms. The main pathogenesis roots in the deficiency of original Qi and the emptiness of brain collaterals，which corresponds to the dysfunction of endothelial cells and neuro-endocrino-immune networks in modern medicine. The symptoms are cerebral blood stasis，occlusion of cerebral arteries and toxic lesion of cerebral arteries，corresponding to cerebrovascular atherosclerosis，hemodynamic changes，hypoperfusion and toxic metabolites-induced injury of white matter in modern medicine. Based on the collateral disease theory，exploring the common pathogenesis of the VBD and CSVD is expected to facilitate the establishment of TCM treatment scheme including the principles，methods and medicines，and improve the clinical prognosis of patients.

Recent advance in endovascular treatment of vertebrobasilar dolichoectasia / 中华神经医学杂志

Vertebrobasilar dolichoectasia is a rare and challenging disorder. Vertebrobasilar dolichoectasia is closely related to enzyme action and hemodynamic changes, and is characterized by ischemic stroke, neurological compression symptoms, hydrocephalus, and other clinical symptoms. With development of interventional techniques and materials in recent years, endovascular treatment of vertebrobasilar dolichoectasia has become the focus. This article summarizes the current endovascular treatment of vertebrobasilar dolichoectasia, aiming to provide references for clinicians.

[Dolichoectasia of the basilar artery caused by cystic medial degeneration, as a cause of neurovascular conflict with damage to the trigeminal, facial and vestibulocochlear nerves]. / Dolikhoektaziya bazilyarnoi arterii, obuslovlennaya kistoznoi medial'noi degeneratsiei, kak prichina neirovaskulyarnogo konflikta s porazheniem troinichnogo, litsevogo i vestibulokokhlearnogo nervov.

Cystic medial degeneration (Gsell-Erdheim syndrome, cystic medial necrosis) is considered to be a nonspecific histological manifestation of a group of diseases characterized by degenerative changes in the media, affecting primarily the aorta and adjacent branches, which leads to destruction of the vessel wall, followed by its expansion and, possibly, rupture. The authors describe a case of a 65-year-old female patient with a neurovascular conflict of the three cranial nerves with dolichoectatic basilar artery due to cystic medial degeneration. As a result, the patient has clinical manifestations in the form of hemifacial spasm, trigeminal neuralgia and vestibular paroxysmia. Data from instrumental studies and treatment provided are presented. Neurovascular conflict can be identified in various diseases and is characterized by the complex etiology. The most common clinical manifestations of neurovascular conflict are trigeminal neuralgia, hemifacial spasm, glossopharyngeal neuralgia, and vestibular paroxysmia.

The cerebellopontine angle cistern volumetric differences in trigeminal neuralgia patients with and without vertebrobasilar compression: a case-matched study.

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.

Basilar artery diameter as neuroimaging biomarker in Chinese Fabry disease patients.

BACKGROUND: Fabry disease (FD) is an X-linked lysosomal storage disease resulting from mutations of α-galactosidase A gene, and has been emphasized as one of the etiologies of young stroke and leukoencephalopathy. Vertebrobasilar dolichoectasia (VBD) is a highlighted finding in FD. We aim to examine the utility of VBD in Chinese FD by comparing the differences in basilar artery (BA) diameter of Chinese FD patients against age-matched controls with and without stroke. METHODS: This was a matched case-control study involving 37 Chinese FD patients. The BA diameters were evaluated on axial T2-weighted magnetic resonance imaging and compared to two age-and-gender matched control groups, one with stroke and one without. The association between BA diameter and stroke occurrences and white matter hyperintensities (WMH) were analyzed among all FD patients. RESULTS: Patients with FD had significantly increased BA diameter compared to controls with and without stroke (p < 0.001). A BA diameter of 4.16 mm could distinguish FD from controls in the stroke subgroup (ROC AUC 0.870, p = 0.001, sensitivity 80% specificity 100%), and with a cut-off of 3.21 mm in the non-stroke subgroup (ROC AUC 0.846, p < 0.001, sensitivity 77.8% specificity 88.9%). Larger BA diameter had more stroke occurrences and was moderately associated with heavier WMH load in terms of higher total FAZEKAS scores. (Spearman's rho = 0.423, p = 0.011). CONCLUSION: VBD was also present in Chinese FD patients. BA diameter has high diagnostic utility in identifying FD from a mixed cohort of stroke and normal controls, and carried predictive value in evaluating neurological complications of FD.

Imaging features of vertebrobasilar dolichoectasia combined with posterior circulation ischemic stroke: A vessel wall magnetic resonance imaging study.

PURPOSE: To elucidate the vessel wall changes of vertebrobasilar dolichoectasia (VBD) with ischemic stroke, using vessel wall magnetic resonance imaging (VW-MRI). METHOD: Thirty-four patients with VBD (22 with stroke and 12 without stroke) who underwent VW-MRI were recruited. Forty-one patients without VBD who underwent VW-MRI were also recruited if they had a recent stroke due to atherosclerosis in the basilar artery or the intracranial vertebral artery. The vessel wall features of VBD were compared between stroke and non-stroke groups. The plaque characteristics were compared between VBD and non-VBD stroke patients. RESULTS: The frequency of plaques was higher (54.5% vs. 8.3%, P = 0.011) in VBD patients with stroke than that in non-stroke patients, while the frequencies of aneurysm, dissection, intraluminal thrombus, and diffuse/concentric wall enhancement did not differ. When the plaque features were compared between plaque-positive stroke patients with and without VBD, the degree of stenosis (31.0% ± 26.8% vs. 71.5% ± 19.0%, P < 0.001), normalized wall index (NWI) (0.7 ± 0.1 vs. 0.9 ± 0.1, P < 0.001), and remodeling index (RI) (1.0 ± 0.4 vs. 1.3 ± 0.4, P = 0.023) were lower in the VBD group, while intraplaque hemorrhage, and enhancement ratio showed no difference. CONCLUSIONS: This preliminary study suggests that atherosclerosis may be an important cause of stroke in VBD patients. Symptomatic plaques in VBD patients have a lower degree of stenosis, NWI, and RI than that in non-VBD patients. VW-MRI may help to assess stroke mechanisms and identify VBD patients at high risk.

Trigeminal neuralgia caused by vertebrobasilar dolichoectasia: efficacy of stepwise decompression technique.

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.

Vertebrobasilar and Basilar Dolichoectasia Causing Audio-Vestibular Manifestations: A Case Series with a Brief Literature Review.

Audio-vestibular symptoms can arise from vertebrobasilar dolichoectasia (VBD) and basilar dolichoectasia (BD). Given the dearth of available information, herein we reported our experience with different audio-vestibular disorders (AVDs) observed in a case series of VBD patients. Furthermore, a literature review analyzed the possible relationships between epidemiological, clinical, and neuroradiological findings and audiological prognosis. The electronic archive of our audiological tertiary referral center was screened. All identified patients had a diagnosis of VBD/BD according to Smoker's criteria and a comprehensive audiological evaluation. PubMed and Scopus databases were searched for inherent papers published from 1 January 2000 to 1 March 2023. Three subjects were found; all of them had high blood pressure, and only the patient with high-grade VBD showed progressive sensorineural hearing loss (SNHL). Seven original studies were retrieved from the literature, overall including 90 cases. AVDs were more common in males and present in late adulthood (mean age 65 years, range 37-71), with symptoms including progressive and sudden SNHL, tinnitus, and vertigo. Diagnosis was made using different audiological and vestibular tests and cerebral MRI. Management was hearing aid fitting and long-term follow-up, with only one case of microvascular decompression surgery. The mechanism by which VBD and BD can cause AVD is debated, with the main hypothesis being VIII cranial nerve compression and vascular impairment. Our reported cases suggested the possibility of central auditory dysfunction of retro-cochlear origin due to VBD, followed by rapidly progressing SNHL and/or unnoticed sudden SNHL. More research is needed to better understand this audiological entity and achieve an evidence-based effective treatment.