Bilateral Distal Internal Carotid Arteries Research Articles

Vessel Wall Magnetic Resonance Imaging of Bilateral Distal Internal Carotid Artery Stenosis in Intracranial Giant Cell Arteritis

Vessel Wall Magnetic Resonance Imaging of Bilateral Distal Internal Carotid Artery Stenosis in Intracranial Giant Cell Arteritis

Carotid Artery Stenosis and Ischemic Strokes in Patients with Giant Cell Arteritis: A Characteristic Pattern-Literature Review and Case Report.

Purpose Ischemic stroke is a relatively rare complication of giant cell arteritis often accompanied by vessel stenosis. Our purpose was to compare the location of internal carotid artery stenosis in GCA patients by performing a literature review suggesting a specific and characteristic pattern. Methods We performed a PubMed research including all articles and cited articles reporting cases and case series about giant cell arteritis patients with internal carotid artery stenosis and ischemic strokes. Results In this case series 39 cases were included. We found a clear tendency of giant cell arteritis-related stenosis to be in the intracranial segments (35/39 (89.7%)). Only in 8/39 (20.5%) patients there was further involvement of extracranial segments. Many cases (27/39 [69.2%]) showed a bilateral involvement. Discussion This literature review reveals a specific pattern of internal carotid artery involvement in patients with giant cell arteritis and ischemic strokes. To our knowledge this pattern has not been reported as a sign strongly pointing toward giant cell arteritis before. We have not found case reports mentioning other common types of vasculitis reporting this involvement pattern. Conclusion Internal carotid artery stenosis and ischemic stroke is a rare complication in patients with giant cell arteritis. Considering the characteristic features of bilateral distal internal carotid artery stenosis giant cell arteritis should be suspected which potentially leads to an early diagnosis and immunotherapy.

Increased tortuosity of bilateral distal internal carotid artery is associated with white matter hyperintensities.

Although the pathophysiology of white matter hyperintensities remains unclear, we can recently explore the possible relationship with white matter hyperintensities by using quantitative parameter. To demonstrate the relationship between bilateral distal internal carotid arterial tortuosity and total brain white matter hyperintensities volume in elderly individuals. A total of 345 patients (age > 65 years) with brain magnetic resonance (MR) examinations were retrospectively included (44.1% men; mean age = 72.1 ± 6.25 years; 55.9% ≥ 70 years). We measured the Tortuosity Index (TI) of the bilateral distal internal carotid artery and basilar artery on MR angiography imaging, and white matter hyperintensities volume on fluid-attenuated inversion recovery MR sequence. Multiple linear regression was used to assess the association of the TI with quantitatively derived brain white matter hyperintensity volume, after adjusting for demographics (age, sex), vascular risk factors (hypertension, diabetes, heart disease), and vessel diameters, total intracranial volume (TIV). Increased tortuosity of bilateral distal internal carotid artery was associated with greater burden of white matter hyperintensity volume (right: β = 11.223, P = 0.016; left: β = 20.701, P < 0.001). This relationship was independent of age and hypertension, both of which have been considered the strongest risk factors for white matter hyperintensities. Our results suggest that tortuosity of the bilateral distal internal carotid artery is associated with white matter hyperintensities, independent of age and hypertension.

Acute Ischemic Stroke in Moyamoya Syndrome Associated with Thyrotoxicosis

Background A few cases of moyamoya syndrome associated with thyrotoxicosis have been reported. However, studies on the association of hyperthyroidism with moyamoya syndrome are insufficient. Case Report Here we report a case of hyperthyroidism associated with moyamoya syndrome in a 41-year-old woman with aphasia and right side weakness. Brain imaging revealed acute cerebral infarction of left middle cerebral artery territory and occlusion of bilateral distal internal carotid arteries. Conclusion Antithyroid medication stabilized the patient’s neurologic deterioration, suggesting that thyrotoxicosis could aggravate acute cerebral infarction caused by moyamoya syndrome. Key Words: Moyamoya disease; Thyrotoxicosis; Cerebral infarction

More Precise Imaging Analysis and Diagnosis of Moyamoya Disease and Moyamoya Syndrome Using High-Resolution Magnetic Resonance Imaging

The diagnosis of moyamoya disease (MMD) is often uncertain. Moyamoya syndrome (MMS) is often misdiagnosed as MMD. High-resolution magnetic resonance imaging (HR-MRI) enables vessel wall assessment to obtain more precise diagnoses. The aim of this study was to determine the true etiologies of arterial steno-occlusion in patients with an angiographic diagnosis of MMD or MMS using HR-MRI. HR-MRI was performed in 21 adult patients with angiographically proven MMD or MMS. A definite diagnosis was based on the HR-MRI findings. The diagnoses made via the 2 different imaging technologies were compared, and significant findings were analyzed. A total of 21 patients were enrolled, including 7 patients with angiographically proven MMD and 14 patients with angiographically proven MMS. Among the 7 patients with MMD, HR-MRI confirmed the diagnosis of MMD in 6; the remaining patient was considered to have atherosclerosis in the bilateral distal internal carotid arteries (ICAs) and the left middle cerebral artery. Among the 14 patients with MMS, HR-MRI confirmed MMD in 6 patients (including 2 patients with unilateral MMD), atherosclerosis in 5 patients (including 3 patients with bilateral atherosclerosis and 2 with unilateral atherosclerosis), arterial dissection of the left ICA in 1 patient, and MMD in the left cerebral hemisphere with atherosclerosis in the right hemisphere in 2 patients. Differentiating MMD from MMS is difficult in certain situations, and HR-MRI may help provide a more in-depth understanding of MMD and MMS, thereby achieving a more reliable diagnosis.

High-resolution magnetic resonance wall imaging findings of Moyamoya disease.

Diagnosis of Moyamoya disease (MMD) is based on the characteristic angiographic findings. However, differentiating MMD from intracranial atherosclerotic disease (ICAD) is difficult. We compared vessel wall imaging findings on high-resolution magnetic resonance imaging between MMD and ICAD. High-resolution magnetic resonance imaging was performed on 32 patients with angiographically proven MMD and 16 patients with acute infarcts because of ICAD. Bilateral internal carotid arteries and steno-occlusive middle cerebral artery were analyzed for wall enhancement and remodeling. Enhancement patterns and distribution were different. Most patients with MMD (90.6%) showed concentric enhancement on distal internal carotid arteries and middle cerebral arteries, whereas focal eccentric enhancement was observed on the symptomatic segment in ICAD. MMD was characterized by middle cerebral artery shrinkage; the remodeling index and wall area were lower in MMD than in ICAD (remodeling index, 0.19±0.11 versus 1.00±0.43; wall area, 0.32±0.22 versus 6.00±2.72; P<0.001). MMD was characterized by concentric enhancement on bilateral distal internal carotid arteries and shrinkage of middle cerebral artery, regardless of symptoms.

“The Fainted Man:” Hypoperfusion Encephalopathy in a Patient With Moyamoya

Back to table of contents Previous article Next article LettersFull Access“The Fainted Man:” Hypoperfusion Encephalopathy in a Patient With MoyamoyaAna Filipa Santos, M.D., João Pinho, M.D., Carla Ferreira, M.D., Celeste Pereira, M.D., Manuel Ribeiro, M.D., and Jaime Rocha, M.D.Ana Filipa SantosSearch for more papers by this author, M.D., João PinhoSearch for more papers by this author, M.D., Carla FerreiraSearch for more papers by this author, M.D., Celeste PereiraSearch for more papers by this author, M.D., Manuel RibeiroSearch for more papers by this author, M.D., and Jaime RochaSearch for more papers by this author, M.D.Published Online:1 Jan 2014https://doi.org/10.1176/appi.neuropsych.13020026AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: Moyamoya disease is a progressive cerebral vasculopathy of unknown etiology, characterized by severe bilateral stenosis/occlusion of the main arteries of the circle of Willis, with a pattern of collateral vessels that look like a “puff of smoke” on angiography.1 It is more common in Asian populations and more prevalent in women.1 The diagnosis peaks in two age-groups: children approximately 5 years old, and adults in their mid-40s.1 It may have variable clinical presentation: ischemic stroke, transient ischemic attack, intracranial hemorrhage, headache, seizures, involuntary movements, cognitive deficits, and psychiatric changes.1 In adults, it usually presents with intracranial hemorrhage or ischemic symptoms.2 Acute cerebral ischemia occurring after intracranial bleeding has rarely been reported and is not well understood.2,3 We describe a case of hypoperfusion encephalopathy after intracerebral hemorrhage occurring as the first manifestation of moyamoya disease.A 77-year-old male patient, with a long-standing history of uncontrolled hypertension, was admitted to our stroke unit upon being found unconscious. His blood pressure was 174/100 mmHg and Glasgow Coma Scale rated 3 (no eye opening, no verbal response, no motor response), with reactive pupils and right extensor plantar reflex. Computed tomographic (CT) scan showed left basal ganglia hemorrhage, extending into the ventricles and subarachnoid space. Antihypertensive and hyperosmotic therapy was started, and the initial evolution was favorable, with recovery of consciousness, confused verbal response, and right hemiparesis Grade 3 (MRC). By Day 14, multifocal myoclonus appeared, with decreased arousal and ensuing severe encephalopathy. Follow-up CT revealed hemorrhage reabsorption and no hydrocephaly. Laboratory studies were unrevealing. An electroencephalogram showed only slow background activity (5–6 Hz). Infections, metabolic, and toxic causes were also excluded. Brain diffusion-weighted imaging (DWI) MRI was negative for acute ischemic lesions. At this point, hypoperfusion encephalopathy was suspected and confirmed by perfusion CT (Figure 1). CT angiography disclosed stenosis of the distal intracranial internal carotid artery (ICA) bilaterally, extending to the proximal anterior (ACA) and middle cerebral (MCA) arteries. Moyamoya disease was diagnosed and was classified as Grade IV (Suzuki Grading System) by conventional angiography (Figure 2). Because of his clinical condition, revascularization was not considered.FIGURE 1. Transverse Perfusion CT Map Shows Global Decreased Cerebral Blood Flow (CBF), Mainly in Middle Cerebral Arteries (MCA) and Anterior Cerebral Arteries (ACA) AreasFIGURE 2. Right (A1/A3) and Left (A2/A4) Internal Carotid Angiograms (ICAs) Show Severe Stenosis of the Bilateral Distal Internal Carotid Artery, With Retrograde Filling of the Peri-Callosal Branches (*) of the ACAs and Basal Moyamoya Vessels “Puff of Smoke” Sign (arrow)We report this case because of the rarity of moyamoya diagnosis in elderly persons and because of the atypical presentation. Our patient seemed to be permanently “fainted;” he had global hypoperfusion, but no real ischemic changes. The initial manifestation was interpreted as a hypertensive intracerebral hemorrhage. Later, when we detected hypoperfusion encephalopathy, Moyamoya was diagnosed. Vasospasm, in association with both subarachnoid and intraventricular hemorrhage, and hypotension and dehydration, resulting from the administration of hyperosmotic agents to control hemorrhage-related increased intracranial pressure, may have caused hemodynamic compromise, being possible contributing factors to the hypoperfusion encephalopathy in our patient. Other factors, such as hypocapnia and shrinkage of the moyamoya vessels, have also been proposed as mechanisms in acute cerebral ischemia occurring after intracranial bleeding.2,3 The initial clinical presentation of hemorrhage and the presence of severe bilateral ICA stenosis affecting cerebral perfusion are predictors of very poor outcome in our patient.Neurology Department, Hospital de Braga, Braga, PortugalInternal Medicine Department, Hospital Santa Maria Maior Barcelos, Barcelos, PortugalNeuroradiology Department, Hospital de Braga, Braga, PortugalCorrespondence: Ana Filipa Santos, M.D.; e-mail: [email protected]comReferences1 Scott RM, Smith ER: Moyamoya disease and moyamoya syndrome. N Engl J Med 2009; 360:1226–1237Crossref, Medline, Google Scholar2 Kim DS, Jang DK, Huh PW, et al.: Ischaemic stroke after acute intracranial haemorrhage in patients with moyamoya disease: six new cases and a short literature review. Acta Neurochir (Wien) 2011; 153:1253–1261Crossref, Medline, Google Scholar3 Shibamoto K, Aoyama K, Taki H, et al.: Acute cerebral ischemia after intracranial bleeding in unilateral moyamoya disease: case report. Neurol Med Chir (Tokyo) 2012; 52:346–349 [Tokyo]Crossref, Medline, Google Scholar FiguresReferencesCited byDetailsCited ByNone Volume 26Issue 1 Winter 2014Pages E31-E33 Metrics PDF download History Published online 1 January 2014 Published in print 1 January 2014

Abstract 2786: Moyamoya Syndrome Associated With Graves' Disease: Report Of 16 Cases

Background and purpose Moyamoya disease is a cerebrovascular disorder characterized by bilateral progressive stenosis and occlusion of terminal portions of internal carotid artery (ICA) accompanying by typical net-like collateral vessels. Patients with both the characteristic moyamoya vasculopathy and the associated conditions are categorized as moyamoya syndrome. These conditions include sickle cell disease, neurofibromatosis type 1, Down’s disease, cranial therapeutic irradiation and other rare diseases. Moyamoya syndrome associated with Graves’ disease has been rarely reported and the underlying coexisting mechanism remains unclear. The aim of this study is to identify the clinical and radiological findings of the patients with simultaneous diagnosis of moyamoya syndrome and Graves’ disease. Possible mechanisms predisposing these individuals to ischemic accidents are discussed. Methods We retrospectively identified 12 patients in Peking Union Medical College Hospital and 4 patients in 307 hospital of PLA between May 2000 and December 2010. All patients were female and mean age (range) was 35.13±12.34 years (11-57 years). The patients were diagnosed with Graves’ disease in endocrinology clinic which meet the full diagnostic criteria and no atherosclerotic factor was found in them. Moyamoya vasculopathy was definitely or probably diagnosed by digital subtract angiography or magnetic resonance angiography. The clinical characteristics and prognosis, laboratory data, vascular radiological characteristics were all collected. Results Stenosis or occlusion of bilateral distal ICA and/or proximal anterior /middle cerebral arteries was found in 13 patients. Three patients had unilateral distal ICA stenosis and abnormal collateral vessels. PCA stenosis was found in 2 patients. Fifteen patients presented with infarction or transient ischemic attack and one with dizziness. Interestingly, thyroid function tests demonstrated predominantly elevated thyroxine and suppressed thyroid stimulating hormone level in 15 patients when cerebrovascular accidents occurred. All patients received antithyroid therapy and two had recurrent ischemic attack after several months of drug withdrawal. Four patients underwent revascularization procedures after normalization of their hormonal conditions in 307 Hospital of PLA and their neurologic status has remained stable during follow-up. Conclusions: Compared with classical moyamoya disease, moyamoya syndrome associated Graves’ disease more commonly presented asymmetric stenosis or occlusion and PCA lesions. Cerebrovascular hemodynamic changes attributable to thyrotoxicosis might be responsible for ischemic attack and further studies are required to verify this hypothesis.

A 10-Year-Old Girl With Coexistent Moyamoya Disease and Graves' Disease

There are rare reports of young women with moyamoya disease associated with Graves' disease; we are unaware of any previous reports of this association in prepubescent girls. We report a 10-year-old girl who presented with multiple bilateral strokes. Cerebral angiography demonstrated moderate to severe stenosis of her bilateral distal internal carotid arteries and proximal anterior and middle cerebral arteries, which was greater on the right. Thyroid function tests demonstrated suppressed thyroid-stimulating hormone and elevated thyroid hormone levels. Serum antiphospholipid antibody screen demonstrated mild elevations of antiocardiolipin IgG. Possible mechanisms predisposing individuals to concurrent moyamoya and Graves' disease are discussed.

Improvement of Cerebral Arterial Stenosis Associated With Basedow's Disease-Case Report-

A 29-year-old female presented with Basedow's disease manifesting as sudden vomiting, diarrhea, fever over 38 degrees C, transient aphasia, and numbness in her extremities. These symptoms were considered due to cerebral ischemia at a local clinic. Magnetic resonance angiography indicated stenosis of the bilateral distal internal carotid arteries and the bilateral proximal anterior cerebral and middle cerebral arteries. Thyroid swelling and exophthalmos were observed. She was transferred to our hospital. Endocrine function tests showed hyperthyroidism. The diagnosis was Basedow's disease. Her symptoms disappeared after receiving intravenous drip infusion of fluid replacement, and antithyroid and antiplatelet medication. After she became euthyroid, cerebral angiography and magnetic resonance angiography revealed improvement of the stenosis of the cerebral arteries. Stenosis of the terminal portion of the internal carotid artery associated with Basedow's disease is extremely rare. Conservative treatment mainly including antithyroid medications for Basedow's disease, and antiplatelet drugs and intravenous replacement fluid for the ischemic manifestations should be the first choice of treatment unless immediate vascular reconstruction is necessary.