Paradoxical association of moyamoya syndrome with large middle cerebral artery aneurysm and subarachnoid hemorrhage. Case report

A 69-year-old woman was admitted to our hospital because of fluctuating dysarthria during the past 2 months. Magnetic resonance imaging revealed old cerebral infarction of the left cerebral hemisphere with acute subarachnoid hemorrhage in the left sylvian fissure. Cerebral angiography showed a large saccular aneurysm, 14 mm in diameter, at the bifurcation of the left middle cerebral artery (MCA) in association with moyamoya vasculopathy with atherosclerosis, including steno-occlusive changes at the bilateral terminal internal carotid arteries and abnormal net-like vessels at the base of the brain. She underwent microsurgical neck clipping of the large aneurysm followed by superficial temporal artery-MCA anastomosis without complication. Intraoperative findings showed no evidence of aneurysm rupture, suggesting that the subarachnoid hemorrhage was due to the intrinsic pathology of moyamoya vasculopathy. The postoperative course was uneventful, and the patient was discharged without neurological deficit. Association of moyamoya syndrome with large MCA aneurysm is extremely rare, and formation of large aneurysm at the vascular territory of an occluded vessel is apparently unique.     

Development of Moyamoya Disease in Pregnancy and Puerperium: Case Report

Progressive moyamoya disease in pregnancy and puerperium has not been reported previously. Here, we present a 39-year-old woman who had been found to have moderate stenosis of right middle cerebral artery (MCA) 4 years prior to her pregnancy, finally suffering minor completed stroke due to progressive moyamoya disease at the early postpartum period. Three days after cesarean section without any complication, she developed cerebral infraction at right hemisphere, when magnetic resonance angiography indicated apparent progression of the proximal MCA stenosis. Catheter angiography demonstrated nearly occlusion of the right terminal internal carotid artery (ICA) and the development of an abnormal vascular network at the base of the brain as well as MCA stenosis, indicating a definitive diagnosis of moyamoya disease with unilateral involvement. The patient underwent superficial temporal artery-middle cerebral artery anastomosis 1 month after the onset of stroke, and she did not manifest as further neurological events during the follow-up period of 2 years. Moyamoya disease could newly develop in pregnancy and puerperium, which should be noted as a pitfall of the management of moyamoya disease with pregnancy.     

Magnetic resonance angiography demonstrating adult moyamoya disease progressing from unilateral to bilateral involvement--case report

A 21-year-old woman presented with moyamoya disease manifesting as speech disturbance and right quadrant hemianopsia on October 22, 1994. Magnetic resonance (MR) angiography showed occlusion of the left internal carotid artery (ICA) with the normal right ICA. The diagnosis was "unilateral" moyamoya disease by conventional angiography. Follow-up MR angiography revealed further occlusive changes of the right middle cerebral artery (MCA) trunk on July 30, 1995, which progressed to occlusion of the MCA on March 25, 1997. Conventional angiography confirmed occlusion of the right terminal ICA to MCA with basal moyamoya vessels. The diagnosis was "bilateral" moyamoya disease. She was successfully treated by bilateral superficial temporal artery-MCA anastomosis. Follow-up MR angiography should be performed in relatively young patients with "unilateral" moyamoya disease to detect any progression to bilateral moyamoya disease.     

A case of iatrogenic carotid artery dissection treated with radial artery graft

A case of iatrogenic intracranial artery dissection is reported. A 52-year-old female developed severe headache and nausea. Brain CT showed diffuse subarachnoid hemorrhage. On admission, carotid angiography revealed an aneurysm in the right middle cerebral artery and the intact right internal carotid artery. The aneurysm was clipped successfully. Carotid angiography on day 7 revealed dissection in the right internal carotid artery. Repeated angiograms at 10 and 31 days showed progression of the carotid artery dissection. Findings of ECD-SPECT on day 31 (Balloon occlusion test) suggested low perfusion of the right internal carotid artery territory. The patient underwent surgical reconstruction of the right internal carotid artery using a radial artery. She presented with right abducens nerve palsy three days after the radial artery graft. The patency of the radial artery graft was proved by the post-operative angiography. Internal carotid artery dissection may occur spontaneously or as a result of trauma. An iatrogenic dissection is an uncommon complication of cerebral angiography. There are no evidence-based guidelines for the treatment although anticoagulation therapy is most commonly used. The present case emphasizes the usefulness of radial artery graft for traumatic carotid artery dissection.     

Endovascular treatment of a ruptured aneurysm associated with unilateral moyamoya disease

We report a case presenting with subarachnoid hemorrhage due to a ruptured aneurysm associated with unilateral moyamoya disease. The patient was a 29-year-old woman exhibiting sudden onset headache. Computed tomography revealed subarachnoid hemorrhage around the brain stem. Cerebral angiography showed a saccular aneurysm at the junction of the left P1 portion of the posterior cerebral artery and its perforator. The right internal carotid artery was occluded at the terminal portion, and the right middle cerebral artery territory was perfused anterogradely via abnormal moyamoya vessels in the basal ganglia. The aneurysm was completely embolized with preservation of the perforator. We suggest that as compared to surgical clipping by craniotomy, endovascular therapy is safe and effective for treatment of intracranial aneurysms associated with moyamoya disease.     

Spontaneous intracranial internal carotid artery dissection treated by intra-arterial thrombolysis and superficial temporal artery-middle cerebral artery anastomosis in the acute stage--case report--

A 22-year-old man presented with sudden onset of right retro-orbital headache followed by left hemiparesis. Right carotid angiography demonstrated almost total occlusion of the intracranial internal carotid artery (ICA) and severe stenosis of the middle cerebral artery (MCA), presumably caused by arterial dissection. Local arterial injection of urokinase was performed 2 hours after onset. The ICA became patent, but the M2 portion of the MCA was still occluded, and the left hemiparesis did not improve. Superficial temporal artery-MCA anastomosis was immediately performed. The left hemiparesis disappeared completely 6 days after this procedure. Angiography 2 weeks after the onset revealed occlusion of the ICA, and maintenance of blood flow to the right cerebral hemisphere via the anastomosis. Magnetic resonance imaging showed small infarcts in the right cerebral cortex. Repeat angiography after 5 months showed recanalization of the right ICA and the right MCA. Combination of thrombolytic therapy and bypass surgery may be a useful treatment option for patients with sudden occlusion of the intracranial artery caused by dissection.     

An adult case of moyamoya disease associated with marked advance of occlusive lesion in the bilateral carotid system

We reported an adult case with moyamoya disease in which unilateral obstructive process proceeded to the bilateral lesion during a period of 45 months. A 30-year-old female was admitted to our institute due to frequent ischemic episodes since she was 25 years old. At the time of admission, she was asymptomatic with slightly disturbed psychogenic reactions. At the time of the initial cerebral angiography when she was 25 years old, the right cerebral angiography demonstrated stenosis of the right ICA terminal portion with moyamoya vessels in the basal area and leptomeningeal anastomosis at the right parietal area. The left cerebral angiography revealed normal ICA system with a lightly narrowing Al portion, without Moyamoya vessels. The second cerebral angiography, taken 45 months after the initial angiography, demonstrated progression stenosis of the right ICA terminal portion, with decreasing moyamoya vessels at the basal area, obstruction of the left ICA terminal portion, stenosis at the Al portion with newly developed moyamoya vessels in the left basal area. We concluded at this time that this case was a definite case of moyamoya disease. The third cerebral angiography after right SDP (synangio-dural plasty), 49 months after the initial angiography, revealed, in the right angiography, newly formed anastomotic vessels perfusing the middle cerebral artery region via the extracerebral arteries and in the left cerebral angiography, and an increased obstruction of the ICA terminal portion, transdural anastomosis via the extracerebral arterial system, and a decrease of moyamoya vessels in the basal area. This rare case provided us a good picture of the pathogenic mechanism of moyamoya disease.     

Post-irradiation vasculopathy of intracranial major arteries in children--report of two cases.

We report two rare cases of post-irradiation vasculopathy of intracranial major arteries in children. A 13-year-old girl suffered from transient right hemiparesis 1 year after irradiation for suprasellar germinoma. Left carotid angiograms revealed marked stenoses of the intracranial internal carotid, middle cerebral, and anterior cerebral arteries, which were previously normal, and moyamoya vessels. A 2.5-year-old girl underwent internal irradiation with 198Au colloid for cystic craniopharyngioma. At the age of 10 years, she suddenly became unconscious after vomiting. Computed tomographic scans showed a right frontal intracerebral hematoma. Right carotid angiograms disclosed complete obstruction of the intracranial internal carotid, middle cerebral, and anterior cerebral arteries and moyamoya vessels, previously not present. The danger of radiation therapy causing occlusive vasculopathy in small and major cerebral arteries in children is emphasized. To prevent permanent ischemic neurological deficits, vasculopathy should be treated either medically or surgically as early as possible.     

Transient crossed cerebellar diaschisis due to cerebral hyperperfusion following surgical revascularization for moyamoya disease: case report

Crossed cerebellar diaschisis (CCD) often occurs after ischemic or hemorrhagic stroke that damages the cortico-ponto-cerebellar pathway. However, CCD due to cerebral hyperperfusion following cerebrovascular reconstruction is rare. A 61-year-old woman presented with transient CCD due to cerebral hyperperfusion following bypass surgery for adult moyamoya disease. She developed transient weakness of the right extremities and was diagnosed with moyamoya disease. First, she underwent superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis with indirect synangiosis on the left. Postoperative course was uneventful. Subsequently, she underwent STA-MCA anastomosis with indirect synangiosis on the right. She complained of mild headache on the right, and single photon emission computed tomography (SPECT) performed on the 7th postoperative day demonstrated hyperperfusion in the right frontal and temporal lobes associated with hypoperfusion in the left cerebellum. Magnetic resonance (MR) imaging demonstrated no new lesions and MR angiography showed patent STA-MCA bypass. Subsequent SPECT showed disappearance of both hyperperfusion and CCD. This case strongly suggests that cerebral hyperperfusion after bypass surgery for moyamoya disease may cause transient CCD. Although the clinical significance is still obscure, this phenomenon indicates the cortico-ponto-cerebellar pathway is interrupted due to hyperperfusion, suggesting the development of hyperperfusion syndrome. Careful observation of cerebral hemodynamics after bypass surgery is warranted to avoid hyperperfusion-related complications.     

Steno-Occlusive Changes in the External Carotid System in Moyamoya Disease

To evaluate the steno-occlusive changes in the external carotid system in moyamoya disease, cerebral angiograms of 39 moyamoya patients were retrospectively reviewed. There were 26 females and 13 males, age ranged from 4 to 62 years with a mean of 26 years. Initial symptoms were ischaemia in 27 patients, haemorrhage in 9, and none in 3. Stenosis, occlusion, and dilatation in the external carotid system were analysed angiographically. No stenosis or occlusion of the superficial temporal artery, middle meningeal artery, or occipital artery was observed in either preoperative or postoperative follow-up angiograms in any patients. Steno-occlusive changes do not occur in the external carotid system, but are confined in the internal carotid system in moyamoya disease.     

Moyamoya disease associated with persistent primitive trigeminal artery--a case report and review of literature

A case of moyamoya disease associated with the persistent primitive trigeminal artery (PTA) is reported. 5 such cases reported in literature are reviewed. A 16-year-old female patient was admitted to our clinic, having complained of right hemiparesis for 8 years. CT scan revealed multiple low density areas in the left frontal, temporal and parietal lobes. In left carotid angiogram, the internal carotid artery was occluded at the C3 portion and basal moyamoya (stage IV) was found. In right carotid angiogram, the internal carotid artery was occluded at the C3 portion and basal moyamoya (stage V) was found. Furthermore the persistent primitive trigeminal artery was seen with marked moyamoya vessels from the posterior cerebral artery. In vertebral angiogram, the posterior cerebral artery was seen bilaterally with moyamoya vessels. In 133Xe-rCBF study, compared with the mean flow in each hemisphere, the flow decreased at the temporal lobe, but, in contrast, increased at the parieto-occipital lobes. Reviewing the literature, the following conclusions are suggested. 1. Persistent primitive trigeminal artery will promote the stage of moyamoya disease. 2. It will protect the brain from hemorrhage and/or ischemia in moyamoya disease.     

Moyamoya disease with persistent primitive hypoglossal artery. Case report

A 44-year-old female developed aphasia and visual disturbance of the left eye. Four-vessel angiography showed occlusion of the extra- and intracranial portions of the right internal carotid artery, severe stenosis of the left internal carotid fork, bilateral moyamoya vessels, and a persistent primitive hypoglossal artery on the left side. Measurement of cerebral blood flow by 133xe inhalation revealed decreased flow and no response to acetazolamide loading in the left cerebral hemisphere. The patient underwent superficial temporal artery-middle cerebral artery anastomosis, first on the left and then on the right side. There have been no other reported cases of moyamoya disease associated with a persistent primitive hypoglossal artery. Although the cause of moyamoya disease remains to be established, it is generally believed that stenotic changes of the carotid fork cause the development of moyamoya vessels as collateral pathways. According to this theory, there is no significant etiological relationship between moyamoya disease and persistent primitive hypoglossal artery. However, persistent primitive hypoglossal artery is compatible with moyamoya disease in terms of providing a collateral pathway via the vertebrobasilar system.     

Internal carotid artery agenesis: correlation by conventional and digital subtraction angiography, and by computed tomography

A case is presented of agenesis of the right internal carotid artery in a fifteen-year-old female with an unrelated seizure disorder. Conventional angiography revealed an absent right internal carotid artery, supply to the right anterior cerebral artery from the left internal carotid artery, and supply to the right middle cerebral artery by anastomosis through the vertebral basilar system. The congenital nature of the anomaly was confirmed by intravenous digital subtraction angiography, which revealed no portion or remnant of the right internal carotid artery to be present, and by computerized tomography of the base of the skull, which demonstrated no bony carotid canal on the right side. This is the first reported case of internal carotid artery agenesis to be elucidated by intravenous digital subtraction angiography. A brief discussion concerning the congenital nature of this anomaly is presented, including the role of angiography and computed tomography in establishing the diagnosis of this condition.     

Constructive interference in steady state imaging of moyamoya disease

The diagnostic accuracy of three-dimensional constructive interference in steady state (CISS) magnetic resonance (MR) imaging was evaluated for the assessment of idiopathic moyamoya disease. Six consecutive patients underwent MR angiography, CISS imaging, and digital subtraction angiography. MR angiography and CISS imaging visualization of the steno-occlusive changes in the distal internal carotid arteries and the development of moyamoya vessels in the basal cistern were compared to the results obtained by digital subtraction angiography. MR angiography revealed the steno-occlusive changes correctly in nine and overestimated the changes in three of 12 hemispheres examined. CISS imaging showed the steno-occlusive changes defined as decreased caliber of the internal carotid artery correctly in two, underestimated the changes in nine, and overestimated the changes in one of the 12 hemispheres. MR angiography detected moyamoya vessels correctly in five and underestimated the vessels in seven of the 12 hemispheres. CISS imaging revealed the moyamoya vessels correctly in 10, underestimated the vessels in one, and overestimated the vessels in one of the 12 hemispheres. CISS imaging can supplement MR angiography in the non-invasive diagnosis of moyamoya disease, especially for the evaluation of moyamoya vessels.     

A case of unilateral atypical moyamoya disease of adult onset with stenosis of the basilar artery]

A 29-year-old, 39-week-pregnant female who had headache and nausea was admitted to our hospital. She bore a baby son by natural delivery after several hours. After labor, her headache was continuous. Brain CT scan demonstrated intracerebral and intraventricular hemorrhage. After conservative treatment for two weeks, her only neurological deficiency was visual field defect. Angiography demonstrated that her left internal carotid artery had partial stenosis at the C2 portion. Her right internal carotid artery had stenosis at the C2 portion. Her right middle cerebral artery was occluded at the M1 portion, and abnormal vascular networks had developed in the ganglionic region. Stenosis was also found in the basilar artery. We diagnosed her as being a case of adult-onset, unilateral, atypical Moyamoya disease with basilar artery stenosis. As our case was of adult-onset, and as she showed no ischemic signs, we did not think that reconstructive surgery was indicated. About the posterior circulation of Moyamoya or atypical Moyamoya disease, it was reported that in cases of juvenile onset the vertebral, basilar or posterior cerebral artery was sometimes stenosed or occluded, but, in adult-onset cases, stenosis or occlusion of the posterior cerebral artery would be an abnormality. Our case is a very rare example of unilateral atypical Moyamoya disease of adult onset with basilar artery stenosis.     

Spontaneous improvement of idiopathic intracranial carotid artery dissection: a case report

We report a rare case of spontaneous intracranial carotid artery dissection presenting with multiple infarctions. A 65-year-old man became aware of transient visual disturbance in the right eye and headache. Magnetic resonance imaging (MRI) at a local neurosurgical clinic depicted fresh multiple infarctions of the right cerebral hemisphere along with severe right carotid artery stenosis and delayed cerebral blood flow on the right side. The patient was transferred to our hospital. Three-dimensional computed tomography (3D-CT) showed an intimal flap in the righ petrous internal carotid artery and we diagnosed spontaneous intracranial carotid artery dissection of the petrous portion. We started conservative therapy including anti-coagulant and antiplatelet drug and blood pressure control. Two weeks after admission, angiography, 3D-CT, MR angiography demonstrated that the dissection had improved and the patient was discharged without neurological deficit. In this case, 3D-CT was effective for the diagnosis of intracranial carotid artery dissection. We report this rare case along with a literature review of the clinical profile and related neuroimaging findings.     

Intracerebral hemorrhage in a patient with moyamoya phenomenon caused by tuberculous arteritis: a case report]

A case of intracerebral hemorrhage which probably had been caused by a rupture of abnormal "moyamoya vessels" due to tuberculous arteritis was reported. A 42-year-old female had a history of tuberculous meningitis at the age of 2 years and suffered from a sudden onset of severe headache in January of 1998. CT scan disclosed a medium-sized intracerebral hematoma in the left frontal base and many calcifications in the basal cistern. Subsequent angiography demonstrated high-grade stenosis in the terminal portion of the right internal carotid artery and near-by "moyamoya vessels". No surgery was performed on the patient. A second angiography was carried out two months later and it newly disclosed ophthalmic artery-feeding "moyamoya vessels" in the place where the intracerebral hematoma had been located. This led us to conclude that a rupture of "moyamoya vessels" was the cause of the intracerebral hemorrhage and "moyamoya vessels" were not visible in the first angiograms because they had been compressed by the hematoma. Although cerebral infarction is common in tuberculous arteritis, cerebral hemorrhage is uncommon. The pathogenesis of cerebral hemorrhage due to tuberculous arteritis and its difference from that of hemorrhage caused by moyamoya disease is discussed.     

Multiple intracranial mycotic aneurysm. Report of a case (author's transl)]

A case of intracranial mycotic aneurysm was reported, in whom repeated cerebral angiographies demonstrated the seccessive appearance of multiple aneurysms in a short term after the septic cerebral infection, and the importance of repeated angiography in the treatment of the intracranial mycotic aneurysm was stressed. A 44-year old male who had heart failure developed suddenly a left hemiparesis with irritable meningial signs on Nov. 3, 1973. The right carotid angiography carried out on Nov. 16 in an admitted hospital showed partial obliteration of cortical branches of the middle cerebral shift of the anterior cerebral artery due to the cerebral infarction. No aneurysm was demonstrated in the angiogram. The second angiography of the right carotid and vertebral artery was done on the admission of Dec. 18. The previously shown contralateral shift of the anterior cerebral artery was remarkably decreased, and an aneurysm of 3 mm in diameter was recognized at the cortical branch of the right middle cerebral artery on the parietal region. Any aneurysm was not revealed by the vertebral angiography. The third angiography of bilateral carotid and vertebral artery was performed on Dec. 25. The previously revealed aneurysm increased in size. Multiple aneurysms was demonstrated at the anterior branches of the insular artery. The vertebral angiography demonstrated multiple aneurysms at the peripheral portion of the bilateral posterior cerebral arteries. The patient was improved neurologically after the administration of antibiotics, and was discharged on Dec. 27 on the convenience of his family and would be followed in conservative cares.     

Dissecting aneurysms at the A1 segment of the anterior cerebral artery--two case reports

Two rare cases of dissections which involve the anterior cerebral artery (ACA) are reported. A 58-year-old woman presented with a ruptured dissecting aneurysm manifesting as sudden onset of severe headache and consciousness disturbance followed by aphasia, right hemiparesis, paresis of the left lower extremity, and choreoathetotic movements of the upper arms and face. Computed tomography and angiography revealed subarachnoid hemorrhage due to a dissecting aneurysm at the left A1 segment. The dissecting aneurysm was trapped surgically on the day of onset. Her neurological deficits disappeared within a month. A 39-year-old woman experienced continuous dull headache from the day before onset, and then suffered right hemiparesis. Magnetic resonance (MR) imaging revealed cerebral infarction at the left globus pallidus. Angiography and MR imaging revealed a dissecting aneurysm at the left A1 segment and occlusion of the left Heubner's artery. She received conservative treatment and her neurological findings were improved. Dissections or dissecting aneurysms involving the ACA can be classified into three types: Extension of a dissection to the ACA from the internal carotid artery, dissection at the A1 segment, and dissection at the A2-A4 segments. These types of dissection have distinct uniform clinical features.     

短暂性脑缺血发作与脑动脉狭窄的脑血管造影研究

目的探讨TIA与颅内外血管狭窄的关系,研究DSA对短暂性脑缺血发作(TIA)血管病变的诊断价值。方法选择符合TIA诊断标准的患者54例行颈部血管B超、经颅多普勒(TCD)、心脏超声以及DSA检查,观察TIA患者颈内动脉和椎动脉颅内、外血管有无狭窄及粥样硬化斑块。结果54例患者中发现46例有血管狭窄,其中颈总动脉狭窄2例,颈内动脉狭窄13例,大脑中动脉狭窄5例,锁骨下动脉狭窄3例,椎动脉狭窄18例,基底动脉狭窄5例。狭窄部位斑块31例。其中大动脉狭窄性TIA 36例,栓塞性TIA 13例,腔隙性TIA 3例,血管痉挛性TIA 2例。结论TIA患者大多存在颅内外动脉狭窄及粥样硬化斑块,颅内外动脉狭窄程度及粥样硬化斑块稳定性决定TIA类型。