Figure - available via license: Creative Commons Attribution-NonCommercial-ShareAlike 3.0 Unported
Content may be subject to copyright.
Four-vessel cerebral angiography showing bilateral stenosis of the supraclinoid segment of both internal carotid arteries and tortuous branches from the anterior and middle cerebral artery. These abnormal vessels were found anastomosing with branches from the posterior cerebral and middle meningeal arteries. (a and b) Left and right carotid arteries (antero-posterior view); (c) right carotid artery (lateral view); (d) left vertebral artery (lateral view)
Source publication
The presentation of moyamoya disease (MMD) as an aneurysmal subarachnoid hemorrhage (SAH) is relatively frequent and in the absence of aneurysms is extremely rare.
A 53-year-old male patient suddenly developed severe headache associated with dysarthria and an altered state of consciousness. At the time of admission, he was found drowsy with global...
Citations
... Notably, TDA was the bleeding origin of subarachnoid hemorrhage (SAH) onset in patients with MMD where aneurysm was not confirmed. [6][7][8] However, an aneurysm arising from TDA, although rare, has been reported. TDA is common in MMD. ...
A 24-year-old woman with hemorrhagic onset moyamoya disease received bilateral indirect bypass surgery 11 years ago. She presented with a life-threatening atraumatic acute right subdural hematoma with temporal lobe intracerebral hemorrhage due to a transdural anastomosis (TDA) aneurysm rupture. We reviewed six cases of rare TDA aneurysms, all of which occurred around the temporal base. TDA aneurysms may occur near the main trunk of the middle meningeal artery (MMA) where hemodynamic stress is high. Therefore, we must pay attention to skull base aneurysms that form near the MMA; otherwise, the prognosis would be poor if such an aneurysm ruptured.
Fullsize Image
... Moreover, intracranial hemorrhage in patients with moyamoya disease is extremely rare unless there is a clear cerebral aneurysm on angiography. Intracranial hemorrhage is most commonly due to rupture of fragile vessels caused by moyamoya disease [8]. There was one case in the literature review similar to our case. ...
Moyamoya disease is a chronic, progressive bilateral occlusion or stenosis of terminal internal carotid arteries as well as the proximal anterior and middle cerebral arteries. Hemorrhage of the splenium of the corpus callosum rarely occurs with moyamoya disease. In this article, we report a case of a 53-year-old woman diagnosed with moyamoya disease by cerebral angiography. She presented to the emergency department complaining of unsteadiness and a tendency to fall forward for one week. The patient was investigated with head computed tomography (CT) scan upon presentation revealing atypical location of hemorrhage in the corpus callosum, mainly in the splenium.
... Moyamoya disease · Subarachnoid hemorrhage · Collateral circulation · Transcranial Doppler ultrasonography A very rare clinical manifestation of Moyamoya Disease (MMD) is subarachnoid hemorrhage, which is not due to the ruptured intracranial aneurysms (NASAH). This is an extremely rare condition and to our knowledge only five other cases [1][2][3][4][5] have been described in the literature; out of them only one was familial [3] . Moreover, there is currently no study reporting the hemodynamic features of intra and extracranial circulation assessed with ultrasound examination. ...
Moyamoya disease is a chronic cerebrovascular disease characterized by progressive stenosis and/or occlusion of the intracranial internal carotid and its proximal branches with a created abnormal collateral network of small arteries. If the vascular occlusion is not bilateral and the patient has been diagnosed with a specific underlying illness, we refer to this condition as Moyamoya syn- drome. The presentation of Moyamoya disease or syndrome in the form of a non-aneurysmal subarachnoid hemorrhage is rare. The 62-year-old woman was admitted for 24 hours lasting pain in the area of the neck, forehead, and temples. She was somnolent, vomited repeatedly, and had double vision. The brain computed tomography discovered subarachnoid hemorrhage. The brain CT angiography revealed an occlusion of the left middle cerebral artery and the digital subtraction angiography, moreover, showed a network of tiny tortuous moyamoya vessels in the left middle cerebral artery territory. The brain perfusion was not impaired. The patient was treated with corticosteroids and anti-platelet drugs. A diagnosis of Moyamoya syndrome was determined. She was dismissed without neurological deficit or subjective problems. After six years, the patient`s general condition deteriorated because of sepsis, and even after getting maximum antibiotic therapy, she died.
Moyamoya disease or syndrome should be considered in the differential diagnosis as the cause of subarachnoid hemorrhage in the absence of other clear vascular pathology as its potential source.
Purpose:
To report three rare causes of fatal spontaneous subarachnoid haemorrhage (SAH) and to discuss the clinical presentations, neuroimaging findings of the patients, and a brief review of the literature on these unusual causes of SAH.
Case reports:
Anomalous branches of the internal carotid artery (ICA) are remarkably rare and multiple aneurysms associated with these aberrant arteries a rarer phenomenon still. A case of multiple proximal and distal aneurysms of the main trunk of an aberrant ICA branch, which resulted in a World Federation of Neurosurgical Societies (WFNS) grade V SAH, was presented. This abnormal vessel had an arterial fenestration, another rare occurrence. The second patient had a craniocervical junction (CCJ) arteriovenous fistula, which was associated with a distal aneurysm extending to the upper cervical spinal canal. The patient presented with recurrent SAH which had been misdiagnosed multiple times in the past. The third case presentation is that of a WFNS grade IV SAH, which occurred secondary to a ruptured giant fusiform aneurysm of the supraclinoid segment of the left ICA. The first two cases manifested with multiple episodes of Fisher grade IV SAH, and all cases proved fatal.
Conclusion:
It is crucial for clinicians to ensure prompt angiographic studies in patients presenting with spontaneous subarachnoid haemorrrhage, as delay in the definitive diagnosis/intervention can be lethal. In particular, a high index of suspicion for a vascular brain lesion should be entertained in cases of repetitive SAH.
Moyamoya, a rare angiographic finding, is characterized by chronic and progressive stenosis at the terminal end of the internal carotid artery, followed by collateralization of the cerebral vasculature at the base of the skull. Coined by Suzuki and Takaku in 1969, the term "moyamoya" means a "puff of smoke" in Japanese, a reference to the angiographic appearance of moyamoya collateralization. Moyamoya is most commonly found in East Asian countries, where much governmental and civilian effort has been expended to characterize this unique disease process. However, despite its rarity, the occurrence of moyamoya in Western countries is associated with significant divergence regarding incidence, gender, sex, age at diagnosis, clinical presentation, and outcomes. Here, we attempted to review the Western literature on moyamoya presentation using the PubMed database to characterize the Western phenotype of moyamoya. We were guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for scoping reviews (PRISMA-ScR). We reviewed papers generated from a search with keywords "moyamoya case report," those reported from a Western institution, and those reported on a relevant association. Our scoping review demonstrated various clinical associations with moyamoya. Moreover, we summarized the demographic profile and clinical symptomatology, as well as reported disease associations to better elucidate the Western phenotype of moyamoya.
Woman of 46 years old that income for left hemiplegia and dysarthria, with brain computed tomography showed right frontoparietal hemorrhage drained ventricles and arteriography with Moyamoya pattern. Readmission 3 months later with deterioration of consciousness and brain computed tomography showed temporoparietal intracerebral hemorrhage with intraventricular extension and deviation from the midline, was managed with tracheostomy and gastrostomy, presented nosocomial pneumonia and sepsis, finally, after 15 days of hospitalization she died at the intensive care unit
Moyamoya disease patients with subarachnoid hemorrhage and cerebral infarction are rare, especially when the subarachnoid hemorrhage is nonaneurysmal. Here, we present one 48-year-old male patient with moyamoya disease identified with digital subtraction angiography. His initial symptoms are associated with increased intracranial pressure. Subsequent computed tomography demonstrated subarachnoid hemorrhage and cerebral infarction. Digital subtraction angiography showed no obvious aneurysms. We assume that subarachnoid hemorrhage is associated with the rupture of the moyamoya vessels and transdural anastomotic vessels. The cerebral infarction can be also explained by hemodynamic mechanisms. We should pay more attention to the recurrent hemorrhagic stroke.
Introduction:
Clinical presentation and etiology of localized nontraumatic convexal subarachnoid hemorrhage (cSAH) have been described in a few patients. They differ from those of aneurysmal subarachnoid bleeding which is diffuse. The purpose of this study was to describe the clinical presentation, the radiologic findings and causes of cSAH.
Methods:
We selected patients admitted to the neurology department of CHU of Nîmes or Montpellier, from May 2008 to May 2011, who presented with cSAH, observed in a single cortical sulcus unrelated to trauma and identified on brain MRI T2* weighted images as a hyposignal in one sulcus of the convexity. Data collection was retrospective.
Results:
Twenty-three patients (14 men and nine women) were included. Mean age was 69.5years (range 29-86). Patients had mostly sensory or sensorimotor deficits which was regressive in less than 30minutes, recurrent, and seldom accompanied by headache. Brain MRI allowed the identification of patients with old brain hematomas (n=2), lobar microbleeds (n=7) and superficial cortical hemosiderosis (n=6). The etiologic diagnosis was determined in 43% (n=10/23): cerebral amyloid angiopathy (n=3), reversible cerebral vasoconstriction syndrome (n=2), primary cerebral angiitis (n=1), posterior reversible encephalopathy syndrome (n=1), cortical vein thrombosis (n=3, two of them associated with dural sinus thrombosis). Cerebral angiography was performed in 11 patients and gave the etiologic diagnosis (angiitis, cortical vein thrombosis) in two. Follow-up was available for 16 patients (mean 12months, range 3months to 5years). Etiology was established during follow-up in two patients, both had cerebral amyloid angiopathy diagnosed after recurrent lobar hematomas.
Conclusions:
cSAH has various causes, but clinical presentations appear to be relatively stereotyped with recurrent and brief episodes of sensorimotor deficits. A comprehensive assessment and monitoring would lead to an etiologic diagnosis in some patients.
