1/95. Central deafness in a young child with moyamoya disease: paternal linkage in a Caucasian family: two case reports and a review of the literature.A case of 'central deafness' is presented in a 3-year-old male Caucasian child with moyamoya disease (MMD); a rare, progressive and occlusive cerebrovascular disorder predominantly affecting the carotid artery system. documentation of normal peripheral auditory function and brainstem pathway integrity is provided by acoustic admittance, otoacoustic emission and brainstem auditory evoked potential measurements. The lack of behavioral response to sound, and absent middle and long latency auditory evoked potentials suggest thalamo-cortical dysfunction. magnetic resonance imaging showed diffuse ischemic damage in subcortical white matter including areas of the temporal lobes. In addition, there were multiple and focal cortical infarctions in both cerebral hemispheres, focused primarily in the frontal, parietal and temporal areas. Taken together, these structural and functional abnormalities in addition to severely delayed speech and language development are consistent with the diagnosis of central deafness and suggest a disconnection between higher brainstem and cortical auditory areas. The child's father also has MMD, but was diagnosed only recently. The presence of paternal linkage is informative since it rules out x-linked recessive and maternal inheritance. To our knowledge, this represents the first documented case of paternal linkage in MMD with central deafness in a Caucasian child with no apparent Japanese ancestry. Herein, we focus on central auditory dysfunction and consider how lesion-induced changes have contributed to a deficit in basic auditory responsiveness, including a severe disturbance in receptive and expressive auditory-based speech and language skills.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/95. moyamoya disease of adult onset brain stem haemorrhage associated with bilateral occlusion of the vertebral arteries--case report.An unusual and first case of moyamoya disease of adult onset brain stem haemorrhage associated with occlusion of both vertebral arteries is reported. A 30-year-old man suddenly suffered from dyspnea, dysphagia, and left-sided hemisensory disturbance. Computed tomography and magnetic resonance imaging revealed a fresh haematoma in the left medulla oblongata and various-sized old infarcts in both parietal lobes. Cerebral angiograms disclosed occlusion of the bilateral internal carotid arteries on both sides at their intracranial portion, accompanied with the developed basal moyamoya vessels. The right vertebral artery occluded at its V2-V3 segment, in which the posterior inferior cerebellar artery was opacified via the posterior spinal artery, and the basilar artery was filled from the anterior spinal artery. The left vertebral artery was also occluded at the craniovertebral junction (V4) with collateral flow. Only one case of moyamoya disease associated with bilateral occlusion of the vertebral artery has been reported previously, and a haemorrhage into the medulla oblongata in moyamoya disease has never been described.- - - - - - - - - - ranking = 220.45662949623keywords = haemorrhage, brain (Clic here for more details about this article) |
3/95. A case of moyamoya-like vessels combined with brain anomaly.We report here a rare case of moyamoya-like vessels combined with brain anomaly. MR imaging revealed a small corpus callosum and stenosis of the internal carotid arteries. T2-weighted images revealed multiple hyperintense lesions in the cerebral deep white matter, suggesting ischemic or abnormal myelinated tissues. cerebral angiography showed aplastic carotid siphons and anomalous aneurysmal dilatation of the petrous portion of the internal carotid arteries. The terminal portion of the internal carotid arteries and horizontal portion of the middle cerebral artery were stenotic with moyamoya-like vessels.- - - - - - - - - - ranking = 1.6666666666667keywords = brain (Clic here for more details about this article) |
4/95. moyamoya disease mimicking a spontaneous internal carotid artery dissection on Doppler ultrasound.moyamoya disease and spontaneous internal carotid artery dissections are rare conditions, but both tend to affect young adults with potentially devastating consequences. A 43-year-old non-Japanese patient presented with neurological symptoms, which, following carotid Doppler ultrasound and angiography, was labelled as being due to a spontaneous internal carotid artery dissection. Repeat imaging at 3 months showed normalisation of the carotid Doppler findings which coincided with the formation of "moyamoya" vessels on the angiogram. This case report illustrates the changes on carotid ultrasound in early moyamoya disease which may mimic the appearances of an internal carotid artery dissection and demonstrates the change of the spectral Doppler waveform that occurs with the formation of new vessels at the base of the brain.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
5/95. Hemorrhagic moyamoya disease during pregnancy.BACKGROUND: Intracranial hemorrhage in pregnant patients with moyamoya disease is rare. We review the case of one such patient who presented with pre-eclampsia and a catastrophic intracerebral hemorrhage in order to highlight the associated management difficulties. methods: A case of a pregnant (31 weeks) female brought to the emergency department with hypertension and a progressive decrease in her level of consciousness is presented. She rapidly developed a dilated right pupil and left extensor posturing. A CT scan of her head showed a large putamenal intracerebral hemorrhage. She was intubated, ventilated and given intravenous mannitol and magnesium sulfate. She underwent a simultaneous craniotomy and cesarean section. Post-operatively the patient's ICP and jugular venous saturation were monitored in the intensive care unit. RESULTS: The patient delivered a 1185 g infant who did well. The patient's ICP was well controlled until the tenth post-operative day when she developed malignant brain edema and died. CONCLUSION: This case highlights three important points. First, simultaneous craniotomy and cesarean section can be performed. Second, intraoperative control of bleeding Moyamoya vessels is described. Third, the difficult post-operative management of these cases is highlighted. The literature regarding moyamoya disease and pregnancy is reviewed and some recommendations for the management of this rare but potentially deadly condition are presented.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
6/95. pulse-inversion contrast harmonic imaging: ultrasonographic assessment of cerebral perfusion.pulse-inversion contrast harmonic imaging is a new ultrasonographic technique that can assess brain perfusion. In an adult with moyamoya disease and multiple recurrent strokes, this method detected subtle hemispheric differences in temporal-lobe perfusion, presumably due to neovascularisation, which were not shown by xenon-computed tomography or magnetic resonance perfusion imaging.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
7/95. radiation-induced aneurysm and moyamoya vessels presenting with subarachnoid haemorrhage.A rare case of a ruptured internal carotid artery (ICA) aneurysm, with ICA stenosis and moyamoya vessels, was diagnosed in a young female patient after radiotherapy for optic glioma. Retrospective analysis revealed that an extraordinarily high dose of radiation (110 Gy) had been delivered to the area around the ICA in two therapeutic sessions. Clipping and wrapping of the aneurysm were performed and the patient made a good recovery. A review of the literature suggested that rupture of radiation-induced aneurysms is a rare event, but may be fatal. Considerable care must therefore be taken when delivering high-dose radiation to cerebral arteries. We suggest that the incidence of radiation-induced moyamoya vessels is higher in younger patients.- - - - - - - - - - ranking = 145.85997521971keywords = haemorrhage (Clic here for more details about this article) |
8/95. Management of residual dysplastic vessels after cerebral arteriovenous malformation resection: implications for postoperative angiography.OBJECTIVE: The verification of surgical resection of cerebral arteriovenous malformations (AVMs) relies on angiography. Abnormal vasculature often is identified after removal of the AVM. Differentiation of dysplastic feeding vessels that resemble the neovascularity of moyamoya disease, as distinct from residual AVM, is crucial for preserving critical brain areas. We review a large experience with immediate postoperative angiography after AVM resection and discuss the implications for management of abnormal dysplastic vessels discovered after AVM resection. methods: Beginning in 1992, 86 consecutive patients with AVMs underwent operations by standard protocol for immediate postoperative angiography under the same general anesthetic. Angiographic interpretation dictated admission to the intensive care unit or return to the operating room for further resection. RESULTS: In 78 patients, the angiogram revealed complete resection. Two patients were returned to the operating room, one for residual malformation with an early draining vein, and one for resection of residual dysplastic vessels. There was one postoperative hemorrhage in a patient whose postoperative angiogram was falsely negative for AVM. Six patients with residual dysplastic vessels mimicking residual AVM, but without an early draining vein, were managed conservatively. Delayed follow-up angiography demonstrated spontaneous involution of these abnormal vessels in all of these patients. CONCLUSION: Residual dysplastic feeding vessels resembling the neovascularity of moyamoya disease but not associated with an early draining vein do not necessarily represent residual malformation after AVM resection. The abnormal vessels will proceed to complete spontaneous resolution. Given the difficulty of interpreting intraoperative angiography, immediate postoperative angiography may be a viable alternative after AVM resection.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
9/95. Hypothalamic-pituitary dysfunction associated with moyamoya disease in children.moyamoya disease is a rare cerebral vascular disease that results in narrowing of the vessels of the circle of willis and the formation of a network of collateral vessels at the base of the brain for compensatory perfusion. moyamoya disease has the highest incidence during the first decade of life, and children present most frequently with transient ischemic attacks. We present two cases of moyamoya disease in children with associated hypothalamic-pituitary dysfunction. Both children presented to the endocrinologist for decreased growth velocity. One child had hypothyroidism and both had growth hormone deficiency. A review of the literature reveals a few isolated case reports of hypothalamic-pituitary dysfunction occurring with moyamoya disease and with other states of cerebral vascular insufficiency. We suggest that children with compromise of cerebral vascular perfusion be monitored closely for clinical signs and symptoms of hypothalamic-pituitary dysfunction.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
10/95. moyamoya disease associated with a brain stem glioma.An 8-year-old boy was found to have primary moyamoya disease associated with a brain stem glioma. For over 3 years the child had experienced transient ischemic attacks induced by hyperventilation. One month before referral to our hospital he had presented with progressive left facial nerve palsy. magnetic resonance imaging showed a cystic mass in the lower pons. Angiography revealed severe bilateral stenosis of the internal carotid arteries and prominent moyamoya vessels in the basal ganglia. Partial resection of the tumor yielded a histological diagnosis of pilocytic astrocytoma. Local radiation therapy reduced the size of the tumor. Anastomosis of the superficial temporal arteries and middle cerebral arteries on both sides was then performed. After direct bypass surgery, the patient remained in a good condition for a 5-year follow-up period. Clinical investigation of the coincidence of primary moyamoya disease and brain stem glioma led the authors to conclude that these two diseases coexisted independently.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
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