1/451. Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease. We diagnosed phakomatosis pigmentovascularis type IIIb in an 11-month-old baby who had a giant nevus spilus, a nevus flammeus, and moyamoya disease. Development of the patient was normal until 6 months of age when he developed a sudden onset of focal seizures and left hemiparesis. This patient represents the sixth case of phakomatosis pigmentovascularis type IIIb, including three cases in the Japanese literature, reported thus far. However, to our knowledge, this is the first case with an association to moyamoya disease. ( info) |
2/451. Simple delineation of misery perfusion areas by superimposition of PET on PET images. oxygen extraction fraction images were superimposed on cerebral blood flow images of positron emission tomography (PET) to depict the so-called misery perfusion area in two patients with moyamoya disease. The superimposed image following extra-intracranial bypass surgery was obtained in one case, which convincingly disclosed attenuation as compared with the preoperative image. This simple PET on PET approach should find an application in the evaluation of misery perfusion and in assessing operative indications for cerebral ischaemic diseases. ( info) |
3/451. Ocular malformations, moyamoya disease, and midline cranial defects: a distinct syndrome. PURPOSE: To report a 10-year-old girl with developmental anomalies of both optic disks, a chorioretinal coloboma, sphenopharyngeal meningoencephalocele, and moyamoya disease. methods: A full ophthalmologic examination, cranial magnetic resonance imaging and magnetic resonance angiography, and cerebral angiography were performed. RESULTS: The patient had a morning glory disk anomaly and microphthalmos of the right eye and optic nerve hypoplasia and retinochoroidal coloboma in the left eye. She had a midfacial cleft and an episode of seizures and a stroke. magnetic resonance imaging showed a sphenopharyngeal meningoencephalocele. magnetic resonance angiography and cerebral angiography demonstrated a pattern consistent with moyamoya disease. CONCLUSIONS: This patient had a distinct syndrome of optic disk, retinochoroidal, and carotid circulation anomalies with midline cranial defects. The recognition and treatment of the vascular abnormalities and cranial defects may prevent complications such as strokes that may occur during or after general anesthesia. ( info) |
4/451. Demonstration of cerebral perfusion abnormalities in moyamoya disease using susceptibility perfusion- and diffusion-weighted MRI. We describe the use of diffusion-weighted imaging and perfusion MRI using a contrast-medium bolus in the preoperative investigation for young man presenting with a cerebral ischaemic episode as a manifestation of moyamoya disease. ( info) |
| We present a woman with moyamoya disease. The patient was born by prolonged vaginal delivery after a long pregnancy lasting 43 weeks. During her first years of life she depicted poor psychomotor development. When she was 9 years old a diagnosis of moyamoya disease was made. Annual neurologic evaluations have revealed moderate motor deficiency, slurred speech and borderline mental capacity. At 26 years of age she became pregnant and was prophylactically treated with 40 mg/day oral nicardipine. She had a healthy child after an uncomplicated 40 week pregnancy. When she was 33 years old a magnetic resonance (MR) study disclosed a zone of ischemic parenchyma. MR angiography yielded results similar to those of conventional arteriography. At present, the patient is 36 years old, lives a normal life and works in a factory. ( info) |
| Moyamoya is a vascular occlusive disease typically limited to the cerebral arterial system. We report a case of severe stenosis of the left main and right coronary arteries occurring in association with moyamoya disease, supporting the concept that moyamoya may be an intracranial manifestation of a systemic arterial disorder. ( info) |
7/451. Moyamoya syndrome in a patient with congenital human immunodeficiency virus infection. A 10-year-old boy with congenital human immunodeficiency virus (hiv) infection developed recurrent episodes of left hemiparesis at age 7 years. The progression of his disease was followed by computed tomography, magnetic resonance imaging, magnetic resonance angiography, and cerebral angiography. The series of images showed progressive stenosis of both carotid arteries at the suprasellar origin with involvement of his anterior and middle cerebral arteries, while prominent collateral vessels developed from his external carotid supply through ophthalmic and middle meningeal arteries. The pattern of cerebrovascular disease is consistent with moyamoya syndrome. We suggest that further studies on the pathophysiology of cerebrovascular disease in patients with hiv could be helpful in understanding the cause of moyamoya disease as well. Also, with the various advances in treatment of hiv, neurovascular complications could be seen more frequently as the long-term survival in these patients improves. ( info) |
| A 7-year-old boy and a 10-year-old girl presented with moyamoya disease showing atypical angiographic findings. In these cases, the internal carotid artery (ICA) had a tapering occlusion just distal to the origin of the ophthalmic artery, whereas the top of the ICA was not occluded and was retrogradely supplied through the posterior communicating artery from the posterior circulation. Surgical treatment resolved the symptoms in both patients. moyamoya disease may include a number of variant types not showing all the characteristic angiographic findings of moyamoya disease. ( info) |
| 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. ( info) |
10/451. 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. ( info) |