Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 4-year-old child who developed hemiplegia 6 months after varicella-zoster virus (VZV) infection. Cerebral angiography showed complete occlusion of the right middle cerebral artery with basal moyamoya vessels. Elevation of anti-VZV antibody in the cerebrospinal fluid indicated central nervous system involvement. The association between VZV cerebral angitis and unilateral occlusion of right middle cerebral artery is discussed.
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PMID:Unilateral occlusion of the middle cerebral artery after varicella-zoster virus infection. 1189 Nov 3

We reported a patient with cardio-facio-cutaneous (CFC) syndrome associated with moyamoya syndrome. The patient was referred at 6 years 5 months with left hemiplegia and right-sided eye deviation. He had an apparently short stature, macrocephaly, left ptosis and atopic skin, and was odd looking. He exhibited an incomplete right bundle branch block on electrocardiogram and an atrial septal defect on ultrasound cardiography. He was diagnosed as having CFC syndrome. Head magnetic resonance imaging showed a flow void in the bilateral basal ganglia, but did not show any ischemic changes. Magnetic resonance angiography showed bilateral stenosis with an internal carotid artery at the Willis artery ring level and bilateral moyamoya. Contrast angiography demonstrated occlusion of both middle cerebral arteries. Cerebrovascular anomalies have not previously been reported in CFC syndrome. This is the first case of CFC syndrome associated with moyamoya syndrome.
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PMID:Cardio-facio-cutaneous syndrome and moyamoya syndrome. 1201 68

The patient is a 35-year-old man who had a medical history of epilepsy in childhood. He came to our hospital because of transient disturbance of consciousness and left hemiplegia just after evacuation. At first, we thought that he had epilepsy with Todd's palsy. But we had to do a differential diagnosis for a transient ischemic attack such as paradoxical embolism, because his symptoms occurred just after evacuation. An electroencephalogram and brain computerized tomography were immediately performed, but no abnormality was detected. Hematologic studies were normal, and no deep vein thrombosis was detected in the veins of the lower extremities by duplex ultrasonography Doppler. But carotid duplex ultrasonography showed an increase in end-diastolic flow velocity and a decrease in vascular resistance in both external carotid arteries. These findings indicated that there was arteriovenous malformation such as moyamoya disease. Brain magnetic resonance imaging showed spotty high signal lesions in the subcortical areas on a fluid-attenuated inversion-recovery(FLAIR) image, and the middle cerebral artery was not visualized on magnetic resonance angiography (MRA). Cerebral angiography demonstrated moyamoya vessels in the brain and collateral circulation from the external carotid artery. Therefore, we diagnosed him as having moyamoya disease. Duplex ultrasonography of the common and, internal carotid, and vertebral arteries is a widely-used technique. Recently, cerebral angiography, MRA and transcranial Doppler have been applied to detect intracranial vascular malformation. But these results suggested that moyamoya disease could be detected by means of carotid duplex ultrasonography. Finally, we considered that carotid duplex ultrasonography was not only a noninvasive screening method but also a useful for the diagnosis of moyamoya disease.
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PMID:[Usefulness of carotid duplex ultrasonography in a patient with moyamoya disease]. 1472 40

Secondary Moyamoya disease, also known as Moyamoya syndrome has been rarely associated with Down syndrome. In pediatric patients, the usual presentation is that of ischemic stroke. Here is reported a 4-year-old child with Down syndrome and Moyamoya syndrome who presented with acute-onset right hemiplegia. A high index of suspicion is necessary to make the diagnosis.
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PMID:Moyamoya syndrome in a child with down syndrome. 1607 50

The aim of this study was to review cases of pediatric arterial ischemic stroke among Chinese subjects and thereby evaluate risk factors, clinical and neuroimaging features, and treatment, to establish a reasonable guideline for assessment and management of the disease. Between 1996 and 2006, 157 children (male:female ratio, 1.4:1) with arterial ischemic stroke were identified at Beijing Children's Hospital. The median age at stroke was 32 months (range, 4-192). Among patients with determined etiology, infections (12.1%), moyamoya disease (12.1%), and trauma (10.8%) were the most common. In 51 patients, there were no obvious risk factors (32.5%). Hemiplegia was the most common presenting feature (81.5%). The region of left middle cerebral artery was most frequently affected (36.3%), followed by the right middle cerebral artery (29.9%). Of the 157 patients, 56 were treated by intravenous thrombolytic agents (35.7%), all but one of them successfully (the one exception involving hemorrhagic complication). Randomized controlled trials are needed to establish primary prevention, acute treatment, and secondary prevention of pediatric ischemic stroke.
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PMID:Arterial ischemic stroke: experience in Chinese children. 1863 65

We experienced a case in which the cause of acute brain swelling following resection of dura matter could not be recognized until the postoperative CT scan. A 30-year-old woman presented with a 4-year history of Moyamoya disease. Right hemiplegia developed a month before operation, so that the anti-platelet therapy was continued. At the end of dural resection the brain started to bulge and the brain swelling increased progressively. The patient was immediately placed on a head up position and received a rapid administration of mannitol for the treatment. The operator performed the echo examination for clarifying the cause of the brain swelling, but they could not find it. As the brain swelling slightly improved by the treatment, the surgery was performed continuously At the end of the operation the patient was moved for a CT scan and it showed mass effect caused by epidural hematoma over the left temporal region distant from the site of craniotomy. Evacuation of the hematoma was carried out urgently. At discharge, she was conscious and had no focal neurological deficits. The occurrence of the epidural hematoma is one of the reasons for unknown brain swelling during surgery. We strongly recommend that any sudden deterioration during the operation should be treated with emergency CT scan.
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PMID:[Postoperative epidural hematoma remote from the site of craniotomy for STA-MCA anastomosis]. 1854 10

Craniopharyngioma management is challenging. Although histology is benign, the tumour can be clinically aggressive with local invasion and frequent recurrences. Extensive morbidity may be present at diagnosis and furthermore, occurs as a consequence of neurosurgery and radiotherapy. Hypothalamic symptoms can have a devastating effect on quality of life and may reduce life expectancy. This case highlights both the challenge of managing hyperphagia and morbid obesity and the importance of initial treatment preserving existing hypothalamic function and the need to avoid tumour recurrence and further surgery. A 11-year old boy presented with hydrocephalus secondary to a craniopharyngioma (he had visual failure and hypopituitarism but few hypothalamic symptoms). He underwent radical resection followed by radiotherapy. Following this treatment, he developed psychological and behavioural problems and hyperphagia. Weight gain in the first year (an increase from +1.4 to +3.7 s.d.) resulted in poor mobility and a fall which caused a slipped femoral epiphysis. In the next year, there was a 6-month period of unexpected weight loss (+4.2 to +3.8 s.d.) that culminated in emergency treatment for diabetic ketoacidosis secondary to severe insulin resistance. He developed a left hemiplegia, and a subsequent cerebral angiogram identified multiple stenoses of the Circle of Willis with a Moyamoya appearance secondary to radiotherapy. Weight gain has continued (+3.8 to +5.5 s.d.) so that bariatric surgery is a management option.
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PMID:'Do no harm': management of craniopharyngioma. 1877 78

Moyamoya disease is the angiographic diagnosis of a clinical syndrome showing bilateral stenosis or occlusion of the distal internal carotid arteries and their major branches with extensive parenchymal, leptomeningeal, or transdural anastomoses. The clinical features normally present as reversible ischemic neurologic deficits, sensory-motor attacks with acute hemiplegia, and motor convulsion. An acute confusional state (ACS) among hospitalized patients is a frequent and serious problem. It is characterized by an acute neurologic deficit with a fluctuating course of impaired attention span, unorganized thinking, and altered levels of consciousness. We report a case of 66-year-old woman who presented with an ACS in the emergency department. The subsequent workups including a neuroradiological examination revealed a rare case of moyamoya disease with bifrontal ischemic infarction. The recognition of an ACS as a manifestation of moyamoya disease should therefore be included in the differential diagnosis of elderly patients who present with an acutely altered neuropsychiatric state. A prompt diagnosis may help to select the most appropriate therapy for this rare disorder especially in elderly patients.
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PMID:Moyamoya disease presenting with an acute confusional state in an elderly patient. 2043 55

Moyamoya vasculopathy is a progressive, occlusive vasculopathy leading to ischemic and hemorrhagic strokes. No treatments are established to treat acute ischemic stroke with moyamoya vasculopathy. A 3-year-old girl with moyamoya syndrome developed acute left hemiplegia. Emergent angiography showed near-occlusion of the supraclinoid segment of the right internal carotid artery. Balloon angioplasty was performed within 6 hours of symptom onset, with significant improvement in the child's neurological symptoms. This is the youngest case of intracranial balloon angioplasty, and this article discusses the paucity of data regarding angioplasty and other forms of endovascular intervention in pediatric cerebrovascular disease and moyamoya vasculopathy. Further study is needed to determine the safety and efficacy of endovascular intervention in these diseases.
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PMID:Angioplasty for acute stroke with pediatric moyamoya syndrome. 2050 56

Moyamoya disease is a rare stroke syndrome of unknown etiology resulting from stenosis or occlusion of the supraclinoid internal carotid artery (ICA) in association with an abnormal vascular network in the basal ganglia. Although the highest incidence of moyamoya disease is in pediatric patients, pathology reports have been primarily limited to adult samples and describe occlusive fibrocellular lesions in the intimae of affected arteries. We describe the case of a young girl with primary moyamoya disease who presented at 18 months of age with right hemiparesis following an ischemic stroke. Angiography showed stenosis of the distal left ICA, left middle cerebral artery, and right ICA. An emergent left-sided dural inversion was performed. Recurrent strokes and alternating hemiplegia necessitated a right dural inversion 6 months later. Nonetheless, her aggressive disease proved uniquely refractory to surgical revascularization, and she succumbed to recurrent strokes and neurological deterioration at 2.5 years of age. Pathological specimens revealed a striking bilateral occlusion of the anterior carotid circulation resulting from intimal proliferation of smooth muscle cells (SMCs). Most strikingly, the ascending aorta and the superior mesenteric artery demonstrated similar intimal proliferation, along with SMC proliferation in the media. The systemic pathology involving multiple arteries in this extremely young child, the first case of its kind available for autopsy, suggests that globally uncontrolled SMC proliferation, in the absence of environmental risk factors and likely resulting from an underlying genetic alteration, may be a primary etiologic event leading to moyamoya disease.
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PMID:Diffuse and uncontrolled vascular smooth muscle cell proliferation in rapidly progressing pediatric moyamoya disease. 2080 8


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