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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Moyamoya disease is a neurological disease rarely seen in children outside Japan. It is difficult to differentiate moyamoya disease in its early stage from mitochondrial disorders in children when nontraumatic ischemic stroke is considered. We present a 14-month-old Taiwanese female child who had suffered from sudden onset of nontraumatic seizure attack, after which progressive left limb weakness was noted. Initial brain imaging and a series of laboratory studies were performed which suggested mitochondrial disorders, especially mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome. The patient underwent an oral glucose lactate stimulation test (OGLST), and mitochondrial disorders were not favored. MR angiography of the circle of Willis showed moyamoya disease. The patient then underwent an encephalo-duro-arterio-myo-synangiosis (EDAMS) operation. Evaluation of the neurodevelopmental and intelligence outcome required close long-term follow-up.
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PMID:Moyamoya disease initially mimicking MELAS syndrome in a 14-month-old child. 1533 22

A 10-year, retrospective review of the etiology, outcome, and complications of ischemic stroke in children from a nonurban population was conducted. Twenty-seven children were identified (14 boys, 13 girls), ages 1.25 to 17 years (mean 7.7 years). Etiologies included undetermined (22%), arterial dissection (19%), coagulopathy (15%), embolism (15%), moyamoya disease (11%), sickle cell disease (11%), isolated angiitis of the central nervous system or vasculitis (11%), or other known source (11%; two fibromuscular dysplasia, one L-asparaginase). More than one risk factor was present in five children. Seventeen (65%) children were anticoagulated, with no adverse events occurring. Nine children were anticoagulated initially with low-molecular-weight heparin. Other treatments included corticosteroids; physical, occupational, and speech therapy; and anticonvulsants for concomitant seizures. Follow-up ranged from 3 to 60 months (mean 17 months) and was as follows: 6 (22%) were normal, 9 (33%) had mild impairment, and 12 (44%) had moderate to severe deficits. There were no deaths. Neurologic complications included seizure (two), behavioral problems (two), and hemorrhagic conversion (one). In this population, the outcome from ischemic stroke was similar to that of other studies, with the majority of children demonstrating persistent neurologic deficits. Etiology could be determined for the majority of patients, with 19% having more than one risk factor.
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PMID:Childhood ischemic stroke in a nonurban population. 1583 8

Moyamoya disease is a cerebrovascular disease characterized by stenosis and occlusion of the arteries of the circle of Willis, with abnormal telangiectatic collateral circulation at the base of the brain. An association between moyamoya disease and neurofibromatosis 1, a neurocutanoeus disorder, is well established in the literature. However, its association with other neurocutaneous syndromes is infrequently reported. Hypomelanosis of Ito, another neurocutaneous syndrome, is characterized by macular hypopigmented skin whorls and variable neurologic involvement. Only one case study of an association between hypomelanosis of Ito and moyamoya disease has been reported in the English literature. We report a 17-year-old girl with both hypomelanosis of Ito and angiographic moyamoya disease. She presented with intractable seizures, progressive left hemiparesis, and skin manifestations of hypomelanosis of Ito. Although one might consider a coincidental association, this second case points to an association between the two disorders. Detailed neuroimaging, in particular angiography, should be considered in children with hypomelanosis of Ito and abnormal neurologic findings.
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PMID:Hypomelanosis of Ito and Moyamoya disease. 1641 66

The authors present the case of a 6-year-old girl with typical absence epilepsy induced by hyperventilation associated with moyamoya disease (MMD). A diffuse 3-Hz spike-and-wave complex induced by hyperventilation was apparent on an electroencephalogram, and her seizures were intractable to medication. Significant ischemia in the bilateral frontal lobes was present. The epilepsy disappeared after superficial temporal artery-middle cerebral artery anastomosis with encephalomyosynangiosis on both sides. In the treatment of children with intractable absence epilepsy, the possibility of underlying MMD and indications that revascularization surgery may be needed should be taken into consideration.
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PMID:Absence epilepsy associated with moyamoya disease. Case report. 1661 38

We report a 1-year-old girl who presented moyamoya disease associated with midaortic syndrome. She had been treated for cardiac failure and severe hypertension due to midaortic syndrome until she suffered seizure and repeated cerebral ischemic attack. Cerebral angiography revealed stenosis of the bilateral internal carotid artery at its terminal portion. She was successfully treated with encephaloduroarteriosynangiosis, and ischemic attack ceased postoperatively. This is the first report of moyamoya disease with midaortic syndrome. Although cerebral ischemic attack has been effectively managed by encephaloduroarteriosynangiosis, renovascular hypertension is still difficult to control.
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PMID:Moyamoya disease associated with midaortic syndrome. 1719 Sep 91

We describe an unusual vasculopathy in two sisters of non-consanguineous parents. The first child developed an acute hemiparesis and focal seizures at the age of 6 months during a febrile illness. Magnetic resonance imaging (MRI) of the brain showed bilateral cortical-subcortical infarction not confined to a vascular territory. Subsequently, the child had a persistent stable neurological deficit. Her younger sister had a similar encephalitis-like episode at the age of 4 months, with left-sided cortical-subcortical ischaemic lesions. Two months later she had left-sided focal seizures. MRI showed a right-sided cortical enhancement, magnetic resonance angiography (MRA) was normal. The neurological deficit was stable and she was seizure free. These episodes were initially interpreted as metabolic strokes, but work-up was normal and mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) was excluded. In their teens both sisters were diagnosed with pulmonary and systemic hypertension and, due to the arterial hypertension, myocardial hypertrophy. Renal artery stenosis, pathological pulmonary arteries, and stenosis and rarefication of coronary arteries were found; the aorta and retinal vessels were normal. Repeat cranial MRI and MRA showed multiple collaterals, while the carotid and basilar arteries were extremely narrowed (moyamoya appearance). We suggest the diagnosis is a hereditary systemic vasculopathy of unknown origin.
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PMID:Siblings with infantile cerebral stroke and delayed multivessel involvement--a new hereditary vasculopathy? 1742 10

Surgical revascularization for moyamoya disease prevents cerebral ischemic attacks by improving cerebral blood flow (CBF). Seizure is known as a rare complication after revascularization for moyamoya disease, although its underlying mechanism is undetermined. We investigated the relationship between seizure attack and postoperative alteration in CBF in patients with moyamoya disease. CBF was routinely measured by N-isopropyl-p-[123I] iodpamphetamine (123I-IMP-SPECT) 1 and 7 days after superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis on 64 sides of the 44 consecutive patients (male:female = 13:31, 2-62 years old) with moyamoya disease. Three patients (male:female = 1:2, 40-55 years old) suffered from seizure attack at 1 to 10 days after surgery. Two of the three patients manifested as seizures at 8-10 days after surgery and presented transient neurologic deteriolation before seizure. Postoperative SPECT revealed significant increase in CBF at the sites of the anastomosis in all three patients. Postoperative magnetic resonance imaging showed no ischemic changes, and magnetic resonance angiography showed the apparently patent STA-MCA anastomosis as thick high signal intensity sign in all three patients. The anatomical location and the temporal profile of hyperperfusion were completely in accordance with the focus of seizure attack. Intensive blood pressure control and the use of antiepileptic agents were instituted. The neurologic deficits were resolved and no seizure attack recurred in three patients. Seizure following STA-MCA anastomosis can be caused by hyperperfusion in patients with moyamoya disease. When seizure attacks occur, routine CBF measurement is recommended to differentiate hyperperfusion and ischemia, since the treatments for these conditions are contradictory.
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PMID:[Seizure following superficial temporal-middle cerebral artery anastomosis in patients with moyamoya disease: possible contribution of postoperative cerebral hyperperfusion]. 1749 42

In this retrospective study, we reviewed the charts and collected clinical and radiographic data on children (age range, 1 month to 18 years) with symptoms and radiographic confirmation of ischemic stroke for the period of January 1996 to July 2006. Ninety-four children were enrolled. Eighty-eight had arterial ischemic stroke and six had sinovenous thrombosis. Twenty-nine percent of the children had seizures. Twenty-six percent had diffuse neurological signs and 76% had focal neurological signs. Risk factors included vascular disease (33%), infection (27%), metabolic disorders (18%), trauma (11%), prothrombotic states (13%), cardiac disease (10%), and mitochondrial disease (6%). Ten percent (n=9) had no identifiable cause. Twenty-two percent of the children had more than one risk factor. Anterior territory (70%) was more involved than posterior territory (18%) in arterial ischemic stroke. Unilateral infarctions were more common on the left side (51%) than on the right (24.5%). Neurological deficits were present in 45% (n=34/75) of the children; the most frequent deficit was motor impairment (24%). Seven children (9%) died in the acute stage. There were 12 children (16%) who had recurrent stroke and 8 children (8/12) who had underlying vascular disease. The vascular disease included moyamoya disease (5), CNS lupus (1) and ill-defined vasculopathy (2). The etiology pattern in Taiwan was different from that in Western countries. Vascular disease was a significant risk factor for recurrence in childhood ischemic stroke.
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PMID:Risk factors and outcomes of childhood ischemic stroke in Taiwan. 1757 20

Cerebrovascular Moyamoya disease (MMD) is a clinical entity characterized by stenotic or occlusive lesions around the terminal portions of the internal carotid arteries (ICA) and the formation of abnormal vascular networks at the base or convexity. This condition is sporadic, and there are no pathological explanations for this cause. A case with unilateral juvenile MMD is reported. A 13-year-old girl with sudden episode of left limbs weakness, accompanied by transient dysphasia and seizures attack, was admitted to our hospital for further investigation. Computed tomography scanning showed an area of localized brain swelling in the right capsular-thalamic region. Angiographic findings demonstra-ted unilateral involvement. Cerebral panangiography reve-aled a high grade stenosis of the right supraclinoid ICA and a peculiar network (moyamoya phenomenon) formed by dilated basal collateral perforating arteries and produced a cloudy image resembling "a puff of smoke". Left carotid angiogram showed a collateral circulation through the anterior communicant artery and apportioned to the right cerebral hemisphere. The vertebrobasilar system was normal. An ultrasoud Doppler study demonstrated a severely decreased blood flow gradient (0.1 l/min) through the right hypoplastic ICA, while the left common and the left ICA showed remarkable increased blood flow, partly due to abondant collateral vessels. Acquired, non-progressive vitiligo with areas of complete pigment loss appeared on flexor wrists, extensor distal extremities, superior eyelids and especially on joints regions (elbow, knee and small joints of the hands and fret). This association has not been reported previosly.
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PMID:[Unilateral moyamoya disease associated with acrofacial vitiligo in a 13-year-old patient--case report]. 1797 97

Moyamoya disease is characterized by progressive steno-occlusive changes at the terminal portions of the bilateral internal carotid arteries with arterial collateral vessels at the base of the brain. As the incidence of moyamoya disease is highest in Japanese and Asians, Japanese researchers have made enormous efforts to illuminate the pathogenesis, the epidemiology, the clinical features and the treatment of this disease for five decades. The disease was called variously according to each researcher's assumption of the pathogenesis in the early period. After presentation of the initial nationwide data summarized by Nishimoto, et al. at the Symposium Neuroradiologicum in Paris in 1967, the disease was named Nishimoto's disease in Europe. Since the publication in the English literature by Suzuki, et al. in 1969, the term "moyamoya disease" has been accepted in the international community. The etiology of moyamoya disease is still unknown. Genetic backgrounds have been suggested in familial moyamoya disease. Angiogenetic cytokines may play a role in progression of steno-occlusive changes and/or angiogenesis of collaterals. The patients with moyamoya disease present with a variety of clinical symptoms, such as ischemic deficits, intracranial hemorrhage, sensory disturbance, involuntary movement, seizures and headache. Ischemic symptoms dominate in children, though intracranial hemorrhage is more common in adults. The ischemic attacks in children are often provoked by hyperventilation. Intracranial hemorrhage is mainly caused by hemodynamic stress on fragile moyamoya vessels. Many surgical options, including indirect bypass, direct bypass and their combination, have been developed to increase cerebral blood flow for patients with ischemic problems. Ongoing studies may elucidate the pathogenesis of the disease and efficacy of bypass surgery for hemorrhagic cases.
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PMID:[Moyamoya disease]. 1823 31


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