Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038454 (stroke)
 147,016 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Moyamoya syndrome has occasionally been seen in association with Down syndrome. We report a child with moyamoya syndrome and Down syndrome who was admitted with repeated episodes of strokes; his magnetic resonance imaging and magnetic resonance angiography findings confirmed the presence of occlusive cerebrovascular disease with basal collateral vessels. His protein C levels were significantly decreased during the stroke. Complete clinical recovery was seen during follow-up. This raises the possibility of a link between protein C deficiency and Down syndrome with moyamoya syndrome.
 ...
PMID:Are the strokes in moyamoya syndrome associated with Down syndrome due to protein C deficiency? 1242 21

Moyamoya syndrome has been reported in association with Down syndrome. In paediatric patients, the usual presentation is that of ischaemic stroke. We report a 9-year-old boy with Down syndrome and moyamoya syndrome who presented with acute-onset left hemiparesis. This is the first such reported case in Hong Kong. There is growing evidence that the chromosomal abnormalities in patients with Down syndrome may contribute to a vulnerability for the development of moyamoya syndrome. A high index of suspicion is necessary to make the correct diagnosis. Medical and surgical management strategies for this disease are discussed. Surgical intervention should proceed without delay, if indicated, to prevent further neurological deterioration. A multidisciplinary approach is recommended for the rehabilitation of these patients.
 ...
PMID:Moyamoya syndrome in a child with Down syndrome. 1254 61

Two patients with Down syndrome and the primary antiphospholipid antibody are described. One patient had a vasculopathy similar to that previously described as Moyamoya. Down syndrome is characterized by immune defects including a tendency to autoimmune phenomena. This report extends the scope of these observations and particularly draws out the potential role of antiphospholipid antibodies. Indeed antiphospholipid antibodies may well explain the well-known association of Down syndrome and stroke.
 ...
PMID:Antiphospholipid antibodies and stroke in Down syndrome. 1258 28

A 56-year-old male presented with thrombus formation manifesting as cerebral embolic infarction suspected to be caused by hemostasis at the carotid bifurcation, not by the intimal abnormalities or hematological disorders. Thrombus repeatedly and reproducibly appeared at the same area in spite of carotid endarterectomy (CEA). Ultrasonography demonstrated a stenotic lesion of the cervical carotid bifurcation. Medical treatment reduced the stenosis, but the thrombus was repeatedly formed at the same area of the cervical carotid bifurcation. CEA was performed. Histological examination of the specimen found only the underlying thin intima consisting of mild fibrous atheromatic change without ulceration or vascular dissection. Ultrasonography following CEA showed reduced blood flow, indicating hemostasis, and moyamoya appearance in that area. The thrombus had recurred in spite of the medical treatment with anti-platelet agent. This repeated thrombus was gradually dissolved and reduced with anticoagulant therapy. Thrombus causing cerebral embolic stroke and originating at the cervical carotid bifurcation is usually due to the intimal atherosclerotic change such as ulcer formation or vascular dissection. The thrombus in this case was probably formed by hemostasis at the cervical carotid bifurcation and CEA was not effective to prevent recurrence.
 ...
PMID:Repeated thrombus formation immediately following carotid endarterectomy. 1276 Apr 98

A previously healthy 24-day-old boy presented with a 2-day history of fever and had a convulsion on the day of admission. MRI showed abnormal signal in the thalami, caudate nuclei and central white matter. Acute necrotising encephalopathy was diagnosed, other causes having been excluded after biochemical and haematological analysis of blood, urine and CSF. He recovered, but with spastic quadriparesis. At the age of 28 months, he suffered sudden deterioration of consciousness and motor weakness of his right limbs. MRI was consistent with an acute cerebrovascular accident. Angiography showed bilateral middle cerebral artery stenosis or frank occlusion with numerous lenticulostriate collateral vessels consistent with moyamoya disease.
 ...
PMID:Moyamoya disease in a child with previous acute necrotizing encephalopathy. 1280 40

Moyamoya disease (MMD) is an uncommon intracranial vasculopathy that typically presents with ischemic or hemorrhagic stroke. Persistent choreoathetosis has been identified as a rare early manifestation of MMD. We present 2 patients with paroxysmal dyskinesia as the initial symptom of MMD, one resembling paroxysmal kinesigenic dyskinesia (PKD) and the other paroxysmal non-kinesigenic dyskinesia (PNKD). We also review the cases of moyamoya-induced chorea reported previously, none of which resembled PKD or PNKD. We hypothesize that both hormonal and ischemic factors may be implicated in the pathogenesis of these abnormal involuntary movements. These cases suggest that MMD should be included in the differential diagnosis of PKD and PNKD.
 ...
PMID:Moyamoya-induced paroxysmal dyskinesia. 1450 75

Moyamoya disease is a relatively uncommon neurovascular complication of sickle cell anemia. We report a case series of six patients with sickle cell anemia who developed moyamoya disease and underwent encephaloduroarteriosynangiosis procedures. These six patients presented with either cerebrovascular accidents, transient ischemic attacks, or seizures, and subsequent magnetic resonance imaging scans were suggestive of moyamoya-like changes in the cerebral vasculature. Conventional cerebral angiography was used to confirm the diagnosis in all six patients. Four of six patients manifested a cerebrovascular accident before surgery, and two of these patients were compliant on a transfusion protocol at the time of their cerebrovascular accident. Bilateral (n = 4) or unilateral (n = 2) encephaloduroarteriosynangiosis procedures were performed without any complications. The patient who was stroke-free preoperatively had a cerebrovascular accident 2 weeks after the procedure; otherwise, all patients have remained free of neurovascular complications with an average follow-up of 33 months. Collateral anastomoses between external and internal carotid arteries were established by magnetic resonance angiography in three patients. The encephaloduroarteriosynangiosis procedure is a safe and effective treatment option in patients with sickle cell anemia who develop moyamoya disease.
 ...
PMID:Sickle cell anemia with moyamoya disease: outcomes after EDAS procedure. 1458 Jun 55

We highlight the first case to our knowledge of vascular parkinsonism with angiographic moyamoya and functional imaging correlates. In addition, transmission electron microscopy of the cutaneous vasculature revealed the mean smooth muscle cell layer of the terminal arterioles to be significantly higher compared with ischemic stroke patients classified by vascular risk factors. Smooth muscle cell thickness was also greater in those with small vessel and large vessel strokes compared with cardioembolic strokes. The marked cutaneous arteriolar smooth muscle cell hypertrophy may be a useful surrogate marker for those at risk of developing angiographic moyamoya.
 ...
PMID:Vascular parkinsonism in moyamoya: microvascular biopsy and imaging correlates. 1468 96

Stroke in children is increasingly recognized. The incidence exceeds 8 per 100,000 per year. Important differences in stroke in newborns and children compared with adults, as well as a paucity of clinical trials, create challenges in the diagnosis and management of pediatric arterial ischemic stroke (AIS). The neurological presentation of AIS can be subtle. Radiographic diagnosis of acute AIS is also challenging because CT scan may be normal early on. Risk factors include vascular, intravascular, and embolic disorders; frequently, there are multiple risk factors in a given child, necessitating thorough investigations. More than 50% have a vasculopathy including postvaricella angiopathy, dissection, moyamoya, or vasculitis. Intravascular mechanisms are frequently present, including dehydration. Hematological or prothrombotic conditions are also associated with AIS in children, and include sickle cell disease and prothrombotic disorders. The latter have been identified in from one third to one half of children with AIS, are usually acquired, and frequently act in concert with other risk factors for stroke. The most common embolic source is congenital heart disease, which is present in 25% of children with AIS. Outcomes include death in 6% and neurological deficits in two thirds of children. Given that no clinical trials have been completed in pediatric stroke to date, treatment is empiric. Initial neuroprotective strategies aim to reduce the size of the infarct. For older children antithrombotic agents (antiplatelet drugs and anticoagulants) are given to reduce the 20 to 30% risk of recurrence. There are coordinated research efforts currently being initiated, which over the next decade will result in clinical trials in this understudied condition.
 ...
PMID:Arterial ischemic strokes in infants and children: an overview of current approaches. 1471 73

Recent development of non-invasive diagnostic technology, such as magnetic resonance imaging (MRI) and angiography (MRA), is believed to have made possible on increase in the diagnoses of asymptomatic moyamoya disease. However, no criteria have been established for the management of such cases. The present study aimed to clarify the natural history of asymptomatic moyamoya disease retrospectively. Ten patients were included in this study. None of them had experienced any episode due to moyamoya disease and were only incidentally diagnosed as having moyamoya disease. There were 4 males and 6 females. Their ages ranged from 30 to 67 years, with the mean age of 46.2. Cerebral angiography showed there was the tendency of disease progression in elder patients. MRI detected cerebral infarction in 3 of 10 patients (30%). Hemodynamic ischemia, such as impaired reactivity to acetazolamide and/or elevated oxygen extraction fraction, was observed in 4 of 10 patients. Only one patient underwent surgical revascularization. Antiplatelet or anticonvulsant medication was administered in 5 of 10 patients. The mean follow-up period was 4.1 years, ranging from 0.5 to 13 years. During follow-up periods, the moyamoya lesion markedly progressed and caused cerebral infarction in one patient. However, neither ischemic nor hemorrhagic stroke occurred in the other 9 patients. Multi-center nation-wide study should be planned to clarify the natural course of asymptomatic moyamoya disease and establish the management guidelines for patients with asymptomatic moyamoya disease.
 ...
PMID:[Clinical features and outcomes of 10 asymptomatic adult patients with moyamoya disease]. 1471 42


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>