Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Moyamoya disease is a chronic occlusive cerebrovascular disorder characterized by stenosis of the intracranial internal carotid artery often accompanied by stenosis of the anterior and/or middle cerebral arteries. This results in cerebral ischemia, which manifests clinically as transient, repetitive episodes of hemiplegia, dysarthria, and involuntary movements. This case report documents a patient in whom an initial extracranial-intracranial bypass (superficial temporal-middle cerebral artery) failed to alleviate the ischemic symptoms. In a subsequent procedure, a pedicle graft of omentum was created and through a subcutaneous tunnel was placed on the right cerebral cortex. Over a 2 1/2-year period, this has resulted in a dramatic resolution of the patient's symptomatology. The report delineates the condition and reviews other therapeutic options.
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PMID:Encephalo-omental synangiosis in the management of moyamoya disease. 173 85

A case of adult Moyamoya disease, with formation of a transcranial external carotid-internal carotid (EC-IC) anastomosis through burr holes which had been made previously. A 43-year-old male suffered sudden headache and vomiting. Neurological examination revealed mild consciousness disturbance and dysarthria. The computed tomography (CT) scans showed intraventricular hemorrhage, which was drained through burr holes bifrontally. The diagnosis of Moyamoya disease was subsequently made by cerebral angiography. A month later he was discharged with mild gait disturbance and mental retardation. Seven years later he suddenly complained of gait disturbance, dysarthria and sensory disturbance involving the right upper extremity. A CT scan revealed a small hemorrhage in the left putamen. Carotid angiograms disclosed transcranial EC-IC anastomosis through the burr holes which had been made previously. It is suggested that revascularization can be expected after opening burr holes and incising the dura matter for Moyamoya disease in adults as well as, possibly, in children.
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PMID:[Adult moyamoya disease with a transcranial internal carotid-external carotid (EC-IC) anastomosis through burr holes]. 237 49

A 58-year-old female was admitted to our hospital because of orthostatic syncope, and difficulty in urination and walking for 2 years. At age 35, she suffered from cerebral hemorrhage and was diagnosed as Moyamoya disease (MD). Neurological examination on admission revealed severe orthostatic syncope so that she could not stand. At that time, her blood pressure and heart rate were 104/74 mmHg and 78/min in supine position and 52/48 mmHg and 52/min in 60 degrees head-up position. She also had severe pollakisuria, cerebellar ataxia and dysarthria. Cranial nerves, motor strength and deep reflexes were normal. ECG, chest X-ray, and EEG were normal. Aschner, Czermak, and cold pressor tests revealed no response. Urodynamic study revealed autonomic bladder. MRI showed only enlarged fourth ventricle because of atrophy of the pons. Bilateral CAG revealed "Moyamoya" vessels in the cerebral basal regions. Cerebral blood flow (CBF) was measured by 133Xe inhalation method. Mean arterial blood pressure changed from 134 mmHg to 126 mmHg in 45 degrees head-up tilting and CBF decreased from 47.5 mg/100 g/min to 37 ml/100 g/min position. Though there was no relationship between SDS and MD, each one shows dysautoregulation of CBF, it is supposed that a severe orthostatic syncope attack was resulted from synergism of both effects.
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PMID:[An association of Shy-Drager syndrome with moyamoya disease--a case report]. 240 Nov 16

10 young patients from 7 to 16 years of age with moyamoya disease were treated by temporal muscle graft (encephalo-myo-synangiosis: EMS) for the purpose of increasing extracranial and intracranial collateral circulation. Ischemic cerebrovascular symptoms and signs were encountered in all of them without intracranial hemorrhage. The patients were observed for 6-26 months after the first operation. Transient ischemic attacks disappeared in 4 out of 7 patients, and improved in frequency and duration in the remaining 3. Paresis of the limbs disappeared in 1 patient and improved in 2. In 2 patients, facial palsy disappeared and dysarthria improved as well. Ataxia disappeared in 2 patients, and involuntary movement of the extremities disappeared in 2. There were no ineffective cases clinically. In postoperative carotid angiography, the middle cerebral arteries were visualized clearly to the peripheral portion mainly via the thickened deep temporal arteries, and the abnormal vascular networks in the region of the basal ganglia reduced in size in most of patients. Improvements in intelligence quotient, electroencephalogram and cerebral blood flow were also obtained in several patients, respectively. EMS seemed to be an effective surgical treatment in young patients with moyamoya disease who suffered from cerebral ischemic symptoms.
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PMID:Treatment of moyamoya disease by temporal muscle graft 'encephalo-myo-synangiosis'. 682 12

We report a case of a rapidly growing cerebral aneurysm in the basal abnormal vascular network associated with spontaneous middle cerebral artery (MCA) occlusion. The aneurysm disappeared spontaneously shortly after performing STA-MCA anastomosis. A 54-year-old female was admitted to our hospital because of repeated attacks of right hemisensory disturbance and dysarthria. CT scan and MRI images showed the infarcted focus in the left parieto-occipital lobe. Bilateral MCAs were undetectable on MRI images. Cerebral angiography revealed that the bilateral MCAs were occluded in their proximal origin with basal abnormal vascular networks. The distal MCA branches were perfused via the vascular networks. A small aneurysm was detected in the distal portion of the left Heubner's artery. There were no abnormalities in the bilateral internal carotid arteries, the anterior cerebral arteries, and the basilar artery. The follow-up angiography performed 29 days after admission revealed a growing aneurysm with a diameter of 3 mm in the distal enhanced lesion consistent with the aneurysm observed in the angiography. An STA-MCA anastomosis was performed for improvement of cerebral misery perfusion. Single photon emission tomography (SPECT) performed 9 days after the bypass operation revealed improvement of cerebral blood flow in the left parieto-occipital lobe, and her TIA attacks disappeared. The aneurysm was undetected in the cerebral angiography performed 24 days after the bypass operation. Spontaneous MCA occlusion is a rare condition of chronic cerebrovascular occlusive diseases. Diagnostic criteria of the disease includes the MCA occlusions or stenosis with basal abnormal vascular networks. Usually the phenomenon is seen unilaterally, which differs from moyamoya disease. Diagnosis must exclude diseases caused by the etiologies such as those of arteriosclerotic origin. There have been 24 reported cases of spontaneous MCA occlusion including our case. Among them, 9 cases presented cerebral aneurysm located in abnormal vascular networks, and all the reported cases presented cerebral hemorrhage at their onset. The abnormal basal vascular network may be developed as collateral vessels to supply blood to the ischemic regions in this disease. An increased hemodynamic stress in the abnormal basal vascular network may produce a true aneurysm in the distal portion of the perforating arteries. STA-MCA anastomosis reduced the TIA attacks, but also decreased the hemodynamic stress on the abnormal basal vascular network and resulted in reduction in size or thrombosis of the aneurysm. STA-MCA anastomosis can be considered effective to treat cerebral aneurysms located in vessels with increased hemodynamic stress.
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PMID:[A case of spontaneous middle cerebral artery occlusion associated with a cerebral aneurysm angiographically disappearing after STA-MCA anastomosis]. 926 66

We present a rare autopsy case of von Recklinghausen's disease with Moyamoya vessels and arteriovenous malformation. A 58-year-old female patient suffered from dysarthria and dysphagia. On examination, Parkinson's signs, pseudobulbar palsy, and muscular weakness of the left extremity and pyramidal tract signs were observed. An enhanced brain computed tomography revealed abnormal high-density network vessels at the thalamus and midbrain. By cerebral angiography, the following changes were observed; occlusion of the right internal carotid artery at the bifurcation, and abrupt narrowing and occlusion of the left internal carotid artery at the bifurcation and siphon. A lateral vertebral arteriography revealed telangiectasia at the basilar tip. The patient died of pulmonary thromboembolism at age 61. The vessels of the circle of Willis were hypoplastic. The optic nerves, infundibulum and mammillary body were covered with a large number of ectastic vessels. Arteriovenous malformations were observed in the bilateral occipital lobes. Histopathologically, the elastic lamina of Moyamoya vessel was conspicuously wavy and often duplicated or triplicated, and discontinued occasionally. Discontinuity of the elastic lamina of the perforating arteries and circumferential arteries supplied by the middle cerebral artery (MCA), anterior CA (ACA) and posterior CA (PCA), was also occasionally observed. It is likely that the Moyamoya vessels in this patient were compensatorily formed by congenital hypoplasia of the internal artery, MCA, ACA and PCA.
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PMID:Moyamoya disease with concurrent von Recklinghausen's disease and cerebral arteriovenous malformation. 965 50

A previously healthy 61-year-old man presented with basal ganglia hemorrhage caused by rupture of a small aneurysm arising from the distal lenticulostriate artery associated with moyamoya-like disease and manifesting as left hemiparesis and dysarthria. The patient underwent frontotemporal craniotomy. Neck clipping of the aneurysm was performed through the hematoma cavity. He was transferred for rehabilitation with left hemiparesis. Three-dimensional computed tomography angiography was very useful for preoperative planning and evaluation of the anatomical correlation between the aneurysm and the hemorrhage.
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PMID:Surgical treatment of a lenticulostriate artery aneurysm. Case report. 1630 16

35-year old patient was admitted to the Department of Neurology, Medical University of Bialystok because of paresis of his left upper limb, progressing over last 10 months and right facial nerve paresis, which started a month ago. During neurological examination he presented with right facial and arm paresis, dysarthric speech, obesity and hypertension. The patient was previously hospitalized in regional hospital, where a lumbar puncture was done revealing normal composition of cerebrospinal fluid. His brain CT revealed bilateral hypodensive areas in frontal and parietal regions of vasogenic character. Doppler ultrasound showed significant slowing of blood flow velocity in both internal carotid arteries. Brain angiography presented with very weak contrast filling of intracranial branches of carotid and vertebral arteries and showed stenosis at the terminal portion of the internal carotid arteries and at the proximal portion of the anterior and middle cerebral arteries. The patient had transthoracic and transesophageal echocardiography, Holter ECG, lab tests (routine lab tests plus coagulation system evaluation with C protein resistance test, anticardiolipne antibodies, antinuclear antibodies, anticytoplasmatic antibodies and thyroid hormones) checked--all tests were within normal range. Based on cerebral angiography and clinical symptoms, after excluding any other reasons of cerebral ischemia, the patient was diagnosed with moyamoya disease and arterial hypertension. The patient was treated pharmacologically with improvement--regression of face assymetry and dysarthria and diminishing of his right arm paresis. The authors of this paper pay attention to moyamoya disease as a rare reason of ischemic strokes in the young in our geographic region. They remind moyamoya disease diagnostic criteria, its etiology and treatment.
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PMID:[Moyamoya disease as a cause of ischemic cerebral stroke in young people]. 1744 85

Headache associated with moyamoya disease (HAMD) is common in moyamoya disease. However, the characteristics and classification of HAMD are largely unknown. We present a case of a 39-year-old woman with HAMD. To characterize and classify the features of this syndrome, the patient was asked to complete a 4-month diagnostic headache diary. There was a total of 15 ictal days. All episodes were without aura. The headache was more commonly pressing (10/15), mild to moderate in severity (14/15), unchanged by physical activity (11/15), and associated with photophobia (10/15). The International Headache Society Classification was utilized to determine that eight episodes met criteria for probable migraine without aura, while seven episodes met criteria for probable frequent episodic tension-type headache. We identified four other case reports of HAMD with partial descriptions of the characteristics. When combined with our patient, the median age was 34 years old (range 6-49, SD 16). Four were female, while the patient with cluster headache was male. The median time from headache onset to diagnosis with moyamoya disease was 9.5 months (range 0-192, SD 88.0). Headaches were described as migraine with aura in two of five cases, hemiplegic migraine in one of five, and cluster headache in one of five. The highest intensity was described as severe in three of three cases, in which headache intensity was reported. Meanwhile, nausea, vomiting, and photophobia were present in two of three cases, where these features were reported, while nausea without vomiting was seen in one of three cases. In all five cases, patients had other neurological symptoms, such as paresis, seizures, visual disturbances, dysarthria, allodynia, ptosis, and unilateral restless leg syndrome. In conclusion, HAMD can present as migraine without aura. It can be the first presenting symptom of moyamoya disease. The headache features are not diagnostic; hence, early neurovascular imaging should be considered in patients with new onset, refractory migraine-like headache, especially in the setting of other neurological symptoms to exclude underlying moyamoya disease. Further reports using headache diaries are needed to better characterize HAMD as well as to determine whether headache with tension-type features is also part of this condition.
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PMID:Headache associated with moyamoya disease: a case story and literature review. 2001 51

Stroke during pregnancy or the puerperium is an extremely rare yet serious cause of perinatal morbidity and mortality. Moyamoya disease, a cerebrovascular occlusive pathology with a female predominance, may become symptomatic for the first time in association with pregnancy. A 36-year-old woman with postpartum pre-eclampsia suddenly developed hemiparesis in the left arm with dysarthria after the initiation of antihypertensive measures. Cranial magnetic resonance imaging and angiography revealed acute ischemic lesions in the right hemisphere along with the steno-occlusive lesions of bilateral terminal portions of the internal carotid artery, indicating Moyamoya disease. With anti-platelet medication, the patient recovered gradually and was diagnosed as having Moyamoya disease using conventional angiography 3 months postpartum. In this case, the fluctuations in blood pressure in association with pre-eclampsia appear to have exacerbated the clinical symptoms of Moyamoya disease. As such, Moyamoya disease should be considered as an underlying disease of ischemic stroke associated with pregnancy.
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PMID:Moyamoya-disease-related ischemic stroke in the postpartum period. 2014 50


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