Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1762617 (weakness)
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One familial case of "moyamoya" disease affecting three patients is reported. The patient in Case 1 was a 28-year-old female. She had suffered from motor weakness of the right limbs in her infantile period. She visited our hospital because of sudden headache and left motor weakness associated with nausea and vomiting. On admission, CT scan revealed cerebral hemorrhage in the right caudate nucleus with intraventricular clots and infarction in the left parietal lobe. Angiography showed stenosis of the left ICA terminal portion and occlusion of the right side, with moyamoya vessels in the basal area. The patient in Case 2 was a 54-year-old female, who was the mother of Case 1. After an operation for acute upper intestinal bleeding, she suffered from cerebral infarction. CT scan revealed large low density areas in the territory of the bilateral MCA. Angiography showed stenosis of the bilateral ICA terminal portions, occlusion of the right MCA, stenosis of the left MCA, and moyamoya vessels in the basal area. The patient in Case 3 was a 40-year-old female, who was a younger sister of Case 2. She had a convulsive attack in her infantile period. She visited our hospital because of gradually worsening headache. CT scan revealed multiple infarctions in the left paraventricle, the right parieto-occipital and occipital lobe. Angiography showed occlusion of the bilateral ICA terminals with moyamoya vessels in the basal and the ethmoidal areas. The patient in Case 2 died immediately. Surgery for reconstruction of hemodynamics was performed in Case 1 and 3.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[One pedigree of "moyamoya" disease]. 189 26

Two cases are reported of occlusive disease of the bilateral internal carotid arteries with dementia in which the anastomosis of the superficial temporal artery to the middle cerebral artery (STA-MCA bypass) resulted in marked improvement in clinical aspects. One patient was a 29-year-old male who complained of transient weakness of the extremities and memory impairment. Computerized tomography (CT) scans showed multiple small infarctions, while cerebral angiography demonstrated findings of Moya-like disease. The cognitive function tests were subnormal and the study of cerebral blood flow (CBF study) showed diffuse low flow in both hemispheres. Based on his clinical symptoms and CBF study, the STA-MCA bypass was performed on both sides in two stages without complications. Postoperatively, his clinical symptoms and cognitive function improved gradually, in accordance with increased CBF in both hemispheres. Six months after the operation, cognitive function tests were within normal limits. Another patient was a 61-year-old hypertensive male who complained of motor weakness, impairment of memory and urinary incontinence. CT scans showed multiple small infarctions, while cerebral angiography revealed occlusion of both internal carotid arteries at the cervical portion. The cognitive function was at the pre-dementia level, and CBF study revealed diffuse low flow in both hemispheres. Based on the clinical symptoms and CBF study, the STA-MCA bypass was performed on both sides in two stages. Postoperatively, clinical symptoms and cognitive function markedly improved. From our results, the diagnosis of vascular dementia, and indications for the use of STA-MCA bypass in this category of patients are discussed.
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PMID:[Anastomosis of the superficial temporal artery to the middle cerebral artery for occlusive disease of the bilateral internal carotid arteries with dementia]. 224 99

A rare case of spontaneous regression of cerebral arterio-venous malformation (AVM) is reported. A 76-year-old male was admitted to Juzen General Hospital due to generalized convulsion on August 24, 1987. On admission, results of physical and neurological examinations were normal. Plain CT scans showed an iso-density lesion with a slightly high density spot in the left frontal lobe, and enhanced CT scans showed multiple, irregularly tubular enhancements in the lesion. Left carotid angiogram (CAG) demonstrated an arteriovenous malformation (AVM) in the left frontal lobe, which was fed by the left ACA and MCA and drained by the ascending cerebral vein and superficial Sylvian vein. There was also an anterior communicating artery aneurysm. At the patient's request, he was treated conservatively and was discharged without neurological deficit. Though he continued to take anticonvulsants thereafter, he felt weakness or numbness of the right extremities once a year. Five years after the first admission, he developed sudden onset of right hemiparesis, aphasia and consciousness disturbance. On admission, his platelet aggregation function was elevated. At this time, enhanced CT scans did not show any enhanced area in the left frontal lobe where AVM had been found previously. T2-weighted magnetic resonance image showed a mixed intensity area without any flow void phenomenon suggesting thrombosis of the nidus. Left CAG demonstrated occlusion of the A1-A2 junction of the anterior cerebral artery and disappearance of the AVM. He was treated conservatively again, and was discharged without neurological deficit.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Spontaneous regression of cerebral arterio-venous malformation following major artery thrombosis proximal to dominant feeders: a case report]. 773 80

We investigated the clinical profile, etiological factors, neuropsychological features and radiological characteristics of 17 cases of striatocapsular infarction (SCI). SCI was defined as the following CT criteria: the area of infarction included the internal capsule and striatum, the maximum diameter of the lesion exceeded 2.0 cm without cortical involvement. There were 9 men and 8 women with mean age of 58 years. Five patients had lesions mainly involving the caudate head (anterior type) and the other 12 had lesions mainly involving the putamen (lateral type), 6 with left side lesion and 6 with right side lesion. Motor weakness was observed in all patients, and the upper extremities were preferentially involved, while in 9 patients face, upper and lower extremities were simultaneously involved. Etiological investigation revealed that 8 patients were cardioembolic stroke, 2 were artery-to-artery embolism and 2 were MCA stem occlusive disease, while the remaining 5 were undetermined. When compared with patients with lacunar infarction (LI), patients with SCI had significantly more frequent cardioembolic sources (47% vs 17%, p < 0.05) and less frequent hypertension (41% vs 80%, p < 0.01). In acute phase, neuropsychological abnormalities were found in 15 patients. Anterior type patients had psychiatric symptoms such as abulia, depression and agitation, while left lateral type patients had aphasia and right lateral type patients had hemispatial neglect or anosognosia. These symptoms gradually improved, although in most patients subtle abnormalities lasted over chronic phase. In 11 out of 13 patients who underwent SPECT using 99mTc-HMPAO, blood flow was decreased in overlying cerebral cortex besides the infarcted area.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Clinical investigation of striatocapsular infarction]. 833 92

We reported a case of adult moyamoya disease showing acute deterioration in which a unilateral occlusive lesion progressed to bilateral involvement during a period of 49 months. A 54-year-old male suffered from progressive weakness in his right extremities and disturbance of speech. On admission, neurological examination demonstrated mild right hemiparesis with global aphasia. A computed tomography (CT) scan showed a strange low density area in the territory of the left ACA and frontal branches of the left MCA. Left carotid angiography showed stenosis of the ICA at the terminal portion, occlusion of the ACA and MCA at each origin, and basal moyamoya vessels. Right carotid angiography demonstrated no occlusive lesions except for the general narrowing of the right ACA, without moyamoya vessels. This case was classified as adult-onset "probable" moyamoya disease according to the diagnostic criteria of the Japan Cooperative Research Committee. Left superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis was performed. The patient showed gradual amelioration of neurological symptoms, and was discharged on foot with mild motor aphasia. However, 4 years later he complained of weakness in his left lower extremity. A second cerebral angiography, performed 49 months after the initial angiography demonstrated severe stenosis of the left ICA at the terminal portion progressing in the proximal direction with decreasing basal moyamoya vessels, while severe stenosis extended from the terminal portion of the right ICA to the M1 portion with newly developed moyamoya vessels in the right basal area. At this time, we considered that this case was a definite case of moyamoya disease. The second cerebral angiography was completed uneventfully. However, the patient then suddenly showed acute deterioration 5 hours after the angiography. Emergent cerebral angiography disclosed an occlusion of the right ICA at the terminal portion. Thereafter he showed acute deterioration in consciousness. A CT scan at the time revealed a huge low density area in the territory of the right ACA and MCA. We performed emergent external decompression, but he did not recover from the sequelae of cerebral infarction and died 3 days later. This rare case suggested the possibility of progression to bilateral involvement although it was a case of adult-onset "probable" moyamoya disease. We consider that acute ischemic deterioration should be kept in mind especially in aged cases of adult-onset moyamoya disease. Careful observation should be performed by the serial MR angiography in adults with "probable" moyamoya disease even though they are clinically asymptomatic.
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PMID:[A case of adult moyamoya disease showing fulminant clinical course associated with progression from unilateral to bilateral involvement]. 899 Apr 73

Transcranial Doppler sonography (TCD) has been increasingly used to detect embolic signals. A 49-year-old woman had repeated episodes of weakness and numbness of the right limbs. She was diagnosed as having transient ischemic attacks (TIAs). A cranial Gadolinium-enhanced MRI revealed multiple small lesions in the cortical-subcortical areas of the left middle cerebral artery territory. A TCD examination on admission detected 4 embolic signals on the left MCA trunk in a 30-minute examination period. TIAs completely ceased after the initiation of an antiplatelet therapy with aspirin. Embolic signals were not detected in later TCD studies. The embolic signals on the TCD strongly indicated that TIAs are caused by an embolic mechanism. TCD is potentially useful for assessing the efficacy of antiplatelet and anticoagulant therapies in patients with TIAs.
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PMID:[Disappearance of embolic signals on transcranial Doppler sonography following antiplatelet therapy in a patient with transient ischemic attacks]. 974 81

In moyamoya disease, progression of carotid occlusive lesion in an adult patient is very rare. We report a case of adult moyamoya disease with acute angiographical stage progression and hemodynamic deterioration. A 56-year-old female complaining of left motor weakness visited our department. On MRI, infarct lesion was found in the right white matter. On cerebral angiography, occlusive lesions in the bilateral internal carotid arterial siphons and moyamoya vessels were found. The right side was stage V and the left side was stage III. On IMP-SPECT, decreased cerebral hemodynamic reserve of the right cerebral hemisphere was found. In this patient, right STA-MCA anastomosis was performed. After operation, she became possible to walk and discharged to home. Five months after operation, good collateral formation and improvement of hemodynamic reserve in the right hemisphere were found. However, on left carotid arteriography, the anterior and middle cerebral arteries were only slightly imaged, and the disease state progressed to stage IV. In addition, decreased blood flow and hemodynamic reserve were appeared in the left hemisphere.
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PMID:A case of adult moyamoya disease showing progressive angiopathy on cerebral angiography. 1053 11

Pure motor stroke is the commonest lacunar syndrome, but it may be associated with nonlacunar mechanisms of infarction. Pure motor brachiofacial weakness has been considered as a partial syndrome depending on a lacunar mechanism. We studied the correlations between stroke type, topography of infarction and etiology in 22 patients with pure motor brachiofacial weakness who were consecutively admitted to our stroke unit during a 10-year period. Seventeen patients had a small deep infarct, 4 had a cortical infarct in the superficial MCA territory and 1 had no specific lesion. The part of the cardiovascular risk factors was about 36% for smoking, 13% for diabetes mellitus, 60% for dyslipidemia and 40% for heart disease. Hypertension was present in 75% of our cases. None of the patients had a large artery stenosis on Doppler ultrasonography. We concluded that brachiofacial pure motor stroke is not always correlated to lacunar infarcts and may be due to a cortical infarct. MRI should be performed when brain CT is normal because of the implications it may have in management and therapy.
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PMID:Brachiofacial pure motor stroke. 1143 77

Nontraumatic arterial dissection of the anterior cerebral artery (NAD-ACA) is a relatively rare disease entity, although case reports have recently been increased. We treated 6 patients suffering from NAD-ACA from January 1996 to December 2003, and the neuroradiological findings together with the clinical courses were reviewed. There were 3 males and 3 females with a mean age of 57.7-year-old, ranging from 41 to 65. Five patients had a past history of hypertension and one diabetes mellitus. At the onset, all patients presented with clinical manifestations of cerebral ischemia. Among them, all exhibited contralateral hemiparesis with greater weakness of the lower extremity, and two patients exhibited headache. Initial angiography revealed the pearl and string sign in four patients and string sign, tapered occlusion in each one. Follow-up angiographies revealed sequential changes in all patients; four improved and two progressed. Main anatomic site of the lesion was as follows; five in the A2 and one in the A1 portion, in addition, one patient was complicated by saccular aneurysm, one by PCA dissection, and two had with saccular aneurysm contralateral ACA & MCA and VA dissection each other. Four patients were treated conservatively by intravenous administration of argatroban, one by intravenous administration of Dextrane and one by anti-platelet agent in the acute stage. All patients were treated by anti-platelet agents in the chronic stage. Good recovery was achieved in five patients, but one who suffered from severe subarachnoid hemorrhage in the chronic stage died. Our experience suggests that hypertension and/or the succeeding abnormal structural changes in the arterial wall may contribute to the occurrence of this disease. NAD-ACA showing clinical manifestations of cerebral ischemia could result in a relatively good prognosis; however, attention should be paid to patients treated conservatively with a very closed follow-up angiography to prevent a possibility of severe hemorrhage.
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PMID:[Nontraumatic arterial dissection of the anterior cerebral artery: six cases report]. 1602 47

Spontaneous intracranial artery dissection (SIAD), leading to occlusion or stenosis of arterial lumen is a frequent but less studied cause of ischemic stroke especially in young adults. We studied 17 patients (10 men, 7 women, mean age 27.5 +/- 8.5 years) with SIAD. All the patients have undergone magnetic resonance imaging of the head, magnetic resonance angiography (MRA) (in 16 patients--follow-up MRA), 1 patient--conventional cerebral angiography. SIAD was located in the middle cerebral artery (MCA, 14 patients); posterior cerebral artery (PCA, 2 patients) and basilar artery (BA, 1 patient). Fifteen patients (88%) with SIAD developed ischemic stroke, 2 patients (12%)--a transient ischemic attack (TIA). The appearance of the local brain ischemia symptoms was combined with headache in 93% cases. The course of ischemic stroke was favorable in most cases with complete or good functional recovery in 66% of patients. A fatal outcome was observed in 1 patient (7%) with massive brain infarct. The initial cerebral angiography carried out in most cases (76%) within 3 weeks after the disease onset revealed occlusion (71%) or stenosis (29%) of corresponding cerebral arteries (MCA, PCA, BA). The last cerebral angiography conducted in 90% cases 4,5 months or later showed positive dynamics--appearance or improvement of the blood flow in these arteries--in 82% patients. The factors provoking SIAD were alcohol, contraceptive drugs and less frequent recent infection. None of patients had atherosclerosis, vasculitis or arterial hypertension. Clinical manifestations of connective tissue weakness were in 71% of patients, hypotension--65%, mitral valve prolapse--46%. In conclusion, SIAD is one of the causes of ischemic stroke and TIA in young adults and characteristic clinical manifestations and follow-up MRA have a great diagnostic importance. The development of SIAD appears to be related to weakness of connective tissue of arterial wall.
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PMID:[Spontaneous intramural intracranial artery dissection and ischemic stroke]. 1819 22


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