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)

Fibromuscular dysplasia (FMD) is well known owing to the characteristic angiographical finding of a "string of beads" appearance, but intracranial involvement with this disease is extremely rare. Moreover, to our knowledge, only seven cases that had repeated angiograms disclosed progression of FMD lesion in the literature. Such cases of intracranial FMD which showed progression in the follow-up angiography are reported. Case 1: A 8-year-old boy was referred to our hospital because of aphasia and right hemiplegia following right hemiconvulsion. Left carotid angiography on the 7th day from the onset revealed a "string of beads" appearance involving the left middle cerebral artery from M1 to M2 portion. He was treated with low molecular dextran, urokinase and steroid. After these drugs were administered, his speech was normalized. A repeat left angiogram performed two months later disclosed definite increase in the degree of stenosis associated with FMD. Perivascular sympathectomy around common and internal carotid artery and superior cervical ganglionectomy on the left side carried out on the 70th day from the onset. Postoperative left carotid angiogram showed improvement of the stenosis markedly, and the motor disturbance was improved gradually. Case 2: A 34-year-old woman presented with head dullness and disorientation suddenly. Left carotid angiogram on the third day from the onset showed a "string of beads" appearance from C1 to M1 portion. Follow-up angiography three days later revealed some progression of the stenosis. Furthermore a repeat left angiogram disclosed occlusion of left internal carotid artery at the C2 portion. Left STA-MCA bypass surgery was performed on the 61st day from the onset.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Two cases of intracranial fibromuscular dysplasia whose repeated angiography disclosed progression of the lesion]. 332 87

A case of dissecting aneurysm of the cervical internal carotid artery due to rare mechanism by non-penetrating injury is described. A 45-year-old right-handed man had complaints of the right lateral neck pain during exercise of KENDO wearing a tight headneck protector. Following sudden dysarthria and left hemiplegia, he developed loss of consciousness and generalized convulsion. Five hours after admission, he became alert and had no neurological deficits. Four days after these episodes, he loss visual acuity of the right eye, and a few days later he showed left hemiplegia, hypotension, hypersomnia and right-sides Horner's syndrome. Right retrograde brachial angiography revealed so-called "string sign" in the right extracranial internal carotid artery and delayed circulation in the right cerebral hemisphere. He was diagnosed as having traumatic dissecting aneurysm due to blunt (rubbing) injury. He was treated with STA-MCA anastomosis 3 weeks after the accident. Usually, carotid dissecting aneurysm due to blunt injury is produced by hyperextension and contralateral rotation of the neck or direct blow to the neck, but our case shows a possible mechanism of rubbing injury such as simple anteroposterior flexion under tight neck fixation.
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PMID:[Carotid dissecting aneurysm due to blunt (rubbing) injury of the Kendo protector]. 395 67

In the present study the pathology and treatment of occlusion of cerebral arteries in children were investigated in an attempt to find out an approach to the surgical treatment of cerebral arterial occlusive disease of childhood. We had a total of 55 children with cerebrovascular disorders seen at our Institute during the past 11 years. In this series there were 19 cases of cerebral arterial occlusive disease. The causes in 15 cases of cerebral arterial occlusive disease except for moyamoya disease were heart disease in 7 cases, trauma in 2 cases and unknown in 6 cases. The cerebral arterial occlusive disease had its onset at the age of less than 6 years in 12 of 15 cases. The disease began with hemiplegia of sudden onset. The disease onset was also attended frequently by a convulsive seizure, which distinguishes the condition from that in adults. In 10 of all 15 lesions the site of occlusion was in the distribution of the middle cerebral artery. One case in which there was occlusion of the basilar artery occurring in association with trauma was described in detail. In one instance the treatment consisted of STA-MCA anastomosis. Paroxysmal black out attacks, which had been of frequent occurrence in addition to hemiplegia were relieved postoperatively. In our opinion, STA-MCA anastomosis for cerebral arterial occlusive disease in children should be considered to be indicated only when 1) cerebral angiographic evidence of occlusion or stenosis of a trunk of cerebral artery is still present after the acute stage or 2) no extensive low density area is demonstrable on CT scan. Surgery is generally less indicated in those instances in which the occlusion is due to embolism. However since abscess may arise from such an arterial lesion, surgery should be considered, or at least its feasibility be evaluated, whenever 1) the underlying cardiac pathology well permits surgical intervention and 2) half a year has passed since an initial attack.
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PMID:[Cerebral arterial occlusive disease in children -- clinical aspects and surgical treatment (author's transl)]. 727 22

This is a report of an endarterectomy performed upon the horizontal portion of the middle cerebral artery of a 45 year-old male patient. He had been suffering from TIAs (left hemiparesis) since 8 days prior to admission. Since the frequency of TIA episodes had increased from 2 times to 5 or 6 times a day and the episodes lasted from approximately 10 minutes to over 20 minutes on the day before admission, the TIA was considered to be of the crescendo type. On admission, neurological examination and CT scans showed no abnormalities. An angiography revealed a severe stenosis of the horizontal portion (M1) of the right middle cerebral artery. An endarterectomy was performed using a pterional approach on the day of admission because of the crescendo TIA. Heparin was not used during the surgery. The patient showed left hemiplegia after the endarterectomy. Angiography was performed immediately after the surgery, and severe stenosis caused by mural thrombus was found at the operative site. An emergency STA-MCA anastomosis was carried out to prevent cerebral infarction. However, left hemiplegia did not abate, and a CT scan taken a few days after the surgery revealed a low density area which included the right, basal ganglia and internal capsule. Two weeks after the surgery, angiography was again performed to determine the patency of the anastomosis, which showed normal configuration of the M1, indicating that the stenosis had disappeared. It was considered that if heparin had been used during the endarterectomy, the acute mural thrombus formation at the M1 would have been prevented, and neurological deficit would not have appeared.
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PMID:[Middle cerebral artery endarterectomy: a case report]. 837 99

Emery-Dreifuss muscular dystrophy (EDMD) is a muscular disorder characterized by 1) early contracture of the elbows. Achilles tendons and post-cervical muscles, 2) slowly progressive muscle wasting and weakness with a humeroperoneal distribution, and 3) life-threatening cardiomyopathy with conduction block. Most of families with EDMD show X-linked recessive inheritance with mutations in the STA gene on chromosome Xq28, which encodes a protein named emerin. A rare autosomal dominant form of EDMD (AD-EDMD) is caused by mutations in lamin A/C gene (LMNA) on chromosome 1q21. Both emerin and lamin A/C are located in the inner surface membrane of the nucleus. A 49-year-old woman was skinny and slow runner from childhood and suspected as having a certain muscular disorder. At 35 years, she was found to have the second degree atrioventricular block. At 45 years, she was admitted to a hospital for right-side hemiplegia after cerebral infarction. Cardiac involvement was also observed including high degree atrioventricular block with chronic atrial fibrillation and frequent paroxysmal ventricular contraction on the electrocardiogram. At 49 years, she was referred to our hospital for further evaluation. She had possible dilated cardiomyopathy with conduction block. She also had muscular atrophy and weakness in all extremities, predominantly in the right-side, and contracture of bilateral Achilles tendon, knee and elbow joints, and postcervical muscles. Biopsied skeletal muscle and electromyogram showed myopathic changes. Since a novel point mutation of Ser303Pro was found in exon 5 of LMNA gene, she was diagnosed as having AD-EDMD and had a permanent pacemaker implantation. Her daughter also had some abnormalities on electrocardiogram. This is the first Japanese case of AD-EDMD. Amiodaron was effective for non-sustained ventricular tachycardia. Early diagnosis and following cardiological examinations and treatments are important and necessary to improve the prognosis of the patients with EDMD.
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PMID:[The first Japanese case of autosomal dominant Emery-Dreifuss muscular dystrophy with a novel mutation in the lamin A/C gene]. 1242 64