Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1762617 (weakness)
37,932 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 31-year-old woman with a history of suspected optic neuritis was admitted with weakness of right-sided extremities and confusion. On admission general physical examination revealed no abnormality. Neurological examination revealed central facial palsy, mild hemiparesis and hemisensory deficit on the right side. Cerebrospinal fluid analysis showed monocytosis of 29 cells, protein of 82 mg/dl, glucose of 62 mg/dl and myelin basic protein of 6.8 ng/ml. No oligoclonal bands were seen. X-ray computed tomographic (CT) scans showed multiple homogeneous contrast-enhancing lesions in the white matter of bilateral parietal lobes without distinct edema or mass effect. Follow-up CT scans showed ring-enhanced lesions. Magnetic resonance image (MRI) showed multiple Gadolinium-enhanced lesions. Additionally, 123I-IMP SPECT [Gamma view-SPCT 2000 H-20 (Hitachi Co.)] was performed at 30 minutes and 5 hours after intravenous administration of IMP (3 mCi). It showed high IMP uptake corresponding to the CT and MRI lesions. Cerebral angiography was considered to be normal. Other laboratory findings were within normal limits. A biopsy was performed. Histological examination showed spongiosis, gliosis and perivascular cuffing. The histological diagnosis was acute demyelinating disease. After therapy with methylprednisolone, she improved gradually. Enhanced lesions in CT and MRI may correspond to active demyelination at acute MS. High uptake of SPECT may also appear in acute stage, although it has not been reported. We should perform neuro-imaging studies including SPECT on acute MS.
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PMID:[A case of acute multiple sclerosis mimicking tumor on the neuro-imaging studies]. 130 32

Case 1. A 46-year-old man suddenly developed mild gait disturbance and left hemiparesis. On examination, gross strength was slightly reduced in the left extremities. The finger-to-nose and heel-to-knee tests disclosed moderate dyssynergia and dysmetria on the left side that could not be explained by the muscular weakness. Deep tendon reflexes were more brisk in the left extremities. There was no Babinski sign. Magnetic resonance imaging showed a region of high signal intensity in the right posterior limb of internal capsule with extension into lateral thalamus. The lesion involved the cortico-ponto-cerebellar pathway and partly the dentato-rubro-thalamo-cortical pathway. No lesions were seen in the brainstem. Single photon emission CT with 123I-IMP showed left cerebellar hypoperfusion termed crossed cerebellar diaschisis by Baron et al. Case 2. A 65-year-old female developed weakness of the left extremities and gait disturbance. On examination, there was a horizontal nystagmus on lateral gaze to each side. She showed dysarthria, mild left hemiparesis and slight left hypesthesia. The finger-nose and heel-knees tests revealed moderate dysmetria and dyssynergia on the left side. Deep tendon reflexes were hyperactive in the left extremities with left Babinski sign. CT showed a low density area in the right basis pontis at about middle level. Intravenous digital subtraction angiography revealed a slight stenosis of right vertebral artery, but no other abnormality. The lesion involved the cortico-ponto-cerebellar pathway. Single photon emission CT with 123I-IMP showed left cerebellar hypoperfusion. The right cerebellar blood flow was normal.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Analysis of hemiparesis with homolateral ataxia by single photon emission CT]. 189 73

We examined the 123I-iodoamphetamine SPECT for 3 patients with ALS, who were clinically diagnosed. Patient 1 was a 31-years-old man, who had bilateral muscle weakness of his upper extremities, and spasticity in lower extremities. Patient 2 was a 51-years-old woman, who had marked weakness of her upper extremities and bulbar sign. Patient 3 was a 68-years-old man, who had severe degree of marked weakness of his upper extremities and mild bulbar signs. Cerebral cognitive function were all normal in three patients. Computed tomographic and magnetic resonance imagings showed moderate degree of cortical atrophy in patient 1, but no abnormalities in patients 2 and 3. In 123I-IMP SPECT, however, hypoperfusion were recognized on the bilateral fronto-parietal border zone areas in these three patients with ALS. It was suggested that patients with ALS showed varying degrees of impaired perfusion in the fronto-parietal border zone areas in spite of normal cognitive functions.
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PMID:[123I-iodoamphetamine single photon emission computed tomography in three patients with amyotrophic lateral sclerosis]. 223 55

Adenylosuccinate (succino-AMP) synthetase and succino-AMP lyase isolated from epimastigotes of Trypanosoma cruzi by chromatography on phosphocellulose. The synthetase was capable of catalyzing the condensation of aspartic acid with IMP and several IMP analogs. The reaction with allopurinol ribonucleotide is of potential chemotherapeutic interest. This analog was slowly converted to its corresponding succino-AMP analog with a Km' of 140 micrometers (cf. IMP at 10 micrometers) and a Vmax' of 0.3 per cent the rate with IMP. The comparable reaction with this analog does not occur with succino-AMP synthetase from a representative mammalian source [T. Spector and R. L. Miller, Biochim. biophys. Acta 455, 509 (1976)]. The protozoal succino-AMP lyase had a broad substrate which was characteristic of this enzyme from many sources. It catalyzed the rapid and efficient cleavage of all the succino-AMP analogs that were produced by succino-AMP synthetase. Thus, these two enzymes appear to be responsible for the selective amination of allopurinol ribonucleotide in T. cruzi. The metabolically produced AMP analog may be the agent or a precursor of the agent that accounts for the anti-growth activity of allopurinol in these organisms. Similar selective amination was observed previously with these enzymes from Leishmania donovani [T. Spector, T. E. Jones and G. B. Elion, J. biol. Chem. 254, 8422 (1979)]. Thiopurinol ribonucleotide was not a substrate of succino-AMP synthetase from T. cruzi, but it was an inhibitor with a K1 = 33 micrometers. Therefore, the weakness of thiopurinol's anti-growth activity with T. cruzi is not due to its inability to inhibit this enzyme.
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PMID:Adenylosuccinate synthetase and adenylosuccinate lyase from Trypanosoma cruzi, Specificity studies with potential chemotherapeutic agents. 703 7

A 62-year-old man developed recurrent TIAs presenting as mild unconsciousness, dysarthria and weakness of the right upper extremity lasting for 15 to 20 minutes. He was found to have severe iron deficiency anemia (hemoglobin: 5.5-5.9g/dl; hematocrit: 18.4-19.5%) which insidiously developed through the chronic bleeding from the gastric ulcer. He had slight hypertension (184/86mmHg), but no orthostatic hypotension. DSA and MR angiography showed severe stenosis at the origin of the bilateral internal carotid arteries and of the left vertebral artery. There was also hypoplasia of the right vertebral artery. Blood circulation detected by 123I-IMP-SPECT was markedly decreased in the whole brain and in the right hemisphere of the cerebellum. TIA was, however, completely disappeared following to the recovery of anemia. The present case suggested that the presence of severe anemia accelerated the occurrence of hemodynamic TIA (regional cerebral anemic hypoxia), which is probably the consequence of the reduced oxygen-transporting capacity of the blood.
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PMID:[Hemodynamic TIA associated with severe anemia--a case report]. 799 47

We report a 30-year-old man with agammaglobulinemia and chronic aseptic meningomyelitis. The patient was diagnosed as having X-linked recessive agammaglobulinemia at 4 years of age and gammaglobulin supplementation was started. He had TIA-like episodes several times since 25 years of age. He developed difficulty in micturition and impotence at 29 years of age. Neurological examination revealed bilateral deafness, contracture of knee joints, slight weakness and areflexia in the lower extremities, Babinski sign and dysuria. There was sensory disturbance in the lower extremity on the left. There was not consciousness disturbance or meningeal irritation sign. The cerebrospinal fluid findings included pleocytosis and increase in protein. Enterovirus RNA was detected in the cerebrospinal fluid by the modified polymerase chain reaction (PCR) method. MRI of lower spinal cord showed syrinx formation in the lumbosacral cord and CT of the brain showed bilateral temporal lobe atrophy and temporoparietal subdural fluid collection on the left. 123I-IMP SPECT showed decrease in the cerebral blood flow in the whole brain. EEG showed diffuse slow activity, suggesting the subclinical encephalopathy. Chronic enteroviral meningoencephalitis with agammaglobulinemia (CEMA) is one of the complications of agammaglobulinemia. However, myelitis without apparent encephalopathy is very rare. To our knowledge, there have been no reports of spinal sylinx formation in CEMA.
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PMID:[A case of agammaglobulinemia with chronic enteroviral meningomyelitis]. 875 85

The manifestations of fatigue, as observed by reductions in the ability to produce a given force or power, are readily apparent soon after the initiation of intense activity. Moreover, following the activity, a sustained weakness may persist for days or even weeks. The mechanisms responsible for the impairment in performance are various, given the severe strain imposed on the multiple organ systems, tissues and cells by the activity. At the level of the muscle cell, ATP utilization is dramatically accelerated in an attempt to satisfy the energy requirements of the major processes involved in excitation and contraction namely sarcolemmal Na+/K+ exchange, sarcoplasmic reticulum Ca2+ sequestration and actomyosin cycling. In an attempt to maintain ATP levels, high-energy phosphate transfer, glycolysis and oxidative phosphorylation are recruited. With intense activity, ATP production rates are unable to match ATP utilization rates, and reductions in ATP occur accompanied by accumulation of a range of metabolic by-products such as hydrogen ions, inorganic phosphate, AMP, ADP and IMP. Selective by-products are believed to disturb Na+/K+ balance, Ca2+ cycling and actomyosin interaction, resulting in fatigue. Cessation of the activity and normalization of cellular energy potential results in a rapid recovery of force. This type of fatigue is often referred to as metabolic. Repeated bouts of high-intensity activity can also result in depletion of the intracellular substrate, glycogen. Since glycogen is the fundamental fuel used to sustain both glycolysis and oxidative phosphorylation, fatigue is readily apparent as cellular resources are exhausted. Intense activity can also result in non-metabolic fatigue and weakness as a consequence of disruption in internal structures, mediated by the high force levels. This type of impairment is most conspicuous following eccentric muscle activity; it is characterized by myofibrillar disorientation and damage to the cytoskeletal framework in the absence of any metabolic disturbance. The specific mechanisms by which the high force levels promote muscle damage and the degree to which the damage can be exacerbated by the metabolic effects of the exercise remain uncertain. Given the intense nature of the activity and the need for extensive, high-frequency recruitment of muscle fibres and motor units in a range of synergistic muscles, there is limited opportunity for compensatory strategies to enable performance to be sustained. Increased fatigue resistance would appear to depend on carefully planned programmes designed to adapt the excitation and contraction processes, the cytoskeleton and the metabolic systems, not only to tolerate but also to minimize the changes in the intracellular environment that are caused by the intense activity.
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PMID:Mechanisms of muscle fatigue in intense exercise. 923 50

A 17-year-old girl was admitted to our hospital due to low-grade fever, confusion, numbness in her right hand and automatism. On admission, she was slightly disoriented but there were no meningeal signs. Weakness and sensory disturbance were observed in her right hand. Automatism and clonic seizures frequently appeared. Electroencephalography revealed frequent delta bursts in her left frontal lobe. 123I-IMP-SPECT study showed abnormally increased isotope uptake in the left cerebral hemisphere. She was diagnosed as status epilepticus of left frontal lobe origin and treated with anti-convulsants including carbamazepine, phenytoin, diazepam, phenobarbital, and thiopental, which were not effective. Then we started corticosteroid therapy. Three cycles of intravenous injections of methylprednisolone, followed by oral prednisolone led to marked improvement in her symptoms. It is known that corticosteroid decreases the threshold of seizure, so we do not use it for idiopathic epilepsy. On the other hand, in some secondary epilepsy due to vasculitis in the brain, corticosteroid is very effective for seizures. It is still unclear whether our patient actually had vasculitis or not. However, it is important to recognize that steroid therapy might be effective in a certain portion of epilepsies resistant to anti-convulsants, especially in young patients with non-infectious fever.
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PMID:[A case of severe status epilepticus of frontal lobe origin successfully treated with corticosteroids]. 980 96

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

We report a case of motor neuron disease (MND) with dementia, presenting motor aphasia as an initial symptom. A 67-year-old man was admitted to our hospital because of speech disturbance slowly progressing for 2 years. On physical examination, he showed no neurological abnormalities except for non-fluent aphasia and increased deep tendon reflexes without laterality. MRI demonstrated bilateral fronto-temporal atrophy, dominating the left hemisphere. This finding was confirmed by surface anatomy scanning (SAS), showing an obvious atrophy in the left inferior frontal gyrus, compared with the right one. SPECT with 123I-IMP revealed some irregular defects in the bilateral frontotemporal region. Because he showed dementia, bulbar palsy with tongue atrophy, weakness of upper extremities and facial muscles, snout reflex, and the atrophy and fasciculation in limbs in addition to motor aphasia soon after the discharge from our hospital, he was diagnosed as having MND with dementia. At age 68, he died of a respiratory failure 3 years after the onset of the disease. MND with dementia seldom shows motor aphasia as an initial symptom. We must include, however, the MND with dementia as an differential diagnosis when we see the patients with progressive aphasia.
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PMID:[A case of motor neuron disease with dementia, presenting motor aphasia as an initial symptom]. 1083 38


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