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)

The record of 67 cases under 15 years of age who were hospitalized during status convulsivus from 1975 to 1978, the 348 cases who visited the hospital for the first time with epilepsy (Oct. 1977 to Sept. 1978) and the 32 cases who were hospitalized during status epilepticus from 1969 to 1974 and who are being followed up as outpatients were studied. The frequency of status epilepticus was 8% among epileptic children. There was no difference in the frequency of incidence between male and female. Patients with mental retardation, however, were revealed to have status epilepticus twice to three times more frequently as compared to cases without mental retardation. The major seizure types of status epilepticus in childhood were generalized tonic clonic convulsion and unilateral clonic convulsion. In 25% of the cases, status epilepticus was the first ictal manifestation. The major cause of status convulsivus was epilepsy, followed by encephalitis and encephalopathy, but cases due to brain tumor were rare. The drug of first choice for status convulsivus is diazepam. If there is any difficulty in controlling status convulsivus with diazepam, it may be worthwhile to consider what the problem is, causes of status convulsivus, seizure type, or basic disease of the patient. The effective dose of diazepam was within the range of 0.3--0.5 mg/kg. When the effect is not sufficient, the dose of diazepam should be increased to 1 mg/kg while watching the general condition of the patient. Factors affecting the prognosis of status convulsivus were its cause, duration, onset age and effectiveness of therapy during the acute stage. The frequency of cases who suffered disability after status epilepticus was 56%. (transient disability 43%, permanent disability 13%) The most frequent type of transient disability was hemiplegia. Most epileptic children who had repetitive status convulsivus revealed psychomotor retardation before first status. Factors which cause repetitive status seem to be hemispheric brain damage or diffuse corticocentrencephalic damage.
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PMID:Therapy and prognosis of status convulsivus in childhood. 52 Sep 66

Acute hemiplegia of obscure cause occurred in 28 children: 13 had had prolonged seizures and a high temperature (considered to have been the direct cause of the brain damage); 5 had had brief seizures, a lower temperature and a depressed level of consciousness; and 10 had a nonfebrile onset of hemiplegia and were found to have vascular abnormalities. Most of the first group were retarded and epileptic at long-term follow-up, as were about half of the second group, whereas children in the third group were of normal intelligence and epilepsy was uncommon among them. Hemiplegia persisted at follow-up in most of the children in each group, the proportion being at least in the third group; if cerebral angiography had demonstrated carotid stenosis or occlusion there was usually poor recovery from the hemiplegia. Bilateral changes on plain skull films or pneumoencephalograms were associated with mental retardation. Failure to control prolonged seizures accompanied by a high temperature predisposes to brain damage; therefore, early and vigorous management is essential.
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PMID:Acute hemiplegia of childhood. 114 81

A prospective longitudinal study involving 233 children with cerebral palsy was carried out to select signs useful for early prognostication of ambulation. All patients were followed from the time they were 12 months old and their progress was observed until they reached the age of 3 to 11 years. The population consisted of 61 children having congenital hemiplegia, 37 having diplegia, 85 having spastic quadriparesis and 28 having the spastic-athetoid clinical type of cerebral palsy. In addition, there were 14 children with athetoid, 6 with ataxic and 2 with hypotonic cerebral palsy. Of the total population, 78.7% achieved some degree of functional walking. Findings indicated that the probability of ambulation was related to the clinical type of cerebral palsy. In some the ultimate functional outcome was rather uniform, as in those with congenital hemiplegic and ataxic types where the prognosis was consistently favorable or in those with hypotonic cerebral palsy in whom the outlook was poor. In spastic diplegic, quadriparetic, spastic-athetoid and athetoid types, on the other hand, expectations varied considerably. For this group of patients, sitting by two years was found to be a predictive sign of high reliability since all children who sat by this age eventually walked. For the group of patients not sitting by two years which included more than half of the eventual ambulators, suppression of obligatory primitive reflex activity between 18 and 24 months provided a sensitive indicator to distinguish the children who ultimately walked from those who would not be expected to do so. These data offer a possibility for predicting future ambulatory status by two years of age in those clinical types of cerebral palsy where difficulties of early accurate prognostication are most likely to be encountered. Observations also suggested that the presence of mental retardation adversely affects ambulation.
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PMID:Cerebral palsy: predictive value of selected clinical signs for early prognostication of motor function. 126 90

A case of alternating hemiplegia in a young girl is presented. The partial benefits of treatment with a calcium antagonist Flunarizine in this patient and in those reported in literature are reviewed. The onset of the disease in this girl was at three months of age with several episodes that were diagnosed as seizures; afterwards she presented, besides, repeated attacks of hemiplegia involving both sides of her body in an alternating way each time with daily frequency and hours of days of duration accompanied of bad mood and irritability as well as autonomic disturbances, oculomotor abnormalities, acquired mental retardation and residual focal neurological abnormalities. After one year of treatment with a calcium-entry blocker: Flunarizine, there was a 30% reduction in the attacks frequency as well as in its severity and stop of the progression of mental retardation. So we report the consequence of precocious diagnosis and treatment of this not well known entity whose clinical signs resemble paroxistic vascular anomalies in the brainstem territory.
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PMID:[Alternating hemiplegia. Partial effectiveness of treatment with flunarizine]. 157 Nov 84

Thirty-one children with right (n = 18) and left (n = 13) congenital hemiplegia were compared for incidence of hand- and foot-preference, eye-dominance, and familial sinistrality. In addition, they were tested with dichotic listening for correct reports of consonant-vowel syllables. The two groups of children were closely matched on IQ and sensory functioning. Children with mental retardation, or epileptic seizures were not included. The results showed that 89% of the left hemisphere impaired (LHI) children were left-handed, all of them preferred the left foot, and 72% were left eye-dominant. In the right hemisphere impaired (RHI) group, everyone (100%) preferred the right hand and foot, and 62% were right eye dominant. The dichotic listening results showed a significant right ear advantage (REA) in the RHI-group, and a significant left ear advantage (LEA) in the LHI-group. The results are discussed in the framework of pathological handedness and shifts in hemisphere control of language in children with early brain injury. It is argued that the homogeneous samples, except for the site of lesion, provides an interesting possibility to compare cognitive effects of left and right hemisphere impairment in children.
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PMID:Pathological left-handedness revisited: dichotic listening in children with left vs right congenital hemiplegia. 162 Mar 27

Developmental language and learning disabilities in children can take many different forms and can result from a variety of causes. Research to date has focused primarily on specific disabilities in learning, which are characterized by a significant delay or disorder in one aspect of learning against a background of otherwise normal development. Learning disabilities affecting language and/or reading acquisition (developmental dysphasia and dyslexia) have been studied most thoroughly. Verbal learning disabilities occur more frequently in boys than in girls, and there is a higher than expected incidence of left-handedness among affected children. Although there are many reasons why a child may have delayed or disordered language development, differential diagnosis of specific developmental language or reading disorders calls for ruling out mental retardation, peripheral auditory or visual dysfunction, autism, frank neurological impairments such as hemiplegia or seizure disorder, and severe social deprivation or lack of educational opportunity. The typical profile of a developmentally dysphasic or dyslexic child is one who shows a marked discrepancy between nonverbal (performance) IQ and verbal IQ, with a history of delayed or disordered speech, language and/or reading development. Such a child usually performs quite normally on visual spatial tasks, while demonstrating severe deficits in tasks of auditory temporal processing, motor sequencing, phonological processing and memory, language, reading and spelling. This characteristic neuropsychological profile may suggest left hemisphere dysfunction or a failure to develop normal cerebral lateralization. The etiology of these developmental learning disorders is unknown, but there is evidence of familial aggregation, indicating a potential genetic basis. Although these children respond to remediation, longitudinal studies have shown that the symptoms often persist into adulthood (see Tallal, 1988, for a more detailed discussion).
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PMID:Hormonal influences in developmental learning disabilities. 196 40

The aims of the study were (1) to replicate previous quantitative studies of motor activity in low-risk and high-risk preterm infants and (2) to apply a new method of systematic analysis of the qualitative characteristics of general movements in these two groups of infants. Sequential one-hour videorecordings of the unstimulated infants in the incubator were made during the preterm period and then continued during the postterm period until about 20 weeks. The high-risk group consisted only of infants with signs of haemorrhage and/or leucomalacia in the repeated ultrasonograms of the brain. The neurological follow-up continued up to a minimum of one and a maximum of three years of corrected age. The quantification of the various motor patterns in 12 matched pairs of low-risk and high-risk preterm infants revealed a slight but significant (P = 0.05) excess of isolated arm movements in the low-risk cases during the activity phase. No other movement pattern differed significantly. The qualitative assessment of general movements during the preterm period resulted in all but one of the 14 low-risk cases having a normal quality of general movements. In the lesion-group (N = 29) all the infants had an abnormal quality during the preterm period. Eight cases later became neurologically normal although 1 of them had strabism. In addition, one infant was blind (ROP) and retarded and one other had mental retardation. Nineteen infants later developed cerebral palsy (two monoplegia of a leg, three hemiplegia, 5 diplegia and 9 quadriplegia). Strabism was present in 48.3% of the whole group of 29 cases. A semi-quantitative estimation of various aspects of the abnormal general movements made a typology of abnormal patterns possible. A graphic display of developmental trajectories of individual cases, depicting the course of abnormal aspects along the time axis, helps document the evolution of abnormal signs. Their course is a better predictor of the neurological outcome than the nature and localization of the lesion, detected by imaging techniques. The qualitative assessment of general movements from videorecordings is a reliable, quick, cheap and totally non-intrusive method in neonatology for the early detection of functional impairment of the nervous system.
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PMID:Qualitative changes of general movements in preterm infants with brain lesions. 225 80

The relationship between serum creatine kinase brain-specific isoenzyme (CK-BB) activity immediately after birth and neurodevelopmental outcome at two and four years corrected age was studied prospectively in 45 preterm infants (less than 34 weeks gestation). Nine infants died during the neonatal period and one was lost to follow-up. Of the 35 children available for follow-up, seven had motor disabilities: four severe diplegia, two mild to moderate diplegia and one hemiplegia. No relationship existed between these motor disabilities and serum CK-BB activity after birth. There seemed to be a relationship between increased serum CK-BB after birth and low scores on the Bayley Scales of Mental Development, but this did not reach statistical significance. At the age of four years, four of the five survivors with high serum CK-BB activity after birth (greater than 25U/L) needed special schooling because of mental retardation. Increased serum CK-BB activity after birth may be associated with delayed mental development, but further study is needed, especially of asphyxiated infants.
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PMID:Serum CK-BB activity in the preterm infant and outcome at two and four years of age. 236 44

We reported a case of mitochondrial encephalomyopathy with repeated stroke-like episodes. A 33-year-old single male was admitted to our hospital because of stroke-like episodes with visual field defect, hemiplegia and convulsion repeated seven times for the past seven years. There were no abnormalities on the physical examination. He was hallucinative and perseverative and had mental deficiency. Muscle weakness and atrophy were not prominent, and generalized hyporeflexia were present without pathological reflexes. Myoclonus was not observed. Serum CK and blood gas analysis were normal (pH 7.398). Although blood levels of lactate and pyruvate were almost within normal limit, lactate was elevated by 20WATT-15 minutes exercises. On the contrary, the CSF levels of lactate and pyruvate were elevated markedly. CT of the brain revealed the presence of the low density areas in the right occipital and the left frontal lobes. Cranial 4 vessels studies were unremarkable. EEG showed the diffuse slowness with spike and wave complex. CT of the muscles were normal. A specimen obtained from the left biceps brachii muscle showed ragged-red fibers without obvious myogenic or neurogenic changes, and accumulations of abnormal mitochondria with paracrystalline inclusion bodies were observed by electron microscopy. However, mitochondrial abnormalities were not seen in the vessel walls in the biopsied muscle. Activities of complex I + III, II + III, IV in mitochondria were normal. Clinical features of this case were consistent with MELAS. However, this case showed no muscle weakness, short stature and lactic acidosis which characterize MELAS, and the onset of this case was later than those cases that were reported before.
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PMID:[A case of mitochondrial encephalomyopathy characterized by repeated stroke-like episodes]. 250 53

We report a 9 year-old boy with MELAS. High dosed oral thiamine administration and high fat diet induced remarkable neurological and biochemical improvement. His mother had episodic headaches and hemiplegia, probably MELAS. He complained muscle weakness and repeated episodes of vomiting started from 2 years of age. High levels of serum lactate and pyruvate were recognized, but with no metabolic acidosis. He developed generalized muscle weakness, growth retardation, generalized convulsions and stroke-like episodes at 5 years old. Optic nerve atrophy and mental retardation gradually appeared. A muscle biopsy at 5 years old revealed numerous ragged-red fibers with excess accumulation of lipid droplets and glycogen particles. Scattered fibers had no cytochrome c oxidase (CCO) activity representing focal CCO deficiency. An electron microscopy showed markedly increased number of giant mitochondria filled with markedly proliferated complicated cristae. Pyruvate dehydrogenase complex level in the fibroblasts was within normal ranges. Serum carnitine level was normal. With oral administration of thiamine hydrochloride (1000 mg) and high fat diet (60-70%), muscle weakness improved, and lactate and pyruvate levels in the serum reduced to normal ranges, whereas the mental deterioration, muscle atrophy, pes cavus progressed very slowly. He died from cardiac and renal failures at 9 years old. Autopsied muscles showed a marked decrease in cytochrome c oxidase activity (biochemically 12.8% of the normal level), and almost all muscle fibers had no cytochrome c oxidase activity histochemically. The progression of the MELAS was probably in parallel with the decrease in CCO activity.
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PMID:[A case of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) with progressive cytochrome c oxidase deficiency]. 255 13


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