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)

Forty-four patients with hemiplegia following stroke and 10 nondisabled subjects were studied to examine the contributions inadequate motor unit recruitment and co-contraction attributable to impaired antagonist inhibition play in the movement disorder of the hemiplegic arm. Electromyographic data were recorded from agonist and antagonist muscles while subjects attempted six specified tasks. Data from subjects who could complete the tasks were compared with those who could not complete the tasks. Differences between the two groups were found in the electromyographic data obtained from the agonist muscles. Electromyographic values were consistently and significantly lower in patients who were unable to complete the tasks than in patients who were able to complete the tasks. In the antagonist muscles, a significant difference was noted only once; in this case, the EMG values were again lower in the group of patients who were unable to complete the task. Inadequate recruitment of agonists, not increased activity in the antagonists, was a consistent finding in patients who were unable to carry out the movement tasks. This study theoretically supports aiming treatment efforts at improving motoneuron recruitment rather than reducing activity in antagonists while retraining arm function.
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PMID:Agonist and antagonist activity during voluntary upper-limb movement in patients with stroke. 849 14

10 cases are presented in which a posterior cerebral artery (PCA) deficit developed suddenly in dramatic fashion with headache, visual symptoms, sensory and motor deficits, and signs of 3rd nerve involvement. There were 9 females and 1 male, ranging in age from 18-51 years with 7 cases under age 35. In 9 of the 10 patients, headache was prominent at the onset; 6 patients reported being dramatically stricken with a severe, sharp localized pain in the forehead or occiput. Visual symptoms were prominent at the onset in 7 patients -- 4 patients experiencing blindness and 3 patients a hemianoptic deficit. Hemisensory symptoms or deficit occurred in 6 instances, a hemiparesis in 3, combined weakness and sensory deficit in 1. Evidence of a 3rd nerve palsy was found in 3 cases. A persisting neurologic deficit occurred in 10 cases -- visual field defect, 6 cases; hemiplegia, 1; slight weakness, 1; and a sensory deficit, 2. A movement disorder developed on the involved side in 7 cases. Evidence of infarction in 1 or both occipital lobes was obtained in 6 patients. 1 patient did not have impaired visual fields, and the other 3 were examined before the days of nuclear medicine and CT scanning. Conventional angiography was performed in 8 patients with the following results: retrothalamic occlusion of 1 PCA (1 patient); distal occlusion of 1 PCA (1 patient); retrothalamic narrowing of 1 PCA (1 patient); irregularity of the wall of the upper basilar artery and both PCAs (1 patient); and in 4 angiography was normal. A digital subtraction angiogram in 1 patient was normal; 1 patient did not have an arteriogram. A history of accompanied migraine was obtained in 3 patients. 1 patient was pregnant; 1 patient was 3 months postpartum. 1 patient was taking oral contraceptives; 1 patient had taken 1 contraceptive pill, and 1 patient was receiving injections of estrogen. These cases represent involvement of the territory of the PCA. They share the same features in varied combinations. The onset or evolution is dramatic, distinctive, or alarming. The cases do not fall easily into any commonly recognized category of cerebrovascular disturbances. The process that most likely applies to this group of cases is migraine. If that is so, the term "catastropic migraine" or "cataclysmic migraine" may have some currency. If it is assumed that the process is ischemic and since vascular obstruction was found in 2 cases, the possibility of using heparin therapy might be considered. In most of the present cases, steroid therapy was used to control brain swelling. If the pathologic process is temporary vasospasm, the use of hemodilution or hyperbaric oxygen could be an option.
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PMID:Unusual vascular events in the territory of the posterior cerebral artery. 395 50

Reports of 62 cases with a movement disorder associated with a focal lesion in the thalamus and/or subthalamic region were analyzed. Thirty-three cases had a lesion confined to the thalamus. Sixteen cases had a thalamic lesion extending into the subthalamic region and/or midbrain. Thirteen cases had a lesion in the subthalamic region or a subthalamic lesion extending into the midbrain. Nineteen cases with dystonia, 18 with asterixis, 17 with ballism-chorea, three with paroxysmal dystonia, and five with clonic or myorhythmic movements have been described. No case with isolated tremor has been described. In 53 cases with unilateral thalamic or subthalamic lesions, all but one with bilateral blepharospasm (associated with right posterior thalamic, pontomesencephalic, and bilateral cerebellar lesions) had dyskinesias in the limbs contralateral to the lesion. The other nine cases had bilateral paramedian thalamic lesions; seven developed bilateral dyskinesias, and the remaining two had unilateral dyskinesias. Regarding the 19 patients with dystonia, the two with bilateral blepharospasm had thalamic and upper brainstem lesions, and one with hemidystonia and torticollis had a subthalamic lesion. The other 16 patients all had a unilateral thalamic lesion with contralateral dystonia (10 hemidystonia, five focal dystonia affecting a hand and/or and one segmental dystonia involving face, arm, and hand). The exact location of the thalamic lesion was mentioned in 10 cases; the posterior or posterolateral thalamus was involved in six and the paramedian thalamus in four. These areas are more posterior or medial to the ventrolateral and ventroanterior thalamic nuclei, which receive pallido-thalamic and nigro-thalamic afferents. Two cases developed dystonia immediately after thalamotomy, and one case developed it 4 days after head trauma. The others initially had a hemiplegia and developed dystonia 1-9 months after the acute insult. Fifteen of the 17 patients with chorea had a unilateral lesion in the subthalamic nucleus or subthalamic region (eight due to infarcts, one to hemorrhage, five to mass lesions, and one to multiple sclerosis). All had contralateral hemichorea or hemiballism. One other case had bilateral chorea of the hands and tongue due to paramedian thalamic infarction. Another case with generalized chorea and thalamic atrophy was complicated by stereotaxic surgery. Thirteen of the 18 cases with asterixis had lesions confined to the thalamus. Eight were associated with thalamotomy, and five others had a stroke (four infarction and one hemorrhage) affecting the contralateral thalamus.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Movement disorders following lesions of the thalamus or subthalamic region. 799 Aug 45

We report 22 cases of alternating hemiplegia of childhood. In addition to repeated episodes of hemiplegia lasting from a few minutes to several days, the disease was characterized by an onset before 18 months of age, the occurrence of tonic or dystonic attacks, nystagmus, dyspnea and other autonomic phenomena, and the development of cognitive impairment and of a choreoathetotic movement disorder. All the patients also had episodes of quadriplegia that occurred either when a hemiplegia was shifting from one side to the other or as an isolated manifestation. Such episodes were often severe and followed by developmental deterioration. In all children, sleep consistently relieved both weakness and associated paroxysmal phenomena, but these would reappear 10 to 20 minutes after the children awakened, during long-lasting episodes. Although six patients also had epileptic seizures, the condition seems to be distinct from epilepsy, and the clinical features and poor outcome differentiate it from migraine. Treatment with the calcium-entry blocker flunarizine was partially effective.
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PMID:Alternating hemiplegia of childhood. 849 42

Alternating hemiplegia of childhood is a rare disorder characterized by episodic paroxysmal attacks of neurologic dysfunction and has historically been thought to represent a migraine equivalent, an unusual form of epilepsy, or a movement disorder. Medical treatment with a variety of anticonvulsants and migraine agents is largely unsuccessful, and progressive mental deterioration is universally expected. Despite extensive laboratory studies, the pathophysiologic basis of this condition has thus far eluded identification. Recently, an international workshop was held to generate testable hypotheses regarding the pathophysiology of alternating hemiplegia of childhood. This paper summarizes the major proceedings of that workshop, and hopes to stimulate further interest in elucidating the molecular and cellular mechanisms underlying this unusual disorder.
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PMID:Alternating hemiplegia of childhood: insights into its pathophysiology. 947 47

We describe a young girl with a novel 1659T>C mutation in the tRNA(Val) gene of mitochondrial DNA (mtDNA) who presented with learning difficulties, hemiplegia, and a movement disorder, together with a raised cerebrospinal fluid (CSF) lactate. The mutation, which was present at high levels of heteroplasmy in patient tissues, interrupts a conserved Watson-Crick basepair in the TPsiC stem and has not previously been described in controls. This report further confirms the frequent association of mitochondrial tRNA mutation with neurological presentations, even in paediatric cases.
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PMID:Childhood neurological presentation of a novel mitochondrial tRNA(Val) gene mutation. 1546 92

Benign nocturnal alternating hemiplegia (BNAH) of childhood is distinct from the classic form of malignant alternating hemiplegia of childhood [1]. It is characterized by hemiplegic attacks occurring exclusively during sleep [2]. It can be misdiagnosed as migraine, nocturnal frontal lobe epilepsy, benign rolandic epilepsy, Panayiotopoulos syndrome, or sleep-related movement disorder [1-4]. Only nine patients have been described to date, with typically, a normal development [1,5-7]. In order to insist about the benignity of the affection, we report two cases: a new three-year-old boy suffering from BNAH and a patient already published to show positive evolution at fourteen years of age. BNAH is a rare disorder but may be underdiagnosed. Making an early diagnosis can help to describe to the parents the good prognosis without treatment.
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PMID:Benign nocturnal alternating hemiplegia of childhood: two cases with positive evolution. 2081 33

Alien Hand Syndrome is defined as unwilled, uncontrollable, but seemingly purposeful movements of an upper limb. Two major criteria for the diagnosis are complaint of a foreign limb and complex, autonomous, involuntary motor activity that is not part of an identifiable movement disorder. After a cerebrovascular accident in the corpus callosum, the parietal, or frontal regions, various abnormal involuntary motor behaviors may follow. Although different subtypes of Alien Hand Syndrome have been distinguished, this classification clearly does not cover the wide clinical variety of abnormal motor behaviors of the upper extremity. And there are few known studies about the neurophysiology of this syndrome using transcranial magnetic stimulation (TMS). We recently experienced 2 rare cases of Alien Hand Syndrome which occurred after anterior cerebral artery (ACA) infarction. A 72 year-old male with right hemiplegia following a left ACA infarct had difficulty with voluntarily releasing an object from his grasp. A 47 year-old female with left hemiplegia following a right ACA infarct had a problem termed 'intermanual conflict' in which the two hands appear to be directed at opposing purposes. Both of them had neurophysiologic studies done, and showed reduced amplitude by single pulse MEP and a lack of intracortical inhibition (ICI) by paired pulse TMS. No abnormalities were found in SSEP.
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PMID:Alien hand syndrome in stroke - case report & neurophysiologic study -. 2297 83

Alternating hemiplegia of childhood (AHC) is a very rare disease characterized by recurrent attacks of loss of muscular tone resulting in hypomobility of one side of the body. The etiology of the disease due to ATP1A3 gene mutations in the majority of patients. Few familial cases have been described. AHC has an onset in the first few months of life. Hemiplegic episodes are often accompanied by other paroxysmal manifestations, such as lateral eyes and head deviation toward the hemiplegic side and a very peculiar monocular nystagmus. As the attack progresses, hemiplegia can shift to the other side of the body. Sometimes the attack can provoke bilateral paralysis, and these patients may have severe clinical impairment, with difficulty in swallowing and breathing. Hemiplegic attacks may be triggered by different stimuli, like bath in warm water, motor activity, or emotion. The frequency of attacks is high, usually several in a month or in a week. The duration is variable from a few minutes to several hours or even days. Sleep can stop the attack. Movement disorders such as dystonia and abnormal movements are frequent. Cognitive delay of variable degree is a common feature. Epilepsy has been reported in 50% of the cases, but seizure onset is usually during the third or fourth year of life. Many drugs have been used in AHC with very few results. Flunarizine has the most supportive anecdotal evidence regarding efficacy.
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PMID:Alternating hemiplegia of childhood. 2362 89

Glucose transporter type 1 deficiency syndrome (GLUT1DS) is the result of impaired glucose transport into the brain. The "classic" GLUT1DS patient presents with infantile seizures (resistant to traditional seizure medications), developmental delay, acquired microcephaly, hypotonia, spasticity, and a complex movement disorder consisting of ataxia and dystonia. However, over the years, other clinical manifestations have been described, such as paroxysmal exertion-induced dystonia with or without seizures, choreoathetosis, alternating hemiplegia, and other paroxysmal events, such as intermittent ataxia, dystonia, and migraine. At the current state of the art in understanding of GLUT1DS, classifying the disease phenotype as "classical" or "non-classical" seems to be of limited clinical utility. It seems more appropriate to think in terms of a broad clinical spectrum in which we can observe intellectual impairment, acquired microcephaly, epilepsy, and movement disorders characterized by different clinical manifestations and degrees of severity. Lumbar puncture, a simple investigation, should be considered the first diagnostic step that, moreover, is feasible worldwide. Thereafter, mutational analysis of the solute carrier family 2 (facilitated glucose transporter) member 1 (SLC2A1) gene should be performed in patients with highly suggestive clinical findings and low cerebrospinal fluid glucose (<50mg/dl or ratio <0.60). Early diagnosis is critical because it allows prompt initiation of treatment with a ketogenic diet (KD). Childhood is the critical period for treatment of GLUT1DS: early diagnosis is crucial for an effective etiological therapy. KD treatment can be useful in adulthood too. Compliance has been found to be much better in GLUT1DS than in the other conditions for which KD treatment is indicated.
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PMID:GLUT1 deficiency syndrome 2013: current state of the art. 2389 Aug 38


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