{"title":"Dysmyelinating and demyelinating conditions in infancy.","authors":"E H Kolodny","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The myelin membrane is essential for rapid conduction of nerve impulses through the central nervous system. Failure of myelination--dysmyelination--may arise through several mechanisms. The synthesis of a particular myelin protein can be defective, as occurs for proteolipid protein in Pelizaeus-Merzbacher disease and for myelin basic protein in the 18q- syndrome. Delay in myelination with a more generalized diminution in white matter is characteristic of many inherited metabolic diseases, including galactosemia, pyridoxine-dependent seizure disorder, glutaric aciduria type 1, and infantile Refsum disease. Demyelination or breakdown in myelin is characteristic of metachromatic leukodystrophy, Krabbe disease, mitochondrial disorders, adrenoleukodystrophy, Canavan disease, Alexander disease, and orthochromatic leukodystrophy. A fourth category is reserved for malformation syndromes. These include Cockayne, Fukuyama, Walker-Warburg, and Angelman syndromes. Demyelination also occurs in HIV-infected individuals with central nervous system findings and in multiple sclerosis. Much of the evidence for leukodystrophy in these disorders comes from neuroimaging. Some of these disorders are treatable.</p>","PeriodicalId":77089,"journal":{"name":"Current opinion in neurology and neurosurgery","volume":"6 3","pages":"379-86"},"PeriodicalIF":0.0,"publicationDate":"1993-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19488591","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Environmental neurotoxicity of chemicals and radiation.","authors":"M A Verity","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Epidemiologic and societal concerns continue to stimulate studies in the field of environmental neurotoxicology. Although the role of heavy metals, aluminum, and iron are unclear in the etiology of human neurodegenerative disorders, these toxins have provided fertile ground for in vivo and in vitro experimental studies to elucidate their role in neurotoxic injury. Experimental models of clinical syndromes are discussed with special relevance to developmental neurotoxicology. Cycloleucine, tellurium, and 1,3-dinitrobenzene provide models of subacute combined degeneration, primary peripheral nerve demyelination, and thiamine deficiency-like lesions, respectively. Increasing attention is being given to irradiation neurotoxicity, especially in the developing or young central nervous system. A fuller understanding of the pathogenesis of low-dose irradiation injury allows for a clearer understanding of its neurobiology and also provides a more rational approach to understanding an interventional therapy associated with brain irradiation for childhood neoplasia.</p>","PeriodicalId":77089,"journal":{"name":"Current opinion in neurology and neurosurgery","volume":"6 3","pages":"437-42"},"PeriodicalIF":0.0,"publicationDate":"1993-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19490576","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Extrapyramidal disorders in childhood.","authors":"M Segawa","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The term extrapyramidal system originally referred to the system concerned with motor function other than the pyramidal tract. This review covers papers that concern the basal ganglia and related structures except disorders relating to the cerebellum. Because the pathophysiologies of these disorders remain unknown, I review those papers that focused or implicated these points.</p>","PeriodicalId":77089,"journal":{"name":"Current opinion in neurology and neurosurgery","volume":"6 3","pages":"387-92"},"PeriodicalIF":0.0,"publicationDate":"1993-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19488592","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Strategies for diagnosis and treatment of childhood epilepsy.","authors":"R Sankar, H T Chugani","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Recent developments in neuroimaging have advanced our capability to diagnose and perform presurgical evaluations in a large proportion of children without recourse to invasive monitoring techniques. Functional imaging is also providing us with novel insights into the mechanism of certain childhood epileptic syndromes. The role of anatomic (computed tomography, magnetic resonance imaging) and functional (single photon emission computed tomography, positron emission tomography) imaging modalities in the diagnosis and therapeutic decision making in major epileptic syndromes of childhood is reviewed.</p>","PeriodicalId":77089,"journal":{"name":"Current opinion in neurology and neurosurgery","volume":"6 3","pages":"398-402"},"PeriodicalIF":0.0,"publicationDate":"1993-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19488594","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pediatric and developmental neurology.","authors":"","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":77089,"journal":{"name":"Current opinion in neurology and neurosurgery","volume":"6 3","pages":"468-88"},"PeriodicalIF":0.0,"publicationDate":"1993-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19490580","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Metabolic disorders and neurotoxicology.","authors":"D M Turnbull","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":77089,"journal":{"name":"Current opinion in neurology and neurosurgery","volume":"6 3","pages":"411-3"},"PeriodicalIF":0.0,"publicationDate":"1993-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19092744","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Transplantation and surgical treatment of parkinsonian syndromes.","authors":"H Widner, S Rehncrona","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Neurosurgical attempts to correct parkinsonism use strategies aimed either at alleviating the underlying dopamine deficiency or at correcting abnormal compensatory effects in neural circuits within the basal ganglia. During the review period, clinical trials of four different neurosurgical approaches were reported. These approaches are intracerebral transplantation of fetal dopamine neurons, intracerebral transplantation of adrenal medullary tissue, tremor-reducing surgical lesions in the ventrolateral thalamus, and ventroposterior pallidotomy aimed at reducing akinesia and rigidity. Experimental studies in rats and monkeys designed to explore mechanisms of graft actions were also reported.</p>","PeriodicalId":77089,"journal":{"name":"Current opinion in neurology and neurosurgery","volume":"6 3","pages":"344-9"},"PeriodicalIF":0.0,"publicationDate":"1993-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19488588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Choreas, hereditary and other ataxias, tics, myoclonus, and other movement disorders.","authors":"E Tolosa, J Berciano","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Developments in the field of Huntington's disease have focused on the potential benefits of predictive testing. Markers have been described for autosomal dominant cerebellar ataxia and for certain subtypes of Friedrich's ataxia. Argentophilic neuronal and glial inclusions appear to be the first specific pathologic hallmark of multiple system atrophy. \"Pure\" hereditary spastic paraplegia is not a multisystem disorder of the central nervous system, but a monomorphic and stereotyped disease. Advances in Tourette's syndrome are limited because the presumed gene eludes identification. A new type of myoclonus, propiospinal myoclonus, has been described. Clinical and electrophysiologic criteria for defining primary orthostatic tremor have been proposed. Understanding of the neurophysiologic substrate of essential tremor and myoclonus is improving. New neurologic disorders presenting clinically with prominent movement disorder continue to be described.</p>","PeriodicalId":77089,"journal":{"name":"Current opinion in neurology and neurosurgery","volume":"6 3","pages":"358-68"},"PeriodicalIF":0.0,"publicationDate":"1993-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19488590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Drug-induced movement disorders.","authors":"O S Gershanik","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>This review covers the major categories of movement disorders secondary to the use of antipsychotic drugs and other pharmacologic agents that interfere with dopaminergic transmission at the basal ganglionic level. In the past year new syndromes were reported, old concepts were either challenged or supported by recent studies, and significant contributions were made in unraveling the complex pathophysiology of these disorders.</p>","PeriodicalId":77089,"journal":{"name":"Current opinion in neurology and neurosurgery","volume":"6 3","pages":"369-76"},"PeriodicalIF":0.0,"publicationDate":"1993-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19092742","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Treatment of Parkinson's disease.","authors":"A Lieberman","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The treatment of Parkinson's disease is reviewed. The rationale for using selegiline (deprenyl) as the first treatment in recently diagnosed patients is presented. Selegiline delays the need for levodopa; however, it is unclear whether this results from a symptomatic or a neuroprotective effect of selegiline. Levodopa combined with a decarboxylase inhibitor is the principal treatment for patients with moderate or marked symptoms. There is little evidence that levodopa has a deleterious effect on the court of Parkinson's disease. The relationship of levodopa to dyskinesias and response fluctuations is discussed. Pharmacokinetic and pharmacodynamic studies suggest that continuous dopaminergic stimulation may be superior to intermittent pulse therapy. The best approximation to continuous stimulation is the use of long-acting levodopa-carbidopa preparations supplemented by dopamine agonists.</p>","PeriodicalId":77089,"journal":{"name":"Current opinion in neurology and neurosurgery","volume":"6 3","pages":"339-43"},"PeriodicalIF":0.0,"publicationDate":"1993-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19488587","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}