Bailliere's clinical neurology最新文献

{"title":"New options for treatment of Parkinson's disease.","authors":"P A LeWitt","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>New medications recently developed for treating Parkinson's disease include two inhibitors of catechol-O-methyltransferase (COMT), entacapone and tolcapone, which, by decreasing the elimination of levodopa, extend the duration of its effects. Increased 'on' time and less 'wearing-off' symptomatology can be expected with the use of these COMT inhibitors. Two non-ergot dopaminergic agonists (pramipexole and ropinirole) and a long-acting ergoline (cabergoline) are also being introduced. These dopaminergic agonists, like the ergot derivatives currently available (bromocriptine, lisuride, and pergolide), are useful as adjuncts to levodopa, and are also efficacious as monotherapies.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"6 1","pages":"109-23"},"PeriodicalIF":0.0,"publicationDate":"1997-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20354905","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":"Genetic aspects of parkinsonism.","authors":"N Wood","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The genetic basis of many monogenic neurological diseases with parkinsonian features has been elucidated over the past few years. The clinical and genetic features are discussed for the diseases with prominent parkinsonian signs. There is also accumulating evidence for a role of genetic factors in the aetiology of idiopathic Parkinson's disease (PD); however, the approach to polygenic diseases is quite different from that to the simpler single-gene disorders. The role of epidemiology in not only establishing genetic susceptibility, but its impact on estimating the size of the problem, is also discussed. A number of candidate genes have been studied in PD but, to date, there is no conclusive proof for any of these. It therefore seems likely that a random genome search is required, and the technical and statistical methods are now available. It is hoped that knowledge of the genes involved will lead to better therapy for this incurable and common disorder.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"6 1","pages":"37-53"},"PeriodicalIF":0.0,"publicationDate":"1997-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20354901","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":"Parkinsonism. Multiple system atrophy.","authors":"G K Wenning,&nbsp;N P Quinn","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Over the last decade multiple system atrophy (MSA) has been confirmed as a distinct clinicopathological entity. For a long time, overlapping pathology in individual cases with striatonigral degeneration (SND), sporadic olivopontocerebellar atrophy (OPCA) or Shy-Drager syndrome (SDS) had often been a source of confusion. The recently discovered glial and neuronal inclusions indeed confirm that these three disorders represent manifestations of the same disease. Parkinsonism is the most frequent motor disorder of MSA. Early diagnosis of these patients is difficult but important, particularly in clinical trials of potential therapies. Patients with only parkinsonism (+/- autonomic failure) account for much of this diagnostic difficulty, particularly early on. Some features that have been associated with SND such as symmetry or absence of tremor are not helpful, and insistence on a poor levodopa response will miss a sizeable minority of patients. A number of further clinical 'red flags' may be helpful. MRI, MRS, PET and SPECT scanning, autonomic function tests and, especially, external sphincter EMG, may also help differentiate between idiopathic Parkinson's disease (IPD) and MSA. Available medical treatments are usually disappointing, so that good therapy services are all the more important. Better animal models of MSA and evaluation of novel treatment strategies are urgently required, and grafting techniques currently applied to IPD and Huntington's disease (HD) patients might be usefully combined in MSA. Epidemiological and case-control studies are needed to determine the prevalence, incidence and risk factors of MSA. The pathogenesis of MSA remains uncertain. Until the discovery of glial cytoplasmic inclusions (GCIs) previous studies had failed to identify abnormalities relevant to pathogenesis rather than reflecting secondary change. The abundance of GCIs points to a fundamental cytoskeletal alteration in glial cells that may eventually result in neuronal degeneration. Mechanisms of formation and distribution of GCIs as well as disordered glial-neuronal interactions should be studied in more detail in MSA brains.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"6 1","pages":"187-204"},"PeriodicalIF":0.0,"publicationDate":"1997-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20356756","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":"New antiepileptic drugs.","authors":"E A Wilson,&nbsp;M J Brodie","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>No fewer than eight new antiepileptic drugs (AEDs) with diverse mechanisms of action have been introduced into clinical practice in the 1990s. Short monographs on lamotrigine, vigabatrin, gabapentin, oxcarbazepine, felbamate, topiramate and vigabatrin have been prepared for this review. Details are provided of mechanisms of action, clinical pharmacokinetics and adverse drug interactions. Each section concentrates on the efficacy, tolerability and practical use of these drugs. The areas where they have potential for superiority over the established AEDs have been highlighted. Specific indications and dosage schedules have been provided. As many of these AEDs have, as yet, limited licences, an attempt has been made to identify ongoing studies and important omissions. Where possible, the eventual place of the new agent in the pharmacological management of epilepsy has been assessed. A more limited summary has been included of zonisamide which, although licensed in Japan, is still regarded as an investigational drug elsewhere. Short discussions of three of the most promising investigational compounds, namely remacemide, losigamone and levetiracetam, complete the picture.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 4","pages":"723-47"},"PeriodicalIF":0.0,"publicationDate":"1996-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20023737","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":"Malignant epileptic encephalopathies in children.","authors":"O J Dulac,&nbsp;C Chiron","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Malignant epileptic encephalopathies in children comprise a series of age-related generalized epilepsy syndromes: neonatal myoclonic encephalopathy and early epileptic encephalopathy in the neonatal period, migrating focal seizures in early infancy, infantile spasms and West syndrome, severe myoclonic epilepsy and non-progressive myoclonic encephalopathies in infancy, and Lennox-Gastaut syndrome, myoclonic astatic epilepsy, continuous spike waves in slow sleep and Rasmussen disease in childhood. Clinical and EEG characteristics are the major diagnostic clues. Aetiology remains unknown but for the majority of the cases, a combination of different factors, particularly lesions, genetic predisposition and age-related features seem to be variously combined to produce an intractable condition.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 4","pages":"765-81"},"PeriodicalIF":0.0,"publicationDate":"1996-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20023740","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":"Status epilepticus.","authors":"D M Treiman","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Status epilepticus is a condition in which multiple epileptic seizures occur without complete recovery from the physiological effects of one seizure before another seizure occurs. There are as many types of SE as there are kinds of epileptic seizures. Generalized convulsive status epilepticus initially presents with repeated generalized convulsions without full recovery of consciousness between seizures. If untreated or undertreated, the convulsive activity becomes progressively subtle and is accompanied by a predictable series of progressive EEG changes. Non-convulsive SE refers to complex partial SE or absence SE, both of which exhibit an epileptic twilight state of altered contact with the environment. In simple partial SE there is no impairment of consciousness, and the behavioural changes reflect focal ictal discharges confined to one area of the cortex. There are between 65,000 and 150,000 cases of the SE in the US each year. Both acute and remote cerebral insults can cause SE, as can severe systemic disease that causes SE secondary to a toxic-metabolic encephalopathy. Mortality is high, but is largely a reflection of underlying aetiology when SE is treated appropriately and aggressively. Treatment is focused on terminating ongoing seizure activity as quickly as possible, both because the longer SE persists the more likely permanent neuronal damage will ensure and also because of strong evidence that the longer SE persists the more refractory to treatment it will be. Currently the most commonly accepted treatment protocol involves rapid initiation of therapy with intravenous lorazepam (0.1 mg/kg), followed, if necessary, by 20 mg/kg of phenytoin, followed, if necessary, by 20 mg/kg of phenobarbital. However, some neurologists still use intravenous diazepam (because of its more rapid antistatus effect) followed by phenytoin. New experimental data in the rat suggest that phenytoin followed by diazepam may be more effective, but this order of administration still has to tested in properly designed clinical trials.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 4","pages":"821-39"},"PeriodicalIF":0.0,"publicationDate":"1996-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20023743","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":"Epilepsy, anticonvulsant drugs and cognition.","authors":"D L Drane,&nbsp;K J Meador","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The use of AEDs in the management of epilepsy requires an ongoing risk-benefit analysis that attempts to maximize seizure control while minimizing adverse cognitive side-effects. Although the effects of other factors on cognition are generally greater than AED effects in patients with epilepsy, the cognitive effects of AEDs are of special concern because they are iatrogenically induced. Baseline evaluation of mental functioning is essential and should be repeated whenever a change in cognitive performance is suspected. The cognitive effects of the major AEDs, including phenytoin, carbamazepine and valproate, appear modest when dosages are kept within standard therapeutic ranges and polypharmacy is avoided. Violation of these guidelines increases the risk of alterations in arousal, attention, memory and psychomotor functioning. In turn, dysfunction in these areas can contribute to deficits in higher cognitive processes. Evidence suggests that these primary and secondary deficits are relatively greater for benzodiazepines, bromide and phenobarbital. Initial studies involving the newer AEDs suggest that the cognitive profile of these drugs is favourable, but further research is required to determine their relative effects to each other and to the older AEDs. For some patients, optimal seizure management may require the use of polypharmacy or AED dosages that exceed the standard therapeutic range. In such cases, the physician should remain sensitive to the increased risk of cognitive side-effects. The impact of such effects will be greatest for those whose daily functioning requires sustained attention or psychomotor speed. Although the cognitive risks of AEDs appear rather modest for most adults, questions remain regarding the impact of AEDs on patients at extremes of age. Initial studies with children and older adults suggest that the effects of the major AEDs are comparable across the developmental lifespan. However, during the formative years of a child's intellectual development, close scrutiny should be paid to the possibility that subtle attentional or arousal deficits could contribute to cumulative deficits in learning or memory. Preliminary studies involving both animals and humans suggest that the impact of AEDs might be greatest during in utero exposure; however, additional research is required to fully delineate the long-term effects of AED exposure in this earliest period of neurodevelopment.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 4","pages":"877-85"},"PeriodicalIF":0.0,"publicationDate":"1996-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20026401","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":"Contraception, pregnancy and lactation in women with epilepsy.","authors":"M S Yerby","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Despite all of our advances women with epilepsy face obstacles when it comes to pregnancy and childbearing. Many of these obstacles are social, based on incorrect and inappropriate attitudes of the public towards persons with epilepsy. Unfortunately many of the uninformed public are health care providers. We must continue to educate not only our patients but our colleagues so that women with epilepsy will cease to face discriminatory behaviour. Most women with epilepsy can conceive and bear healthy children. They have higher probabilities of infertility but this is often amenable to treatment. Complications of pregnancy are higher and revolve primarily around the increased risk of maternal seizures. Careful monitoring of the clinical condition of the patient and her free anticonvulsant levels will obviate much of this difficulty. Maternal seizures themselves can pose hazards for women with epilepsy and their offspring and generalized convulsive seizures are clearly to be avoided. Adverse pregnancy outcomes tend to be seen more often in particular: congenital malformations 4-6%; dysmorphic features < 10%; neonatal haemorrhage < 7%; fetal death and neonatal and infant mortality a two to threefold increase over the general population; and an uncertain risk of developmental delay particularly in the area of language acquisition. Of the potential variables of interest: anticonvulsants, maternal seizures during gestation, and the genetics of maternal epilepsy, it is at present unclear which is the most important in determining a good pregnancy outcome. Current research suggests that anticonvulsant drugs are probably responsible for the increased risk of malformations. Malformations are, however, only one of the adverse outcomes of concern. Risks can be reduced by ensuring good seizure control; monotherapy: preconceptual use of multivitamins with folate. The plethora of new anticonvulsants offers us new opportunities for improving the function and control of persons with epilepsy. Unfortunately we are uncertain how hazardous the newer anticonvulsant drugs are in pregnancy. Felbamate, gabapentin, lamotrigine, vigabatrine, and topiramate have all been recently introduced. The number of exposed women is so small that no pattern or estimates of risk can be determined at this time. Careful monitoring as is being performed by the Lamotrigine and North American Epilepsy and Pregnancy Registries will hopefully provide the necessary safety information in the near future. All of the risks aside, the majority of women with epilepsy can and will have healthy children.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 4","pages":"887-908"},"PeriodicalIF":0.0,"publicationDate":"1996-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20026402","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":"Established antiepileptic drugs.","authors":"E Perucca","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The major established drugs used in the management of epilepsy are carbamazepine, valproic acid, phenytoin, phenobarbital, primidone, ethosuximide and benzodiazepine drugs. Carbamazepine and phenytoin are used mainly in the treatment of partial seizures and primarily or secondarily generalized tonic-clonic seizures. Valproic acid is effective against all types of seizures, but it is used most extensively in the management of generalized epilepsies. Ethosuximide is effective against absence seizures. Phenobarbital and primidone are effective against all types of seizures (except for absences) although they are less commonly used because of their sedative properties and adverse effects on cognition. Benzodiazepines are most valuable in the treatment of status epilepticus, but their long-term use is often associated with undesirable sedation and development of tolerance to their antiepileptic effect. Irrespective of the drug used, optimal clinical management requires individualization of dosage and dosing schedules based on careful evaluation of clinical response and sound knowledge of the pharmacokinetics and interaction potential of the individual compounds. Monitoring serum drug concentrations may provide a useful guide to dosage adjustments, particularly in the case of phenytoin, which shows dose-dependent kinetics within the therapeutic dosage range.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 4","pages":"693-722"},"PeriodicalIF":0.0,"publicationDate":"1996-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20023736","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":"Epilepsy in children.","authors":"S T Arnold,&nbsp;W E Dodson","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Childhood epilepsies comprise a broad range of disorders which vary from benign to progressive and disabling. Accurate diagnosis of epilepsy type and determination of aetiology, when possible, are essential for appropriate treatment. The most common seizure type encountered in children is febrile seizures. These represent a benign condition which is not, in fact, epilepsy and usually does not require antiepileptic medication. When partial seizures occur in childhood, benign syndromes with spontaneous remission, such as rolandic epilepsy, must be distinguished from symptomatic epilepsies which may be refractory to medical management. Complex partial seizures in young children may appear different than in adults. The adverse effect profiles and dosing regimens of antiepileptic drugs in children are also different than in adults, and influence the choice of treatment. Epilepsy surgery should be considered for some children with intractible partial seizures. Generalized epilepsies also have a broader spectrum in children. The idiopathic generalized absence epilepsies are usually easy to control with medication. They range from childhood absence epilepsy which tends to remit in adolescence to juvenile myoclonic epilepsy which is a lifelong condition. In contrast, the seizures of West syndrome and Lennox-Gastaut syndrome are difficult to control, and treatment involves therapeutic modalities rarely used in adults such as ACTH and the ketogenic diet. Many childhood epilepsy syndromes have a familial predisposition, and the genetic bases for several disorders have been described.</p>","PeriodicalId":77030,"journal":{"name":"Bailliere's clinical neurology","volume":"5 4","pages":"783-802"},"PeriodicalIF":0.0,"publicationDate":"1996-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20023741","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}