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Query: UMLS:C0036572 (seizures)
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Approximately 0.8% of people in the general population have epilepsy. Within this group are specific sub-populations who co-present with other additional conditions, learning disability being one such example. Epilepsy rates are the highest of all in this subgroup, between 21% and 50% and positively correlated with degree of learning disability. In addition, in the more severe categories, problems frequently arise when attempting to differentiate epileptic events from other phenomenon, such as stereotyped behaviours and involuntary movements. The individual is unable to communicate changes in consciousness and perception and observers often find it difficult to detect such changes, particularly with regard to the partial epilepsies. Intensive monitoring using EEG and video equipment can often prove valuable in such a situation in assisting carers to recognize epileptic episodes and respond accordingly.
Seizure 1997 Apr
PMID:Epilepsy or stereotypy? Diagnostic issues in learning disabilities. 915 23

A. Digital EEG is an established substitute for recording, reviewing, and storing a paper EEG record. It is a clear technical advance over previous paper methods. It is highly recommended. (Class III evidence, Type C recommendation). B. EEG brain mapping and other advanced QEEG techniques should be used only by physicians highly skilled in clinical EEG, and only as an adjunct to and in conjunction with traditional EEG interpretation. These tests may be clinically useful only for patients who have been well selected on the basis of their clinical presentation. C. Certain quantitative EEG techniques are considered established as an addition to digital EEG in: C.1. Epilepsy: For screening for possible epileptic spikes or seizures in long-term EEG monitoring or ambulatory recording to facilitate subsequent expert visual EEG interpretation. (Class I and II evidence, Type A recommendation as a practice guideline). C.2. OR and ICU monitoring: For continuous EEG monitoring by frequency-trending to detect early, acute intracranial complications in the OR or ICU, and for screening for possible epileptic seizures in high-risk ICU patients. (Class II evidence, Type B recommendation as a practice option). D. Certain quantitative EEG techniques are considered possibly useful practice options as an addition to digital EEG in: D.1. Epilepsy: For topographic voltage and dipole analysis in presurgical evaluations. (Class II evidence, Type B recommendation). D.2. Cerebrovascular Disease: Based on Class II and III evidence, QEEG in expert hands may possibly be useful in evaluating certain patients with symptoms of cerebrovascular disease whose neuroimaging and routine EEG studies are not conclusive. (Type B recommendation). D.3. Dementia: Routine EEG has long been an established test used in evaluations of dementia and encephalopathy when the diagnosis remains unresolved after initial clinical evaluation. In occasional clinical evaluations, QEEG frequency analysis may be a useful adjunct to interpretation of the routine EEG when used in expert hands. (Class II and III evidence as a possibly useful test, Type B recommendation). E. On the basis of current clinical literature, opinions of most experts, and proposed rationales for their use, QEEG remains investigational for clinical use in postconcussion syndrome, mild or moderate head injury, learning disability, attention disorders, schizophrenia, depression, alcoholism, and drug abuse. (Class II and III evidence, Type D recommendation). F. On the basis of clinical and scientific evidence, opinions of most experts, and the technical and methodologic shortcomings, QEEG is not recommended for use in civil or criminal judicial proceedings. (Strong Class III evidence, Type E recommendation). G. Because of the very substantial risk of erroneous interpretations, it is unacceptable for any EEG brain mapping or other QEEG techniques to be used clinically by those who are not physicians highly skilled in clinical EEG interpretation. (Strong Class III evidence, Type E recommendation).
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PMID:Assessment of digital EEG, quantitative EEG, and EEG brain mapping: report of the American Academy of Neurology and the American Clinical Neurophysiology Society. 922 9

People with learning disabilities often have seizures in addition to other disorders. Precise diagnosis may be difficult, but accuracy can be improved using electroencephalographic and video investigations. Following the establishment of a diagnosis of epilepsy, individually tailored care is necessary taking into account other health, behavioural and therapeutic issues. Neuroimaging may indicate a need for surgery which should not be automatically excluded as a treatment option. Rational antiepileptic drug use is advised, with emphasis upon the newer agents due to their better tolerance and ease of use. A programme of regular review will prevent over-medicating. Drug therapy may be withdrawn in a seizure-free patient. Realistic goals should be established for each individual coupled with an optimistic approach to care. However, future developments require a solid evidence base combined with rationality in all aspects of management. The community learning disability epilepsy nurse specialist is the key health-care professional who can ensure that a learning disabled individual with epilepsy is able to take full advantage of all available services. Education, closer collaboration and the mutual recognition of skills will ensure more cohesive and comprehensive care for this disadvantaged patient population.
Seizure 1998 Feb
PMID:Epilepsy and learning disabilities--a challenge for the next millennium? 954 20

A major component of the population of people who have epilepsy are people with a learning disability. As a group, such individuals often have complex epilepsy which is refractory to treatment. Current available measures to assess the outcomes of therapeutic interventions in epilepsy are based on seizure frequency, seizure severity and quality-of-life measures, but have not been validated in people with a learning disability. Thus, we do not know if such measures of outcome serve the needs of this group. This review examines how able we are to assess the efficacy of our interventions to control epilepsy in people with learning disability. It is suggested that a standard data set is necessary as the basis of the assessment of any therapeutic intervention. Central components of this data set would encompass a definition of important characteristics of an individual, a description of their epilepsy and an assessment of the impact of their condition on both their own and their carer's health. The approach to obtaining this information should employ a methodology which can allow for environmental influences.
Seizure 1997 Oct
PMID:Learning disability and epilepsy. 1, towards common outcome measures. 966 96

A survey of the prescription of the novel anticonvulsants lamotrigine, vigabatrin, gabapentin and topiramate was carried out in an outpatient setting to investigate the outcome of prescription and factors affecting response. One hundred and fifty randomly selected patient notes were analysed retrospectively. Drug continuation and seizure freedom were used as measures of response. Twenty-nine percent of patients had a brain lesion, 14% a psychiatric disorder, 7% neonatal seizures, 21% a family history of epilepsy and 15% a learning disability. On average at the moment of initiation of the novel anticonvulsant the patients had had a diagnosis of epilepsy for 18 years, were 33 years old, had 19 seizures per month and had previously used two drugs which failed to control their epilepsy. The first novel anticonvulsant was in 55% of cases lamotrigine, in 43% vigabatrin and in 1 % gabapentin. The overall percentage of patients who stayed on their first novel anticonvulsant was 55%, and 17% became seizure-free. No factors were found to influence the response in terms of drug continuation. For seizure freedom, the presence of a psychiatric disorder and partial seizures predicted a significantly poorer response. Length of seizure disorder, seizure frequency at initiation, the number of previously used failed drugs and the total number of drugs previously used were all significantly lower in the seizure-free group.
Seizure 1998 Oct
PMID:The outcome of prescribing novel anticonvulsants in an outpatient setting: factors affecting response to medication. 980 13

Tuberous sclerosis (TSC) is an autosomal dominant disorder characterised by tumour-like malformations (hamartomas) of the brain, skin, and other organs, often associated with seizures and learning disability. There is genetic heterogeneity with loci for TSC on chromosomes 9q34 (TSC1) and 16p13.3 (TSC2). The recently cloned TSC1 gene has 23 exons spanning some 40 kb of genomic DNA with an 8.6 kb transcript. We now report the results of mutation screening by SSCP and heteroduplex analysis of genomic DNA for all 21 coding exons of TSC1 in 83 unrelated cases of tuberous sclerosis. TSC1 gene mutations were found in 16 of the 83 cases (19%). These comprised base substitutions, small insertions, or small deletions giving rise to six nonsense mutations, eight frameshifts, and two splice site mutations, all of which would be expected to result in a truncated or absent protein. In the 10 cases predicted to have TSC1 mutations by linkage analysis or loss of heterozygosity studies, the mutation was identified in eight (80%). In the remaining 73 unassigned cases, only eight mutations were found (11%). From these data we estimate that TSC1 mutations accounted for 24% of the cases in this sample (and an estimated 22% of all TSC cases). This contrasts with data from linkage studies suggesting that TSC1 and TSC2 mutations account for approximately equal numbers of families.
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PMID:Mutations in the TSC1 gene account for a minority of patients with tuberous sclerosis. 986 90

People with a learning disability are often disadvantaged due to the nature of their disability. Up to a third are likely to have concomitant epilepsy which adds to the health loss experienced by this group. It is important to manage their epilepsy in such a way as to limit the debilitating effects of both the illness and the medication. Rectal diazepam remains the gold standard rescue medication for prolonged, recurrent seizures or seizures associated with hypoxia. Some of the drawbacks are highlighted in this paper and we go on to explore a novel means of treating these seizures. Midazolam, via the intranasal route, has been used extensively in children, mostly as a sedative but also in the treatment of epilepsy. We present two cases, both are adults with a learning disability, who have benefited significantly from the use of intranasal midazolam. Ongoing research into the safe use of this form of treatment, training of staff and carers and the impact on the individual is being conducted.
Seizure 1998 Dec
PMID:Midazolam via the intranasal route: an effective rescue medication for severe epilepsy in adults with learning disability. 988 98

In spite of the high prevalence of epilepsy and the importance of preserving cognitive function in people with learning disabilities, this population has received relatively little research attention. This study sets out systematically to investigate possible predictive factors of inter-ictal states of arousal and attention. The daytime function of 28 people with epilepsy and severe learning disabilities was assessed by performance on a two-choice reaction time vigilance task, behavioural analysis of time-sampled video recordings taken in naturalistic settings, and carer ratings on visual analogue scales. This methodology yielded eight discrete functional measures, from which two further index measures were derived after principal components analysis. A range of clinical and psychosocial assessments was completed and subjects had 36 hour ambulatory EEG and sleep EEG monitoring. Regression models identified significant predictors of cognitive function from a range of potential explanatory variables i.e. demographic, clinical, pharmacological, background EEG rhythms and sleep parameters. Results indicated that greater severity of learning disability, longer bedtime periods, poor sleep efficiency, frequent seizures and antiepileptic drug polytherapy were significant predictor variables. Explained variance (adjusted R2) was greater than 50% for six of 10 outcome variables (range up to 85%). Furthermore, significant regression equations (P < 0.05) were obtained for all but one variable. Thus, these results appear reasonably robust. Results support an interactional model of daytime arousal and attention in people with epilepsy plus severe learning disabilities. Inter-ictal cognitive function appears to be mediated by a combination of organic, circadian (sleep wake), clinical and pharmacological factors.
Seizure 1999 Apr
PMID:Cognitive functioning in people with epilepsy plus severe learning disabilities: a systematic analysis of predictors of daytime arousal and attention. 1022 97

Digital EEG (DEEG) and quantitative EEG (QEEG) are recently developed tools present in many clinical situations. Besides showing didactic and research utility, they may also have a clinical role. Although a considerable amount of scientific literature has been published related to QEEG, many controversies still subsist regarding its clinical utilization. Clinical applications are: 1. DEEG is already an established substitute for conventional EEG, representing a clear technical advance. 2. Certain QEEG techniques are an established addition to DEEG for: 2a) screening for epileptic spikes or seizures in long-term recordings; 2b) Operation room and intensive care unit EEG monitoring. 3. Certain QEEG techniques are considered possible useful additions to DEEG: 3a) topographic voltage and dipole analysis in epilepsy evaluations; 3b) frequency analysis in cerebrovascular disease and dementia, mostly when other tests have been inconclusive. 4. QEEG remains investigational for clinical use in postconcussion syndrome, learning disability, attention disorders, schizophrenia, depression, alcoholism and drug abuse. EEG brain mapping and other QEEG techniques should be clinically used only by physicians highly skilled in clinical EEG interpretation and as an adjunct to traditional EEG work.
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PMID:[Guidelines for recording/analyzing quantitative EEG and evoked potentials. Part II: Clinical aspects]. 1034 40

The study examined changes in the use of antiepileptic drugs (AEDs) in a large hospital for people with a learning disability over a 2 year period, the use of investigations, and the presence of medication side-effects. The surveys were carried out in 1993 and 1995/6. In 1993, 27% of patients were being treated for epilepsy and in 1995/6, 30.1%. Ninety percent and 82.4% of patients, respectively, were receiving one or two AEDs. In the second survey there were fewer prescriptions for phenobarbitone (5.8% vs. 12.5%) and an increase in the use of lamotrigine (21.6% vs. 5%), gabapentin (5.8% vs. 0) and vigabatrin (3.9% vs. 2.5% in 1993). Side-effects were recorded in 6 (11.8%) patients. Seven (21.2%) patients receiving carbamazepine were found to have hyponatraemia. Of the 54 electroencephalograms (EEGs) requested, 41 (76%) were reported as abnormal. Six CT brain scans had been conducted, of which five were abnormal. People receiving antipsychotic drugs had fewer seizures than average.
Seizure 1999 May
PMID:The management of epilepsy in a hospital for people with a learning disability. 1035 77


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