Age-related fractures in people with intellectual disability and epilepsy

The present paper describes age-related fractures in 68 people with intellectual disability and epilepsy (39 females and 29 males). A higher incidence of fractures in epileptic subjects (269%) was noted when they were compared with non-epileptic patients (15%). In the sample of 263 epileptics (121 females and 142 males), a higher number of females (32%) sustained fractures than their male counterparts (20%). The peak period of all fractures is between 40 and 49 years of age. The highest incidence of fractures in females occurred during the periods from 10 to 19 and 40 to 49 years, while the peak was between 30 and 39 years for males. The causes of fractures and preventative measures are discussed, and further avenues for research are indicated.     

Topiramate: uses in people with an intellectual disability who have epilepsy

The novel anticonvulsant topiramate has been shown to have efficacy across a range of seizure types including both generalized and partial seizures in several well-designed randomized controlled trials. It has also been shown to be effective in atonic seizures associated with Lennox-Gastaut syndrome. Tolerability data show a tendency to neuropsychiatric side-effects, such as confusion and word finding difficulties, when topiramate is used in polytherapy; these side-effects are reduced in monotherapy usage. The efficacy and spectrum of seizures treated by topiramate suggests that it has an important role in managing epilepsy in people with intellectual disability. The predictable side-effects can be monitored in clinical practice and possibly reduced by slow dose increments. The data set of patients with intellectual disability is still too small to rule out idiosyncratic drug reaction.     

Tiagabine: a new therapeutic option for people with intellectual disability and partial epilepsy

Tiagabine exerts its antiepileptic drug (AED) activity by selectively inhibiting the uptake of gamma-aminobutyric acid (GABA) onto the transporter molecules, and thus, increasing extracellular concentrations of GABA in the brain. The absorption and elimination of tiagabine follow linear pharmacokinetics. Tiagabine is metabolized by hepatic cytochrome P450 enzymes and enzyme-inducing AEDs increase tiagabine clearance by 50-65%. Tiagabine has shown no clinically important interactions with other drugs, including oral contraceptives. In the perforant pathway stimulation model of status epilepticus, tiagabine reduced the seizure number and severity, and also prevented the loss of pyramidal cells in the hippocampus as well as alleviated impairment of the spatial memory impairment associated with hippocampal damage. Tiagabine has both antiepileptogenic and anticonvulsant effects in the kindling model of epilepsy. Based on the data from the short- and long-term add-on studies, tiagabine is effective adjunctive therapy for all partial seizure types in adolescents and adults. Conversion to tiagabine monotherapy has been also possible in substantial amount of patients with partial seizures in three trials. Tiagabine is generally well-tolerated. The most common adverse events in controlled studies involve the central nervous system; for example, dizziness, asthenia, nervousness, tremor, depressed mood and emotional lability. Special safety analyses with formal neuropsychological testing suggest that tiagabine does not adversely affect cognition or mood. Tiagabine represents an important new therapeutic option for patients with treatment-refractory partial seizures. The role of tiagabine in the management of partial epilepsy of patients with intellectual disability is especially emphasized since tiagabine has a low side-effect profile in the cognitive area.     

Carbamazepine in the treatment of epilepsy in people with intellectual disability

Carbamazepine is a major antiepileptic drug which is primarily used to treat epileptic patients suffering from partial seizures with or without secondary generalization, but which also has applications in those suffering from primary generalized tonic-clonic seizures. Besides its antiepileptic effect, carbamazepine is also indicated in the treatment of trigeminal and occipital neuralgia, and in manic depressive disorders. Because of its minimal unwanted effects on cognition and behaviour, carbamazepine is an excellent drug for the treatment of people with intellectual disability and epilepsy. Carbamazepine is still one of the most commonly prescribed medications in the treatment of epileptic disorders.     

Lamotrigine in the treatment of epilepsy in people with intellectual disability

Information about the mechanism of action and pharmacology of lamotrigine is summarized. A brief review of the literature on the use of this drug in people with intellectual disability is followed by a suggested framework for evaluating antiepileptic drugs in this population. The role of lamotrigine is systematically examined against the suggested framework. This leads to the conclusion that lamotrigine is a very favourable drug for treating epilepsy in people with intellectual disability because it has a broad spectrum of action, is effective in treating subtle seizures, shows no loss of effect with time, is not usually sedative, does not produce difficult-to-manage adverse effects, appears to have no direct adverse behavioural effects and is available in a range of 'patient friendly' preparations. However, it is important to use the drug wisely. This implies starting with low doses of lamotrigine and escalating the dose slowly to avoid adverse effects, especially rash, and being aware of drug interactions which could cause difficulty, including the prolongation of half-life with valproate, the pharmacodynamic interaction when it is added to carbamazepine and the pharmacokinetic interactions of lamotrigine with a number of antiepileptic drugs.     

Barbiturates in the treatment of epilepsy in people with intellectual disability

Barbiturates are effective drugs in the treatment of epileptic disorder. The systemic side-effects are minimal. The main limiting factor is the presence of cognitive and behavioural problems. Relevant research is presented in this paper; however, it is somewhat difficult to extrapolate some of these experiences to a population of children and adults with intellectual disability and epilepsy. Recent reviews of this subject have suggested that, although the cognitive deficiencies seem to be a serious problem when phenobarbital is given in high doses, the problem is much less severe when the doses are on the low side. The most consistent findings with regard to behaviour are the exacerbation of behaviour disorders (mostly hyperactivity), as well as sleep disorders and depression in individuals who already have a predisposition to these disorders. However, the clinical experience of many professionals involved with the care of people with intellectual disability strongly suggests that barbiturates, and especially phenobarbital, produces intolerable side-effects at the point that the use of phenobarbital has been reduced to a minimum, and it is no longer considered a drug of choice. It is probably that the simultaneous presence of brain damage, epilepsy, intellectual disability and psychiatric disorders in people with intellectual disability is responsible for the high incidence of behaviour problems observed by clinicians.     

Valproate in the treatment of epilepsy in people with intellectual disability

Valproate is a major broad-spectrum antiepileptic drug effective against many different types of epileptic seizures. Valproate is a first-line drug in the treatment of primary generalized seizures and syndromes, but it is also effective in other seizure and epilepsy types. The possible mechanisms of action and the pharmacokinetics of valproate are outlined. A limited number of studies on the efficacy and safety of valproate treatment in patients with West syndrome and Lennox-Gastaut syndrome have shown that even therapy-resistant people with intellectual disability can benefit from add-on valproate medication. In status epilepticus, valproate can be effective either intravenously, by gastric drip or following rectal administration. Patient tolerance towards valproate is generally good. The most serious adverse effect of valproate include hepatotoxicity and teratogenicity.     

Benzodiazepines in the treatment of epilepsy in people with intellectual disability

All the benzodiazepines (BZDs) in clinical use have the capacity to promote the binding of the major inhibitory neurotransmitter, gamma-amino-butyric acid (GABA), to sub-types of GABA receptors which exist as multi-subunit ligand-gated chloride channels. Thus, the BZDs facilitate the actions of GABA in the brain. The BZDs in use as antiepileptic drugs are diazepam, clonazepam, clobazam, nitrazepam, and lately, also lorazepam and midazolam as emergency therapy. The BZDs have a wide-spectrum of proven clinical efficacy in the prevention of different kind of seizures. Clonazepam and clobazam, as well as nitrazepam in some cases, can be useful as an adjunct treatment in refractory epilepsies. However, the clinical use of BZDs for the prophylactic treatment of epilepsy is associated with two major problems which have limited the long-term use of these drugs: the potential for side-effects, especially sedative effects, and the high risk of development of tolerance. Despite the limitations of BZDs in the prophylactic treatment of epilepsies, these drugs play a prominent role in clinical practice in the emergency management of acute seizures and status epilepticus. Diazepam, clonazepam and lorazepam are all considered first-line agents in the emergency management of acute seizures and status epilepticus. Furthermore, the value of midazolam as an emergency therapy in epilepsy has been increasingly recognized in recent years.     

Antiepileptic efficacy of vigabatrin in people with severe epilepsy and intellectual disability

The short- and long-term clinical efficacy of add-on vigabatrin treatment was evaluated in a group of 36 patients with intellectual disability and drug-refractory epilepsy. The results were compared to the efficacy of vigabatrin in 75 non-retarded patients with drug-resistant complex partial and secondarily generalized seizures. After 3 months, 42% of the patients with intellectual disability had experienced a reduction in seizure frequency of more than 50% (responders). The percentage of responders was still 22% after 6 years. No impairment in psychological function was observed during vigabatrin treatment compared with baseline values. However, one patient was excluded from long-term treatment because of psychotic depression and two patients because of psychomotor slowing after 1-2 years of treatment The need for extra supervision appeared to diminish and three patients were able to be discharged from institutional care during the follow-up. In the group of non-retarded patients, the percentages of the responders were 55% and 27% after 3 months and 6 years of treatment, respectively. The results from these studies suggest that vigabatrin is effective and relatively well tolerated, and that the successful treatment of epilepsy also has socio-economic consequences in patients with intellectual disability and severe epilepsy.     

Phenytoin: effective but insidious therapy for epilepsy in people with intellectual disability

Phenytoin (5,5-diphenylhydantoin), which has been in use for 60 years, is still an important antiepileptic drug. Its primary mechanism of action is modulation of the sustained repetitive firing of neurones by direct inhibition and blockage of voltage-gated sodium channels in the neuronal cell membrane, and by delay of cellular reactivation. The plasma protein binding of phenytoin is normally between 90% and 95%. The drug is rapidly distributed from the blood to the tissues and is almost completely metabolized in the liver. The plasma phenytoin concentration normally reaches the steady-state level within 1-2 weeks. The half-life of phenytoin is less than 20 h in low doses, but is prolonged in high doses, newborn infants and elderly people. The half-life is shortened when phenytoin is given concomitantly with an enzyme-inducing drug, such as phenobarbital or carbamazepine. Phenytoin is effective for treating generalized tonic-clonic seizures, partial seizures with or without generalization, and convulsive status epilepticus. Over the years, many new, and even serious, adverse effects of phenytoin have been recognized. Phenytoin encephalopathy, manifesting as cognitive impairment and a cerebellar syndrome, is an important adverse neurological effect, the development of which depends on the saturation kinetics of phenytoin, individual differences in phenytoin metabolism, an inhibitory effect of certain drugs on phenytoin metabolism, or the ability of certain drugs to displace phenytoin from plasma proteins, leading to an increase in the plasma level of unbound phenytoin. Because of its potentially adverse effects, phenytoin is not recommended as the first choice for treating epileptic seizures, except as a co-drug for managing convulsive status epilepticus. In patients with epilepsy who also have intellectual disability, and are susceptible to balance disturbances and cognitive dysfunction, it is wise to replace phenytoin with another drug, such as carbamazepine or oxcarbazepine. The long-term use of phenytoin is not recommended for patients with loss of locomotion, marked cognitive impairment, or symptoms and signs of cerebellar disease. The prevention of phenytoin intoxication, with the subsequent development of phenytoin-induced encephalopathy, depends on careful observation of the patients and frequent monitoring of plasma levels of phenytoin and other concomitantly administered antiepileptic drugs.     

Homosexuality among people with a mild intellectual disability: an explorative study on the lived experiences of homosexual people in the Netherlands with a mild intellectual disability

Background Empirical research on homosexuality among people with an intellectual disability (ID) is limited and, to date, very little is known regarding the personal experiences of gay and lesbian people with an ID. This study set out to answer the question: What are the lived experiences of a specific cohort of homosexual people with an intellectual disability living in the Netherlands? Method To answer this question, a qualitative study was performed in which 21 people with a mild ID were interviewed via semi‐structured interviews. In this study, 19 participants were men and two were women (average age = 40.5 years). Results Participants reported positive and negative experiences, and talked about their gay or lesbian identity. Almost half of the participants (n = 10) reported that they had experienced sexual abuse including partner violence (n = 6). Additionally, they indicated that there was a lack of support for homosexual people with an ID. Conclusion Specific problems impact the lives of homosexual people with ID, namely the high prevalence of negative sexual experiences, the lack of support, training and sex education, and their search for a suitable partner.     

Life expectancy of people with intellectual disability: a 35-year follow-up study

A 35‐year follow‐up study based on a nation‐wide population study of the life expectancy of people with intellectual disability (ID) was undertaken. The study population consisted of a total of 60969 person‐years. A prospective cohort study with mortality follow‐up for 35 years was used and the life expectancy of people with ID was calculated for different levels of intelligence. Proportional hazard models were used to assess the influence of level of intelligence and associated disorders on survival. People with mild ID did not have poorer life expectancy than the general population and subjects with mild ID did not have lower life expectancy in the first 3 decades of life. In cases with profound ID, the proportion of expected life lost was > 20% for almost all age groups. The female preponderance was manifested from the age of 60 years onwards, 25 years later than in the general population. Respectively, survival between sexes differed less. Epilepsy and/or hearing impairment increased the relative risk of death for all levels of ID. The prevalence of people with ID over 40 years was 0.4%. People with ID now live longer than previously expected, and the ageing of people with mild ID appears to be equal to that of the general population, posing new challenges to health care professionals.     

Use of antiepileptic drugs in the treatment of epilepsy in people with intellectual disability

The main principles of antiepileptic drug treatment of epilepsy in patients with intellectual disability are basically the same as for other patients with epilepsy. However, some specific issues need to be taken into account These are primarily associated with the diagnostic difficulties of epilepsy in this population. In addition, a number of other relevant issues, including the degree and location of brain lesion, the nature of the underlying disease, the higher frequency of difficult-to-treat epilepsies, the additional intellectual impairment caused by inappropriate antiepileptic medication, or by frequent and prolonged seizures, the appropriate use of monotherapy versus rational polytherapy, and the use of broad-spectrum antiepileptic drugs will be discussed in the present paper. Although the goals of treatment are to keep the patient seizure-free and alert while preventing possible mental deterioration, we have to accept compromises between these primary goals in many cases. Some people with epilepsy and intellectual disability are very vulnerable to insidious neurotoxic effects; for example, sedative effects caused by phenobarbital, or cognitive and/or cerebellar dysfunction caused by long-term phenytoin, especially together with other drugs. Because of the adverse effects of phenobarbital and phenytoin, these drugs are no longer recommended as a first-choice drugs when long-term antiepileptic medication is required. In primary generalized tonic-clonic seizures, valproate, oxcarbazepine/carbamazepine and lamotrigine are recommended in this order of preference. The corresponding recommendations are: in typical absences, valproate, ethosuximide and lamotrigine; in atypical absences, valproate and lamotrigine; in juvenile myoclonic epilepsy, valproate, lamotrigine and clobazam; in infantile spasms vigabatrin, ACTH and valproate; in Lennox-Gastaut syndrome, valproate, lamotrigine and vigabatrin; in atonic seizures, valproate and lamotrigine; in simple and complex partial seizures with or without secondary generalization, oxcarbazepine/carbamazepine, valproate/ vigabatrin and lamotrigine; and in status epilepticus lorazepam, diazepam and clonazepam together with phenytoin or fosphenytoin. In cases of poor response to the monotherapy recommended above, the following combinations may be indicated: in primary generalized tonic-clonic epilepsy, valproate and oxcarbazepine/ carbamazepine, or valproate and lamotrigine; in typical absences, valproate and lamotrigine, or valproate and ethosuximide; in juvenile myolonic epilepsy, valproate and lamotrigine, or valproate and clonazepam; and in partial epilepsies, add to the monotherapy one of the following drugs, vigabatrin, lamotrigine, gabapentin, tiagabine, topiramate, zonisamide or clobazam. So far, the order of preference of these new drugs remains undetermined. More data are needed on the efficacy and adverse effects of the new drugs based on controlled studies on patients with intellectual disability and epilepsy.     

Bullying and people with severe intellectual disability

Although bullying has been shown to reduce quality of life in many spheres, anti‐bullying strategies have yet to be incorporated into services for adults with severe intellectual disability (ID). The present study employed a survey of staff and parent concerns about 54 previously surveyed students who had left a school for pupils with severe ID. A content analysis of follow‐up interviews was performed in 10 cases. Staff identified 19% of the survey sample as bullying others and 11% as being picked on. Neither gender nor communication ability had an impact. There was no significant change in bully or victim status over time, although some people did change. Parents or staff raised bully/victim problems in more than half of the interviews. There is sufficient evidence of bullying behaviour to warrant the adoption of anti‐bullying strategies.     

Estimating the risk of crime and victimisation in people with intellectual disability: a data-linkage study

Purpose

People with intellectual disability (PWID) appear more likely to be victims and perpetrators of crime. However, extant evidence pertaining to these risks is limited by methodological weaknesses and the absence of consistent operational definitions. This research aimed to estimate the prevalence of criminal histories and victimisation using a large, well-defined sample of PWID.

Methods

A case-linkage study was conducted comprising 2220 PWID registered with disability services in Victoria, Australia, whose personal details were linked with a state-wide police database. Criminal charges and reports of victimisation were compared to a non-disabled community comparison sample (n?=?2085).

Results

PWID were at increased risk of having a history of criminal charges, particularly for violent and sexual offences. Although the non-disabled comparison group had a greater risk of criminal victimisation overall, PWID had a greatly increased risk of sexual and violent crime victimisation.

Conclusions

PWID are at increased risk of victimisation and perpetration of violent and sexual crimes. Risk of sex offending and victimisation is particularly elevated, and signalling the need for specialised interventions to prevent offending and to ensure victims is assisted with access to justice, support, and treatment.