Stress and residential staff who work with people who have an intellectual disability: a factor analytic study

The present study describes the development of a questionnaire, and the subsequent collection of data, to gather information on the demands and supports which influence stress (as measured by anxiety and depression scales) in direct care staff who work with people with intellectual disability. The results from the questionnaire were also used to explore the relationships between the factors derived from the questionnaire and to consider how these may be influential in building organizational models. The questionnaire was specifically constructed to measure the demands and supports experienced by direct care staff. The participants included 216 staff working in community residential services for people with intellectual disability. These individuals included staff working for two British health trusts and similar staff working for one local authority. The questionnaire consisted of 33 demands items and 23 supports items. Separate factor analyses were conducted on the demands and supports scales. Analyses of the reliability and validity of the resulting factors were conducted. Stepwise multiple regressions were conducted to further explore the relationship of factors with the measures of anxiety and depression. Four factors were extracted from the demands scale and three factors from the support scale. There was some concern over levels of reliability and validity for individual factors; however, the reliability of the main scales appeared to be satisfactory. All but one support factor correlated significantly with levels of anxiety and depression. The factors explained a modest amount of the variance in the regressions. While there are some concerns about the psychometric properties of the questionnaire, it is argued that the scales and factors can be used to confirm and further understanding of the relationship between groups of demands, supports and stress in staff. The usefulness of the questionnaire as a means of diagnosing specific sources of demand and support is considered. It is suggested that the questionnaire could form the basis for assessment and subsequent intervention in houses where staff are reporting relatively high levels of anxiety and depression.     

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.     

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.     

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.     

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.     

Self-injury in people with intellectual disability and epilepsy: a matched controlled study

We aimed to identify the presence of self-injurious behavior in a sample of 158 people with intellectual disability and epilepsy as compared with a control sample consisting of 195 people with intellectual disability without epilepsy. The Italian Scale for the Assessment of self-injurious behaviors was used to describe self-injurious behavior in both groups. The groups were matched for ID degree: mild/moderate (20 and 20 respectively), severe/profound (45 in both samples) and unknown (4 in both samples). Seventy-four percent of the first sample were diagnosed with symptomatic partial epilepsy. The prevalence of self-injurious behaviors was 44% in the group with intellectual disability and epilepsy and 46.5% in the group with intellectual disability without epilepsy (difference not significant). The areas most affected by self-injurious behaviors in both samples were the hands, the mouth and the head. The most frequent types of self-injurious behaviors were self-biting, self-hitting with hands and with objects. Self-injurious behavior is frequently observed in individuals with epilepsy and intellectual disability. Our study does not suggest that the presence of epilepsy is a risk factor for self-injurious behavior in this patient group.     

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.     

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.     

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.     

Topiramate in patients with learning disability and refractory epilepsy

PURPOSE: Management of seizures in learning disabled people is challenging. This prospective study explored the efficacy and tolerability of adjunctive topiramate (TPM) in patients with learning disability and refractory epilepsy attending a single centre. METHODS: Sixty-four patients (36 men, 28 women, aged 16-65 years) were begun on adjunctive TPM after a 3-month prospective baseline on unchanged medication. Efficacy end points were reached when a consistent response was achieved over a 6-month period at optimal TPM dosing. These were seizure freedom or > or =50% seizure reduction (responder). Appetite, behaviour, alertness, and sleep were assessed by caregivers throughout the study. RESULTS: Sixteen (25%) patients became seizure free with adjunctive TPM. There were 29 (45%) responders. A further 10 (16%) patients experiencing a more modest improvement in seizure control continued on treatment at the behest of their family and/or caregivers. TPM was discontinued in the remaining nine (14%) patients, mainly because of side effects. Final TPM doses and plasma concentrations varied widely among the efficacy outcome groups. Many patients responding well to adjunctive TPM did so on < or =200 mg daily. Mean carer scores did not worsen with TPM therapy. CONCLUSIONS: TPM was effective as add-on therapy in learning-disabled people with difficult-to-control epilepsy. Seizure freedom is a realistic goal in this population.     

Invisible victims: Sexual assault of people with an intellectual disability

This paper discusses the lack of attention which has been given by helping professionals, police, welfare and legal systems concerning the sexual assault of adults who are intellectually disabled. Reasons why people with intellectual disability are particularly vulnerable to sexual assault are also explored. A research project conducted in 1989–1990 by the author for the NSW Women's Co-ordination Unit is described and the major findings discussed.