The mortality associated with epilepsy, with particular reference to sudden unexpected death: a review.

Recent studies indicate that the overall mortality rate for persons with epilepsy is elevated two- or threefold compared with the general population. The standardized mortality ratio (SMR) is greatest in the first few years after diagnosis and in symptomatic epilepsies. Idiopathic epilepsies also have a small increase in SMR. The sudden unexpected death rate in those with epilepsy (SUDEP) depends on the population examined. In the general population of persons with epilepsy, the risk is between 1:500 and 1:1,000 person-years. For those with severe epilepsy or other neurologic impairments, the risk appears to be 1:200 person-years. The risk in children remains uncertain. Estimates of mortality should be borne in mind when patients are counseled about the risks and benefits of various treatment strategies.

Assessing the psychosocial consequences of epilepsy: a community-based study.

BACKGROUND: Few studies have measured, using validated scales, the psychosocial handicap of epilepsy in a general practice setting. AIM: To assess the prevalence of psychosocial problems associated with epilepsy. METHOD: A survey was undertaken of 309 subjects, with one or more non-febrile epileptic seizures, drawn from two general practices in the United Kingdom (UK). The outcome measures were the Subjective Handicap of Epilepsy Scale (SHE), the SF-36, and the Hospital Anxiety and Depression scale (HAD). RESULTS: One-third of persons with active epilepsy were significantly handicapped by their condition. The severity of subjective handicap was related to seizure frequency and to the duration of remission of seizures. Between one-third and one-half of subjects scored as 'cases' on the HAD scale and on the mental health subscale of the SF-36. Only one-third of the psychiatric morbidity revealed by the questionnaires had been recognized by the general practitioner (GP). Scores on the SF-36 indicated that people with active seizures perceived themselves as significantly less healthy than those in remission, and that, for persons in remission, drug treatment had a detrimental effect on certain aspects of well-being. CONCLUSIONS: The occurrence of seizures, even at low frequencies, is associated with psychosocial handicap, and this may remain covert in general practice.

How rational is current anti-epileptic drug treatment?

The epilepsy community has often failed to recognise that, at present, anti-epileptic drug therapies are empirical rather than rational. Our current practice may be termed "rational prescribing" rather than "rational therapy". Significant recent improvements in the quality of the available empirical evidence, though welcome, cannot be considered as a substitute for a more scientific approach to epilepsy. The commitment to a molecular biological approach that is at the forefront of cancer and HIV research needs to be adopted by physicians treating people with epilepsy.

Myoclonic epilepsy of late onset in trisomy 21.

We report the case of a patient with trisomy 21 (T21) with late onset epilepsy. The electroclinical features were of myoclonic jerks on awakening and generalised tonic clonic seizures, with generalised spike and wave on EEG, and a progressive dementia. As familial Alzheimer's dementia and progressive myoclonic epilepsy (Unverricht-Lundborg type) are both linked to the chromosome 21, this case may represent a distinct progressive myoclonic epilepsy related to T21.

Allergic skin rash with lamotrigine and concomitant valproate therapy: evidence for an increased risk.

Cutaneous rash is one of the commonest adverse events associated with lamotrigine. We assessed whether the risk is increased in patients receiving concomitant valproate therapy in a population of 103 adult patients with intractable epilepsy, who had lamotrigine added to their treatment. Of the 33 patients taking valproate, 10 (30%) developed a rash, whilst of the 70 not taking valproate, only 6 (8%) developed a rash. This suggests a significantly higher risk of cutaneous rash when starting lamotrigine in patients already taking valproate (p < 0.02).

A historical perspective on the mortality associated with chronic epilepsy.

OBJECTIVE: To examine the secular trend in the mortality associated with chronic epilepsy. MATERIAL AND METHODS: Using data from the Chalfont Centre for Epilepsy, UK, a residential centre for people with epilepsy, we determined the standardized mortality ratio (SMR) in the Chalfont population for each 5-year epoch from 1896 to 1965. RESULTS: The SMR was found to be between 2 and 3 for most 5 year epochs from 1896 to 1965. CONCLUSION: Changes in the average age and disability of the residents at the Chalfont Centre confound a definite conclusion, but the evidence suggests the possibility that an excess mortality has been associated with chronic epilepsy for 100 years despite major changes in treatment.

The subjective handicap of epilepsy. A new approach to measuring treatment outcome.

It is now widely acknowledged that the impact of epilepsy on the individual extends beyond the occurrence of seizures, and that there is a need for outcome measures sensitive to these consequences. Until now these instruments have largely been developed within a 'quality of life' framework. The technical and conceptual difficulties that arise with measuring quality of life have led us to develop a more focused measurement model, the 'Subjective Handicap of Epilepsy' (SHE) scale, based on the World Health Organization's concept of handicap. The scale contains 32 items in six subscales: 'Work and activities' (eight items), 'Social and personal' (four items), 'Self-perception' (five items), 'Physical' (four items), 'Life-satisfaction' (four items) and a 'Change' scale (seven items); and it takes on average < 10 min to complete. The scale's test-retest reliability was found to be satisfactory (intra-class correlation coefficient was 0.8-0.9 in 110 subjects). The test-retest interval (24 h to 8 weeks) had no influence on the reliability. The reliability was also not affected by minor recent fluctuations in seizure frequency. The internal consistency of the scales was 0.8-0.9 (Cronbach's alpha). The construct validity of the scale was examined in a sample of 287 clinic attendees at a university neurology clinic in the UK. The scales were highly sensitive to the handicapping effects of increasing seizure frequency, employment status, the impact of epilepsy on career choice and the subject's own opinion as to the major determinant of their quality of life. The scales were also sensitive, retrospectively, to the benefits of successful epilepsy surgery in a cohort of 105 patients. Scales focusing specifically on handicap were more sensitive to group differences in seizure frequency in the clinic population, and to outcome after epilepsy surgery, than the 'Life-satisfaction' scale and the Epilepsy Surgery Inventory 55 (ESI-55) scales. This supports the contention that measuring 'subjective handicap' may be a more sensitive, and more useful, approach to assessing the impact of interventions on the long-term consequences of epilepsy than current methods.

The National Hospital Seizure Severity Scale: a further development of the Chalfont Seizure Severity Scale.

Seizure severity scales have recently been identified as an important additional outcome measure in trials of new antiepileptic drugs (AEDs). The National Hospital Seizure Severity Scale (NHS3) is presented as a refined version of the Chalfont Seizure Severity Scale. The principal advantages of the new version are that it is quicker and simpler to apply, the limits of reliability are now clearly defined, and construct validity for the scale is available. The scale is administered by a health professional during an interview with a patient and a witness to the seizures. It contains seven seizure-related factors and generates a score from 1 to 27. An intraclass correlation coefficient of 0.90 was obtained during interobserver and test-retest reliability assessment, suggesting that the scale is sufficiently reliable for group studies. Scores for an individual patient should be interpreted with caution in light of the limits of agreement obtained. Validation experiments indicate that NHS3 measures seizure severity in a manner compatible with the subjective impression of people with epilepsy. We suggest that the NHS3 is a valid, easily applicable measure of seizure severity that is acceptably reliable for use in trials of novel AEDs.

Myoclonic epilepsy of late onset in trisomy 21

Pacientes com trissomia 21 (T21), com o passar dos anos, sao propensos a desenvolver crises epilépticas parciais concomitantes ao aparecimento de degeneraçao cerebral do tipo Alzheimer. Pacientes com T21 e demência parecem ter o risco maior de apresentarem crises epiléticas que outros pacientes com degeneraçao cerebral do tipo Alzheimer. O caso relatado é de um paciente com T21 com epilepsia de início tardio. A história clínica consiste de crises mioclônicas ao despertar. ocasionais crises generalizadas tônico-clônicas, demência e ponta onda generalizada no EEG. Demência do tipo Alzheimer familial é ligada ao cromossoma 21, bem como epilepsia mioclônica progressiva (tipo Unverricht-Lundborg) Isto sugere que este caso possa representar um tipo distinto de epilepsia mioclônica progressiva, ligado ao cromossoma 21.

Allergic skin rash with lamotrigine and concomitant valproate therapy

Cutaneous rash is one of the commonest adverse events associated with lamotrigine. We assessed whether the risk is increased in patients receiving concomitant valproate therapy in a population of 103 adult patients with intractable epilepsy, who had lamotrigine added to their treatment. Of the 33 patients taking valproate, 10 (30 per cent) developed a rash, whilst of the 70 not taking valproate, only 6 (8 per cent) developed a rash. This suggests a significantly higher risk of cutaneous rash when starting lamotrigine in patients already taking valproate (p