Introduction
Epilepsy is the third most common neurological diagnosis in old people after dementia and stroke. The peak ages of onset of epilepsy are in childhood and old age (Table 61.1).1 About 25% of newly diagnosed patients and 23% of people with epilepsy in the community are over the age of 60 years. However, little epilepsy research has been undertaken in elderly subjects and there have been only three double-blind randomized trials of antiepileptic drugs (AEDs) in the elderly.2 Epilepsy is defined as the tendency to recurrent seizures, excluding febrile convulsions. A single seizure is not considered as ‘epilepsy’, although studies have suggested that up to 75% of people go on to have a second seizure and in the elderly this may be as high as 90%.3
Age (years) | Incidence |
0–10 | 60:105 |
20–40 | 40:105 |
60–69 | 101:105 |
70–79 | 150:105 |
80–89 | 190:105 |
Reproduced from Tallis et al.1 by permission of Oxford University Press.
A seizure results from a paroxysmal abnormal synchronous electrical discharge in the brain. A diagnosis of epilepsy is not an endpoint diagnosis—it should automatically lead to investigations for classifying the type of seizure (compare making a diagnosis of jaundice) as epilepsy is the result of many diverse neurochemical, neuropathological and neurophysiological abnormalities. Epilepsy is a clinical diagnosis based on an eyewitness description of an episode. At least 10% of people with a diagnosis of ‘epilepsy’ do not have the disorder. One of the commonest misdiagnoses is reflex anoxic seizures. These occur when someone feels faint and is kept upright, a few muscle jerks occur and a diagnosis of an ‘epileptic’ seizure is made. As epilepsy has such serious consequences with regard to activities of daily living, in particular driving, it is a diagnosis which should not be made lightly. If there is doubt, it is better to await clear evidence. An electroencephalogram (EEG) does not make the diagnosis of epilepsy as many trivial abnormalities are often overinterpreted. An EEG simply helps classify the kind of epilepsy—localization-related or generalized.
Aetiology
In the majority of elderly patients who develop epilepsy, it will be presumed to be secondary to cerebrovascular disease (Table 61.2).4 Although the majority of patients are worried that they have an underlying brain tumour, this is rare. Neurodegenerative diseases such as Alzheimer’s disease account for a significant percentage of those developing epilepsy. With the advent of MRI scanning, it is likely that in a higher proportion of patients an aetiological factor will be found in a higher proportion of elderly patients.
Cerebrovascular disease | 30–42% |
Tumour | 2–14% |
Dementia | 2–14% |
Toxic/metabolic | 6–12% |
Other | 22–58% |
Reproduced from Hildick-Smith4 by permission of Royal Society of Medicine Press.
Dementia
Epileptic seizures are common in demented patients, especially in the later stages of the disease. A study in Dundee suggested that 9% of patients had had an epileptic seizure; 92% of reported seizures were tonic–clonic. As complex partial seizures are generally more common than tonic–clonic seizures, this would suggest that many partial seizures probably go unrecognized. Patients developing epilepsy were significantly younger and more severely demented. On the whole, the epilepsy appeared to be reasonably well controlled.5 Another study found that the mean onset of epilepsy was 6.5 years (range 2–15 years) after the onset of the dementia. There was a threefold increase in the incidence of epilepsy compared with a reference population.6 A study of 446 autopsy-proven cases of Alzheimer’s disease found that 17% were documented as having seizures during their lifetime. However, two-thirds of patients had less than three seizures, suggesting that on the whole the seizure disorder was mild.7
Cerebrovascular Disease
Cerebrovascular disease is the commonest cause of seizures in the elderly. Cerebrovascular disease increases the risk of an epileptic seizure by a factor of 20 and is the commonest cause of seizures in the elderly. There is an increased risk of developing epilepsy in the first year after a cerebrovascular accident by a factor of 17 compared with the risk in age-matched patients in the general population.8 The overall incidence of seizures after a cerebrovascular accident varies between 4.4 and 12.5%, depending on the population studied and the methods used. Studies looking at the risk factors for the subsequent development of epilepsy have suggested that patients with haemorrhagic strokes, embolic stroke, cortical lesions, hippocampal involvement or lesions involving more than one lobe are at greater risk of developing epilepsy. Early seizures within 2 weeks of a cerebrovascular accident (2–8%) are due to acute biochemical abnormalities, and late seizures (after 2 weeks) are secondary to chronic processes such as removal of inhibition and formation of new synaptic connections. Epileptic seizures in old age are often the first signs of cerebrovascular disease and therefore people presenting with a first seizure should be evaluated for risk factors for vascular disease as they have a 2.8–9 greater risk [95% confidence interval (CI) 2.45–3.40] of having a stroke within 5 years.2, 9 A post-ictal Todd’s paresis is often misdiagnosed as a stroke recurrence and if the patient has a seizure with a weakness then this alternative diagnosis needs to be considered.
Diagnosis
The diagnosis of epilepsy is the same in elderly as in younger patients. It is a clinical diagnosis based on eyewitness description of the episodes. One of the major problems in diagnosing epilepsy in the elderly is that they may be unable to give a description of what is actually happening to them before or after an attack and, as they often live on their own, an episode may not have been witnessed. A classical tonic–clonic seizure is relatively easy to diagnose from eyewitness descriptions. Minor attacks such as absence or complex partial seizures are much more difficult to diagnose. Often they may consist of staring episodes where the person is not responsive for a few seconds. These can be followed by automatisms or confusion, often short-lived. An EEG is useful for the classification of seizure type. Only rarely can it be used to make a diagnosis, that is, if a seizure occurs while an EEG is being performed or specific diagnostic EEG changes occur. In addition, the post-ictal period can be longer in the elderly, lasting from hours to days, leading to the incorrect diagnosis of dementia.10 In more than 50% of elderly patients who were finally diagnosed with epilepsy, epilepsy was not the initial suspected diagnosis.11 In the elderly, after the first seizure the risk of recurrent seizures is very high, greater than 90%.3 Suggestions have been made that elderly patients should be treated with AEDs after the first seizure.
Differential Diagnosis
Transient Ischaemic Attacks (TIAs)
A TIA is a vascular event lasting less than 24 h. It tends to be associated with negative phenomena such as weakness or sensory loss. Conversely, epilepsy tends to have positive phenomena such as paraesthesias or jerking of a limb. However, at times it is difficult to differentiate simple partial seizures from TIAs. If consciousness is lost, it is very unlikely to be a TIA. It also has to be remembered that cerebrovascular disease is an important aetiological factor in the development of epilepsy.
Transient Global Amnesia
This is not an uncommon disorder occurring in later life, when a person has no memory for a significant period of between a few hours and a day or so. Eyewitnesses say that the person appeared normal and was able to perform complex tasks. On closer questioning, the person often was repeating the same questions such as ‘what time is it?’ A person tends to have only a single episode, so if repeated the diagnosis needs to be reconsidered. The symptoms are due to a defect in memory for events of the present and recent past. During the episode, consciousness is retained and there is no impairment in intellectual functioning. Studies have suggested that there is no increase in risk factors for vascular disease and no subsequent increase in cerebrovascular disease. There is, however, an increased incidence of migraine. Transient global amnesia is an important diagnosis to make in that, unlike epilepsy, people in the UK with this condition, after reporting to the Driving Vehicle Licensing Authority (DVLA), are allowed to continue driving.
Cardiac Arrhythmias
These are very important in the differential diagnosis of epilepsy in the elderly. If the brain is starved of oxygen, for whatever reason, a tonic–clonic seizure will result. Both tachy- and bradyarrhythmias can result in tonic–clonic seizures. Clues to the fact that the seizure may be a secondary phenomenon may be found in the prodrome. A history of feeling faint or palpitations should make a clinician suspect a cardiac cause for the episodes of loss of consciousness. A 24 h EEG with an ECG lead, videotelemetry and reveal devices can be useful in trying to differentiate between a cardiac and an epileptic episode. If the episode is cardiac in nature, the arrhythmia will begin before the abnormal electrical activity in the brain by a significant time lapse. It has to be remembered that during a seizure it is not uncommon for a cardiac arrhythmia to occur, but it will begin at about the same time as the abnormal electrical activity in the brain.
Syncope
If a person is kept upright during a vasovagal attack, it is not uncommon for a few myoclonic jerks to occur and in susceptible people a tonic–clonic seizure can result. The events leading up to the episode of loss of consciousness and also eyewitness descriptions are very important. The feeling of faintness, dizziness and the need to get fresh air preceding the episode make it likely that the episode was syncopal rather than epileptic in nature. The person is usually upright during the episode. They feel dizzy, queasy and giddy and often things recede or go black. Pallor is noted and often a cold perspiration. Nausea and vomiting occasionally accompany these symptoms. After falling to the ground, consciousness is regained very fast, and if the person lies down the episode can often be aborted. A few myoclonic jerks can occur, as can incontinence if the bladder is full. Micturition syncope, especially in men having to get up during the night, is a relatively common cause of loss of consciousness; so too is cough syncope. The elderly are more prone to syncope as they may have impaired cardiovascular reflexes or be on vasodilating drugs such as glyceryl trinitrate (GTN) or have carotid sinus hypersensitivity.
Psychogenic Epileptic Attacks
Psychogenic epileptic attacks are thought to be rare beginning in the elderly but are commonly misdiagnosed as people often have underlying organic problems.
Panic Attacks
It is rare for these to present de novo in the elderly as usually there is a life-long history. The person feels that something is going to happen. He/she feels the need to take deep breaths, becomes dizzy and light-headed, develops paraesthesias in the limbs and around the mouth and the legs become heavy. Symptoms can be reproduced by getting the person to hyperventilate. The initial precipitant was often a faint and panic attacks develop subsequently because of a fear of a further episode.
Drop Attacks
These are episodes of unknown aetiology where a middle-aged or older person, usually a woman, will suddenly drop to the ground. There is no preceding warning and consciousness is not lost. There is often bruising to face, hands and knees. After the episode, the person can get up immediately. The EEG is normal, even during a fall. There is no treatment.
Hypoglycaemia
Hypoglycaemia is a rare cause of tonic–clonic seizures. It obviously needs excluding in diabetic patients on treatment. Rarely an insulinoma can produce tonic–clonic seizures. Other metabolic causes such as hypocalcaemia can precipitate seizures.
Alcohol and Drugs
Alcohol abuse and withdrawal can precipitate seizures and it is important to take an alcohol history, even in the elderly. Many drugs, particularly tricyclic antidepressants, antimalarials, phenothiazines and butyrophenones, lower the seizure threshold and can precipitate seizures. The withdrawal of benzodiazepines occasionally can produce seizures.
Sleep Disorders
Seizures during sleep need to be differentiated from parasomnias, but usually epilepsy can be diagnosed by taking a good history from the patient and obtaining an eye-witness description of the episode. A REM (rapid eye movement) sleep behaviour disorder occurs when somebody acts out their dreams. Periodic limb movement disorders are characterized by periodic movements occurring during non-REM sleep. Most commonly people dorsiflex their ankles and flex their knees and hips every 20–40 s and this can be associated, in about 30% of patients, with restless legs syndrome. Hypnic jerks are a normal phenomenon occurring in about 70% of the population and consist of brief myoclonic jerks at sleep onset.
Seizure Classification
Epilepsy is classified in two ways. There is classification of the seizure itself (Table 61.3)12 and then there is syndromic classification.13 Epilepsy is divided into two main groups. The generalized epilepsies are when the abnormal electrical activity rises from both hemispheres together (generalized spike wave). Localization-related epilepsy is when the abnormal electrical activity arises in one place in the brain and then spreads.
I. Partial seizures A. Simple partial seizures B. Complex partial seizures 1. With impairment of consciousness at onset 2. Simple partial onset followed by impairment of consciousness C. Partial seizures evolving to generalized tonic–clonic convulsions 1. Simple evolving to generalized tonic–clonic convulsion 2. Complex evolving to generalized tonic–clonic convulsion (including those with simple partial onset) II. Generalized seizures A. Absence seizures 1. Typical absence seizures 2. Atypical absence seizures B. Myoclonic seizures C. Clonic seizures D. Tonic seizures E. Tonic–clonic seizures F. Atonic seizures III. Unclassified seizures |
Generalized Epilepsies
The generalized epilepsies are divided into two broad categories, primary and secondary.
Primary generalized epilepsy is probably a channelopathy that manifests itself in various syndromes such as childhood absence epilepsy and juvenile myoclonic epilepsy. Various types of seizures can occur, such as absence seizures, myoclonic and tonic–clonic seizures in association with generalized spike wave (three per second) on the EEG. These syndromes tend to occur in people of normal intelligence and begin in childhood or early adult life, although occasionally new-onset cases have been seen in the elderly or the diagnosis has been missed. Minor seizures beginning for the first time in the elderly are not absence seizures or petit mal. They are likely to be complex partial seizures. Primary generalized epilepsy responds best to treatment with sodium valproate or lamotrigine or levetiracetam as second-line therapy. The outlook for total seizure control is excellent. The majority of children with childhood absence epilepsy will be seizure free and off treatment by adult life. Most patients with juvenile myoclonic epilepsy become seizure free but will relapse if treatment is stopped even if the patient is elderly. Many elderly people with a long-standing seizure disorder will never have had their epilepsy classified. In particular, the diagnosis of juvenile myoclonic epilepsy is commonly missed. Changing someone on to a more effective AED such as valproate can render them seizure free even in extreme old age.
Symptomatic secondary generalized epilepsy is more commonly seen in people with learning disabilities.
Localization-Related Epilepsy
In localization-related seizure disorders, the abnormal electrical activity begins in one area of the brain and spreads. The seizure manifestations depend on the site of onset of the abnormal electrical activity and the rate of involvement and spread to other areas of the brain. For example, abnormal electrical activity arising in the temporal lobe may begin with a feeling of fear or déjà vu