What Is Epilepsy? An Australian Guide

What is epilepsy? An Australian guide

Epilepsy is the second most burdensome neurological condition in Australia, after dementia. It accounts for about 14.6 per cent of the national neurological disease burden, and its burden on the health system exceeds that of prostate cancer.

Almost nobody knows that, including a lot of people who have it.

The figures come from a Deloitte Access Economics report prepared for Epilepsy Australia, which put numbers on a condition that had largely been described in adjectives. 142,740 Australians had active epilepsy. 14,603 new cases arrive each year. The annual cost is $12.3 billion, and the lifetime cost of a single year’s new diagnoses is $22.2 billion. Epilepsy Action Australia puts the current figure closer to 250,000 Australians, which reflects a different prevalence estimate and a later population base — the honest answer is that it is somewhere in that range and that Australia does not count it precisely.

Three to four per cent of Australians will develop epilepsy at some point in their lives. That is roughly one person in thirty.

And yet it is a condition that most people’s mental image of gets substantially wrong. Most seizures are not convulsions. Most people with epilepsy become seizure-free on medication. Most people with epilepsy drive, work and have children. And the single biggest cause of epilepsy-related death is something most people with epilepsy have never had explained to them.

This guide covers what epilepsy actually is, the numbers, what causes it, what the types are, who gets it, what the realistic outlook is, and where the Australian system lets people down. Each of those has a guide of its own, linked throughout. The practical side of supporting somebody with epilepsy in a supported living arrangement — management plans, emergency medication, overnight supervision, worker training — is covered in epilepsy and seizure support.

What epilepsy is, and what it is not

A seizure is a burst of abnormal, excessive, synchronised electrical activity in the brain. What it looks like depends entirely on which part of the brain it starts in and how far it spreads — which is why seizures range from a few seconds of staring to a full convulsion to a strange smell nobody else can detect.

Epilepsy is the tendency to have recurrent, unprovoked seizures. The usual clinical definition requires either two or more unprovoked seizures more than 24 hours apart, or one unprovoked seizure plus a high likelihood of further seizures — which is why a single seizure with a clearly abnormal EEG and MRI can be enough for a diagnosis.

Three distinctions that matter:

A seizure is not epilepsy. Many people have one seizure and never have another. Around one in ten people will have a seizure in their lifetime; far fewer develop epilepsy.

A provoked seizure is not epilepsy. A seizure caused by a reversible trigger — acute alcohol withdrawal, a severe electrolyte disturbance, very low blood sugar, a high fever in a young child, an acute head injury, certain drugs — is a symptom of that trigger rather than evidence of an ongoing tendency. Treat the cause, and the seizures usually stop.

And not everything that looks like a seizure is one. This is a bigger issue than most people realise and it has its own guide: Epilepsy Action Australia’s own material states that approximately one in five people referred to a neurologist for drug-resistant epilepsy have functional or dissociative seizures rather than epilepsy. See functional and dissociative seizures.

The Australian numbers

Three of those rows deserve more than a glance.

The 60 to 70 per cent figure is the most important and the most reassuring. Most people diagnosed with epilepsy in Australia will become seizure-free on medication, often on the first or second drug tried, often within a year. Epilepsy Action Australia notes that after two to five years seizure-free, medication can be withdrawn without relapse in about 70 per cent of children and 60 per cent of adults. For a substantial number of people, epilepsy is a condition they have, treat and eventually stop treating.

The 30 per cent figure is where the harm concentrates. Around 42,800 Australians have seizures that medication does not control, and the Australian evidence is that they wait far too long for the assessment that could change it. See drug-resistant epilepsy.

And the 725 figure is the one that should stop you. Seven hundred and twenty-five NDIS participants with epilepsy as their primary disability, out of 142,740 Australians with active epilepsy — against 19,656 on the Disability Support Pension with epilepsy as their primary condition. Epilepsy is, in NDIS terms, almost invisible, and the reasons are structural rather than accidental. See epilepsy and the NDIS.

What causes it

In a lot of cases, nobody knows, and that is a legitimate answer rather than an incomplete investigation.

Structural causes. Something physically different about the brain: a stroke, a brain injury, a tumour, a vascular malformation, a cortical malformation present from birth, hippocampal sclerosis, or scarring from infection or surgery. Structural epilepsy is the group most likely to be drug-resistant and also the group most likely to be surgically treatable, which is why MRI matters.

Genetic causes. Not usually “inherited” in the way people assume. Most genetic epilepsies arise from variants that either occurred new in the person or involve several genes with modest effects. A family history raises risk modestly for most epilepsies. A small number of epilepsies are clearly single-gene conditions with specific implications — Dravet syndrome being the best known — and genetic testing has become standard in early-onset and severe epilepsies because the result sometimes changes which medication is used.

Infectious causes. Meningitis, encephalitis, cerebral malaria, neurocysticercosis — the last of which is the commonest cause of epilepsy worldwide and rare in Australia.

Metabolic and immune causes, including autoimmune encephalitis, which is treatable with immunotherapy and which is a genuinely important diagnosis not to miss in new-onset epilepsy with psychiatric or cognitive features.

And unknown, in a substantial proportion.

Two Australian notes. Epilepsy is most often diagnosed in early childhood, adolescence, and after 65 — the last of those surprises people, and is largely stroke-related. And Indigenous Australians had a seizure hospitalisation rate around five times the non-Indigenous rate in the period studied, which is a disparity with no clinical explanation and a lot of structural ones.

The types, in outline

Epilepsy Action Australia counts around 40 types of epilepsy and epilepsy syndromes. The modern classification works in layers, and knowing which layer you are being told about avoids a lot of confusion.

Seizure type is the first layer: where the seizure starts and what it does. Focal seizures begin in one part of the brain; generalised seizures involve both sides from the outset. This is covered properly in seizure types explained.

Epilepsy type is the second: focal epilepsy, generalised epilepsy, combined, or unknown.

Epilepsy syndrome is the third, and it is the most useful when it applies. A syndrome is a recognisable package — a particular age of onset, particular seizure types, particular EEG findings, a particular outlook, and sometimes a particular treatment. Examples:

Childhood absence epilepsy, with brief staring spells many times a day, which commonly resolves.

Juvenile myoclonic epilepsy, starting in the teens with morning jerks, strongly provoked by sleep deprivation and alcohol, usually very treatable and usually lifelong.

Temporal lobe epilepsy, the commonest focal epilepsy in adults, frequently drug-resistant and frequently surgically treatable.

Self-limited epilepsy with centrotemporal spikes, a childhood focal epilepsy that resolves in adolescence.

Infantile epileptic spasms syndrome (formerly West syndrome), Lennox-Gastaut syndrome and Dravet syndrome — the developmental and epileptic encephalopathies, which are severe, drug-resistant, and associated with intellectual disability. These are the epilepsies most relevant to disability services and they are covered in epilepsy and intellectual disability.

Why the syndrome matters practically: it changes the medication. Some drugs that work well for focal seizures make generalised seizures worse. Carbamazepine can aggravate absence and myoclonic seizures. Sodium valproate is first-line for the genetic generalised epilepsies and carries particular risks in people who may become pregnant. Getting the syndrome right is the difference between treatment working and treatment making things worse. See epilepsy medications.

What epilepsy is actually like

The thing most people get wrong is assuming the seizures are the problem.

For many people with epilepsy, the seizures take up a few minutes a month and the rest of it takes up everything else: the medication side effects, the driving ban, the employment conversation, the memory problems, the sleep rules, the mood, and the constant low-grade calculation about where the next seizure might happen.

Depression and anxiety are substantially more common than in the general population. The Epilepsy Foundation’s material cites around 50 per cent experiencing depression and about 20 per cent anxiety; an Epilepsy Action Australia survey found about 20 per cent of respondents with depression — double the general population rate — and roughly one in six with anxiety, with over half rating their quality of life as low to average. The figures vary by how they are measured; the direction does not. See memory, mood and the other things epilepsy brings.

Memory complaints are near-universal and are a mixture of the epilepsy, the medication and the mood, in proportions that vary between people.

Employment is affected. The Deloitte report, using UK data as a proxy because Australian figures were not available, found employment rates of 60.8 per cent for men with epilepsy against 80.6 per cent for the general population, and 68.0 per cent against 81.1 per cent for women. Twenty-nine per cent of Australian survey respondents had been absent from work because of epilepsy in the previous year, averaging 33 days.

Driving is the loss people name most often, and the rules are specific rather than arbitrary. See driving, work and daily life.

And there is stigma, still, which is why a lot of people with controlled epilepsy tell nobody.

Where the Australian system falls down

Four gaps, each documented rather than asserted.

Recognising drug resistance. An Australian study found that only 35 per cent of surveyed GPs knew the definition of drug-resistant epilepsy, and 88 per cent wanted more information about epilepsy. There are roughly 550 neurologists nationally, 93 per cent working in capital cities, and only a handful of tertiary epilepsy centres. No national guideline governs referral or management.

The delay to specialist assessment. Diagnosis can be delayed one to four years, and some people wait up to 17 years for epilepsy surgery — a procedure suitable for around a third of people with drug-resistant epilepsy and achieving seizure freedom in an estimated 62 per cent of those who have it.

Talking about SUDEP. Australian clinical guidance acknowledges that families want information about sudden unexpected death in epilepsy and that doctors often do not provide it, with the barriers being clinician comfort, knowledge and opportunity rather than any evidence that disclosure harms people. See SUDEP.

And funding. 725 NDIS participants, 19,656 on the DSP, $12.3 billion a year in costs, and $28.8 million a year in research funding.

What to do if this is a new diagnosis

1. Get the diagnosis in writing, with the seizure type, the epilepsy type, and the syndrome if one has been identified.
2. Ask whether an MRI and EEG have been done, and ask what they showed.
3. Ring a free specialist service. Epilepsy Action Australia on 1300 37 45 37 offers telehealth appointments with an epilepsy nurse. The National Epilepsy Support Service on 1300 761 487 is the Epilepsy Foundation’s line. Both are free, both are staffed by people who do this all day, and both will answer things a seven-minute appointment could not.
4. Ask about the driving rules for your situation, and notify the licensing authority — it is a legal obligation.
5. Ask what the triggers are for you, and take the sleep advice seriously, because sleep deprivation is the most consistently reported trigger across epilepsy types.
6. If you are a woman or girl who might become pregnant, ask specifically about which medication you are being started on and why. This matters more than almost anything else in the first appointment. See women and epilepsy.
7. Ask about SUDEP. If it is not raised, raise it. The guidance says the best time is at diagnosis.
8. Start a seizure diary, with dates, times, descriptions, and what was happening beforehand. It is the single most useful thing you will bring to your next appointment.
9. And if you are still having seizures after two medications have been properly tried, ask for referral to a comprehensive epilepsy centre. That sentence is the most valuable one in this entire library.

Seizure first aid, in sixty seconds

Worth knowing whether or not you have epilepsy, because around one in ten people will have a seizure at some point and most seizures happen in front of somebody who has no idea what to do.

For a convulsive seizure:

Stay. Do not leave them.

Time it, from the start, on a clock or phone. This is the most useful single thing you can do, because the decision to call an ambulance turns on the number and people routinely overestimate duration.

Protect the head. Move hard objects away, put something soft underneath, loosen anything tight at the neck.

Do not restrain them, and do not try to stop the movements.

Do not put anything in their mouth. Not a spoon, not your fingers, nothing. Tongue biting is not prevented by it, and injuries are caused by it.

Once the convulsion eases, roll them onto their side to protect the airway.

Stay with them until they are properly recovered, which can take anywhere from minutes to an hour or more, and be calm about the confusion — people frequently wake frightened and disoriented.

Afterwards: tell them what happened, because they will not know, and note the time it started and stopped.

For a focal impaired awareness seizure — the staring, fumbling, wandering kind — do not grab or restrain. Guide them gently away from danger, speak calmly, and stay until they are oriented. People in this state have been arrested, ejected from shops and assumed drunk, and a calm bystander prevents most of that.

Call 000 if: the convulsion lasts more than five minutes; seizures repeat without recovery in between; it is a first-ever seizure; it happened in water; there is significant injury; breathing does not return to normal; the person is pregnant; or you are simply unsure. If there is a management plan, follow it.