Transcript First seizure: diagnosis / management

The many faces of seizures in epilepsy
in people with cavernomas
International Cavernoma Alliance
UK Forum
London, 13 June 2015
Dr Tim Wehner
National Hospital for Neurology & Neurosurgery
University College London
United Kingdom
Take home messages
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Epileptic seizures are the most common first
presentation of a cavernous angioma
The cavernoma is often, but not always, the cause of
the seizures
The risk of seizures and the manifestation of seizures
depend on the location of the cavernoma in the brain
The first line treatment are seizure medications
(Lamotrigine, Carbamazepine, Levetiracetam, and
others)
Epilepsy surgery should be considered in people with
cavernoma who do not become seizure free on seizure
medications
What are epileptic seizures?
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An epileptic seizure is a sudden change of behaviour
and/or subjective experience,
caused by hypersynchronous discharges of neurons in
the brain (“electrical storm“)
Epileptic seizures may indicate an underlying medical or
neurological disorder
In principle, epileptic seizures can be provoked in any
person‘s brain, e. g. through sleep deprivation, drugs,
electrical stimulation,…
1 in 20 people have an epileptic seizure in their life
Importantly, not all seizures are epileptic seizures.
What is Epilepsy? – Clinical definition
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A brain disorder that is characterized by occurence of
unprovoked epileptic seizures
About 1 in 131 people in the UK, i. e. ~ 500.000
people, have epilepsy
>50 Million people have epilepsy worldwide
Most common disabling chronic neurological disorder
Classification of Seizures & Epilepsies
Generalised
- absence
- atonic
- myoclonic
- tonic
- tonic-clonic
Focal / partial
-without alteration of
conciousness (“simple partial”)
-with alteration of conciousness
(“complex partial”)
GENETIC (“idiopathic”)
STRUCTURAL – METABOLIC (“symptomatic”)
CRYPTOGENIC (“unknown”)
Symptomatogenic areas
Left hemisphere, lateral aspect
Symptomatogenic areas
Left hemisphere, mesial aspect
Symptomatogenic areas
Left Insula
Relationship of Cavernoma and
Epilepsy
Definite CRE: This term refers to epilepsies in patients with at least
one CCM and with evidence of a seizure-onset zone in the
immediate vicinity of the CCM. Example: A patient with left hand
tonic–clonic seizures and a right M1 hand area CCM.
Probable CRE: This is defined as epilepsy in a patient with at least
one CCM and with evidence that the epilepsy is focal and arises
from the same hemisphere as the CCM but not necessarily in its
immediate vicinity. At the same time, there is no evidence of other
causes for the epilepsy. Example: A patient with a left occipital lobe
CCM and a history of a right versive seizure indicating a left
hemisphere seizure onset.
Cavernomas unrelated to epilepsy: This is defined as epilepsy in
a patient with at least one CCM with evidence that the CCM and the
epilepsy are not causally related. Example: A patient with bilateral
myoclonic seizures after awakening, generalized 3–4 Hz spike and
wave complexes, a photoparoxysmal response on
electroencephalography (EEG) (suggestive of juvenile myoclonic
epilepsy [JME]), and a right temporal lobe CCM.
Rosenow et al, Cavernoma related epilepsy, ILAE task force, Epilepsia 2013
Risk factors for seizures in people with
cavernoma
Established risk factors
 Cavernomas in the grey matter of the brain
 Cavernomas in the mesial temporal region of the brain
Uncertain risk factors
 Number of cavernomas
 Size of cavernoma
 Presence or absence of hemosiderin rim around the
cavernoma
Rosenow et al, Cavernoma related epilepsy, ILAE task force, Epilepsia 2013
Prognosis for epileptic seizures in
people with cavernoma
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In people with an incidental cavernoma and no history
of seizures, the risk of seizures is 4% over 5 years.
Prophylactic treatment is therefore not necessary.
In people with cavernoma related seizures, the risk of
further seizures without treatment is 94% over 5 years.
=> Treatment is recommended after the first seizure.
50-60% of people with cavernoma – related epilepsy
become seizure free on medications.
60-80% of people with cavernoma become seizure free
after epilepsy surgery for cavernoma over 2-5 years.
Rosenow et al, Cavernoma related epilepsy, ILAE task force, Epilepsia 2013
Acknowledgements
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Colleagues in Neurology, Neurosurgery,
Neurophysiology and Video telemetry unit at NHNN
Patients in the videos who consented to show their
seizures during lectures