The many faces of seizures in epilepsy in people with cavernomas International Cavernoma Alliance UK Forum London, 13 June 2015 Dr Tim Wehner National.

The many faces of seizures in epilepsy in people with

cavernomas

International Cavernoma Alliance UK Forum

London, 13 June 2015

Dr Tim WehnerNational Hospital for Neurology & Neurosurgery

University College LondonUnited Kingdom

Take home messages

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?

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

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

Generalised- absence - atonic- myoclonic- tonic- tonic-clonic

Focal / partial-without alteration of conciousness (“simple partial”)-with alteration of conciousness (“complex partial”)

Classification of Seizures & Epilepsies

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 factorsCavernomas in the grey matter of the brainCavernomas in the mesial temporal region of the brain

Uncertain risk factorsNumber of cavernomasSize of cavernomaPresence 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

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

• Colleagues in Neurology, Neurosurgery, Neurophysiology and Video telemetry unit at NHNN

• Patients in the videos who consented to show their seizures during lectures