Epilepsy recent classification and definitions, dr. amit vatkar, pedaitric neurologist

Dr. Amit VatkarMBBS, DCH, DNB Pediatrics

Fellow in Pediatric Neurology, MumbaiTrained in Neurophysiology & Epilepsy, USA

Contact No. : +91-8767844488Email: [email protected]

EpilepsyRecent Classification

Definitions

Definitions

• Seizure– An episode of altered behavior or awareness

– Associated with too much excitation of a population of nerve cells (neurons)

• Epilepsy– The tendency to have recurrent, unprovoked seizures

(brain makes seizures happen)

• Acute symptomatic (provoked) seizure– A seizure occurring in the setting of some systemic

provoking factor (normal brain, body makes seizures happen)

Iowa Comprehensive Epilepsy Program

ILAE Definition

Most Recent ILAE Definition April 2014: (1) At least two unprovoked (or reflex) seizures

occurring >24 h apart(2) One unprovoked (or reflex) seizure and a

probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years

(3) Diagnosis of an epilepsy syndrome

Epilepsy Syndrome

• Epilepsy Syndrome - ILAE - “a complex of signs and symptoms that define a unique epileptic condition.”

• Epilepsy syndromes denote specific constellations of clinical seizure type(s),

– EEG findings, other characteristic clinical features such as

– age at onset

– course of epilepsy

– associated neurologic and neuropsychological findings, and underlying pathophysiologic or genetic mechanisms.

Definitions

• Acute repetitive seizures (“cluster”)– A period of increased severity or frequency of seizures

in an epilepsy patient

• Status epilepticus– A single prolonged seizure (> 5-10 min)– Repeated seizures without recovery to baseline

• SUDEP– Sudden unexpected death in epilepsy patients

• 1-6 per 1000 patients per year• Probably under recognized, under reported• Needs further study

Definitions

• Drug resistant epilepsy

– Failure of at least TWO seizure medications to completely control seizures

• Appropriately chosen for seizure type

• Taken as prescribed

• Well tolerated (not failed due to side effects)

Drug Resistant Epilepsy

• 470 patients with previously untreated epilepsy

– Seizure-free to 1st medication 47%

– Seizure-free to 2nd medication 13%

– Seizure-free to 3rd medication or beyond 4%

• 36% of epilepsy patients are drug resistant!

• The new generation of medications are generally safer

(fewer side effects), but are not significantly more

effective.

Kwan P, Brodie M. NEJM 2000; 342(5)

Generalized - reconceptualized

For seizures– originating at some point

within

BUT– Rapidly engaging,

bilaterally distributed networks

– Cortical and subcorticalstructures

– Not necessarily include the entire cortex.

Focal reconceptualized

• For seizures:

– originating within networks

– limited to one hemisphere.

Genetic

• Concept: the epilepsy is the direct result of a known or inferred genetic defect(s). Seizures are the core symptom of the disorder.

• Evidence: Specific molecular genetic studies (well replicated) or evidence from appropriately designed family studies.

• Genetic does not exclude the possibility of environmental factors contributing

www.ilae.org

Structural-Metabolic

• Concept:

– distinct other structural

– metabolic condition or disease present.

– eg. Tuberous sclerosis

• Evidence: Must have demonstrated a substantially increased risk of developing epilepsy in association with the condition.

Elements of a seizure

• Subjective experience of the patient

• Motor and behavioural manifestations

• Awareness, language and cognition

• Autonomic manifestations

• May coexist in any combination, even in brief seizures, and are a function of how well the patient is assessed during the seizure

Describing Seizure

• Define the nature of the disease (epileptic paroxysmal episode = epilepsy)

• DIMENSION #1: define the location of the disease (location of the epileptogenic zone)

• DIMENSION #2: define the cause of the disease (etiology)

• DIMENSION #3: define the main symptomatology(ictal symptomatology, frequency and “triggering factors” of seizures or status epilepticus)

• DIMENSION #4: define related medical conditions

a. Myoclonic seizures - short muscle contractions lasting <400 ms.b. Tonic seizures - sustained muscle contractions, usually lasting >3 s

“positioning.” c. Epileptic spasms - muscle contractions of variable duration which affect

predominantly axial muscles.vary from a short myoclonic jerk to a sustained tonic postur-ing. “salaam” posture.

d. Clonic seizures - series of myoclonic contractions that regularly recur at a rate

e. Tonic-clonic seizures - initial tonic posturing of all limbs. The sustained muscle contractions that determined the tonic phase then tend to slow, evolving into a clonic phase with contractions of progressively decreasing frequency until the con-tractions disappear completely

f. Versive seizures - conjugate eye movement to one side or moves the head, and occasionally the whole body, to one side.

• Hypomotor seizures are characterized by immobility or a marked decrease in motilityin newborns, infants, or in severely mentally retarded patients in whom the cause of immobility (alterations of consciousness, inability to move, distraction by an aura, etc) can not be determined.

• Akinetic seizures during which the patient is conscious but he is unable to move (i.e. he tries to move but cannot). The muscle tone is not affected. Only patients in whom consciousness is preserved during the seizures can have akinetic seizures because they .can be tested or tell about the “akinetic” symptoms.

Dr. Amit VatkarPediatric Neurologist, Navi Mumbai

MBBS, DNB

Email: [email protected] No.: +91-8767844488

Visit us at: http://pediatricneurology.in/

THANK YOU !