Liga Chilena contra la Epilepsia - Classification of Status … · 2017. 8. 16. · Axis 1 =...

Classification of Status

Epilepticus:

A New Proposal Dan Lowenstein, M.D.

University of California, San Francisco

for the ILAE Taskforce for Classification of

Status Epilepticus: Eugen Trinka, Hannah Cock,

Dale Hesdorffer, Andrea O. Rossetti, Ingrid E

Scheffer, Shlomo Shinnar, Simon Shorvon

Definition of Status Epilepticus

„a seizure that persists for a sufficient length of time or is repeated frequently enough that recovery between attacks does not occur“ ILAE 19811

1 Commission on Classification ILAE, Epilepsia 1981; 2 Engel et al. Epilepsia 2006; 3 Theodore et al. 1994; 4 Lowenstein et al. 1999; 5: Dobesberger et al. Epilepsy & Behavior 04/2015; DOI:10.1016/j.yebeh.2015.03.023;

Majority of GTCS < 2-3 min3,5

Operational definition: 5min4

Traditional classification:

• There were as many types of status as there were types of epileptic seizures (draft 1962; final 1967)

• SE classification mirrored the seizure classification (1964, final 1970)

…“Mechanistically, SE represents the failure of the natural homeostatic seizure-suppressing mechanisms for seizure termination.”…2

Definition of Non Convulsive Status Epilepticus

1 Commission on Classification ILAE, Epilepsia 1981; 2 Drislane et al. Epilepsy Behav. 2000;1(5):301-14; 3 Trinka Lowenstein et al. Epilepsia 2015 (R1 submitted)

NCSE Definition 30 min

Traditional classification:

• There were as many types of status as there were types of epileptic seizures (draft 1962; final 1967)

• SE classification mirrored the seizure classification (draft 1964, final 1970)

…“Mechanistically, SE represents the failure of the natural homeostatic seizure-suppressing mechanisms for seizure termination.”…2

„enduring epileptic condition with reduced/altered consciousness, behavioural and vegetative abnormalities or merely subjective symptoms, without major convulsive movements“1

NCSE Definition 10 min3

Definition of Status Epilepticus

Status epilepticus is the failure of the mechanisms responsible for seizure termination or the initiation of mechanisms, which lead to abnormally prolonged seizures (t1) that might have long-term consequences (t2), including neuronal death, neuronal injury, and alteration of neuronal networks, depending on the type and duration of seizures

Conceptual (=“mechanistic”) definition 2 Operational dimensions: length of seizure (t1) time (t2) to long term consequences

Trinka, Lowenstein et al. ILAE Task Force on Classification of SE, Epilepsia 2015 (R1 submitted)

Type of SE

Time (t1), when a seizure is likely to be prolonged leading to continuous

seizure activity

Time (t2), beyond which long term consequences

are increasingly likely (including neunonal injury, neuronal

death, alteration of neuronal networks and functional deficits)

Tonic clonic SE 5 minutes <30 minutes

Focal NCSE with impaired consciousness

10 minutes* 30-60 minutes*

Absence status epilepticus

2 minutes** unknown*

Time point 2 determines the time at which status should be controlled to

prevent long term consequences

Time point 1 determines the earliest time when treatment should be

considered or started

Implications to treatment

* Best available evidence, but insufficient data to give a definite timepoint

1: Dobesberger and Ristic et al. Epilepsy & Behavior 04/2015; DOI:10.1016/j.yebeh.2015.03.023

How long do seizures last in patients with epilepsy?1


Mean seizure duration +4 SD



Cumulative 99% seizure duration of focal complex

seizures


Axis 1 Semiology

Axis 2 EEG

Axis 3 Etiology

Axis 4 Age

Clinical Classification of SE Types

Classification of SE



Axis 1 = semiology Classification of SE types

A) With prominent motor symptoms

B) Without prominent motor symptoms (i.e. NCSE)


Along two taxonomic criteria: motor symptoms and impairment of consciousness



1. Convulsive SE (syn.: tonic clonic SE, CSE)

2. Myoclonic SE (prominent epileptic myoclonic jerks)

3. Focal motor (including EPC)

4. Tonic SE

5. Hyperkinetic SE







1. Convulsive SE (syn.: tonic clonic SE, CSE)

2. Myoclonic SE (prominent epileptic myoclonic jerks)

3. Focal motor (including EPC)

4. Tonic SE

5. Hyperkinetic SE


1. NCSE with coma

2. NCSE without coma

a. Generalised

b. Focal





A) With prominent motor symptoms 1. Convulsive SE (syn.: tonic clonic SE, CSE)

a) Generalised convulsive

b) Focal onset evolving into bilateral convulsive SE

c) Unknown whether focal or generalised

2. Myoclonic SE

a) With coma

b) Without coma

3. Focal motor

a) Repeated focal motor seizures (Jacksonian)

b) Epilepsia Partialis Continua (EPC)

c) Adversive status

d) Oculoclonic status

e) Ictal paresis

4. Tonic SE

5. Hyperkinetic SE






1. NCSE with coma

2. NCSE without coma

a) Generalised

a) Typical absence status

b) Atypical absence status

c) Myoclonic absence status

b) Focal

a) Without impaired consciousness (Aura continua)

(With autonomic*, sensory symptoms, visual, olfactory, gustatory, emotional, auditory symptoms

b) Aphasic SE

c) With impaired consciousness (dyscognitive SE)

c) Unknown

a) Autonomic SE*





Currently indeterminate conditions**

1. Epileptic encephalopathies

2. Coma with epileptiform EEG patterns

3. Behavioural disturbance (e.g psychosis) in patients with epilepsy

4. Acute confusional states (e.g delirium) with epileptiform EEG patterns



**= Boundary syndromes


Axis 1 Semiology

Axis 2 EEG

Axis 3 Etiology

Axis 4 Age

Ictal EEG correlates



Trinka, Lowenstein et al. ILAE Task Force on Classification of SE, Epilepsia 2015 (R1 submitted) Trinka, Lowenstein et al. ILAE Task Force on Classification of SE, Epilepsia 2015 (R1 submitted)

• EDs > 2.5 Hz, or • EDs ≤ 2.5 Hz or rhythmic delta/theta activity (>0.5 Hz) AND one

of the following: EEG and clinical improvement after IV AED*, or Subtle clinical ictal phenomena, or Typical spatiotemporal evolution**

*If EEG improvement without clinical improvement, or if fluctuation without definite evolution, this should be considered possible NCSE. **Incrementing onset (increase in voltage and change in frequency), or evolution in pattern (change in frequency >1 Hz or change in location), or decrementing termination (voltage or frequency).

Beniczky et al. Epilepsia. 2013;54 Suppl 6:28-9.

Proposed EEG Criteria for NCSE – For patients without epileptic encephalopathies:


Beniczky et al. Epilepsia. 2013;54 Suppl 6:28-9.

Proposed EEG Criteria for NCSE – For patients without epileptic encephalopathies:

• Increase in prominence or frequency when compared to baseline with observable change in clinical state

• Improvement of clinical and EEG* features with IV AEDs

*If EEG improvement without clinical improvement, or if fluctuation without definite evolution, this should be considered possible NCSE.


clinical suspicion of NCSE (without preexisting epileptic encephalopathy)

- transition from praemorbid to current ill state within minutes to hours - patient did not improve significantly in last minutes to hours, apart from waxing and waning - no information from brain imaging sufficiently explaining EEG-pattern (e.g. brain stem haemorrhage) - no metabolic/ toxicological derangement sufficiently explaining EEG-pattern (e.g. acute renal or liver failure)

clinical data

ED...epileptiform discharge RA...rhythmic activity clin. ...clinical IV AED...intravenous antiepileptic drug NCSE...non-convulsive status epilepticus

frequency of epileptiform discharges > 2.5 Hz

“NCSE”

EEG data

EEG data AND clinical data appropriate

(1)

typical ictal spatio-

temporal evolution

ED RA >0.5Hz

(2a) (2b)

subtle ictal clinical

phenomena

ED RA >0.5Hz

(3a) (3b)

careful consideration of clinical situation

appropriate AED treatment

EEG – clin. –

document improvement*

4a) EDs ≤2.5Hz with fluctuation, or 4b) RA >0.5 Hz with fluctuation, or 4c) RA >0.5 Hz wihtout fluctuation

EEG + clin. –

EEG – clin. +

EEG + clin. +

*important note regarding improvement to IV AED: for clinical practice: all four constellations qualify for NCSE. for research projects: patient qualifies for NCSE if EEG and/or clinical improvement

Trinka and Leitinger; Epilepsy and Behavior 2015 (in press) Trinka, Lowenstein et al. ILAE Task Force on Classification of SE, Epilepsia 2015 (R1 submitted)

Typical spatiotemporal

evolution*

Evolution in pattern:

Change in frequency > 1 Hz

Change in location

Decrementing termination

Voltage Frequency

*In order to qualify as present, a single frequency or location must persist at least 3 cycles, and at least two consecutive changes in the same direction has to be present in order to qualify as evolution. If changes are present but do not qualify as evolution, it is considered to be fluctuation, and it is scored as possible NCSE.

Incrementing onset:

EEG

Voltage Frequency

Typical spatiotemporal evolution

1: Leitinger et al. Epilepsy and Behavior 2015 (in press)


Axis 1 Semiology

Axis 2 EEG

Axis 3 Etiology

Axis 4 Age

Categories of etiology




1. Known (syn. symptomatic)

a) Acute (e.g.: stroke, intoxication, malaria, encephalitis, etc.)

b) Remote (e.g. posttraumatic, postencephalitic, poststroke, etc.)

c) Progressive (e.g. glioblastoma, Lafora´s disease and other PMEs)

d) SE in defined electroclinical syndromes

2. Unknown (syn. cryptogenic)1

1: Synonymous translations germ.: ungeklärt; french: xxx, span.: yyy; ital.: zzz, russ.: aaa ...)

Classification of SE: Axis 3 Etiology


Axis 1 Semiology

Axis 2 EEG

Axis 3 Etiology

Axis 4 Age

Age groups




1. SE occurring in the neonatal and infantile epilepsy syndromes

a) Tonic Status in Ohtahara Syndrome

b) Myoclonic status in Dravet´s syndrome

c) Febrile SE

i. With bilateral motor symptoms

ii. With unilateral motor symptoms

2. SE occurring predominately in childhood and adolescence

a) NCSE in Early-onset Childhood Occipital Epilepsy (Panayiotopoulos syndrome)

b) NCSE in childhood epileptic encephalopathies (e.g., myoclonic-astatic epilepsy, Ring chromosome 20, Angelman syndrome see appendix)

c) Tonic status in Lennox Gastaut Syndrome

d) Myoclonic Status in Down Syndrome

e) Absence Status in Juvenile Absence Epilepsy

f) Myoclonic Status in Juvenile Myoclonic Epilepsy

g) Myoclonic Status in Progressive Myoclonus Epilepsies

h) FIRES

3. SE occurring only in adults and elderly

a) De Novo (or relapsing) absence status of later life

Age Related Disorders and Electroclinical Syndromes with SE


www. Status-epilepticus.net

Online audit of the treatments being used in status epilepticus around the world and their outcome Short and easy to complete Questionnaires !! PLEASE REGISTER AND TAKE PART !!

Liga Chilena contra la Epilepsia - Classification of Status … · 2017. 8. 16. · Axis 1 =...

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