Seizures and epilepsy

Epilepsy is a chronic cerebral disorder characterized by recurrent

seizures.

Seizures are paroxistic clinical manifestations taking usually seconds

to minutes and having a variable content: motor, somato-sensory,
psychiatric, cognitive, or autonomic. Usually we have to deal with
positive phenomena (clonias, visual hallucinations, ictal speech etc.)
but some negative symptoms might occur as well (aphasia,
hemianopia, ictal motor deficit).

Seizures occuring during an acut neurological disorder are strictly

related to the acute damage of cortical neurons as a consequence of
pathological process. Seizures are nothing more than a clinical sign (as
Babinski sign is). This is not epilepsy!!!!!

For instance acute encephalitis could be manifested by fever, altered mental status
and seizures.
Seizures in this case are a clinical hallmark of the inflammatory process.
If seizures restart at distance from acute episode, after healing of inflammatory
lesions we have to deal this time with postinfectious epilepsy a chronic disorder.
 Physiopathological base for seizures is a process of hyperexcitability in a
group of cortical neurons that we call seizure focus.
As a consequence, seizures are the clinical expression of a lesion that
affects cortical neurons.
Seizure semiology is highly dependant on the location of the seizure focus .
We call seizures originating in restricted parts of the cerebral cortex - focal
(partial) seizures. Focal seizures are encountered in localization related
epilepsies.
On the contrary when seizures appear to begin bilaterally, neuronal
hyperexcitability or loss of inhibitory mechanisms affects more widespread
the cortex of cerebral hemispheres and clinical expression is a generalized
seizure (with loss of counsciousness from the very first moment). Primary
generalized seizures occurs in idiopathic epilepsy and have a genetic basis
or could be provoked by toxic or metabolic conditions
Paraclinical diagnosis of epilepsy is based on two critical aspects:
Prove the epileptogenic process electroencephalography
Search of a structural lesion that could be the cause MRI and
functional imagistic studies
 Seizures classification
Generalized seizures abrupt initial loss of consciousness
Generalized tonic clonic seizures
Absence seizures
Myoclonic seizures
Atonic seizures (one of the most severe type, onset only during
childhood, hallmark of intractable, deteriorating epilepsies like
Lennox Gastaut syndrome)

Focal (partial) seizures consciousness retained, patient able to

describe the clinical content of his seizures
Simple focal seizures consciouness retained during the whole episode
Complex focal seizure complex semiology, impairment of consciousness at
some point during the episode. Seizure focus located in a cortical zone
responsible for highly complex, integrative functions usually temporal or
frontal lobes or propagation of seizures into such complex cortical territories
(hippocampal structures, orbitofrontal cortex etc.)
Simple or complex focal seizures wirh secondary tonic-clonic generalization.
 Trained doctors or nurses are generally able to adequately interact with a patient
during a seizure and interrogate after, to reveal all the reliable clinical aspects of a
seizure.

Impairment of consciousness is considered

in a nonresponsive patient and unable to
perform tasks during seizures also
amnestic for the episode (total or partial)
when patient is questioned afterward.
 Generalized tonic-clonic seizure (epileptic grand-mal)

Sudden loss of consciousness

Patient is falling and usually is screaming (laringian muscles contraction)
Apnea and cyanosis occur as consequence
Tonic phase (~ 40 sec) generalized tonic contraction initially in flexion,
then extension,
Eyes wide open,
Autonomic signs: hypersalivation and drooling, sweating, tachycardia,
rising blood pressure
Clonic phase (~20 sec) intermittent relaxation from the tonic contraction
status
Urinary incontinence occurs and tongue is bitten on its lateral aspect
postictal coma nonlateralized coma status with generalized loss of
muscles tone, midriasis and bilateral Babinski sign
Progressively patient becames reoriented but has no recollection of the
ictal episode
 Absence seizure (petit mal)
Abrupt loss of contact
Motor arrest but patient does not fall
Some activities might continue (like driving
a car or manipulating an object etc..)
Fine myoclonic movements occur
rhythmical (3Hz) on the eyelids or involves
other muscles as well
Might be very frequent pyknolepsia
Rarely have their onset under 4 years of
age or after puberty
 Simple partial seizures
Consciousness retained
Motor, sensory paresthetic, visual,
vertiginous, olfactory, aphasic
Patient able to describe in detail what
happens during the episode
 Complex partial seizures
Aura initial event in the seizure frequently temporal
epilepsy consist in rising epigastric sensation or fear or
experiential sensations (dj vu, deja vecu)
Period of altered behavior and responsiveness for which
the patient is found later to be amnesic (impairment of
consciousness)
The motor component of a seizure occurs later during
the seizure and takes form of so called automatisms
oroalimentary (chewing, swallowing), gestual, verbal.
 Each type of epilepsy presents with its own
unique combination of seizure type, typical age
of onset, EEG findings, treatment, and prognosis.
The most widespread classification of the
epilepsies devides epilepsy syndromes bylocation
or distribution of seizures (as revealed by the
appearance of the seizures and by EEG) and by
cause.
Syndromes are divided into
localization-related epilepsies
generalized epilepsies
epilepsies of unknown localization.
 Localization related epilepsies
Presence of lesional zone in the brain (frontal,
temporal, parietal , occipital) that has been acquired at
some point in life
from the first weeks of gestation (intrauterine
developmental lesions like focal cortical dysplasia,
disembrioplastic tumors etc)
to older ages: vascular, traumatic, inflammatory
Early lesions have a great impact on cerebral
development and could be associated with cognitive
delay
 Generalized epilepsies
Idiopathic - Genetic disorders, age dependant
penetrance
Onset typically before 18 y of age
Non - lesional MRI
Generalized discharges on EEG - very typical EEG
patterns that confirms the diagnosis
Very sensitive to sleep deprivation
Most representative:
Childhood absence epilepsy onset around 6-8y of
age, good prognosis,
Juvenile myoclonic epilepsy onset in adolescence,
life long condition, myoclonic seizures, generalized
tonic clonic seizures, absence seizures
 Diagnosis
Anamnesis precise interrogation about the
seizure semiology asking the patient, relatives,
witnesses
Standard EEG recording scalp electrodes, 30
min recording, patient awaken, relaxed, 3
hiperventilation, eyes open, eyes shut.
If no abnormalities activation procedures like
sleep deprivation, sleep recordings (especially
in children)
 Electroencephalography
Normal background patient awake, eyes
shut posterior alpha rhythm, well
modulated
 interictal epileptiform abnormalities

Interictal sharp waves

 Right anterior temporal spikes common
abnormality in temporal epilepsy
Generalized polispike and waves discharges
encountered in juvenile myoclonic epilepsy
 Imaging studies
Emergency condition first seizure or
cluster of seizures - CT scan is likely to
reveal the most striking lesions
responsible for seizures:
Tumors
Stroke
Subdural hematoma
Traumatic scars
Cerebral venous thrombosis etc..
 Imaging studies
Most of the lesions responsible for focal
epilepsies are very well depicted by MRI
1,5 T in T1 weighted images, T2 and FLAIR
Metabolic studies are used in MRI negative
cases:
PET using fluorodeoxiglucose (FDG) is used
interictal (interval between seizures) to reveal
the hypometabolic area associated to the
seizure focus
SPECT is used to reveal ictal (during seizure)
hyperperfusion associated to the seizure focus
 Imaging studies
Right hippocampal sclerosis hypersignal right
hippocampal structures
in a patient with
mesiotemporal epilepsy
 Right temporoparietal
Polymicrogiria malformation
of cortical development
 Acute seizures
Treatment to stop seizing and finding
cause is an emergency
Depending of the clinical pattern:
Generalized: toxic, metabolic screening, CSF
analysis (LP)
Focal: valuable clinical sign for a focal
cortical lesion generally contralateral to the
limbs with motor symptoms

Imiging studies are prevalent to electroencephalography!!!

Etiology of seizures and epilepsy
Onset 0-3 y of age
Injury at birth: anoxia, vascular
Traumatic
Postinfectious
Vascular
Metabolic
Toxic
Idiopathic
 Onset 3-18 y of age
Idiopathic genetic
Traumatic
Infectious
Vascular
Tumors
Metabolic
 Onset 18-40 y of age
Traumatic
Primary cerebral tumors
Infectious
Metabolic
Toxic
Vascular
Rarely idiopathic
 Onset over 40y
Vascular
Metastatic tumors
Degenerative
Infectious
Traumatic
Metabolic
Toxic
 Treatment
Acute treatment of a seizure:
Check vital functions and start support if
needed
Iv. Benzodiazepine with short T1/2
(diazepam, lorazepam)
 Status epilepticus: seizing for more then
30 min.
Generalized tonic-clonic status medical
emergency life threatening condition
Support vitals
O2, iv line
Short half life Benzodiazepine
Iv phenitoin 20mg/kc (caution with cardiac
block)
Iv phenobarbital 20mg/kc
Intubated patient - pentobarbital
 Chronic treatment
Principles:
Seizure freedom
No side effects
Start an AED in monotherapy
If failure switch to an other AED monotherapy
Make the switch slowly, progressive
If failure try a reasonable combination
If failure after 1 year of combination treatment
declare pharmacoresistance and refer to
presurgical evaluation if is a focal epilepsy
 Chronic treatment
Focal epilepsies
First line: carbamazepine
Second line: lamotrigine, levetiracetam,
oxcarbazepine, topiramate
Idiopathic Generalized epilepsies
First line: valproat
Second line: etosuximid (only for absence
seizures), lamotrigine (may aggravate
myoclonic seizures), levetiracetam (may not
be very effective)
 Side effects of AED
CBZ: vetigo, ataxia, sedation, double vision,
hematologic, cardiac, hyponatremia, rash
LTG: rash, allergic - inflammatory reaction,
insomnia
LEV: sedation, agressivity, irritability, affective
reactions
VPA: sedation, hepatic toxicity, ponderal gain,
hair fall, ovarian policystosis???, parkinsonism
OXC: vertigo, ataxia, double vision,
hyponatremia
 Epilepsy surgery
Candidates: patients with focal
pharmacoresistant epilepsy
Target: remove the epileptogenic zone
preserving the eloquent, functional corte
and rend the patient seizure free
The most common intervention with
70-90% chance of healing is for
mesiotemporal epilepsy due to unilateral
hippocampal sclerosis
 Presurgical protocol
Surface video-EEG long term monitoring
with the aim to record habitual seizures and
interpret data anatomo-electro-clinical to
establish a hypothesis
If data obtained are concordant with the
lesion present on MRI and functional
cortex (language areas, motor, visual
cortex) could be safely spared planning
surgery with high chance of seizure freedom
 Discordant data or MRI negative cases
impose implantation of intracerebral
electrodes and further verification of
initial hypothesis.
If safe and beneficial tailored cortical
resection
After surgery, if successful therapy might
be tapered down only after 2 years of
good outcome.