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Epilepsy

István FEKETE
2017.
Epilepsy

Epileptic seizures:
an abnormal and excessive discharge
of brain neurons involving
hypersynchrony accompanied by
some behavioral change.
Definition of epilepsy
• Epilepsy: – two or more seizures
– occur in attacks
– the behavioural changes
are usually stereotyped
– epileptiform abnormalities
on EEG
• Acute and recurrent seizures, status
epilepticus, occasional convulsion
Epidemiology of epilepsy

1. The prevalence within a particular


population: 0,3-0,6-1,0%. In children: >1%
2. Man/woman: 1,1-1,7
3. The incidence: 0,4-0,7-1,0‰ /year
4. The chances of having at least one
seizure during a lifetime: 8-10%
New classification of epilepsy (ILAE, 2017)

Epilepsia
8 MAR 2017 DOI: 10.1111/epi.13709
Classification of epileptic seizures

1. Partial (focal, local) seizures


1.1. Simple partial seizures
• with motor signs
• with somatosensory or special
sensory symptoms
• with autonomic symptoms
• with psychic symptoms
Classification of epileptic seizures

1.2. Complex partial seizures


• Simple partial onset followed by
impairment of consciousness
- only with impairment of
consciousness
- with automatism
• With impairment of consciousness at
onset
Classification of epileptic seizures

1.3. Partial seizures evolving to secondary


generalized seizures
• Simple partial seizures 
• Complex partial seizures 
• Simple partial seizures  Complex
partial seizures 
Classification of epileptic seizures

2. Generalized seizures
Absence seizures (typical and atypical)
Myoclonic seizures
Clonic seizures
Tonic seizures
Tonic-clonic seizures
Atonic (astatic) seizures
3. Unclassified epileptic seizures
Situation-related seizures

• Febrile convulsions
• Seizures occurring only with an acute
or toxic event, due to factors such as
alcohol, drugs, eclampsia, nonketotic
hyperglycaemia
Pathophysiology and
neurobiochemistry of epilepsy

 The role of ION channels: Na+, Ca2+, Cl- and


GABA receptor system and the glutamate
(NMDA) receptors:

some antiepileptics have effect on the closing


and the opening mechanism of the Na-
channel, prolonging the refractor time.
Pathophysiology and
neurobiochemistry of epilepsy

 GABA : The most important inhibitor


neurotransmitter of the brain on the GABA
A-Benzodiazepine-Phenobarbital- receptor
complex, controlling the opening and closing
mechanism of the Chloride-channel.
 GABA B-receptor connects to the K+-ion
channels and influences the NMDA mediated
excitability.
Pathophysiology and
neurobiochemistry of epilepsy

 GABA hypofunction causes seizure. The


GABA agonists can protect (valproate,
vigabatrin, tiagabine).
Developmental cortical
malformations with epilepsy

• Pachygyria, heterotopia (nodular),


lissencephaly
• polymicrogyria, shizencephaly, cortical
dysplasia (gliosis),microdysgenesis
Developmental cortical
malformations with epilepsy

2. Malformations due to abnormal neuronal


and glial proliferation:
microencephaly, non-neoplastic (tuberous
sclerosis), neoplastic (ganglioglioma)
3. Neurocutaneous disorders: Sturge-Weber
syndrome, neurofibromatosis
4. Others: subarachnoid cyst, porencephaly
Etiologies of epilepsy
genetic factors
• The concordance rate in monozygotic
twins: >70%, in dizygotic twins: 15%

• Occurrence of epilepsy in relatives


In idiopathic epilepsy: 1.3 - 8%
In symptomatic epilepsy: 0.5 - 5%
In normal population: 0.5%
Etiologies of epilepsy
genetic factors
• Known genes associated with epilepsy:
6p, 16p, 1q, 8q, 10q, 20q, 21q,
mitochondrial DNA disorder: MERRF

• Epilepsy may occur in 141 genetically


determined neurological diseases
Diagnosis of epilepsy

• Seizures are diagnosed primary by the


history (anamnesis, heteroanamnesis)
• Laboratory tests, ECG (Holter), Doppler
examination of supraaortic arteries,
examination of CSF, toxicology
Diagnosis of epilepsy

EEG: routine, sleep


deprivation, photic
stimulation, HV, digital
analysis of power
spectrum, sphenoidal
recording, Holter EEG,
split screen EEG
(video+EEG)
LORETA (A low resolution electromagnetic tomography)
Diagnosis of epilepsy

• Neuroimaging
MRI, (CT scan), SPECT, PET
• Genetic examination
• Psychological, psychiatric examination
Nonepileptic paroxysmal disorders

 Cardiovascular
Syncope
Breath-holding spells (cyanotic, noncyanotic)
Mitral valve prolapse

 Cerebrovascular (transient ischemic attack)

 Migraine
Nonepileptic paroxysmal disorders

 Movement disorder

Tics, Tourette’s syndrome


Myoclonus
Chorea and paroxysmal choreoathetosis
Nonepileptic paroxysmal disorders

 Sleep disorders
Narcolepsy
Sleep terrors and somnambulism
Rapid eye movement (REM) sleep disorder
Benign sleep jerks
Periodic leg movements (nocturnal
myoclonus)
Nonepileptic paroxysmal disorders
 Metabolic-toxic (e.g., pheochromocytoma, drug
ingestion)

 Gastrointestinal (vomitus, diarrhoea: disturbance of


ion levels

 Psychiatric
Psychogenic seizures
Somatization and dissociative disorders
Panic disorder
Intermittent explosive disorder
Malingering
Therapy of epilepsy
Sixty-seventy percent of patients are seizure
free on AE therapy and another 25 percent’s
are decreased

Phytoterápia
Status epilepticus

 After 5 min, after 10 min, should be stopped in an


hour
 Life threatening event
 Medication:
-first choice BDZ: lorazepam, diazepam, clonazepam
-IV. valproate, levetiracetam, lacosamide
-IV. phenytoin
-IV. propofol, midazolam
Surgical treatment of epilepsy

Ten percent to 15% of patients with refractory


seizures may be surgical candidates.
The aim of surgery is
 to remove the focus of origin of the seizures
 or to prevent spread of the seizure discharge
 DBS (deep brain stimulation)
VNS
Presurgical evaluation

 Patient selection
 Clinical evaluation
 EEG (interictal recordings, ictal recordings to
document seizure onset, EEG/video telemetry
monitoring is required, intracranial EEG recording:
epidural electrodes subdural strip electrodes, IC
electrodes)
 Neuroimiging (MRI, SPECT, PET, functional MRI)
 Neuropsychological Psychiatric Assessment
Subdural records
Surgical procedures

 Lesionectomy (dysplasia, ganglioma, low grade


gliomas, vascular anomalies, neuronal
migration, in presence of mesial-temporal
sclerosis)

 Selective amygdalohippocampectomy (focal


mesio-temporal sclerosis)
Surgical procedures

 Anterior temporal lobectomy (amygdala,


anterior hippocampus, anterior temporal
neocortex)
 Hemispherectomy (Sturge-Weber syndrome,
Lennox-Gastaut Syndrome)
 Corpus callosotomy (anterior two-thirds or
complete; atonic seizures, Lennox-Gastaut
syndromre)
 Multiple subpial transection
Temporal lobectomy

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