Epilepsy is a symptom of paroxysmal (also continuous) attacks of transient (also ongoing) electrical discharges in the brain's nerve cells. The discharges may occur episodically or continuously.
Epilepsy is the tendency to have epileptic seizures. Usually (not always), at least two unprovoked seizures are required for the diagnosis of epilepsy be be confirmed.
An epileptic seizure is not a disease but a symptom
Epilepsy versus epileptic seizures (brain tumor with epileptic seizures)
In practice, the word "epilepsy" is usually used instead of epileptic seizures
400 B.C. Hippocrates wrote the first book on epilepsy;
When the phlegm (from the brain) runs down through the veins, the patient loses his speech and foams at the mouth, his hands are contracted, the eyes contorted, he becomes insensible, and in some cases, the bowels are emptied (…). The patient kicks with his feet (…). The patient must endure all these symptoms when the cold phlegm flows into the warm blood.
(four humors: blood, phlegm, yellow bile, and black bile)
1859-1906: Modern medical thinking about epilepsy is started.
1904: William Spratling, the first epileptologist
1912: Luminal discovered (Phenobarbital)
1929: Hans Berger, a German psychiatrist, uses the new method and calls it electroencephalography (EEG).
Socrate (470-399 BC)
Julius Caesar (100-44 BC)
Napoleon Bonaparte (1769-1821)
Fyodor Mikhailovich Dostoyevsky (1821–1880)
Vincent van Gogh (1853 – 1890)
Vladimir Lenin (1870-1924)
Prevalence in Sweden is approximately 0.6% (600/100 000). No difference between men and women. Debuts of all ages. Highest incidence in young children and older people (> 65).
14% of the general population has had at least one attack during their life.
Metabolic/endocrinologic: hypocalcemia, hypomagnesemia, hyponatremia, hypernatremia, hypoglycemia, pyridoxin dependent, hypoparathyroidism
Infection: sepsis, meningitis, encephalitis, brain abscess, TORCH
Iatrogenic: ECMO, neurosurgical operation
Drug Withdrawal: barbiturates, benzodiazepines, heroin, methadone, opium
Trauma: bleeding, contusion, epidural hematoma, subdural hematoma, diffuse brain injury
Syndrome: Di George, Osler Weber Rendo,
Neurocutaneous (phakomatosis) syndromes: tuberous sclerosis, Von Rekling Hasen syndrome, Von Hippel Lindau syndrome, Sturge Weber syndrome
Malformation: migration defect: Micro: small heterotopias av neurons. Macro: Lissencephaly, Schizencephaly, cortical dysplasia (Taylors och Icke Taylors type)
Tumor: teratoma, craniopharyngioma, medulloblastoma
Genetic: ion channel derangement (kalium, calcium, and chloride channel rubbing): generalized epilepsy with febrile seizures, autosomal dominant nocturnal frontal lobe epilepsy, benign familial neonatal convulsion, and episodic ataxia type 1 with partial seizures
Cerebrovascular: arteriovenous vascular malformation, thromboembolism
Others: stress, sleep deprivation
Self-induced
Idiopathic: (epilepsy)
1- Hyperexcitability
2- Synchronicity
+ Spreading
Ion channels:
Na-channels, Ca-channels
K-channels, Cl-channels
Neurotransmitters:
Excitatory neurotransmitters open Na-
och Ca-channels
Inhibitory neurotransmitters
open K- and Cl-channels
GABA regulates K- and Cl-channels
Glutamate regulates Na-channels
Focal synchronicity (simple partial, complex partial)
Generalized synchronicity (generalized seizures, generalized absence epilepsy)
Intrahemispheric:
Simple partial epilepsy --> Complex partial epilepsy
Interhemispheric:
Partial epilepsy --> Secondary generalized epilepsy
Epileptic seizures can be expressed in the form of abnormal motor, sensory, behavioral or autonomic functions
Motor (tonic, clonic, tonic-clonic, myoclonic, atonic, Todds paralysis)
Consciousness (absence epilepsy)
Speech disorders (stuttering, aphasia)
Winking
Tremor (cortical tremor, titubation)
Vomiting
Sensory (pain, numbness, auditive and visual perceptional disorders)
Paroxysmal pain attack (headache)
Dizziness, nausea, vertigo
Apnea, bradycardia, tachycardia
Enuresis, encopresis
Sleep disorders (CSWS= continuous spikes and waves during slow sleep, ESES= electrical status epilepticus during slow sleep)
Status epilepticus
Cardiac arrhythmias, ictal and postictal asystole
Temporal lobe epilepsy:
Relatively long seizure duration (>1 min)
Long postictal phase and often postictal amnesia
Insular epilepsy: EEG is usually expected as normal because of operculum acting as a small lid.
Medial temporal lobe epilepsy: (epileptic activity originates from hippocamp/amygdala) anxiety, panic, dizziness, nausea, apnea
Lateral temporal lobe epilepsy: auditive or visual hallucinations/illusions, dream state, and language disturbance (if the dominant hemisphere is affected)
Frontal lobe epilepsy:
Complicated automatism
Short duration (seconds to few minutes)
The postictal phase is relatively short
Often secondary generalization
Half-sided tonic or clonic seizures
Head rotation
Smell hallucinations
Parietal lobe epilepsi:
Relatively unusual
Rarely generalization
Simple sensory seizures (half sided paresthesia or aphasia)
Occipital lobe epilepsy:
Flashes in front of the eyes
Visual hallucinations: micropsia/macropsia
Nausea or vomiting
The postictal headache could be considered a migraine headache!
Epilepsy is a clinical diagnosis being verified by neurophysiological examinations (EEG and MEG)
Completion of the investigation by structural (CT, MRI) and functional neuroradiology (PET, fMRI, SPECT)
Investigation of underlying causes (neurometabolic, neuroendocrinological, and neurogenetic examinations)
EEG: electroencephalography
Electroencephalography is a method for recording electrical activities in the cortex and, to a lesser extent, in subcortical areas of the brain.
This method is used to confirm or exclude epileptic and encephalopathic (dementia, metabolic encephalopathy) changes in the brain.
Continuous EEG can be used for patients with lowered consciousness (skull trauma, severe infections/sepsis, circulatory shock, etc).
When must EEG be done?
Evaluation of answer?
Sensitivity: 25-56%
Specificity: 78-98%
These percentages are dependent on different factors:
Who does it? How is it done? Who judges the result?
How to increase the sensitivity and specificity of EEG?
Stimulation (intermittent photic or phonic stimulation)
Registration of deeper area of the brain via sphenoidal electrode
Sleep deprivation and hyperventilation increase the sensitivity of EEG
Video-EEG: video electroencephalography
Video EEG (VEEG) is a monitoring system that correlates the clinical symptoms to neurophysiological changes.
Intracranial EEG: intracranial electroencephalography
Intracranial EEG (I-EEG) or subdural-EEG (SD-EEG)
Deep electrodes (hypothalamus, amygdala)
Used in decision making for epilepsy surgery
MEG: magnetoencephalography
Magnetoencephalography is an imaging technique used to measure magnetic fields produced by electrical activity in the brain.
CT: computed tomography
Computed tomography provides better information about skeletal changes (f ex calcification in the basal ganglia).
It is not a promising imaging method for visualizing the posterior fossa.
MRI: magnetic resonance imaging
Magnetic resonance imaging/ tomography provides higher sensitivity and specificity than CT (regarding changes in brain tissue).
MRI is a better imaging method for visualizing the posterior fossa.
It is not a promising imaging method for visualizing skeletal changes or calcification.
PET: positron emissions tomografi
Based on the increased metabolic profile of examined tissue
SPECT: single-photon emission computed tomography
Uses gamma-ray
fMRI: functional MRI
Based on diffusion and perfusion
Focality (kliniskt eller EEG-mässigt)
Coexisting symptoms (speech disorders, developmental disturbances, motor difficulties, visual problems, etc.)
Therapy resistant epilepsy
Neurosurgery is indicated
Pharmacological therapy: AED, neuroleptics, medicines reducing high intracranial pressure, calcium channel blocker, IVIg (intravenous immunoglobulin), melatonin, mm.
Non-pharmacological therapy: neurosurgery, ketogenic diet, VNS, TNS and EBT
AED (antiepileptic drugs):
Na channel blocker: carbamazepine, oxcarbazepine, phenytoin, lamotrigine
Ca channel blocker: valproic acid, ethosoximide,
GABA-receptor agonist: clonazepam, clobazam, phenobarbital, valproic acid, bensodiazepines
Glutamate blocker: topiramate, felbamate
Complex mechanism: levetiracetam
Hormon: corticosteroider, ACTH
Immunotherapy: IVIg (KIOVIG, Oktagam)
Medicines reducing high intracranial pressure: osmotic diuretitcs, diamox (acetazolamide)
Hypnotics (sleep medicines): melatonin
Calcium channel blocker: verapamil, nifedipine
Anesthesia (status epilepticus)
Epilepsy surgery:
Corpus callosotomy
Hemispherotomy (peri-insular hemispherotomy)
Partial hemispherectomy
Extirpation of cortical dysplasia, CNS tumor etc
Embolization or surgical resection of intracranial vascular malformations
VNS: vagus nerve stimulation
TNS: trigeminus nerve stimulation
Ketogenic diet: high fat and low carbohydrate content
Unknown mechanism (PH changes? Keton hypothesis? Stabilising of ion channels or synapses?)
Side effects of the ketogenic diet: nephrolithiasis, hypercholesterolemia, dehydration, and constipation
EBT: "EEG Biofeedback Therapy" is a learning strategy that helps the patient to control his/her brain activity.
School/education:
Extra pedagogical assistance
Information for school staff
Handling the school staff's fear!
Law:
Driving license
Crime and accident
Economy:
Employment
Insurance
Drugs and healthcare
Sickness compensation
Human rights:
Discrimination
Neuropsychologic evaluation is essential!
-------------------------------------------------
By AmirHossein Mahdavian, MD
Pediatric neurologist