By Johneilia Bariffe & Richele Hardy What is Epilepsy? ● The word Epilepsy comes from a Greek word which means, “to seize, possess, or afflict” ● It is classified by having at least 2 seizures within the timespan of 24 hours with unknown causes ● Seizures are the only visible symptoms of epilepsy ● Next to migraines, it’s the most common neurological disorder Within the Nervous System What is wrong in the brain? ● When you have a seizure there may be too much or too little activity which causes an imbalance of excitability and stopping. ● Most of the time the cells in the brain are too excitable which produces a sudden surge of electrical activity in the brain. ● Normally the brain has a homeostatic balance of cells that can excite and those that can stop messages. Neurological Activity - Epilepsy is the disorder of the central nervous system, specifically the brain - A person with epilepsy experience glitches in their neurological channels - The body runs on electrical signals and with seizures the electrical homeostasis is all over the place - Signals either act when they should not or do not act at all http://www.chm.bris.ac.uk/webprojects2002/wrigglesworth/brainimaging.htm https://www.youtube.com/watch?v=THOdtXlQXPU&list=PLQ2LF5nDPNka_vhRn25vMBZG-HrY-YeWM&index=45 Neurological Activity - depends on how many cells are signaling and which area of the brain is involved - also, depends on the seizure threshold which is the balance between the excitatory and inhibitory - the hippocampus is susceptible to epilepsy. Neurological Activity - Glutamate is the brain’s prominent excitatory neurotransmitter in the body and its receptors are in charge of the neural communication. As well as indicating for GABA. - Glutamate is a ligand, which is an ion that binds to the central metal atom, that signals Ca 2+ and Na channels so open - The prolonged excitability causes the glutamate to become toxic to the body. All of this due to the overwhelming messages and can cause a stroke which could lead to epilepsy or lead straight to Epilepsy Neurological Activity - GABA the brain’s primary inhibitory neurotransmitter - Studies show that abnormalities in this neurotransmitter can initiate the symptoms of epilepsy - Also, there is research that reasoned that since there was not a sufficient uptake of GABA, then the next demand lacks any power. - This lack of power causes the overproduction of excitable neurotransmitters http://medicine.yale.edu/neurology/people/ognen_petroff-3.profile History of Epilepsy Timeline Timeline continued... Timeline continued...again Timeline continued... 21st Century How would one diagnosis someone with Epilepsy? All epileptologists are neurologists, but not all neurologists are epileptologists. Epilepsy Syndromes The type of Epilepsy is classified in categories depending on: 1. 2. 3. 4. 5. 6. 7. Type of seizure (ex. twitching or still) How severe and how frequent Genetic History Which part(s) of the brain is involved Cause of seizure Age EGG, MRI, CT scan images https://www.youtube.com/watch?v=kJOC_ZGKDr8 Types of Epilepsy Idiopathic - there is no apparent cause (ex genetics) Cryptogenic - there is a cause but it hasn't been identified Symptomatic - the cause has been identified Triggers - Genetics - A scar, a tangle of blood vessels or anything that interrupts the flow of electricity in the brain - Head injury, brain tumor, birth trauma and stroke - Abuse of substances - Aftermath of diseases like measles - brain damage that has not been identified Generalized v.s. Partial Generalized widespread electrical discharge across both sides of the brain Partial occur in one part of brain 60% of people with epilepsy have this type Generalized Epilepsy - mostly idiopathic which means they have no apparent cause - both sides of the brain are affected by the abnormal electrical activity - many have loss of consciousness Benign Rolandic Epilepsy of Children (BREC) - seizures start around the central sulcus or Rolandic fissure (a fold in the cerebral cortex) age 3-13 typically involves twitching, numbness, or tingling of the face or tongue lasts no more than 2 minutes and child remains fully conscious usually happens at night common trigger is sleep deprivation almost all outgrow by age 15 15% of all epilepsy in children most common in children who have a close relative with epilepsy the cause is genetics there is a theory that there is a link with the mutation of chromosome 15q14 which is involved in excitability of nerve cells How is BREC Diagnosed Diagnosed with a MRI (magnetic resolance imaging) or EEG (electroencephalogram) - usually the MRI appears normal - on the EGG the child will have spikes in the temporal lobe and motor area *Since most experience less than 10 seizures in the 2 - 4 years of the BREC’s peak most children do not take any medication* **Medication is optional if seizures are frequent or during the day (neurontin, tegretol, keppra, vimpat, zonegran, trileptal are most common)** Childhood Absence Epilepsy (petit mal) - child has a staring spell and is unaware and unresponsive - lasts about 10 seconds - episodes can occur 1 to 50 times per day - begin between ages 4-8 - 2%-8% of people with epilepsy **predominantly genetic** 10% chance of a child developing if a close relative has a past - treatment includes Zarontin and Depakote - 65% of children with CAE respond to the treatment and the seizures disappear by mid adolescence Myoclonic - “myo” means muscle and “clonic” means jerk - three specific types (juvenile, progressive, and LennoxGastaut syndrome) - they happen due to an increase in muscle tone - the rapid change from relaxing to contracting in a muscle - usually cause abnormal jerking or twitching movements on both sides of the body but can be only one sided - genetic Continued ● react as they have been hit by a single jolt of electricity ● often drop the objects in their hands ● mistaken for clumsiness or tremors because they last only for a second Juvenile Myoclonic (JME) - emerge from mid to late childhood they can have staring spells or full on convulsions seizures involved neck, shoulders, and upper arms usually after they wake up genetically determined controlled with medication but continued throughout life the most common triggers are flashing lights, stress, and sleep deprivation - important to have an outlet for stress and get plenty of rest to reduce chances of seizures Clonic - Clonic means jerking - Rare; varies in age, even newborns can develop this -sometimes the seizures are brief and disappear on their own or some may last into adulthood - Causes constant, rhythmic jerking of the arms and legs - Sometimes it affects both sides of the body - Cannot be stopped if the person is restrained - Don’t mistake it with jitteriness because a person with clonic is less alert than a person who is jittery - Different from Tonic-Clonic because a period of tiredness or confusion doesn’t follow the episode Tonic - Muscle “tone” is the muscle’s normal tension at rest but a “tonic” seizure causes the tension to increase in the body, arms and legs, thus everything stiffens. - Occurs most often during sleep and involves all or most of the brain, which affects both sides of the body - Can last < 20 seconds - Common in people who have Lennox-Gastaut syndrome but can occur in anyone - After an episode the person can be either sleepy or confused Tonic Continued... - Some people might have one seizure per episode or back to back seizures in one episode - EEGs are used to diagnose the seizures - Medicines can be used to treat these. Tonic-Clonic (Grand Mal) - First: Tonic Phase- All muscles stiffen, air is being forced out so a groan or cry is heard, person loses consciousness, may turn blue, and there might be blood due to biting of the tongue or cheek, - Second Phase: Clonic- the rapid and rhythmic jerking of the limbs, causes one to relieve themselves involuntarily, consciousness returns and person maybe drowsy, depressed or irritated - Can last for 1 to 3 minutes Tonic-Clonic (Grand Mal) - affects both adults and children - the severity of the illness depends on the person (add a little tidbit) - The only way to truly tell if a person has Tonic-Clonic is through video EEGs - Special medicines can be used in preventing further seizures - VNS can help with this type of epilepsy https://www.youtube.com/watch?v=BvqA3vbNYPc&index=12&list=PLQ2LF5nDPNka_vhRn25vMBZG-HrY-YeWM Atonic (Akinetic) - Muscle “tone” = normal muscle tension - Atonic means “without tone” so during a seizure the muscles lose strength. Akinetic means “without movement” - Called “drop seizures” or “drop attacks” since they become limp as they fall - Person usually remains conscious and it lasts < 15 seconds - Begins in childhood and can last into adulthood - Can be treated by surgery or medicines and devices - EEGs are used but checking blood pressure and BPM is needed Partial Epilepsy - partial seizures may affect one small part (simple) of the brain or the whole hemisphere (complex) Simple Partial ● Simple Partial seizures can either be motor or sensory ● Motor seizures involve abnormal twitching or stiffening of the muscles ● Sensory seizures involve smelling and tasting things that are not there. Clicking and ringing are the most common experiences during a sensory seizure but visual hallucinations may happen as well. ● Anyone can get them but more likely after a head injury ● Lasts no more than 2 mins and the person remains fully aware Complex Partial - start in one specific area (ex. frontal lobe) and spread to other areas of that hemisphere that can affect awareness and memory - lasts up to 2 minutes and the person may feel tired or confused afterwards in extreme cases they may not return to feeling normal for hours - during the seizure most people start to lose awareness and smack their lips or do other meaningless movements https://www.youtube.com/watch?v=WP4NZcfadIk&index=11&list=PLQ2LF5nDPNka_vhRn25vMBZG-HrYYeWM Temporal Lobe - most common of partial epilepsy - auras or warnings often are the first sign (up to one minute long) ● might experience hallucinations of people, music, voices, smells or tastes - most common form of temporal epilepsy is medial - internal structures of temporal lobe - begins with hippocampus and surrounding structures - resistant to medications so surgery is the best way to go - mostly found by an MRI - hippocampal sclerosis is considered a factor in the development of this type of epilepsy Frontal Lobe Epilepsy - 2nd most common form of epilepsy - Lasts for less than a minute - These are considered partial but the symptoms vary depending on the area - someone could be experiencing a tonic-clonic seizure, while someone else could be laughing or cry or not be able to speak - Often occurs during sleep and can cause them to unconsciously scream, do bicycle movements et cetera - Treatment is dependent on what symptoms are exhibited Occipital Lobe Epilepsy - Accounts for 5-10 % of epilepsy syndromes - occurs in both adults and children - Can be either idiopathic or symptomatic - Begin with visual hallucinations (flickering , colored light or anything that is related to eyes or vision - Usually spontaneous but can be triggered by flashing lights or a repeating pattern - Symptoms: nausea/ vomiting, partial blindness, eye jerking, pain in the eyes, et cetera Parietal Lobe Epilepsy - Considered a rare form of epilepsy - Occurs at any age and any gender - since this part deals with touch, sensory information and planned movements, these operations are affected - Can last for a few seconds and up to a few minutes - Somatosensory seizures: patients feel numbness, tingling, heat, pressure and on rare occasions pain. Sometimes they experience involuntary marching - Somatic illusions: Feel like posture is abnormal, or that they are missing body parts Parietal Lobe Epilepsy - Vertigo: the environment begins to spin or its their body that is moving’ - Visual Illusions/ Hallucinations: objects being too far or too close while other objects appear to be there but are nonexistent - Language Disturbances: difficulty understanding words and language, reading and doing basic math Treatments Besides the common treatments include: 1. 2. 3. 4. 5. 6. 7. Antiepileptic Drugs Bromides Barbiturates Ketogenic Diet Vagus Nerve Stimulator (VNS) Responsive Neuropace Stimulator Surgery Anti-epileptic Drugs - There are around 26 AEDs used to treat seizures, and different AEDs work for different seizures. - There are around 4 generations of AED, some were mentioned in the timeline - they help to decrease the severity of the seizures and/or the frequency of these seizures - They attack the Na channels, Ca 2+ channels as well as enhancing the GABA mechanisms in the body. Block any repetitive actions which causes inactivity - Examples: Acetazolamide (partial,tonic-clonic & absence), Phenytoin (everything but absence) Bromides - Sodium Bromide (NaBr) and Potassium Bromide (KBr) - used as a sedative and anticonvulsant - used until replaced by barbiturates - still used today but primarily by veterinarians - 1st medical treatment geared toward epilepsy Barbiturates (Phenobarbital) - comes from barbituric acid - it suppresses the activity of the CNS - they enhance the activity of GABA (inhibitory neurotransmitter) - used to treat all types of seizures except for absence seizures Used to Treat - Temporal Lobe Epilepsy - Clonic Seizures, Tonic Seizures and Tonic-clonic Seizures - Simple Partial Seizures - Et Cetera Ketogenic Diet - ketogenic means that it produces ketones in the body - ketones are what the body uses fat as its source of energy instead of carbohydrates - successful mostly on children 2-12 with difficult to control seizures or do not respond to medication - not a cure justs reduces the chance for a seizure - the ratio of fat to carbs and protien is 3:1 - the diet is very strict and hard to follow Ketogenic Diet (continued) - the diet is not long term but typically used for 2 years - mineral supplements are important (ex calcium and vitamin d to prevent bone thinning) - one of the oldest treatments Vagus Nerve Stimulator ● sends regular mild pulses of electrical energy to the brain via the vagus nerve ● vagus nerve controls involuntary functions like heart rate ● passes through neck as it goes from between chest and abdomen and lower part of brain ● device is placed under the skin on chest ● wire from the stimulator is wound around the vagus nerve on the left side of neck ● alternates from sending signals to resting ● lasts 5 - 10 years Responsive Neuropace Stimulator ● should be treated for people who do not respond well to medicine and who cannot have surgery to remove part of the brain ● device is placed onto skull ● when a seizure is identified then the device tries to stop the seizure activity by sending an electrical current ● not a cure just an aid Surgery - This option is here because sometimes the seizures cannot be controlled by medicines - Also, it can reduce the frequency of some seizures - The earlier a surgery is performed, the better the outcome - Beneficial to those suffering from epilepsy induced by brain tumors, strokes or blood vessels degradation - Every surgery is tailored to the individual - At least single drugs or 2 combinations must be tried before performing surgery Surgery: Types - Resection or resective surgery: removes the part of the brain that causes the seizure to happen - most common form is temporal lobectomy - frontal lobectomy - parietal and occipital lobectomy Surgery: Types - Disconnection surgery: the surgeon interrupts the nerve pathways that allow the seizures to spread - more of a relief than a cure - Corpus callosotomy: severs the pathway that seizure can travel on - Multiple subpial transections: great for when some areas that the seizures begin are too important to remove - Thermal Ablation: laser surgery which utilizes MRIs in order to pinpoint where in the brain there will be an operation - has a very high accuracy rate Florence Griffith Joyner (Flo-Jo) - - - Considered the fastest woman in the world suffered from the tonic-clonic form of epilepsy in 1990 treated for it in 1993 and 1994 she had a brain abnormality which caused her to experience these seizures Cavernous hemangioma is what she suffered from This was the malformation of blood vessels and causes leakage in the brain She died in 1998 due to suffocation during an epileptic seizure Prince - - He was born with epilepsy and suffered throughout his infancy These episodes lasted until he was 7 years old Even though he grew out of the seizures, the isolation that he felt made him turn to music He tried to restrict being flashy in his early years He tried to being flashy and loud in his later years in order to make up for the illness About ⅓ of childhood epilepsy disappears in childhood https://www.youtube.com/watch?v=agD9qVUVHrI#t=23 Organizations ● Organizations provide the tools to raise awareness for epilepsy and raise money for further research. ● http://www.epilepsy.com/ is the largest foundation geared to helping this disorder but there are others such as https://www.epilepsyct.com/ http://www.epilepsynewengland.org/