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Benign Rolandic Epilepsy (BRE) Same as BECTS. Children between 4-8 y.o. No convulsions. Genetic. Most common epilepsy in childhood. Usually nocturnal. Brief. SP (focal onset) sz involving mouth & face or GTC sz - Starts at one side of face or mouth & may spread to arm or leg.
Juvenile Myoclonic Epilepsy (JME) (Janz Syndrome) Begins near puberty (after 8-10 yo). Idiopathic GE early in the morning (Nearly exclusively 15 to 30 min after awakening but usually within 1-2 hours). Myoclonic Jerks. Also GTC, CT, CTC or variation. No cognitive delays. No LOC. Genetic. Life long AEDs.
Simple Febrile Seizures Begins in children 3 mo - 5 yo with 2 yo most common. Not considered 'epilepsy' if these szs are reoccurring unless other factors develop looks like TC sz. Most common cause of sz in children. 1% develop epilepsy.
Infantile Spasms (IS) Other Names: - West's Syndrome - Jackknife convulsions - Salaam spasms - Generalized Flexion Epilepsy - Massive Myoclonia - Infantile Epileptic Encephalopathy - Infantile Myoclonic Encephalopathy 2-7 months up to 2 years. Sudden onset, regression, cerebral malformation, perinatal brain damage. Quick jerky, lean forward from waist, abrupt head drop to waist. Tonic or myoclonic seizures usually around sleep time. Mental and cognitive impairments.
Myoclonic Seizures Generalized. Sudden brief (20-100 msec) muscle contraction - Generalized. May involve 1 or few muscles, Reoccurring every few seconds. Usually synchronous. No impairment of consciousness. No post ictal period.
Atonic Seizures (Akinetic / Astatic Seizures) 'Drop Attacks'. No warning - a sudden total loss of muscle tone. Pt loses consciousness during attack but may regain immediately after falling. May need helmet or collosectomy to decrease if not able to tx.
Tonic Seizures (TC) Brief (10-30 s) episodes of generalized increase in muscle tone. Air rushes to lungs (pt may yell out). Pt loses consciousness and falls due to stiffing/rigid Usually activated by onset of sleep. Postictal. impairment ~Seen in LGS (about 92%).
Clonic Seizures Rhythmic bilateral myoclonic jerks of arms and legs occurring in rapid succession. Eyes may roll back, tongue may suffer bruising by strong jaw contractions, may have incontinence, teeth clench, irregular breathing may occur. Post ictal symptoms.
Typical Absence Seizures (Petit-Mal) Normal ictal period, no warning before a seizure and no post ictal period. Awareness and responsiveness are impaired. Very brief starring. Complete alertness immediately afterward. Last less than 5-10 seconds. Usually a family hx.
Atypical Absence Seizures (Petit-Mal) Starring and include a change in muscle activity, usually eye blinks, eye rolling, lip smacking, chewing, change in postural tone, and other automatisms. Usually more than 10 seconds.
Nocturnal Seizures Seizures occur only while sleeping. It is focal onset (may be very brief) and secondarily generalized to TC. If pt stays awake at time of sleep, s/he can have the seizure while awake, which will have symptoms of motor szs on one side of the body.
Tonic Clonic Seizures (TC) (Grand Mal or Gran Mal) Generalized. Not usually during first months of life but can any age after. Aura (which is SP sz) then lose consciousness and make odd/pointless repetitive mvts. such as picking, smacking towards the end of the aura (sz is a CP now) then generalizes.
Complex Partial Seizures (Psycho-Motor Seizures / Temporal Lobe Seizures / Psychomotor Seizures) Focal followed by impairment of consciousness at onset. Usually preceded by an aura. Usually includes psychiatric symptoms. Automatisms (some autonomic - vomit, etc.) Can resembles drunk. Loss of consciousness ~Long confusion.
Simple Partial (Focal Motor or Focal Sensory - Aura) Focal. Originates from Rolandic. Usually precursor to larger sz. Sudden and very brief. No impairment of conscience. Symptoms will be contralateral. Ex: Jacksonian March. May include: Motor, Somatosensory, Autonomic, Psychic symptoms.
Aversive Seizures Caused by a lesion on either of the frontal motor eye fields. Focal ~During a sz, head and eyes or trunk deviate away from the location of the lesion.
Affective Seizure A focal or temporal lobe seizure accompanied by emotional reactions.
Eneuretic Seizures Seizures involving bed wetting. Not the same as nocturnal bed wetting. This is also often called incontinence.
Jacksonian (March) Seizures Unique - travel through motor cortex in succession, affecting corresponding muscles, usually fingers first. Begin SP sz, then motor symptoms like twitching, turning head, paralysis. The progression has a particular rhythm.
Gelastic Seizures Automatism of laughing. Rare ~Involves a sudden burst of energy. Usually occurs for no obvious reason and is uncontrollable. Slightly more common in males than females.
Lacrimonic Seizures Automatism of crying.
Neonatal Seizures (Unclassified Seizures) First 28 days of life (at gestational age 44 weeks). Focal and symptomatic with shifting clonic movements that go from one area of the body to another in a disorganized fashion. Eye 'swimming' is common. Only affected a few days. Relatively common.
Sensory Reflex Seizures Rare. Induced by specific stimuli. Partial or generalized. Usually: Reading (myoclonic jerks of jaw). Auditory, somatic, olfactory, gustatory, or visceral, photo (most common). Others: eating, musicogenic (CP), mvt. induced, 'Tapping Sz' by touch.
Status Epilepticus (SE) Pt does not fully recover between Szs or single sz lasting longer than 30 minutes Life-threatening. Always considered an emergency because 5 mins. is sufficient to damage neurons and szs are unlikely to self-terminate. The mortality (at least 20%).
Simple Partial Status Epilepticus (SPSE) - (also Epilepsia Partialis Continua (EPC) and Focal Motor Status) Constant repetitive clonic activity if a limited muscle segments contralateral to the cortical focus. Pt is conscious. Some may be surgical candidates. Non-convulsive.
Absence Status Epilepticus (Petit Mal Status / Spike & Wave Stupor) Most pts seem dull and confused but able to carry out daily living such as eating, drinking, dressing. Seen in any age but most often adults who failed to out grow childhood disorder. Automatisms of face and hands can occur. Alters consciousness.
Complex Partial Status Epilepticus (CPSE) Rare. May be convulsive or one of the only non-convulsive forms in category. Twilight state b/t szs w/ partial responsiveness and long-lasting stupor & staring. Automatism of speech and purposeful movements, eye twitching. Dangerously misdiagnosed.
Partial Seizures Symptoms vary according to location of sz -Frontal lobe symptoms include wave-like sensation in the head -Temporal lobe, feeling of déjà vu; -Parietal lobe, numbness or tingling; -Occipital lobe, visual disturbance or hallucination.
PGE Includes Idiopathic TC, Classic Absence, Myoclonic. Normal cognitive functioning. Easily managed. Most common.
SGE Includes Atypical Absence, Tonic, Atonic, Infantile Spasms, LGS Has an identifiable cause and there is usually some cognitive delay and difficulty controlling.
Frontal Lobe Seizures Mostly SP Sz Effects reasoning and higher learning characterized by unusual symptoms of ‘forced thinking’ and ‘forced acts’. The patient is forced into an obsessive thought associated with attempt to act on it. Gaze and gestures seem aggressive.
Temporal Lobe Seizures More Complex Partial. Can be SP or complex focal, and SGE szs, or combinations. Effects emotions. Simple focal - autonomic and/or psychic symptoms. Complex focal - often but not always begin with motor arrest typically followed by automatisms.
Parietal Lobe Seizures Simple focal w/o impairment of consciousness Aura ~CP szs may arise become secondarily generalized ~Phenomena of tingling, electricity, may spread in a Jacksonian manner. May feel mvt of body part or like it's being moved. Muscle tone may be lost.
Occipital Lobe Seizures Visual manifestations: Perceptive illusions, sparks, flashes, decreases sight. Sensations appear contralateral to the discharge, but can spread to the entire visual field. ~Usually SP and SGE szs.
Continuous Spike and Wave Discharges During Sleep (CSWDS) There appears to be an overlap between Landau-Kleffner syndrome and this syndrome.
Electrographic Status Epilepticus of Sleep (ESES) Status w/ no clinical symptoms seen only during sleep. Commonly seen in LKS.
Partial with Secondary Generalization Simple Partial or Complex Partial evolving to Generalized or all three phases in sequence.
Seizure Classification I - Simple Partial (Focal) with motor sign: With or without march, Focal motor with Jacksonian March, Versive, Postural, Phonotory. Will affect muscle activity, causing jerking movements of the foot, face, arm, or another part of the body.
Seizure Classification II - Generalized, Convulsive and Non-Convulsive: Typical Absence (Petit Mal): Impairment of consciousness only, with mild coloic, atonic, tonic, automatisms, or autonomic components. Atypical Absence with Myoclonic, Clonic, Tonic, TC, or Atonic Szs. Myoclonic, Clonic, Tonic, TC, Atonic
Seizure Classification III - Unclassified Epileptic Seizures: Any that cannot be classified due to incomplete data or defying classification into category. Ex: Neonatal with Swimming Movements.
Seizure Classification IV Status Epilepticus
Tonic Phase of TC Sz Abrupt increase in muscle tone. Stiffening of the extremities occurs (arms and legs extend, spine arches and jaw clamps shut), pupils dilate and are non-reactive and consciousness is lost. Spasms of the respiratory muscles also occur.
Clonic Phase of TC Sz Occurs as the tonic phase ends – begins suddenly and by quick, bilateral, severe jerking movements. The patient may have irregular and labored respiration, sweat profusely, froth at the mouth, experience incontinence and have clenched jaws.
Post-Ictal Phase of TC Sz Begins when all muscles are relaxed and the pt falls into a deep sleep. May last 1-2 hours breathing becomes more regular, pupils become more reactive. Pt may appear confused and drowsy.
Focal Epilepsies Szs that emanate from an epileptogenic focus anywhere within the brain. Ictal symptoms determined by localisation and not by aetiology. Mesial temporal lobe epilepsy (MTLE) with hippocampal sclerosis (most common and most distinct) is a striking example.
Subacute Sclerosing Panencephalitis (SSPE) Rare and chronic 'disease-revealing' sz, dominated by partial szs, secondarily generalized (86%), suggests focal encephalitic process and assoc w/ myo jerks, burst-suppression, Rademecker Complex. Children and young adults in Morocco affected.
Mesial Temporal Lobe Epilepsy (MTLE) This has been associated with HHV-6 (Neuro Human Herpes Virus) infection. Is the most common form of chronic epilepsy and its underlying mechanism is not fully understood.
What type of epilepsy would a pt experience an aura of Rising Epigastric Sensation (RES) or fear of olfactory? CPE.
What part of the brain does Partial Epilepsy come from? Hippocampus.
What are the primary causes of Partial Epilepsy? It usually originates from infancy due to prolonged labor, birth trauma, hypoxia, or febrile szs.
What part of the brain does psychomotor variants arise from? Cortex - not deep.
Benign Partial Epilepsy of Childhood with Centro-temporal Spikes (BECTS) Same as BRE. Most common forms of epilepsy in children. Most szs occur from sleep. Child has partial szs with a EEG pattern in the centrotemporal region. Easily controlled with AEDs and usually cured by the age of 16.
Sylvian Seizures Produced by BECTS/BRE.
What are the 8 important childhood epilepsy syndromes? 1. Simple Febrile Convulsions 2. Complex Febrile Convulsions 3. IS (West Syndrome) 4. LGS 5. Absence 6. BECTS/BRE 7. JME 8. BECTS w/ Occipital Paroxyms
Complex Febrile Seizures Abnormal neurological exam and usually a family hx of sz. This type increases a child's risk for epilepsy.
Lennox-Gastaut Syndrome (LGS) 3-5 years. Mental delay. Multiple sz types including TC, atonic, myoclonic, atypical absence, and may have a family hx of sz. Usually is a continuation of IS.
GCSE Overt Szs 30 minutes or more without complete recovery between events (back to back). Easy to identify.
GCSE Subtle Sustained activity lasting 10 minutes in a comatose, brain damaged, disease monitoring, pt. Continuous or near continuous epileptiform activity such as PLEDS.
NCSE Varying terms are used. Sz state with altered consciousness and sometimes minor clinical symptoms. Progression is very different by cause of cerebral insult vs. generalized convulsive status. 2 Types. Absence and CP. Pt may be conscious b/t events or not.
Epilepsia Partialis Continua (Kojevnikov's Epilepsia) A rare type of brain disorder in which a patient experiences recurrent motor epileptic seizures that are focal (hands and face), and recur every few seconds or minutes for extended periods (days or years).
PLEDS Occurs mostly in acute cerebral lesions such as stroke and encephalitis. May also be caused by tumor.
What is the difference between a Jacksonian Sz and a TC sz proceeded by a sensory aura? Location of the focus.
What does the Jacksonian Sz begin and end with? Clonic activity, usually involving the hand contralateral to a lesion in the motor cortex and progresses to a TC sz.
Simple Partial Sz Aura The origin of this kind of sz is in the somatosensory area of the brain, contralateral to the limb experiencing he feeling.
Clonic Sz Involves maximal contraction of skeletal muscles, followed by relaxation, with the cycle usually repeated a few times per second.
Most Szs are... Non-Epileptic.
What is the proper definition of a Sz? A paroxysmal, time-limited event that results from abnormal neuronal activity in the brain.
What is the proper definition of Epilepsy? A condition in which an individual is predisposed to recurrent szs because of a CNS disorder.
What is the most common cause for epilepsy in the elderly? Cerebrovasuular diseases (stroke). Tumor and head injury are also common.
What is the most common cause for epilepsy in the young? Genetic syndromes and birth traumas.
What are the factors used to identify Epileptic Syndrome? Sz type, EEG, Etiology, AED response, Inheritance, Natural hx.
Focal Epilepsies and Syndromes (Local & Partial): Idiopathic with age-related onset. Can have impaired consciousness or not. BRE and BOE. Symptoms are based on localization.
Generalized Epilepsies and Syndromes, Idiopathic with age related onset (listed chronologically by age): Benign neonatal Familial Convulsions, Benign Neonatal convulsions, Benign Myoclonic Epilepsy in Infancy, Absence, JME, Grand Mal on Awakening.
Generalized Epilepsies and Syndromes, Idiopathic & symptomatic (listed chronologically by age): IS, LGS, Myoclonic Astatic, Myoclonic Absence.
Generalized Epilepsies and Syndromes, Nonspecific etiology (listed chronologically by age): Early myoclonic encephalopathy.
Epilepsies and Syndromes Undetermined as to whether they are Focal or Generalized, with both generalized and vocal szs (listed chronologically by age): Neonatal szs, Severe myoclonic epilepsy in infancy, Epilepsy with continuous spike and wave during slow wave sleep, LKS.
Epilepsies and Syndromes, Special Syndromes - Situation related, Isolated, unprovoked, specific modes, chronic progressive: Febrile, Hormonal, Drugs, Alcohol, Sleep Deprivation.
Syndromes by Age - Newborn: Benign neonatal convulsions (fifth day fits), Familial benign neonatal convulsions, Early myoclonic encephalopathy, Severe idiopathic status epilepticus, Early infantile epileptic encephalopathy with suppression-burst.
Syndromes by Age - Infants: Febrile, IS, Benign myoclonic epilepsy, Severe myoclonic epilepsy, Myoclonic epilepsy in non-progressive encephalopathies (myoclonic status), Epileptic szs caused by inborn errors of metabolism, Myoclonic-astatic epilepsy of early childhood, LGS.
Syndromes by Age - Children: Absence, Myoclonic Absence, Generalized convulsive, Benign partial, BRE, Benign psychomotor epilepsy, BOE, LKS, Spike and Wave during sleep, Photosensitive epilepsy, Self-Induced.
Syndromes by Age - Older Children and Adolescents (Juvenile): Juvenile Absence, JME, GMA, Benign partial szs of adolescence, Kojewnikoff's Syndrome, Progressive Myoclonus epilepsies, Unverricht-Lundborg Disease.
GMA Grand Mal on Awakening.
Progressive Myoclonus Epilepsies Juvenile Gaucher's, Juvenile neuronal ceroid lipofuscinosis (NCL), Lafora's body disease.
Unverricht-Lundborg Disease (Finnish or Baltic myoclonus epilepsy) Cherry-red spot myoclonus (neuraminidase deficiency), Dyssynergia cerebellaris myoclonicia (Ramsay Hunt Syndrome), Mitochondrial encephalopathy.
Some causes of Localized Epilepsies - Vascular: Stroke, Infantile Hemiplegia, Arteriovenous malformation, Sturge-Weber Syndrome, Aneurysms (subarachnoid hemorrhage), Venous thrombosis, Hypertensive encephalopathy, Sickle cell anemia.
Some causes of Localized Epilepsies - Infectious: Abscess, Meningitis, Encephalitis, Toxoplasmosis, Rubella, Rasmussen's syndrome, Cysticercosis.
Some causes of Localized Epilepsies - Tumors: Meningiomas, Gliomas, Hamartomas, Metastatic.
Some causes of Localized Epilepsies - Degenerative: Alzheimer's, Multiple Sclerosis.
Some causes of Localized Epilepsies - Congenital: Heterotopias, Cortical dysplasias.
Some causes of Localized Epilepsies - Traumatic: Prenatal and Perinatal injuries, Head injuries.
Some causes of Localized Epilepsies - Cryptogenic: No identified cause.
Akinetic Seizure The same as an Atonic Sz.
What is the recommended tx for managing epilepsy in pregnant women? Educate risks, determine need for continued AED, monotherapy, start folic acid, eliminate substance abuse, do not change AED, check levels every trimester and adjust dose accordingly, eval for neural tube defect at 12-16w, levels before delivery.
When is the best time to tx in attempt to prevent Status Epilepticus? After 1 generalized TC sz lasting more than 5 minutes or After 2 generalized TC szs occurring in 1 hour.
What are the driving laws for sz pts? There is a sz free period of 90 days - 18 months, depending on the state. Some states give discretion to the DMV or physician.
What type of tx do you use for szs caused by a condition? No sz tx. Tx condition (tumor, infection, head injury, etc) - unless sibling has sz hx.
Are non-epileptic szs fake? Not always. Can be psychogenic (from brain - not fake, but not sz) or any other cause rather than CNS.
Seizure Classification I - Simple Partial (Focal) with Somatosensory or Special Sensory symptoms: Somatosensory, Visual, Auditory, Olfactory, Gustatory, Vertiginous.
Seizure Classification I - Simple Partial (Focal) with Psychic Symptoms: Affects parts of the brain that trigger emotions or previous experiences: it may cause feelings of fear, anxiety, déjà vu (the feeling that something has been experienced before), etc.
Seizure Classification I - Simple Partial (Focal) with Autonomic Signs or Symptoms: May cause changes in blood pressure, heart rhythm, bowel function, etc.
Seizure Classification I - Complex Partial: With Simple Partial features, with automatisms.
Seizure Classification I - Complex Partial with impairment of consciousness at onset: Impairment of consciousness only, with automatisms.
Seizure Classification I - Complex Partial evolving to Secondarily generalized Seizures: Simple Partial, Complex Partial, Simple Partial to Complex Partial.
De Novo Automatisms Associated with longer spells and most commonly associated with rubbing the face or hands or smiling.
In Absence Szs, clinical effects generally accompany discharges lasing longer than... 3 seconds.
Clonic Szs are similar to GTC except for what? Only consist of rhythmic or semirthytmic contractions of a group of muscles.
There are two different types of Tonic Szs. The Tonic type and Flexor Spasms. What is the difference? In the first type, the pt extends body and falls. The body remains in that state after fall. In the second type, the hypertonicity appear in in bursts of forward flexations of the upper body and flinging outward and resembles IS.
What are the two divisions (dichotomies) of epileptic syndromes? Generalized and Partial (Focal) related.
Benign Occipital Epilepsy (BOE) (Benign focal epilepsy with occipital paroxysms) Panayiotopoulos Type: Ages 15 months-17 yrs. Infrequent szs typically at night shortly after falling asleep. >10 minutes, may include vomiting, gazing to side. Often evolve to rhythmic muscle contractions, one or both sides of body. Triggers may be turning off lights etc.
Benign Occipital Epilepsy (BOE) (Benign focal epilepsy with occipital paroxysms) Gastaut Type: Begins between the ages of 3 and 16 years and has a peak onset between ages 7 and 9. Children may experience visual hallucinations with the seizure. Headaches are common before, during, or after the seizures.
Doose Syndrome (Myoclonic-Astatic Epilepsy MAE) Early childhood age 1-5, 3 mean. Often resistant to AED. Idiopathic, generalized. Normal development. Boys 3:1. Myoclonic and/or myoclonic-astatic (or drop) szs. The -astatic (loss of muscle tone) feature is rare. May also have TC or Absence.
Landau–Kleffner Syndrome (LKS) Ages of 3 and 7 years of age. Sudden or gradual aphasia and always abnormal EEG ~Affects Broca's and Wernicke's area.
Pt with medically refractory epilepsy often have... Structural brain abnormalities.
Most age related epilepsies are associated with particular types of... CNS lesions. Ex: TS can cause IS.
During childhood, the most common cause of epilepsy is... Congenital abnormality.
Generalized Myoclonic Status Epilepticus (MSE) A prolonged state of repetitive spontaneous epileptic myoclonic activity. Occurs in several forms. Most severe form occurs after anoxia and is always fatal.
Tonic Status Epilepticus (TSE) Rare. Primarily in children, particularly with LGS. Both sides of the body are usually involved, but may move asynchronously.
Non-Epileptic Seizures (NES) Paroxysmal events that mimic epileptic szs but do not involve abnormal, rhythmic discharges of cortical neurons. Caused by either physiological or psychological conditions. The latter is usually discussed more fully in this.
Benign Rolandic Spikes High voltage spike discharges seen primarily at C3 and T3 and accentuated during sleep.
Slow wave focus indicating a structural lesion involving entire left hemisphere... Polymorphic 1-2 Hz activity seen over entire left hemisphere, which does not block with EO.
What is the most common sz with BECTS? A focal seizure without impairment of consciousness, although a common presentation is a secondary generalized tonic clonic seizure during sleep.
Metabolic Encephalopathies Tri/Biphasic waves may be seen but other patterns are more common, such as diffuse slowing and epileptiform discharges. With progression, multifocal and synchronous spikes. Background is usually disrupted. Spikes resolve with disease correction.
Hyporcalcemia Marked slowing with high amplitude sharply contoured delta waves in the bifrontal head regions.
Created by: kmburg5840