Seizure Disorders Flashcards
How is epilepsy defined?
Epilepsy is defined as having two or more unprovoked seizures at least 24 hours apart.
What is the most common type of epilepsy in childhood?
Generalized epilepsy is the most common type in children and involves diffuse abnormal electrical activity from both cerebral hemispheres from the beginning of the seizure.
List 5 classic features of the seizures seen in generalized epilepsy.
Abrupt onset; loss or alteration of consciousness; variable bilateral symmetric motor activity associated with changes in muscle tone; no warning of the impending attack (no aura); and epileptiform activity that is bilateral and synchronous on EEG.
How do myoclonic seizures classically present?
Myoclonic seizures are classically defined by: short duration; rapid muscle contractions, often in one limb; isolated or repetitive jerks; and likelihood of the patient falling (if severe and involving the axial muscles).
How is consciousness usually affected during myoclonic seizures?
Consciousness is typically preserved during myoclonic seizures.
List four drugs/drug classes most effective in the treatment of myoclonic seizures.
Valproate, benzodiazepines, lamotrigine, or other broad-spectrum anticonvulsants.
Name the disorder that presents with morning myoclonic jerks and with onset between 8 and 20 years of age.
Juvenile myoclonic epilepsy
List six characteristics of juvenile myoclonic epilepsy.
Morning myoclonic jerks (often w/ dropping of items in hands); +/- history of absence seizures; generalized tonic-clonic seizures occurring just after awakening or during sleep; normal intelligence; family history of similar seizures; and onset between 8 and 20 years of age.
Do patients grow out of juvenile myoclonic epilepsy?
No, it is usually a lifelong condition.
What (3) factors typically worsen seizure activity in patients with juvenile myoclonic epilepsy?
Sleep deprivation, stress, and alcohol use.
What is the first line treatment for juvenile myoclonic epilepsy?
Valproate is the drug of choice and is often effective at very low doses.
List two alternative treatment options for patients with juvenile myoclonic epilepsy for whom valproate is contraindicated or undesirable.
Levetiracetam or lamotrigine
List the (6) characteristic features of absence seizures.
Episodes of extremely short lapses in awareness (5-15 seconds); absence of aura; amnesia during the episodes; abrupt onset and end (frequently in midconversation or activity); staring episodes (w/ possible flickering eyelids or eye rolling); absence of postictal period.
What three characteristics of focal seizures with impaired consciousness can be used to distinguish them from absence seizures?
Focal seizures with impaired consciousness often last longer (>30 seconds), have associated aura, and have slow return to consciousness postictally.
What is the general age range and peak age of occurance of absence seizures?
Absence seizures generally occur between 3 and 12 years of age, with a peak between 4 and 8 years.
Which sex is more likely to have absence seizures?
Absence seizures are more common in girls than boys.
What are the classic EEG findings in a patient with absence seizures? How might one provoke a seizure during the examination?
EEG classically shows a 3-Hz generalized spike and wave discharge. Hyperventilation for 3-4 minutes usually provokes an absence seizure.
What drug is used as first-line treatment for absence seizures?
Ethosuximide
List five characteristic features of atonic seizures.
Without warning, a sudden and complete loss of tone in the limbs, neck, and trunk muscles; can be brief or longer duration; loss of consciousness; complete awareness returns very quickly after the attack; and one or more myoclonic jerks can occur immediately before muscle tone is lost.
An elementary-aged child presents with focal hemifacial/extremity seizures shortly after going to sleep and just before or after waking up. During the episodes, the child seems to have difficulty with speech despite preserved cognition. Bilateral centrotemporal spikes are seen on EEG. What diagnosis do you suspect?
Benign rolandic epilepsy of childhood (AKA Benign epilepsy with centrotemporal spikes (BECTS))
What is the typical prognosis for patients with benign rolandic epilepsy of childhood?
Most children outgrow it within 2 years or, at the latest, by puberty. Long term prognosis is excellent.
How are patients with benign rolandic epilepsy of childhood typically treated?
Treatment is typically with oxcarbazepine.
What is the classic EEG finding in patients with benign rolandic epilepsy of childhood?
EEG classically shows bilateral centrotemporal spikes.
What is the definition of focal seizures without impaired consciousness?
Focal seizures without impaired consciousness refer to seizures in which the patients can still interact with their environment without loss of consciousness.
How do many focal seizures without impaired consciousness present?
Motor findings are most common and include asynchronous clonic or tonic movements that tend to involve the face, neck, and extremities.
What locations of the body are most commonly affected in focal seizures without impaired consciousness?
The face, neck, and extremities.
Define Todd paralysis.
Todd paralysis is a transient paralysis of the affected body part following seizure activity which can last for minutes to hours following the seizure.
Define focal seizures with impaired consciousness.
Focal seizures with impaired consciousness have variable symptoms but usually include alterations in consciousness, unresponsiveness, and automatisms.
What are automatisms?
Repetitive, purposeless, undirected, and inappropriate motor activities which can occur with certain types of seizures. They commonly include repetitive lip smacking, swallowing, chewing, or fidgeting of the fingers or hands.
What three antiepileptic medications are most commonly used in the treatment of focal seizures?
The drugs of choice for treatment of focal epilepsy are carbamazepine, oxcarbazepine, and phenytoin.
Describe a “jackknife” flexor spasm.
Sudden, simultaneous flexion of the neck and trunk, with associated flexion and adduction of the extremities. Jackknife spasms are typically brief and occur in clusters of diminishing severity.
What is the classic spasm seen in children with infantile spasms?
The “jackknife” flexor spasm (sudden, simultaneous flexion of the neck and trunk, with associated flexion and adduction of the extremities. Jackknife spasms are typically brief and occur in clusters of diminishing severity).
What percentage of patients with infantile spasms are found to have tuberous sclerosis?
Up to 30% of patients with infantile spasms are found to have tuberous sclerosis.
What is the classic EEG finding in patients with infantile spasms?
Hypsarrhythmia: high-voltage, irregular, slow waves that occur out of sync and randomly over all head regions intermixed with spikes from multiple foci.
What is the long-term prognosis for an infant with infantile spasms?
Infantile spasms resolve over time even without specific therapy; however, most surviving children have severe intellectual disability and other types of seizure disorders (most typically Lennox-Gastaut syndrome).
What is West syndrome?
West syndrome is a severe epilepsy syndrome that includes the triad of infantile spasms, intellectual disability, and hypsarrhythmia.
What are the various therapeutic options for infantile spasms in the U.S.?
The two most efficacious treatments include intramuscular adrenocorticotropic hormone (ACTH) and oral vigabatrin.
What are the (3) main adverse effects of ACTH therapy?
Hypertension, irritability, and increased risk of infection.
What is the drug of choice for treatment of infantile spasms in patients with concurrent tuberous sclerosis?
Vigabatrin
What is the black box warning associated with vigabatrin?
Permanent visual field deficits
Define Lennox-Gastaut syndrome.
Lennox-Gastaut syndrome refers to a varied group of symptoms with severe seizures, intellectual disability, and a characteristic EEG pattern: generalized, bilaterally synchronous, sharp- and slow-wave complexes, occurring in repetitive fashion in long runs at ~1.5-2.5 Hz (AKA “slow spike and wave”).
What percentage of patients with Lennox-Gastaut syndrome have a history of infantile spasms?
25-40%
