What is the age range for Startle Epilepsy?
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2 to 12 years.
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What is the age range for Startle Epilepsy?
2 to 12 years.
Is Startle Epilepsy more common in males or females?
It is equally common in both genders.
What is the etiology of Startle Epilepsy?
Unknown.
What is the prognosis for Startle Epilepsy?
Poor prognosis with intractable seizures.
With what conditions is Startle Epilepsy more commonly associated?
Localized/diffuse static encephalopathies, neurological deficits, and abnormal imaging.
What typically triggers seizures in Startle Epilepsy?
Unexpected sensory stimuli, particularly auditory.
What type of stimulus triggers seizures in Startle Epilepsy?
Stereotyped single stimuli within the patient.
What is the characteristic progression of seizures in West Syndrome?
Rapidly progressive multifocal/migrating clusters of partial seizures that become almost continuous by 1 year.
What are the three components of the triad in West Syndrome?
Infantile spasms, intellectual disability, and hypsarrhythmia EEG.
What is the typical age range for the onset of West Syndrome?
3 months to 12 years, with a peak at 4-6 months.
What is the prognosis for primary idiopathic/cryptogenic West Syndrome?
Better prognosis with normal psychomotor development prior to onset.
What is Landau-Kleffner Syndrome (LKS)?
A syndrome of acquired aphasia with convulsive disorder, typically affecting children aged 3-8 years.
What is the common etiology for secondary symptomatic West Syndrome?
Bad birth or cortical dysplasia, often with poor prognosis.
What are the common cerebral pathologies associated with LKS?
Acute inflammatory disease, tumors, and arachnoid cysts.
What is the typical treatment for West Syndrome?
Vigabatrin (VGB) and steroids.
What is the age range for Benign Neonatal Seizures (BNS)?
1 to 7 days (average 5 days).
What is the primary treatment for LKS?
Steroids and antiepileptic medications (ASMs).
What is the age range for Myoclonic Epilepsy of Infancy (MEI)?
6 months to 3 years.
What characterizes the language disorder in LKS?
Regression of previously acquired language.
What is a key characteristic of seizures in Benign Neonatal Seizures (BNS)?
Clonic clusters, with or without apnea.
What is the typical seizure manifestation in Myoclonic Epilepsy of Infancy?
Bilateral myoclonic jerks of upper extremities/head more than lower extremities.
What is the prognosis for Benign Neonatal Seizures (BNS)?
Good prognosis: self-limited with normal development.
What types of seizures are associated with LKS?
Partial motor, atypical absence, generalized tonic-clonic (GTC), and atonic seizures.
What is the prognosis for Benign Infantile Seizures (BIS)?
Great prognosis, neurologically normal with little to no seizure recurrence.
What is the typical age range for Benign Familial Neonatal Epilepsy (BFNE)?
2 to 8 days.
What does the interictal EEG show in LKS?
Normal background with bursts of temporal or temporo-occipital spike waves.
What type of seizures are characteristic of Benign Infantile Seizures (BIS)?
Complex partial seizures (CPS) with focal impaired awareness.
What is a notable feature of seizures in Benign Familial Neonatal Epilepsy (BFNE)?
Focal clonic or generalized tonic-clonic component (not GTC).
What is the typical interictal EEG finding in Benign Infantile Seizures (BIS)?
Normal.
What is Autosomal Dominant Nocturnal Frontal Lobe Epilepsy (ADNFLE)?
A genetic epilepsy characterized by brief clusters of nocturnal motor seizures, typically occurring in infancy to adulthood.
What is the prognosis for Early Myoclonic Encephalopathy (EME)?
Poor prognosis with >50% mortality rate by 1-2 years.
What is the seizure frequency in ADNFLE?
70% of patients are seizure-free on carbamazepine (CBZ).
What is the treatment for Epilepsy with Myoclonic Absences?
VPA + Lamotrigine.
What age group is primarily affected by Rasmussen’s Syndrome?
4 to 14 years old, with a peak between 5 to 10 years.
What type of seizures are associated with Early Myoclonic Encephalopathy (EME)?
Erratic/massive myoclonus and focal seizures.
What are the characteristics of seizures in ADNFLE?
Nocturnal motor seizures that are stereotyped, with no loss of consciousness.
What is the age range for Febrile Seizures?
6 months to 5 years, with peak incidence at 18-22 months.
What age group is primarily affected by Epilepsy with Myoclonic Absences?
11 months to 12 years.
What triggers Rasmussen’s Syndrome?
An autoimmune disorder triggered by viral infection.
What is the typical age range for Ohtahara Syndrome (EIEE)?
1 day to 3 months (average 10 days).
What does the interictal EEG show in ADNFLE?
Infrequent anterior quadrant epileptiform activity.
What triggers Febrile Seizures?
A sudden rise in temperature (fever >38.5°C).
What is the prognosis for Epilepsy with Myoclonic Absences?
Poor prognosis; may evolve to Lennox-Gastaut Syndrome (LGS).
What is the prognosis for Rasmussen’s Syndrome?
Very poor prognosis with intractable seizures.
What is a common EEG finding in Ohtahara Syndrome (EIEE)?
Suppression-burst pattern.
What type of seizures are associated with Rasmussen’s Syndrome?
Focal seizures from the affected hemisphere.
What are the characteristics of Lennox-Gastaut Syndrome (LGS)?
Triad: multiple types of epileptic seizures, specific EEG abnormalities, and slow mental development with varying degrees of intellectual disability.
What is the treatment for Febrile Seizures?
No seizure treatment; treat the fever.
What is a significant risk associated with Ohtahara Syndrome (EIEE)?
High mortality and intractable seizures.
What defines Simple Febrile Seizures?
1 seizure within 24 hours, generalized tonic-clonic (GTC) only, lasting less than 15 minutes.
What are common symptoms of Rasmussen’s Syndrome?
Progressive unilateral weakness, hemiparesis, intellectual disability, and possible aphasia.
What age range is most affected by Lennox-Gastaut Syndrome (LGS)?
1 to 8 years, with a peak at 2 to 5 years.
What defines Complex Febrile Seizures?
1 or more seizures in 24 hours, clusters, focal features, lasting more than 15 minutes.
What is a characteristic feature of Gelastic Seizures?
Ictal laughing or forced smiling.
What types of seizures are common in Lennox-Gastaut Syndrome (LGS)?
Atonic, tonic, atypical absence, and less commonly myoclonic and generalized tonic-clonic seizures.
What is Febrile Seizures Plus (GEFS+)?
Febrile seizures that continue beyond 5 years or other seizure types develop, often genetic.
What is the prognosis for Epileptic Encephalopathy with Continuous Spike Wave in Sleep (CSWS/ESES)?
Reserved; seizures may resolve but abnormal development and behavioral issues may persist.
What is a hypothalamic hamartoma?
A benign growth in the brain affecting the hypothalamus.
What is Dravet Syndrome (SMEI)?
The most severe form of epilepsy that begins in infancy, highly intractable with a poor prognosis.
What is a key EEG finding in CSWS/ESES?
Continuous diffuse slow waves in slow wave sleep.
What behavioral issues can arise from Gelastic Seizures?
Increased irritability, aggression, and behavioral problems.
What types of seizures predominate in CSWS/ESES?
Simple partial motor and generalized tonic-clonic seizures.
What are common seizure types in Dravet Syndrome?
Prolonged clonic seizures, myoclonic seizures, atypical absence seizures, and complex partial seizures.
What treatment options are available for Gelastic Seizures?
Surgery can be effective; if not surgical candidates, lifelong anti-seizure medications (ASMs) are needed.
What age range is Juvenile Absence Epilepsy (JAE) most commonly diagnosed?
7 to 16 years, typically around 10 to 12 years.
What is the typical EEG finding in Dravet Syndrome?
Frequent generalized spike-wave or polyspike-wave activity, with photosensitivity.
How is Juvenile Absence Epilepsy (JAE) characterized?
It is a primary generalized epilepsy that is well-controlled and usually lifelong.
What is Migrating Partial Epilepsy in Infancy (MPEI)?
A poorly controlled epilepsy with multifocal/migrating clusters of partial seizures, onset within the first 6 months of life.
What type of seizures are common in Juvenile Absence Epilepsy (JAE)?
Typical absence seizures with longer duration and clusters, as well as generalized tonic-clonic seizures upon awakening.
What are common symptoms of MPEI?
Eye jerks, eyelid twitching, limb jerks, chewing movements, apnea, flushing, and salivation.
What is the interictal EEG finding in Juvenile Absence Epilepsy (JAE)?
Normal background with possible PBDA/OIRDA and common polyspikes & SW fragments.
What is the age range for Familial Focal Epilepsy with Variable Foci (FFEwVF)?
2 months to 43 years, with an average of 10 years.
What is the age range for epilepsy with GTCs?
6 to 47 years, with a peak at 16-17 years.
What is the most common idiopathic generalized epilepsy?
Juvenile Myoclonic Epilepsy (JME).
What is the inheritance pattern of FFEwVF?
Autosomal dominant with 60% penetrance.
What is the male to female ratio in epilepsy with GTCs?
8:1.
What type of jerks are characteristic of myoclonic seizures in Juvenile Myoclonic Epilepsy (JME)?
Bilateral, single or repetitive, arrhythmic, and irregular jerks, more frequent in the morning.
What types of seizures are associated with FFEwVF?
CPS, SPS, and most secondary GTC seizures.
What type of seizures are primarily observed in epilepsy with GTCs?
Generalized Tonic-Clonic seizures (GTCs).
What is a common EEG finding during an ictal event in Juvenile Myoclonic Epilepsy (JME)?
Generalized SW/PSW with myoclonus and occasional irregular 3/s SW bursts.
What is the typical EEG finding in FFEwVF?
Focal epileptiform activity correlated with seizures, both ictal and interictal.
What are common triggers for GTC seizures?
Sleep deprivation, fatigue, and excessive alcohol consumption.
What factors can influence the occurrence of seizures in Juvenile Myoclonic Epilepsy (JME)?
Sleep deprivation and waking up.
What is the age range for Idiopathic Photosensitive Occipital Lobe Epilepsy?
6 to 18 years, typically around puberty.
What is the prognosis for Progressive Myoclonic Epilepsies (PME)?
Unfavorable prognosis with intractable seizures and mental deterioration.
What should be assessed regarding clumsiness in patients with Juvenile Myoclonic Epilepsy (JME)?
Whether they feel more clumsy in the morning compared to the afternoon or evening, and if they experience falls.
What are common symptoms of Idiopathic Photosensitive Occipital Lobe Epilepsy?
Visual auras, post-ictal headache, and possible secondary GTC.
What are some symptoms associated with Progressive Myoclonic Epilepsies?
Unsteadiness, muscle rigidity, and dexterity problems.
What is the prognosis for Idiopathic Photosensitive Occipital Lobe Epilepsy?
Good prognosis, often seizure-free on monotherapy.
What is the genetic inheritance pattern for Myoclonic Tonic-Clonic seizures?
Autosomal Dominant.
What is the age range for Primary Reading Epilepsy?
6 to 44 years, with an average of 18 years.
What is a characteristic feature of Autosomal Dominant Partial Epilepsy?
Auditory features.
What triggers seizures in Primary Reading Epilepsy?
Reading.
What is a common characteristic of familial temporal lobe epilepsies?
They can have auditory features.
What EEG findings are associated with Primary Reading Epilepsy?
3-6 Hz bilateral synchronous generalized SW discharges.
What is a common characteristic of Visual Sensitive Epilepsies?
Strictly due to photic/flicker stimulation.
What is the age range for Self-Limited Epilepsy with Centrotemporal Spikes (SLECS)?
2 - 13 years, with a peak between 5 - 10 years.
What is the gender prevalence for Self-Limited Epilepsy with Centrotemporal Spikes?
Males are more affected than females.
What is the prognosis for Self-Limited Epilepsy with Centrotemporal Spikes?
Good prognosis with spontaneous resolution by age 15.
What types of seizures are associated with Self-Limited Epilepsy with Centrotemporal Spikes?
Simple partial seizures (SPS) and focal aware seizures.
What is the typical EEG finding in Self-Limited Epilepsy with Centrotemporal Spikes?
Normal background with Rolandic dipole spikes in temporal/central regions.
What is the age range for Childhood Absence Epilepsy (CAE)?
3 - 12 years.
What is the gender prevalence for Childhood Absence Epilepsy?
Females are more affected than males.
What type of seizures are characteristic of Childhood Absence Epilepsy?
Typical absence seizures with or without complex automatisms.
What is the EEG finding during an ictal event in Childhood Absence Epilepsy?
Generalized synchronous 3 Hz spike-and-wave discharges.
What is the age range for Epilepsy with Myoclonic-Atonic Seizures?
2 - 6 years.
What is the gender prevalence for Epilepsy with Myoclonic-Atonic Seizures?
Females are more affected than males.
What types of seizures are associated with Epilepsy with Myoclonic-Atonic Seizures?
Myoclonic and atonic seizures, with no tonic seizures.
What is the typical EEG finding in Epilepsy with Myoclonic-Atonic Seizures?
Generalized spike-and-wave discharges, especially during sleep.
What is the age range for Myoclonic Encephalopathy in Nonprogressive Dravet Syndrome?
1 day to 5 years, with a peak at 12 to 17 months.
Which gender is more affected by Myoclonic Encephalopathy?
Females are more affected than males.
What is the etiology of Myoclonic Encephalopathy?
It is rare with an unknown etiology, likely genetic or chromosomal.
What are some pre-existing conditions associated with Myoclonic Encephalopathy?
Neuro impairment with encephalopathy, severe hypotonia, abnormal movements (flapping), and intellectual disability.
What is the prognosis for Myoclonic Encephalopathy?
Poor prognosis; antiepileptic medications may temporarily stop seizures but won't control abnormal EEG.
What types of seizures are common in Myoclonic Encephalopathy?
Massive myoclonus, myoclonic status, myoclonic-absence, partial motor, and rare generalized tonic-clonic or unilateral clonic seizures.
What is a characteristic EEG finding in Myoclonic Encephalopathy?
Abnormal background with difficulty in capturing due to constant movement and smiling.
In which syndrome is Myoclonic Encephalopathy commonly found?
Angelman’s syndrome.
What is a colloquial term used to describe affected females in Myoclonic Encephalopathy?
“Happy flapping girls.”
