CLTM Epilepsy disorders Exam
Questions and Answers 100% Pass
Angelman Syndrome SZ onset - ✔✔1-3 years
Angelman Syndrome SZ type - ✔✔all; myoclonic and absence most common
Angelman Syndrome treatments - ✔✔difficult to control with AEDs, ketogenic diet and
VNS can be tried
ADNFLE - ✔✔Autosomal Dominant Nocturnal Frontal Lobe Epilepsy
ADNFLE sz onset - ✔✔between 1-60 years, most often before 20 yrs
ADNFLE sz type - ✔✔frequent, brief, hypermotor sz during sleep
ADNFLE is acquired: - ✔✔usually inherited
ADNFLE treatment - ✔✔AEDs: most often with OXC, CAR; 30% resistant to AEDs
ADNFLE outlook - ✔✔lifelong, but not progressive, most are intellectually normal
Benign Rolandic Epilepsy (BRE or BECTS) sz onset - ✔✔3-13 years, average 6-8 years
BRE tx - ✔✔many don't need meds; AEDs of choice: OXC, CAR, GAB, ZON, LEV, LAC
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,BRE prognosis - ✔✔szs usually stop by 15 yrs of age
CDKL5 disorder - ✔✔nervous system and cognitive development issues
mutation in Rett syndrome
F>M
CDKL5 disorder sz onset - ✔✔3-6 months
CDKL5 disorder EEG - ✔✔initial is normal or BS, hyppsathythmia may be seen later on
CDKL5 disorder sz types - ✔✔infantile spasms, myoclonic, tonic, tonic-clonic; only seen
in sleep early on
CDKL5 disorder tx - ✔✔AEDs, steroids, ketogenic diet, VNS, surgery
Childhood Absence Epilepsy sz onset - ✔✔3-11 years old, usually 5-8 years old
Childhood Absence Epilepsy tx - ✔✔ethosuximide (Zarontin) #1 choice; also, valporate,
lamitrogine
Childhood Absence Epilepsy prognosis - ✔✔in 2/3 of pts, szs resolve in adolescence,
10-15% will develop new szs (usually myoclonic or GTC)
Doose Syndrome (Myoclonic-astatic epilepsy) sz onset - ✔✔7 months-6 years; most 2-4
years
Doose Syndrome gender preferred? - ✔✔M>F
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, Part of GEFS+ (febrile szs plus) - ✔✔Doose Syndrome
Doose Syndrome sz type - ✔✔myoclonic and myoclonic followed by atonic; some also
GTC, GTC w/ fever, absence; 1/3 will have episode of NCSE
in Doose Syndrome szs often happen at what time of day? - ✔✔in the morning
Doose Syndrome tx - ✔✔does not respond well to medication
AEDs that can make Doose Syndrome worse - ✔✔Carbamezapine, oxacarbazepine,
phenytoin, vigabatrin
Doose Syndrome prognosis - ✔✔2/3 of childrens' szs may remit and have normal
intelligence; others may have mild disabilities
Doose Syndrome poor prognosis indicators - ✔✔GTC in first 2 years, abnormal EEG
background, early episodes of SE, szs when falling asleep, development of myoclonis
szs after 4 years old
Dravet syndrome onset - ✔✔onsets in infancy, often first sz w/fever
Dravet syndrome sz type - ✔✔85% myoclonic, presents between 1 and 5 years
Dravet syndrome szs are sensitive to: - ✔✔temperature, photic, stress
Dravet syndrome prognosis - ✔✔typically delayed after age 2; correlated w/sz
frequency
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Questions and Answers 100% Pass
Angelman Syndrome SZ onset - ✔✔1-3 years
Angelman Syndrome SZ type - ✔✔all; myoclonic and absence most common
Angelman Syndrome treatments - ✔✔difficult to control with AEDs, ketogenic diet and
VNS can be tried
ADNFLE - ✔✔Autosomal Dominant Nocturnal Frontal Lobe Epilepsy
ADNFLE sz onset - ✔✔between 1-60 years, most often before 20 yrs
ADNFLE sz type - ✔✔frequent, brief, hypermotor sz during sleep
ADNFLE is acquired: - ✔✔usually inherited
ADNFLE treatment - ✔✔AEDs: most often with OXC, CAR; 30% resistant to AEDs
ADNFLE outlook - ✔✔lifelong, but not progressive, most are intellectually normal
Benign Rolandic Epilepsy (BRE or BECTS) sz onset - ✔✔3-13 years, average 6-8 years
BRE tx - ✔✔many don't need meds; AEDs of choice: OXC, CAR, GAB, ZON, LEV, LAC
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,BRE prognosis - ✔✔szs usually stop by 15 yrs of age
CDKL5 disorder - ✔✔nervous system and cognitive development issues
mutation in Rett syndrome
F>M
CDKL5 disorder sz onset - ✔✔3-6 months
CDKL5 disorder EEG - ✔✔initial is normal or BS, hyppsathythmia may be seen later on
CDKL5 disorder sz types - ✔✔infantile spasms, myoclonic, tonic, tonic-clonic; only seen
in sleep early on
CDKL5 disorder tx - ✔✔AEDs, steroids, ketogenic diet, VNS, surgery
Childhood Absence Epilepsy sz onset - ✔✔3-11 years old, usually 5-8 years old
Childhood Absence Epilepsy tx - ✔✔ethosuximide (Zarontin) #1 choice; also, valporate,
lamitrogine
Childhood Absence Epilepsy prognosis - ✔✔in 2/3 of pts, szs resolve in adolescence,
10-15% will develop new szs (usually myoclonic or GTC)
Doose Syndrome (Myoclonic-astatic epilepsy) sz onset - ✔✔7 months-6 years; most 2-4
years
Doose Syndrome gender preferred? - ✔✔M>F
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, Part of GEFS+ (febrile szs plus) - ✔✔Doose Syndrome
Doose Syndrome sz type - ✔✔myoclonic and myoclonic followed by atonic; some also
GTC, GTC w/ fever, absence; 1/3 will have episode of NCSE
in Doose Syndrome szs often happen at what time of day? - ✔✔in the morning
Doose Syndrome tx - ✔✔does not respond well to medication
AEDs that can make Doose Syndrome worse - ✔✔Carbamezapine, oxacarbazepine,
phenytoin, vigabatrin
Doose Syndrome prognosis - ✔✔2/3 of childrens' szs may remit and have normal
intelligence; others may have mild disabilities
Doose Syndrome poor prognosis indicators - ✔✔GTC in first 2 years, abnormal EEG
background, early episodes of SE, szs when falling asleep, development of myoclonis
szs after 4 years old
Dravet syndrome onset - ✔✔onsets in infancy, often first sz w/fever
Dravet syndrome sz type - ✔✔85% myoclonic, presents between 1 and 5 years
Dravet syndrome szs are sensitive to: - ✔✔temperature, photic, stress
Dravet syndrome prognosis - ✔✔typically delayed after age 2; correlated w/sz
frequency
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