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