CLTM Epilepsy disorders Exam

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