INSTRUCTIONS READ EACH QUESTION GIVE IT YOUR BEST SHOT GOOD LUCK! Israel Alfonso, MD.

INSTRUCTIONS READ EACH QUESTION GIVE IT YOUR BEST SHOT GOOD LUCK! Israel Alfonso, MD

1. INFANCY INCLUDES FROM: A. BIRTH TO 1 MONTH OF AGE B. 1 MONTH TO 1 YEAR OF AGE C. 1 YEAR TO 6 YEARS OF AGE D. 6 YEARS TO 12 YEARS OF AGE

SHOW ME THE QUESTION AGAIN GO TO THE NEXT QUESTION I WANT TO SEE THE CLUE, ANYWAY

I WANT TO TRY AGAIN JUST GIVE ME THE ANSWER GIVE ME A CLUE

CLASSIFICATION OF EPILEPSY BY AGE NEONATAL PERIOD (UP TO 1 MONTH) INFANCY (1 MONTH TO 1 YEAR) EARLY CHILDHOOD (1 YEAR TO 6 YEARS) LATE CHILDHOOD (FROM 6 TO 13 YEARS) ADOLESCENCE (FROM 13 TO 20 YEARS)

1. INFANCY INCLUDES FROM: A. BIRTH TO 1 MONTH OF AGE B. 1 MONTH TO 1 YEAR OF AGE C. 1 YEAR TO 6 YEARS OF AGE D. 6 YEARS TO 12 YEARS OF AGE

2. SYNDROMIC CLASSIFICATION OF SEIZURES IS BASED ON: A. AGE B. COURSE C. SEIZURE TYPE D. ALL OF THE ABOVE

CLASSIFICATION OF EPILEPSY AGE NEONATES INFANTS CHILDHOOD EARLY LATE ADOLESCENCE

CLASSIFICATION OF EPILEPSY SEIZURE TYPE GENERALIZED FRONTAL PARTIAL PARIETAL TEMPORAL OCCIPITAL SYNDROME ? YES N0 INSULA

CLASSIFICATION OF EPILEPSY AGE NEONATES INFANTS CHILDHOOD EARLY LATE ADOLESCENCE SYNDROME ? YES N0

CLASSIFICATION OF EPILEPSY CAUSE INHERITED OTHERS SYNDROME ? YES N0

CLASSIFICATION OF EPILEPSY COURSE GOOD BAD SYNDROME ? YES N0

2. SYNDROMIC CLASSIFICATION OF SEIZURES IS BASED ON: A. AGE B. COURSE C. SEIZURE TYPE D. ALL OF THE ABOVE

3. SYNDROMIC CLASSIFICATION OF SEIZURES IS BETTER THAN OTHER CLASSIFICATIONS: A. TRUE B. FALSE

SYNDROME AGE SEIZURE TYPE CAUSE COURSE CLINICAL UNKNOWN FAMILIAL OTHERS EEG EFFECTIVE AED NEUROLOGICAL EXAM NORMALABNORMAL IF YOU KNOW 2 FACTORS, YOU KNOW ALL

3. SYNDROMIC CLASSIFICATION OF SEIZURES IS BETTER THAN OTHER: A. TRUE B. FALSE

4. WHICH OF THE FOLLOWING SEIZURE TYPES IS NOT FREQUENTLY ENCOUNTERED IN NEONATES? A. GENERALIZED SEIZURES B. FOCAL SEIZURES C. MULTIFOCAL SEIZURES D. MYOCLONIC SEIZURES E. SUBTLE SEIZURES

THIS IS A VERY RARE TYPE OF SEIZURES THIS IS A VERY FREQUENT TYPE OF SEIZURES (IT IS A LONG VIDEO, LOOK AT LEFT FOOT)

4. WHICH OF THE FOLLOWING SEIZURE TYPES IS NOT FREQUENTLY ENCOUNTERED IN NEONATES? A. GENERALIZED SEIZURES B. FOCAL SEIZURES C. MULTIFOCAL SEIZURES D. MYOCLONIC SEIZURES E. SUBTLE SEIZURES

5. ALL SEIZURES IN NEONATES ARE EPILEPTIC. A. TRUE B. FALSE

CLINICAL SEIZURES ELECTRICAL SEIZURES EPILEPTICNONEPILEPTIC CLASSIFICATION OF SEIZURES NO ANTIEPILEPTIC DRUGS

5. ALL SEIZURES IN NEONATES ARE EPILEPTIC. A. TRUE B. FALSE

6. AICARDI SYNDROME IS CHARACTERIZED BY ALL OF THE FOLLOWING, EXCEPT: A. COLOBOMAS OF THE IRIS B. RETINAL LACUNAE C. ABSENCE OF THE CORPUS CALLOSUM D. TESTICULAR TUMOR

AICARDI SYNDROME AGENESIS OF THE CORPUS CALLOSUM RETINAL LACUNAE

6. AICARDI SYNDROME IS CHARACTERIZED BY ALL OF THE FOLLOWING, EXCEPT: A. COLOBOMAS OF THE IRIS B. RETINAL LACUNAE C. ABSENCE OF THE CORPUS CALLOSUM D. TESTICULAR TUMOR

7. A NEONATE WITH HYPOCALCEMIC SEIZURES RESISTANT TO CALCIUM SHOULD MAKE YOU THINK OF: A. HYPOMAGNESEMIA B. DIGEORGE SYNDROME C. TUBEROUS SCLEROSIS D. INCONTINENTIA PIGMENTI

INTRAMUSCULAR MAGNESIUM 50 % SOLUTION (MgSO ) 4 0.2 mL/kg

7. A NEONATE WITH HYPOCALCEMIC SEIZURES RESISTANT TO CALCIUM SHOULD MAKE YOU THINK OF: A. HYPOMAGNESEMIA B. DIGEORGE SYNDROME C. TUBEROUS SCLEROSIS D. INCONTINENTIA PIGMENTI

8. HYPOCALCEMIA IN AN INTERICTALLY ALERT GOOD LOOKING LEAN NEONATES IS LIKELY TO BE DO TO A. HIGH-PHOSPHATE FEEDINGS B. DIGEORGE SYNDROME C. MATERNAL DIABETES D. HYPOMAGNESEMIA

9. A YELLOWISH (XANTHOCHROMATIC) SUPERNATANT CSF SUGGESTS ALL OF THE FOLLOWING, EXCEPT: A. JAUNDICE B. SUBARACHNOID BLEED C. INTRAVENTRICULAR BLEED D. XANTHINE OXIDASE DEFICIENCY

CSF JAUNDICE SUBARACHNOID BLEED INTRAVENTRICULAR BLEED CAROTINEMIA

9. A YELLOWISH (XANTHOCHROMATIC) SUPERNATANT CSF SUGGESTS ALL OF THE FOLLOWING, EXCEPT: A. JAUNDICE B. SUBARACHNOID BLEED C. INTRAVENTRICULAR BLEED D. XANTHINE OXIDASE DEFICIENCY

10. FAILURE TO RESPOND TO 200 MG OF IV PYRIDOXINE EXCLUDES THE POSSIBILITY OF PYRIDOXINE RESPONSIVE SEIZURES. A. TRUE B. FALSE

PYRIDOXINE DEPENDENCY MONITOR PATIENT, MAY STOP BREATHING IT MAY TAKE HOURS FOR EEG TO BE NORMAL (NOT SECONDS) FAILURE OF IV; TRY 20 MG/D PO FOR 6 WK

10. FAILURE TO RESPOND TO 200 MG OF IV PYRIDOXINE EXCLUDES THE POSSIBILITY OF PYRIDOXINE RESPONSIVE SEIZURES. A. TRUE B. FALSE

11. WHICH OF THE FOLLOWING SYNDROME DO NOT OCCUR IN THE NEONATAL PERIOD A. BENIGN FAMILIAL NEONATAL SEIZURES B. OHTAHARA SYNDROME C. LANDAU-KLEFFNER SYNDROME D. GELASTIC SEIZURES

EPILEPSY SYNDROMES OF NEONATAL - ONSET BENIGN NEONATAL CONVULSIONS BENIGN NEONATAL FAMILIAL CONVULSIONS OHTAHARA SYNDROME (EARLY INFANTILE EPILEPTIC ENCEPHALOPATHY WITH BURST SUPPRESSION) GELASTIC SEIZURES * * HYPOGLYCEMIA, HYPOCALCEMIA, PYRIDOXINE, FOLINIC ACID, MENINGITIS, HYPERAMMONEMIA, BIOTINIDASE, STROKE, BRAIN MALFORMATION MAPLE SYRUP URINE DISEASE

11. WHICH OF THE FOLLOWING SYNDROME DO NOT OCCUR IN THE NEONATAL PERIOD A. BENIGN FAMILIAL NEONATAL SEIZURES B. OHTAHARA SYNDROME C. LANDAU-KLEFFNER SYNDROME D. GELASTIC SEIZURES

12. A 5-DAY-OLD NEONATE STARTS HAVING SEIZURES, NEUROLOGICAL EXAMINATION IS NORMAL. THE MOST LIKELY DIAGNOSIS IS BENIGN NEONATAL SEIZURES. A. TRUE B. FALSE

SEIZURE TYPE: EEG: TREATMENT: BENIGN NEONATAL CONVULSIONS CLONIC, APNEA, NEVER TONIC THETA POINTU ALTERNANT (NOT SPECIFIC) DIAGNOSIS OF EXCLUSION ONSET OF SEIZURES AT 5 DAYS OF AGE (FIFTH DAY FITS) NORMAL NEUROLOGICAL EXAMINATION NO ETIOLOGY (LOOK HARD FOR ONE) PHENOBARBITAL

BENIGN NEONATAL CONVULSIONS AGE SEIZURE TYPE CAUSE GOOD CLINICAL UNKNOWN FAMILIAL OTHERS EEG PHENOBARBITAL NEUROLOGICAL EXAM NORMALABNORMAL < 1M NORMALNOT TONIC

12. A 5-DAY-OLD NEONATE STARTS HAVING SEIZURES, NEUROLOGICAL EXAMINATION IS NORMAL. THE MOST LIKELY DIAGNOSIS IS BENIGN NEONATAL SEIZURES. A. TRUE B. FALSE BENIGN NEONATAL SEIZURES (5 DAY SEIZURES) IS A DIAGNOSIS OF EXCLUSION

13. BENIGN FAMILIAL NEONATAL SEIZURES ARE CHARACTERIZED BY ALL OF THE FOLLOWING EXCEPT: A. NORMAL EEG BACKGROUND B. NO DETECTABLE CAUSE C. POSITIVE FAMILY HISTORY OF SEIZURE DURING THE NEONATAL PERIOD D. LINKED TO CHROMOSOME 15

SEIZURE TYPE: EEG: TREATMENT: BENIGN NEONATAL FAMILIAL CONVULSIONS CLONIC, APNEA, NEVER TONIC NONE DIAGNOSIS: FAMILY HISTORY OF NEONATAL CONVULSION ONSET OF SEIZURES AT 2 TO 3 DAYS OF AGE ( <4 WEEKS) NORMAL NEUROLOGICAL EXAMINATION NO ETIOLOGY PHENOBARBITAL IN FIRST DEGREE RELATIVES WITH NORMAL DEVELOPMENT (14 % MAY HAVE SEIZURES AS ADULTS BUT NO MENTAL RETARDATION) AUTOSOMAL DOMINANT, LINKAGE: 20p AND 8p SEIZURES STOP USUALLY BY 6 WEEKS

BENIGN FAMILIAL NEONATAL CONVULSIONS AGE SEIZURE TYPE CAUSE GOOD CLINICAL UNKNOWN FAMILIAL OTHERS EEG PHENOBARBITAL NEUROLOGICAL EXAM NORMALABNORMAL < 1M NORMALNOT TONIC

13. BENIGN FAMILIAL NEONATAL SEIZURES ARE CHARACTERIZED BY ALL OF THE FOLLOWING EXCEPT: A. NORMAL EEG BACKGROUND B. NO DETECTABLE CAUSE C. POSITIVE FAMILY HISTORY OF SEIZURE DURING THE NEONATAL PERIOD D. LINKED TO CHROMOSOME 15

14. A 3-DAY-OLD NEONATE STARTS HAVING SEIZURES, NEUROLOGICAL EXAMINATION IS NORMAL; THE FATHER, WHO IS MENTALLY RETARDED ALSO HAD SEIZURES AS A NEONATE. THE MOST LIKELY DIAGNOSIS IS BENIGN FAMILIAL NEONATAL CONVULSIONS. A. TRUE B. FALSE

BENIGN FAMILIAL NEONATAL CONVULSIONS AGE SEIZURE TYPE CAUSE GOOD CLINICAL UNKNOWN FAMILIAL OTHERS EEG PHENOBARBITAL NEUROLOGICAL EXAM NORMALABNORMAL < 1M NORMALNOT TONIC

14. A 3-DAY-OLD NEONATE STARTS HAVING SEIZURES, NEUROLOGICAL EXAMINATION IS NORMAL; THE FATHER, WHO IS MENTALLY RETARDED ALSO HAD SEIZURES AS A NEONATE. THE MOST LIKELY DIAGNOSIS IS BENIGN FAMILIAL NEONATAL CONVULSIONS. A. TRUE B. FALSE

15. FAMILIAL BENIGN NEONATAL CONVULSION IS LINKED TO CHROMOSOMES: A. 7 AND 4 B. 16 AND 12 C. 20 AND 8 D. 23 AND 58

16. FAMILIAL BENIGN NEONATAL CONVULSION IS A _______ DISORDER. A. SEX LINKED RECESSIVE B. AUTOSOMAL RECESSIVE C. AUTOSOMAL DOMINANT D. SEX LINKED DOMINANT

17. WHAT EEG PATTERN IS CHARACTERISTIC OF OHTAHARA SYNDROME? A. BURST SUPPRESSION PATTERN B. GENERALIZED 3-PER-SECOND SPIKE AND WAVE C. HYPSARHYTHMIA D. FOCAL SHARP WAVES IN THE CENTROTEMPORAL REGION

OHTAHARA SYNDROME AGE SEIZURE TYPE CAUSE COURSE CLINICAL UNKNOWN FAMILIAL OTHERS EEG AED ARE INEFFECTIVE < 1M BURST SUPPRESSION PATTERN POOR

OHTAHARA SYNDROME BURST SUPPRESSION PATTERN BURST SUPPRESSION BURST

17. WHAT EEG PATTERN IS CHARACTERISTIC OF OHTAHARA SYNDROME? A. BURST SUPPRESSION PATTERN B. GENERALIZED 3-PER-SECOND SPIKE AND WAVE C. HYPSARHYTHMIA D. FOCAL SHARP WAVES IN THE CENTROTEMPORAL REGION

18. OHTAHARA SYNDROME IS A BENIGN CONDITION. A. TRUE B. FALSE

SEIZURE TYPE: EEG: SEARCH FOR ETIOLOGY: WOODS LAMP, METABOLIC DISORDERS (BIOTINIDASE), TRIALS OF B6 & FOLINIC ACID; ANTIEPILEPTIC DRUGS NOT EFFECTIVE, BUT TRY OHTAHARA SYNDROME (EARLY INFANTILE EPILEPTIC ENCEPHALOPATHY WITH BURST SUPPRESSION) TONIC AND OTHERS BURST SUPPRESSION (ESTABLISHES DIAGNOSIS) VARIETIES: IDIOPATHIC (RARE) SYMPTOMATIC CRYPTOGENIC EXAMCAUSE NEGATIVENONE ABNORMALNONE PRESENTEITHER PROGNOSIS: POOR

18. OHTAHARA SYNDROME IS A BENIGN CONDITION. A. TRUE B. FALSE

19. GIGGLING IN A NEONATE CAN BE A SIGN OF A. MENINGITIS B. HYPOTHALAMIC HAMARTOMA C. TEMPORAL LOBE SCLEROSIS

SEIZURE TYPE: EEG: TREATMENT: GELASTIC SEIZURES BRIEF EPISODES OF PLEASANT LAUGHTER AND GIGGLING, FACIAL FLUSHING AND MYDRIASIS NORMAL, EVEN DURING EPISODES SURGERY, PHENOBARBITAL ETIOLOGY: HYPOTHALAMIC HAMARTOMA

19. GIGGLING IN A NEONATE CAN BE A SIGN OF A. MENINGITIS B. HYPOTHALAMIC HAMARTOMA C. TEMPORAL LOBE SCLEROSIS

20. WHICH OF THE FOLLOWING EPILEPTIC SYNDROMES DO NOT USUALLY OCCUR DURING INFANCY? A. FEBRILE SEIZURES B. WEST SYNDROME C. TYPICAL MYOCLONIC EPILEPSY OF EARLY CHILDHOOD D. MYOCLONIC EPILEPSY OF JANZ

EPILEPSY SYNDROMES OF ONSET - DURING INFANCY WEST SYNDROME (INFANTILE SPASM) FEBRILE SEIZURES BENIGN MYOCLONIC EPILEPSY OF INFANCY

20. WHICH OF THE FOLLOWING EPILEPTIC SYNDROMES USUALLY OCCUR DURING INFANCY? A. FEBRILE SEIZURES B. WEST SYNDROME C. TYPICAL MYOCLONIC EPILEPSY OF EARLY CHILDHOOD D. MYOCLONIC EPILEPSY OF JANZ

21. A 2-MINUTE SEIZURE IN A 3-YEAR- OLD WITH MENINGITIS AND HIGH FEVER MAY SHOULD BE CLASSIFIED AS: A. A SIMPLE FEBRILE SEIZURES B. A COMPLEX FEBRILE SEIZURES C. NONE OF THE ABOVE

SIMPLE FEBRILE SEIZURES FEBRILE SEIZURES SEIZURE THAT OCCURS BETWEEN 3 MONTHS AND 5 YEARS OF AGE (PEAK 14-18 M), ASSOCIATED WITH FEVER, WITHOUT INTRACRANIAL DEMONSTRABLE PATHOLOGY LESS THAN 15 MINUTES GENERALIZED NO RECURRENCE IN 48 HOURS NORMAL NEUROLOGICAL EXAMINATION COMPLEX FEBRILE SEIZURES IF NOT SIMPLE TREATMENT: TEMPERATURE CONTROL DIAZEPAM (PO): 0.33 MG/KG Q 8 HOURS DIASTAT 2.5;5;10 MG (RECTAL): < 10 MG PHENOBARBITAL (PO): 3-5 MG/KG/D AGE/Y MG/KG 2-5 0.5 5-11 0.3 >12 0.2 DIASTAT

21. A 2-MINUTE SEIZURE IN A 3-YEAR- OLD WITH MENINGITIS AND HIGH FEVER MAY CAN BE CLASSIFIED AS: A. A SIMPLE FEBRILE SEIZURES B. A COMPLEX FEBRILE SEIZURES C. NONE OF THE ABOVE

22. AT WHAT AGE ARE FEBRILE SEIZURES MOST FREQUENT? A. 6 TO 10 MONTHS B. 14 TO 18 MONTHS C. 24 TO 28 MONTHS D. 36 TO 40 MONTHS

SIMPLE FEBRILE SEIZURES FEBRILE SEIZURES SEIZURE THAT OCCURS BETWEEN 3 MONTHS AND 5 YEARS OF AGE (PEAK 14-18 M), ASSOCIATED WITH FEVER, WITHOUT INTRACRANIAL DEMONSTRABLE PATHOLOGY LESS THAN 15 MINUTES GENERALIZED NO RECURRENCE IN 48 HOURS NORMAL NEUROLOGICAL EXAMINATION COMPLEX FEBRILE SEIZURES IF NOT SIMPLE TREATMENT: TEMPERATURE CONTROL DIAZEPAM (PO): 0.33 MG/KG Q 8 HOURS DIASTAT 2.5;5;10 MG (RECTAL): < 10 MG PHENOBARBITAL (PO): 3-5 MG/KG/D AGE/Y MG/KG 2-5 0.5 5-11 0.3 >12 0.2 DIASTAT

22. AT WHAT AGE ARE FEBRILE SEIZURES MOST FREQUENT? A. 6 TO 10 MONTHS B. 14 TO 18 MONTHS C. 24 TO 28 MONTHS D. 36 TO 40 MONTHS

23. THE FEBRILE SEIZURE GENE HAS BEEN MAPPED TO CHROMOSOME 19p AND 8q13- 21 IN SOME FAMILIES. A. TRUE B. FALSE

SIMPLE FEBRILE SEIZURES FEBRILE SEIZURES IN LARGE FAMILIES LINKED TO 19 AND 8. SOMETIMES AUTOSOMAL DOMINANT. AFFECT 3 TO 4 % OF KIDS LESS THAN 15 MINUTES GENERALIZED NO RECURRENCE IN 48 HOURS NORMAL NEUROLOGICAL EXAMINATION COMPLEX FEBRILE SEIZURES IF NOT SIMPLE TREATMENT: TEMPERATURE CONTROL DIAZEPAM (PO): 0.33 MG/KG Q 8 HOURS DIASTAT 2.5;5;10 MG (RECTAL): < 10 MG PHENOBARBITAL (PO): 3-5 MG/KG/D AGE/Y MG/KG 2-5 0.5 5-11 0.3 >12 0.2 DIASTAT THINK, CNS PROBLEMS

23. THE FEBRILE SEIZURE GENE HAS BEEN MAPPED TO CHROMOSOME 19p AND 8q13- 21 IN SOME FAMILIES. A. TRUE B. FALSE

24. ORGANIC CNS PATHOLOGY IS MORE LIKELY IN PATIENTS WITH: A. BENIGN ROLANDIC EPILEPSY B. SIMPLE FEBRILE SEIZURE C. EPILEPSY OF JANZ D. COMPLEX FEBRILE SEIZURES

SIMPLE FEBRILE SEIZURES FEBRILE SEIZURES IN LARGE FAMILIES LINKED TO 19 AND 8. SOMETIMES AUTOSOMAL DOMINANT. AFFECT 3 TO 4 % OF KIDS LESS THAN 15 MINUTES GENERALIZED NO RECURRENCE IN 48 HOURS NORMAL NEUROLOGICAL EXAMINATION COMPLEX FEBRILE SEIZURES IF NOT SIMPLE TREATMENT: TEMPERATURE CONTROL DIAZEPAM (PO): 0.33 MG/KG Q 8 HOURS DIASTAT 2.5;5;10 MG (RECTAL): < 10 MG PHENOBARBITAL (PO): 3-5 MG/KG/D AGE/Y MG/KG 2-5 0.5 5-11 0.3 >12 0.2 DIASTAT THINK, CNS PROBLEMS

24. ORGANIC CNS PATHOLOGY SUCH AS INFECTIONS OR TOXIC PROCESS IS MORE LIKELY IN PATIENTS WITH: A. BENIGN ROLANDIC EPILEPSY B. SIMPLE FEBRILE SEIZURE C. EPILEPSY OF JANZ D. COMPLEX FEBRILE SEIZURES

25. ALL OF THE FOLLOWING ARE RISK FACTORS FOR EPILEPSY AS A COMPLICATION OF FEBRILE SEIZURES, EXCEPT: A. COMPLEX FEBRILE SEIZURES B. ONSET BEFORE 9 MONTHS OF AGE C. OCCURRENCE DURING LOW- GRADE TEMPERATURE D. FAMILY HISTORY OF EPILEPSY

NO RISK FACTORS FEBRILE SEIZURES DURATION: MORE THAN 15 MINUTES FOCAL RECURRENCE IN 48 HOURS ABNORMAL NEUROLOGICAL EXAMINATION EPILEPSY: 1% RISK FACTORS FAMILY HISTORY OF EPILEPSY DEVELOPMENTAL DELAY THE PRESENCE OF SEVERAL OF THIS FACTORS MAKES THE RISK OF EPILEPSY: 9%

25. ALL OF THE FOLLOWING ARE RISK FACTORS FOR EPILEPSY AS A COMPLICATION OF FEBRILE SEIZURES, EXCEPT: A. COMPLEX FEBRILE SEIZURES B. ONSET BEFORE 9 MONTHS OF AGE C. OCCURRENCE DURING LOW- GRADE TEMPERATURE D. FAMILY HISTORY OF EPILEPSY

26. AN EEG IS INDICATED IN PATIENTS WITH THE FIRST SIMPLE FEBRILE SEIZURE. A. TRUE B. FALSE

27. AN EFFECTIVE AND SAFE METHOD TO REDUCE FEBRILE SEIZURES IS: A. RECTAL DIAZEPAM AT THE ONSET OF FEVER B. ORAL DIAZEPAM AT THE ONSET OF FEVER C. RECTAL VALPROATE ACID AT THE ONSET OF FEVER D. DAILY ORAL PHENOBARBITAL

SIMPLE FEBRILE SEIZURES FEBRILE SEIZURES IN LARGE FAMILIES LINKED TO 19 AND 8. SOMETIMES AUTOSOMAL DOMINANT. AFFECT 3 TO 4 % OF KIDS LESS THAN 15 MINUTES GENERALIZED NO RECURRENCE IN 48 HOURS NORMAL NEUROLOGICAL EXAMINATION COMPLEX FEBRILE SEIZURES IF NOT SIMPLE TREATMENT: TEMPERATURE CONTROL DIAZEPAM (PO): 0.33 MG/KG Q 8 HOURS FROM FEVER ONSET TO FINISH DIASTAT 2.5;5;10 MG (RECTAL): < 10 MG PHENOBARBITAL (PO): 3-5 MG/KG/D AGE/Y MG/KG 2-5 0.5 5-11 0.3 >12 0.2 DIASTAT THINK, CNS PROBLEMS

27. AN EFFECTIVE AND SAFE METHOD TO REDUCE FEBRILE SEIZURES IS: A. RECTAL DIAZEPAM AT THE ONSET OF FEVER B. ORAL DIAZEPAM AT THE ONSET OF FEVER C. RECTAL VALPROATE ACID AT THE ONSET OF FEVER D. DAILY ORAL PHENOBARBITAL

28. THE FOLLOWING STATEMENTS ABOUT TYPICAL MYOCLONIC EPILEPSY OF EARLY CHILDHOOD ARE TRUE, EXCEPT: A. OCCURS BETWEEN 6 MONTHS AND 4 YEARS OF AGE B. MENTAL RETARDATION IS FREQUENT C. LANGUAGE AND EMOTIONAL PROBLEMS ARE FREQUENT D. FAMILY HISTORY OF EPILEPSY IN A THIRD OF CASES

TYPICAL MYOCLONIC EPILEPSY OF EARLY CHILDHOOD NORMAL EEG BACKGROUND FAST SPIKE WAVES COMPLEX > 2.5 Hz 50 % ARE SEIZURE FREE AFTER FEW YEARS 33 % POSITIVE FAMILY HISTORY OFTEN DEVELOP PROBLEMS WITH: LEARNING AND LANGUAGE EMOTIONS AND BEHAVIOR MENTAL RETARDATION IS RARE

SEIZURE TYPE: EEG: TREATMENT: TYPICAL MYOCLONIC EPILEPSY OF EARLY CHILDHOOD UPPER LIMB MYOCLONUS, AWAKE AND SLEEP GB: NORMAL, ICTAL GENERALIZED SPIKE & WAVE DIAGNOSIS: EXCLUDE SLEEP MYOCLONUS (NO ICTAL ONSET OF SEIZURES: 6 MONTHS TO 3 YEARS OF AGE NORMAL NEUROLOGICAL EXAMINATION NO ETIOLOGY VALPROIC ACID ? FAMILY HISTORY OF SEIZURES (SOMETIMES) EEG CHANGES)

28. THE FOLLOWING STATEMENTS ABOUT TYPICAL MYOCLONIC EPILEPSY OF EARLY CHILDHOOD ARE TRUE, EXCEPT: A. OCCURS BETWEEN 6 MONTHS AND 4 YEARS OF AGE B. MENTAL RETARDATION IS FREQUENT C. LANGUAGE AND EMOTIONAL PROBLEMS ARE FREQUENT D. FAMILY HISTORY OF EPILEPSY IN A THIRD OF CASES

29. ALL THE SPASMS IN INFANTS WITH INFANTILE SPASM LOOK THE SAME. A. TRUE B. FALSE

WEST SYNDROME (INFANTILE SPASM) GENERALIZED FLEXOR SPASM PARTIAL LEFT ARM SPASM HIP FLEXION SPASMHEAD DROP

29. ALL THE SPASMS IN INFANTS WITH INFANTILE SPASM LOOK THE SAME. A. TRUE B. FALSE

30. ALL OF THE FOLLOWING ARE POSSIBLE CAUSES OF WEST SYNDROME, EXCEPT: A. TUBEROUS SCLEROSIS B. FEBRILE SEIZURES C. A NEONATAL STROKE D. HYPOXIC ISCHEMIC ENCEPHALOPATHY

SEIZURE TYPE: EEG: SEARCH FOR ETIOLOGY: WOODS LAMP, METABOLIC DISORDERS (BIOTINIDASE), TRIALS OF B6 & FOLINIC ACID; ANTIEPILEPTIC DRUGS EFFECTIVE (ACTH, VPA, VIGABATRIN [BLINDNESS] FOR TUBEROUS SCLEROSIS); FREQUENTLY HISTORY OF OHTAHARA SYNDROME WEST SYNDROME (INFANTILE SPASM) FLEXOR SPASM, EXTENSION SPASM, OTHERS (SOMETIMES PRECEDED BY CRY) VARIETIES: IDIOPATHIC (RARE) SYMPTOMATIC CRYPTOGENIC EXAMCAUSE NEGATIVENONE ABNORMALNONE PRESENTEITHER HYPSARHYTHMIA (ESTABLISHES DIAGNOSIS) PROGNOSIS: POOR NOT PRODUCED BY IMMUNIZATIONS! *

SEIZURE TYPE: EEG: WEST SYNDROME (INFANTILE SPASM) FLEXOR SPASM, EXTENSION SPASM, OTHERS (SOMETIMES PRECEDED BY CRY) VARIETIES AS PER NELSON SYMPTOMATICCRYPTOGENIC NEURO EXAM NORMAL IF NOT CRYPTOGENIC PREGNANCY BAD HYPSARHYTHMIA (ESTABLISHES DIAGNOSIS) PROGNOSIS * NORMAL BIRTH HISTORY DEVELOPMENT BRAIN CT & MRI RISK FACTORS NORMAL NONE GOOD

30. ALL OF THE FOLLOWING ARE POSSIBLE CAUSES OF WEST SYNDROME, EXCEPT: A. TUBEROUS SCLEROSIS B. FEBRILE SEIZURES C. A NEONATAL STROKE D. HYPOXIC ISCHEMIC ENCEPHALOPATHY

31. ALL OF THE FOLLOWING ARE POSSIBLE CAUSES OF WEST SYNDROME, EXCEPT: A. PYRIDOXINE DEPENDENCY B. LISSENCEPHALY C. CONGENITAL INFECTIONS D. IMMUNIZATIONS

32. A THERAPEUTIC TRIAL OF B6 AND FOLINIC ACID SHOULD BE INSTITUTED IN ALL INFANTS WITH WEST SYNDROME. A. TRUE B. FALSE

32. A THERAPEUTIC TRIAL OF B6 AND FOLINIC ACID SHOULD BE INSTITUTED IN ALL INFANTS WITH WEST SYNDROME. A. TRUE B. FALSE (NOT IF AN ALTERNATIVE CAUSE IS KNOW)

33. THE DIFFERENTIAL DIAGNOSIS OF MYOCLONIC ACTIVITY IN A NEUROLOGICALLY NORMAL INFANT INCLUDES: A. BENIGN MYOCLONIC EPILEPSY OF EARLY CHILDHOOD B. WEST SYNDROME C. SLEEP MYOCLONUS D. ALL OF THE ABOVE

SEIZURE TYPE: EEG: TREATMENT: TYPICAL MYOCLONIC EPILEPSY OF EARLY CHILDHOOD UPPER LIMB MYOCLONUS, AWAKE AND SLEEP GB: NORMAL, ICTAL GENERALIZED SPIKE & WAVE DIAGNOSIS: EXCLUDE SLEEP MYOCLONUS (NO ICTAL ONSET OF SEIZURES: 6 MONTHS TO 3 YEARS OF AGE NORMAL NEUROLOGICAL EXAMINATION NO ETIOLOGY VALPROIC ACID ? FAMILY HISTORY OF SEIZURES (SOMETIMES) EEG CHANGES)

33. THE DIFFERENTIAL DIAGNOSIS OF MYOCLONIC ACTIVITY IN A NEUROLOGICALLY NORMAL INFANT INCLUDES: A. BENIGN MYOCLONIC EPILEPSY OF EARLY CHILDHOOD B. WEST SYNDROME C. SLEEP MYOCLONUS D. ALL OF THE ABOVE

34. WHAT EEG PATTERN IS CHARACTERISTIC OF WEST SYNDROME? A. BURST SUPPRESSION PATTERN B. GENERALIZED 3-PER-SECOND SPIKE AND WAVE C. HYPSARHYTHMIA D. FOCAL SHARP WAVES IN THE CENTROTEMPORAL REGION

34. WHAT EEG PATTERN IS CHARACTERISTIC OF WEST SYNDROME? A. BURST SUPPRESSION PATTERN B. GENERALIZED 3 PER SECOND SPIKE AND WAVE C. HYPSARHYTHMIA D. FOCAL SHARP WAVES IN THE CENTROTEMPORAL REGION

35. ALL OF THE FOLLOWING SYNDROMES MAY PRESENT DURING EARLY CHILDHOOD, EXCEPT A. LENNOX GASTAUT SYNDROME B. LANDAU KLEFFNER SYNDROME C. SLOW SLEEP STATUS D. OHTAHARA SYNDROME

EPILEPSY SYNDROMES OF ONSET - DURING EARLY CHILDHOOD LENNOX-GASTAUT SYNDROME LANDAU-KLEFFNER SYNDROME SLOW SLEEP STATUS SYNDROME TYPICAL MYOCLONIC EPILEPSY OF EARLY INFANCY

35. ALL OF THE FOLLOWING SYNDROMES MAY PRESENT DURING EARLY CHILDHOOD, EXCEPT A. LENNOX GASTAUT SYNDROME B. LANDAU KLEFFNER SYNDROME C. SLOW SLEEP STATUS D. OHTAHARA SYNDROME

36. WHICH OF THE SEIZURE TYPES ARE MOST PREVALENT CHILDREN WITH LENNOX GASTAUT SYNDROME ? A. ABSENCE OR HEAD DROPS B. RIGHT ARM PARTIAL SEIZURES C. LEFT ARM CONVULSIONS D. GENERALIZED TONIC-CLONIC SEIZURES

LENNOX-GASTAUT SYNDROME PARTIAL SEIZURE HEAD DROPDROP ATTACK

36. WHICH OF THE SEIZURE TYPES ARE MOST PREVALENT CHILDREN WITH LENNOX GASTAUT SYNDROME ? A. ABSENCE OR HEAD DROPS B. RIGHT ARM PARTIAL SEIZURES C. LEFT ARM CONVULSIONS D. GENERALIZED TONIC-CLONIC SEIZURES

37. MOST CHILDREN WITH LENNOX GASTAUT SYNDROME ARE: A. MENTALLY RETARDED B. AUTISTIC C. HEMIPLEGIC D. ALL OF THE ABOVE

LENNOX-GASTAUT SYNDROME PARTIAL SEIZURE HEAD DROPDROP ATTACK

37. MOST CHILDREN WITH LENNOX GASTAUT SYNDROME ARE: A. MENTALLY RETARDED B. AUTISTIC C. HEMIPLEGIC D. ALL OF THE ABOVE

38. LENNOX GASTAUT SYNDROME IN A CHILD WITH A NORMAL NEUROLOGICAL EXAMINATION, NORMAL DEVELOPMENT AND MULTIPLE HYPOPIGMENTED SPOTS SHOULD BE CLASSIFIED AS: A. CRYPTOGENIC B. SYMPTOMATIC C. IDIOPATHIC

EEG: SEARCH FOR ETIOLOGY: WOODS LAMP, METABOLIC DISORDERS, TRIAL OF B6; ANTIEPILEPTIC DRUGS INEFFECTIVE, BUT TRY! FREQUENTLY HISTORY OF WEST SYNDROME LENNOX-GASTAUT SYNDROME SEIZURE TYPE: ABSENCE, ATONIC & MYOCLONIC FITS VARIETIES: IDIOPATHIC (RARE) SYMPTOMATIC CRYPTOGENIC EXAMCAUSE NEGATIVENONE ABNORMALNONE PRESENTEITHER SPIKE OR POLYSPIKE AND WAVES (1 TO 2.5 Hz) FREQUENT STATUS EPILEPTICUS GENERALIZED (MAXIMALLY FRONTAL) PROGNOSIS: POOR

38. LENNOX GASTAUT SYNDROME IN A CHILD WITH A NORMAL NEUROLOGICAL EXAMINATION, NORMAL DEVELOPMENT AND MULTIPLE HYPOPIGMENTED SPOTS SHOULD BE CLASSIFIED AS: A. CRYPTOGENIC B. SYMPTOMATIC C. IDIOPATHIC

39. AN IV TRIAL OF PYRIDOXINE SHOULD BE PREFORMED WHILE CONDUCTING AN EEG AND WHILE THE PATIENT IS BEING MONITORED. A. TRUE B. FALSE

PYRIDOXINE IN A PATIENT WITH PYRIDOXINE DEPENDENCY LEADS TO A SUDDEN PRODUCTION OF GABA A SUDDEN PRODUCTION OF GABA IS AS IF THE PATIENT IS GETTING A DOSE OF BENZODIAZEPINE IV BENZODIAZEPINE MAY PRODUCE RESPIRATORY ARREST

39. AN IV TRIAL OF PYRIDOXINE SHOULD BE PREFORMED WHILE CONDUCTING AN EEG AND WHILE THE PATIENT IS BEING MONITORED. A. TRUE B. FALSE

40. THE CLINICAL FINDING THAT SHOULD MAKE YOU CONSIDER THE POSSIBILITY OF LANDAU KLEFFNER SYNDROME IS: A. LEFT ARM SEIZURES B. REGRESSION OF PREVIOUSLY ACQUIRED LANGUAGE C. GENERALIZED SEIZURES PRECEDED BY ABDOMINAL PAIN

SEIZURE TYPE: EEG: TREATMENT: LANDAU-KLEFFNER SYNDROME ABSENCE, TONIC-CLONIC, PARTIAL, NONE SHARPS AND SPIKE-WAVE COMPLEXES IN THE CENTRAL, TEMPORAL AND PARTIAL REGIONS REGRESSION OF PREVIOUSLY ACQUIRED LANGUAGE BEHAVIORAL DYSFUNCTION (AUTISTIC LIKE BEHAVIOR) COGNITIVE DYSFUNCTION TEGRETOL, CORTICOSTEROIDS, EEG GUIDED SUBPIAL SURGICAL TRANSECTION

40. THE CLINICAL FINDING THAT SHOULD MAKE YOU CONSIDER THE POSSIBILITY OF LANDAU KLEFFNER SYNDROME IS: A. LEFT ARM SEIZURES B. REGRESSION OF PREVIOUSLY ACQUIRED LANGUAGE C. GENERALIZED SEIZURES PRECEDED BY ABDOMINAL PAIN

41. ALL OF THE FOLLOWING EPILEPTIC SYNDROMES SHOULD BE CONSIDERED IN A CHILD WITH REGRESSION OF PREVIOUSLY ACQUIRED LANGUAGE, AUTISTIC-LIKE BEHAVIOR AND SHORT ATTENTION SPAN, EXCEPT: A. LANDAU KLEFFNER SYNDROME B. SLOW SLEEP STATUS C. OHTAHARA SYNDROME

SEIZURE TYPE: EEG: TREATMENT: SLOW SLEEP STATUS SYNDROME ABSENCE, TONIC-CLONIC, PARTIAL, NONE CONTINUOS GENERALIZED SPIKE-WAVE COMPLEXES DURING NON-REM SLEEP REGRESSION OF PREVIOUSLY ACQUIRED LANGUAGE BEHAVIORAL DYSFUNCTION (AUTISTIC LIKE BEHAVIOR) COGNITIVE DYSFUNCTION (LEARNING DISABILITIES) VALPROIC ACID

SEIZURE TYPE: EEG: SEARCH FOR ETIOLOGY: WOODS LAMP, METABOLIC DISORDERS (BIOTINIDASE), TRIALS OF B6 & FOLINIC ACID; ANTIEPILEPTIC DRUGS NOT EFFECTIVE, BUT TRY OHTAHARA SYNDROME (EARLY INFANTILE EPILEPTIC ENCEPHALOPATHY WITH BURST SUPPRESSION) TONIC AND OTHERS BURST SUPPRESSION (ESTABLISHES DIAGNOSIS) VARIETIES: IDIOPATHIC (RARE) SYMPTOMATIC CRYPTOGENIC EXAMCAUSE NEGATIVENONE ABNORMALNONE PRESENTEITHER PROGNOSIS: POOR

41. ALL OF THE FOLLOWING EPILEPTIC SYNDROMES SHOULD BE CONSIDERED IN A CHILD WITH REGRESSION OF PREVIOUSLY ACQUIRED LANGUAGE, AUTISTIC-LIKE BEHAVIOR AND SHORT ATTENTION SPAN, EXCEPT: A. LANDAU KLEFFNER SYNDROME B. SLOW SLEEP STATUS C. OHTAHARA SYNDROME

42. ALL THE FOLLOWING EPILEPTIC SYNDROMES FREQUENTLY OCCUR IN LATE CHILDHOOD, EXCEPT: A. BENIGN ROLANDIC EPILEPSY B. PYKNOLEPSIES C. WEST SYNDROME D. BENIGN OCCIPITAL EPILEPSY

EPILEPSY SYNDROMES OF ONSET - DURING LATE CHILDHOOD BENIGN ROLANDIC SEIZURES PETIT MAL, PYKNOLEPSY BENIGN OCCIPITAL EPILEPSY

42. ALL THE FOLLOWING EPILEPTIC SYNDROMES FREQUENTLY OCCUR IN LATE CHILDHOOD, EXCEPT: A. BENIGN ROLANDIC EPILEPSY B. PYKNOLEPSIES C. WEST SYNDROME D. BENIGN OCCIPITAL EPILEPSY

43. IN CHILDREN WITH BENIGN ROLANDIC EPILEPSY, SPEECH ARREST IS USUALLY FOLLOWED BY: A. LEFT ARM SEIZURES B. RIGHT ARM SEIZURES C. GENERALIZED SEIZURES

SEIZURE TYPE: EEG: TREATMENT: BENIGN ROLANDIC EPILEPSY FACIAL-ORAL-PHARYNGEAL SEIZURES, JACKSONIAN SPREAD, GENERALIZATION AT NIGHT ONLY (NIGHT SEIZURES) PERI-ROLANDIC SPIKES AND SHARP WAVES (HORIZONTAL DIPOLE), ACTIVATED BY SLEEP AND SHIFTING FROM SIDE TO SIDE TEGRETOL, TRILEPTAL (DO NOT TREAT AFTER THE FIRST ONE) SPEECH ARREST DURING THE DAY OR GUTTURAL NOISES AT NIGHT FOLLOWED BY UPPER LIMB CONVULSIONS NORMAL NEUROLOGICAL EXAMINATION SEIZURES STOP BY 16 YEARS OF AGE

43. IN CHILDREN WITH BENIGN ROLANDIC EPILEPSY, SPEECH ARREST IS USUALLY FOLLOWED BY: A. LEFT ARM SEIZURES B. RIGHT ARM SEIZURES (LANGUAGE IS ON THE LEFT) C. GENERALIZED SEIZURES

44. BENIGN OCCIPITAL EPILEPSY MAY BE ASSOCIATED WITH: A. CELIAC DISEASE B. MAPLE SYRUP URINE DISEASE D. DIABETES MELLITUS

SEIZURE TYPE: EEG: TREATMENT: BENIGN OCCIPITAL EPILEPSY OCCIPITAL LOBE SEIZURES REPETITIVE OCCIPITAL DISCHARGES ON EYE CLOSURE TEGRETOL, TRILEPTAL SIMPLE OR COMPLEX HALLUCINATIONS, VISUAL DISTORTIONS, HEMIANOPSIA, AND AMAUROSIS WITH PRESERVATION OF CONSCIOUSNESS DO NOT MISDIAGNOSE AS MIGRAINE MAYBE ASSOCIATED WITH CELIAC DISEASE AND - OCCIPITAL LOBE CALCIFICATION NORMAL NEUROLOGICAL EXAMINATION

44. BENIGN OCCIPITAL EPILEPSY MAY BE ASSOCIATED WITH: A. CELIAC DISEASE B. MAPLE SYRUP URINE DISEASE D. DIABETES MELLITUS

45. WHICH OF THE FOLLOWING EPILEPTIC SYNDROMES SHOULD YOU CONSIDER IN A 5-YEAR-OLD CHILD WITH ABSENCE, NO AURA AND NO POSTICTAL CONFUSION? A. PETIT MAL B. PSYCHOMOTOR SEIZURES C. SIMPLE PARTIAL SEIZURES

SEIZURE TYPE: EEG: TREATMENT: PETIT MAL, PYKNOLEPSY ABSENCE, AUTOMATISMS, SMALL JERKS 3 Hz SPIKES AND WAVES, TRIGGER BY HV 30 TO 40 % DEVELOP GENERALIZED CONVULSIONS LATER NORMAL NEUROLOGICAL EXAMINATION GENETIC PREDISPOSITION ETHOSUXIMIDE, VALPROATE NO AURA NO POST-ICTAL CONFUSION

PETIT MAL, PYKNOLEPSY F F F F O O O

SEIZURE TYPE: EEG: TREATMENT: PSYCHOMOTOR SEIZURES ABSENCE, CONVULSIONS FOCAL TEMPORAL OR FRONTAL SPIKES TEMPORAL LOBE SCLEROSIS (FEBRILES SEIZURES ?) ABNORMAL NEUROLOGICAL EXAMINATION FRONTAL LOBE TUMORS TEGRETOL OR VALPROIC ACID AURA: TEMPORAL LOBE: METALLIC SMELL FRONTAL LOBE : ABNORMAL BEHAVIOR POST-ICTAL CONFUSION

45. WHICH OF THE FOLLOWING EPILEPTIC SYNDROMES SHOULD YOU CONSIDER IN A 5-YEAR-OLD CHILD WITH ABSENCE, NO AURA AND NO POSTICTAL CONFUSION? A. PETIT MAL B. PSYCHOMOTOR SEIZURES C. SIMPLE PARTIAL SEIZURES

46. COMPLEX PARTIAL SEIZURES ALWAYS COINCIDE WITH THE ONSET OF AN ALTERED STATE OF CONSCIOUSNESS. A. TRUE B. FALSE

SIMPLE PARTIAL SEIZURES (SENSORY OR MOTOR PHENOMENA WITHOUT LOSS OF CONSCIOUSNESS) COMPLEX PARTIAL SEIZURES (ALTER STATE OF CONSCIOUSNESS) GENERALIZED SEIZURES

46. COMPLEX PARTIAL SEIZURES ALWAYS COINCIDE WITH THE ONSET OF AN ALTERED STATE OF CONSCIOUSNESS. A. TRUE B. FALSE SOME NEUROLOGIST DO NOT AGREE WITH THIS ANSWER!

47. AN INFANT THAT PRESENTS WITH PROLONGED AND REPETITIVE ALIMENTARY AUTOMATISM AND A BLANK STARE IS LIKELY TO HAVE? A. WEST SYNDROME B. INFANTILE SPASM C. COMPLEX PARTIAL SEIZURES D. SIMPLE PARTIAL SEIZURES

INFANT ONSET EPILEPSIES SYNDROMES OTHERS COMPLEX PARTIAL SEIZURES SIMPLE PARTIAL SEIZURES EPILEPSY SYNDROMES OF ONSET DURING INFANCY WEST SYNDROME (INFANTILE SPASM) FEBRILE SEIZURES BENIGN MYOCLONIC EPILEPSY OF INFANCY OTHER EPILEPSY ALIMENTARY AUTOMATISM

SEIZURE TYPE: EEG: TREATMENT: PSYCHOMOTOR SEIZURES ABSENCE, CONVULSIONS FOCAL TEMPORAL OR FRONTAL SPIKES TEMPORAL LOBE SCLEROSIS (FEBRILES SEIZURES ?) ABNORMAL NEUROLOGICAL EXAMINATION FRONTAL LOBE TUMORS TEGRETOL OR VALPROIC ACID AURA: TEMPORAL LOBE: METALLIC SMELL FRONTAL LOBE : ABNORMAL BEHAVIOR POST-ICTAL CONFUSION

47. AN INFANT THAT PRESENTS WITH PROLONGED AND REPETITIVE ALIMENTARY AUTOMATISM AND A BLANK STARE IS LIKELY TO HAVE? A. WEST SYNDROME B. INFANTILE SPASM C. COMPLEX PARTIAL SEIZURES D. SIMPLE PARTIAL SEIZURES

48. WHICH OF THE FOLLOWING AUTOMATISM IS LESS FREQUENT IN OLDER CHILDREN WITH COMPLEX PARTIAL SEIZURES? A. PICKING AT CLOTHING B. RUBBING OBJECTS C. FEARFUL BEHAVIOR D. LIP SMACKING

TEMPORAL LOBE SEIZURES RIGHT ARM TONIC THEN CONFUSION ABNORMAL BEHAVIOR

FEAR RAGE SECONDARY GENERALIZED TONIC POSTURE HEAD TURNING FRONTAL (PREMOTOR) LOBE SEIZURES

48. WHICH OF THE FOLLOWING AUTOMATISM IS LESS FREQUENT IN OLDER CHILDREN WITH COMPLEX PARTIAL SEIZURES? A. PICKING AT CLOTHING B. RUBBING OBJECTS C. FEARFUL BEHAVIOR D. LIP SMACKING

49. A NORMAL INTERICTAL ROUTINE EEG EXCLUDES THE POSSIBILITY OF COMPLEX PARTIAL SEIZURES. A. TRUE B. FALSE

A NORMAL INTERICTAL ROUTINE EEG OCCURS IN 20% OF INFANT AND CHILDREN WITH COMPLEX PARTIAL SEIZURES. MRI ALL PATIENTS WITH COMPLEX PARTIAL SEIZURES. POSSIBLE FINDINGS ARE TUMORS, CYSTS, ARTERIOVENOUS MALFORMATIONS, INFARCTION IN THE FRONTAL OR TEMPORAL LOBE OR MESIAL TEMPORAL SCLEROSIS

49. A NORMAL INTERICTAL ROUTINE EEG EXCLUDES THE POSSIBILITY OF COMPLEX PARTIAL SEIZURES. A. TRUE B. FALSE

50. RASMUSSEN ENCEPHALITIS IS A FREQUENT CAUSE OF: A. BENIGN ROLANDIC EPILEPSY B. EPILEPSY OF JANZ C. EPILEPSIA PARTIALIS CONTINUA D. OHTAHARA SYNDROME

RASMUSSEN ENCEPHALITIS NO CAUSE KNOWN EPILEPSIA PARTIALIS CONTINUA POOR PROGNOSIS

50. RASMUSSEN ENCEPHALITIS IS A FREQUENT CAUSE OF: A. BENIGN ROLANDIC EPILEPSY B. JANZ SYNDROME C. EPILEPSIA PARTIALIS CONTINUA D. OHTAHARA SYNDROME

51. THE HALLMARK OF JANZ SYNDROME IS: A. SPEECH ARREST B. MYOCLONIC JERKS WITHIN 30 MINUTES OF AWAKENING C. ROLANDIC SEIZURES D. MENTAL RETARDATION

EPILEPSY SYNDROMES OF ONSET - DURING ADOLESCENCE JANZ’S SYNDROME (JUVENILE MYOCLONIC EPILEPSY)

SEIZURE TYPE: EEG: TREATMENT: (JUVENILE MYOCLONIC EPILEPSY) MYOCLONIC SEIZURES WITHIN 30 MINUTES OF AWAKENING, SOMETIMES PRECEDED BY ABSENCE 3.5 TO 6 MULTI-SPIKE AND WAVE COMPLEXES DURING SEIZURES AND INTERICTALLY (MAYBE TRIGGER BY PHOTIC) VALPROIC ACID (CLONAZEPAM) BILATERAL SINGLE OR REPETITIVE JERKING MOVEMENTS OF THE ARMS DO NOT STOP MEDICATION LINKED TO CHROMOSOME 6 NORMAL NEUROLOGICAL EXAMINATION JANZ’S SYNDROME

(JUVENILE MYOCLONIC EPILEPSY) JANZ’S SYNDROME MYOCLONIC JERK TRIGGER BY PHOTIC STIMULATION

51. THE HALLMARK OF JANZ SYNDROME IS: A. SPEECH ARREST B. MYOCLONIC JERKS WITHIN 30 MINUTES OF AWAKENING C. ROLANDIC SEIZURES D. MENTAL RETARDATION

52. JUVENILE MYOCLONIC EPILEPSY IS LINKED TO: A. 20q B. 8q C. 6p D. 21q

SEIZURE TYPE: EEG: TREATMENT: (JUVENILE MYOCLONIC EPILEPSY) MYOCLONIC SEIZURES WITHIN 30 MINUTES OF AWAKENING, SOMETIMES PRECEDED BY ABSENCE 3.5 TO 6 MULTI-SPIKE AND WAVE COMPLEXES DURING SEIZURES AND INTERICTALLY (MAYBE TRIGGER BY PHOTIC) VALPROIC ACID (CLONAZEPAM) BILATERAL SINGLE OR REPETITIVE JERKING MOVEMENTS OF THE ARMS DO NOT STOP MEDICATION LINKED TO CHROMOSOME 6 NORMAL NEUROLOGICAL EXAMINATION JANZ’S SYNDROME

52. JUVENILE MYOCLONIC EPILEPSY IS LINKED TO: A. 20q B. 8q C. 6p D. 21q

53. WHICH OF THE FOLLOWING STRUCTURES HAS AN INTEGRAL ROLE IN THE DEVELOPMENT OF GENERALIZED SEIZURES? A. GLOBUS PALLIDUS B. RED NUCLEUS C. SUBSTANTIA NIGRA D. CAUDATE NUCLEUS

GLOBUS PALLIDUS RED NUCLEUS SUBSTANTIA NIGRA PUTAMEN CLAUSTRUM CAUDATE NUCLEUS

53. WHICH OF THE FOLLOWING STRUCTURES HAS AN INTEGRAL ROLE IN THE DEVELOPMENT OF GENERALIZED SEIZURES? A. GLOBUS PALLIDUS B. RED NUCLEUS C. SUBSTANTIA NIGRA D. CAUDATE NUCLEUS

54. THE MINIMUM WORK-UP FOR THE FIRST AFEBRILE SEIZURE, IN AN OTHERWISE HEALTHY CHILD, SHOULD INCLUDE ALL OF THE FOLLOWING, EXCEPT: A. FASTING GLUCOSE B. MAGNESIUM C. EEG D. AMMONIA

THE MINIMUM WORK-UP FOR THE FIRST AFEBRILE SEIZURE IS IN AN OTHERWISE HEALTHY CHILD SHOULD INCLUDE ALL OF THE FOLLOWING: (1) FASTING GLUCOSE, (2) CALCIUM, (3) MAGNESIUM, (4) SERUM ELECTROLYTES, (5) EEG.

54. THE MINIMUM WORK-UP FOR THE FIRST AFEBRILE SEIZURE, IN AN OTHERWISE HEALTHY CHILD, SHOULD INCLUDE ALL OF THE FOLLOWING, EXCEPT: A. FASTING GLUCOSE B. MAGNESIUM C. EEG D. AMMONIA

55. INTERICTAL EEG RECORDINGS ARE NORMAL IN APPROXIMATELY ___ PERCENT OF PATIENTS WITH FIRST AFEBRILE SEIZURE. A. 20 B. 40 C. 60 D. 80

40% NORMAL

55. INTERICTAL EEG RECORDINGS ARE NORMAL IN APPROXIMATELY ___ PERCENT OF PATIENTS WITH FIRST AFEBRILE SEIZURE. A. 20 B. 40 C. 60 D. 80

56. BEFORE SCHEDULING A ROUTINE EEG, THE SEIZURE MEDICATION SHOULD BE STOPPED. A. TRUE B. FALSE

57. MRI OF THE BRAIN SHOULD BE DONE IN ALL PATIENTS WITH AFEBRILE SEIZURES. A. TRUE B. FALSE

MRI OF THE BRAIN SHOULD BE DONE IN PATIENTS WITH AFEBRILE SEIZURES IF AN INTRACRANIAL LESION IS SUSPECTED

57. MRI OF THE BRAIN SHOULD BE DONE IN ALL PATIENTS WITH AFEBRILE SEIZURES. A. TRUE B. FALSE

58. NO ANTIEPILEPTIC DRUGS ARE INDICATED AFTER THE FIRST AFEBRILE CONVULSION IF ALL OF THE FOLLOWING CONDITIONS ARE TRUE, EXCEPT: A. A POSITIVE FAMILY HISTORY OF SEIZURES B. NORMAL NEUROLOGICAL EXAMINATION C. NORMAL EEG D. A COOPERATIVE AND COMPLIANT FAMILY

A POSITIVE FAMILY HISTORY OF SEIZURES IS AN INDICATION TO TREAT

58. NO ANTIEPILEPTIC DRUGS ARE INDICATED AFTER THE FIRST AFEBRILE CONVULSION IF ALL OF THE FOLLOWING CONDITIONS ARE TRUE, EXCEPT: A. A POSITIVE FAMILY HISTORY OF SEIZURES B. NORMAL NEUROLOGICAL EXAMINATION C. NORMAL EEG D. A COOPERATIVE AND COMPLIANT FAMILY

59. MONITORING OF ANTICONVULSANT LEVELS SHOULD BE DONE IN ALL OF THE FOLLOWING SITUATIONS, EXCEPT: A. AT THE ONSET OF ANTICONVULSANT THERAPY B. DURING ACCELERATED GROWTH SPURTS C. IN PATIENTS WITH COGNITIVE OR PHYSICAL DISABILITIES D. IN PATIENTS TAKING CARBAMAZEPINE

MONITORING OF ANTICONVULSANT LEVELS SHOULD BE DONE: (1) AT THE ONSET OF ANTICONVULSANT THERAPY, (2) DURING ACCELERATED GROWTH SPURTS, (3) NONCOMPLIANT FAMILIES, (4) TIME OF STATUS EPILEPTICUS, (5) FOR PATIENTS IN POLYTHERAPY, (6) SEIZURES THAT CHANGE, (7) UNCONTROLLABLE SEIZURES, (8) SIGNS OF DRUG TOXICITY, (9) IN PATIENTS WITH LIVER OR KIDNEY PROBLEMS, AND (10) IN PATIENTS WITH COGNITIVE OR PHYSICAL DISABILITIES

59. MONITORING OF ANTICONVULSANT LEVELS SHOULD BE DONE IN ALL OF THE FOLLOWING SITUATIONS, EXCEPT: A. AT THE ONSET OF ANTICONVULSANT THERAPY B. DURING ACCELERATED GROWTH SPURTS C. IN PATIENTS WITH COGNITIVE OR PHYSICAL DISABILITIES D. IN PATIENTS TAKING CARBAMAZEPINE

60. ALL OF THE FOLLOWING ARE RISK FACTORS FOR SEIZURE RECURRENCE AFTER 2 SEIZURE-FREE YEARS EXCEPT: A. AGE GREATER THAN 12 YEARS AT ONSET OF SEIZURES B. A HISTORY OF NEONATAL SEIZURES C. A NEUROLOGIC DYSFUNCTION D. AGE LESS THAN 12 YEARS AT ONSET OF SEIZURES

THE FOLLOWING ARE RISK FACTORS FOR SEIZURE RECURRENCE AFTER 2 SEIZURE-FREE YEARS: (1) AGE GREATER THAN 12 YEARS AT ONSET OF SEIZURES, (2) A HISTORY OF NEONATAL SEIZURES, (3) A NEUROLOGIC DYSFUNCTION, (4) NUMEROUS SEIZURES BEFORE ACHIEVING SEIZURE CONTROL

60. ALL OF THE FOLLOWING ARE RISK FACTORS FOR SEIZURE RECURRENCE AFTER 2 SEIZURE-FREE YEARS EXCEPT: A. AGE GREATER THAN 12 YEARS AT ONSET OF SEIZURES B. A HISTORY OF NEONATAL SEIZURES C. A NEUROLOGIC DYSFUNCTION D. AGE LESS THAN 12 YEARS AT ONSET OF SEIZURES

61. THE PROCESS OF WEANING ANTIEPILEPTIC DRUGS SHOULD OCCUR FOR: A. 1 TO 3 MONTHS B. 3 TO 6 MONTHS C. 6 TO 9 MONTHS D. 9 MONTHS TO 1 YEAR

WEANING PROCESS OF ANTIEPILEPTIC DRUGS SHOULD OCCUR FOR: MONTH 612390

61. THE PROCESS OF WEANING ANTIEPILEPTIC DRUGS SHOULD OCCUR FOR: A. 1 TO 3 MONTHS B. 3 TO 6 MONTHS C. 6 TO 9 MONTHS D. 9 MONTHS TO 1 YEAR

62. BENZODIAZEPINES EXERT ANTICONVULSANT ACTIVITY BY: A. OPENING CHLORIDE CHANNELS B. BLOCKING SODIUM-DEPENDENT CHANNELS C. DECREASING THE POLARIZATION- DEPENDENT CALCIUM UPTAKE D. INCREASING GABA TURNOVER

MUST SEIZURE MEDICATION WORK EITHER BY “CLOSING” Na CHANNELS OR “OPENING” Cl CHANNELS Cl CHANNELS Cl BARBITURATES BENZODIAZEPINES GABA BY OPENING Cl CHANNELS THE MEMBRANE POTENTIAL IS FIXED AT THE NERNST POINT FOR Cl (-60  V)

= 10  V + NEURONAL MEMBRANE AT REST 60  V DEPOLARIZATION ACTION POTENTIAL: Na CHANNELS ARE OPEN HYPER POLARIZATION NERNST POINT = 73  V K = 60  V Cl Na = + 57  V Na CHANNELS ARE CLOSED + 50  V MUST SEIZURE MEDICATION WORK EITHER BY “CLOSING” Na CHANNELS OR “OPENING” Cl CHANNELS

62. BENZODIAZEPINES EXERT ANTICONVULSANT ACTIVITY BY: A. OPENING CHLORIDE CHANNELS B. BLOCKING SODIUM-DEPENDENT CHANNELS C. DECREASING THE POLARIZATION- DEPENDENT CALCIUM UPTAKE D. INCREASING GABA TURNOVER

63. PATIENTS ON CARBAMAZEPINE SHOULD HAVE CBC WITH DIFFERENTIAL AND LIVER FUNCTION TEST: A. MONTHLY DURING THE FIRST 3 TO 4 MONTHS OF THERAPY B. EVERY 3 MONTHS DURING THERAPY C. ONCE A YEAR D. NEVER

PATIENTS ON CARBAMAZEPINE SHOULD HAVE CBC AND DIFFERENTIAL AND LIVER FUNCTION TEST MONTHLY DURING THE FIRST 3 TO 4 MONTHS OF THERAPY (AS PER NELSON) MUST NEUROLOGIST DO NOT AGREE WITH THIS STATEMENT, BUT IS RIGHT FOR EXAM (2002)

CARBAMAZEPINE (CBZ) DOSE/PO START UP/W BY USUAL MAX MG/KG/D 5-10 5-10 15-20 30 LEVEL: 4-12 MG/L SIDE EFFECTS CNS: LETHARGY, ATAXIA, DIPLOPIA GI: NAUSEA, WEIGHT GAIN, PANCREATITIS, HEPATITIS BLOOD: LOW WHITE & RED CELLS &/OR PLATELETS OTHERS: LOW SODIUM, LUPUS LIKE, ARRHYTHMIA INTERACTION CBZ LOWERS ANTICOAGULANT AND “PILL” CBZ INCREASED BY ERYTHROMYCIN STOP IF ABSOLUTE NEUTROPHILIC COUNT IS < 1000 CBC & LFT: ONSET, 6 WEEKS, & Q 3-6 MONTHS

63. PATIENTS ON CARBAMAZEPINE SHOULD HAVE CBC WITH DIFFERENTIAL AND LIVER FUNCTION TEST: A. MONTHLY DURING THE FIRST 3 TO 4 MONTHS OF THERAPY B. EVERY 3 MONTHS DURING THERAPY C. ONCE A YEAR D. NEVER

64. WHICH OF THE FOLLOWING DRUGS RAISES THE LEVEL OF CARBAMAZEPINE? A. ERYTHROMYCIN B. PHENYTOIN C. PHENOBARBITAL D. VALPROIC ACID

PHENOBARBITAL PHENYTOIN VALPROIC ACID CARBAMAZEPINE LEVEL

ERYTHROMYCIN CARBAMAZEPINE LEVEL

64. WHICH OF THE FOLLOWING DRUGS RAISES THE LEVEL OF CARBAMAZEPINE? A. ERYTHROMYCIN B. PHENYTOIN C. PHENOBARBITAL D. VALPROIC ACID

65. TOXICITY DESPITE THERAPEUTIC CARBAMAZEPINE LEVELS IS LIKELY TO OCCUR WHEN ______ IS ADDED. A. ERYTHROMYCIN B. PHENYTOIN C. PHENOBARBITAL D. VALPROIC ACID

PHENOBARBITAL PHENYTOIN VALPROIC ACID CARBAMAZEPINE LEVEL EPOXIDE (TOXICITY)

65. TOXICITY DESPITE THERAPEUTIC CARBAMAZEPINE LEVELS IS LIKELY TO OCCUR WHEN ______ IS ADDED. A. ERYTHROMYCIN B. PHENYTOIN C. PHENOBARBITAL D. VALPROIC ACID

66. CARBAMAZEPINE EXERTS ANTICONVULSANT ACTIVITY BY: A. OPENING CHLORIDE CHANNELS B. BLOCKING SODIUM-DEPENDENT CHANNELS C. BLOCKING CALCIUM CHANNELS ASSOCIATED WITH THALAMOCORTICAL CIRCUITRY D. INCREASING GABA TURNOVER

MUST SEIZURE MEDICATION WORK EITHER BY “CLOSING” Na CHANNELS OR “OPENING” Cl CHANNELS Na CHANNELS Na BY CLOSING Na CHANNELS THE MEMBRANE POTENTIAL DOES NOT MOVES TO THE NERNST POINT FOR Na (+57  V) CARBAMAZEPINE

66. CARBAMAZEPINE EXERTS ANTICONVULSANT ACTIVITY BY: A. OPENING CHLORIDE CHANNELS B. BLOCKING SODIUM-DEPENDENT CHANNELS C. BLOCKING CALCIUM CHANNELS ASSOCIATED WITH THALAMOCORTICAL CIRCUITRY D. INCREASING GABA TURNOVER

67. ETHOSUXIMIDE EXERTS ANTICONVULSANT ACTIVITY BY: A. OPENING CHLORIDE CHANNELS B. BLOCKING SODIUM-DEPENDENT CHANNELS C. BLOCKING CALCIUM CHANNELS ASSOCIATED WITH THALAMOCORTICAL CIRCUITRY D. INCREASING GABA TURNOVER

ETHOSUXIMIDE EXERTS ANTICONVULSANT ACTIVITY BY BLOCKING CALCIUM CHANNELS ASSOCIATED WITH THALAMOCORTICAL CIRCUITRY

67. ETHOSUXIMIDE EXERTS ANTICONVULSANT ACTIVITY BY: A. OPENING CHLORIDE CHANNELS B. BLOCKING SODIUM-DEPENDENT CHANNELS C. BLOCKING CALCIUM CHANNELS ASSOCIATED WITH THALAMOCORTICAL CIRCUITRY D. INCREASING GABA TURNOVER

68. WHEN A PATIENT RECEIVING LAMOTRIGINE DEVELOPS A RASH, YOU SHOULD: A. DECREASE THE DOSE BY HALF B. CONTINUE THE SAME DOSE BUT DO NOT INCREASE C. STOP THE MEDICATION AND REINTRODUCE IT LATER WHEN THE RASH IS GONE D. STOP THE MEDICATION AND DO NOT USE IT AGAIN

69. IN A PATIENT RECEIVING PHENOBARBITAL, THE INTRODUCTION OF VALPROIC ACID WILL: A. INCREASE PHENOBARBITAL LEVEL B. DECREASE PHENOBARBITAL LEVEL C. NOT AFFECT PHENOBARBITAL LEVEL D. ALL OF THE ABOVE

PHENOBARBITAL (PB) DOSE/PO USUAL MAX MG/D MG/KG/D 3-5 250 TR: 15-45 MG/L SIDE EFFECTS CNS: LETHARGY, HYPERACTIVITY, COGNITIVE CHANGES GI: LIVER FAILURE BLOOD: APLASTIC ANEMIA OTHERS: LARYNGO- & BRONCO-SPASM, HYPOTENSION INTERACTION PB: LOWERS THEOPHYLLINE, ANTICOAGULANT & “PILL” BIDQD CONTRAINDICATIONS: OBSTRUCTIVE LUNG, PORPHYRIA RICKETS PERSONALITY CHANGES

VALPROIC ACID PHENOBARBITAL LEVEL

MUST SEIZURE MEDICATION WORK EITHER BY “CLOSING” Na CHANNELS OR “OPENING” Cl CHANNELS Cl CHANNELS Cl BARBITURATES BENZODIAZEPINES GABA BY OPENING Cl CHANNELS THE MEMBRANE POTENTIAL IS FIXED AT THE NERNST POINT FOR Cl (-60  V)

69. IN A PATIENT RECEIVING PHENOBARBITAL, THE INTRODUCTION OF VALPROIC ACID WILL: A. INCREASE PHENOBARBITAL LEVEL B. DECREASE PHENOBARBITAL LEVEL C. NOT AFFECT PHENOBARBITAL LEVEL D. ALL OF THE ABOVE

70. VALPROIC ACID EXERTS ANTICONVULSANT ACTIVITY BY ALL OF THE FOLLOWING, EXCEPT: A. BLOCKING VOLTAGE-DEPENDENT SODIUM CHANNELS B. INCREASE CALCIUM-DEPENDENT POTASSIUM CONDUCTANCE C. DECREASE THE POLARIZATION- DEPENDENT UPTAKE

MUST SEIZURE MEDICATION WORK EITHER BY “CLOSING” Na CHANNELS OR “OPENING” Cl CHANNELS, BUT A FEW WORK BY INCREASING PERMEABILITY TO POTASSIUM Na CHANNELS BY CLOSING Na CHANNELS THE MEMBRANE POTENTIAL DOES NOT MOVES TO THE K NERNST POINT (-73  V) VALPROIC ACID K CHANNELS BY OPENING K CHANNELS THE MEMBRANE POTENTIAL MOVES TOWARD THE Na NERNST POINT (+57  V)

VALPROIC ACID (VPA) DOSE/PO START UP/W BY USUAL MAX MG/KG/D 15 5-10 20-30 60 TR: 50-130 MG/L SIDE EFFECTS CNS: TREMOR, HYPERACTIVITY, BRAIN ATROPHY GI: NAUSEA, WEIGHT GAIN, PANCREATITIS, HEPATITIS BLOOD: LOW PLATELETS, APLASTIC ANEMIA OTHERS: POLYCYSTIC OVARIAN SYNDROME INTERACTION VPA LEVEL ARE INCREASED BY SALICYLATES TIDQID CBC, AMMONIA, AMYLASE & LFT: ONSET CBC, & LFT: IN 6 WEEKS, & Q 3-6 MONTHS LOW FACTOR VII, LOW CARNITINE CONTRAINDICATIONS: PORPHYRIA, LIVES DISEASE GIRL > 10 Y: FOLIC ACID

70. VALPROIC ACID EXERTS ANTICONVULSANT ACTIVITY BY ALL OF THE FOLLOWING, EXCEPT: A. BLOCKING VOLTAGE-DEPENDENT SODIUM CHANNELS B. INCREASE CALCIUM-DEPENDENT POTASSIUM CONDUCTANCE C. DECREASE THE POLARIZATION- DEPENDENT UPTAKE

71. IN A PATIENT TAKING MULTIPLE ANTICONVULSANTS INCLUDING VALPROIC ACID, THE PRESENCE OF ABDOMINAL PAIN, ANOREXIA, WEIGHT LOSS, AND WRENCHING SHOULD MAKE YOU CONSIDER: A. ANGIONEUROTIC EDEMA B. HEPATOTOXIC SYNDROME C. TOXIC EPIDERMAL NECROLYSIS D. ALL OF THE ABOVE

VALPROIC ACID (VPA) DOSE/PO START UP/W BY USUAL MAX MG/KG/D 15 5-10 20-30 60 TR: 50-130 MG/L SIDE EFFECTS CNS: TREMOR, HYPERACTIVITY, BRAIN ATROPHY GI: NAUSEA, WEIGHT GAIN, PANCREATITIS, HEPATITIS BLOOD: LOW PLATELETS, APLASTIC ANEMIA OTHERS: POLYCYSTIC OVARIAN SYNDROME INTERACTION VPA LEVEL ARE INCREASED BY SALICYLATES TIDQID CBC, AMMONIA, AMYLASE & LFT: ONSET CBC, & LFT: IN 6 WEEKS, & Q 3-6 MONTHS LOW FACTOR VII, LOW CARNITINE CONTRAINDICATIONS: PORPHYRIA, LIVER DISEASE GIRL > 10 Y: FOLIC ACID

71. IN A PATIENT TAKING MULTIPLE ANTICONVULSANTS INCLUDING VALPROIC ACID, THE PRESENCE OF ABDOMINAL PAIN, ANOREXIA, WEIGHT LOSS, AND WRENCHING SHOULD MAKE YOU CONSIDER: A. ANGIONEUROTIC EDEMA B. HEPATOTOXIC SYNDROME C. TOXIC EPIDERMAL NECROLYSIS D. ALL OF THE ABOVE

72. BEFORE INITIATING VALPROIC ACID IN A 2-YEAR-OLD, WHICH OF THE FOLLOWING SHOULD BE RULED OUT: A. METABOLIC DISORDERS B. CARDIAC DISORDERS C. KIDNEY DISORDERS D. ENDOCRINOPATHIES

BEFORE INITIATING VALPROIC ACID IN A 2-YEAR-OLD, METABOLIC DISORDERS SHOULD BE RULE OUT BY OBTAINING: (1) AMMONIA, (2) AMINO ACIDS, (3) BLOOD GASES, (4) LACTATE TO PYRUVATE RATIO, (5) URINE ORGANIC ACIDS, AND (6) FREE AND TOTAL SERUM CARNITINE.

72. BEFORE INITIATING VALPROIC ACID IN A 2-YEAR-OLD, WHICH OF THE FOLLOWING SHOULD BE RULED OUT: A. METABOLIC DISORDERS B. CARDIAC DISORDERS C. KIDNEY DISORDERS D. ENDOCRINOPATHIES

73. WHICH OF THE FOLLOWING MEDICINES SHOULD BE GIVEN TO A PATIENT YOUNGER THAN 2 YEARS OF AGE TAKING VALPROIC ACID? A. VITAMIN B6 B. CARNITINE C. VITAMIN B12 D. VITAMIN B1

74. ACTH AND PREDNISONE ARE EQUALLY AFFECTIVE IN THE TREATMENT OF INFANTILE SPASMS: A. TRUE B. FALSE

PREDNISONEACTH SUCCESS FOR CRYPTOGENIC AND SYMPTOMATIC WEST SYNDROME 70 % I DO NOT THINK SO, BUT …

74. ACTH AND PREDNISONE ARE EQUALLY AFFECTIVE IN THE TREATMENT OF INFANTILE SPASMS: A. TRUE B. FALSE

75. ALL OF THE FOLLOWING DRUGS LOWER THEOPHYLLINE LEVELS, EXCEPT: A. PHENOBARBITAL B. PHENYTOIN C. PRIMIDONE D. VALPROIC ACID

PRIMIDONE (PRM) TR: PB: 15-45 MG/L SIDE EFFECTS CNS: LETHARGY, HYPERACTIVITY, COGNITIVE CHANGES GI: LIVER FAILURE BLOOD: AGRANULOCYTOSIS, MEGALOBLASTIC ANEMIA OTHERS: LARYNGO- & BRONCO-SPASM, HYPOTENSION INTERACTION PB: LOWERS THEOPHYLLINE, ANTICOAGULANT & “PILL” TIDQID CONTRAINDICATIONS: OBSTRUCTIVE LUNG, PORPHYRIA RICKETS PERSONALITY CHANGES DOSE/PO START UP/W BY USUAL MAX MG/D 50-60 50 375-750 2000

PHENYTOIN (PHT) DOSE/PO USUAL MAX MG/D MG/KG/D 3-5 300 TR: 10-20 MG/L SIDE EFFECTS CNS: LETHARGY, ATAXIA, NYSTAGMUS, DYSTONIA GI: LIVER FAILURE BLOOD: LYMPHOCYTIC REACTION, PANCYTOPENIA, OTHERS: HIRSUTISM, COARSE FACE, BIG GUM (HYGIENE) INTERACTION PHT: LOWERS THEOPHYLLINE, ANTICOAGULANT & “PILL” BIDTID CONTRAINDICATIONS: CONDUCTION HEART PROBLEMS.5-3 MG/L (FREE) LYMPHADENOPATHY, LEARNING PROBLEMS MEGALOBLASTIC ANEMIA

75. ALL OF THE FOLLOWING DRUGS LOWER THEOPHYLLINE LEVELS, EXCEPT: A. PHENOBARBITAL B. PHENYTOIN C. PRIMIDONE D. VALPROIC ACID

76. THE HIGHEST DOSE OF ORAL PHENOBARBITAL IN A CHILD SHOULD NOT EXCEED: A. 250 MG PER DAY B. 250 MG/KG PER DAY C. 500 MG PER DAY D. 500 MG/KG PER DAY

76. THE HIGHEST DOSE OF ORAL PHENOBARBITAL IN A CHILD SHOULD NOT EXCEED: A. 250 MG PER DAY B. 250 MG/KG/DAY C. 500 MG/ DAY D. 500 MG/KG/DAY

77. A 3-YEAR-OLD BOY WITH STATIC ENCEPHALOPATHY MANIFESTED BY MENTAL RETARDATION, SPASTIC QUADRIPARESIS AND SEIZURES DEVELOPS DYSTONIC POSTURES. WHICH OF THE FOLLOWING ANTIEPILEPTIC DRUGS IS HE LIKELY USING? A. PHENOBARBITAL B. PHENYTOIN C. PRIMIDONE D. VALPROIC ACID

78. LARYNGOSPASM AND BRONCHOSPASM ARE COMPLICATIONS OF: A. PHENOBARBITAL B. PHENYTOIN C. CARBAMAZEPINE D. VALPROIC ACID

79. WHICH OF THE FOLLOWING ANTIEPILEPTIC DRUGS SHOULD BE AVOIDED IN PATIENT WITH CARDIAC DISEASE? A. PHENOBARBITAL B. PHENYTOIN C. CARBAMAZEPINE D. VALPROIC ACID

80. A CHILD JUST AFTER CARDIAC SURGERY IS USING ORAL ANTICOAGULATION. WHICH OF THE FOLLOWING DRUGS SHOULD YOU INITIATE IF HE HAS A SEIZURE? A. PHENOBARBITAL B. PHENYTOIN C. OXCARBAMAZEPINE D. VALPROIC ACID

OXCARBAMAZEPINE (OXC) DOSE/PO START UP IN USUAL WEIGHT (KG) G/D MG/KG/D 8-10 2 W 20-29:.9;29-39:1.2;>39:1.8 TR: 12-30 MG/L* SIDE EFFECTS CNS: HEADACHE, DIZZINESS, ATAXIA, DIPLOPIA GI: NAUSEA, VOMITING OTHERS: LOW SODIUM INTERACTION OXC: LOWERS “PILL” BIDTID SERUM SODIUM * 10-MONOHYDROXYL METABOLITE (MHD)

80. A CHILD JUST AFTER CARDIAC SURGERY IS USING ORAL ANTICOAGULATION. WHICH OF THE FOLLOWING DRUGS SHOULD YOU TRY TO INITIATE IF HE HAS A SEIZURE? A. PHENOBARBITAL B. PHENYTOIN C. OXCARBAMAZEPINE D. VALPROIC ACID

81. WHICH OF THE FOLLOWING DRUGS SHOULD BE AVOIDED DURING INFANCY BECAUSE OF THE POSSIBILITY OF HEPATIC FAILURE? A. PHENOBARBITAL B. PHENYTOIN C. PRIMIDONE D. VALPROIC ACID

82. A 12-YEAR-OLD WITH STATIC ENCEPHALOPATHY ON CARBAMAZEPINE DEVELOPS SUDDEN DETERIORATION IN HIS MENTAL STATUS AND RECURRENT SEIZURES. WHICH OF THE FOLLOWING TESTS SHOULD YOU ORDER TO DETERMINE THE CAUSE OF THE SUDDEN DETERIORATION OF MENTAL STATUS? A. CBC WITH DIFFERENTIAL B. LIVER FUNCTION TEST C. ELECTROLYTES D. AMMONIA LEVEL

82. A-12-YEAR OLD WITH STATIC ENCEPHALOPATHY ON CARBAMAZEPINE DEVELOPS SUDDEN DETERIORATION IN HIS MENTAL STATUS AND RECURRENT SEIZURES. WHICH OF THE FOLLOWING TESTS SHOULD YOU ORDER TO DETERMENT THE CAUSE OF THE SUDDEN DETERIORATION OF MENTAL STATUS? A. CBC WITH DIFFERENTIAL B. LIVER FUNCTION TEST C. ELECTROLYTES D. AMMONIA LEVEL

83. WHICH ANTIBIOTIC CAN PRODUCE DIPLOPIA AND ATAXIA IN A CHILD TAKING CARBAMAZEPINE? A. PENICILLIN B. AUGMENTIN C. SULFA D. ERYTHROMYCIN

84. LYMPHADENOPATHY IS A COMPLICATION OF WHICH OF THE FOLLOWING ANTIEPILEPTIC DRUGS: A. OXCARBAMAZEPINE B. PHENOBARBITAL C. VALPROIC ACID D. PHENYTOIN

85. GUM HYPERTROPHY IS AN UNAVOIDABLE COMPLICATION OF PHENYTOIN THERAPY? A. TRUE B. FALSE

86. FOLIC ACID SHOULD BE USED IN ALL FEMALES OVER 14 YEARS OF AGE WHO ARE TAKING WHICH OF THE FOLLOWING ANTIEPILEPTIC DRUGS? A. PRIMIDONE B. PHENOBARBITAL C. VALPROIC ACID D. PHENYTOIN

86. FOLIC ACID SHOULD BE USE IN ALL FEMALE OVER 14 YEARS OF AGE WHO IS TAKING WHICH OF THE FOLLOWING ANTIEPILEPTIC DRUGS? A. PRIMIDONE B. PHENOBARBITAL C. VALPROIC ACID D. PHENYTOIN

87. HIRSUTISM, GUM HYPERTROPHY, AND COARSE FACIES ARE SIDE EFFECTS OF: A. PRIMIDONE B. PHENOBARBITAL C. VALPROIC ACID D. PHENYTOIN

88. SALICYLATES INCREASED THE LEVELS OF A. PHENOBARBITAL B. VALPROIC ACID C. CARBAMAZEPINE D. OXCARBAMAZEPINE

ASPIRIN VALPROIC LEVEL

88. SALICYLATES INCREASED THE LEVELS OF: A. PHENOBARBITAL B. VALPROIC ACID C. CARBAMAZEPINE D. OXCARBAMAZEPINE

89. WHICH OF THE FOLLOWING ANTIEPILEPTIC DRUGS DO NOT AFFECT THE EFFICACY OF CONTRACEPTIVE DRUGS? A. PHENOBARBITAL B. CARBAMAZEPINE C. CLONAZEPAM D. ETHOSUXIMIDE

CLONAZEPAM (CLN) -DOSE START UP BY/W USUAL MAX MG/KG/D 0.01 0.02 0.1 0.2 TR:.02-.08 MG/L SIDE EFFECTS CNS: LETHARGY, ATAXIA, HYPERACTIVITY, PSYCHOSIS GI: INCREASED SALIVATION, HEPATITIS BLOOD: EOSINOPHILIA OTHERS: INCREASED BRONCHIAL SECRETION INTERACTION CLONAZEPAM IS DECREASED BY OTHER AED TID URINARY RETENTION

ETHOSUXIMIDE (ETZ) DOSE/PO START UP/W BY USUAL MAX MG/KG/D 10 5-10 15-20 40 TR: 40-100 MG/L SIDE EFFECTS CNS: SEDATION, HEADACHES, PSYCHOSIS GI: NAUSEA, VOMITING, HEPATITIS BLOOD: LOW WHITE & RED CELLS &/OR PLATELETS OTHERS: LUPUS-LIKE SYNDROME INTERACTION ETHOSUXIMIDE: LOWER VALPROIC AND “PILL” ETHOSUXIMIDE LEVEL INCREASED BY VALPROIC BIDTID CBC & LFT: ONSET, 3 WEEKS, & Q 3-6 MONTHS CONTRAINDICATION: PORPHYRIA, allergy: SUCCINIMIDE

89. WHICH OF THE FOLLOWING ANTIEPILEPTIC DRUGS DO NOT AFFECT THE EFFICACY OF CONTRACEPTIVE DRUGS? A. PHENOBARBITAL B. CARBAMAZEPINE C. CLONAZEPAM D. ETHOSUXIMIDE

90. RENAL STONES ARE A FREQUENT COMPLICATION OF WHICH OF THE FOLLOWING ANTIEPILEPTIC DRUGS? A. PHENOBARBITAL B. CLONAZEPAM C. TOPIRAMATE D. ETHOSUXIMIDE

CLONAZEPAM (CLN) -DOSE START UP BY/W USUAL MAX MG/KG/D 0.01 0.02 0.1 0.2 TR:.02-.08 MG/L SIDE EFFECTS CNS: LETHARGY, ATAXIA, HYPERACTIVITY, PSYCHOSIS GI: INCREASED SALIVATION, HEPATITIS BLOOD: EOSINOPHILIA OTHERS: INCREASED BRONCHIAL SECRETION INTERACTION CLONAZEPAM IS DECREASED BY OTHER AED TID URINARY RETENTION

TOPIRAMATE (TPM) DOSE/PO START UP/W BY USUAL MAX MG/KG/D 1-3 1-3 5-9 15-24 TR: 4-10 MG/L SIDE EFFECTS CNS: LETHARGY, ATAXIA, DIPLOPIA, AGGRESSIVITY GI: INCREASE SALIVATION, PANCREATITIS, ANOREXIA BLOOD: ACIDOSIS, BONE MARROW DEPRESSION OTHERS: RENAL STONES, ARRHYTHMIA INTERACTION TPM LOWERS DIGOXIN AND “PILL” BID CBC & LFT: ONSET, 3 WEEKS, & Q 3-6 MONTHS POOR CONCENTRATION, VISUAL FIELD DEFECT

ETHOSUXIMIDE (ETZ) DOSE/PO START UP/W BY USUAL MAX MG/KG/D 10 5-10 15-20 40 TR: 40-100 MG/L SIDE EFFECTS CNS: SEDATION, HEADACHES, PSYCHOSIS GI: NAUSEA, VOMITING, HEPATITIS BLOOD: LOW WHITE & RED CELLS &/OR PLATELETS OTHERS: LUPUS-LIKE SYNDROME INTERACTION ETHOSUXIMIDE: LOWER VALPROIC AND “PILL” ETHOSUXIMIDE LEVEL INCREASED BY VALPROIC BIDTID CBC & LFT: ONSET, 3 WEEKS, & Q 3-6 MONTHS CONTRAINDICATION: PORPHYRIA, allergy: SUCCINIMIDE

90. RENAL STONES ARE A FREQUENT COMPLICATION OF WHICH OF THE FOLLOWING ANTIEPILEPTIC DRUGS? A. PHENOBARBITAL B. CLONAZEPAM C. TOPIRAMATE D. ETHOSUXIMIDE

91. TO DEFINE ANY CONTINUOUS SEIZURES OR INTERMITTENT SEIZURES WITHOUT REGAINING CONSCIOUSNESS AS STATUS EPILEPTICUS, THE EVENT MUST LAST FOR: A. 10 MINUTES B. 20 MINUTES C. 30 MINUTES D. 60 MINUTES

CONVULSIVE STATUS EPILEPTICUS ABC, 100% OXYGEN MASK OR TUBE, VITAL SIGNS, BRIEF EXAM; VENOUS BLOOD: DEXTROSTIX, GLUCOSE, CALCIUM, ELECTROLYTES, AED LEVELS, (TOXICOLOGY AND METABOLIC SCREENING IF APPROPRIATE); ARTERIAL BLOOD GASES. > 30 MIN OF CONTINUOS CONVULSION > 30 MIN OF INTERMITTENT CONVULSIONS WITHOUT RECOVERY OF CONSCIOUSNESS DEXTROSTIX < 60: D 25 PUSH, 2-4 ML/KG (MAX: 100 ML) ON PHENOBARBITAL: FOSPHENYTOIN (FOS) NOT ON PHENOBARBITAL: LORAZEPAM & FOS LORAZEPAM MAY BE REPEATED IN 10 MINUTES, MAXIMUM OF 3 DOSES IF CONVULSION PERSISTS IN INFANTS: PYRIDOXINE 50-100 MG IV IF SEIZURES PERSISTS: EEG; INTUBATE, DIAZEPAM, PENTOTHAL;

0 15 30 45

91. TO DEFINE ANY CONTINUOUS SEIZURES OR INTERMITTENT SEIZURES WITHOUT REGAINING CONSCIOUSNESS AS STATUS EPILEPTICUS, THE EVENT MUST LAST FOR: A. 10 MINUTES B. 20 MINUTES C. 30 MINUTES D. 60 MINUTES

92. WHICH OF THE FOLLOWING IS NOT A MAJOR SUBTYPE OF STATUS EPILEPTICUS IN CHILDREN: A. PROLONGED FEBRILE SEIZURES B. IDIOPATHIC STATUS EPILEPTICUS C. SYMPTOMATIC STATUS EPILEPTICUS D. CRYPTIC STATUS EPILEPTICUS

MAJOR SUBTYPE OF STATUS EPILEPTICUS IN CHILDREN ARE PROLONGED FEBRILE SEIZURES, IDIOPATHIC STATUS EPILEPTICUS AND SYMPTOMATIC STATUS EPILEPTICUS

92. WHICH OF THE FOLLOWING IS NOT A MAJOR SUBTYPE OF STATUS EPILEPTICUS IN CHILDREN: A. PROLONGED FEBRILE SEIZURES B. IDIOPATHIC STATUS EPILEPTICUS C. SYMPTOMATIC STATUS EPILEPTICUS D. CRYPTIC STATUS EPILEPTICUS

93. WHICH OF THE FOLLOWING IS NOT A CAUSE OF STATUS EPILEPTICUS IN CHILDREN WITH SEIZURES: A. IRREGULAR USE OF ANTICONVULSANTS B. SLEEP DEPRIVATION C. COLD WEATHER D. INTERCURRENT INFECTION

COLD WEATHER IS NOT A CAUSE OF STATUS EPILEPTICUS IN CHILDREN WITH SEIZURES

93. WHICH OF THE FOLLOWING IS NOT A CAUSE OF STATUS EPILEPTICUS IN CHILDREN WITH SEIZURES: A. IRREGULAR USE OF ANTICONVULSANTS B. SLEEP DEPRIVATION C. COLD WEATHER D. INTERCURRENT INFECTION

94. WHICH OF THE FOLLOWING IS NOT AN INITIAL STEP IN THE TREATMENT OF STATUS EPILEPTICUS: A. NASOGASTRIC TUBE PLACEMENT B. INTRAVENOUS CATHETER PLACEMENT C. BLOOD PRESSURE MONITORING D. ENDOTRACHEAL INTUBATION

CONVULSIVE STATUS EPILEPTICUS ABC, 100% OXYGEN MASK OR TUBE, VITAL SIGNS, BRIEF EXAM; VENOUS BLOOD: DEXTROSTIX, GLUCOSE, CALCIUM, ELECTROLYTES, AED LEVELS, (TOXICOLOGY AND METABOLIC SCREENING IF APPROPRIATE); ARTERIAL BLOOD GASES. > 30 MIN OF CONTINUOS CONVULSION > 30 MIN OF INTERMITTENT CONVULSIONS WITHOUT RECOVERY OF CONSCIOUSNESS DEXTROSTIX < 60: D 25 PUSH, 2-4 ML/KG (MAX: 100 ML) ON PHENOBARBITAL: FOSPHENYTOIN (FOS) NOT ON PHENOBARBITAL: LORAZEPAM & FOS LORAZEPAM MAY BE REPEATED IN 10 MINUTES, MAXIMUM OF 3 DOSES IF CONVULSION PERSISTS IN INFANTS: PYRIDOXINE 50-100 MG IV IF SEIZURES PERSISTS: EEG; INTUBATE, DIAZEPAM, PENTOTHAL;

ENDOTRACHEAL INTUBATION IS NOT AN INITIAL STEP IN THE TREATMENT OF STATUS EPILEPTICUS ENDOTRACHEAL INTUBATION IS DONE ONLY IF RESPIRATORY DEPRESSION IS LIKELY

94. WHICH OF THE FOLLOWING IS NOT AN INITIAL STEP IN THE TREATMENT OF STATUS EPILEPTICUS: A. NASOGASTRIC TUBE PLACEMENT B. INTRAVENOUS CATHETER PLACEMENT C. BLOOD PRESSURE MONITORING D. ENDOTRACHEAL INTUBATION

95. ALL OF THE FOLLOWING DRUGS CAN POTENTIATE OR PRECIPITATE STATUS EPILEPTICUS, EXCEPT: A. AMPHETAMINES B. TRICYCLIC ANTIDEPRESSANTS C. PHENYLTHIAZINES D. GENTAMICIN

DRUGS THAT CAN POTENTIATE OR PRECIPITATE STATUS EPILEPTICUS ARE: (1) AMPHETAMINES, (2) TRICYCLIC ANTIDEPRESSANTS, (3) PHENYLTHIAZINES, (4) TOXIC LEVEL OF THEOPHYLLINE, (5) TRICYCLIC ANTIDEPRESSANTS, AND (6) COCAINE

95. ALL OF THE FOLLOWING DRUGS CAN POTENTIATE OR PRECIPITATE STATUS EPILEPTICUS, EXCEPT: A. AMPHETAMINES B. TRICYCLIC ANTIDEPRESSANTS C. PHENYLTHIAZINES D. GENTAMICIN

96. ALL OF THE FOLLOWING DRUGS CAN POTENTIATE OR PRECIPITATE STATUS EPILEPTICUS, EXCEPT: A. TOXIC LEVEL OF THEOPHYLLINE B. TRICYCLIC ANTIDEPRESSANTS C. SULFA D. COCAINE

DRUGS THAT CAN POTENTIATE OR PRECIPITATE STATUS EPILEPTICUS ARE: (1) AMPHETAMINES, (2) TRICYCLIC ANTIDEPRESSANTS, (3) PHENYLTHIAZINES, (4) TOXIC LEVEL OF THEOPHYLLINE, (5) TRICYCLIC ANTIDEPRESSANTS, AND (6) COCAINE

96. ALL OF THE FOLLOWING DRUGS CAN POTENTIATE OR PRECIPITATE STATUS EPILEPTICUS, EXCEPT: A. TOXIC LEVEL OF THEOPHYLLINE B. TRICYCLIC ANTIDEPRESSANTS C. SULFA D. COCAINE

97. WHICH OF THE FOLLOWING SHOULD BE USED TO TREAT STATUS EPILEPTICUS: A. INTRAMUSCULAR FOSPHENYTOIN B. PHENYTOIN MIXED WITH GLUCOSE SOLUTION C. INTRAVENOUS DIAZEPAM AT A DOSE OF 1 MG PER KILO D. INTRAVENOUS LORAZEPAM AT A DOSE 0.05 – 0.1 PER KILO

DIAZEPAM 1 MG/KG GLU RIP LORAZEPAM 0.05 – 0.1 MG/KG, MAXIMUM OF 5 MG PER DOSE, MAXIMUM OF 3 DOSES PER EVENT PHE

97. WHICH OF THE FOLLOWING SHOULD BE USED TO TREAT STATUS EPILEPTICUS: A. INTRAMUSCULAR FOSPHENYTOIN B. PHENYTOIN MIXED WITH GLUCOSE SOLUTION C. INTRAVENOUS DIAZEPAM AT A DOSE OF 1 MG PER KILO D. INTRAVENOUS LORAZEPAM AT A DOSE 0.05 – 0.1 PER KILO

98. DIAZEPAM RATE OF INFUSION SHOULD BE NO GREATER THAN: A. 2 MG PER MINUTE B. 3 MG PER MINUTE C. 4 MG PER MINUTE D. 5 MG PER MINUTE

DIAZEPAM (DZP) DOSE IV=.1-.3 MG/KG/DOSE MAY REPEAT 15 MIN TR: 0.1-1 MG/L SIDE EFFECTS CNS: SEDATION, IRRITABILITY, HEADACHE GI: NAUSEA OTHERS: THROMBOPHLEBITIS, INTERSTITIAL NEPHRITIS CONTRAINDICATIONS: GLAUCOMA, SEVERE PAIN HYPOTENSION, RESPIRATORY DEPRESSION DIZZINESS, DEPRESSION DRIP (50 MG IN 250 ML.9% NaCl OR D5W) AT A RATE OF.15-.6 MG/KG/HR. NEW INFUSION Q 12 H WEAN OVER 8-12 H IF NO SEIZURE IN 12-24 H FLUMAZENIL (ANTIDOTES) RELATIVE: HYPERBILIRUBINEMIA (BENZOATE) INFUSE AT A RATE NOT FASTER THAN 2 MG/MIN

98. DIAZEPAM RATE OF INFUSION SHOULD BE NO GREATER THAN: A. 2 MG PER MINUTE B. 3 MG PER MINUTE C. 4 MG PER MINUTE D. 5 MG PER MINUTE

99. WHICH OF THE FOLLOWING STATEMENTS IS NOT TRUE REGARDING THE USE OF PHENYTOIN DURING STATUS EPILEPTICUS: A. THE RATE OF INFUSION SHOULD BE 1 MG PER KILO PER MINUTE (UP TO 50 MG /MIN) B. PHENYTOIN SHOULD BE MIXED WITH NORMAL SALINE C. EKG TRACING IS RECOMMENDED DURING INFUSION D. SYSTEMIC HYPERTENSION MAY COMPLICATE INTRAVENOUS PHENYTOIN

PHENYTOIN (PHE) DOSE/IV LOAD USUAL/D IN RATE/MIN MG/KG 15-20 4-8 1 MG (< 50 MG) TR: 10-25 MG/L SIDE EFFECTS CNS: LETHARGY, ATAXIA, PSYCHOSIS, MOVEMENTS GI: LIVER FAILURE BLOOD: LOW WHITE CELLS &/OR PLATELETS OTHERS: ITCHING, NYSTAGMUS, HYPOTENSION INTERACTION FOS: LOWER ANTICOAGULANTS AND “PILL” FOS LEVEL INCREASED BY ETZ; DECREASED BY CBZ TID MONITOR HEART WHEN FIRST USED CONTRAINDICATION: HEART BLOCK OR LOW RATE BID

99. WHICH OF THE FOLLOWING STATEMENTS IS NOT TRUE REGARDING THE USE OF PHENYTOIN DURING STATUS EPILEPTICUS: A. THE RATE OF INFUSION SHOULD BE 1 MG PER KILO PER MINUTE B. PHENYTOIN SHOULD BE MIXED WITH NORMAL SALINE C. EKG TRACING IS RECOMMENDED DURING INFUSION D. SYSTEMIC HYPERTENSION MAY COMPLICATE INTRAVENOUS PHENYTOIN

100. THIOPENTAL SHOULD BE TITRATED TO ACHIEVE A BURST-SUPPRESSION EEG PATTERN. A. TRUE B. FALSE

PENTOBARBITAL (PTB) LOAD IV 5-15 MG/KG/1 HOUR EEG: B/S TR: 20-40 MG/L SIDE EFFECTS CNS: LETHARGY, HYPERACTIVITY, COGNITIVE CHANGES GI: NAUSEA AND VOMITING OTHERS: CORNEAL EDEMA, MYOCARDIAL DEPRESSION INTERACTION PTB: LOWERS ANTICOAGULANT & “PILL” CONTRAINDICATIONS: PORPHYRIA HYPOTENSION, HYPOTHERMIA PERSONALITY CHANGES THEN IV 1-5 MG/KG/1 HOUR EEG: B/S /24 H AFTER IV DECREASED 1 MG/KG/1 HOUR Q6H B/S: BURST SUPPRESSION

100. THIOPENTAL SHOULD BE TITRATED TO ACHIEVE A BURST-SUPPRESSION EEG PATTERN. A. TRUE B. FALSE

101. THE MORTALITY RATE OF STATUS EPILEPTICUS IS APPROXIMATELY: A. 5% B. 10% C. 15% D. 20%

102. RICKETS ASSOCIATED WITH ANTICONVULSANT THERAPY CAN BE PREVENTED BY PROVIDING: A. VITAMIN C B. VITAMIN D2 C. VITAMIN B1 D. VITAMIN B6

103. THE USUAL DOSE OF PYRIDOXINE IV IN AN INFANT WITH CONTINUOS SEIZURES IS: A. 100 MG/KG B. 200 MG/KG C. 300 MG/KG D. 400 MG/KG

CONVULSIVE STATUS EPILEPTICUS ABC, 100% OXYGEN MASK OR TUBE, VITAL SIGNS, BRIEF EXAM; VENOUS BLOOD: DEXTROSTIX, GLUCOSE, Ca, Mg, ELECTROLYTES, AED LEVELS, (TOXICOLOGY AND METABOLIC SCREENING IF APPROPRIATE); ARTERIAL BLOOD GASES. > 30 MIN OF CONTINUOS CONVULSION > 30 MIN OF INTERMITTENT CONVULSIONS WITHOUT RECOVERY OF CONSCIOUSNESS DEXTROSTIX < 60: D 25 PUSH, 2-4 ML/KG (MAX: 100 ML) ON PHENOBARBITAL: FOSPHENYTOIN (FOS) NOT ON PHENOBARBITAL: LORAZEPAM & FOS LORAZEPAM MAY BE REPEATED IN 10 MINUTES, MAXIMUM OF 3 DOSES IF CONVULSION PERSISTS IN INFANTS: PYRIDOXINE 50-100 MG IV IF SEIZURES PERSISTS: EEG; INTUBATE, DIAZEPAM, PENTOTHAL ;

103. THE USUAL DOSE OF PYRIDOXINE IV IN AN INFANT WITH CONTINUOS SEIZURES IS: A. 100 MG/KG B. 200 MG/KG C. 300 MG/KG D. 400 MG/KG

104. WHICH OF THE FOLLOWING TESTS SHOULD NOT BE ROUTINELY DONE IN A PATIENT WITH CONVULSIVE STATUS? A. CALCIUM B. ELECTROLYTES C. LIVER FUNCTION TESTS D. DEXTROSTIX

CONVULSIVE STATUS EPILEPTICUS ABC, 100% OXYGEN MASK OR TUBE, VITAL SIGNS, BRIEF EXAM; VENOUS BLOOD: DEXTROSTIX, GLUCOSE, CALCIUM, ELECTROLYTES, AED LEVELS, (TOXICOLOGY AND METABOLIC SCREENING IF APPROPRIATE); ARTERIAL BLOOD GASES. > 30 MIN OF CONTINUOS CONVULSION > 30 MIN OF INTERMITTENT CONVULSIONS WITHOUT RECOVERY OF CONSCIOUSNESS DEXTROSTIX < 60: D 25 PUSH, 2-4 ML/KG (MAX: 100 ML) ON PHENOBARBITAL: FOSPHENYTOIN (FOS) NOT ON PHENOBARBITAL: LORAZEPAM & FOS LORAZEPAM MAY BE REPEATED IN 10 MINUTES, MAXIMUM OF 3 DOSES IF CONVULSION PERSISTS IN INFANTS: PYRIDOXINE 50-100 MG IV IF SEIZURES PERSISTS: EEG; INTUBATE, DIAZEPAM, PENTOTHAL ;

104. WHICH OF THE FOLLOWING TESTS SHOULD NOT BE ROUTINELY DONE IN A PATIENT WITH CONVULSIVE STATUS? A. CALCIUM B. ELECTROLYTES C. LIVER FUNCTION TESTS D. DEXTROSTIX

105. NONCONVULSIVE STATUS EPILEPTICUS IS ALWAYS DUE TO PETIT MAL: A. TRUE B. FALSE

NONCONVULSIVE STATUS EPILEPTICUS ABC, 100% OXYGEN MASK OR TUBE, VITAL SIGNS, BRIEF EXAM; VENOUS BLOOD: DEXTROSTIX, LFT, GLUCOSE, Ca, Mg, ELECTROLYTES, AED LEVELS, (TOXICOLOGY AND METABOLIC SCREENING IF APPROPRIATE); ARTERIAL BLOOD GASES. EEG > 30 MIN OF UNCONSCIOUSNESS DEXTROSTIX < 60: D 25 PUSH, 2-4 ML/KG (MAX: 100 ML) ON PHENOBARBITAL: FOSPHENYTOIN (FOS) NOT ON PHENOBARBITAL: LORAZEPAM & FOS LORAZEPAM MAY BE REPEATED IN 10 MINUTES, IF CONVULSION PERSISTS IN INFANTS: PYRIDOXINE 50-100 MG IV COMPLEX PARTIALPETIT MALIV VALPROATE EEG

105. NONCONVULSIVE STATUS EPILEPTICUS IS ALWAYS DUE TO PETIT MAL: A. TRUE B. FALSE

106. THE TREATMENT OF PETIT MAL STATUS IS: A. FOSPHENYTOIN B. PHENOBARBITAL C. VALPROATE D. PHENYTOIN

NONCONVULSIVE STATUS EPILEPTICUS ABC, 100% OXYGEN MASK OR TUBE, VITAL SIGNS, BRIEF EXAM; VENOUS BLOOD: DEXTROSTIX, LFT, GLUCOSE, Ca, Mg, ELECTROLYTES, AED LEVELS, (TOXICOLOGY AND METABOLIC SCREENING IF APPROPRIATE); ARTERIAL BLOOD GASES. EEG > 30 MIN OF UNCONSCIOUSNESS DEXTROSTIX < 60: D 25 PUSH, 2-4 ML/KG (MAX: 100 ML) ON PHENOBARBITAL: FOSPHENYTOIN (FOS) NOT ON PHENOBARBITAL: LORAZEPAM & FOS LORAZEPAM MAY BE REPEATED IN 10 MINUTES, IF CONVULSION PERSISTS IN INFANTS: PYRIDOXINE 50-100 MG IV COMPLEX PARTIALPETIT MALIV VALPROATE EEG

DEPACON DOSE IV: 30 MG/KG (5 MG/KG/MIN) IF CONTINUES TR: 50-120 MG/L SECOND DOSE: 15 MG/KG WEAN BY 1 MG/KG/2 H IF NO SEIZURE IN 12-24 H DRIP: 5 MG/KG/H SIDE EFFECTS CNS: TREMOR, HYPERACTIVITY, BRAIN ATROPHY GI: NAUSEA, WEIGHT GAIN, PANCREATITIS, HEPATITIS BLOOD: LOW PLATELETS, APLASTIC ANEMIA OTHERS: POLYCYSTIC OVARIAN SYNDROME INTERACTION VPA LEVEL ARE INCREASED BY SALICYLATES LOW FACTOR VII, LOW CARNITINE CONTRAINDICATIONS: PORPHYRIA, LIVER DISEASE

106. THE TREATMENT OF PETIT MAL STATUS IS: A. FOSPHENYTOIN B. PHENOBARBITAL C. VALPROATE D. PHENYTOIN

107. LORAZEPAM SHOULD BE REPEATED 3 TIMES, IF IT INITIALLY DOES NOT WORK? A. TRUE B. FALSE

LORAZEPAM (LZP) DOSE IV=.05-.1 MG/KG/DOSE MAY REPEAT IN 15 MINUTES 3 TIMES TR: 0.2-0.5 MG/L SIDE EFFECTS CNS: SEDATION, DYSARTHRIA, OROFACIAL DYSKINESIA GI: INCREASED SALIVATION OTHERS: CARDIAC AND RESPIRATORY DEPRESSION CONTRAINDICATIONS: GLAUCOMA, APNEA, SEVERE PAIN HYPOTENSION, HYPERTENSION HALLUCINATION, ANTEROGRADE AMNESIA MAX PER DOSE: 5 MG

107. LORAZEPAM SHOULD BE REPEATED 3 TIMES, IF IT INITIALLY DOES NOT WORK? A. TRUE B. FALSE

108. WHEN GIVING FOSPHENYTOIN, WHICH OF THE FOLLOWING ORGANS SHOULD BE MOST CLOSELY MONITORED: A. LUNG B. HEART C. KIDNEY D. THYROID

FOSPHENYTOIN (FOS) DOSE/IV LOAD USUAL/D IN RATE/MIN MG/KG 15-20 4-8 1 MG (< 50 MG) TR: 10-25 MG/L SIDE EFFECTS CNS: LETHARGY, ATAXIA, PSYCHOSIS, MOVEMENTS GI: LIVER FAILURE BLOOD: LOW WHITE CELLS &/OR PLATELETS OTHERS: ITCHING, NYSTAGMUS INTERACTION FOS: LOWER ANTICOAGULANTS AND “PILL” FOS LEVEL INCREASED BY ETZ; DECREASED BY CBZ TID MONITOR HEART WHEN FIRST USED CONTRAINDICATION: HEART BLOCK OR LOW RATE BID

108. WHEN GIVING FOSPHENYTOIN, WHICH OF THE FOLLOWING ORGANS SHOULD BE MOST CLOSELY MONITOR: A. LUNG B. HEART C. KIDNEY D. THYROID

109. THE INFUSION OF FOSPHENYTOIN SHOULD NOT EXCEED __ MG/KG PER MIN? A. 1 B. 2 C. 3 D. 4

109. THE INFUSION OF FOSPHENYTOIN SHOULD NOT EXCEED __ MG/KG/MIN ? A. 1 B. 2 C. 3 D. 4

110. IN A 70 KG CHILD FOSPHENYTOIN SHOULD BE PUSH AT A RATE OF: A. 50 MG/KG PER MIN B. 70 MG/KG PER MIN C. 30 MG/KG PER MIN D. 10 MG/KG PER MIN

111. THE MOST FREQUENT SIDE EFFECT OF LORAZEPAM IS? A. MYOCARDIAC DEPRESSION B. APNEA C. LIVER FAILURE D. KIDNEY FAILURE

LORAZEPAM (LZP) DOSE IV=.05-.1 MG/KG/DOSE MAY REPEAT IN 15 MINUTES 3 TIMES TR: 0.2-0.5 MG/L SIDE EFFECTS CNS: SEDATION, DYSARTHRIA, OROFACIAL DYSKINESIA GI: INCREASED SALIVATION OTHERS: RESPIRATORY DEPRESSION, APNEA CONTRAINDICATIONS: GLAUCOMA, APNEA, SEVERE PAIN HYPOTENSION, HYPERTENSION, HALLUCINATION, ANTEROGRADE AMNESIA MAX PER DOSE: 5 MG

111. THE MOST FREQUENT SIDE EFFECT OF LORAZEPAM IS? A. MYOCARDIAC DEPRESSION B. APNEA C. LIVER FAILURE D. KIDNEY FAILURE

112. WHICH OF THE FOLLOWING DRUGS IS MORE EFFECTIVE AS DRIP? A. DIAZEPAM B. PHENOBARBITAL C. LORAZEPAM D. FOSPHENYTOIN

DIAZEPAM (DZP) DOSE IV=.1-.3 MG/KG/DOSE MAY REPEAT 15 MIN TR: 0.1-1 MG/L SIDE EFFECTS CNS: SEDATION, IRRITABILITY, HEADACHE GI: NAUSEA OTHERS: THROMBOPHLEBITIS, INTERSTITIAL NEPHRITIS CONTRAINDICATIONS: GLAUCOMA, SEVERE PAIN HYPOTENSION, RESPIRATORY DEPRESSION DIZZINESS, DEPRESSION DRIP (50 MG IN 250 ML.9% NaCl OR D5W) AT A RATE OF.15-.6 MG/KG/HR. NEW INFUSION Q 12 H WEAN OVER 8-12 H IF NO SEIZURE IN 12-24 H FLUMAZENIL (ANTIDOTES) RELATIVE: HYPERBILIRUBINEMIA (BENZOATE) INFUSE AT A RATE NOT FASTER THAN 2 MG/MIN

112. WHICH OF THE FOLLOWING DRUGS IS MORE EFFECTIVE AS DRIP? A. DIAZEPAM B. PHENOBARBITAL C. LORAZEPAM D. FOSPHENYTOIN

113. THE USUAL LOADING DOSE OF PHENOBARBITAL IN PATIENTS WITH STATUS EPILEPTICUS IS? A. 10 MG PER KG B. 20 MG PER KG C. 30 MG PER KG D. 40 MG PER KG

PHENOBARBITAL (PB) DOSE/IV LOAD AFTER MG/KG 20/DOSE 2-3/DAY ÷ 2 TR: 15-45 MG/L SIDE EFFECTS CNS: LETHARGY, HYPERACTIVITY, COGNITIVE CHANGES GI: LIVER FAILURE BLOOD: APLASTIC ANEMIA OTHERS: LARYNGO- & BRONCO-SPASM, HYPOTENSION INTERACTION PB: LOWERS THEOPHYLLINE, ANTICOAGULANT & “PILL” CONTRAINDICATIONS: OBSTRUCTIVE LUNG, PORPHYRIA PHLEBITIS PERSONALITY CHANGES INFUSION/RATE 1 MG/KG/MIN

113. THE USUAL LOADING DOSE OF PHENOBARBITAL IN PATIENTS WITH STATUS EPILEPTICUS IS? A. 10 MG PER KG B. 20 MG PER KG C. 30 MG PER KG D. 40 MG PER KG

114. ALL OF THE FOLLOWING ANTIEPILEPTIC DRUGS ARE ENZYME INDUCERS, EXCEPT: A. CARBAMAZEPINE B. PRIMIDONE C. PHENYTOIN D. PHENOBARBITAL

114. ALL OF THE FOLLOWING ANTIEPILEPTIC DRUGS ARE ENZYME INDUCERS, EXCEPT: A.CARBAMAZEPINE B.PRIMIDONE C.PHENYTOIN D.PHENOBARBITAL

115.WHICH OF THE FOLLOWING DRUGS INHIBITS THE ENZYME INVOLVED IN CARBAMAZEPINE METABOLISM? A. PENICILLIN B. ERYTHROMYCIN C. SULFA D. VANCOMYCIN

115.WHICH OF THE FOLLOWING DRUGS INHIBITS THE ENZYME INVOLVED IN CARBAMAZEPINE METABOLISM? A.PENICILLIN B.ERYTHROMYCIN C.SULFA D.VANCOMYCIN

116.BEHAVIORAL CHANGES OCCUR IN ____ PERCENT OF CHILDREN ON PHENOBARBITAL. A. 25% B. 50% C. 75% D. 100%

116.BEHAVIORAL CHANGES OCCUR IN ____ PERCENT OF CHILDREN ON PHENOBARBITAL. A.25% B.50% C.75% D.100%

117.WHICH OF THE FOLLOWING ANTIEPILEPTIC DRUGS HAS A HIGHER RISK OF COMPLICATIONS WHEN USED IN CONJUNCTION WITH THE KETOGENIC DIET? A. VALPROIC ACID B. PRIMIDONE C. PHENYTOIN D. PHENOBARBITAL

118.STATUS EPILEPTICUS IN CHILDREN HAS AN AVERAGE MORTALITY RATE OF: A. 2.5% B. 10% C. 15% D. 20%

119.THE DIFFERENTIAL DIAGNOSIS OF NONCONVULSIVE STATUS EPILEPTICUS INCLUDES ALL OF THE FOLLOWING, EXCEPT: A. TRANSIENT ISCHEMIC EVENT B. MIGRAINE C. FUGUE STATES D. REYE SYNDROME

120.A SAFE RATE OF FOSPHENYTOIN INFUSION IS: A. 1 MG/KG PER MINUTE B. 5 MG/KG PER MINUTE C. 10 MG/KG PER MINUTE D. 20 MG/KG PER MINUTE

120.A SAFE RATE OF FOSPHENYTOIN INFUSION IS: A. 1 MG/KG PER MINUTE (UP TO 3) B. 5 MG/KG PER MINUTE C. 10 MG/KG PER MINUTE D. 20 MG/KG PER MINUTE

121.FREQUENT AURA IN PATIENTS WITH COMPLEX PARTIAL SEIZURES ARE ALL OF THE FOLLOWING, EXCEPT: A. EPIGASTRIC DISCOMFORT B. FEAR C. UNPLEASANT FEELING D. SCOTOMAS

EPIGASTRIC DISCOMFORT FEAR UNPLEASANT FEELING

121.FREQUENT AURA IN PATIENTS WITH COMPLEX PARTIAL SEIZURES ARE ALL OF THE FOLLOWING, EXCEPT: A. EPIGASTRIC DISCOMFORT B. FEAR C. UNPLEASANT FEELING D. SCOTOMAS

122.WHICH OF THE FOLLOWING SYNDROMES HAS THE WORST PROGNOSIS? A. LENNOX-GASTAUT SYNDROME B. LANDAU-KLEFFNER SYNDROME C. ROLANDIC SEIZURES D. EPILEPSY JANZ

LENNOX-GASTAUT SYNDROME USUALLY LEAD TO MENTAL RETARDATION AND SEIZURES LANDAU-KLEFFNER SYNDROME SELDOMLY RECOVERS TALK, BUT SEIZURES ARE BETTER ROLANDIC SEIZURES: GOOD PROGNOSIS (NO MENTAL RETARDATION NO SEIZURES) JANZ EPILEPSY: NO MENTAL RETARDATION, BUT SEIZURES ARE FOR LIFE

122.WHICH OF THE FOLLOWING SYNDROMES HAS THE WORST PROGNOSIS? A. LENNOX-GASTAUT SYNDROME B. LANDAU-KLEFFNER SYNDROME C. ROLANDIC SEIZURES D. EPILEPSY OF JANZ

123.AFTER THE FIRST AFEBRILE SEIZURE, CHILDREN WITH NORMAL EEG, MRI, AND NO FAMILY HISTORY OF SEIZURES HAVE A _____ PERCENT CHANCE OF NOT HAVING ANOTHER SEIZURE: A. 25 B. 50 C. 75 D. 100

SHOW ME THE QUESTION AGAIN I WANT TO SEE THE CLUE, ANYWAY

123.AFTER THE FIRST AFEBRILE SEIZURE, CHILDREN WITH NORMAL EEG, MRI, AND NO FAMILY HISTORY OF SEIZURES HAVE A _____ PERCENT CHANCE OF NOT HAVING ANOTHER SEIZURE A.25 B.50 C.75 D.100

I HOPE GOING THROUGH THESE QUESTIONS WILL HELP YOU TO PASS YOUR BOARDS AND MAKE YOU A BETTER DOCTOR Israel Alfonso, MD THE END

INSTRUCTIONS READ EACH QUESTION GIVE IT YOUR BEST SHOT GOOD LUCK! Israel Alfonso, MD.

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INSTRUCTIONS READ EACH QUESTION GIVE IT YOUR BEST SHOT GOOD LUCK! Israel Alfonso, MD.

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