Less than 2: Nonfebrile Seizures

Question 1

seizures that present as myoclonus and myoclonic seizures

Answer 1

infantile spasms
benign myoclonus of infancy
benign myoclonic epilepsy
severe myoclonic epilepsy of infancy
biotinidase deficiency
lennox-gastaut syndrome

Question 2

infantile spasms

Answer 2

age-dependent myoclonic seizures with underlying causes including: congenital malformations and perinatal asphyxia and tuberous sclerosis

Question 3

Aicardi syndrome

Answer 3

combination of infantile spasms, genesis of the corpus callous (as well as other midline cerebral malformations), and retinal malformations
always female - X-linked dominant

Question 4

infantile spasms onset

Answer 4

peak age is between 4 and 7 months, always before 1 year

Question 5

infantile spasm clinical feature

Answer 5

can be flexor, extensor, or mixed movement
generally occur in clusters during drowsiness, feedings, and shortly after the infant awakes from sleep
whether flexor or extensor, the movement is usually symmetrical and brief and tends to occur in clusters with similar intervals between spasms

Question 6

infantile spasm characteristic

Answer 6

rapid flexor spasm involving the neck, trunk, and limbs followed by a tonic contraction sustained for 2-10 seconds

Question 7

infantile spasm: flexor

Answer 7

rapid flexor spasm involving the neck, trunk, and limbs followed by a tonic contraction sustained for 2-10 seconds
less severe flexor spasms consist of dropping of the head, abduction of the arms, or by flexion at the waist

Question 8

infantile spasm: extensor

Answer 8

resemble the second component of the Moro reflex: the head moves backward and the arms suddenly spread

Question 9

infantile spasm: EEG

Answer 9

hypsarrhythmia
slow spike and wave
burst suppression

Question 10

benign myoclonus: EEG

Answer 10

normal

Question 11

benign myoclonic epilepsy: EEG

Answer 11

spike and wave (3 cps)

Question 12

severe myoclonic epilepsy: EEG

Answer 12

polyspike and wave (3 cps)

Question 13

lennox-gastaut: EEG

Answer 13

spike and wave (2-2.5 cps)

polyspike and wave (2-2.5cps)