Neurology

Question 1

Most common cause of seizures during the 1st 24 hours of life

Answer 1

HIE

Question 2

Characteristics of benign neonatal myoclonus

Answer 2

Occurs during sleep, suppressible by touch

Question 3

Jacksonian march occurs during which siezure type

Answer 3

Simple partial

Question 4

Automatisms occur during which seizure type

Answer 4

Complex partial

Question 5

Aura occurs prior to which seizure type

Answer 5

Simple partial

Question 6

Recurrence rate for febrile seizure

Answer 6

33%

Question 7

Age limit for febrile seizure

Answer 7

6 yo

Question 8

Vaccinations associated with febrile seizure

Answer 8

MMR, DTaP

Question 9

EEG findings of infantile spasms

Answer 9

hypsarrhythmia

Question 10

West syndrome

Answer 10

infantile spasms + arrest of psychomotor development

Question 11

Treatment for infantile spasms

Answer 11

ACTH, vigabratin

Question 12

EEG findigns in Landau-Kleffner

Answer 12

during sleep

Question 13

Characteristic of Landau-Kleffner

Answer 13

age 3-5 years, language regression

Question 14

EEG findings in absence seizures

Answer 14

3/second spike and slow wave complexes

Question 15

Treatment of absence seizures

Answer 15

ethosuximide, valproate

Question 16

Juvenile myoclonic epilepsy age group

Answer 16

adolescents

Question 17

Characteristics of juvenile myoclonic epilepsy

Answer 17

occur during waking, require lifelong treatment

Question 18

EEG findings of juvenile myoclonic epilepsy

Answer 18

generalized poly spikes

Question 19

Lennox-Gastaut prognosis

Answer 19

MR, intractable seizures

Question 20

Characterisitics of temporal lobe seizures

Answer 20

complex partial seizure with automatisms

Question 21

Characterisitics of benign focal epilepsy with centro-temporal spikes

Answer 21

age 8-10 yo, remit by 16 yo, nocturnal GTC

Question 22

Phenytoin side effects

Answer 22

hirsutism, gingival hypertrophy

Question 23

Topiramate side effects

Answer 23

weight loss, kideny stones, cognitive difficulties

Question 24

Valproate side effects

Answer 24

weight gain, PCOS, pancreatitis, hepatotoxicity

Question 25

Carbamazepine side effects

Answer 25

BM suppresssoin, hyponatremia

Question 26

Breath holding spells association

Answer 26

iron deficiency anemia

Question 27

Trigger for pallid breath holding spells

Answer 27

trivial head trauma, ocular pressure

Question 28

PANDAS

Answer 28

tic disorder or OCD triggered by GAS infection

Question 29

Stereotypies

Answer 29

non-purposeful repetitive movements in neurologically impaired children

Question 30

Spasmus nutans

Answer 30

head tilt, head titubation, nystagmus (begins in 1st month and remits by 1-2 years)

Question 31

Cataplexy

Answer 31

sudden loss of tone

Question 32

Migraine epidemiology

Answer 32

More common in girls AFTER puberty

Question 33

Acute confusional migraine trigger

Answer 33

mild head trauma

Question 34

Basilar artery migraine symptoms

Answer 34

ataxia, nausea, dysarthria, hemiparesis, followed by occipital headache

Question 35

Most prevalent neurocutaneous disorder

Answer 35

NF type 1

Question 36

NF1 genetics

Answer 36

AD

Question 37

Diagnostic criteria for NF1

Answer 37

cafe au lait macules, neurofibromas. axillary/inguinal freckling, optic glioma, lisch nodules, osseous lesions

Question 38

NF1 association

Answer 38

learning disabilities (35%)

Question 39

NF2 genetics

Answer 39

AD

Question 40

NF2 presentation

Answer 40

hearing loss, late onset (avg. age 22 years)

Question 41

Tuberous sclerosis genetics

Answer 41

AD

Question 42

Disorder associated with infantile spasms

Answer 42

Tuberous sclerosis

Question 43

Tuberous sclerosis findings

Answer 43

ash leaf spots, shagreen patch, adenoma sebaceum, subungal fibromas, MR, intracranial calcifications

Question 44

High risk for Sturge-Weber

Answer 44

bilateral, V1, V2+V3, eyelids

Question 45

Sturge-Weber complications

Answer 45

glaucoma, retinal detachment, MR, seizures

Question 46

PHACE syndrome

Answer 46

posterior fossa brain malformation, hemagiomas, arterial anomalies, cardiac anomalies, eye abnormalities, sternal cleft

Question 47

SMA genetics

Answer 47

AR

Question 48

Muscular dystrophy genetics

Answer 48

XLR - Duchenne's, Beckert | AD - myotonic dystrophy

Question 49

Myotonic dystrophy genetics

Answer 49

maternal transmission, trinucleotide repeat disorder

Question 50

Dandy-Walker

Answer 50

agenesis of cerebellar vermis, dilation of 4th ventricle, enlarged posterior fossa

Question 51

Most common inherited ataxic condition

Answer 51

Friedreich's ataxia

Question 52

Friedreich's ataxia genetics

Answer 52

trinucleotide repeat disorder (GAA)

Question 53

Findings in Friedreich's ataxia

Answer 53

dysarthria, high-arched foot, cardiomyopathy, afib, diabetes

Question 54

Rett syndrome genetics

Answer 54

X-linked dominant

Question 55

Wilson's disease presentation

Answer 55

de novo psychiatric illness and a movement disorder

Question 56

Alpers-Huttenlocher syndrome

Answer 56

DD, intractable seizures (may be focal), cortical atrophy, liver failure

Question 57

Congenital myopathy with ptosis and ophthalmoplegia

Answer 57

myotubular myopathy

Question 58

Congenital myopathy associated with malignant hyperthermia

Answer 58

central core, nemaline rod, minicore myopathies

Question 59

Nemaline rod myopathy facies

Answer 59

elongated, narrow face with facial diplegia, lower jaw open

Question 60

Difference between BMD and DMD

Answer 60

``` DMD = no dystrophin BMD = decreased dystrophin ```

Question 61

Emery-Dreifuss MD natural history

Answer 61

slowly progessive weakness, survival depends on cardiac involvement

Question 62

Myotonic dystrophy findings

Answer 62

distal weakness, ptosis, elongated facies, frontal balding, testicular atrophy, cataracts, heart disease

Question 63

Congential muscular dystrophy that is more severely affected

Answer 63

merosin negative

Question 64

Limb-Girdle muscular dystrophy findings

Answer 64

proximal muscle weakness (face sparing), muscle wasting

Question 65

Ulrich muscular dystrophy

Answer 65

hypotonia, proximal joint contractures, hyperlaxity of hands, prominent calcenei (merosin negative)

Question 66

Drug-drug interaction: Carbamazepine

Answer 66

macrolide antibiotics

Question 67

Vital sign abnormality in hyponatremic seizures

Answer 67

hypothermia