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Compiled PPS board reviewer > Neuro COPY > Flashcards

Neuro COPY Flashcards

1
Q

Cracked pot sound on percussion of the skull indicating hydrocephalus

A

MacEwen sign

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2
Q

Malformation consisting of cystic expansion of the 4th ventricle int eh posterior fossa and midline cerebellar hypoplasia, resulting from a developmental failure of the roof of the 4th ventricle during embryogenesis

A

Dandy-walker malformation

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3
Q

Brain weight:volume ratio >98th percentile for age (≥ 2 SD above the mean)

A

Megalencephaly

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4
Q

Most common megalencephalic syndrome; cerebral gigantism

A

Sotos syndrome

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5
Q

Cerebral hemispheres are absent or represented by membranous sacs with remnants of frontal, temporal, or occipital cortex dispersed over the membrane

A

Hydranencephaly

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6
Q

EEG findings in typical absence seizure

A

3 Hz spike-and-slow wave discharges

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7
Q

EEG findings in atypical absence seizure

A

1-2 Hz spike-and-slow wave discharges

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8
Q

__ seizures often occur at night and can be very numerous and brief

A

frontal lobe

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9
Q

EEG in patients with partial seizures usually show __ in the lobe where the seizure originates

A

focal spikes or sharp waves

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10
Q

Most common benign epilepsy syndrome with partial seizures
Child wakes up at night owing to simple partial seizures causing buccal and throat tingling and tonic or clinic contractions of one side of the face, with drooling and inability to speak but with preserved consciousness

A

Benign childhood epilepsy with centrotemporal spikes (BECTS)

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11
Q

EEG findings in BECTS

A

broad-based centrotemporal spikes that are markedly increased in frequency during drowsiness and sleep

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12
Q

Most common cause of surgically remediable partial epilepsy in adolescents and adults
Preceded by febrile seizures

A

Mesial temporal sclerosis

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13
Q

Form of chronic encephalitis that manifests with unilateral intractable partial seizures, epilepsia partialis continua, and progressive hemiparesis of the affected side, with progressive atrophy of the contralateral hemisphere

A

Rasmussen encephalitis

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14
Q

Typical absence seizures usually start at

A

5-8 yr

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15
Q

Consists of myoclonic and other seizures during the 1st yr of life, with generalized 3 Hz spike-and-slow wave discharges

A

Benign myoclonic epilepsy of infancy

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16
Q

Most common generalized epilepsy in young adults
Starts in adolescence with one or more of the ff: myoclonic jerks in the morning, often causing the patient to drop things; generalized tonic clonic or clonic-tonic-clonic seizures upon awakening; and juvenile absences
EEG shows generalized 4-5 Hz polyspike-and-slow wave discharges

A

Juvenile myoclonic epilepsy (Janz syndrome)

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17
Q

Starts during the first 2 mo of life with severe myoclonic seizures and burst suppression pattern on EEG

A

Early myoclonic infantile encephalopathy (EMIE)

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18
Q

Starts during the first 2 mo of life
Manifests as tonic seizures and is usually caused by brain malformations or syntaxin binding protein 1 mutations
Same EEG pattern as EMIE

A

Early epileptic infantile encephalopathy (EEIE) or Ohtahara syndrome

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19
Q

Starts as focal febrile status epilepticus and later manifests myoclonic and other seizure types

A

Severe myoclonic epilepsy of infancy or Dravet syndrome

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20
Q

Starts between the ages of 2 and 12 mo and consists of a triad of infantile spasms that usually occur in clusters, developmental regression and a typical EEG picture called hypsarrhythmia

A

West syndrome

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21
Q

Typically starts between the age of 2 and 10 yr and consists of a triad of:

  1. Developmental delay
  2. Multiple seizure types that as a rule include atypical absences and myoclonic, astatic and tonic seizures
  3. EEG findings 1-2 Hz spike-and-slow waves, polyspike bursts in sleep and a slow background in wakefulness
A

Lennox-Gestaut Syndrome

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22
Q

First line treatment of absence seizures

A

Ethosuximide

or valproic acid

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23
Q

First line treatment of complex partial seizures

A

oxcarbazepine

or carbamazepine

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24
Q

Drug of choice for juvenile myoclonic epilepsy

A

valproate

lamotrigine

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25
Q

Drug of choice for Lennox-Gestaut syndrome

A

valproate
topiramate
lamotrigine
rufinamide

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26
Q

Drug of choice for infantile spasms

A

ACTH

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27
Q

Drug of choice for West syndrome

A

ACTH

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28
Q

The most important factor for seizure relapse

A

abnormal EEG before medication is discontinued

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29
Q

Seizures that occur __ after AEDs are completely discontinued indicate relapse, and resumption is usually warranted

A

> 2-3 mo

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30
Q 
EEG manifestations of status epilepticus
Stage I
Stage II
Stage III
Stage IV
A

Stage I: initial distinct electrographic seizures
Stage II: waxing and waning electrographic seizures
Stage III: continuous electrographic seizures
Stage IV: Continuous ictal discharges punctuated by flat periods
Periodic epileptiform discharges on flat background

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31
Q

Migraine that persists beyond 72 hr

A

status migrainous

32
Q

Most commonly used preventive therapy for headache and migraine is

A

amitriptyline

33
Q

Classification of tension type headaches

A

Infrequent <12 times per year
Frequent 1-15 times per mo
Chronic >15 times per mo

34
Q

Criteria NF-1

CAFE SPOT

A

2/7:

  • 6 or more cafe-au-lait macules over 5 mm in greatest diameter in prepubertal individuals and over 15 mm in greatest diameter in postpubertal individuals
  • axillary or inguinal freckling consisting of multiple hyperpigmented areas 2-3 mm in diameter
  • 2 or more Lisch nodules
  • 2 or more neurofibromas or 1 plexiform neurofibroma
  • osseous lesion such as sphenoid dysplasia or cortical thinning of long bones
  • optic gliomas
  • first degree relative with NF-1
    mnemonic: CAFE SPOT
35
Q

Criteria NF-2

A

1/4:

  • bilateral vestibular schwannomas
  • a parent, sibling or child with NF-2 and either unilateral vestibular schwannomas or any 2 of the ff: meningioma, schwannoma, glioma, neurofibroma, posterior lenticular opacities
  • unilateral vestibular schwannoma and any 2: (same list as above)
  • multiple meningiomas (2 or more) and unilateral vestibular schwannoma or any 2: (same list as above
36
Q

Autosomal dominant disorder resulting from the loss of either tuberin or hamartin gene manifesting as numerous benign tumors (hamartomas)

A

tuberous sclerosis

37
Q

Criteria tuberous sclerosis

A

2 major or 1 major plus 1 minor features

38
Q

Major features of tuberous sclerosis

A
  • cortical tuber
  • subependymal nodule
  • subependymal giant cell astroyctoma
  • facial angriofibroma or forehead plaque
  • ungula or periungual fibroma
  • hypomelanotic macules (>3)
  • Shagreen patch
  • multiple retinal hamartomas
  • cardiac rhabdmyoma
  • renal angiomyolipoma
  • pulmonary lymphangioleiomyomatosis
39
Q

Minor features of tuberous sclerosis

A
  • cerebral white matter migration lines
  • multiple dental pits
  • gingival fibromas
  • bone cysts
  • retinal achromatic patch
  • confetti skin lesions
  • nonrenal hamartomas
  • multiple renal cysts
  • hamartomatous rectal polyps
40
Q

Lesons found along the wall of the lateral ventricles where they undergo calcification and project into the ventricular cavity, producing a candle-dripping appearance

A

subependymal nodules

41
Q

most common neurologic manifestation of TSC

A

epilepsy, cognitive impairment, autism spectrum disorders

42
Q

Hypomelanotic skin macules on the trunk and extremities seen in tuberous sclerosis

A

ash leaf lesions

43
Q

Roughened, raised lesion with an orange-peel consistency located primarily in the lumbosacral region

A

Shagreen patch

Tuberous sclerosis

44
Q

Treatment of subependymal giant cell astrocytomas in TBC patients not candidates for surgery

A

everlimus

45
Q

Routine follow-up of TBC patients

A

brain MRI every 1-3 yr
renal imaging every 1-3 yr
neurodevelopmental testing at the time of beginning 1st grade

46
Q

Sporadic vascular disorder and consists of a constellation of symptoms and signs including port-wine stain, leptomeningeal angioma and abnormal blood vessels of the eye leading to glaucoma

A

Sturge-Weber syndrome

47
Q

Typical seizure in SWS

A

focal tonic-clonic and contralateral to the side of the facial capillary malformation

48
Q

Pulsed dye laser therapy is used in?

A

clearing of port-wine stain in SWS

49
Q

Autosomal dominant disorder with features of cerebellar hemangioblastomas and retinal angiomas
Also associated with cystic lesions of the kidneys, pancreas, liver, and epididymis as well as pheochromocytomas

A

Von Hippel-Lindau disease

50
Q

Most common cause of death in Von Hippel-Lindau

A

renal carcinoma

51
Q

Sporadic condition characterized by a facial nevus and neurodevelopmental abnormalities

A

Linear nevus syndrome

52
Q

Components of PHACE syndrome

A 
Posterior fossa malfromations
Hemangiomas 
Arterial anomalies
Coarctation of the aora and other cardiac defects
Eye abnormalities
53
Q

The most common brain abnormality in PHACE syndrome

A

Dandy-Walker malformation

54
Q

Most common neuropathologic finding in spastic diplegia

A

PVL

55
Q

Most common lesions seen on pathologic examination or on MRI in spastic quadriplegia

A

severe PVL and multicystic cortical encephalomalacia

56
Q

Most common type of CP most likely associated with birth asphyxia

A

Athetoid CP/choreoathetoid, extrapyramidal, dyskinetic

57
Q

Treatment for uncomplicated penicillin-sensitive S. pneumoniae meningitis

A

3rd generation cephalosporin or penicillin x 10-14 days

58
Q

Treatment for uncomplicated N. meningitidis meningitis

A

penicillin x 5-7 days

59
Q

Treatment for uncomplicated H. influenzae b meningitis

A

3rd generation cephalosporin x 7-10 days

60
Q

Gram negative bacillary meningitis should be treated for __ or at least __ after CSF sterilization, which may occur after 2-10 days of treatment

A

3 weeks

or 2 weeks after CSF sterilization

61
Q

Data support the use of IV dexamethasone in treatment of children older than 6 wk with acute bacterial meningitis caused by __

A

H. influenzae type b

62
Q

Most common sequelae of bacterial meningitis

A

sensorineural hearing loss

63
Q

Most common cause of viral meningoencephalitis

A

Enterovirus

64
Q

Most common manifestation of CNS involvement in VZV infection

A

cerebellar ataxia

65
Q

Part of the brain commonly affected by:

  1. HSV
  2. Arboviruses
  3. Rabies
A
  1. HSV - cortex, esp temporal lobe
  2. Arboviruses - entire brain
  3. Rabies - basal structures
66
Q

EEG findings in viral encephalitis

A

diffuse slow-wave activity, usually without focal changes

67
Q

Brain abscess most common in which age group?

A

4-8 yr and neonates

68
Q

Most common pathogen in brain abscess in neonates

A

Citrobacter

69
Q 
A brain abscess can be treated with antibiotics without surgery if: 
1. size of abscess
2. duration
3. 
4.
A
  1. <2 cm
  2. <2 wk
  3. No signs of inc ICP
  4. Neurologically intact
70
Q

Indications for surgery

A
  • > 2.5 cm in diameter
  • gas is present in the abscess
  • lesion is multiloculated
  • located in the posterior fossa
  • fungus is identified
71
Q

Main pathogen in purulent meningitis in newborn to 3 months

A 
GBS
E. coli
L. monocytogenes
Enterococci
S. aureus and other Staph species
72
Q

Main pathogen in purulent meningitis in 2 m to 6 yr

A

H. influenzae type b
S. pneumoniae
N. meningitidis

73
Q

Main pathogen in purulent meningitis in >6 yr

A

N. meningitides

S. pneumoniae

74
Q 
Normal CSF
pressure
leukocyte count
protein
glucose
A

pressure 50-180 mmH2O
leukocyte <4, L: 60-70%, M 30-40%, N 1-3%
protein 20-45 mg/dL
glucose >50% of serum glucose

75
Q

Diagnostic criteria for transverse myelitis

A
  • bilateral sensorimotor and autonomic spinal cord dysfunction
  • clearly defined sensory level
  • progression to nadir of clinical deficits between 4 hr to 21 d after symptom onset
  • demonstration of spinal cord inflammation: CSF pleocytosis, elevated IgG index, or MRI revealing GAD enhancing cord lesion
  • exclusion of compressive, postradiation, neoplastic and vascular causes
76
Q

Most common hereditary neuromuscular disease

A

Duchenne muscular dystrophy

Compiled PPS board reviewer (60 decks)

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