Pediatric Neurology Flashcards

1
Q

Normal Developmental Age upper range for:
Mimics action of others

A

18 months

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2
Q

Normal Developmental Age upper range for:
Supports weight on forearms

A

3 months

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3
Q

Normal Developmental Age upper range for:
Pincer grasp

A

9 months

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4
Q

Normal Developmental Age upper range for:
Babbles

A

6 months

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5
Q

Normal Developmental Age upper range for:
Runs

A

24 months

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6
Q

Normal Developmental Age upper range for:
Feeds self from spoon

A

18 months

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7
Q

Normal Developmental Age upper range for:
Shows likes and dislikes

A

6 months

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8
Q

Normal Developmental Age upper range for:
1-2 meaningful words

A

12 months

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9
Q

Normal Developmental Age upper range for:
Smiles appropriately

A

3 months

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10
Q

Normal Developmental Age upper range for:
Walks with 1 held hand

A

12 months

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11
Q

Normal Developmental Age upper range for:
Plays with others

A

24 months

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12
Q

Normal Developmental Age upper range for:
Pulls to stand

A

9 months

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13
Q

Normal Developmental Age upper range for:
Coos, laughs

A

3 months

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14
Q

Normal Developmental Age upper range for:
Comes when called

A

12 months

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15
Q

Normal Developmental Age upper range for:
Transfers objects

A

6 months

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16
Q

Normal Developmental Age upper range for:
Walks upstairs with assistance

A

18 months

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17
Q

Normal Developmental Age upper range for:
Releases an object on command

A

12 months

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18
Q

Normal Developmental Age upper range for:
Imitates sounds

A

9 months

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19
Q

Normal Developmental Age upper range for:
Opens hands spontaneously

A

3 months

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20
Q

Normal Developmental Age upper range for:
2- to 3-word sentences

A

24 months

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21
Q

Normal Developmental Age upper range for:
Plays pat-a-cake, peek-a-boo

A

9 months

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22
Q

Normal Developmental Age upper range for:
Builds a tower of 6 blocks

A

24 months

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23
Q

Normal Developmental Age upper range for:
At least 6 words

A

18 months

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24
Q

Platelet count that contraindicates lumbar puncture

A

<20 x10^9/L

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25
Q

Lumbar puncture positioning for neonates

A

seated position

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26
Q

Vertebral level for Lumbar puncture

A

Between L4-L5 interspace
or
L5-S1

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27
Q

Volume of CSF allowed to drip from lumbar puncture

A

10-15 mL

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28
Q

Imaging method of choice for detecting
- Intracranial hemorrhage
- Periventricular leukomalacia
- Hydrocephalus in infants with patent anterior fontanelles

A

Cranial ultrasonography

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29
Q

Age that may require sedation for brain MRI

A

< 8 yo

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30
Q

This NTD covers the largest proportion of congenital anomalies in the CNS

A

Hydrocephalus

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31
Q

When does the neural tube close in utero?

A

3rd or 4th week

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32
Q

What is VACTERL-H?

A

Vertebral, Anal, Cardiac, Tracheoesophageal, Renal, and Limb anomalies, plus hydrocephalus

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33
Q

Most common type of hydrocephalus in children

A

Obstructive or Noncommunicating hydrocephalus

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34
Q

Most prominent sign of hydrocephalus in an infant

A

accelerated rate of enlargement of the head

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35
Q

Most prominent sign of hydrocephalus in older patients

A

headache

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36
Q

This percussion sign of the skull seen in hydrocephalus indicates separation of the sutures

A

Cracked pot sound or Macewen sign

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37
Q

This deformity consists of displacement of the cerebellar tonsils into the cervical canal, although not usually associated with hydrocephalus

A

Type I Chiari malformation

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38
Q

This deformity consists of displacement of the inferior vermis, pons, and medulla into the cervical canal, and it is characterized by progressive hydrocephalus with myelomeningocele

A

Type II Chiari malformation

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39
Q

Consists of a cystic expansion of the fourth ventricle in the posterior fossa and midline cerebellar hypoplasia, which results from a developmental failure of the roof of the fourth ventricle during embryogenesis

A

Dandy-Walker Malformation

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40
Q

Medications that may reduce the rate of CSF production providing a temporary relief in hydrocephalus in cases such as Dandy-Walker Syndrome

A

acetazolamide and furosemide

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41
Q

Other name for Spina Bifida Occulta

A

Occult Spinal Dysraphism

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42
Q

The most severe form of dysraphism

A

Myelomeningocele

Open or Spina bifida aperta

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43
Q

Folic acid supplementation should be initiated before conception and continued until at least how many weeks of gestation?

A

12 weeks

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44
Q

All women of childbearing age who can become pregnant should take how much folic acid daily?

A

0.4 mg

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45
Q

Women with child previously affected with NTD shall be started with how much folic acid daily, 1 month prior to planned conception?

A

4 mg

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46
Q

Large defect of the calvarium, meninges, and scalp associated with a rudimentary brain

A

Anencephaly

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47
Q

Characteristic of seizure described as increased tone or rigidity usually lasting 2 sec up to several minutes

A

Tonic

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48
Q

Characteristic of seizure described as rhythmic, fast muscle contractions and slightly longer relaxations

A

Clonic

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49
Q

Characteristic of seizure described as a shock-like contraction of a muscle of < 50 msec

A

Myoclonic

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50
Q

Characteristic of seizure described as flaccidity or lack of movement

A

Atonic

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51
Q

The most common aura experience by children consists of:

A

Epigastric discomfort or pain and a feeling of fear

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52
Q

Routine _____ should be performed in all cases of a first unprovoked nonfebrile seizure to help predict risk of recurrence

A

EEG

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53
Q

________ is preferred over CT scan and performing this on a nonemergent basis should be considered in patients with seizures

A

Brain MRI

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54
Q

This is previously referred to as pseudotumor cerebri

A

Idiopathic intracranial hypertension

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55
Q

T/F:
Lumbar puncture is contraindicated in critical illnesses and skin infection at the site of puncture

A

True

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56
Q

This useful tool is done by administering a light bolus of iodinated contrast through a large-bore intravenous catheter then acquiring CT images as contrast passes through the arteries within the cranium

A

Cranial CT angiography

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57
Q

Non-invasive procedure, well-suited for detecting a variety of abnormalities, including those of the posterior fossa and spinal cord

A

Brain MRI

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58
Q

Give 4 disorders of structure specification in CNS congenital anomalies

A

Gray matter structures
Neuronal migration disorders
Disorders of connectivity
Commissure and tract formation

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59
Q

Which of the following is not an etiologic factor for hydrocephalus?
A. Hypothermia
B. Valproic acid intake
C. Low red cell folate levels
D. Maternal obesity or diabetes

A

A.

It should be hyperthermia

Valproic acid intake
Low red cell folate levels
Maternal obesity or diab

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60
Q

The putative genetic link for VACTERL-H

A

PTEN

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61
Q

Abnormality of which structures in the brain most often causes non-communicating hydrocephalus in children?

A

Aqueduct of Sylvius and Fourth Ventricle

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62
Q

Type II Chiari malformation represents an anomaly of the hindbrain, probably owing to a failure of _______ development during embryogenesis

A

pontine flexure

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63
Q

This is the more accurate diagnostic tool for Spinal Bifida occulta at any age

A

MRI

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64
Q

This is a fluctuant midline mass of the spinal column that might transilluminate usually in the lower back

A

Meningocele

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65
Q

How many percent of myelomeningocele is located in the lumbosacral region?

A

75%

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66
Q

At what locations does myelomeningocele typically have either a sac-like cystic structure covered by a thin layer of partially epithelialized tissue or an exposed flat neural placode without overlying tissues?

A

Mid-lumbar or high lumbo-thoracic region

67
Q

T/F: Surgery of Myelomeningocele can be delayed

A

True except when there is CSF leak

68
Q

Percent recurrence of Myelomeningocele if one affected child is ________ vs with two affected children: ___________

A

One = 3-4%
Two = 10%

69
Q

This agent in the conversion of homocysteine to methionine with Folic Acid is important for in vivo methylation

A

S-adenosylmethionine

70
Q

Which of the following is not an enzyme important in homocysteine metabolism in the pathogenesis of meningomyelocele?
A. 5,10-methylenetetrahydrofolate reductase
B. Cystathionine B-synthase
C. γ-glutamyl hydrolase
D. Methionine synthase

A

C.

71
Q

Folic acid supplementation reduced incidence of NTDs in pregnancies at risk by at least ____%

A

50%

72
Q

This contains a cranial sac plus cerebral cortex, cerebellum, or portions of the brainstem

A

Encephalocele

73
Q

Microcephaly is head circumference that measures __________ SD below the mean for age and sex

A

> 3 SD

74
Q

Microcephaly and MR are most severe with radiation exposure before ____ wk of gestation

A

15th

75
Q

Significant fever during the first _______ wk has been reported to cause microcephaly, seizures, and facial anomalies

A

4-6 wk

76
Q

A ________ is a transient occurrence of signs and/or symptoms resulting from abnormal excessive or synchronous neuronal activity in the brain

A

seizure

77
Q

Determining if the epilepsy fits into a particular epilepsy syndrome is at which level of diagnostic and classification scheme of epilepsies?

A

Level 3

1: Know if the event was epileptic seizure
2: type of epilepsy
4: dx

78
Q

Which type of seizure manifests as forceful turning of the head and eyes to one side, unilateral clonic movements beginning in the face or extremities, or a sensory disturbance (e.g., paresthesia, pain) localized to a specific area?

A

Focal seizure

79
Q

Febrile seizures in children typically occur between what age range?

A

6-60 mos

80
Q

What is the temperature of children having febrile seizures

A

38C/100.4F or higher

81
Q

What is the maximum duration for a simple febrile seizure before it can be considered as complex febrile seizure?

A

15 min

82
Q

Which type of febrile seizure manifests as Focal?

A

Complex Febrile Seizure

83
Q

Which type of febrile seizure recurs within 24 hours?

A

Complex Febrile Seizure

84
Q

Which type of febrile seizure manifests as primary generalized, usually tonic-clonic?

A

Simple Febrile Seizure

85
Q

Do Simple Febrile Seizures recur?

A

No

86
Q

This pertains to a febrile seizure lasting longer than 30 min

A

Febrile status epilepticus

87
Q

This disorder is seen predominantly in older (>5 years) usually male children and associated with an encephalitis-like illness but without an identifiable infectious agent.

A

Febrile Infection-Related (Refractory) Epilepsy

aka FIRES

88
Q

What are the major criteria for the recurrence of febrile seizure?

A

Age < 1 year
Duration of fever < 24 hours
Fever of 38-39C (100.4-102.2F)

89
Q

What gender is a minor risk factor for Febrile Seizure recurrence?

A

Male

90
Q

T/F:
Blood studies are not routinely recommended in Simple Febrile Seizures

A

T

91
Q

What laboratory test should be requested or measured initially and with prolonged postictal obtundation or with poor oral intake during febrile seizures?

A

Blood glucose

92
Q

Which meds are used for the acute treatment of Febrile seizures?

clue: 3 benzodiazepines

A

Lorazepam, midazolam, or diazepam

93
Q

T/F:
Use of continuous antiepileptic therapy in febrile seizures is justified

A

F

due to lack of demonstrated long-term benefits

94
Q

T/F:
Antipyretics reduce the risk of having a recurrent febrile seizure

A

F

These can decrease the discomfort but not the risk of recurrence

95
Q

Absence seizures start at what age range?

A

5-8 yrs

96
Q

T/F:
Eyelid flutter or upward rolling of eyes are manifestations of absence seizure

A

T

97
Q

DOC for Absence seizure

A

Ethosuximide

98
Q

What is the most common generalized motor seizures?

A

Generalized tonic-clonic seizures

99
Q

DOC for Janz syndrome

A

Valproic acid

100
Q

This is the most common general epilepsy in young adults, accounting for 5% of all epilepsies

A

Juvenile Myoclonic Epilepsy (Janz syndrome)

101
Q

Severe generalized epilepsy occurring as early as 2 month-old due to inborn errors of metabolism, characterized as severe myoclonic seizure with burst suppression pattern of EEG

A

Early Myoclonic Infantile Encephalopathy

102
Q

Severe generalized epilepsy occurring as early as 2 month-old due to brain malformation or syntaxin-binding protein 1 mutations, characterized as tonic seizures with burst suppression pattern in ECG

A

Early Infantile Epileptic Encephalopathy

Ohtahara syndrome

103
Q

West syndrome often starts between what age?

A

2-12 months

104
Q

What is the triad of West Syndrome?

A

SRH
infantile epileptic Spasms
developmental Regression
Hypsarrhythmia in EEG

105
Q

Gene mutations in males with West Syndrome

A

ARX gene

106
Q

What is the triad of Lennox-Gastaut Syndrome?

A

DMT

Developmental Delay
Multiple Seizure types
Typical EEG

107
Q

Autoimmune reaction to which parasite can trigger Lennox-Gastaut Syndrome?

A

Onchocerca volvulus

108
Q

A child with one unprovoked seizure can permitted for moderate-risk sports if he’s seizure free from ____ mos?

A

> 12 mos

109
Q

Allergic hepatitis and agranulocytosis should be monitored especially on the first ____ months of Anti-epileptic drugs (AED) therapy

A

3-6 months

110
Q

Discontinuation of AEDs is usually indicated when children are free of seizures for at least ____ years

A

2 years

111
Q

What are the factors that should be considered before discontinuing AEDs?

A

Likelihood of remaining seizure-free after drug withdrawal
Risk of injury in case of seizure recurrence

112
Q

Most common type of Status Epilepticus in children

A

Febrile Status Epilepticus

113
Q

2 molecular mechanisms of status epilepticus

A

Failure of desensitization of AMPA glutamate
Reduction of GABA-mediated inhibition due to internalization of GABAA receptors

114
Q

At least how many attacks is required in diagnosis of Migraine with Typical or Brainstem Aura

A

2 attacks

115
Q

How long should vestibular symptoms last in the criteria for vestibular migraine with vertigo?

A

5 min to 72 hours

116
Q

How many days in a month for how many months should headaches occur to fulfill the criteria for chronic migraine

A

15 days or more in a month for more than 3 months

117
Q

Acute treatment strategy should be developed for stopping a headache attack on a consistent basis with return to function as soon as possible, with the goal being ___ hours maximum

A

2 hours

118
Q

How many days per year are headaches required to occur for the diagnosis of infrequent episodic tension-type headache?

A

<12 days/yr

10 episodes of headache occuring on <1 day per month or <12 days per yr

119
Q

This neurocutaneous syndrome manifests as distinctive skin lesion appearing in 4 stages (bullous, verrucous, pigmentary, atretic)

A

Incontinentia pigmenti

120
Q

PHACE syndrome

A

Posterior fossa malformation
Hemangioma
Arterial lesions
Cardiac defects (COA)
Eye or ocular defect
Ventral defect (sternal clefting)

121
Q

These are autosomal dominant disorders that cause tumors to grow on nerves and result in other systemic abnormalities

A

Neurofibromatosis

122
Q

Number of Cafe au lait spots suggestive of NF1

A

6 or more

123
Q

Diameter of cafe au lait spots in children with NF1

A

> 0.5 cm

124
Q

Diameter of cafe au lait spots in adolescents with NF1

A

> 1.5 cm

125
Q

This is the pathognomonic sign for von Recklinghausen neurofibromatosis

A

Axillary Freckling or Crowe’s sign

126
Q

These are pigmented hamartomas of the iris seen in NF1

A

Lisch nodules

127
Q

This is a multisystemic AD neurocutaneous disease manifesting with hypomelanotic macules, facial angiofibromas, Shagreen patch, retinal hamartomas, or lymphangioleiomyomatosis

A

Tuberous sclerosis complex

128
Q

Tremors result from the action of which muscles?

A

Antagonistic muscles

129
Q

This is the most common and costly form of chronic motor disability that begins in childhood

A

Cerebral Palsy

130
Q

Most common neuropathologic finding in children with spastic diplegia that is visualized on MRI in more than 70% of cases

A

Periventricular Leukomalacia

131
Q

Most severe form of CP

A

Spastic quadriplegia

132
Q

This medication given to mothers in premature labor (before 32 wk AOG) showed significant reduction of CP at 2 yrs of age

A

Magnesium sulfate

133
Q

Stroke in pediatrics often involve which artery territory?

A

Middle cerebral artery

134
Q

The most common focal presentation of stroke in children is:

A

hemiparesis

135
Q

Instantaneous or thunderclap headaches are feature of which Stroke?

A

Acute Hemorrhagic stroke

136
Q

Imaging tool that is highly sensitive to acute hemorrhagic stroke

A

CT scan

137
Q

Which infections remain a significant cause of CNS disease

A

Viral infections

138
Q

Pair the treatment duration for the following type of meningitis:
1. Meningococcal
2. Pneumococcal
3. Influenza

A. 5-7 days
B. 7-10 days
C. 10-14 days

A
  1. A
  2. C
  3. B
139
Q

Name the medications for the following type of meningitis:
1. Meningococcal
2. Pneumococcal
3. Influenza

A
  1. M: IV penicillin or ceftriaxone
  2. P: third-gen cephalosporin, IV penicillin, vancomycin
  3. I: ampicillin or third-gen cephalosporin
140
Q

Medications for patients without identifiable pathogens in LP but have evidence of bacterial meningitis in CSF profile

A

Cefotaxime or Ceftriaxone for 7-10 days

141
Q

These medications reduce hearing loss in patients with Hib meningitis

A

Corticosteroids

142
Q

What is the chemoprophylaxis for Meningococcal meningitis?

A

Rifampin 10mg/kg/day q 12hrs x 2 days (600 mg max)

143
Q

Instead of antibiotic prophylaxis for contacts of children with pneumococcal meningitis, what interventions should be routinely given?

A

PCV13 vaccine for children < 5 years old

144
Q

How many percent of cases do multiple brain abscess present?

A

18%

145
Q

How may percent of cases have no predisposing risk factor for brain abscess was identified?

A

20%

146
Q

Frontal lobe abscesses are often caused by extension of infections from:

A

sinusitis or orbital cellulitis

147
Q

Temporal lobe or cerebellar abscesses are often caused by extension of infections from:

A

Otitis media or mastoiditis

148
Q

Predominant pathogenic organisms that cause brain abscesses

A

Streptococci

149
Q

Diagnostic test of choice for differentiating brain abscesses from cysts and necrotic tumors

A

Brain MRI

150
Q

Empiric therapy for brain abscess

A

Combination of third-gen cephalosporin, metronidazole, and vancomycin

151
Q

4 obligatory criteria of muscular dystrophy

A

Primary myopathy
Genetic basis
Progressive course
Degeneration and death of muscle fibers

152
Q

Gower’s sign in DMD is nearly always evident by age:

A

5 or 6 years

seen by 3 years of age

153
Q

Most common pathogens for GBS:
1. GI
2. Respiratory
3. Systemic

A
  1. Campylobacter jejuni
  2. Mycoplasma pneumoniae
  3. Zika virus
154
Q

T/F:
GBS may follow administration of vaccines

A

T

155
Q

Maximal severity of weakness in GBS is reached by _____ weeks

A

4 weeks after onset

156
Q

Which patterns of GBS causes ataxia and external ophthalmoplegia?

A

Miller-Fisher syndrome
Bickerstaff’s brainstem encephalitis

157
Q

Which pattern of GBS causes hypersomnolence?

A

Bickerstaff’s brainstem encephalitis

158
Q

T/F:
There should be FEWER than 10 WBC per mm3 in GBS

A

T

159
Q

T/F:
CSF glucose is elevated more than twice in GBS

A

F

CSF protein is elevated more than twice
Glucose level is normal

160
Q

Treatment for severe or rapidly progressive muscle weakness in GBS

A

IVIG 0.4 g/kg/day x 5 days or 1g/kg/day x 2 days
(does not alter long-term outcome)

161
Q

What is the most common long-term residuum of GBS?

A

Fatigue

162
Q

Common infections that cause Bell’s palsy

A

HSV1, VZV, Lyme disease

163
Q

This is recommended to be started within the first 3 days of onset of bell’s palsy

A

oral prednisone

164
Q

T/F: Acute motor axonal neuropathy has no feature of demyelination in Nerve conduction studies

A

T