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pediatric Boards > neurology > Flashcards

neurology Flashcards

0
Q

What diagnosis should you consider in a patient with confusion and irritability after being in contact with a person with diagnosis of meningitis?

A

Enterovirus encephalitis

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1
Q

What diagnosis should you consider in a patient who has signs of encephalitis after mosquito bites and lives in a warm climate ?

A

Arbovirus - west Nile or St Louis encephalitis

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2
Q

What diagnosis should you consider in a teenager who is unimmunized and presents with parotitis and neurological symptoms ?

A

Mumps encephalitis

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3
Q

What medication should you give in a febrile toddler who presents with a fever and lethargy for one hour post seizure?

A

Acyclovir and antibiotics

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4
Q

What is the treatment for a teenager with frontal headache which is “band like” and “pressure”?

A

Eliminating the environmental stressors

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5
Q

What is the first line treatment for migraine headache?

A

Ibuprofen
Tylenol
Fluids
Rest

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6
Q

What is the appropriate next step in a patient with several months of frontotemporal headache unrelieved wth daily Tylenol and Motrin ?

A

Stop medication as effect is blunted by chronic use

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7
Q

What diagnosis should you consider in a patient with headache and progressive and intermittent visual disturbances ?

A

Increased ICP

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8
Q

How can you differentiate migraines from increased ICP in a patient with headache and visual disturbances?

A

Migraines present with blurry vision only before the headache
Progressive visual loss is consistent with increased ICP

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9
Q

If a patient presents with abrupt onset of altered mental status what are the two most likely causes?

A

Trauma or CVA

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10
Q

If a patient presents with altered mental status that occurred over several hours, what is the more likely diagnosis?

A

Toxic ingestion

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11
Q

What is the probable diagnosis in a patient with hypoglycemia that is resistant to glucose replacement ?

A

Ingestion of hypoglycemic medication

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12
Q

If you are presented with a non responsive neonate, what labs should you order (other than to rule out sepsis)?

A

Ammonia

Organic acid levels

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13
Q

What diagnosis should you consider if you see greenish-yellow rings at the edge of the cornea on slit lamp exam?

A

Wilson’s disease

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14
Q

What are 4 abnormal lab results that help in diagnosis of Wilson’s disease?

A

Low ceruloplasmin
Low serum copper
Increased urinary copper
Hemolytic anemia

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15
Q

How would you treat Wilson disease ?

A

Penicillamine and low copper diet

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16
Q

What diagnosis presents as a neonate with hydrocephalus and hx of heart failure who has a cranial bruit on physical exam?

A

Vein of Galen malformation

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17
Q

What diagnosis should you consider in a patient with tremors, depression and abnormal eye movements as well as acute liver failure ?

A

Wilson’s disease

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18
Q

What are the 5 major causes of acute ataxia?

A 
Toxic ingestion
Infection
Metabolic problems
Trauma
Neoplasm
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19
Q

What types of malignancy are patients with ataxia telangiectasia at risk for?

A

Hodgkin lymphoma and leukemia

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20
Q

What diagnosis should you consider in a patient with abnormal gait and abnormal pigmentation of the eyes?

A

Ataxia telangiectasia

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21
Q

Why do children with ataxia telangiectasia present with frequent upper and lower respiratory infections ?

A

Decreased levels of immunoglobulin and T cell dysfunction

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22
Q

What disease presents in late childhood with slow/clumsy gait as well as elevated plantar arch and absence of DTR in lower extremities?

A

Friedreich ataxia

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23
Q

Why do children with friedreich ataxia present with abnormal gait?

A

Cerebellar problem and loss of proprioception

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24
Q

What 2 (non neurological) problems are common in children with friedreich ataxia?

A

Diabetes

Cardiomyopathy leading to CHF

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25
Q

What is the treatment for friedreich ataxia?

A

Supportive

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26
Q

What diagnosis should you consider in a patient with quick random jerky movements and poor tone who has difficulty paying attention in school and emotional lability?

A

Sydenham chorea

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27
Q

What lab test effectively rules out Sydenham chorea?

A

None! A negative ASO does not rule out Sydenham chorea!!!

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28
Q

What is the treatment for Sydenham chorea?

A 
Supportive
Antipsychotics (dopamine blocking agents)
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29
Q

What is inheritance pattern of Huntington chorea?

A

Autosomal dominant

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30
Q

What is the classic triad of Huntington chorea?

A

Chorea
Hypotonia
Emotional lability (dementia)

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31
Q

What type of medication reaction should you consider in an alert patient with fixed upward gaze? How would you treat?

A

Dystonic reaction - treat with benadryl

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32
Q

What diagnosis and treatment would you provide to an ADHD child with brief episodes of eye blinking?

A

Simple motor tic - no intervention needed

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33
Q

What are choreiform movements?

A

Repetitive jerking movements that cannot be suppressed

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34
Q

What constitutes that diagnosis of Tourette syndrome?

A

Tic disorder present for at least one year

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35
Q

What are two things that were previously thought to cause tics but are now known not to be a cause of tics ?

A

Anxiety

Stimulant medications

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36
Q

What are 5 main classes of medications that cause tremors?

A 
Methylxanthines (caffeine)
Amphetamines
Valproic acid 
Phenothiazine
TCAs
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37
Q

What are the main symptoms associated with posterior fossa tumors?

A

Afebrile child with ataxia and headache

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38
Q

What diagnosis should you consider in a patient with growth delay, headaches and chronic progressive visual field deficits?

A

Craniopharyngioma (supratentorial tumor)

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39
Q

What Brain CT finding is seen in craniopharyngioma?

A

Calcification in sella turcica

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40
Q

What diagnosis should you consider in a patient with double vision, headache and papilledema?

A

Pseudotumor cerebri

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41
Q

What 5 types of medications can cause pseudotumor cerebri?

A 
Vitamin A (megadoses)
Steroids 
Thyroxine
Lithium
Antibiotics
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42
Q

What are 3 possible treatment options for pseudotumor cerebri?

A

Carbonic anhydrase inhibitor (acetazolamide)
Steroids
Shunt surgery

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43
Q

What should you be concerned about in a patient with HTN, bradycardia and abducens paresis?

A

Increased intracranial pressure

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44
Q

What are 3 contraindications to performing LP?

A

Focal neurological signs
Coagulapathy
Cardiorespiratory instability

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45
Q

What are 5 causes of acute peripheral nerve disorder with loss of deep tendon reflexes ?

A 
Guillain barre
Polio
Diphtheria
Tick paralysis
Lead poisoning
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46
Q

What cause progressive onset of neuromuscular junction disorder? what causes a rapid onset NMJ disorder?

A 
Myasthenia gravis (progressive)
Botulism (rapid, descending)
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47
Q

What are 3 causes of chronic peripheral nerve disease and loss of deep tendon reflexes?

A

Chronic demyelinating poly neuropathy
Hereditary neuropathy
Leukodystrophy

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48
Q

What are 5 major causes of spinal cord weakness with loss of motor/sensation as well as loss of bladder/bowel function and increased reflexes?

A 
Transverse myelitis
Anterior spinal artery infarction
Spinal cord compression
Epidural abscess
Tethered cord
49
Q

What are 3 causes of acute proximal muscle weakness ?

A

Polymyositis
Dermatomyositis
Electrolyte imbalance

50
Q

What are 3 causes of chronic proximal muscular weakness?

A

Muscular dystrophy
Mitochondrial myopathy
Congenital myopathy

51
Q

In patients with Guillain Barre Syndrome, what needs to be closely followed after diagnosis?

A

Lung function - PFTs or vital capacity (oxygen saturation is inadequate as it only declines once patient is severely compromised)

52
Q

What diagnosis presents in a child with diarrhea who is having difficulty standing up from sitting and exam shows areflexia?

A

Guillain barre syndrome

53
Q

What are common findings on physical exam in a patient with Guillain barre syndrome ?

A

Ascending paralysis / unsteady gait
Areflexia
Cranial nerve defects
Dysautonomia (hypotension, tachycardia)

54
Q

What may be seen in lumbar puncture results in a patient With Guillain barre syndrome ?

A

Increased protein but normal cell count

55
Q

What are 2 treatment options with Guillain barre syndrome ?

A

Plasmapheresis & IVIG (steroids have no proven benefit)

56
Q

What medical condition is associated with myasthenia gravis and should be evaluated for once the diagnosis of myasthenia has been made?

A

Thymoma

57
Q

How is myasthenia gravis diagnosed?

A

Tensilon test - edrophonium briefly inhibits acetylcholinesterase

58
Q

What diagnosis should you consider in a patient who presents with droopy eyes and fatigue that is worse as the day progresses ?

A

Myasthenia gravis

59
Q

What is the pathogenesis of myasthenia gravis ?

A

Autoimmune antibodies against acetylcholine receptor at neuromuscular junction

60
Q

What are 4 treatment options for myasthenia gravis ?

A

Pyridostigmine (inhibits acetylcholinesterase)
Plasmaphesis
Prednisone
Thymectomy

61
Q

What is transient myasthenia gravis? How long does it last and how can you differentiate it from congenital myasthenia gravis?

A

Due to mothers antibodies - resolves in 6 weeks and does not involve the eyes

62
Q

What is the cause of congenital myasthenia gravis?

A

Genetic defect of the neuromuscular junction and is lifelong disease

63
Q

What diagnosis should you consider in an infant with a weak cry, constipation and acute onset of hypotonia that is descending ?

A

Botulism

64
Q

What is the pathogenesis of botulism?

A

Toxin blocks release of Acetylcholine from the neuromuscular junction

65
Q

What diagnosis should you consider in a patient with malar rash and proximal muscle weakness? What lab finding would be elevated?

A

Dermatomyositis - elevated CPK

66
Q

What diagnosis should you consider in a child with poor head control and large calf muscles ?

A

Duchenne muscular dystrophy

67
Q

What are the methods of diagnosis of duchenne muscular dystrophy ?

A

Muscle biopsy

Genetic testing

68
Q

What lab finding is abnormal in children with duchenne muscular dystrophy ?

A

Elevated CPK (even before symptoms)

69
Q

What is the cause of calf hypertrophy in patients with duchenne muscular dystrophy?

A

Deposition and proliferation of collagen

70
Q

Other than muscle weakness, what are 3 other medical problems in patients with duchenne muscular dystrophy ?

A

Mild intellectual disability
Cardiomyopathy
Respiratory failure

71
Q

What is the inheritance pattern of duchenne muscular dystrophy? Could a child develop this disease in a mother who is not a carrier of the gene mutation?

A

X linked recessive (only found in males)

Yes - there is 1/3 chance of spontaneous mutations!!

72
Q

What is the inheritance of myotonic muscular dystrophy ?

A

Autosomal dominant

73
Q

What disease presents with distal muscle wasting and slow muscle relaxation of the hand as well as endocrine problems?

A

Myotonic muscular dystrophy (aka. Steinert disease)

74
Q

What types of muscles are affected in patients with myotonic muscular dystrophy ?

A

Striated and smooth muscles (includes then heart and GI tract)

75
Q

What is myotonia?

A

Slow muscle relaxation after contraction

76
Q

What is the emperor antibiotic regimen for a patient with brain abscess?

A

Vancomycin, metronidazole and ceftriaxone

77
Q

What diagnosis should you consider in a patient with back pain, decreased anal tone and reduced sensation in the legs as well as fever?

A

Epidural abscess

78
Q

What is the treatment for epidural abscess?

A

Antibiotic with antistaphylococcal coverage and emergency spinal decompression

79
Q

What diagnosis presents after febrile illness with abrupt onset of weakness and hypotonia followed by increased tone and hyperreflexia ?

A

Acute transverse myelitis

80
Q

What is the pathogenic cause of acute transverse myelitis?

A

Lymphocytic infiltration and demyelination of nerves secondary to inflammation

81
Q

What CSF findings are found in patients with acute transverse myelitis? What should be performed prior to LP in these patients and what will those results be?

A

Csf with increased polys but negative gram stain

MRI with gadolinium shows cord swelling

82
Q

What is the most immediate treatment for extrinsic spinal cord mass lesion?

A

High dose IV dexamethasone to reduce risk for ischemia

83
Q

What diagnosis presents in infancy with hypotonia, poor suck and tongue fasciculations?

A

Spinal muscle atrophy type 1 (werdnig Hoffmann disease)

84
Q

What is the pathophysiology of spinal muscle atrophy ?

A

Degeneration of anterior horn cells (only affects motor function with no sensory deficits)

85
Q

What are two important evaluations that should be performed on every newborn with spina bifida?

A

Orthopedic and urological evaluation

86
Q

What mental/learning issues might a patient with spina bifida have later in life?

A

Difficulty with organization, memory or other learning skills.
iQ low-average

87
Q

What might you suspect in a patient with leg length discrepancy, elevated arches and gait abnormalities as well as lipoma to the lower back?

A

Occult spinal dysraphism

88
Q

How would you treat a patient with focal spine pain after a traumatic event?

A

Immobilization and methylprednisolone 30mg/kg over an hour

89
Q

What type of seizure presents with localized movement while the patient remains awake?

A

Simple partial seizure

90
Q

What type of seizure presents with localized movement disorder and loss of consciousness?

A

Complex partial seizure

91
Q

What type of seizure presents as brief staring episodes?

A

Absence seizure

92
Q

What are 5 causes of metabolic related seizures?

A 
Pyridoxine deficiency
Hypoglycemia
Hyponatremia
Hypocalcemia
Urea cycle disorders
93
Q

What should you do in a patient with history of seizures who takes phenobarbital but presents with breakthrough seizure?

A

Phenobarbital level

94
Q

What is the term that describes motor weakness after a seizure?

A

Todds paralysis

95
Q

What are the 3 components that make a febrile seizure complex?

A

Last >15 min
>1 episode in 24 hour period
Focal signs

96
Q

What is the only blood test indicated after a new onset seizure?

A

Glucose

97
Q

What are 1st and 2nd line meds for treatment of status epilepticus?

A

Ativan then fosphenytoin (phenytoin causes skin necrosis)

98
Q

Under what condition should a child get a lumbar puncture after a febrile seizure?

A

In a child <18months of age

99
Q

What is the risk of epilepsy in a patient who has had a febrile seizure compared to the risk in the general population?

A

0.5% in general population

1% chance in child with febrile seizure

100
Q

What is the diagnosis in a patient with a seizure and EEG findings showing 3 per second spike and wave ?

A

Absence seizure

101
Q

What medication is specific for treatment of absence seizures?

A

Ethosuximide

102
Q

What can induce an absence seizure and can be used in diagnosis?

A

Hyperventilation

103
Q

What is the drug of choice for generalized tonic clonic seizures ?

A

Valproic acid

104
Q

What are the 3 components associated with infantile spasms (aka West Syndrome)?

A

Infantile spasm
Hypsarrhythmia on EEG
Developmental delay

105
Q

What type of seizure presents with loss of muscle tone and sudden brief shock-like muscle twitches with the child “falling” forward but no impairment of consciousness ?

A

Myoclonic seizures

106
Q

What diagnosis should you consider in a 4 month old patient described as having repetitive flexion of head, trunk and extremities?

A

Infantile spasms

107
Q

What genetic syndrome is associated with infantile spasms? What is this conditions inheritance pattern?

A

Tuberous sclerosis

Autosomal dominant

108
Q

What is the first line treatment for infantile spasms ? What are 3 other treatment options?

A

ACTH (first line)
Steroids
Anti-epileptics
Focal resection

109
Q

What type of seizure presents with lip snaking and moaning followed by post ictal state?

A

Complex partial seizure

110
Q

What is the drug of choice in a patient with abnormal movement of one extremity and normal consciousness?

A

Carbamazepine

111
Q

What diagnosis should you consider in a child with facial motor seizures with unilateral sensory involvement that occurs while asleep? What is the treatment of choice?

A

Benign rolandic seizures - carbamazepine

112
Q

When can withdrawal from seizure medications be attempted? In what condition should withdrawal not be attempted?

A

If seizure free 2 years

Unless dx of juvenile myoclonic epilepsy on valproic acid

113
Q

What diagnosis presents with difficulty concentrating and clumsiness with adduction of the contralateral leg with stimulation of the patellar reflex?

A

Adrenleukodystrophy

114
Q

What is the most common risk factor for cerebral palsy in premature newborns?

A

Perinatal infection

115
Q

What type of cerebral palsy presents with bilateral spasticity of legs, and toe walking

A

Spastic dysplegia

116
Q

What type of CP presents with spasticity of either the right or left side of the body ?

A

Spastic hemiplegia

117
Q

What type of CP presents with increased tone in all 4 extremities plus dystonia and strange movements?

A

Dyskinetic or athetoid cerebral palsy

118
Q

What diagnostic study would be appropriate for a child with acute unilateral hemiparesis after blunt trauma to the chest?

A

Carotid angiography

119
Q

What type of disorder would you consider in a patient with stroke, elevated lactate level and sensorineural deafness?

A

Mitochondrial disorder

120
Q

If you are presented with a patient with CVA confirmed with CT, what would be the next step to identify the diagnosis?

A

Cerebral angiography

pediatric Boards (23 decks)

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