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US Pediatric Boards Edited > neurology > Flashcards

Flashcards in neurology Deck (121):
1

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

Enterovirus encephalitis

2

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

Mumps encephalitis

3

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

Acyclovir and antibiotics

4

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

Eliminating the environmental stressors

5

What is the first line treatment for migraine headache?

Ibuprofen
Tylenol
Fluids
Rest

6

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

Stop medication as effect is blunted by chronic use

7

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

Increased ICP

8

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

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

9

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

Trauma or CVA

10

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

Toxic ingestion

11

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

Ingestion of hypoglycemic medication

12

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

Ammonia
Organic acid levels

13

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

Wilson's disease

14

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

Low ceruloplasmin
Low serum copper
Increased urinary copper
Hemolytic anemia

15

How would you treat Wilson disease ?

Penicillamine and low copper diet

16

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

Vein of Galen malformation

17

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

Wilson's disease

18

What are the 5 major causes of acute ataxia?

Toxic ingestion
Infection
Metabolic problems
Trauma
Neoplasm

19

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

Hodgkin lymphoma and leukemia

20

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

Ataxia telangiectasia

21

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

Decreased levels of immunoglobulin and T cell dysfunction

22

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

Friedreich ataxia

23

Why do children with friedreich ataxia present with abnormal gait?

Cerebellar problem and loss of proprioception

24

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

Diabetes
Cardiomyopathy leading to CHF

25

What is the treatment for friedreich ataxia?

Supportive

26

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?

Sydenham chorea

27

What lab test effectively rules out Sydenham chorea?

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

28

What is the treatment for Sydenham chorea?

Supportive
Antipsychotics (dopamine blocking agents)

29

What is inheritance pattern of Huntington chorea?

Autosomal dominant

30

What is the classic triad of Huntington chorea?

Chorea
Hypotonia
Emotional lability (dementia)

31

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

Dystonic reaction - treat with benadryl

32

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

Simple motor tic - no intervention needed

33

What are choreiform movements?

Repetitive jerking movements that cannot be suppressed

34

What constitutes that diagnosis of Tourette syndrome?

Tic disorder present for at least one year

35

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

Anxiety
Stimulant medications

36

What are 5 main classes of medications that cause tremors?

Methylxanthines (caffeine)
Amphetamines
Valproic acid
Phenothiazine
TCAs

37

What are the main symptoms associated with posterior fossa tumors?

Afebrile child with ataxia and headache

38

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

Craniopharyngioma (supratentorial tumor)

39

What Brain CT finding is seen in craniopharyngioma?

Calcification in sella turcica

40

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

Pseudotumor cerebri

41

What 5 types of medications can cause pseudotumor cerebri?

Vitamin A (megadoses)
Steroids
Thyroxine
Lithium
Antibiotics

42

What are 3 possible treatment options for pseudotumor cerebri?

Carbonic anhydrase inhibitor (acetazolamide)
Steroids
Shunt surgery

43

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

Increased intracranial pressure

44

What are 3 contraindications to performing LP?

Focal neurological signs
Coagulapathy
Cardiorespiratory instability

45

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

Guillain barre
Polio
Diphtheria
Tick paralysis
Lead poisoning

46

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

Myasthenia gravis (progressive)
Botulism (rapid, descending)

47

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

Chronic demyelinating poly neuropathy
Hereditary neuropathy
Leukodystrophy

48

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?

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

49

What are 3 causes of acute proximal muscle weakness ?

Polymyositis
Dermatomyositis
Electrolyte imbalance

50

What are 3 causes of chronic proximal muscular weakness?

Muscular dystrophy
Mitochondrial myopathy
Congenital myopathy

51

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

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

52

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

Guillain barre syndrome

53

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

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

54

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

Increased protein but normal cell count

55

What are 2 treatment options with Guillain barre syndrome ?

Plasmapheresis & IVIG (steroids have no proven benefit)

56

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

Thymoma

57

How is myasthenia gravis diagnosed?

Tensilon test - edrophonium briefly inhibits acetylcholinesterase

58

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

Myasthenia gravis

59

What is the pathogenesis of myasthenia gravis ?

Autoimmune antibodies against acetylcholine receptor at neuromuscular junction

60

What are 4 treatment options for myasthenia gravis ?

Pyridostigmine (inhibits acetylcholinesterase)
Plasmaphesis
Prednisone
Thymectomy

61

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

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

62

What is the cause of congenital myasthenia gravis?

Genetic defect of the neuromuscular junction and is lifelong disease

63

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

Botulism

64

What is the pathogenesis of botulism?

Toxin blocks release of Acetylcholine from the neuromuscular junction

65

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

Dermatomyositis - elevated CPK

66

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

Duchenne muscular dystrophy

67

What are the methods of diagnosis of duchenne muscular dystrophy ?

Muscle biopsy
Genetic testing

68

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

Elevated CPK (even before symptoms)

69

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

Deposition and proliferation of collagen

70

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

Mild intellectual disability
Cardiomyopathy
Respiratory failure

71

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?

X linked recessive (only found in males)
Yes - there is 1/3 chance of spontaneous mutations!!

72

What is the inheritance of myotonic muscular dystrophy ?

Autosomal dominant

73

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

Myotonic muscular dystrophy (aka. Steinert disease)

74

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

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

75

What is myotonia?

Slow muscle relaxation after contraction

76

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

Vancomycin, metronidazole and ceftriaxone

77

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

Epidural abscess

78

What is the treatment for epidural abscess?

Antibiotic with antistaphylococcal coverage and emergency spinal decompression

79

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

Acute transverse myelitis

80

What is the pathogenic cause of acute transverse myelitis?

Lymphocytic infiltration and demyelination of nerves secondary to inflammation

81

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?

Csf with increased polys but negative gram stain
MRI with gadolinium shows cord swelling

82

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

High dose IV dexamethasone to reduce risk for ischemia

83

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

Spinal muscle atrophy type 1 (werdnig Hoffmann disease)

84

What is the pathophysiology of spinal muscle atrophy ?

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

85

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

Orthopedic and urological evaluation

86

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

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

87

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

Occult spinal dysraphism

88

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

Immobilization and methylprednisolone 30mg/kg over an hour

89

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

Simple partial seizure

90

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

Complex partial seizure

91

What type of seizure presents as brief staring episodes?

Absence seizure

92

What are 5 causes of metabolic related seizures?

Pyridoxine deficiency
Hypoglycemia
Hyponatremia
Hypocalcemia
Urea cycle disorders

93

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

Phenobarbital level

94

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

Todds paralysis

95

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

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

96

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

Glucose

97

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

Ativan then fosphenytoin (phenytoin causes skin necrosis)

98

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

In a child <18months of age

99

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

0.5% in general population
1% chance in child with febrile seizure

100

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

Absence seizure

101

What medication is specific for treatment of absence seizures?

Ethosuximide

102

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

Hyperventilation

103

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

Valproic acid

104

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

Infantile spasm
Hypsarrhythmia on EEG
Developmental delay

105

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 ?

Myoclonic seizures

106

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

Infantile spasms

107

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

Tuberous sclerosis
Autosomal dominant

108

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

ACTH (first line)
Steroids
Anti-epileptics
Focal resection

109

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

Complex partial seizure

110

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

Carbamazepine

111

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?

Benign rolandic seizures - carbamazepine

112

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

If seizure free 2 years
Unless dx of juvenile myoclonic epilepsy on valproic acid

113

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

Adrenleukodystrophy

114

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

Perinatal infection

115

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

Spastic dysplegia

116

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

Spastic hemiplegia

117

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

Dyskinetic or athetoid cerebral palsy

118

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

Carotid angiography

119

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

Mitochondrial disorder

120

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

Cerebral angiography

121

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

Arbovirus - west Nile or St Louis encephalitis