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NEURO CLERKSHIP > Pediatric Neurology > Flashcards

Pediatric Neurology Flashcards

1
Q

What are the 5 types of movement disorders?

A
  1. Chorea
  2. Athetosis
  3. Dystonia
  4. Dyskinesia
  5. Tics (motor and vocal)
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2
Q

What are the types of ataxia?

A

Ocular, Truncal, Appendicular and Gait

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3
Q

What are possible causes of pediatric movement disorders?

A
  1. Infectious
  2. Lesional (vascular, demyelinating, traumatic or mass)
  3. Toxin
  4. Metabolic
  5. Hereditary
  6. Neurochemical (dopamine)
  7. Seizures
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4
Q

What are the physical exam findings of chorea and athetosis?

A
  1. Frequent, brief and unpredictable movements
  2. Flow from one body part to another
  3. Chaotic
  4. “Piano playing”, “Milkmaid’s grip” and “Chameleon tongue”
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5
Q

What diseases may present with chorea or athetosis?

A
  1. Sydenham’s Chorea
  2. Huntingtons
  3. Cerebral Palsy
  4. Kernicterus
  5. Stimulant Meds
  6. “Post-Pump Chorea”
  7. Chorea Gravidarum
  8. Infection
  9. Degenerative Disease
  10. Metabolic
  11. ADEM
  12. SLE
  13. Hyperthyroidism
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6
Q

With what condition is Sydenham’s Chorea associated?

A

Acute Rheumatic Fever

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7
Q

Which gender is more commonly affected by Sydenham’s Chorea?

A

Female

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8
Q

Describe the onset of Sydenhams Chorea

A

Gradual onset weeks after a GBS infection with progressive emotional lability and choreiform movements

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9
Q

Is Sydenhams Chorea symmetric?

A

No- unilateral

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10
Q

What lab test can confirm Sydenhams Chorea?

A

Antistreptolysin O or antiDNAse B antibodies

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11
Q

What other condition may be associated with SC?

A

Carditis

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12
Q

Describe the prognosis of SC?

A

Waxing and waning course for months, usually resolves

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13
Q

What medications may be used to treat SC?

A
  1. Pimozide (Dopamine antagonist)
  2. Carbamazepine
  3. Valproate
  4. Benzodiazepines
  5. Penicillin
  6. Steroids or IVIg
  7. Haloperidol
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14
Q

What medications worsen chorea?

A
  1. Stimulants
  2. Cocaine
  3. Lithium
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15
Q

What is dystonia?

A

Contraction of agonist and antagonist muscle group

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16
Q

What are the primary causes of dystonia?

A
  1. Hereditary

2. Metabolic

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17
Q

What are the secondary causes of dystonia?

A
  1. Cerebral Palsy
  2. Post-Traumatic
  3. After a Stroke
  4. Toxin
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18
Q

What are the types of dystonia?

A
  1. Focal
  2. Segmental
  3. Multifocal
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19
Q

What are features of dystonia?

A
  1. Worsen w/ stress or excitement
  2. Fluctuate over time
  3. Triggered by certain movements
  4. NOT present during sleep
  5. May be suppressed by touch of a certain body part (geste antagoniste)
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20
Q

What are the features of dopa-responsive dystonia?

A
  1. Common
  2. Responds to levodopa
  3. Begins with foot dystonia
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21
Q

What are the features of Idiopathic Generalized Torsion Dystonia?

A
  1. Autosomal Dominant DYT1 Mutation
  2. Begins in lower limb
  3. Generalizes within 5 years
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22
Q

What is Glutaric Acidemia Type 1?

A

Autosomal Recessive error in the metabolism of lysine, hydroxylysine and tryptophan due to deficiency in glutaryl-Coenzyme A Dehydrogenase

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23
Q

What are the symptoms of Glutaric Acidemia Type 1?

A
  1. Macrocephaly

2. Chorea

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24
Q

What is the treatment for Glutaric Acidemia Type I?

A

Carnitine supplementation and supportive care during febrile illness

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25
Q

What is Wilson’s Disease?

A

An autosomal recessive disorder in copper transport due to abnormality on 13q14-q21

26
Q

What are the symptoms of Wilson’s Disease?

A
  1. Dysarthria
  2. Dystonia (risus sardonicus)
  3. Rigidity
  4. Gait disturbance
  5. Tremor
27
Q

What is spasticity?

A

Velocity dependent tone- felt when patient is at rest

28
Q

What is dystonia?

A

Felt at action, patient may not be able to be “at rest”

29
Q

How are the affected muscles in dystonia and spasticity?

A

Weak

30
Q

What is the treatment for dystonia and spasticity?

A
  1. Muscle Relaxants (Baclofen + Tizanidine)
  2. Anticholinergics (Trihexyphenidyl)
  3. Benzos (Diazepam)
31
Q

What are surgical treatment options for dystonia?

A
  1. Intrathecal baclofen pump

2. DBS

32
Q

What are common causes of drug-induced movement disorders?

A
  1. Dopamine antagonists (antipsychotics and anti nausea)
    • -> acute dystonic rxn, TD, PD and NMS
  2. Amphetamines and Stimulants
    • -> chorea and tremor
  3. Cocaine –> Chorea
  4. Lithium –> Chorea and tremor
33
Q

What are examples of acute dystonic reactions?

A
  1. Oculogyric Crisis
  2. Torticollis
  3. Axial Dystonia
34
Q

What is the treatment for Acute Dystonic Reactions?

A

IV Benadryl or Cogentin

35
Q

What is the treatment for Stereotypies?

A

SSRIs

36
Q

If no comorbidities are present, what is the treatment for a tic?

A

Alpha 2 Agonists

37
Q

What are PANDAS?

A

Pediatric Autoimmune Neurophyschiatric Disorders Associated with Streptococcal infections

38
Q

What is the autoimmune mechanism for PANDAS?

A

Group A Strep –> tics + OCD

39
Q

What features are associated with PANDAS?

A
  1. Prepubertal onset
  2. Episodic course
  3. Temporal relationship with GABHS and tic/OCD exacerbation
40
Q

How are PANDAS treated?

A
  1. Use standard Tic medication for exacerbations

2. ABX prophylaxis after ARF

41
Q

What are the acute causes of ataxia?

A
  1. Cerebellitis
  2. Neuroblastoma
  3. Brain Tumor
  4. Ingestion
  5. Miller Fischer Syndrome
  6. MS
  7. Post-Concussive Syndrome
  8. Vertebrobasilar Hemorrhage
  9. Kawasaki Disease
42
Q

What are the paroxysmal causes of ataxia?

A
  1. Metabolic
  2. Hereditary
  3. Migraine
  4. Benign Paroxysmal Vertigo
43
Q

What are the chronic causes of ataxia?

A
  1. Congenital
  2. Brain Tumor
  3. MS
  4. Friedrich’s Ataxia
  5. Ataxia-Telangiectasia
44
Q

What are the 3 metabolic causes of paroxysmal ataxia?

A
  1. Hartnup Disease
  2. Maple Syrup Urine Disease
  3. Pyruvate Dehydrogenase Deficiency
45
Q

What is Hartnup Disease?

A

AR disease due to chromosome 5p15 that results in deficient amino acid transport in the kidney

46
Q

What are the symptoms of Hartnup Disease?

A
  1. Developmental Delay
  2. Pellagra-like disease
  3. Limb ataxia
47
Q

What is the treatment for Hartnup Disease?

A

High protein diet

48
Q

What is Maple Syrup Disease?

A

AR Disorder of branched chain amino acid metabolism

49
Q

What are the clinical features of Maple Syrup Urine Disease?

A
  1. Urine with syrup odor

2. Stress, infection, ataxia, lethargy and irritability

50
Q

What is the treatment for MSUD?

A

Protein restriction with Thiamine supplements

51
Q

What is Pyruvate Dehydrogenase Deficiency?

A

Defect in the E1 component of pyruvate dehydrogenase complex

52
Q

What are the symptoms of Pyruvate Dehydrogenase deficiency?

A
  1. Increased lactate and pyruvate

2. Attacks of ataxia and lethargy

53
Q

What is the treatment for pyruvate dehydrogenase deficiency?

A
  1. Daily acetazolamide (helps reduce attacks)

2. Ketogenic diet

54
Q

Which channelopathies cause paroxysmal ataxia?

A
  1. Episodic Ataxia Type 1

2. Episodic Ataxia Type 2

55
Q

What is the mutation, clinical presentation and treatment for Episodic Ataxia Type 1?

A
  1. KCNA1
  2. Brief attacks of ataxia
  3. Phenytoin, carbamazepine and acetazolamide
56
Q

What is the mutation, clinical presentation and treatment for Episodic Ataxia Type 2?

A
  1. CACNA1A
  2. Attacks of ataxia lasting days
  3. Acetaolamide and Flunarizine
57
Q

Where is the lesion causing truncal ataxia?

A

Vermis

58
Q

Where is the lesion causing limb ataxia?

A

Hemispheres

59
Q

Where is the lesion causing eye movements?

A

Brainstem

60
Q

Which causes of ataxia have increased protein in CSF?

A

MS and Miller Fisher

61
Q

Describe peripheral vertigo

A

Episodic, unidirectional, possible hearing loss but no other neuro sx

62
Q

Describe central vertigo

A

Constant, variable direction, no hearing loss, cranial nerve and cerebellar deficits

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