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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
What is Wilson's Disease?
An autosomal recessive disorder in copper transport due to abnormality on 13q14-q21
26
What are the symptoms of Wilson's Disease?
1. Dysarthria 2. Dystonia (risus sardonicus) 3. Rigidity 4. Gait disturbance 5. Tremor
27
What is spasticity?
Velocity dependent tone- felt when patient is at rest
28
What is dystonia?
Felt at action, patient may not be able to be "at rest"
29
How are the affected muscles in dystonia and spasticity?
Weak
30
What is the treatment for dystonia and spasticity?
1. Muscle Relaxants (Baclofen + Tizanidine) 2. Anticholinergics (Trihexyphenidyl) 3. Benzos (Diazepam)
31
What are surgical treatment options for dystonia?
1. Intrathecal baclofen pump | 2. DBS
32
What are common causes of drug-induced movement disorders?
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
What are examples of acute dystonic reactions?
1. Oculogyric Crisis 2. Torticollis 3. Axial Dystonia
34
What is the treatment for Acute Dystonic Reactions?
IV Benadryl or Cogentin
35
What is the treatment for Stereotypies?
SSRIs
36
If no comorbidities are present, what is the treatment for a tic?
Alpha 2 Agonists
37
What are PANDAS?
Pediatric Autoimmune Neurophyschiatric Disorders Associated with Streptococcal infections
38
What is the autoimmune mechanism for PANDAS?
Group A Strep --> tics + OCD
39
What features are associated with PANDAS?
1. Prepubertal onset 2. Episodic course 3. Temporal relationship with GABHS and tic/OCD exacerbation
40
How are PANDAS treated?
1. Use standard Tic medication for exacerbations | 2. ABX prophylaxis after ARF
41
What are the acute causes of ataxia?
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
What are the paroxysmal causes of ataxia?
1. Metabolic 2. Hereditary 3. Migraine 4. Benign Paroxysmal Vertigo
43
What are the chronic causes of ataxia?
1. Congenital 2. Brain Tumor 3. MS 4. Friedrich's Ataxia 5. Ataxia-Telangiectasia
44
What are the 3 metabolic causes of paroxysmal ataxia?
1. Hartnup Disease 2. Maple Syrup Urine Disease 3. Pyruvate Dehydrogenase Deficiency
45
What is Hartnup Disease?
AR disease due to chromosome 5p15 that results in deficient amino acid transport in the kidney
46
What are the symptoms of Hartnup Disease?
1. Developmental Delay 2. Pellagra-like disease 3. Limb ataxia
47
What is the treatment for Hartnup Disease?
High protein diet
48
What is Maple Syrup Disease?
AR Disorder of branched chain amino acid metabolism
49
What are the clinical features of Maple Syrup Urine Disease?
1. Urine with syrup odor | 2. Stress, infection, ataxia, lethargy and irritability
50
What is the treatment for MSUD?
Protein restriction with Thiamine supplements
51
What is Pyruvate Dehydrogenase Deficiency?
Defect in the E1 component of pyruvate dehydrogenase complex
52
What are the symptoms of Pyruvate Dehydrogenase deficiency?
1. Increased lactate and pyruvate | 2. Attacks of ataxia and lethargy
53
What is the treatment for pyruvate dehydrogenase deficiency?
1. Daily acetazolamide (helps reduce attacks) | 2. Ketogenic diet
54
Which channelopathies cause paroxysmal ataxia?
1. Episodic Ataxia Type 1 | 2. Episodic Ataxia Type 2
55
What is the mutation, clinical presentation and treatment for Episodic Ataxia Type 1?
1. KCNA1 2. Brief attacks of ataxia 3. Phenytoin, carbamazepine and acetazolamide
56
What is the mutation, clinical presentation and treatment for Episodic Ataxia Type 2?
1. CACNA1A 2. Attacks of ataxia lasting days 3. Acetaolamide and Flunarizine
57
Where is the lesion causing truncal ataxia?
Vermis
58
Where is the lesion causing limb ataxia?
Hemispheres
59
Where is the lesion causing eye movements?
Brainstem
60
Which causes of ataxia have increased protein in CSF?
MS and Miller Fisher
61
Describe peripheral vertigo
Episodic, unidirectional, possible hearing loss but no other neuro sx
62
Describe central vertigo
Constant, variable direction, no hearing loss, cranial nerve and cerebellar deficits

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