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Neuro Clerkship > Pediatric Movement Disorders > Flashcards

Pediatric Movement Disorders Flashcards

1
Q

Abnormal paroxysmal movements should cause you to consider what problem?

What 4 things would lead you to think this as opposed to run of the mill movement disorder?

A

Consider Seizures

    • Should see LOC
    • EEG changes
    • Possible worsening with sleep
    • Less frequent than run of the mill movement disorder
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2
Q

What are the 5 types of childhood movement disorders?

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

What is Athetosis?

With what disease is this abnormal movement associated?

What two areas of the body are typically affected?

A

Abnormal muscle contractions that cause writing movements; frequent, brief, unpredictable–flow from one body part to another

Typically affect patients with CP

Will cause abnormal face and hand movements

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4
Q

Define opsiclonus?

A

“dancing eyes” –irregular bouncing movement of the eyelids

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5
Q

What are the four types of ataxia?

A
  1. Ocular
  2. Truncal
  3. Appendicular
  4. Gait
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6
Q

What is the triad for paraneoplastic syndrome associated with neuroblastoma?

A
  1. Opsiclonus
  2. Ataxia
  3. Myoclonus

**Kids are often irritable or encephalopathic

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7
Q

How do we diagnose neuroblastoma? (3)

A
  1. Nucleotide scan
  2. Urinalysis (VMA, HVA)
  3. CT scan** (most important)
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8
Q

How do we treat neuroblastoma? (3)

A
  1. IVIG
  2. ACTH
  3. Tumor removal if one is found
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9
Q

What are the common clinical findings for Familial Episodic Cerebellar Ataxia (2)?

How many variants of this disease exist?
What is the etiology of this disease?

A
  • Dysmmetria
  • Dystaticokinsia

There are two types of familial episodic cerebellar ataxia; they are channelopathies

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10
Q

What is one common finding in family hx of patients with familial episodic cerebellar ataxia?

A

Hemiplegic migraines

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11
Q

How do we treat familial episodic cerebellar ataxia?

A

Acetazolamide; has similar ADR profile to topirimate–causes metallic taste to soda

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12
Q

When a patient presents with unexplained chorea or athetosis, what are 4 important things to ask?

A
  • Birth hx
  • Congenital disease (heart esp.)
  • Recent infection
  • Stimulant med use
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13
Q

What are three PE tests to assess chorea or athetosis?

A
  • Piano playing (fingers in hand)
  • Milkmaid grip
  • Chameleon tongue
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14
Q

List 8 disease presentations associated with Chorea or Athetosis:

A
  • Juvenile onset Huntington Disease
  • Sydenham’s Chorea
  • Post-Pump chorea (following cardio surg.)
  • CP
  • Kernicterus
  • Infection +/- ADEM
  • Metabolic disorders
  • SLE, hyperthyroidism
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15
Q

What is the most common cause of chorea in childhood?

A

Acute Rheumatic Fever (ARF)–> Sydenham’s chorea

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16
Q

What is the most common age range for Sydenham’s Chorea to occur?

What is the sex distribution?

What causes it?

A
  • Ages 5-15 yoa
  • 2:1 F/M ratio

Occurs avg 4 mos after GAS infection–> ARF (JONES)

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17
Q

How does Sydenham’s Chorea present?

What might you see on imaging?

A

Gradual progressive emotional lability (may begin with school difficulties) –> Choreiform movements–> waxes and wanes for mos w complete resolution

May have T2 increased signal in putamen and GP; will resolve when chorea recovers

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18
Q

What are the antibodies implicated in Sydenham’s chorea (2)

A
  • ASO

- AntiDNAse B

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19
Q

What are treatments for Sydenham’s Chorea? (6)

A
  • DA antagonists (Pimozide, Haloperidol)
  • Antiepileptics (Carbamazepine, Valproate)
  • BDZs
  • Corticosteroids
  • IVIG
  • Penicillin
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20
Q

List 3 examples of secondary dystonia:

A
  • CP
  • Post trauma or stroke
  • Toxin induced
21
Q

What are the three types of dystonias– i.e. how might dystonia present in three ways?

A
  • focal
  • segmental
  • multifocal

**Note that it may fluctuate in severity over time

22
Q

What two things may stop dystonia?

A
  • Sleep: NEVER PRESENT DURING SLEEP

- “Geste antagoniste”: suppressed by touch of a certain body part

23
Q

What two things may worsen dystonia?

A
  • Certain body movements

- Stress/ excitement/ pain

24
Q

What is the most common primary dystonia of childhood?

How does it initially present and how is it treated?

A

Dopa-responsive dystonia

  • Initially presents with foot dystonia
  • Treated well with low dose levodopa
25
Q

Which mutation is associated with Idiopathic Generalized Torsion Dystonia? What is the inheritance pattern?

How does the disease initially present? How long does it take to generalize?

A
  • (AD) DYT1 mutation
  • Begins in lower extremity
  • Generalizes within 5 years
26
Q

Gluteric Acidemia Type 1:

What is the deficient enzyme?
What three aa’s can’t be made?
What is one hallmark feature, and at what age does chorea present?
How do we treat it?

A
  • (AR) Glutaryl-CoA Dehydrogenase
  • Lysine, Hydroxylysine, Tryptophan
  • Presents with macrocephaly at birth, and chorea onset by 1 yoa
  • Treat with carnitine supplementation
27
Q

Wilson’s Disease:

Which gene and inheritance pattern?
What is the problem?
How does the disease manifest (5)?

A
  • (AR) Chromosome 13
  • Defective Cu transporter

Presentation:

  • Dysarthria
  • Dystonia (risus sardonicus)
  • Rigidity
  • Gait disturbance
  • Tremor
28
Q

Dystonia and spasticity often occur in the same affected limb; what is the difference between the two?

What makes them both worse?
What is common amongst all affected muscles?

A

Dystonia: action–can’t completely relax muscle

Spasticity: felt at rest–velocity dependent tone

Both are worse with stress, excitement, pain
Affected muscles are all WEAK

29
Q

List three drug classes used to treat spasticity:

A
  • Muscle relaxants (baclofen, tizanidine) **May surgically implant intrathecally
  • Anticholinergic (trihexylphenidyl)
  • BDZs
30
Q

Why does DBS not work for spasticity?

A

Circuit for “action” is interrupted; therefore it can only be used to treat dystonia

31
Q

DA antagonists (antipsychotic and anti nausea meds) can cause what 4 movement disorders?

A
  • Acute dystonic reaction
  • Tardive Dyskinesia
  • Parkinsonism
  • Neuroleptic malignant syndrome
32
Q

What are the three types of acute dystonic reaction to DA agonists?

What is the treatment?

A

Oculogyric crisis, torticollis, axial dystonia

Tx with IV Benadryl or Benztropine

33
Q

Tics:
What constitutes transient vs. chronic?
What is required for Tourette’s Syndrome dx?

A

Transient tics last less than 1 year; chronic are more than 1 year

Tourettes dx must be under 18 yoa w/ both chronic vocal and motor tics

34
Q

Tics usually begin by what age?
Which come first: motor or vocal?
What is one hallmark feature of tics in general?
What is the best treatment?

A

Begin by 6yoa–> worse by 10 yoa–> Lessen by 18 yoa

Motor tics usually happen first

**Tics change over time

Treat comorbidities first,–CBT and alpha 2 adrenergic blocker (clonidine) (if no comorbidities)

35
Q

How do stereotypic movements differ from tics? (2)

What is the best treatment?

A

Repetitive purposeless movements that DO NOT change over time; may or may not be involuntary

Treat with SSRI, especially for anxiety

36
Q

PANDAS:

What does this mean?
How does it present and at what age?

A

Pediatric Autoimmune Neuropsychiatric Disorders Assocated with Strep (GAS)

Presents following GAS infection w/ pre-pubertal tics and OCD exacerbations; course is episodic

37
Q

Do ABX help tic exacerbations associated with GAS infection?

A

No; treat with ABX to prevent rheumatic fever only

38
Q

What is Hartnup Disease?
Inheritance pattern and chromosome?
What problems does it cause?
How do we treat it?

A

AR, chromosome 5

Defective aa transporter in kidney–> Vit B3 deficiency–> Pellagra, Developmental delay, paroxysmal limb ataxia

Treat with high protein diet

39
Q

What is Maple Syrup Urine Disease?

Inheritance pattern and problem?

How and at what age does it present?
What makes it worse?
How do we treat?

A

AR defective branched chain aa metabolism

Presents by 2 yoa with maple syrup smelling turing and paroxysmal ataxia, lethargy, irritability

Treat by restricting protein and giving thiamine supplements

40
Q

What is defective in Pyruvate Dehydrogenase Deficiency?

How does this disease present?
How do we treat it (2)?

A

E1 component of PDH complex defective–> ^ lactate, ^ pyruvate–> paroxysmal ataxia and lethargy

Treat with acetazolamide and ketogenic diet

41
Q

Episodic Ataxia Type 1:
Mutant gene?
Duration of attacks?
Treatment? (3)

A
  • KCNA1 channel (K+)
  • Brief attacks of ataxia
  • Phenytoin, carbamazepine, acetazolamide
42
Q

Episodic Ataxia Type 2:
Mutant gene?
Duration of attacks?
Treatment (2)?

A
  • CACNA1A channel (Ca++ )
  • Day-long attacks of ataxia
  • Acetazolamide and flunarizine
43
Q

For each type of ataxia, site the location of the lesion?

  1. Truncal
  2. Limbs
  3. Eyes
A
  1. Cerebellar vermis
  2. Hemispheres
  3. Brainstem
44
Q

Infections that can cause ataxia (4)

A

Varicella
HSV
EVB
Mumps

45
Q

Heavy metal poisons that can cause ataxia (3)

A

Mercury
Lead
Thallium

46
Q

Three brain tumors that can cause ataxia w poor prognosis:

A

Meduloblastoma
Ependymoma
Pilocytic astrocytoma

47
Q

Three disease states that can cause vertigo

A
  • acoustic neuroma
  • posterior fossa tumor
  • demyelinating disease
48
Q

Features of peripheral vertigo (4):

A
  • episodic
  • unidirectional
  • +/- hearing loss

**no other neuro sx

49
Q

Features of central vertigo (4):

A
  • constant
  • spinning direction varies
  • NO HEARING LOSS OR TINNITUS
  • CN deficit/ cerebellar disease present

Neuro Clerkship (13 decks)

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