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Peds neuro boards, V2 > Movement D/O and other Paroxysmal Events NOS > Flashcards

Movement D/O and other Paroxysmal Events NOS Flashcards

1
Q

Benign Paroxysmal Vertigo

  1. What does it look like?
  2. Onset and natural history
  3. Family history note
  4. Diagnostic test
  5. Treatment
A
  1. Description
    1. like PPV, but not always positional
    2. Abrupt episodes of unsteadiness / ataxia lasting 1-2 minutes
    3. Appears startled / frighteneed, may run to parent
    4. Assocaited rotary nystagmus, nausea, vomiting, pallor
    5. Consciousness always preserved
  2. Onset
    1. Commonly before age 4, but remit by age 11 (or become migraines)
  3. Family history of migraine is classic
  4. Diagnosis: Dix Hallpike test
  5. Treatment: Epley maneuver
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2
Q

Treatment for Tics (5)

A
  1. A2 - adrenergic agonists
    1. Clonidine
    2. Guanfacine
  2. Neuroleptics
    1. Risperidone
    2. Pimozide
  3. Habit reversal training
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3
Q

Sydenham’s Chorea

  1. Cause
  2. Typical disease association
  3. Treatment
    1. Category 1 (1)
    2. Category 2 (2)
    3. Category 3 (3)
    4. Category 4 (3)
  4. Prognosis
A
  1. Group A beta-hemolytic streptococci
  2. 20-30% of patients with acute rheumatic fever
  3. Treatment
    1. Clonidine
    2. ASM
      1. VPA
      2. CBZ
    3. Neuroleptics
      1. Risperidone
      2. haloperidol
      3. pimozide
    4. Immunosuppresssion
      1. Steroids
      2. IVIG
      3. Plasmapharesis
  4. 50% recover after 2-6 months but some persist for >2 years
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4
Q

Wilson’s Disease

  1. Mutation / chromosome / inheritance
  2. Symptoms (3)
  3. Diagnostic findings (7)
  4. Treatment (7)
A
  1. ATP7B (chromosome 13), autosomal recessive
  2. Symptoms
    1. Liver disease
    2. Neurologic symptoms***
    3. Renal Tubular Acidosis
  3. Diagnostic tests
    1. Abnormal LFT’s
    2. Low ceruloplasmin
    3. Low copper
    4. Neuroimaging (Bilateral T2 hyperintensities of BG and Thalami)
    5. slit lamp exam > Kayser-Fleisher rings
    6. Liver biopsy
  4. Treatment
    1. Diet: avoid mushrooms, nuts, broccoli, chocolate, dried fruit, liver, and shellfish
    2. Zinc
    3. Chelation
      1. Penicillamine
      2. trientine hydrochloride
      3. Tetrahiomolybate
    4. Liver transplant
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5
Q

Paroxysmal Kinesigenic Choreoathetosis

  1. Inheritance
  2. Genes (4)
  3. Characterization of movements
  4. Treatment (2)
  5. Prognosis
A
  1. Autosomal Dominant
  2. Genes
    1. 16p11.2-q12.1
    2. DYT8 2q35
    3. DYT9 1p
    4. 16p12-q12 ICCA
  3. No LoC, tiggered upon initiation of movement
  4. Treatment
    1. Carbamazepine
    2. Benzos
  5. Prognosis
    1. Usually goes away by 18
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6
Q

Hallervorden Spatz

  1. AKA
  2. pathogenesis
  3. Mutation / inheritance
  4. Onset
    1. A
    2. B
    3. C
  5. Finding
    1. Category A (3)
    2. Category B (2)
  6. Prognosis
A

Hallervorden-Spatz

  1. Neurodegeneration with brain iron accumulation (NBIA1)
  2. Mutation in pantothenate kinase gene (PANK2) (20p13-p12.3) AR
  3. Onset
    1. Classic: 1st decade, rapid progresssion
    2. Atypical: 2nd decade, slow progression
    3. Intermiediate:
      1. 1st decade with slow progression
      2. 2nd decade with rapid progression
  4. Findings
    1. Eye:
      1. Pigmentary Retinopathy
      2. retinal degeneration
      3. optic atrophy
    2. Neurologic
      1. Facial grimacing / dysphagia
      2. spasticity
  5. Death 20 years after onset
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7
Q

DYT1

  1. Mutation and inheritance
  2. Onset
  3. Presentation
  4. Epidemiology note
  5. Treatment
A
  1. 9q34 - AD
  2. Onset often mid-to-late childhood but occasionally in adulthood
  3. Wide phenotypic variability and penetrance
  4. High incidence of ashkenazi Jews
  5. Treatment: symptomatic (including DBS
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8
Q

What does this show?

A

Caudate and putamen atrophy

(juvenile huntington’s disease)

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9
Q

4 year old comes in with 2-3 Hz yes-no head movements.

Before diagnosis motor tics, what should you exclude?

A

Dilation of third ventricle

“Bobble-headed-doll syndrome”

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10
Q

Patient with parkinson’s disease Complains of nausea after starting Sinimet

What can you do and why?

A

Increase amount of carbidopa (levodopa in periphery can cause nausea)

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11
Q

pregnant woman develops chreaform movements of arms and legs.

What two treatments can be done for this?

A

Plasma exchange

1st generation antipsychotics (haloperidol is preferred)

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12
Q
A
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13
Q

Mechanism of action: Carbidopa

A

Inhibits dopa decarboxylase (prevents peripheral conversion of Levodopa to Carbidopa)

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14
Q

Friedrich’s ataxia Menmonic + chromosome

A

Mnemonic: Fred told his wife to PUSH, then the baby went “GAA”

GAA repeat in FXN (frataxin) gene on 9q13

  • *P**es cavus, Loss of Proprioception
  • *U**pgoing toes
  • *S**coliosis, Speech problems (dysarthria)
  • *H**yporeflexia
  • *G**ait
  • *A**taxia
  • *A**rrythmias
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15
Q

Region damaged to cause Hemiballismus

A

Contralateral Subthalamic nucleus

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16
Q

Patient presents with exaggerated startle response, nocturnal myoclonus, and stimulus episodes of apnea

what is this called?
what amino acid is typically involved?
what is a possible way to abort these episodes?

A

Hyperekplexia

Glycine

can try to treat by flexing child’s neck or hips

17
Q

Hypokalemic periodic paralysis:

Preventative Dietary changes and Medication

A

Low salt, low carbohydrate diet

Dichlorphenamide

18
Q

Antidepressant preferred for RLS and why?

A

Buproprion, because it doesn’t exacerbate or precipitate RLS

19
Q

How to differentiate between Dopa-responsive dystonia and Paroxysmal exertional dyskinesia

A

DRD = positive response to low-dose Dlevodopa

PED = TRIGGERED by exercise, also with absence seizures and complex partial seizures

20
Q

Difference between Carbidopa and Entacapone

A

Carbidopa = Dopa decarboxylase inhitor

Entacapone = COMPT inhibitor

21
Q

Patient presents with jerking movements after being treated for rheumatic fever

How do you treat?

A

IM penicillin (or 10 days oral penicillin) + haloperidol

22
Q

Patient with wilsons disease treated with penicillamine develops neurologic deterioration and contractures. What do you do?

A

Switch to trientine hydrochloride

23
Q

MRI Finding in CJD

A

increased signal in basal ganglia

24
Q

19 year old man presents with bradykinesia, dystonia, and rigidity, but also to a lesser extent chroeoform movements.

What genetic condition do you suspect?

A

Westphal variant of Huntington’s

25
Q

Patient presents with parkinsonian features and prominent nasal speech

What regions of the brain are affected (5)

What type of disorder is this?

A
  1. Atrophy of:
    1. midbrain
    2. pallidum
    3. thalamus
    4. Subthalamic nucleus
    5. Mild symmtric frontal atrophy as well
  2. What type of disorder?
    1. Tauopathy
26
Q

Histopathologic features of Progressive supranuclear palsy (supercategory and 3 components)

A

Glial tau pathology:

  • tufted astrocytes
  • Thorn shaped astrocytes
  • Oligodendroglial coiled bodies
27
Q

Tests to order before initiating Cabergoline (2) and why?

A

CXR and Echo, due to retroperitoneal, pulmonary, and cardiac fibrosis

28
Q

20 year old male presents with external ophthalmoplegia, ataxia, as well as spasticity, dystonia, athetosis, and dysarthria.

His father and grandmother died of a similar condition.

What disorder to you expect?
What is the mutation?
What exam feature can you use to confirm?

A
  1. Machado-Josheph disease type 1 (aka spinocerebellar ataxia type 1)
  2. Trinucleotide repeat of ATXN3 gene (chromosome 4)
  3. Look for tongue fasciculations
29
Q

50 year old man comes in for second opinion on TMJ. You note frequent blinking and jaw tightening.

What is this and what could you offer as treatment?

A

Meige syndrome (oromandibular dystonia + blepharospasm)

No cure, but botox can help

30
Q

Genes associated with Restless leg syndrome

A

“I’m restless about boards. Beat-the-boards meissed somet hings. I want a map to make sure i’m prepaired”

BTBD9

MEIS1

PTPRD

MAP2K5/LBXCOR

Peds neuro boards, V2 (29 decks)

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